1st CONGRESS of the EUROPEAN YOUNG PAEDIATRICIANS

Transcription

1st CONGRESS of the EUROPEAN YOUNG PAEDIATRICIANS
YOU
N
TPA
1930
NS
IA
G
DI
PAE ATRIC
1st CONGRESS of
the EUROPEAN
YOUNG
PAEDIATRICIANS’
ASSOCIATION
Abstract Book & Congress Information
Wyndham Grand Levent Hotel
4 - 6 December 2015
Istanbul/TURKEY
CONTENTS
WELCOME.................................................................................2
COMMITTEES............................................................................3
SPEAKERS AND MODERATORS ....................................................4
LOCAL INFORMATION..............................................................5-6
USEFUL TURKISH EXPRESSIONS..................................................7
CONGRESS INFORMATION..........................................................8
SOCIAL PROGRAM.....................................................................8
VENUE PLAN.............................................................................9
OUR SUPPORTERS................................................................... 10
SCIENTIFIC PROGRAM......................................................... 11-16
ABSTRACTS .................................................................... 17-128
INDEX.................................................................................. 129
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Dear Friends and Colleagues,
EURYPA (European Young Paediatricians’ Association) is founded in January 2015 primarily by the
Turkish Young Paediatricians and ONSP (Italian National Observatory for Paediatric Residents).
Our main aim is to facilitate networking among paediatricians all over Europe and support them
on their quest for better education during and post-specialization, research opportunities.
We’re delighted to welcome you to the 1st ever Congress of EURYPA where newly specialized
paediatricians and paediatric residents will meet, present their interesting cases with an
emphasis on the main topic supported by their senior doctors. There will be workshops about
the problems we’re facing and the future projects to tackle them as well.
No need to say, there will also be a rich social programme for us to get to know each other in
the heart of Istanbul.
EURYPA 1st Congress Organizing Committee
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EURYPA 1st CONGRESS
ORGANIZING COMMITTEE
EPA-UNEPSA
COUNCIL
SERHAT GÜLER
DAVIDE VECCHIO
MİNE ÖZDİL
SALVATORE AVERSA
ADEM KARBUZ
PINAR ÜRENDEN ELİÇİN
PAOLA BERLESE
ERTUĞRUL KIYKIM
ROBERTO RASCHETTI
LEYLA NAMAZOVA-BARANOVA
President
MASSIMO PETTOELLO-MANTOVANI
Secretary General
JULIJE MESTROVİC
Vice President
MANUEL MOYA
Vice President
GİZEM PAMUK
ZEYNEP İŞÇAN
TUĞÇE KALAYCI ORAL
ÇİĞDEM ORUÇ
AYŞE AYZIT KILIÇ
SERAP SAPMAZ
SEVİNÇ GÜMÜŞ
ZEYNEP ALP ÜNKAR
ÜMMÜHAN ÖNCÜL
BURÇİN NAZLI KARACABEY
TUBA KOÇKAR
ELİF ARSLAN
GULNARA HEYDAROVA
ADA BULUT
DORUK GÜL
ZEYNEP KARACA
PINAR YAMAÇ
ENSAR DURAS
MELDA TAŞ
NAZLI KAVCIK
AYŞE PEHLEVAN
HATİCE EREN
MUSTAFA ÖZDEMİR
ERKAN ERFİDAN
REFİA GÖZDENUR SAVCI
FÜGEN ÇULLU ÇOKUĞRAŞ
Councilor
SIMON LENTON
Councilor
JOCHEN EHRICH
Treasurer
ANDREAS KONSTANTOPOULOS
Past President
MARIN BURLEA
President of 8th Europaediatrics
MEHMET VURAL
President of Balkan Pediatrics Association
Secretary of TPA
GENERAL SECRETARY OF EURYPA 1st CONGRESS
ÖMER FARUK BEŞER
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SPEAKERS AND MODERATORS
SPEAKERS AND MODERATORS FROM TURKEY
Ahmet Arvas
Ada Bulut Sinoplu
Adem Karbuz
Aysun Karabay Bayazıt
Ayşe Ayzıt Atabek
Ayşe Güler Eroğlu
Beyhan Tüysüz
Burak Doğangün
Burçin Nazlı Karacabey
Bülent Hacıhamdioğlu
Çiğdem Oruç
Diğdem Bezen
Elif Arslan
Emel Gür
Emin Ünüvar
Emine Olcay Yasa
Engin Tutar
Ergin Çiftçi
Ertuğrul Kıykım
Esra Özek
Fatih Varol
Fatma İrioğlu Kocael
Fuat Gürkan
Gizem Pamuk
Gözde Apaydın
Gözde Yücel
Gülnara Heydarova
Hacer Ergin
Haluk Çokuğraş
Hasret Ayyıldız Civan
Hatice Öztürk
Hilmi Apak
Hüray Kök
Hüseyin Kılıç
Kenan Barut
Levent Saltık
M.Tayyib Kadak
Mehmet Satar
Mehmet Vural
Melike İrem Petan
Merve Erdemir Kula
Mine Özdil
Mustafa Akçam
Mustafa Kemal Özdemir
Müjgan Alikaşifoğlu
Münevver Kaynak Türkmen
Nafiye Urgancı
Nazlı Kavçık
Nihat Sapan
Nilay Güneş
Olcay Evliyaoğlu
Oya Ercan
Ozan Özkaya
Ömer Faruk Beşer Övgü Kul Çınar
Özden Aksu Sayman
Özgür Kasapçopur
Pınar Yamaç Dilaver
Raşit Vural Yağcı
Refia Gözdenur Savcı
Salim Çalışkan
Sebuh Kuruğoğlu
Sema Saltık
Serhat Güler
Sezgin Şahin
Sultan Kavuncuoğlu
Süheyla Ocak
Şükrü Çekiç
Tiraje Celkan
Tuba Koçkar
Tuğba Erener Ercan
Tülay Erkan
Ümmühan Öncül
Vildan Ertekin
Yıldız Perk
Yücel Taştan
Zeynep İşçan
Zeynep Tamay
INTERNATIONAL SPEAKERS AND MODERATORS
Alma Topi
Ana Skelin
Ana Stefanescu
Ancuta Ignat
Angelica Cristina Marin
Bendavid Matthieu
Davide Vecchio
Evda Vevecka
Jelena Sabljic
Julia Jillich
Julije Mestrovic
Kerbl Reinhold
Manuel Ferreira-Magalhaes
Margareta Seher Zupancic
Marin Burlea
Massimo Pettoello Mantovani
Norbert Varga
Paola Berlese
-4-
Rita Lónyai
Roberto Raschetti
Sara Gorgoglione
Sebastian Grey
Sharat Varma
Tatjana Ardalic
Tudor L Pop
Ulrike Wanz
Vasile Valeriu Lupu
Zsuzsanna Almassy
LOCAL INFORMATION
Hotel Name
Wyndham Grand Levent Hotel
Hotel Address
Esentepe Mah. Büyükdere Cad. No:177-183
34394 Şişli – İstanbul
Getting Around
Istanbul has an extensive public transport network with its highway, rail and funicular systems
and sea transport services. The easiest way to reach the Congress Venue, is the take the metro
line and use station “ Levent”.
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Transportation Cards
The city has an integrated electronic ticket system which uses smart RFID cards called
İstanbulKart. It’s a contactless smart card used at public transport in the city and can be
acquired at major transport interchanges. The main benefit of İstanbulKart is to save time
and money while travelling. The easy to use and easy to obtain reasonably priced İstanbulKart
may be used in any public transportation throughout the city eith the benefit of cheaper rates
compared to buying a ticket or token. Also, the card can be loaded at special machines or some
kiosques which can be found almost in every main bus stations and ferry stations.
Weather in Istanbul
Current forecast show warmer than usual temperature,
however be ready for cold weather and occasional rain
during congress dates.
Banking and Exchange Facilities
Currency is the Turkish Lira. Foreign money can be changed
by banks during working days (09.00- 17.00 Monday Friday) as well as hotels, exchange offices. Exchange rates
are set daily by the Central Bank. All major credit cards
are accepted in most of the hotels, restaurants and shops.
Automated bank machines are available at many points
throughout the city and the airport.
Shopping
One of the most enjoyable parts of a trip to Turkey is shopping for the rich variety of Turkish
crafts. Telephones
Pay phones and credit card phones are available at the meeting venue as well as in the city.
Electricity
The electrical power supply in Turkey is 220 volts.
Tipping
Although service charge is included in most restaurants, leaving a tip at one’s own discretion
is appreciated.
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USEFUL TURKISH EXPRESSIONS
English
Turkish
Pronunciation
Hello
Merhaba!
MARE-HA-BA see above
Good evening
İyi akşamlar!
EE AK-SHAM-LAR
Hoşçakal!
HOSH-CHA-KAL
Güle güle!
GUU-LEH GUU-LEH
How are you?
Nasılsın?
NA-SEL-SIN
I am fine, and you?
İyiyim, sen nasılsın?
EE-IM, SEN NA-SEL-SIN
Thank you
Teşekkür ederim.
TE-SH-QU-ERR ED-ERR-IM
Good-bye (said by person
leaving)
Good-bye (said by person
staying)
You’re welcome
Birşey değil. (in response to
“Thank you”)
BEER-SHEY DEY-EEL
Please
Lütfen.
LUT-FEN
I don’t speak Turkish
Türkçe bilmiyorum!
TURK-JEH BILL-MI-YOR-UM
I don’t understand
Sizi anlamıyorum.
SI-ZI ANN-LA-MA-YOR-UM
Could you please repeat
Şunu tekrar edebilir misiniz?
SHU-NU TEK-RA ED-EBB-ILL-EAR MISS-IN-IZ
Tanıştığımıza sevindim./Memnum
TAN-ISH-TI-IM-IZA SEV-IN-DIM/ MEM-NUM-
oldum
OLL-DUM
How much does it cost?
Ne kadar?
NAY KAD-AR
I am sorry
Özür dilerim.
OUZ-UR DILL-EAR-IM
Excuse me
Afedersiniz.
AFF-ED-DAR-SAN-IZ
Can you help me?
Yardım edebilir misiniz?
YARR-DIM ED-EBB-ILL-EAR MISS-IN-IZ
Yes
Evet.
EV-ET
No
Hayır.
HI-EAR
I don’t know
Bilmiyorum.
BILL-ME-YOR-UM
Okay/Alright
Tamam.
TAM-AM
Welcome
Hoş Geldiniz.
HOSH GEL-DIN-IZ
Pleased to meet you
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CONGRESS INFORMATION
Certification of Attendance
All congress participants will be issued a certification of attendance. You can collect your
certificates from the registration desk from December 06th, 2015.
Name Badge
All participants and accompanying people are kindly advised to wear their name badges at all
times during the congress. If you lose your name badge, please apply to registration desk.
Poster Exhibition
Poster area is located in the Hezarfen Ahmet Celebi Hall. All poster presenters are expected to
be with their posters 6 December 2015, between 18.00-19.00 for poster evaluation.
Congress Registration Desk
Congress registration desk will be at the entrance of the congress center and will be open
from 07:00 to 19:00 during congress dates for giving information about registration and
accommodation.
SOCIAL PROGRAM
4 December 2015 – Wyndham Grand Hotel
18.00-22.00 Opening Ceremony
- Violin and Piano Performance, by Müge Sak and Zümrüt Alieva
- Opening Remarks
- Jazz Concert and Dance Party- Ezgi Ayça Kızıldere
5 December 2015 – Wyndham Grand Hotel
19.00-22.00 Gala Dinner
- Concert: Demet Tuncer : Demet Tuncer started her musical career in Las Vegas and
Santa Fe with Austin Miskel and Andre Garcia Nuthman. Demet also had the privilege
of working with the current Dean of Drama and Dance at the University of Houston,
Steve Wallace, who also pioneered an academic play ‘Prymates’, in Broadway.
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WE GRATEFULLY THANK TO OUR SPONSORS FOR THEIR VALUABLE
SUPPORT FOR THE 1ST CONGRESS OF EURYPA
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04 Aralık 2015, Cuma
SAAT
PEDİATRİK
RADYOLOJİ KURSU
ÇOCUK
KARDİYOLOJİ
KURSU
BESLENME KURSU
SAĞLAM ÇOCUK
İZLEMİ KURSU
YENİDOĞAN KURSU
09.00
10.00
Pediatrik Toraks
Radyolojisi
Sebuh Kuruğoğlu
Üfürümü Olan
Çocuğa Yaklaşım
Ayşe Güler Eroğlu
Çocukluk Çağında
Besin İhtiyaçları ve
Malnütrisyonun
Tanınması
Engin Tutar
Sağlam Çocuk
İzlemi
Emel Gür
Olgularla
Yenidoğanda
Mekanik Ventilasyon
Mehmet Vural
Çocukluk Çağında
Aşılanma
Ahmet Arvas
Sarılığa Güncel
Yaklaşım
Yıldız Perk
Adölesan İzlemi
Müjgan Alikaşifoğlu
Prematüre Bebeğin
Parenteral-Enteral
Beslenmesi
Sultan Kavuncuoğlu
10:00
10:15
10:15
11:15
KAHVE ARASI
Pediatrik
Nöroradyoloji
Sebuh Kuruğoğlu
Telekardiyografinin
Değerlendirmesi
Ayşe Güler Eroğlu
11:15
11:30
11:30
12:30
KAHVE ARASI
Pediatrik Batın
Radyolojisi
Sebuh Kuruğoğlu
Elektrokardiyografinin Değerlendirmesi
Ayşe Güler Eroğlu
12.30
13.30
13.30
15.00
Malnütrisyon
Tedavisinde Enteral
Beslenme
Vildan Ertekin
Malnütrisyon
Tedavisinde
Parenteral
Beslenme
Nafiye Urgancı
ÖĞLE YEMEĞİ
13.30-14.15 Kistik Fibroz; Beslenmede Güncel Yaklaşımlar ve Öneriler
Fügen Çullu Çokuğraş
Gülnara Heydarova
Ömer Faruk Beşer 14.15-15.00 Kistik Fibroz; Akciğer Tutulumunda Koruyucu Önlemler ve Tedavide Güncel Yaklaşımlar
Haluk Çokuğraş
Ayşe Ayzıt Atabek
Tuba Koçkar 15.00
15.30
KAHVE ARASI UYDU SEMPOZYUMU
15.30
16.30
16.30
18.00
18.00
19.00
19.00
21.30
Erken Dönem Beslenmenin Önemi ve Flora Gelişimine Etkisi
Mehmet Vural
Raşit Vural Yağcı
16.30-17.15 Demir Eksikliği Anemisi
Hilmi Apak
Nazlı Kavçık
Hüray Kök
17.15-18.00 Lenfadenopatili Çocuğa Yaklaşım
Tiraje Celkan
Süheyla Ocak
Gözde Yücel
AÇILIŞ TÖRENİ ve MÜZİK DİNLETİSİ (Müge Sak, Zümrüt Alieva)
JAZZ KONSERİ ve DANS PARTİSİ (Ezgi Ayça Kızıldere)
- 11 -
05 Aralık 2015, Cumartesi
SAAT
PROF. DR. SEZAİ BEDRETTİN TÜMAY SALONU
PROF. DR. ŞÜKRAN YALÇINDAĞ SALONU
09.00
10.30
09.00-09.45 Çocuklarda Boy Kısalıkları
Oya Ercan
Ada Bulut Sinoplu
Övgü Kul Çınar
09.45-10.30 Erken Püberte
Olcay Evliyaoğlu
Diğdem Bezen
Hatice Öztürk
09.00-09.45 Ateşli Hastaya Yaklaşım
Yücel Taştan
Çiğdem Oruç
Fatih Varol
09.45-10.30 Çocuklarda Antibiyotik Kullanımında
Doğrular-Yanlışlar - AKILCI İLAÇ KULLANIMI OTURUMU
Emin Ünüvar
Adem Karbuz
Ümmühan Öncül
10.30
11.00
11.00
12.00
KAHVE ARASI
Aşılar Hakkında Kanıta Dayalı Bilimsel Yaklaşımlar
Ahmet Arvas
Aşı takviminde uygulanan aşılar
Gizem Pamuk
Yeni aşılar
Gözde Apaydın
UYDU SEMPOZYUMU
(Besin Alerjileri; Güncel Tedavi Yaklaşımları)
Haluk Çokuğraş
Ömer Faruk Beşer
12.00
13.00
13.0014.00
14.00
15.00
15.00
16.00
16.00
16.30
Hangi Çocukta Metabolik Hastalık Düşünelim?
Ertuğrul Kıykım
Melike İrem Petan
Refia Gözdenur Savcı
Özden Aksu Sayman
UYDU SEMPOZYUMU
(Nörolojik Hastalarda Malnütrisyon ve Tedavi Yaklaşımları)
Tülay Erkan
Serhat Güler
Yenidoğan Solunum Problemlerine Güncel Yaklaşımlar
Mehmet Satar
Mine Özdil
Zeynep İşçan
Genetik; Depo hastalıklarında Güncel Tanı-Tedavi
Yaklaşımları
Beyhan Tüysüz
Nilay Güneş
Ertuğrul Kıykım
KAHVE ARASI
- 12 -
05 Aralık 2015, Cumartesi
SAAT
PROF. DR. SEZAİ BEDRETTİN TÜMAY SALONU
PROF. DR. ŞÜKRAN YALÇINDAĞ SALONU
16.30
17.30
Probiyotiklerin hayatımızda ve güncel tıptaki yeri
Raşit Vural Yağcı
Akut ishalde Probiyotikler
Adem Karbuz
Alerjik hastalıklarda Probiyotikler
Pınar Yamaç Dilaver
Fonksiyonel Sindirim Sistemi Hastalıklarında
Probiyotikler
Merve Erdemir Kula
Enflamatuar Barsak Hastalıklarında Probiyotikler
Hasret Ayyıldız Civan
Febril Konvülsiyon: Yaklaşım Ve Tedavide Yenilikler
Sema Saltık
Hüseyin Kılıç
Burçin Nazlı Karacabey
Alerjik Cilt Hastalıkları
Zeynep Tamay
Esra Özek
17.30
18.00
18.00
19.00
POSTER
20.00
22.00
GALA YEMEĞİ
KONSER: DEMET TUNCER
06 Aralık 2015, Pazar
SAAT
PROF. DR. SEZAİ BEDRETTİN TÜMAY SALONU
09.00
10.30
09.00-09.45 Çocuk Psikiatrisinde Gelişimsel Bozukluklar
Burak Doğangün
M.Tayyib Kadak
09.45-10.30 Solunum yolu alerjileri; Güncel Tedavi ve Önlemler
Nihat Sapan
Fatma İrioğlu Kocael
Şükrü Çekiç
10.30
11.00
11.00
12.30
KAHVE ARASI
11.00-11.45 Çocuklarda Otoenflamatuar Hastalıklar
Özgür Kasapçopur
Sezgin Şahin
Kenan Barut
11.45-12.30 Tekrarlayan İdrar Yolu Enfeksiyonlarında Güncel Yaklaşımlar
Salim Çalışkan
Elif Arslan
Mustafa Kemal Özdemir
12.30
13.30
ÖĞLE YEMEĞİ
13.30
KAPANIŞ
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04 December 2015, Friday
TIME
PROF. DR. ÖZDEMİR İLTER HALL
09.00
10.30
COURSE
10.30
11.00
COFFEE BREAK
11.00
12.30
COURSE
12.30
13.30
LUNCH
14.15
15.00
“Aspects of Gastroesophageal Pathology in Children”
Moderator: Marin Burlea
• Particularities of the Gastroesophageal Reflux Disease in Childhood”
Vasile Valeriu Lupu • Gastritis in Childhood”
Ancuta Ignat
• Helicobacter Pylori Infection in Children”
Angelica Cristina Marin
15.00
15.30
COFFEE BREAK
SATELLITE SYMPOSIUM *
15.30
16.30
16.30
17.30
Importance of Early Life Nutrition and Effect on Development of Microbiota
Mehmet Vural
Raşit Vural Yağcı
“Paediatric/neonatal emergencies”
Moderator: Julije Mestrovic
• “Coma, case presentation” Tatjana Ardalic
• “Apnoea, case presentation”
Ana Skelin
• “Dyspnoea, case presentation”
Jelena Sabljic
18.0019.00
OPENING CEREMONY and CONCERT (Müge Sak, Zümrüt Alieva)
19.0021.30
JAZZ CONCERT and DANCE PARTY (Ezgi Ayça Kızıldere)
*The symposium will be Turkish, but simultaneous translation will be available in English.
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05 December 2015, Saturday
TIME
09.00
10.30
PROF. DR. ÖZDEMİR İLTER HALL
09.00-09.45 “Sudden Infant Death Syndrome”
Moderator: Kerbl Reinhold
• “Epidemiology and pathogenesis”
Julia Jillich
• “Risk factors and prevention”
Ulrike Wanz
09.45-10.30 “EURYPA Executive Commitee and Board Meeting”
Moderator:
Pınar Ürenden Eliçin
Davide Vecchio
Mine Özdil
Sebastian Grey
10.30
11.00
11.00
12.00
12.00
13.00
COFFEE BREAK
EURYPA Executive Commitee and Board Meeting
Moderator:
Pınar Ürenden Eliçin
Davide Vecchio
Mine Özdil
Sebastian Grey
SATELLITE SYMPOSIUM *
Food allergies; Current Treatments
Haluk Çokuğraş
Ömer Faruk Beşer
13.00
14.00
14.00
15.00
15.00
16.00
LUNCH
SATELLITE SYMPOSIUM *
Malnutrition in neurological diseases
Tülay Erkan
Serhat Güler
“Ins and Outs of the New Neonatal Resuscitation Guidelines”
Moderators: Massimo Pettoello Mantovani and Mehmet Vural
• “Interactive and multimedial case reports”
Davide Vecchio
Paola Berlese
Roberto Raschetti
• Lecturer: Davide Vecchio
• Commentator: Mehmet Vural
16.00
16.30
16.30
17.30
COFFEE BREAK
“Career Planning for Young Pediatricians”
Moderator: Massimo Pettoello-Montovani
(Round table session)
17.30
18.30
POSTER SESSION
20.00
22.00
GALA DINNER-CONCERT: DEMET TUNCER
*The symposium will be Turkish, but simultaneous translation will be available in English.
- 15 -
06 December 2015, Sunday
TIME
09.00
10.30
PROF. DR. ÖZDEMİR İLTER HALL
09.00-09.45 “Supplementation of vitamins and micronutrients in infants”
Moderator: Fügen Çullu Çokuğraş
• “D vitamin supplementation and prophylaxis”
Zeynep Hızlı
• “Iron supplementation and prophylaxis”
Erkan Erfidan
• “Supplementation of the other micronutrients”
Zeynep Alp Ünkar
09.45-10.30 “Pediatric hepatology”
Moderator: Tudor Pop
• “Non-invasive evaluation of the fibrosis in children”
Ana Stefanescu
• “Indications of liver transplantation in children”
Sharat Varma
10.30
11.00
11.00
12.30
COFFEE BREAK
11.00-11.45 ‘’Metabolic diseases in children’’
Moderator: Zsuzsanna Almassy
• “’A Rare Cause Behind Developmental Regression in Infancy”
Rita Lónyai
• “’The Presence of Hunter Sydrome in a Family”
Norbert Varga
11.45-12.30 “Acute respiratory disease in infancy; last evidence”
Moderator: Ergin Çiftçi
• Bordetella pertussis infection in a 1 month old infant, with respiratory failure
Manuel Ferreira-Magalhaes
• High flow nasal canula treatment in bronchiolitis
Sebastian Gray
12.30
13.30
LUNCH
13.30
CLOSING CEREMONY
- 16 -
ABSTRACTS
- 17 -
[P-001]
[P-002]
G. V. Volynets, T. A. Skvortsova, E. L. Semikina, A. N.
Surkov, A. V. Nikitin, A. O. Anushenko
Gastroenterology and Hepatology Department,
Scientific Centre of Children Health of the Ministry of
Health of the Russian Federation, Moscow, Russian
Federation
Background&Aims: The aim of the study was to
determine the predictors of sustained virologic response
(SVR) to interferon alpha-2b plus ribavirin therapy in
chronic hepatitis C children.
Fatih Çelmeli1, Abdullah Kocabaş2, A.ishak Işık3, Mesut
Parlak4, Serdar Ceylaner5
1
Department of Pediatric Allergy-Immunology, Antalya
Education and Research Hospital, Antalya, Turkey
2
Department of Pediatric Cardiology, Antalya Education
and Research Hospital, Antalya, Turkey
3
Department of Pediatric Gastroenterology, Antalya
Education and Research Hospital, Antalya, Turkey
4
Department of Pediatric Endocrinology, Antalya
Education and Research Hospital, Antalya, Turkey
5
İntergen Genetic Diseases Diagnostic Center, Ankara,
Turkey
A study of best treatment predictors of
sustained virologic response to interferon
alpha-2b plus ribavirin therapy in children
with chronic hepatitis C
Methods: 29 children and adolescents (mean age 9.1±0.8
years) were chronically infected with HCV genotype
1 (58.6%) and HCV genotype 2 or 3 (41.4%). All
the patients were treated with PEG-IFN alfa-2b (60
microg/m(2)/week) plus RBV (15 mg/kg/day). The
prediction performance was assessed using receiver
operating characteristics (ROC), correlation analyse
of various clinical, laboratory and anthropometric
parameters.
Results: The following pre-treatment predictors of SVR
showed a good correlation: age (r=0.429, p=0.02),
weight (r=0.85, p=0.000) and stature (r=0.966,
p=0.000). Children with weight >23 kg (AUC=0.834),
older than 6 yo (AUC=0.808), higher than 115 cm
(AUC=0.828) were more likely to attain SVR. Other
predictors of SVR were absolute
lymphocyte count >2.5*109/L (R=0.506, p=0.05),
absolute
neutrophil count 2-3.4*109/L (R=0.561, p=0.002)
and serum interferon level 16-22 U/ml (R=0.669,
p=0.000).
Conclusions: The identified factors significantly predict
SVR to PEG-INF and ribavirin on pretreatment stage.
Success rate of treatment based on these predictors
reaches up to 83% regardless of HCV genotype.
Keywords: HCV, predictors, SVR
Unexplained cyanosis revealing
hepatopulmonary syndrome in a girl with
APECED syndrome caused by a novel AIRE
mutation
Background&Aims: Autoimmune polyendocrinopathy
type 1, also called autoimmune polyendocrinopathy
candidiasis ectodermal dystrophy (APECED) is a rare
primary immunodeficiency disease caused by lossof-functions mutations in the AutoImmune REgulator
(AIRE) gene on chromosome 21q22.3. AIRE gene is
an important mediator of tolerance to self-antigens.
The clinical spectrum of the disease is characterized
by a wide heterogeneity because of the highly variable
pattern of destructive autoimmune reactions toward
different endocrine and non-endocrine organs.
Results: A 17-year-old girl was referred to our hospital
with complaints of cyanosis and dyspnea. Peripheral
oxygen saturation was 73%. Also, she had diffuse
oropharyngeal moniliasis, skin hyperpigmentation,
central cyanosis and digital clubbing. She was following
by pediatric endocrinology department with the diagnoses
of Addison’s disease, hypoparathyroidism, and hyper
gonadotropic hypogonadism. She had recurrent chronic
mucocutaneous candidiasis since 6 years of age and pangastritis. Her clinical manifestations were consistent with
APECED, which was later confirmed by the identification
of a novel homozygous mutation p.R15H (c.44G>A) in the
AIRE gene. The blood gase assessment revealed PaO2:
55.8 mmHg, PaCO2: 21.7 mmHg, Sat O2: 88 %. CT
revealed nodular contour and heterogeneity of the liver.
The patient experienced upper gastrointestinal bleeding,
and esophagoduedenoscopy showed esophageal varices
and candiasis. The portal venous Doppler showed a dilated
portal vein with a large thrombus. Liver biopsy revealed
autoimmune hepatitis which is a rare manifestation of
APECED Syndrome. She had prominent platypnea and
orthodeoxia. The contrast-enhanced echocardiography
with agitated saline was compatible with an
intrapulmonary right-to-left shunt after 4th cardiac cycle,
thus the diagnosis hepatopulmonary syndrome (HPS) was
confirmed in the patient.
Conclusions: Herein, we reported a girl with a novel
mutation in the AIRE gene, and complicated by HPS due
to autoimmune hepatitis and portal hypertension. To the
best of our knowledge, HPS has not been reported as a
manifestation of APECED Syndrome previously.
Keywords: APACED, hepatopulmonary syndrome,
novel mutation
- 18 -
[P-003]
present this in the following case study.
Articular Involvement in Childhood Familial
Mediterranean Fever
Kenan Barut1, Ada Bulut Sinoplu2, Gözde Yücel2,
Gizem Pamuk2, Amra Adrovic1, Sezgin Şahin1, Özgür
Kasapçopur1
1
Istanbul University, Cerrahpasa Medical Faculty,
Department of Pediatric Rheumatology
2
Istanbul University, Cerrahpasa Medical Faculty,
Department of Pediatrics
Introduction: FMF is an autosomal recessively inherited
autoinflammatory disease which is clinically manifested
with periodic episodes of fever, serositis and arthritis.
Articular involvement is also a frequent presentation
after fever and peritonitis in childhood FMF.
Objective: The aim of this study is to evaluate the
demographic and clinical features of the common
articular findings in the course of FMF and compare these
with the relatively rare occasions of chronic arthritis and
FMF comorbidity.
Patients and Results: Among the 708 patients
diagnosed with FMF; arthritis was defined in 288
(40,7%) cases. In 192 (66%) children the affected joints
were observed primarily as the ankles, subsequently
followed by knees in 148 (51%) cases. Articular findings
were mainly acute monoarthritis without leading to
sequelae. When the children presented with arthritis
were considered, the mean age at disease onset was
estimated to be 5,2 ± 3,7 years and the mean age
at diagnosis was 8,1±4 years. The mean duration of
the episodes of FMF mainly manifested by articular
symptoms was yielded as 97 ± 53,6 hours (with the
median of 72 hours,ranging between 24-168 hours) and
when compared with the cases primarily manifested
with abdominal pain and fever that was declared to be
59,4±34,3 hours in a former study. In 23 (8%) cases
with the diagnosis of FMF and articular manifestations,a
coincidental diagnosis of chronic arthritis was considered
to be more consistent as the disease progressed.
Conclusion: Articular involvement in FMF is acute
monoarthritis typically affecting the lower extremities
in a periodic manner with short duration and without
sequelae formation. The clinical entity of chronic arthritis
and FMF coincidence can emerge as a rare occasion.
Especially with the cases of oligoarticular JIA that
show recurrence and quickly respond to treatment, the
diagnosis of FMF should also be kept in mind.
Keywords: arthritis, familial mediterranean fever
[P-004]
Case report of a dystrophic child
Ádám Schmidt, Margit Lörincz
Heim Pál Children’s Hospital, Gastroenterology
Department, Budapest, Hungary
Background: It seems so, that the differential diagnosis
of gluten-sensitive enteropathy can be still difficult.
The illness can be so progressive that it can be close
to coeliac crisis at the time it is diagnosed. I would like
Case: A 20,5 month-old fatigue child was brought to the
hospital’s Emergency Unit with the following symptoms:
chronic diarrhoea, meteoristic belly, sleepiness. His GP
was following the child’s case since he was 6 month
old because of dystrophy, but there was no previous
examination. Suspicion of Malabsorption syndrome
indicated hospitalisation.
Examinations shown hypoproteinaemia,
hypalbuminaemia, iron deficiency anaemia. Endoscopy
was indicated.
Results: The critical symptoms were explained when the
child was diagnosed with severe coeliac disease. It is still
not clear what caused the flattening of his weight percentile
before the exposition of gluten and what were the causes
of the late diagnoses of this rather frequent disease.
The health of the child improved rapidly with the coeliac
diet. The follow-up showed that beside the coeliac disease
there was another malabsorption disease present in the
child’s system, which we could not identify.
Conclusions: It is necessary to be aware that coeliac
disease is frequent and it must be diagnosed in time.
There is also the possibility that it appears in conjunction
with other illnesses.
Keywords: Gastroenterology, Coeliac disease,
Dystrophy
[P-005]
A Well Known, But Less Diagnosed Disease:
Pharyngoconjuntival Fever
Adem Karbuz, Mustafa Kemal Özdemir, Alper Kaçar,
Vefik Arıca
Okmeydanı Eğitim ve Araştırma Hastanesi, Çocuk
Kliniği, İstanbul, Türkiye
Introduction: Pharyngoconjuntival fever is a common
syndrome, include benign follicular conjunctivitis,
pharyngitis, fever and cervical adenitis caused by
Adenovirus serotype 3 and 7. It typically involves
bilateral eyes. This syndrome generally self-limited
and seen in pediatric population sporadically.
In our case report, we would like to remember that a
child with pharyngitis, non-purulent conjunctivitis and
continuous fever unresponsive to antibiotic therapy is
not necessarily sign of a serious infection.
Case: 2.5 years old boy apply to emergency service
with high fever, redness in eyes and cough. Reddening
of the eyes began 6 days ago in left eye. Family doctor
prescribed an antibacterial eye drop, 2 days later
symptoms were aggravated, eyes became red, and body
temperature was raised. Patient applied to emergency
service, doctor prescribed amoxicillin & clavulanic
acid for upper respiratory tract infections. Patient was
experienced fever within 2 days, then patient applied
to emergency service secondarily. Parenteral cefuroxim
treatment was begun, but fever continued. Patient
was hospitalized because has got fever unresponsive
to oral and parenteral antibiotic treatment, bilateral
conjunctivitis for 4 days. In physical examination, he has
bilateral serous conjunctivitis, oropharyngeal hyperemia,
- 19 -
tonsillar hypertrophy and multiple jugulodigastric and
anterior cervical lymphadenopathies up to 1,5*1 cm
in diameter. The other part of body is normal. Patient’s
laboratory findings are; white blood cells: 8700/mm3,
hemoglobin level: 10,9 g/dl, platelet count: 277000/
mm3, C-reactive protein: 70 mg/l. Other blood
biochemical levels are normal. Adenovirus PCR test is
positive in nasopharynxgeal aspiration specimen. We
have started intavenous fluid treatment and stopped
antibiotic treatment. We observed the patient, symptoms
are relieved, was not experience fever and, conjunctivitis
was healed after 2 days hospitalization.
Keywords: Pharyngoconjunctival Fever, adenovirus
[P-006]
Premature Baby Vaccination; Could It Be
Done On Time?
Adil Umut Zübarioğlu, Mesut Dursun, Sinan Uslu, Ali
Bülbül, Duygu Besnili Acar, Evrim Kıray Baş, Ebru
Türkoğlu Ünal
Department of Neonatology, Sisli Hamidiye Etfal
Education and Research Hospital, Istanbul, Turkey
Background&Aims: In this study the aim is to
investigate vaccination status during hospitalization and
factors that effects this situation in babies followed in
outpatient clinics.
Methods: The study is cross-sectional; all babies
admitted in high risk newborn outpatient clinic between
October 2013 to end of March 2014 and October 2014
to end of March 2015 (palivizumab prophylaxis session)
included. Babies divided in 2 groups according to hospital
that gave care. Group 1 was our hospital, Group 2 was
external center. Demographic features, hospitalization
time, gestational weeks, weight and vaccination status
at discharge recorded.
Results: One hundred thirty eight babies included in
study whose all information could be taken. Sixty-seven
babies (48.5 %) were in group 1. Mean gestational week
was 27.4±1.8 weeks in group 1 and 28.5±1.9 weeks
in group 2. Mean birthweight was 1085.2±268 grams
in group 1 and 1216.8±415.7 grams in group 2. There
were no statistical significance in demographic features
between groups. First dose hepatitis B vaccine was
delayed in 14 (10%) babies. From these babies 12 of
them were in group 2. Reasons for delay were clinical
instability in 6 babies, transfer to outpatient follow-up
in 5 babies, neglect of family in 3 babies. First dose
combination vaccine was delayed in 63 (45.6%) babies.
Reasons for delay were clinical instability in 31 babies,
doctor’s not recommended in 16 babies, neglect of
family in 10 babies and fever of baby in 6 cases. Thirtysix percent of babies delayed in group 1 and 54.9 % of
babies delayed in group 2.
Conclusions: Healthy premature and low birthweight
newborns should be vaccinated according to vaccination
schedule at recommended chronological age for term
babies. However in daily practice, these proposal can
not be fully achieved. We suggest that with educational
programs to care givers and families vaccination rates
can be increased.
Keywords: premature baby, vaccination
[P-007]
Introduction of an infant’s feeding disorder
through a case study
Ágnes Rita Martonosi, Éva Németh, Noémi Scheuring,
László Szabó
Heim Pal Children Hospital
Background&Aims: Beside curing the somatic
symptoms of patients with chronic diseases, leading a
psychological support and a mental care to their families
also play a major role during the healing procedure.
Methods: Through a follow-up method we are
introducing a treatment of an infant called Barnabas with
multiplex congenital limb malformation having a cardiac
surgery and feeding with nasogastric tube (NGT). While
stabilizing his status we reached the exclusively oral
feeding by paying attention not only to his physical but
also his mental health condition.
Results: After having multiplied heart failures caused
emergency cardiac surgery as of eleven days old,
Barnabas required long-term mechanical ventilation.
Further care in our department began through feeding
exclusively by NGT. After fighting infections, his vital
parameters allowed attempting minimal oral feeding,
still supplemental artificial nutrition was necessary.
Becoming two months old, he started to refuse NGT by
his body language or pulling out the tube, manifesting
also repeated retching, vomiting, without detectable
organic cause. Oral feeding was given during his arousal
period, supplemented by NGT while resting. During
our care we completed the treatment with a parentinfant consultation every day. Barnabas went home at
three months old, with positive weight tendency. 1.5
months later, during a necessary re-hospitalization his
attempts with putting all his power for pulling out his
tube paired with oral underfeed, retching made his
nourishment critical. Treating his infection, replenishing
the required fluids and nutrition, continuing the parentinfant consultations, the willpower of Barnabas and
persistent cooperation, patience and confidence of
his parents allowed reaching the solely oral feeding.
Conclusions: Creating a complex and long-lasting care
for the patients with chronic diseases and their family
members is challenging. Instead of focusing only the
somatic symptoms we should apply to bio-psycho-social
approach to reach an effective healing.
Keywords: feeding disorder, parent-infant
consultation, psychological support
[P-008]
Gitelman Syndrome Case Report
Ahmet Ibrahim Bektasoglu, Burcu Karakayalı, Ismail
Islek, Ahmet Sami Yazar, Julide Cavus
Pediatric Clinic of Umraniye Training and Research
Hospital, Istanbul, Turkey
Context: Gitelman syndrome (GS) is an inherited
autosomal recessive kidney disorder. It can diagnosed
during adolescence or early adulthood based on clinical
and biochemical findings.
- 20 -
Case Presentation: We present a case of 12 years old
male, admitted our emergency service with rash and
itching. Questioning revealed, patient had carpal spazm,
hand paresthesia, increased thirst, frequent urination
and nocturnal enurezis. Family never appealed for this
further symptoms. Patient diagnosed parvovirus B19
infection and GS. Parvovirus B19 serology performed.
Typical biochemical abnormalities for GS as hypokalemia,
hypomagnesemia, hypocalciuria, metabolic alkalosis and
hyperreninemic hyperaldosteronizm were detected. The
patient was treated with oral potasium, magnesium and
desmopressin, biochemical abnormalities and symptoms
gradually regressed.
Conclusion: Gitelman syndrome presentation and
diagnosis is usually made during adolescence or early
adulthood. It should be considered as a differential
diagnosis of hypokalemia, especially in late childhood
and adolescent age group, but must be noted that it
can even occur under 6 years, also be noted that typical
symptoms may not be the admitting reason.
Keywords: Gitelman Syndrome, Hypokalemia,
Metabolic acidosis
[P-009]
A Rare Cause Of Acute Kidney Failure Caused
By Rabdomyolysis: Weil Disease
Alkim Beyhan
Akdeniz University School of Medicine, Pediatrics
Deparment, Antalya, Turkey
Leptospirosis is a commonly seen zoonosis caused by a
bacteria called Leptospira. Even though Weil Disease is
seen only in %5-10 of Leptospiral infections, because
it affects a variety of organs it’s course is quite severe.
An 18 year old male patient is presented with a greenish
colored feces which had diarrheic characteristics.
He had no fever but he noticed that his daily urine
output has decreased. The patient had normal blood
pressure(115 systolic, 80 diastolic mmHg). He had a
2cm hepatomegaly on the midclavicular line, periorbital
and peripheral edema. His lab results showed that
he had thrombocytopenia, an increase in creatinine,
BUN, creatinine kinase and myoglobin levels.(Table 1)
The second case was a 15 year old male patient who
had had fever for 4 days, diffuse myalgia and muscle
weakness. In his physical examination, he had diffuse
jaundice, a 3cm hepatomegaly on the midclavicular
line and crackles on the middle and inferior lobes of
the right lung. Similar to the first patient, the second
patient had thrombocytopenia, lymphocytopenia, an
increase in creatinine, BUN and creatinine kinase levels.
In addition to this he had conjugated hyperbilirubinemia.
Upon further examination, both patients were found to
have swam in the same river, approximately 10 days
before the symptoms began. Because of the similar
history, we studied microscobic agglutination test for
Leptospira, which were both found positive(1/800
Leptospira
Icterohaemorrhagiae
for
the
former
and 1/100 Leptospira Pomona for the latter)
The first patient had 2 courses and the second one
had 3 courses of hemodialysis and ultrafiltration.
The first patient was administered meropenem
and the second one meropenem and doxycycline.
With both of these cases we wanted to emphasize that
patients who were presented with acute kidney failure
secondary to rabdomyolysis could have an infectious
origin such as Leptospira.
Keywords: Leptospirosis, Rabdomyolysis
[P-010]
Portal cavernoma in a child – case report
Ancuta Ignat1, Vasile Valeriu Lupu1, Gabriela Paduraru1,
Angelica Cristina Marin1, Bogdan Ciubara2, Marin
Burlea1
1
Pediatrics Department, University of Medicine and
Pharmacy “Gr. T. Popa”, Iasi, Romania
2
Anatomy Department, University of Medicine and
Pharmacy “Gr. T. Popa”, Iasi, Romania
Cavernous transformation of the portal vein (also called
portal cavernoma) occurs when the native portal vein is
thrombosed and it is replace the normal single channel
portal vein with numerous tortuous venous channels.
Cavernous transformation has been shown to form as
early as 6 to 20 days after acute thrombosis of the portal
vein. In about 30% of cases no underlying cause is found.
It occurs much more commonly in patients without
underlying liver disease. Often, it is associated with portal
hypertension because the collateral veins are not able
to adequately handle the splenic and mesenteric inflow.
We report the case of a child of 13-year-old female
presented with asthenia, headache, somnolence and
hematemesis (an isolated instance 7 days prior to
admission). Associated findings in this case included
esophageal varices grade III, signs of splenomegaly
(severe microcytic hypochromic anemia, leukopenia
and thrombocytopenia). The diagnosis was confirmed
with liver computerized tomography after obtaining a
normal ultrasound. Better control of hemorrhagic and
thrombotic complications in the patients with portal
cavernoma substantially improves their life span and
the clinical outcome. Whereas portal hypertension can
in some cases be treated with transjugular intrahepatic
portosystemic shunt (TIPS), but the absence of normal
portal circulation usually makes this impossible.
Keywords: portal cavernoma, esophageal varices,
splenomegaly
- 21 -
[P-011]
[P-012]
Analysis of pediatric pancreatitis (APPLE).
Multicenter prospective data collection and
analysis by the Hungarian Pancreatic Study
Group (HPSG)
Observational Clinical Trial About Pain
IN EArly Phase of Pediatric pancreatitis
(PINEAPPLE)
Andrea Parniczky1, Dóra Mosztbacher2, Natália
Lásztity1, Anna Tóth3, Noémi Vass3, Judit Czelecz7, Csilla
Andorka5, Gábor Veres5, Ildikó Guthy4, Veronika Ila4,
Balázs Csaba Németh6, Péter Hegyi6
1
Heim Pál Children’s Hospital
2
János Balassa County Hospital, Department of
Pediatrics, Szekszárd, Hungary
3
University of Szeged, Faculty of Medicine, Department
of Pediatrics and Pediatric Health Center,
4
Szabolcs-Szatmár-Bereg County Hospitals, Jósa András
University Teaching Hospital, Nyíregyháza,
5
Semmelweis University, First Department of Pediatrics
and Pediatric Health Center, Budapest
6
University of Szeged, Faculty of Medicine, First
Department of Medicine, Hungary
7
Bethesda Children’s Hospital, Budapest, Hungary
Background&Aims: The incidence of pediatric
pancreatitis(PP) has increased in the last decade. Some
of the recent studies showed that the occurrence of the
disease has grown over 10/100.000 which is not much
less than in adults. We have established the Paediatric
Section of the Hungarian Pancreatic Study Group in
order to organize nationwide data collection and improve
the management of the disease. Our aim was to analyse
the epidemiology, risk factors, management and clinical
outcome of PP in Hungary.
Methods: 56 children suffering from PP were enrolled
from 7 centres between 2012 and 2014.
Results: 61% of the children were female. 31 acute(AP),
11 recurrent acute(RAP) and 14 chronic pancreatitis(CP)
cases were recorded in the registry. 84% of the AP
patients had mild and 16% moderate episodes, however,
no severe AP was observed. In RAP patients pancreatitis
seemed to be more severe than in patients having
isolated episodes (mild: 73% moderate: 18%, severe:
9%). Mortality was not observed at all. Without genetic
testing we could identify the etiological factors only in
44% of the cases, the others remained idiopathic.In 17
cases, genetic analyses of PRSS1, SPINK1, CFTR and
CTRC genes have been completed. Genetic alterations
in PRSS1 were found in 3 cases(all CP), in SPINK1 in
4 cases(1 RAP and 3 CP), in CFTR in 1 case(CP) and
in CTRC in 10 cases(3 AP and 7 CP), In 5 CP patients
mutations in two genes were observed(3 SPINK1-CTRC,
1 PRSS1-SPINK, 1 CFTR-CTRC).
Conclusions: Genetic testing is essential to identify the
etiological factors in children with pancreatitis.
Keywords: pediatric pancreatitis, genetic testing,
multicenter prospective trial
Dóra Mosztbacher1, Andrea Párniczky2, Fanni Zsoldos2,
Anna Tóth3, Veronika Ila4, Maisam Abu El Haija5, István
Tokodi6, Boglárka Fehér7, Károly Bakó7, Dénes Molnár8,
Andrea Szentesi9, Péter Hegyi9
1
Department of Pediatrics, János Balassa County
Hospital, Szekszárd, Hungary
2
Heim Pál Children’s Hospital, Budapest
3
Department of Pediatrics and Pediatric Health Center,
University of Szeged, Faculty of Medicine, Szeged,
Hungary
4
Department of Pediatrics, Dr. Kenessey Albert Hospital,
Balassagyarmat, Hungary
5
Division of Gastroenterology, Hepatology and Nutrition,
Cincinnati Children’s Hospital Medical Center, Cincinnati,
USA
6
Department of Pediatrics, Szent György Teaching
Hospital of County Fejér, Székesfehérvár, Hungary
7
Department of Pediatrics, University of Debrecen,
Faculty of Medicine, Debrecen, Hungary
8
Department of Pediatrics, University of Pécs, Faculty of
Medicine, Pécs, Hungary
9
First Department of Internal Medicine, University of
Szeged, Faculty of Medicine, Szeged, Hungary
Background&Aims:
The
documented
incidence
of pediatric pancreatitis (PP) is very low, less than
1/100,000 in almost all European countries, whereas it
is around 3.6-13.2/100,000 in the USA and Australia.
Moreover, there are unexpectedly large differences
between the incidences of PP among the countries and
hospitals in Europe. The aim of the PINEAPPLE study
(ISRCTN35618458) is to understand the current practice
of diagnosis of PP, to demonstrate the difference of the
incidence of PP between the various countries based
on the pancreatic enzyme measurement(PEM) and to
develop EBM guidelines that helps to evaluate (in a
reliable and cost efficient way) the necessity of PEM and
abdominal ultrasonography when a child has abdominal
pain.
Methods: PINEAPPLE is a registered, observational,
multinational clinical trial. The PINEAPPLE-R subtrial is
a retrospective review on children records appearing
at ER units, whereas, the PINEAPPLE-P subtrial
is a prospective part of the study where detailed
patients data (concerning medical history, complaints,
symptoms, physical examination) are collected, PEM and
abdominal imaging are performed in all cases. Until now
23644 patients records/PINEAPPLE-R and 113 patients/
PINEAPPLE-P were enrolled from eight pediatric centres.
Results: PINEAPPLE-R: 8,3% (1970/23644) of the
patients appearing at ER unit had abdominal pain.
Only 9,7% (192/1970) of them had PEM, whereas
30% (592/1970) had transabdominal ultrasonography.
Pancreatitis was diagnosed in five cases only. In case
where 21,6% (157/728) PEM were performed, the
incidence of pancreatitis (4/728) was six times higher,
than in case where just 2,8 % (35/1242) PEM were
performed (1/1242). PINEAPPLE-P: 1 pancreatitis of 113
patients with abdominal pain was diagnosed.
- 22 -
Conclusions: The PINEAPPLE-R clearly shows that the
number of PEM performed at ER units are unacceptably
low in children, which could be the reason of low
incidences of PP. More patients are crucially needed for
PINEAPPLE-P in order to develop EBM guidelines.
Keywords: pancreatitis, abdominal pain, EBM
[P-013]
An Infant with Congenitally Corrected
Transposition; Case Report
Aras Tulunoglu1, Osen Arı1, Helen Bornaun1, Aylin
Öztarhan2, Murat Şahin1, Kazım Öztarhan1
1
Kanuni Sultan Suleyman Hospital, Pediatrics &
Pediatric Cardiology Department, Istanbul, Turkey
2
Sisli Etfal Hospital, Gynegology and Obstetrics
Department, Istanbul, Turkey
Introduction: Congenitally corrected transposition of
the great vessels (CCTGA) is a rare congenital heart
defect associated with multiple cardiac morphological
abnormalities and conduction defects.
Case Description: One year old girl with no medical
history was referred with tachypnea, dyspnea and
tachycardia. Her height, weight and head circumference
were between 10 to 25 percentile. No significant
neuromotor and pulmonary abnormalities were
detected. Initial ECG report showed narrow QRS
complex supraventricular tachycardia. Rapid intravenous
adenosine administered, after stabilization the ECG
monitor revealed that the PR interval had shortened, a
delta wave were present right before the QRS complex
and Q waves were present in the right precordial leads
but absent in the left precordial leads. The patient was
suspected with supraventricular tachycardia and WolffParkinson-White syndrome associated with congenitally
corrected transposition of the great arteries (CCTGA).
Echocardiography had revealed ventricular inversion.
The patient was prescribed propranolol. A Holter monitor
is used for assessment of AV block and atrial arrhythmia,
none had seen. A week later the patient had discharged
for follow-up.
Discussion: CCTGA is a rare defect that reported in
0.5% of patients with congenital heart disease. In situs
solitus, the morphologic right ventricle is anatomically
positioned to the left of the morphologic right ventricle.
Blood flows in an effective sequence; however, the
right ventricle supports the systemic circulation in
this disorder. Prognosis depends on AV conduction,
arrhythmias, structural abnormalities and degree of
hemodynamic disturbance.
Conclusion: Supraventricular tachycardia is the most
common symptomatic dysrhythmia in infants and
children and CCTGA should be considered. The diagnosis
of CCTGA is usually made by echocardiography. ECG can
be helpful to show the ventricular bundle branches are
inverted and the initial activation is oriented from rightto-left.
Keywords: congenitally corrected transposition,
supraventricular tachycardia, Wolff-Parkinson-White
syndrome
[P-014]
Isolated Left Ventricular Non-Compaction
(LVNC) and Restrictive Cardiomyopathy
with missense mutation in MYH7 gene; Case
Report
Kazım Öztarhan1, Şükrü Candan4, Tuğba Erener5, Helen
Bornaun1, Aras Tulunoğlu2, Elif Yılmaz Güleç3, Akl
Fahed6, Zeynep Ocak3
1
Kanuni Sultan Suleyman Education and Research
Hospital, Department of Pediatric Cardiology Istanbul,
Turkey
2
Kanuni Sultan Suleyman Education and Research
Hospital, Department of Pediatrics, Istanbul, Turkey
3
Kanuni Sultan Suleyman Education and Research
Hospital, Department of Medical Genetics, Istanbul,
Turkey
4
Ataturk General Hospital, Department of Medical
Genetics, Balikesir, Turkey
5
Maltepe University Faculty of Medicine, Department of
Neonatology, Istanbul, Turkey
6
Harvard Medical School, Department of Genetics,
Seidman Laboratory
Introduction: Isolated left ventricular non-compaction
(LVNC) is a myocardial disorder characterised by
prominent ventricular trabeculations and deep recesses
extending from the LV cavity to the subendocardial
surface of the LV wall with or without LV dysfunction.
Case Report: A-4 year-old girl with a history of
respiratory problems was referred with palpitations,
sweating, dyspnea and syncope. After the initial
examination and treatment the patient was suspected
for cardiac failure. Transthoracic echocardiography had
revealed prominent ventricular trabeculations extending
from the LV cavity, dilatation of the left atrium (LA) and
moderate mitral valve insufficiency. On doppler tissue
imaging, there was a diastolic dysfunction of the LV in
restrictive pattern. MRI and cardiac catheterization had
confirmed the trabeculations and restrictive pattern of
the LV. The patient prescribed with diuretics and ACE
inhibitors and added to cardiac transplantation list.
Molecular analysis had performed to family members
with target capture sequencing which is created for
known cardiomyopathy genes group. The mutation in
our case was a novel missense mutation located in exon
14 (GCA/GTA) A426V-MYH7 coding region. Segregation
analysis within the family revealed that both parents
and unaffected brother were homozygous wild genotype
(GCA/GCA).
Discussion: Isolated left ventricular non-compaction
(LVNC) is a morphological abnormality of excessive
trabeculation of the LV, often complicated by ventricular
dysfunction, arrhythmias and cardioembolism. Screening
of first-degree relatives is warranted, asymptomatic
family
members
may
have
good
prognosis.
Some of the genetic mutations associated with LVNC
include the G4.5 gene, DTNA (α-dystrobrevin), Cypher/
ZASP, lamin A/C cardiac troponin T (TNNT2), myosin
heavy-chain gene (MYH7), cardiac actin (ACTC1), SCN5A
and MYBPC3.
Conclusion: LVNC is a form of cardiomyopahty that
there are no evidince of significant difference from the
- 23 -
other forms of cardiomyopathies. In this case we report
LV dysfunction in a restrictive pattern with a novel
missense mutation of the MYH7 gene.
Keywords: Isolated left ventricular non-compaction,
restrictive cardiomyopathy, MYH7 gene
[P-015]
A case of primary immunodeficiency:Severe
combined immunodeficiency
Arzu Aras, Hatice Öztürk, Aynur Bedel, Zehra Esra
Onal, Çağatay Nuhoğlu
Haydarpaşa Numune Education and Research
Hospital,Pediatri Department,Istanbul
Background&Aims:
Methods:
Results:
Conclusions:
Primary immune deficiencies, depending on hereditary
gene defects, associated with disorders in the
functioning of the immune system with increased
susceptibility to infections are characterized by the
tendency to the formation of autoimmune and malign
disorders. Combined immunodeficiency is called
severe combined immunodeficiency due to genetic
defects in various T-cell differentiation disorders
occur as a result of fully blocked T-cell differentiation.
2-month old girl was admitted to our hospital with
cough and fever.She had cough and fever during the
last 20 days.With a diagnosis of pneumonia, she was
treated with ceftriaxone and vancomycin in previous
hospitalization. Because of no recovery of fever and
cough with the current treatment, the patient was
hospitalized in our clinic for further evaluation and
treatment. She had no health problem after birth.
Her family history showed epilepsy presence of her
father, death of four brothers without any reason when
they were approximately 2 months of aged, and the
parents are the 1st degree cousin (uncle’s children).
Lung infection in an early age and early age death of
brothers due to no reason in history, immune deficiency
was considered. Laboratory results showed the
detection of lymphopenia and hypogammaglobinemia
strengthened our diagnosis as primary immunodeficiency.
Lymphocyte subsets; CD3 %6, CD3CD4 %3.85,
CD3CD8 %2.29, CD19 %0.06, CD1656 %89,25
T(-) B(-), were detected respectively. She was
diagnosed with severe combined immunodeficiency.
Early diagnosis and treatment of primary immune
deficiency, is very important in order to reduce the risk
of death and complications. Therefore, early recognition
of immune deficiency by clinicians is important for the
prevention and reduction of mortality and appropriate
treatment of complications due to recurrent infections.
Keywords: Severe combined immune deficiency,
children
[P-016]
septo-optic displasia syndrome caused by
congenital cmv infection
Asuman Kıral, İsmet Düşmez, Doruk Gül, Asena Pınar
Sefer
Goztepe Research Hospital Istanbul/Turkey
Introduction: Septo-optic displasia syndrome( De
Morsier Syndrome ),is a rare congenital malformation
syndrome.Its prevelance is 1:50000 and has 3
characteristic speciality which are optic nerve
atrophy(causes nistagmus),septum pellucidum agenesia,
hypothalamic-pituitary disfunction and %50 cases have
schizencephaly.Congenital CMV infection rarely causes
this syndrome in embriological stage.
In this case;we describe the importance of differential
diagnosis in prolonged jaundice
Case: 3 month aged patient who had jaundice,central
hypotiroidism,hypotonia and hepatosplenomegaly,was
sent to our hospital because suspecting metobolic
disesase from another hospital.
He had jaundice, hypotonia,rotatuar
nistagmus,macrocephaly,2-3/6 cardiac
murmur,hepatosplenomegaly in physical examination
Laboratory results; WBC:7280/mm^3,PNL 25%,LYM
66%,Hb:9.73 g/dL,Hct: 27.6%,ALT:356 IU/L,AST:527
IU/L,GGT:70 IU/L,AF:796 IU/L,T.Bil:9.18 mg/dL,D.
bil:5.96 mg/dL,electrolits and urinalyse was normal.
Hemolytic anemia,viral hepatitis,aminoacid metabolic
diseases,CMV infection,galactosemia,peroxysmal
diseases,pituitary disfunction,biliary tract agenesia,
Wolman disease,intracranial pathology was tought in
differential diagnosis.
Anti CMV IgG (+), IgM (-) was the only meaningful
result in viral serology Furthermore, urinalysis for
Galactosemia was negative,fitanic and pyristanic
acid
was
negative(for
peroxysismal
diseases).
The other methabolic labratory results was normal
Secundum ASD was detected in ECHO.
Lateral ventricules was enlarged in Cranial USG
Hepatosplenomegaly and grade 1 hepatosteatosis was
detected in abdominal USG.Surrenal glands were intact.
Bilateral optic disc atrophy was realized in ophtalmologic
examination.
Pediatric Endocrinology detected panhypopituitarsm as a
result of further investigation.
BERA test for audition was normal.
Pediatric Gastroenterology advised liver biopsy and the
result was neonatal hepatitis.
In Cranial MRI; right frontal lobe closed lib
schizencephaly,septum
pellicidum
agenesia
was
detected.
His CSF CMV PCR<79.7copy/ml,Urine CMV
PCR:19434copy/ml
The results were meaningful for Congenital CMV Infection
and Pediactric infectious disease advised ganciclovir
treatment for 6 weeks.
The baby who has bilateral optic disc hipoplasia, septum
pellucidum agenesia, shizencephaly, CMV infection,
rotatuar nistagmus, panhipopituitarism was diagnosed
to Septo-optic displasia Syndrome.
- 24 -
[P-018]
Keywords: septo-optic displasia syndrome, congenital
cmv infection
Isolated exocrine gland involvement
associated with mutation of 1677 delta
heterozygote and M470V heterozygote
polymorphism on the CFTR Gene
[P-017]
Syndromic Severe Congenital Neutropenia:
JAGN1 Mutation
Avniye Kübra Baskın, Funda Çipe, Çiğdem Aydoğmuş,
Gonca Keskindemirci, Kaan Boztug
Division of Pediatric Allergy and Immunology,
Kanuni Sultan Suleyman Research and Training
Hospital,Istanbul,Turkey
Severe congenital neutropenia (SCN) is characterized
by susceptibility to recurrent life threatening bacterial
infections due to maturation arrest of neutrophils. Different
studies have showed mutations in ELA 2, HAX 1, G6PC3,
WAS, GF11 and VPS45 genes. Jagunal homolog 1 (JAGN
1) mutations were recently defined as a rare genetic
cause severe congenital neutropenia defect in 2014.
Our patient was a 10 years old male born from first
degree cousin parents at 1900 gram birth weight. He
was admitted due to neonatal sepsis for a month at
fourth day of postnatal age. He was re-hospitalized
because of wounds on bilateral lower extremities and
severe neutropenia has been noticed. Bone marrow
aspiration revealed maturation arrest of neutrophils.
He was started to treat with G-CSF (5 µg/kg) with
the initial diagnosis of Kostmann syndrome. Physical
examination was not notable except triangular face and
extrovert ears. Initial laboratory evaluation revealed
only neutropenia. Despite treatment he had recurrent
infections including pneumonia, otitis, sinusitis and skin
abscess even pulmonary tuberculosis. In addition, he
suffered from asthma due to house dust mite allergy.
Mutation analysis for common mutations for SCN were
found to be negative. After identification of JAGN1
deficiency, it was sequenced the relevant gene and
detected a homozygous missense mutation in exon 2
of JAGN1 gene (c 130 c>T, p. His 44 Tyr). The patient
is still followed up and receiving high dose G-CSF(10 µg
/kg). Matched unrelated donor screening is continuing
for bone marrow transplantation because of lack of
family matched donor. In conlusion, JAGN 1 mutations
must be considered in patients with severe congenital
neutropenia especially with facial dismorphism and
multisystemic disorder.
Keywords: JAGN1 mutation, Severe congenital
neutropenia
Ayla Balcı1, Duygu Övünç Hacıhamdioğlu2, David
Terence Thomas3, Ayça Sözen1, Arif Kut4, Ismail
Göçmen1
1
Department of Paediatrics, Faculty of Medicine,
Maltepe University
2
Department of Pediatric Nephrology, Faculty of
Medicine, Maltepe University
3
Department of Pediatric Surgery, Faculty of Medicine,
Maltepe University
4
Department of Pediatric Pulmonology, Faculty of
Medicine, Maltepe University
Introduction: CF is a genetic disease that commonly
involves the lungs, gastrointestinal system, pancreas and
exocrine glands. It is rare for a patient to have normal
lungs yet other system involvement. Herein we report
a patient affected by only exocrine gland dysfunction
presenting with anemia due to cystic fibrosis (CF).
Case: A 2.5 month-old boy was born meconium stained
from unconsanguineous healthy parents at 38th weeks
of gestational age, weighing 2600 g. Prenatal history was
insignificant. Family history revealed Cystic Fibrosis (CF)
in his mother’s cousin (exocrine involvement, female)
and father’s cousin (exocrine and lung involvement,
male). The patient was admited to our hospital at 2.5
months of age (weight: 6300 g. 50th percentile, height:
61 cm, 50th percentile) due to weakness, facial edema
and paleness. Deep anemia (hemoglobin 6 g/dl) was
detected, requiring recurrent erythrocyte transfusions.
Hypokalemic metabolic alkalosis was found during
investigation of etiology of anemia. There was no renal
potassium, calcium or sodium loss. Analysis of blood
and urine was evaluated normally. Sweat chloride test
results were 68 mmol/L, 92 mmol/L and 98 mmol/L
(normal: <60 mmol/L). A diagnosis of CF was made.
Vitamins A, D, E and K, plus potassium, and pancrelipase
supplementation was commenced and his anemia
resolved. CFTR gene analysis was performed on 10th
exon and heterozygote mutation of 16677delta (M470V
polymorphism) was detected. The patient currently
has no signs of lung involvement and his neurological
development is appropriate for his age.
Discussion: It is rare for CF to effect other systems
without lung involvement. We report a small infant where
CF presented with anemia, exocrine gland dysfunction
but no lung involvement. Heterozygote polymorphisms
are not known to lead to disease. However, interestingly,
our patient’s 1667delta heterozygote mutation (M470V
polymorphism) seems to have led to CF. Further genetic
analysis has been planned.
Keywords: cystic fibrosis, deep anemia, infant
- 25 -
[P-019]
Viral Encephalitis With Atypical and
Fulminant Prognosis (Case Report)
Aynur Bedel, Hatice Öztürk, Tamay Gürbüz, Dilşad
Koca, Çağatay Nuhoğlu
Haydarpaşa Numune Eğitim ve Araştırma Hastanesi
Encephalitis in childhood can be occured fulminant and
mortal. Permanent morbidity is present even over 35%
in treated patients.
In this study atypical encephalitis which is compatible
with radiological encephalitis, but can not be determined
by serologically active fulminant course and get benefit
by acyclovir treatment with antibiotics, steroids and
IVIG is presented.
Keywords: Viral encephalitis, atypical, fulminant
[P-020]
Anti N Methyl D Aspartate(NMDA) reseptor
encephalitis after healing of herpes simplex
virus (HSV) encephalitis
Aynur Guliyeva1, Türkan Uygur Şahin2, Ayşegül Doğan
Demir1, Akın İşcan2
1
Bezmi Alem Üniversitesy Medical Faculty, Paediatrics
2
Bezmi Alem Üniversitesy Medical Faculty, Children
Neurology
Anti NMDA (N-methyl D-aspartate) receptor antibody
encephalitis caused by an autoimmune reaction of the
NDMA receptor.
Sometime this disorder relapses after herpes simplex
virus1 (HSV1) encephalitis. Anti NMDA (N-methyl
D-aspartate) receptor antibody encephalitis have been
hipothesied to be secondary to postviral autoimmunity.
25 month old girl was admitted with a fever, seizures,
agitation.
Prenatal and postnatal history wasn’t signifancy. No
family history feature.
Acute phase reactants were negative and other laboratory
tests were normal range. Cranial magnit resonant image
(MRI) is compatible with herpes encephalitis: bilateral
themperoparietal edema were found.
In cerebrospinal fluid HSV 1DNA-PCR were positive.
Spike waves were seen in EEG. Because of these findgins
acyclovir treatment were applied for 3 weeks.
After 10 days from discharge in patient began speech
disorder,
gait
disturbance,
choreiform
movies,
oropharingeal dyskinesia.
With the diagnosis of limbic encephalitis began IVIG
therapy. At the same time sent cerebrospinal liquid antiNMDA receptor antibody was positive. Admitted pulse
steroid therapy.
Seven times plasmapheresis treatment - refractory
patients began cyclophosphamide therapy. After receive
2 dose of cyclophosphamide treatment response
were taken. Gradually improved swallowing and and
disappeared dyskinesia and choreiform movies.
Control cranial MRI was compatible with anti NMDAR
antibody encephalitis (cerebellar and cerebral volume
loss and ventricular dilatation).
The
treatment
of
patients
is
continuing.
Treated patients with encephalitis and after develop
choreiform movie, agitation and dyskinesia must come
to mind NMDA encephalitis.
Treatment options is IVIG, pulse steroid, plasmapheresis
and cyclophosphamide.
Keywords: Anti NMDA (N-methyl D-aspartate)
receptor antibody encephalitis, oropharıngeal
dyskinesia, choreiform movies
[P-021]
α-Melanocyte-Stimulating Hormone and Agouti-Related
Protein: Do They Play a Role in Appetite Regulation in
Childhood Obesity?
Aysel Vehapoğlu1, Serdar Türkmen2, Şule Terzioğlu3
1
Department of Pediatrics, Bezmialem Vakıf University,
School of Medicine, Istanbul, Turkey
2
Department of Biochemistry, Gaziosmanpaşa Taksim
Training and Research Hospital, Istanbul, Turkey.
3
Department of Medicinal Biology, Bezmialem Vakıf
University, School of Medicine, Istanbul, Turkey.
Background&Aims: The hypothalamus plays a
crucial role in the regulation of feeding behavior. The
anorexigenic neuropeptide α-melanocyte-stimulating
hormone (α-MSH) and the orexigenic neuropeptide
agouti-related protein (AgRP) are among major peptides
produced by hypothalamus. This study investigated
the plasma concentrations of α-MSH and AgRP in
underweight and obese children and their healthy peers,
and the association between α-MSH and AgRP levels and
anthropometric and nutritional markers of malnutrition
and obesity.
Methods: Subjects aged 2 to 12 years were divided into
3 groups:57 underweight (due to loss of appetite and
less frequent hunger episodes) prepubertal children,
61 healthy obese children, and 57 healthy age and
sex-matched children of normal weight. Plasma fasting
concentrations of α-MSH and AgRP were measured by
enzyme-linked immunosorbent assay. The relationships
between plasma concentrations of α-MSH and AgRP and
anthropometric data, serum biochemical parameters,
and homeostatic model assessment of insulin resistance
were also evaluated.
Results: Obese children had significantly lower α-MSH
levels than did underweight (1194±865 vs. 1904±1312
ng/ml, p= 0.006) and normal-weight (1194±865 vs.
1762±1463 ng/ml, p= 0.036) children; there were no
significant differences in the α-MSH levels between the
underweight and normal-weight children (p= 0.811).
Also, no significant differences were observed between
the underweight and obese children in the AgRP levels
(742±352 vs. 828±417, p=0.125). We found a significant
positive correlation between the plasma α-MSH and AGRP
levels (p< 0.001) and a negative correlation between
the α-MSH levels and weight z-score (p= 0.017), BMI
(p= 0.019), BMI z-score (p= 0.014), BMI percentile (p=
0.017), insulin (p= 0.045), and CRP (p< 0.001).
Conclusions: This study is the first to demonstrate
body weight-related changes in α-MSH and AgRP levels
in children. Circulating plasma α-MSH levels in obese
children were markedly lower than those of underweight
and normal-weight children. This suggests that α-MSH
- 26 -
[P-023]
could play a role in appetite regulation.
Keywords: α-MSH, AgRP, underweight children
[P-022]
Role of the circulating adipocytokines
vaspin, apelin, and visfatin in loss of appetite
in underweight children: A pilot trial
Aysel Vehapoğlu1, Feyza Ustabas1, İlker Tolga Özgen2,
Şule Terzioğlu3, Banu Çermik1, Ömer Faruk Özer4
1
Department of Pediatrics, Bezmialem Vakıf University,
School of Medicine, Istanbul, Turkey.
2
Department of Pediatric Endocrinology, Bezmialem
Vakıf University, School of Medicine, Istanbul,
3
Department of Medicinal Biology, Bezmialem Vakıf
University, School of Medicine, Istanbul, Turkey.
4
Department of Biochemistry, Bezmialem Vakıf
University, School of Medicine, Istanbul, Turkey.
Background&Aims: In anorexic children who eat slowly,
consume a limited number of foods, lack interest in food,
display an irregular eating pattern, and experience loss
of appetite and less frequent hunger episodes, body
weight and fat are reduced. Children require sufficient
nutrients to support the immune system, and to help
the body maintain health and normal bodily functions.
Present study aims to investigate serum concentrations
of vaspin, apelin, and visfatin in underweight children
and their association with anthropometric and nutritional
markers of malnutrition.
Methods: We recruited 44 underweight prepubertal
children (youngest age = 2 years) with thinness
grades of 1, 2, and 3, and body weights <90% of ideal
body weight due to loss of appetite and less frequent
hunger episodes. Forty-one healthy age- and gendermatched controls were also included in the study. Serum
fasting vaspin, apelin, and visfatin concentrations were
measured by enzyme immunoassay.
Results: Mean vaspin and apelin levels in the
malnourished group (0.44 ± 0.18 and 483.37 ± 333.26
pg/mL, respectively) were significantly lower compared
to healthy controls (0.58 ± 0.35 and 711.71 ± 616.50
pg/m, P = 0.024 and P = 0.041, respectively). No
significant group differences were observed in visfatin
levels (malnourished group: 177.81 ± 158.01 pg/mL;
control group: 221.15 ± 212.94 pg/mL, P = 0.119).
Vaspin and apelin levels were positively correlated with
BMI z-scores (r = 0.297, P = 0.007; and r = 0.267, P =
0.016, respectively) in the malnourished group.
Conclusions: In underweight children, decreased
vaspin and apelin levels should be considered in the
etiology of anorexia. Our data suggest that plasma
apelin and vaspin levels represent a sensitive index of
nutritional status that reflects changes in the fat levels
of underweight children.
Keywords: appetite regulation, vaspin, apelin
Reduced antioxidant capacity and increased
subclinical inflammation markers in
prepubescent obese children and their
relationship with nutritional markers and
metabolic parameters
Aysel Vehapoğlu1, Serdar Türkmen2, Nilüfer Göknar1,
Ömer Faruk Özer3
1
Department of Pediatrics, Bezmialem Vakıf University,
Faculty of Medicine, Istanbul, Turkey.
2
Department of Biochemistry, Gaziosmanpaşa Taksim
Training and Research Hospital, Istanbul, Turkey.
3
Department of Biochemistry, Bezmialem Vakıf
University, School of Medicine, Istanbul, Turkey.
Background&Aims: There are associations between
some inflammatory and oxidative markers and obesity
in adults, but whether prepubescent children of different
weights also have such markers has not been studied.
We investigated multiple inflammatory markers and
levels of erythrocyte oxidant/antioxidant enzymes in
prepubescent children of different weights.
Methods: Children aged 2–11 years were divided into
three groups: 80 were underweight, 90 were obese
but otherwise healthy, and 80 were healthy age- and
sex-matched children of normal weight. We analyzed
inflammatory markers and the total oxidant status,
total antioxidant status, and total thiol level were also
determined, and the oxidative stress index was calculated
as an indicator of the degree of oxidative stress.
Results: The obese group exhibited higher levels of
fasting glucose, insulin, total cholesterol, triglycerides,
the homeostatic model assessment of insulin resistance,
and the homeostatic model assessment of β-cell function,
CRP, neutrophils, and neutrophil/lymphocyte ratio, as
well as lower TAS and total thiol levels than the other
two groups (all p< 0.001). Moreover, TAS and total thiols
were negatively correlated with age in the obese group
(r= -0.212, p=0.001; r= -0.231, p<0.001, respectively).
CRP levels in plasma were positively correlated with
the body mass index (BMI), insulin and glucose levels,
HOMA-IR, HOMA-β, WBC and neutrophil counts, and the
NLR, and were negatively correlated with TAS and total
thiol levels in the overall studied population.
Conclusions: The coexistence of increased obesityrelated
subclinical
inflammation
and
decreased
antioxidant capacity can be observed even in
prepubescence, and may eventually increase the risk of
long-term vascular damage.
Keywords: Inflammation markers, Oxidative/
antioxidative status, Prepubescent children
- 27 -
[P-024]
Clinical significance of human
metapneumovirus in refractory status
epilepticus and encephalitis: case report
Aysel Vehapoğlu1, Özden Türel2, Türkan Uygur Şahin3,
Nurettin Onur Kutlu4, Akın İşcan3
1
Department of Pediatrics, Bezmialem Vakıf University,
Faculty of Medicine, Istanbul, Turkey.
2
Department of Pediatrics Infection, Bezmialem Vakıf
University, Faculty of Medicine, Istanbul,
3
Department of Pediatric Neurology, Bezmialem Vakıf
University, Faculty of Medicine, Istanbul, Turkey.
4
Department of Pediatric Emergency, Bezmialem Vakıf
University, Faculty of Medicine, Istanbul, Turkey.
Human metapneumovirus (hMPV) is a common cause
of respiratory tract infections in children. Here we
describe an infant with refractory status epilepticus and
encephalitis in whom hMPV was detected in respiratory
samples. A previously healthy 4-months-old male infant
presented with ongoing seizure activity. He had 3 days
history of mild rhinorrhea and suddenly experienced
twitching at right extremity and eye deviation to the
right. The seizure was unresponsive to rectal diazepam
and could only be controlled by bolus midazolam and
phenytoin infusion. His EEG tracing was consistent
with multifocal epileptiform activity. Cerebrospinal fluid
(CSF) examination showed 100 leukocytes/mm3 (all
lymphocytes), glucose 51 mg/dl, and protein 24 mg/dl.
Acyclovir and ceftriaxone were initiated pending culture
results. Cranial magnetic resonance imaging showed no
abnormalities. No bacterial growth was detected in blood,
urine, respiratory, and CSF cultures. Investigation of viral
pathogens on nasal scrapings by reverse transcription
PCR revealed hMPV. CSF herpes simplex type 1 and 2
PCR were negative. He had no seizures during the next
days and was discharged on the 25th day of admission.
A broad spectrum of neurologic disorders ranging from
seizures to fatal encephalitis/encephalopathy, have been
reported in association with upper respiratory tract
disease due to hMPV. Given the increasing evidence that
hMPV may be one of causative agents rather than an
incidental finding, we suggest that it should be tested
along with other pathogens in children with status
epilepticus and encephalitis.
Keywords: Human metapneumovirus, refractory
status epilepticus
[P-025]
Malnutrition in Spina Bifida
Banu Yazici1, Busra Duzyol2, Huseyin Canaz3, Nursu
Kara4, Ibrahim Alatas3
1
Department of Pediatrics, Florence Nightingale
Hospital, Istanbul Bilim University, Istanbul, Turkey
2
Department of Dieticianary, Florence Nightingale
Hospital, Istanbul, Turkey
3
Department of Neurosurgery, Florence Nightingale
Hospital, Istanbul Bilim University, Istanbul, Turkey
4
Department of Neonatology, Florence Nightingale
Hospital, Istanbul Bilim University, Istanbul, Turkey
The aim of this research is to determine if there’s any
difference between malnutrition in children with spina
bifida and normal population. The study included 112 girl
and 129 boys in total 241 children with spina bifida whom
we tracked their height, weight and head circumference
in our clinic and we compared their results against the
same age healthy children’s percentil. We found that
the Spina Bifida Group is always get behind of healthy
children percentil. The gap starts from 0-3 mounths and
it grows with time. Even Spina Bifida Group’s and control
group’s social and economic statue are same, there is
a significant percentil gap (p<0.05). There’s significant
gap between control group and children with spina
bifida. Measurements of SB group is always behind the
control group and lag increases with age. That’s why we
declare the lag in percentil of children with spina bifida is
not only based on malnutrition but also their condition.
Keywords: Spina bifida, malnutrition, growth percentil
[P-026]
False-positive pneumococcal antigen test in
a case of pediatric pleural empyema
Bendavid Matthieu1, Caseris Marion1, Cointe Aurélie2,
Mariani Patricia2, Pierron Charlotte3, Bidet Philippe2,
Bonacorsi Stéphane4
1
AP-HP, General Paediatrics, Robert-Debré hospital,
F-75019 Paris, France
2
AP-HP, Microbiology laboratory, Robert-Debré hospital,
F-75019 Paris, France
3
AP-HP, Intensive Care unit, Robert-Debré hospital,
F-75019 Paris, France
4
Paris Diderot University, Sorbonne Paris Cité, IAME,
UMR 1137, F-75018 Paris, France
Background&Aims: empyema is a serious complication
of pediatric pneumonia requiring a rapid diagnosis of
the causative agent for effective treatment and good
outcome. The BINAX NOW® Streptococcus pneumoniae
Antigen Card is an in vitro rapid and simple test used for
the detection of S. pneumoniae soluble antigen and may
be used directly on the pleural fluid. We report a falsepositive pneumococcal antigen test in pleural fluid of an
8-year’s old with pleural empyema following aspiration
pneumonia.
Case report: an 8-year-old girl was admitted in
intensive care unit for fever, dyspnea and sepsis evolving
for 24 hours. Medical history included encephalopathy
due to hypoxic-ischemic brain injury, and respiratory
insufficiency managed by tracheostomy. Investigations
showed right empyema. Gram examination on the
pleural fluid was negative but the S. pneumoniae antigen
test performed on it was positive. The patient therefore
received Amoxicillin. Cultures, mass spectrometry and
16S rRNA gene amplification then identified Parvimonas
micra, Eikenella corrodens and Fusobacterium nucleatum.
Microbiological analysis: we separately tested
Parvimonas micra and Eikinella corrodens isolates from
the pleural fluid with the BINAX NOW® S. pneumoniae
test. As we didn’t isolate the strain of Fusobacterium
nucleatum, we challenged another strain of Fusobacterium
nucleatum from our collection. We observed a falsepositive in vitro test for Parvimonas micra and a
negative test for Eikenella and Fusobacterium species.
Finally, we performed specific pneumococcal polymerase
- 28 -
chain reaction on the pleural fluid that proved
negative eliminating a co-infection with this pathogen.
Consequently, the false-positive result for our patient was
due to the presence of Parvimonas micra in the pleural fluid.
Conclusions: clinicians should be aware that BINAX
NOW ® S. pneumonia test performed on fluid from
pleural infection may be falsely positive in case of
anaerobic infection including Parvimonas micra and
should interpret with caution this test result in context
compatible with aspiration pneumonia.
Keywords: empyema, false-positive pneumococcal
antigen test, Parvimonas micra
[P-027]
A rare case of prenatally diagnosed galen
vein aneurysm
Betül ÇINAR1, Royala Babayeva1, Mine Özdil2, Ersin
Ulu2, Yıldız Perk2, Mehmet Vural2, Civan Işlak3
1
İ.Ü Cerrahpasa Medical Faculty Department of
Pediatrics,
2
İ.Ü Cerrahpasa Medical Faculty Department of
Pediatrics, Neonatalogy,
3
İ.Ü Cerrahpasa Medical Faculty Department of
Neuroradiology, Istanbul, Turkey
Background&Aims: Galen vein aneurysms are rarely
seen intracranial malformations which develop as
a result of the presence of an arteriovenous fistula
between choroidal branches of the cerebral artery
and/or branches of the basilar artery and the dilated
veins of the mesencephalon. Aneurysm of Galen
vein malformation (VGAM) is the most common
cerebrovascular malformation symptomatic in neonatal
and infantile periods. Children with VGAM presenting
during the neonatal period have a worse prognosis.
Clinical manifestations include high-output congestive
heart failure, bruit on head auscultation, hydrocephalus
and drug-resistant seizures. Better clinical results and
cognitive function are achieved with endovascular
interventions to occlude interweaving vessels.
Methods: A term, male baby whose prenatal ultrasound
exposed 11mm Galen vein aneurysm, was born with
cesarean section and admitted to the neonatal intensive
care unit. The baby was active neurologically, anterior
fontanelle was normal on palpation but a systolodiastolic
bruit was notable on auscultation. On cardiac examination
a 2/6 systolic murmur was audible. The cranial
ultrasonography and MR venography detected a vascular
malformation in proximity to quadrigeminal cysterna
(13,6 mm). The echocardiography uncovered right
ventricular hypertrophy, PDA, pulmonary hypertension
and atrial septal defect. On the second day, the patient
developed tachycardia, tachypnea and hypotension
and treatment for heart failure was started. On the
6th day, endovascular embolisation was performed
through transarterial approach. After the procedure,
his hemodynamics gradually improved, cardiomegaly
diminished but the patient developed generalized clonic
convulsions, responsive to anticonvulsive treatment. On
the 32nd day he was discharged with normal neurological,
cardiac findings and breastfed.
Results: Galen vein aneurysm is a rare, prenatally
recognizable malformation which must be kept in mind
in a neonate presenting with early onset high-output
heart failure and seizures. Endovascular treatment
seems to be the best primary treatment for VGAM.
Excellent long-term cognitive and functional outcomes
after endovascular occlusions of VGAM are reported.
Keywords: Galen vein, heart failure, seizure
[P-028]
Complications with Rotavirus: Is it worth
vaccination?
Bilge Aldemir Kocabaş1, Adem Karbuz1, Halil Özdemir1,
Anıl Tapısız1, Nurşen Belet1, Haluk Güriz2, Ergin Çiftçi1,
Erdal Ince1
1
Division of Pediatric Infectious Diseases, Department
of Pediatrics, Faculty of Medicine, Ankara University,
Ankara, Turkey
2
Department of Clinical Microbiology, Faculty of
Medicine, Ankara University, Ankara, Turkey
Background&Aims: Rotavirus gastroenteritis (RG)
causes severe fluid and electrolyte imbalance and
development of fatal complications during the course of
the disease, if untreated. Herein, we evaluated the clinical
characteristics, incidence and types of the complications
during RG in 276 patients in this retrospective study.
Methods: We evaluated a total of 276 patients (164
male, 112 female) diagnosed with RG between January
2006 and February 2013 at the department of Pediatric
Infectious Disease. Results: Male to female ratio was
1.5 and the median age at admission was 12 months
(1-102 months). None of the patients had a history of
Rotavirus immunization. While overall complication rate
was 44.2%, the most common complication related
RG was electrolyte imbalance and the second common
complication was septicemia (32.6% and 6.5%,
respectively). Klebsiella, E. coli and Candida species
were the most common isolated microorganisms from
the blood cultures.
Conclusions: RG is still an important cause of morbidity
in children which can cause serious complications such
as sepsis. Lack of rotavirus vaccine in our national
immunization program might contribute the RG related
complications and prolonged hospitalization, health care
expenses, family work loss, as well.
Keywords: complications, Rotavirus, septicemia
- 29 -
[P-029]
[P-030]
Bilge Aldemir Kocabaş1, Adem Karbuz1, Ergin Çiftçi1,
Fırat Beğde2, Selver Ametoglou2, Ali Adil Fouad3, Ayşe
Kalkancı3, Zeynep Ceren Karahan4, Derya Aysev2, Erdal
Ince1
1
Division of Pediatric Infectious Diseases, Department
of Pediatrics, Faculty of Medicine, Ankara University,
Ankara, Turkey.
2
Department of Pediatrics, Faculty of Medicine, Ankara
University, Ankara, Turkey.
3
Department of Medical Microbiology, Faculty of
Medicine, Gazi University, Ankara, Turkey
4
Department of Medical Microbiology, Faculty of
Medicine, Ankara University, Ankara, Turkey
Background&Aims: Kerion celsi (KC) is inflammatory
form of tinea capitis. Herein, we report a ten year-old
boy who had KC related to Trichosporon asteroides
and successfully treated with fluconazole within eight
weeks. This is the first KC case in the literature due to T.
asteroides to our knowledge.
Fatih Çelmeli1, Bilge Aldemir Kocabaş2, Ömer Bek3,
Kemal Gökkuş4
1
Department of Pediatric Allergy-Immunology, Antalya
Education and Research Hospital, Antalya, Turkey
2
Department of Pediatric Infectious Diseases, Antalya
Education and Research Hospital, Antalya, Turkey
3
Department of Pediatrics, Memorial Hospital, Antalya,
Turkey
4
Department of Orthopaedics, Memorial Hospital,
Antalya, Turkey
Background&Aims: Osteomyelitis caused by Salmonella
typhi is rare in patients with no haemoglobinopathies
or immunodeficiency diseases. There have been a
few reports on septic arthritis and osteomyelitis in the
humerus secondary to Salmonella infection in children.
Trichosporon asteroides: A novel ethiological
agent of Kerion celsi in a child
Salmonella typhi septic arthritis and
osteomyelitis in an infant
Results: A 10-year-old boy was admitted to our hospital
with complaints of swelling, discharge and pain behind
of the right ear. There was no trauma history and initial
physical examination revealed a painful and yellow
colored purulent discharge and hair loss at an about
3x2 cm area on the scalp behind the right ear. There
was also a mobile, painful palpable lymphadenopathy
in the postauricular region. White blood cell count and
acute phase reactants were within normal ranges. The
skin lesion was consistent with KC and intravenous
fluconazole (10 mg/kg/day) was initiated taking into
account his previous history of terbinafine treatment.
Also, ketoconazole shampoo was given two days a week.
The reduction of pain and erythema was observed on the
lesion within the first three days of treatment. Discharge
was significantly reduced at the end of the first week and
incrustation was observed on the lesion surface. He did not
have fever episodes during the follow-up. Trichosporon
colonies were detected on Sabouraud Dextrose Agar
(SDA) plates. DNA sequencing was additionally performed
for the molecular identification Trichosporon isolate.
The patient was discharged on the 8th day of intravenous
treatment with oral fluconazole (4 mg/kg/day) next
week. The lesion was found to be significantly resolved
at the control visit. At the end of the 8th week, new hair
formation was observed on the lesion and the treatment
was stopped.
Results: An 11-montn-old male infant was referred to
us with complaints of prolonged diarrhea, fever, and
pain, swelling and restricted movement of left shoulder.
He was born full term neonate and her parents were
not consanguineous. He had no a history of any signs
of immunodeficiency, sickle-cell disease or trauma.
On his physical examination, he had fever (39º C),
swelling and restriction of movement of the left shoulder.
Laboratory tests revealed: absolute neutrophil count
6,160 /mm3, absolute lymphocyte count 4,900/mm3,
absolute monocyte count 1,250/mm3, CRP 137 mg/dl,
sedimentation rate 114 mm/hr. IgG 695 mg/dl, IgM 110
mg/dl, IgA 77 mg/dl, IgE 96 kU/L, anti Hbs 195 mg/dl.
Flow cytometric evaluation of lymphocyte subsets were
as following: CD3 59%, CD4 34%, CD8 18%, CD19 35%,
CD20 35%, CD16-56 3%, HLA-DR %34. Pus and blood
cultures yielded Salmonella. HIV was negative. NBT
was positive. Hemoglobin electrophoresis was normal.
Ultrasonography revealed left shoulder joint effusion.
Radiographs and magnetic resonance imaging of the
left shoulder was taken and showed osteomyelitis in
humerus and the patient was referred to the orthopedic
surgeon. Aspiration was performed which revealed 200
segmented WBCs per high-power field. Intraoperative
left shoulder arthrotomy with irrigation, debridement,
and a synovial biopsy were performed. The joint aspirate
culture was subsequently positive for Salmonella. The
species was later identified as Salmonella serotype typhi.
The organism was susceptible to ampicillin, cefotaxime,
and
trimethoprim/sulfamethoxazole.
Parenteral
ceftriaxone was initiated.
Conclusions: Differential diagnosis with bacterial
pyoderma, abscess may avoid unnecessary and
inappropriate surgical drainage.
Keywords: Kerion celsi, tinea capitis, Trichosporon
asteroides
Conclusions: We report a case of a previously healthy
infant who diagnosed with Salmonella typhi osteomyelitis
of humerus. We think that possible molecular mechanism
might be IL 12Rβ1 or IL 12p40 mutation.
Keywords: osteomyelitis, Salmonella typhi
- 30 -
[P-031]
Atypical Sturge-Weber syndrome
Borbala Zsigmond1, Beata Rosdy1, Katalin Kollar1, Eva
Kovacs2, Gyorgy Varallyay3, Marton Hernadi1
1
Neurology Department, Heim Pal Children Hospital,
Budapest, Hungary
2
CT and Interventional Radiology Department, Heim Pal
Children Hospital, Budapest, Hungary
3
MRI Research Center, Semmelweis University,
Budapest, Hungary
Introduction: Sturge-Weber syndrome is a rare,
congenital neuro-cutaneous disease, which, in typical
cases, contains one-sided facial port-wine stains,
ipsilateral ocular lesions, leptomeningeal angiomas and
epilepsy. However, in atypical cases these abnormalities
can be found without dermal lesions, as well.
The Case: 7- year-old child has been treated with
epilepsy since the age of 6 month. For the neonatal
focal seizures,which were not controlled sufficiently with
carbamazepine alone, valproate was added to therapy.
After three years without manifest seizures the medical
treatment was gradually tapered during the next two
years.
After one-and-a-half year without anticonvulsant
medication the patient experienced cumulative habitual
seizures during a febrile infection at home, ending up
in status epilepticus with focal initiation in our hospital.
Parenteral midazolam was only temporarily effective, so
intravenous valproate loading was successfully applied,
and it was later per oral administered. The postictal EEG
showed serious slowing in the right parieto-occipital
region. Because of the possible infectious trigger of the
seizures, emergency cranial MRI was performed with a
suspicion of parainfectious encephalitis. The MRI showed
significant atrophy of the right parieto-occipital region,
and a streak signal change was observed between the
cortex and the white matter in the same region. The lesion
in question was identified as calcification or subacute
hemorrhage. Cranial CT confirmed it as calcification and
verified the diagnosis of Sturge-Weber syndrome.
Conclusion: This case points out that the phakomatoses
could present themselves even without the characteristic
cutaneous abnormalities.
Keywords: Neurology, Phacomatosis, Epilepsy
[P-032]
Imcomplete Kawasaki Disease in a twomonth old infant
Burcu Karakol1, Burcu Karakayalı1, Burak Deliloğlu1,
Taner Yavuz2, Şirin Güven1, İsmail İşlek1
1
Ümraniye Eğitim ve Araştırma Hastanesi, İstanbul
2
Zeynep Kamil Kadın ve Çocuk Hastalıkları Eğitim ve
Araştırma Hastanesi, İstanbul
Introduction: Kawasaki disease (KD) is an acute febrile
multi-systemic vasculitis of unknown etiology. The
incidence rate is very low in patients younger than 3
months of age. We reported here an infantile case of
incomplete KD aged two months.
Case: A two- month old infant with fever, and mild
diarrhea for three days was admitted to the emergency
department. Physical examination revealed polymorphic
diffuse maculopapular rash and erythema on trunk
and both upper extremities, he had oropharyngeal
erythema, nonpurulent conjunctivitis on both eyes, a
unilateral cervical lymphadenopathy measuring 1.5
cm. His initial laboratory tests included elevated CRP
(7.5 mg/dl), no leukocytosis, hemoglobin of 9.4 g/dl,
440,000/µL platelets. Tests for TORCH infections were
negative. He was admitted to the hospital. On day four,
he had still fever, diarrhea and rash, tests were repated.
A drop of hemoglobin (7. g/dl) and mild thrombocytosis,
hypoalbuminemia, elevated erythrocyte sedimentation
rate (ESR) (78 mm/hr), elevated fibrinogen were
noted. Cultures remained sterile and antibiotics were
stopped. The patient underwent echocardiography;
showing a saccular dilated LCA and LAD. Intravenous
immunoglobuline 2 g/kg and aspirin 60 mg/kg/day were
given on the seventh day of fever. Fever dropped and the
rash disappeared dramatically with treatment. At day
13, laboratory tests showed thrombocytosis (620,000/
µL) and elevated ESR (67 mm /hr). Control echo on the
14th day showed amelioration of findings and aspirin
dose was lowered to antitrombotic dosage. The third
echo on the 35th day of disease showed normal coronary
vascular anatomy. Echocardiography on the second
month remained normal.
Conclusion: Because of higher risk for developing
coronary artery abnormalities (CAA), precise diagnosis
of KD is important. KD should be considered in the
differantial diagnosis of all febrile ilnesses in infants
where the fever persists for more than 5 days.
Keywords: Incomplete, Infant, Kawasaki Disease
[P-033]
Septic arthritis in an 8 month-old,a
challenging diagnosis
Burcu Karadas1, Sule Nursah Ayata2, Nurcan Yusufoglu1,
Zehra Esra Onal1, Cagatay Nuhoglu1
1
Department Of Pediatrics,Haydarpasa Numune
Research and Training Hospital,İstanbul,Turkey
2
Department Of Family Medicine,Haydarpasa Numune
Research and Training Hospital,İstanbul,Turkey
Introduction: Septic arthritis is the invasion of a joint by an
infectious agent. Septic arthritis in infancy is a challenging
clinical problem,because signs and symptoms may be
subtle and overlap with those found in other conditions.
Case presentation: A 8 month-old boy presented
to our institution with an inability to weight bear on
affected limb.His hip joint was pulled by his older sister
a month ago,at that time he was seen at emergency
department, radiologic findings were normal.On his
second presentation the diagnosis was clinched when
he developed more definitive signs and symptoms, with
fever and increased white blood cell count; which are
considered independent variables that best distinguishes
septic arthritis from transient synovitis.The earliest
plain film radiographic findings were soft tissue swelling
around the joint and a widened joint space from joint
- 31 -
effusion,which our case was diagnosed. Magnetic
resonance imaging revealed septic arthritis of hip
joint. He underwent surgery and 4 weeks of antibiotics
administered intravenously.During his stay at our clinic,
he diagnosed with varicella and also complicated with
cellulitis of knee.The entire duration of stay was 4
weeks until normalization of the C-reactive protein level.
We are happy to report a good recovery without any
complications at his 12-month follow up.
(iv, 30 mg/kg/day) for 3 days and 2mg/kg once daily
per oral after pulse therapy. During the period of
interruption that we reduced steroid doses, patient’s
pulmonary hemorrhage attack repeated. Immunological
tests were already normal and no signs of renal
involvement for vasculitis. With all of these findings,
2mg/kg/day cyclophosphamide added to the therapy
at follow-up. We planned to perform open lung biopsy
when patient’s attack ended to check our diagnose.
Conclusions: Septic arthritis of the hip is a true
orthopedic emergency; delay in diagnosis or treatment
may result in irreversible damage to the joint and
disastrous complications in the pediatric patient,including
complete destruction of the articular cartilage and the
underlying epiphysis, loss of the adjacent growth plate,
and dislocation of the joint.
Conclusions: Idiopathic pulmonary hemosiderosis is a
diagnose that exclude other alveolar diseases. Pulmonary
hemorrhage can be seen with atipic systemic involvement
and immunologic signs, expecially in childhood. It should
be kept in mind that patient’s clinic and laboratory signs
can get clear in the follow up period.
Keywords: Pulmonary Hemorrhage, Anemia, Children
With prompt treatment, all complications might be
avoided, and normal function and future growth may be
preserved. The keys to proper management are a high
index of suspicion in any child with joint dysfunction and
strict adherence to the principles for treatment.
Keywords: septic arthritis, early diagnosis, treatment
[P-034]
Management of pulmonary hemorrhage in a
pediatric patient: Case report
Coşkun Armağan1, Seda Şirin Köse2, Suna Asilsoy2,
Nevin Uzuner2
1
Department of Pediatrics, Dokuz Eylul University, Izmir,
Turkey
2
Department of Pediatric Allergy & Immunology, Dokuz
Eylul University, Izmir, Turkey
Background&Aims: We aimed to raise awareness to
management of pulmonary hemorrhage and vasculitis in
pediatric patients.
Methods: This is a case report of pulmonary hemorrhage
in a 13-year-old child.
Introduction: Pulmonary hemorrhage is a clinical
condition characterized by severe clinical signs which
includes respiratory distress, hemorrhage into the
alveolus, cough, hemoptysis and anemia. Bilateral diffuse
alveolar radiopacities are seen on chest radiograph.
Etiology is reviewed by the presence of alveolar
capillaritis, promote lung biopsy and immunofluorescence
staining. In this study, a 13-year old child diagnosed
with pulmonary hemorrhage at our clinic was presented.
Case: 13-year-old boy, referred to our hospital with
anemia in blood count and respiratory distress findings,
consistent with bilateral pulmonary hemorrhage was
detected in tomography. Patient’s pulmonary function
test was FEV1:55%, FVC:52%, FEV1/FVC:103%,
PEF:96%, MEF25-75:59%. All of immunological markers
was negative. We performed flexible bronchoscopy.
Lung biopsy results was non-specific and there were
hemosiderin-containing macrophages in bronchoalveolar
lavage. With all these findings, idiopathic pulmonary
hemosiderosis was thought as preliminary diagnose.
We started to give pulse methylprednisolone therapy
[P-035]
A case of brucella admitting with bicytopenia
and deep thrombocytopenia
Deniz Aygun1, Duhan Hopurcuoglu2, Fatih Varol3, Turkay
Rzayev2, Haluk Cokuras1
1
Division of Pediatric Infectious Diseases, Clinical
Immunology and Allergy, Istanbul University
Cerrahpasa Faculty of Medicine
2
Department of Pediatrics, Istanbul University
Cerrahpasa Medical Faculty, Istanbul, Turkey
3
Department of Pediatric Emergency Medicine, Istanbul
University Cerrahpasa Medical Faculty, Istanbul, Turkey
Introduction: Brucellosis is a widespread and potentially
life-threatening multisystem zoonotic disease caused by
intracelluler, gram-negative, non spore-forming bacteria
of the genus Brucella. Clinical signs and symptoms are
quite variable because all the organs can be affected
by the microorganism. Hematologic findings are also
non-specific and return to normal with appropriate
antimicrobial therapy. Herein, we present a case of
brucellosis presenting with massive hepatosplenomegaly,
bicytopenia and deep thrombocytopenia.
Case: A previously healthy 10-year-old male patient was
admitted with a two-month history of weakness, high
fever, weight loss. He was a member of a family living
in a village and he consumed unpasteurized milk and
milk products. On physical examination he had impaired
general condition with 38.60C body temperature,
firm hepatomegaly of 6 cm and splenomegaly of 9
cm. Laboratory analysis revealed anemia (8.5 g/dl),
leucopenia (2200/mm3) and thrombocytopenia (4000/
mm3). The results of blood chemistry were within normal
limits. Peripheral blood smear and bone marrow revealed
no blast. The tube agglutination test was positive with
1/320 titer for Brucella. Bone marrow culture and
hemoculture yielded gram negative coccobacilli, as
Brucella spp. Rifampicin, trimethoprim sulfamethoxazole
and amikacin combination therapy was started. The
patient is currently in the third week of treatment, he is
asymptomatic and pancytopenia returned normal levels.
Conclusion: Brucellosis is still a challenging worldwide
health problem caused by ingestion of unpasteurized
milk or undercooked meat from infected animals, or
- 32 -
close contact with their secretions, milk and excrement.
Various degrees of hematological manifestations can be
seen during the course of the disease. Brucella should be
kept in mind in patients admitting with bicytopenia and
pancytopenia especially for our country which has a high
Brucella seroprevalence.
Keywords: brucella, bicytopenia, thrombocytopenia
[P-036]
A case of embryonal rhabdomyosarcoma
presenting as parotitis
Deniz Aygun1, Başak Koç2, Emre Gök3, Haluk Cokugras1
1
Division of Pediatric Infectious Diseases, Clinical
Immunology and Allergy, Istanbul University
Cerrahpasa Faculty of Medicine, Istanbul, Turkey
2
Instution of Oncology, Istanbul University Cerrahpasa
Faculty of Medicine, Istanbul, Turkey
3
Department of Pediatrics, Istanbul University
Cerrahpasa Faculty of Medicine, Istanbul, Turkey
Rhabdomyosarcoma(RMS) is the most common soft tissue
neoplasm seen in childhood that usually arises in the head
and neck region. Rhabdomyosarcoma is an uncommon
malignant mesenchymal neoplasm, accounting for 5%
of all childhood malignancies. Unfortunately, it is often
misdiagnosed as common benign conditions. Here we
describe a case of embryonal RMS presenting with
swelling of parotis region and fever mimicking parotitis.
A previously healthy 3 years old boy admitted with a
10 days history of fever, irrability, pain and left sided
swelling of parotis region. Physical examination revealed
a diffuse, tender, unilateral swelling of 6x5 cm in diameter
in the left parotid region. It was hard and not fluctuated.
The rest of the physical examination was unremarkable.
A complete blood count revealed a total leukocyte count
of 14600/mm3, Haemoglobin 10.5 gr/dl, platelet count
as 359,000/mm3. Erythrocyte sedimentation rate was
39 mm/hour, CRP level was 2.5 mg/dl. Ultrasonography
displayed soft tissue swelling with multilobulated septation
in left parotid gland which measured 32x25 milimeters
approximately. Concerning clinical symptoms, increased
inflammatory markers and radiological findings he was
diagnosed as parotitis and empirically, intravenous
ampicillin-clindamycin combination was started. After
four days of therapy, the swelling was not regressed.
Because of the atypical clinical course and unresponsive
to antibiotic treatment, computed tomography was
performed which suggested malignancy. Biopsy was
performed and the histopathological evaluation of
the sample adjusted embryonal rhabdomyosarcoma.
Rhabdomyosarcomas of the parotid gland are the most
lethal forms of salivary gland malignancy in children.
They have a rapid onset and usually present with
advanced disease. So every swelling of parotis gland is
not always parotitis and rhabdomyosarcoma should be
kept in mind in patients unresponsive to antibiotics.
Keywords: Rabdomyosarcoma, parotitis
[P-037]
A case of antenatal diagnosed
hyperimmunoglobulin M syndrome
Deniz Aygun1, Ayper Somer2, Zehra Yavas3, Mine
Kucuk3, Nuran Salman2
1
Department of Pediatric Infectious Diseases,
Clinical Immunology and Allergy, Istanbul University,
Cerrahpasa Medical Faculty, Istanbul, Turkey
2
Department of Pediatric Infectious Diseases and
Clinical Immunology, Istanbul University, Istanbul
Medical Faculty, Istanbul, Turkey
3
Department of Pediatrics, Istanbul University, Istanbul
Medical Faculty, Istanbul, Turkey
Hyper-IgM syndrome (HIGM) is a rare primary
immunodeficiency disorder characterized by severe
defects of both cellular and humoral immunity due
to impaired expression of CD40 ligand on activated T
lymphocytes. Affected patients typically have elevated
or normal serum IgM and decreased Ig G levels.
Herein, we report an antenatal diagnosed hyperIgM syndrome having a sibling of the same disease.
A 2-year-old male patient admitted with gingival redness
and hypertrophy. His brother was diagnosed as X linked
hyper-IgM syndrome at 10 years old due to recurrent
infections and chronic diarrhea. He was the son of nonconsanguineous parents and genetic analysis of the
mother showed a heterozygote pattern, indicating carrier
status. Our patient was also diagnosed antenatally
as X linked hyper-IgM syndrome and gene analysis
of the CD40L gene revealed GG to CC substitution of
Try 140 in 440-441 position. But the parents refused
medical abortus and did not come to hospital until
two years old despite of history of recurrent infection.
The rest of his physical examination was unremarkable.
Laboratory analysis revealed bicytopenia and neutropenia.
Bone marrow aspiration did not reveal malignancy. Flow
cytometric analysis of T, B, and NK cells revealed normal
values. Serum immunoglobulin G level was low, 139
mg/dl, IgA: 47 mg/dl and IgM: 147 mg/dl. Antenatal
diagnosis of hyper-IgM syndrome was confirmed
and he was placed on intravenous immunoglobulin.
Hyper-IgM syndrome is a combined immunodeficiency
disorder characterized by elevated or normal serum
IgM and decreased IgG, IgA, and IgE due to defective
immunoglobulin class switching. Identification of
mutations in this rare disorder will help in genetic
counselling in affected families which could be further
useful in carrier detection and prenatal diagnosis.
Keywords: hyperimmunoglobulin M, antenatal
- 33 -
[P-038]
Ataxia-telangiectasia presenting with
hyperimmunoglobulin M syndrome: report of
two cases
Deniz Aygun1, Ayper Somer2, Mine Kucuk3, Burak Tatlı4,
Nuran Salman2
1
Department of Pediatric Infectious Diseases,
Clinical Immunology and Allergy, Istanbul University,
Cerrahpasa Medical Faculty, Istanbul, Turkey
2
Department of Pediatric Infectious Diseases and
Clinical Immunology, Istanbul University, Istanbul
Medical Faculty, Istanbul, Turkey
3
Department of Pediatrics, Istanbul University, Istanbul
Medical Faculty, Istanbul, Turkey
4
Department of Pediatric Neurology, Istanbul University,
Istanbul Medical Faculty, Istanbul, Turkey
Ataxia-telangiectasia is an immunodeficiency syndrome
with a wide-spectrum of findings including progressive
cerebellar ataxia, oculocutaneous telangiectasia, ionizing
radiation hypersensitivity and recurrent sinopulmonary
infections. Patients usually have low levels of IgG, IgA,
and IgE. About 10% of patients show raised serum IgM
concentrations during the course of the disease, but it is
unusual to find a high level of IgM at onset of the disease.
Herein, we report two cases of ataxia-telangiectasia
presenting with hyperimmunoglobulin M syndrome.
Case 1: A 2-year-old male patient admitted with ataxia
and recurrent infections including pneumonia. He was
the son of consanguineous parents and he had a brother
diagnosed as ataxia-telangiectasia. Physical examination
revealed bilateral ocular telangiectasia and ataxia. Serum
immunoglobulin G level was 0 mg/dl, IgA: 2.5 mg/dl and
IgM: 618 mg/dl. In lymphocyte subtypes CD3 was %42,
CD4 %25, others were normal. Serum alfa feto protein
was elevated. He was diagnosed as ataxia-telangiectasia
with hyperimmunoglobulin M syndrome and started
monthly intravenous immunoglobulin infusion.
Case 2: A 3-year-old female patient admitted with
neuromotor retardation and recurrent sinopulmonary
infections. She was the son of consanguineous parents
and had a cousin diagnosed as ataxia-telangiectasia.
Physical examination revealed only ataxia, the rest of
was unremarkable. Serum immunoglobulin G level was
148 mg/dl, IgA: 44 mg/dl and IgM: 414 mg/dl. The
proportions of lymphocyte subtypes and serum alfa feto
protein were normal. She was also diagnosed as ataxiatelangiectasia with hyperimmunoglobulin M syndrome
and started monthly intravenous immunoglobulin
infusion.
Ataxia-telangiectasia
and
hyper-immunoglobulin
M syndrome are both primary immunodeficiency
diseases caused by different genetic defects. Ataxiatelangiectasia patients presenting with decreased serum
immunoglobulin G and A and with normal or elevated
immunoglobulin M levels are often misdiagnosed as
hyperimmunoglobulin M syndrome.
Keywords: Ataxia-telangiectasia, hyperimmunoglobulin M
[P-039]
Congenital lobar emphysema with glaucoma
and inguinal hernia
Deniz Aygun1, Gonca Tekant2, Yusuf Can Dogan3, Haluk
Cokugras1, Yıldız Camcıoglu1
1
Department of Pediatric Infectious Diseases,
Clinical Immunology and Allergy, Istanbul University,
Cerrahpasa Medical Faculty, Istanbul, Turkey
2
Department of Pediatric Surgery, Istanbul University,
Cerrahpasa Medical Faculty, Istanbul, Turkey
3
Department of Pediatrics, Istanbul University, Istanbul
Medical Faculty, Istanbul, Turkey
Congenital lobar emphysema (CLE) is a rare congenital
abnormality that is characterized by air trapping
and overdistension of one or more lobes of lung with
subsequent compression of adjacent structures leading
to an atelectasis of the remaining lung parenchyma and
mediastinal displacement. CLE presents with recurrent
cough and wheezing within six months of life. Herein, we
report congenital lobar emphysema with glaucoma and
inguinal hernia in a 1 year old boy because of rare assosiation.
A 1 year old boy having glaucoma and inguinal hernia
admitted with history of recurrent pulmonary infections,
chronic cough. Physical examination revealed bilateral
crackles and rhonchi at lower zones. The blood
parametere were normal. Chest X-ray shows hyperlucent
and hyperexpanded left lung with mediastinal shifting to
the right side without any visible visceral pleural lining.
Computed tomography (CT) scan of the thorax revealed
an emphysematous left upper lobe with tracheal and
mediastinal shift to the right side. Both chest X-ray and
CT scan confirmed the congenital lobar emphysema.
On the echocardiography, there was no evidence of
congenital cardiac anomalies. The patient had a left
posterolateral thoracotomy with left upper lobectomy
by pediatric surgery department. He had an uneventful
postoperative course and was discharged from the
hospital within one week. Histopathological examination
of the surgical specimen revealed an emphysematous
parenchyma with peribronchiolar lymphocyte infiltration.
Chest x-ray should be performed in patients having
history of recurrent pulmonary infections to rule out
congenital lobar emphysema.
Cardiovascular abnormalities are the most common
accompanying congenital anomalies to CLE. Renal,
gastrointestinal, musculoskeletal system anomalies are
reported but to our knowledge this is the only case in
the litherature having glaucoma and inguinal hernia with
CLE.
Keywords: Congenital lobar emphysema, glaucoma,
hernia
- 34 -
[P-040]
A case of idiopathic hypereosinophilic
syndrome in a two years old girl
[P-041]
Deniz Aygun1, Hande Kumkumoglu3, Turkay Rzayev2,
Hilmi Apak3, Tiraje Celkan3, Haluk Cokugras1
1
Department of Pediatric Infectious Diseases,
Clinical Immunology and Allergy, Istanbul University,
Cerrahpasa Medical Faculty, Istanbul, Turkey
2
Department of Pediatrics,Istanbul University,
Cerrahpasa Medical Faculty, Istanbul, Turkey
3
Department of Pediatric Hematology and Oncology,
Istanbul University, Cerrahpasa Medical Faculty,
Istanbul, Turkey
Idiopathic hypereosinophilic syndrome(HES) is defined
as the presence of prolonged and over-production of
eosinophils (>1.5 × 109/L) without an identifiable
underlying cause. The clinical manifestations range
from asymptomatic eosinophilia to aggressive disease
complicated by life-threatening end organ involvement.
Herein, we report idiopathic hypereosinophilic syndrome
in a two years old girl, because it is extraordinarily rare
in children.
A previously healthy 2 years old girl admitted with fever
and abdominal pain. She did not have any history of
asthma, allergic disease or any disease before. Her
physical examination was completely normal.
A complete blood count (CBC) showed significant
eosinophilia (77%), with an abso¬lute esosinophil
count of 49,100. The total leukocyte count was 63,600/
mm3, haemoglobin 10.8 gr/dl and platelet count
220,000/mm3. Erythrocyte sedimentation rate, CRP and
laboratory analysis were normal. Serum immunoglobulin
levels were normal. Food allergy panel was negative. The
chest x-ray, echocardiography and ultrasonography were
normal. Investigations for parasitic infections, including
toxocariasis, enterobius vermicularis and feces analysis
were all negative. Bone marrow was full of eosinophils, but
otherwise, including immunophenotype and cytogenetic
analysis, it was normal. Serum tryptase level was
normal. Purified protein derivative skin test was negative.
A diagnosis of the hypereosinophilic syndrome was made
on the basis of the presence of hypereosinophilia with no
other identifiable cause or organ involvement. Treatment
with prednisolone was started in a dose of 1 mg/kg/day.
Hypereosinophilic syndromes is a rare condition in
the pediatric age-group with a wide range of clin¬ical
manifestations.
Hypereosinophilic
syndromes
is
diagnosed after exclusion of underlying diseases known
to cause and the natural course of the disease is usually
self-limiting in children as in our patient.
Keywords: hypereosinophilic syndrome, child,
idiopathic
The effect of phototherapy treatment in
the neonatal period, on the development
of asthma, allergic rhinitis and atopic
dermatitis
Mehtap Sak, Mahmut Doğru, Feyza Mediha Yıldız,
Serpil Değirmenci, Özlem Temel, Özlem Erdede, Derya
Gültekin Başboğa
Zeynep Kamil Training and Research Hospital
Background&Aims: Icterus is a visible symptom of
indirect hyperbilirubinemia. When serum total bilirubin
(STB) level exceeds 5-6 mg/dl, icterus becomes visible to
eye. Within the first week of birth, icterus can be seen in
60-80% of newborns and more than 80% of premature
babies. 10-15% of the patients with hyperbilirubinemia
should be treated with phototherapy. Allergic diseases
including asthma, allergic rhinitis and atopic dermatitis
are frequently observed in childhood period and the
frequency is increasing for the last 30 years, especially
in developed countries. Genetic effects cannot be the
only reason behind this increase, environmental factors
should be considered as well.
Methods: This study analyzes 621 children between the
ages of 3 and 17, including a patient group of 371 who
received phototherapy and a control group of 250 who
did not receive phototherapy. ISAAC survey was applied
to all cases to diagnose allergic diseases. For patients
with allergic diseases, eosinophil and total IgE levels
were analyzed and skin prick test was applied.
Results: In 25.5% (87 patients) of patient group and
27.2% (68 patients) of control group diagnosed asthma
and/or allergic rhinitis and/or eczema were observed.
There was no meaningful statistical different between
patient and control groups in terms of diagnosed
asthma and/or allergic rhinitis and/or atopic dermatitis
(p=0,290). Atopy distribution in patient and control
group is respectively 50% (16 patient) and 57,8% (22
patients). In skin tests applied to patients with allergic
diseases in patient and control groups sensitivity (atopy)
diagnose did not show meaningful statistical difference
(p=0.509).
Conclusions: In this study, we analyzed the relationship
between phototherapy and the frequency of childhood
asthma, allergic rhinitis and atopic dermatitis diagnosis.
As a result of this study, we did not find a meaningful
relationship between phototherapy treatment and
asthma, allergic rhinitis and atopic dermatitis.
Keywords: phototherapy, asthma, atopic dermatitis
- 35 -
[P-042]
[P-043]
The general features of children and
adolescents with Hashimoto’s Thyroiditis
Alstrom Syndrome: A case report
Didar Arslan, Heves Kırmızıbekmez, Feyza Mediha
Yıldız, Özlem Temel, Özlem Erdede, Serpil Değirmenci,
Derya Gültekin Başboğa
Zeynep Kamil Training and Research Hospital
Background&Aims: Hashimoto’s thyroiditis is the most
common reason of goiter and acquired hypotyroidism in
children and adolescents. It is an autoimmune disease
intrinsic to thyroid tissue mediated by genetic and
environmental factors. In this study, we aimed to review
our patients’ data with Hashimoto’s thyroiditis and
compare results with literature findings.
Methods: In this study, patients data with Hashimoto’s
thyroiditis, who were being followed up in Zeynep Kamil
Training and Research Hospital, Pediatric Endocrinology
Department were reviewed retrospectively.
Results: A total of 114 patient were evaluated; 102
female (89,5%) and 12 male (10,5%), the ratio of female
to male was 8,5/1.The average age of the patients at
diagnosis was 11,91±3,26 years. Based on symptoms,
23 patient (20,7%) had swelling in neck,19 (17,1%) had
increased weight, 5 (4,5%) had the complaints of short
stature and growth retardation. 8 patient were tested
(7%) because of accompanying Diabetes Mellitus, and
6 (5,3%) for a positive family history of Hashimoto’s
thyroiditis, 10 (8,8%) patient were diagnosed through
routine laboratory tests. In the examination of thyroid
gland on the first admission; 55 (48,2%) patients have
goiter. Laboratory evaluations revealed; 47 (41,2%)
patients were euthyroid, 46 (40,4%) patients were
subclinical hypothyroid, 10 (8,8%) patients were
overt hypothyroid, 3 (2,6%) patients were subclinical
hyperthyroid, 8 (7,0%) patients were overt hyperthyroid.
Antithyroid peroxidase antibodies were positive in 109
(95,6%) of the patients and antithyroglobulin antibodies
were positive in 105 (92,1%) of the patients. Nodule
was found in 22 of patients determined by thyroid
ultrasonography. Radiation exposure was statistically
significant between groups: 7 (13,2%) patient under
12 years old, only 1 (1,9%) patient over the age of 12
years.
Conclusions: The patients who have clinical and
laboratory evidence for thyroid disease should be tested
for autoimmune thyroiditis.
Keywords: children, adolescents, Hashimoto’s
Thyroiditis
Didem Ocal Topcu, Gamze Ozgurhan, Bahar Caran,
Bulent Hacıhamdioglu
Suleymaniye Maternity and Children’s Training and
Research Hospital, Department of Pediatrics, Istanbul,
Turkey
Introduction: Alstrom syndrome is a very rare
autosomal recessively inherited disorder that can lead
to infantile-onset of dilated cardiomyopathy, blindness,
hearing impairment, obesity, severe insulin resistance,
hepatic and renal dysfunction. We report a 14 year-old
boy who diagnosed as Alstrom Syndrome.
Case Presentation: A 14-year-old boy was admitted
to pediatric endocrinology department due to obesity.
He was the first child of consanguineous parents,
and he was born with a normal birth weight. There
was a history of transient heart failure due to dilated
cardiomyopathy in infantile period. Also he has been
followed by retinal dystrophy which was first detected in
infantile period. He has been treated with levothyroxine
and metformine because of hypothyroidism and insulin
resistance respectively. Severe acanthosis nigricans
was noted in the neck, axillary region and fingers
(Figure 1) on physical examination. His testes were
15/15 ml with normal penile length on external genital
examination. His anthropometric measures were as
follows: weight 107,8 kg (>95th percentile), height 172
centimeters (50-75th percentile). His body mass index
was 36.2 kg/m2 (>95th percentile).His blood pressure
was measured as 140 mmHg/90 mmHg. He had been
suffering from photophobia, decreased vision and
hearing loss for a long time. Cone-rod dystrophy and
sensorineural hearing loss was detected by fundoscopic
examination and audiogram respectively. In laboratory
investigations; serum fasting glucose 98 mg/dl, fasting
insulin 285.2 mIU/ml, C-peptid 13.18 ng/ml, HbA1c:
7.8%, alanine aminotransferase 131 IU/l and aspartate
aminotransferase 80 IU/l, creatinine 0.8 mg/dl. Patient
had hypertriglyceridemia with low HDL cholesterol level
(361 and 29 mg/dl respectively). He has been diagnosed
as Alstrom syndrome clinically.
Conclusions: There is a need of detailed medical
history and physical examination by pediatricians to
confirm diagnosis of rare syndromes. Alstrom Syndrome
should be kept in mind by pediatricians in obese children
with early onset severe insulin resistance and retinal
dystrophy.
Keywords: Alstrom syndrome, insulin resistance,
retinal dystrophy
- 36 -
[P-044]
[P-045]
Dilara Ece Toprak1, Tanyel Zübarioğlu2, Ertuğrul
Kıykım2, Mine Aslan3, Mehmet Şerif Cansever4, Ayşe
Çiğdem Aktuğlu Zeybek2
1
Istanbul University, Cerrahpasa Medical Faculty,
Department of Pediatrics, Istanbul, TURKEY
2
Istanbul University Cerrahpasa Medical Faculty,
Department of Pediatrics Division of Nutrition and
Metabolism, Istanbul, TURKEY
3
Istanbul University Cerrahpasa Medical Faculty,
Department of Radiology, Istanbul, TURKEY
4
Istanbul University, Cerrahpasa Medical Faculty Central
Laboratory, Istanbul, TURKEY
Wolman disease is caused by the complete loss of lysosomal
acid lipase (LAL) activity which is essential for hydrolysis
of cholesterol esters and triglyserides. It presents with
vomiting, diarrhea, poor weight gain and hepatomegaly
subsequently leading to death in early infancy. Diagnosis
is based on the evaluation of enzymatic activity for LAL
and LIPA gene sequencing. Here, an 18 days old female
patient who was diagnosed as Wolman disease is reported.
Diagnosis was confirmed with LIPA gene sequencing by
showing novel mutation that was not reported before.
An 18 days old female patient was admitted to our department
for the etiological evaluation of hepatosplenomegaly and
elevated liver enzymes. The first two children of the
family who had similar complaints died in early infancy.
Her physical examination revealed mild coarse facial
features, mild macroglossia and hepatosplenomegaly.
In her initial laboratory investigations, elevated plasma
triglyceride and total cholesterol levels accompanied by
remarkably reduced plasma high density lipoprotein level
was found. Her liver enzymes were moderately elevated
and she had a mild hypoalbuminemia. Abdominal
sonography revealed hepatosplenomegaly and surrenal
calcification bilaterally. Magnetic resonance imaging of the
abdomen demonstrated significant enlargement of liver
and spleen, as well as the surrenal glands. Having a family
history of two siblings’ death, elevated liver enzymes
and dyslipidemia in our patient and radiological findings
led us to consider LAL deficiency in differential diagnosis.
Decreased LAL activity was detected in dried blood
sample. LIPA gene sequence was performed to confirm
the diagnosis, and she was found in homozygote state for
novel mutation that has not been previously described in
the literature before.
Wolman disease is a rare cause of hepatic failure
that causes death in early infancy. Diagnosis should
be considered in the existence of dyslipidemia and
hypertransaminasemia. Diagnostic confirmation should
be performed for genetic counseling.
Keywords: Wolman Disease, LAL deficiency, genetic
mutation
Dilara Fucucuoglu, Ozlem Bostan Gayret, Meltem Erol,
Ozgul Yigit, Emine Erdogan Kutanis, Ahmet Sener
Bagcilar Training and Research Hospital, Pediatrics,
Istanbul, Turkey
Background&Aims: Recurrent abdominal pain is a
common complaint in childhood. Abdominal epilepsy
(AE) is a very rare cause of this clinical entity which
is characterized by episodic paroxysmal abdominal
and periumbilical pain, various abdominal complaints,
specific abnormalities on electroencephalography (EEG)
and good response to antiepileptic drugs.
Wolman Disease In A Turkish Infant: A Novel
Mutation Of Lysosomal Acid Lipase Gene
An Uncommon Cause of Abdominal Pain:
Abdominal Epilepsy
Case: In this report, we present the case of a 12-year-old
girl diagnosed with AE who was admitted to the emergency
department because of recurrent periumblical abdominal
pain, vomiting and dysuria. She had been admitted to
the hospital several times in the last month. Her medical
history did not include seizures or trauma. She had
been followed up by pediatric nephrology department
for having bilaterally grade 4 vesicoureteral reflux. One
year ago she had bilaterally Cohen operation. She was
hospitalized several times for urinary tract infections and
uses drugs for hypertension, antibiotics for prophylaxia.
On physical examination, the patient’s weight was 43
kg (50 p), and her height was 147 cm (50 p). System
examinations were normal. Routine biochemical tests,
acute phase reactants, hemogram, complete urinalysis
and blood gas analysis were normal. There were no
parasites or occult blood on stool examination. Urine
and stool cultures were negative. Familial Mediterranean
fever (FMF) gene mutation analysis and serum amiloid-A
level were normal. The patient was assessed in the
pediatric surgery department using abdominal USG and
supine and erect abdominal graphy; however, pathology
requiring surgical intervention was not detected.
Electroencephalography revealed epileptiform activity
characterized by sharp and slow wave complexes.
Valproic acid was started. After treatment with the drug,
the abdominal pain attacks did not recur.
Conclusions: After exclusion of known causes, diagnosis
of AE should be considered in children with episodes of
paroxysmal abdominal pain. Anticonvulsant therapy
should be initiated after a diagnosis of AE.
Keywords: Abdominal epilepsy, recurrent abdominal
pain, childhood
[P-046]
Spontaneous Pneumomediastinum in an
4-Year-Old Girl
Okan Yüce, Dilara Fucucuoglu, Ozgül Yiğit, Meltem Erol,
Zübeyde Kilinc Yaprak
Bagcilar Treaning and Research Hospital, Istanbul,
Turkey
Background&Aims:
Pneumomediastinum
is
an
uncommon entity in pediatric practice. It is defined as
free air or gas contained within the mediastinum, which
- 37 -
almost invariably originates from the alveolar space or
the conducting airways. It is rarely symptomatic and
diagnosed coincidentally. Clinical appearance spreads
from asymptomatic to the life-threatening respiratory
insufficiency. Spontaneous pneumomediastinum in
children mostly caused by elevated intrathoracic
pressure, but malnutrition can decrease the quality of
the alveolar walls. Thoracic pain and dyspnea are the
most common symptoms. Pneumomediastinum is
diagnosed by posterior-anterior and lateral chest X-ray
radiologically. In this article we present a 4 years old girl
with spontaneous pneumomediastinum.
Case: Our patient presented to emergency room with
retrosternal chest pain. Her personal and family medical
history revealed no significant findings. She wasn’t injured.
Body weight was 13.4 kg(3-10 p). Her vital signs were
normal: hemoglobin oxygen saturation 99% in room air,
heart rate 80 beats/min, respirations rate 28/min, blood
pressure 88/63 mm Hg and body temperature 36.8°C.
Electrocardiography was normal. Physical examination
revealed no visible haematoma, echymosis, redness
or swelling on the chest wall. There was no palpable
tenderness or subcutaneous emphysema over the neck,
chest, face or shoulders. There weren’t any external
signs of sternal and rib contusions or fractures. On the
chest radiogram there was a suspect thin band of gas
revealed between the heart and diaphragm. Computed
tomograpyh of the chest revealed the presence of air
in the parabronchial and perivascular connective tissue
and around the anterior to the heart. We consigned the
patient to a university hospital to the pediatric surgery
department.
Conclusions: In this article we want to emphasize that
pneumomediastinum should be considered in children
with chest pain without distinct clinical signs and
symptoms.
Keywords: Spontaneous Pneumomediastinum, Chest
pain, childhood
[P-047]
Vitamin D deficiency’s effect on the severity
of bronchiolitis
Huseyin Kaya, Ozgul Yigit, Meltem Erol, Ozlem Bostan
Gayret, Dilara Fucucuoglu, Can Polat
Bagcilar Treaning and Research Hospital, Pediatric
Department, Istanbul, Turkey
Background&Aims: Vitamin D deficiency is frequent
in children. Vitamin D takes an important role in bone
metabolism, it also takes place in inflammatory conditions
and in the course of infections. Bronchiolitis is a common
infection in children younger than 2 years old. The aim of
this study is to try to determine the relationship between
vitamin D deficiency and the severity of bronchiolitis.
Methods: We questioned in children younger than
2 years old who presented to the children emergency
department in Bagcilar Training and Research hospital,
the bronchiolitis frequency and the usage of the Vitamin
D. Blood samples were taken from all children to
measure the serum vitamin D levels. We separated the
children in two groups according to the severity of the
bronchiolitis: mild and severe. In classification we used
the data hemoglobin oxygen saturation in room air, heart
rate per minute, respirations rate per minute, having
apnea, cyanosis and retractions. It has been classified
statistically.
Results: In our study there were 112 children between
the ages 6,1 ± 4,9 months (median: 5 month). 47
children were female (%42) and 65 were male (% 58).
The levels of vitamin D were significantly lower in severe
group than the mild one. In the hospitalized group the
levels were significantly lower than the outpatient group.
The level of Vitamin D did not differ significantly the
number of bronchiolitis attack.
Conclusions: In our study we found that vitamin D takes
an important role in the severity of bronchiolitis. If we
can avoid the children from the vitamin D deficiency, we
believe that we can reduce the duration of hospitalization.
Keywords: Vitamin D deficiency, bronchiolitis, chilhood
[P-048]
Vitamin B12 Deficiency in infancy
Gulsum Karakus, Ozgul Yigit, Ozlem Bostan Gayret,
Meltem Erol, Dilara Fucucuoglu, Esra Ozcan
Bagcilar Treaning and Research Hospital, Pediatric
Department, Istanbul, Turkey
Background&Aims: Vitamin B12 defiency is most
ımportant cause of megabalastıc anemia. In this report,
we presented two infant that exclusively breastfed
by their mothers who consumed less animal protein.
Case 1: Twelve month old male infant who exclusively
breastfed, was admitted to hospıtal with paleness,
vomiting, irritability, failure to gain weight, growth
retardation, cough and breathing difficulty. His body
weight and height were under 3 percentage. He was
letharjic, hypotonic, he had hyperpigmention of palms ve
knuckles. His lung finding has compatible with pneumonia
and he has hepatomegaly. His hemoglobin was 5,9g/
dL, hematocrit 18%, MCV 90fl, plt: 41000 /mm3, the
neutrophils were seen as hypersegmented. Iron and ferritin
levels, biochemical profile and urine test results were
normal, with normal blood and urine aminoacidography
and plasma homocysteine level. Serum vitamin B12 level
was 76pg/mL and serum folate level was 12,3ng/m.
Case 2: Eleven month old female infant who exclusively
breastfed was admitted paleness, growth retardation
and failure to gain weight. She was irritable, letharjic,
hypotonic. Her body weight and height were 10
percentage. Her hemoglobin was 5,8g/dL, hematocrit
17%, MCV 113 fl, plt: 118 000 /mm3, the neutrophils
were seen as hypersegmented. Iron and ferritin levels,
biochemical profile and urine test results were normal,
with normal blood and urine aminoacidography and
plasma homocysteine level. Serum vitamin B12 level
was 30pg/mL and serum folate level was 15,6ng/m.
The patients mother’s vitamin B12 level were low.
The patients were given intramusculer cobalamin
(200 μg/day for first week). After the start of therapy,
their hemoglobin levels are rapidly improved, and a
clinical improvement was observed after a few weeks.
- 38 -
Conclusions: During infancy, vitamin B12 deficiency
may cause serious hematologic and neurological
problems. The treatment of vitamin B12 deficiency is
very crucial to prevent permanent neurological damage.
Keywords: Vitamin B12, infancy, neurological deficit
[P-049]
Milroy Disease in a newborn
Can Polat, Meltem Erol, Ozlem Bostan Gayret, Sahin
Hamilcıkan, Ozgul Yigit, Dilara Fucucuoglu
Bagcilar Treaning and Research Hospital, Pediatric
Department, Istanbul, Turkey
Background&Aims: Milroy disease is characterized by
lower-limb lymphedema. Milroy disease, also known as
primary congenital herediter lymphedema, is a genetic
disorder. FLT4 (VEGFR3) is the only gene known to be
associated with Milroy disease. Milroy disease is the
rarest form of inhareted lymphedema. The disease
is inhareted in an autosomal dominant manner. The
most prominent feature at birth of Milroy’s disease
is swelling in the feet. In this report we presented
a newborn who diagnosed Milroy disease whose
mother was also diagnosed with the same disease.
Case: Our patient was given birth in an unknown
gestastional week from an unfollowed syrian mother.
His birth weight was 4140. He was admietted to the
newborn intensive care unit. On physical exemination
he had distinct lower-limb lymphedema and a systolic
1/6 degree murmur. Routine biochemical tests, acute
phase reactants, hemogram, complete urinalysis and
blood gas analysis were normal. There was no pathology
in abdominal ultrasound graphy. On the transfontanel
ultrasound graphy there were two cyctic lesions in the
caudothalamic channels. The left one’s size was 3,5*2
mm, the right ones 4,1*2 mm. In the echocardiography
we determined an atrioseptal defect. A lymphosintigraphy
was planned. We referated the patient to the medical
genetic department
Conclusions: Milroy disease is a rare condition in
pediatric clinics. We want to remember this rare disease
because of this case with lower-limb lymphedema
Keywords: milroy, lower-limb lymphedema, newborn
[P-050]
Two Patients With 22q11 Deletion Syndrome
Presenting With Short Stature And Palatal
Insufficiency
Dilem Eriş, Gamze Özgürhan, Vildan Küçüksümbül,
Rabia Nur Temiz, Bülent Hacıhamdioğlu
Suleymaniye Maternity and Children’s Training and
Research Hospital, Department of Pediatrics, Istanbul
22q11 Deletion Syndrome (22q11DS, DiGeorge
Syndrome) presents with a very wide phenotypic
spectrum, including the following: facial dysmorphisms;
cardiac defects; velopharyngeal insufficiency; thymic
hypoplasia; parathyroid hypoplasia; developmental
delay. Growth deficiencies are common in patients,
particularly during infancy and early childhood. Here, we
report two children who diagnosed as 22q11DS while
have been evaluated by short stature.
Both patients were evaluated with short stature. Patient
1 was a 12-year-old girl and there was a history of
palatal insufficiency (hypernasal speech and nasal
regurgitation), which was occurred after adenoidectomy
when she was eight years old. She was followed with
left renal agenesis and atrioventricular septal defect.
On physical examination, her height was 132.5 cm
height (-3.7 SDS) she had mild facial dysmorphism
(Figure-1), goiter, cubitis valgus, abdominal adiposity
and rhinolalia. Patient 2 was a 7-year-old girl, and there
was history of cleft palate surgery and adenoidectomy.
She had medical history of frequent upper respiratory
infections, speech difficulty and nasal regurgitation.
On physical examination, her height was 113 cm
height (-1.81 SDS) she had mild facial dysmorphism
(Figure-2) and rhinolalia. Both patients had palatal
insufficiency, mild facial dysmorphism and frequent
upper respiratory infections; therefore, FISH screening
had be planned for 22q11.2 deletion. FISH analysis
revealed chromosome 22q11.2 deletion both patients.
The decision to test for 22q11DS is easy if multiple
defects and/or symptoms associated with the syndrome
are present simultaneously. However, physicians should
note that the signs and symptoms might be subtle.
FISH screening should be planned for 22q11DS in every
child with symptoms associated with velopharyngeal
insufficiency. Short stature is associated with a number
of syndromes, there is need detailed medical history and
carefully physical examination for correct diagnosis of
these syndromes.
Keywords: 22q11, Palatal Insufficiency, Short Stature
[P-051]
A Girl Newborn with Male Phenotype
Diagnosed as Congenital Adrenal
Hyperplasia
Dr. Mahmut Can Kızıl1, Prof.Dr. Birgül Kırel2, Dr. Tuğba
Barsan Kaya3, Dr. Meliha Demiral2, Doç.Dr.Özge
Aydemir3, Yrd.Doç.Dr.Hüseyin Aslan4
1
Pediatrics and Child Health, Osmangazi University
Medical Faculty, Eskişehir, Turkey
2
Pediatric Endocrinology Department, Osmangazi
Unıversity Medical Faculty, Eskişehir,Turkey
3
Neonatalogy Department, Osmangazi University
Medical Faculty, Eskişehir, Turkey
4
Department of Medical Genetics, Osmangazi Unıversity
Medical Faculty, Eskişehir, Turkey
In childhood, congenital adrenal hyperplasia is the most
common cause of adrenal insufficiency. It is an autosomal
recessive inherited disease that is characterized with
enzyme deficiencies in steroidogenesis. 21-hydroxylase
deficiency with salt losing form is the most common type.
CASE REPORT: Three weeks old infant was referred
to our hospital for deteoriating in clinical condition with
hyponatremia and hyperpotassemia after one week
follow up in another hospital.He was born to a 23 years
- 39 -
old mother at 38th gestational weeks and did not need
resuscitation. On physical examination body weight 2800
g, height 51 cm.He showed 2nd degree dehydratation
symptoms, his genital examination was compatible with
male sexuality, uretral orifice was in normal localization
(compatible with prader stage 5 )but his gonads were
not palpable.Laboratory evaluation revealed sodium:112
mEq/l, potassium:7,9 mEq/l,glucose:87 g/l, 17(OH)
progesteron:92
ng/ml,
DHEA-SO4:113,4
ug/dl,
testosteron:70,7 ngdl, androstenedion:22,03 ng/ml.
He was treated for adrenal crisis and congenital adrenal
hyperplasia –salt wasting type. Karyotype analyse was
resulted as XX and SRY gene research was resulted as
negative.Genetic analysis for 21-hydroxylase deficiency
was resulted as I2 splice and Del 8bp E3 homozygot
mutations positive. Council consisting of geneticist,
pediatric endocrinologist, neonatologist and pediatric
surgeon concluded that feminizing corrective surgery is
required.
DISCUSSION: Female newborns with complete
deficiency of 21-hydroxylase may have full developed
male genitalia and if unrecognized, they are growth up as
boys.Careful examination reveals undescended gonads
which can be a clue for congenital adrenal hyperplasia.
Early diagnosis, treatment and surgery can prevent
adrenal crisis in this patients, it can also prevent families’
social and pshycological problems.So congenital adrenal
hyperplasia is one of the most important disease should
be implemented in national disease screening program
in Turkey because of the high rates of intermarriage.
Keywords: congenital adrenal hyperplasia,
21-hydroxylase deficiency, adrenal crisis
[P-052]
A Case with Fever and Rash Later Diagnosed
as Hemophagocytic Syndrome
Durdugül Ayyıldız Emecen1, Sermin Özcan1, Gülperi
Yağar Keskin1, Dildar Bahar Genç1, Zeynep Yıldız
Yıldırmak1, Gülşen Köse1, Ahmet Naci Emecen2
1
Şişli Hamidiye Etfal Education and Research Hospital,
Pediatric Clinics, Istanbul, Turkey
2
Istanbul Medeniyet University Göztepe Education and
Research Hospital Infectious Disease Department,
Istanbul, Turkey
Background&Aims:
Hemophagocytic
syndrome
(hemophagocytic lymphohistiocytosis, HLH) is a
potentially fatal phenomenon characterized by the
activation of macrophages and T lymphocytes,
dysfunction of cytotoxic T lymphocytes and naturel killer
cells, overproduction of cytokines and hemophagocytosis
leads to fever, hepatosplenomegaly and pancytopenia.
HLH is divided into familial and secondary subtypes.
With this case we aim that HLH should be kept in mind
with febrile children despite treatment.
Case: 3.5 months aged girl admitted to hospital with fever,
millimetric maculopapular rash in scalp and body for two
days. Parents were not relative. Respiratory, cardiac and
neuromotor examinations were normal. Liver was 4 cm
and spleen was 3 cm palpable. Hb: 9.9 g/dl, WBC:16000/
mm3, Neu:3040/mm3, Le:10570/mm3, Plt:18000/mm3,
CRP:20 mg/L. No atypic cells in peripheral blood smear.
Ceftriaxone and 1 gr/kg IVIG started with the diagnosis
of sepsis. Fever, notropenia and trombocytopenia
continued at the third day of treatment and the second
dose of IVIG applied. No atypical cells were observed
in bone marrow aspiration. Lymphocyte predominance
and one macrophage with phagocytosed erythrocytes
were seen; hypertriglyceridemia, hyperferritinemia,
hypofibrinogenemia detected and diagnosis of HLH
was made. Perforin mutation, TORCH panel, EBV
and Parvovirus tests were negative. CMV IgM was
positive but CMV PCR was negative. Immunoglobulines
and lymphocytes subtypes were normal. Fever and
pancytopenia persisted and dexamethasone 10 mg/kg
started. Bone marrow aspiration was repeated on the
15th day of dexamethasone to indicate the response
to the treatment and again phagocytosed erythrocytes
were seen. The patient received third dose of IVIG but
hepatosplenomegaly didn’t resolved. Neutrophil count
was >1000/mm3 but no significant improvement in
platelet and hemoglobin count observed so HLH 2004
protocol and bone marrow transplantation planned.
Results: Because of HLH is a rare but fatal disease; it
must be considered in patients with prolonged fever,
hepatomegaly and pancytopenia. Early initition of
treatment may be life-saving.
Keywords: hemophagocytic syndrome, persistent
fever
[P-053]
A thanatophoric dysplasia type I case with
the mutation of p.R248C on FGFR3 gene
Ebru Atas Aslan1, Hamdi Ograg2, Suzan Sahin2,
Abdullah Barıs Akcan2, Huriye Nursel Elcioglu3,
Munevver Kaynak Turkmen2
1
Department of Pediatrics, Faculty of Medicine, Adnan
Menderes University, Aydın, Turkey
2
Division of Neonatology, Department of Pediatrics,
Faculty of Medicine, Adnan Menderes University, Aydın,
Turkey
3
Division of Genetics, Department of Pediatrics, Faculty
of Medicine, Marmara University, Istanbul, Turkey
Introduction: Thanatophoric dysplasia (TD) represents
the most common lethal skeletal dysplasia (1).
Molecular genetic analysis of the fibroblast growth
factor receptor 3 (FGFR3) gene is useful for the prenatal
diagnosis of TD, because FGFR3 is the only gene known
to cause TD (2, 3).
Here, a TD case with suspicion of the disease in prenatal
ultrasonography, born with the dysmorphic features and
confirmed diagnosis with molecular analysis is presented.
Case: A baby boy, weighted 2985 grams, born at the
37th gestational week by cesarean section with APGAR
scores of 7/8 as the first baby of the family, was
hospitalized due to respiratory distress and dysmorphic
features. Amniosynthesis performed due to the shortness
of the longbones on ultrasonography during the second
trimester revealed constitutional karyotype. On physical
examination, the length of the baby was 39 cm (<10p),
weight 2985 g (50-75p) and head circumference 39 cm
(>90p). The baby had hypotonia, short neck, depressed
nasal root, frontal bossing, edema of the scalp and
- 40 -
marked shortening of the longbones. He was intubated
due to respiratory insufficiency and died on the 162nd
day of his life. TD type I diagnosis was confirmed by
molecular analysis of FGFR3 gene which showed
heterozygote p.R248C mutation.
Discussion: TD, as could be imagined from its
name, is a very severe congenital anomaly and most
of the cases are either stillbirth or lost during the
first several hours of their life due to respiratory
insufficiency. The rear only few case reports in the
literature regarding to TD cases who could survive
beyond the childhood period (4). Molecular diagnosis
can relieve parental anxiety in such a low risk couple.
This case is presented due to the relative long life span of
a baby with TD and whose diagnosis could be confirmed
by molecular analysis during his hospital stay.
Keywords: thanataphoric dysplasia, FGFR3, genetic
counselling
[P-056]
[P-054]
Macrophage activation syndrome secondary
to systemic juvenile idiopathic arthritis
Elif Arslan, Soner Sazak, Saniye Berna Hamilçıkan,
Emine Türkkan, Yelda Türkmenoğlu, Muhammet
Nurullah Yakut, Ozan Özkaya
Okmeydanı Training and Research Hospital, Department
of Paediatric, Istanbul
Macrophage activation syndrome (MAS) is caused by
the excessive activation and uncontrolled proliferation
of T-cells and well-differantiated macrophages. MAS has
been reported in patients with almost any rheumatic
diseases and most strongly associated with Systemic
Juvenile Idiopathic Arthritis (SJIA).
MAS can be dignosed by the presence of 5 of the
following: fever, splenomegaly, cytopenia of 2 cell
lines, hypertriglyceridemia or hypofibrinogenemia,
hyperferritinemia, elevated soluble CD 25 (interleukin-2
receptor), reduced or absent NK cells, and bone marrow,
CSF, or lymph node evidence ofhemophagocytosis.
A case of 7 year old boy is presented here who refered
to hospital with arthritis and fever. After hospitalization
hyperferritinemia and hemophagocytosis was detected
by the biopsy of bone marrow. With immunotherapy the
patient induced remission.
Keywords: macrophage activation, children, juvenile
idiopathic arthritis
[P-055]
Coagulation Disorder And Direct
Hyperbilirubinemia In A Newborn With
Galactosemia
disorder. Classical galactosemia appears due to deficiency
of galactose-1-P uridyl transferase (GALT) which is
essential for galactose metabolism pathway. Signs of
disease begins with taking galactose rich nutrition as
breast milk, cow milk or formulas containing lactose.
Usually the disease begins after the first week of life with
jaundice, vomiting, hypoglycaemia, cataract, nutrition
problems, irritability, convulsion, weight loss, sepsis or
liver failure. Restriction of galactose intake is lifesaving.
In this case an eight days old infant brought to
hospital with jaundice and weight loss is presented. In
labaratory studies, baby had direct hyperbilirubinemia
and prolonged coagulation parameters. With suspicion
of galactosemia breast milk had been stopped and
started lactose-free formula. In a few days bilirubin and
coagulation parameters turned to normal. GALT level of
baby was detected so low.
Keywords: galactosemia, GALT, lactose free formula
The Correlation Between Aortic Intima Media
Thickness, Blood Lipid Profiles and Leptin
Levels in Newborns with Intrauterin Growth
Retardation
Emel Akıncı Ataoğlu1, Vasfi Onur Vermezoğlu1, Öznur
Vermezoğlu1, Derya Büyükkayhan2, Murat Elevli1
1
Istanbul Haseki Training and Research Hospital,
Department of Pediatrics
2
Istanbul Medeniyet University, Department of
Pediatrics
Background&Aims: In this study,it was aimed to
determine the aortic intima-media thickness (aIMT) as an
indicator of preclinic atherosclerosis and its relationship
betweenblood lipid profiles and leptin levels in newborns
with intrauterin growth retardation (IUGR) hospitalised
in NICU of Haseki Research and Education Hospital.
We also aimed to to adress which children are prone to
atherosclerosis and which mechanisms are responsible
for this.
Methods: 30 babies with IUGR and 30 babies with
appropriate growth for gestational age (AGA) were
included in the study.The demographic characteristics,
biochemical values, arteriel blood pressures, weights and
CRP levels were recorded. Aortic intima-media thickness
and its relation with lipid profiles and leptin levels were
also determined. The results were compared between
groups.
Results: The lipid profiles, abdominal aortic intimamedia thicknesses and leptin levels of IUGR babies were
not signifacantly different than that of AGA babies
Elif Arslan, Mustafa Kemal Özdemir, Evrim Çeliker
Yapıcı, Berna Gümüşoğlu Akşahin, Avidan Kızılelma
Yiğit, Yeşim Acar, Ömer Faruk Beşer, Bekir Yiğit Develi,
Ozan Özkaya
İstanbul Okmeydanı Eğitim ve Araştırma Hastanesi
Çocuk Sağlığı ve Hastalıkları
Galactosemia is a rarely seen carbonhydrate metabolism
Conclusions: According to these results, IUGR babies
are not more likely to develop atherosclerosis compared
to AGA babies.
Keywords: lipid profile, intima media thickness
- 41 -
[P-057]
The Correlation Between Aortic Intima
Media Thickness in Different Gestational Age
Newborns
Emel Akıncı Ataoğlu1, Öznur Vermezoğlu1, Vasfi Onur
Vermezoğlu1, Derya Büyükkayhan2, Murat Elevli1
1
İstanbul Haseki Training and Research Hospital
2
İstanbul Medeniyet University
Background&Aims: In this study,it was aimed to
determine the aortic intima-media thickness (aIMT)
as an indicator of preclinic atherosclerosis and its
relationship between blood lipid profiles and gestational
age in newborns those hospitalised in NICU of Haseki
Research and Education Hospital. We also aimed to to
adress which children are prone to atherosclerosis and
which mechanisms are responsible for this.
Methods: 60 babies have different gestational age
(AGA) were included in the study. The demographic
characteristics, biochemical values, arteriel blood
pressures, weights and CRP levels were recorded. Aortic
intima-media thickness and its relation with lipid profiles
and gestational age were also determined. The results
were compared between groups.
Results: The lipid profiles and abdominal aortic intimamedia thicknesses were not significantly different in
different gestational age groups.
Conclusions: According to these results, Premature
babies are not more likely to develop atherosclerosis
compared to term babies.
Keywords: intima media thickness, gestational age
[P-058]
A retrospective evaluation of causes of
morbidity, and mortality among babies
delivered in the Haseki Training and
Research Hospital within the 3 years
Ali Alkan1, Emel Akıncı Ataoğlu1, Derya Buyukkayhan2,
Murat Elevli1
1
İstanbul Haseki Training and Research Hospital
2
İstanbul Medeniyet University
Background&Aims: Our aim in this study is to
evaluate morbidity, and mortality rates of the babies
delivered between January 2009, and December
2011 so as to shed light on prospective planning
studies, and relevant applications to be performed.
Methods: We compared mortality, and morbidity data
of the babies delivered in newborn registry between
January 2009, and December 2011, and analyzed
epidemiological data of these babies delivered in our
hospital at gestational age of > 22 weeks with birth
weights below 500 gr.
0.61, and 1.65 %, respectively. Causes of perinatal
death were stillbirths (68.1 %), and congenital
malformations (11.3 %), prematurity-related factors,
and other etiologies in order of decreasing frequency.
The most frequent indications for hospitalizations were
neonatal hyperbilirubinemia (35.3 %), and suspect
sepsis (13.4 %). Our median mortality rate among
babies delivered before 32. gestational weeks within
indicated three years was 14.5 percent.
Conclusions: Providing adequate antenatal, and
postnatal care for pregnants, and their newborns, and
realization of deliveries under optimal conditions will
dramatically decrease neonatal mortality rates.
Keywords: Neonatal, mortality, morbidity
[P-059]
Congenital Syphlis Case Presented with
Purulent Rhinorea and Skin Findings
Emel Akıncı Ataoğlu1, Özlem Kosvalı1, Derya
Buyukkayhan2, Murat Elevli1
1
İstanbul Haseki Training and Research Hospital
2
İstanbul Medeniyet University
Congenital syphilia is a serious condition that transmit
from mother to baby through transplasentally in all stages
of pregnancy. The various clinical presentations depend
on the gestational age of fetus, whether the mother was
diagnosed or not, treatment of mother and immunologic
response of fetus. Half of the syphilitic pregnancies could
be resulted with spontaneous abortus, perinatal death
and premature birth. The infection commonly transmit
by intrauterine way, however ocaasionally the infection
could transmit at the time of birth with genital contact.
The two third of infected fetuses are asymptomatic
at birth. Prematurity, low birth weight, elevated
liver functions, hyperbilirubinemia, skin findings,
thrombocytopenia, hemolitic anemia, lymphadenopathy,
bone deformities, central nervous system abnormalities
and fever are signs of symptomatic neonates.
Our case was borned at 32 gestational age weighing
1930 gr. His mother had no control in her pregnancy
and known to have condyloma. He was entubated and
brought to neonatal intensive care unit. The lung X-ray
of newborn showed the signs of respiratory distress
syndrome and surfactan had been given. On follow
up purulent rhinorrhea and maculopapular rashes
occured. Laboratory findings showed pozitive tests for
VDRL and TPHA. According to lomber punction he had
no neurological involvement. On babygram there was
no bone deformity. Congenital syphilis diagnosis was
made and 10 days penicilin treatment was started. After
two months hospital care, he was discharged with his
controls were planned.
Keywords: congenital, syphilis
Results: 1905 live babies, and 32 stillbirths were
delivered in the year 2011. During early neonatal
period 12 babies exited. Rates of perinatal, and early
neonatal mortality, and stillbirth were found to be 2.27,
- 42 -
[P-060]
Other system examinations were normal.
Diagnostic value of serum procalcitonin and
C-reaction protein levels in neonatal sepsis
Mehmet Ali Erceylan1, Emel Akıncı Ataoğlu1, Derya
Büyükkayhan2, Murat Elevli1
1
İstanbul Haseki Training and Research Hospital
2
İstanbul Medeniyet University
Early diagnosis of neonatal sepsis mortality reduction of
morbidity is of great importance. Hemoculture growth
factor is produced before the diagnosis of acute phase
reactants are supported. Clinical applications, C-reactive
protein (CRP) levels, monitoring is used for this purpose.
In recent years, procalcitonin (PCT) has come into use
in neonatal sepsis. PCT increase is earlier than CRP. In
this study, neonatal sepsis diagnosis and follow-up CRP
and PCT levels were compared. Study was conducted on
40 patients with the diagnosis of neonatal sepsis in the
intensive care unit and 40 controls. Study group of the
PCT and CRP levels before treatment were significantly
higher than the control group (p <0.05). CRP and PCT
levels before treatment, the treatment received after
72.hours compared CRP and PCT levels, showed a
statistically significant decrease (p <0.05). PCT in sepsis
as a result of early diagnosis and in monitoring response
to therapy are used routinely to CRP were found to be
useful.
Keywords: Procalcitonin, neonatal sepsis
[P-061]
recurrent bacterial menengitis after
traumatic head injury
Emine Olcay Yasa, Asena Pınar Sefer, Ismet Dusmez,
Doruk Gül
Goztepe Research Hospital Istanbul/Turkey
Introduction: Recurrent bacterial menengitis is defined
as two or more episodes of meningitis separated by a
period of convalescense and the complete resolution of
all signs and symptoms and it is a rare condition which
usually caused by head trauma, congenital anatomical
defects,immunological deficiencies.
An accurate diagnosis of underlying pathologly is crucial
to prevent further episodes from occuring.
In this case we highlighted the importance of investigation
and diagnosis of underlying pathology which causes
recurrent bacterial menegitis episodes.
Case: 8 year old male patient applied to hospital by high
fever,severe headache,nausia,vomiting and malaise. He
took diagnosis of acute bacterial menengites by lumbar
puncture and CSF examination. He was transported to
our infectious disease deparment for internalitazion.
[P-062]
Varicella zoster case with ataxia and
dyslexia
Emine Olcay Yasa, Asena Pınar Sefer, Doruk Gül, İsmet
Düşmez
Goztepe Research Hospital Istanbul/Turkey
Introduction: Acute cerebellitis is an inflamatory
condition characterized by cerebellar disfunction.
It’s generally bening condition however rarerly it may
cause herniation and death. It may occur after some
kind of infections and vaccinations.Infections such
as varicella zoster,EBV,coxsacie,rubella,diphteria and
pertusis; are the most common causes.Lead or cyanide
poising, demiyelinating diseases and vasculitis may
cause cerebellar inflamation. However;sometimes the
etiology cant be detected.To diagnose idiopathic acute
cerebellitis; other etiological factors should be excluded.
In this case we describe varicella zoster cerebellitis who
applied with vomiting, speaking and walking disability.
Case: 5 years old male patient,6 days ago he was
diagnosed as varicella zoster with the complain about
multiple vesiculer skin eruptions.After 3 days he
developed ataxia,dyslexia and severe nause -vomiting.
He was internalized infectious dieases department.
At admission, he was lethargic.Physical examination;he
had generilazed multiple vesiculer skin eruption.ataxia
+,romberg +, dysdiadokinesia +.He had no pathological
reflexes, no meningial irritation finding.Other physical
examination sings were normal.
Hb:11.4 g/
dl,Plt:368000,Wbc:7.7,Neu:%42,Lenf:%49,Glucose:98
mg/dl,creatinin:0.46 mg/dl,Ast:30 u/lt,Alt:19 u/
lt,Na:141 meq/lt,K:4.5meq/lt,Cl:108 meq/lt,Ca:9.1mg/
dl,Crp:0.33 mg/dl
Differential diagnosis; vzv cerebellitis,ADEM,mass
lesion at posterior fossa of cranium,vasculitis,drug
intoxication
In order to exclude ADEM and posterior fossa tumor
we took cranial MRI of the patient and the result was
normal.
He was diagnosed varicella zoster cerebellitis and
30 mg/kg/day acyclovir treatment was started.
With the treatment;signs and symptoms of patient
dramatically decreased. And the treatment was
completed at 10 days.Then he was externated from
hospital.
Keywords: varicella zoster cerebellitis, ataxia, dyslexia
Medical history;4 years ago he had traffic accident which
cause traumatic head injury and he operated due to
subdural hematoma.
1.5 year ago he had another acute bacterial meningites
episode.
At
admission,
he
was
lethargic,Gks:12,body
temperature:38.5.Physical examination; neck stiffness
+,Kerning+,dlr:+/+ ılr:+/+,had no patological reflexes..
Babinski -,motor exam of lower exst.:5/5 upper exst:5/5.
- 43 -
[P-063]
[P-064]
Emine Yurdakul Ertürk1, Özlem Özdemir2, İsmail
Taşkıran3, Abdullah Erdil1
1
Department of Pediatrics, Medical Faculty, Ordu
University, Ordu, Turkey
2
Department of Internal Medicine, Medical Faculty, Ordu
University, Ordu, Turkey
3
Department of Gastroenterology, Medical Faculty, Ordu
University, Ordu, Turkey
Background&Aims: The upper gastrointestinal system
(GIS) hemorrhages are the hemorrhage into the lümen
from the upper oesophagus to the treitz ligament.It
occurs in the childhood rarely then the adult ages.İn this
case,we have presented an adolescent with the upper
GIS hemorrhage after a one dose use of the paracetamol.
Emine Yurdakul Ertürk1, Şükrü Küçüködük2, Canan
Aygün2, Kemal Baysal3
1
Department of Pediatrics, Medical Faculty, Ordu
University, Ordu, Turkey
2
Division of Neonatalogy, Medical Faculty, 19 Mayıs
University, Samsun, Turkey
3
Division of Pediatric Cardiology, Medical Faculty, 19
Mayıs University, Samsun, Turkey
Background&Aims: The transposition of the great
arteries (TGA) which is a cyanotic cardiac abnormality
constitutes approximately 5% of congenital cardiac
disease. In this study, the investigation of some
demographic and clinical characteristics of thirty patients
who were diagnosed with neonatal TGA between 20062010 periods was aimed.
İs the one dose of the paracetamol can be
the cause of the gastrointestinal system
hemorrhage?
Transposition of the great arteries:
Evaluation of the thirty neonatal patients
The Case: The male,seventeen age adolescent who
explain that he had any complaint before,has came to
the emergency clinic with the handful hemorrhage after
a nausea and vomiting.There was no characteristic in his
brief and family history.We have learned that he took a
one 500mg paracetamol tablet for the abdominal pain
reason,five days ago.His appearance,vital parameters
and physical examination were normal.Hb:14,6 gr/
dl, WBC:7000/mm3, platelet:259000/mm3, BUN:24
mg/dl, Cr:0,6 mg/dl, AST:17 U/L, ALT:12 U/L, Na:139
mmol/L, K:4,3 mmol/L, Cl:105 mm/L were determined.
The coagulation parameters were normal.A nasogastric
tube has been placed,the oral take has stopped,fluid
replacement
and
antisecretuar
treatment
have
started.180 cc bloody fluid from NG and melaena were
determined,abdominal ultrasonography was normal.
After the four hours of the treatment the hemorrhage
was stopped and the hemoglobin level was stabil.The
upper GIS endoscopy was done and a antral gastritis
and a one bulber ulcer at 2-3 mm dimension (forrest3)
were determined.İn the following,the patient had any
complaint,had oral nutrition and he was discharged with
the prescription and the advice for the polyclinic control.
Conclusions: In many research,the paracetamol was
detected as the reason for the upper GIS hemorrhage.
Even the dosages age-appropriate or the using for the
short term can be a reason to a hemorrhage.In our
study the hemorrhage was associated with the one
dose paracetamol take but we need the many similar
studies for to be sure.On the other hand,the commun
information must be done about the adverse effects
of the paracetamol which is accepted safety for the
stomach.
Keywords: adolescent, hemorrhage, gastrointestinal
system
Methods: Within the 300 patients who were hospitalized
in the Neonatal Intensive Care Unit of the Ondokuz
Mayıs University with the diagnosis of congenital heart
disease between January 2006 and December 2010, met
the criteria to be included into the study and whose files
were accessible, the files of the TGA detected patients in
echocardiography (ECHO) were analyzed retrospectively.
Results: The frequency of the transposition of the great
arteries was determined as 10% (30/300). Nine of the
cases (30%) were female and 21(70%) were male.
The average diagnosis age of the patients was found
as 9.53 days. While 16(53.4%) of patients were d-TGA,
14(46.6%) of them were isolated TGA and 26(86.6%)
cases had additional cardiac disorder. In 30 cases,
because of the cyanosis 16(53.3%) of the patients and
because of the heart murmur 14(46.7%) of the patients
were performed cardiological evaluation and ECHO.
The arterial switch operation were performed on the
18(60%) of the cases. While the 14(46.7%) cases were
discharging, 16(53.3%) of them died.
Conclusions: The transposition of great arteries is
the most common cyanotic congenital heart disease
in newborns and infants. If the surgical interventions
aren’t applied, the mortality rate is over 90% in the
first year of life. Prenatal diagnosis of TGA is possible
with a fetal ECHO. The fetal ECHO which will be applied
in the prenatal period is extremely important for the
prevention of cases that can be mortal due to complex
congenital heart abnormalities and the prediction of
surgical strategy which will be required after birth.
Keywords: newborn, transposition of the great arteries
- 44 -
[P-065]
[P-066]
Emmanouela Sdona1, Dimitris Papamichail2, Takis
Panagiotopoulos2, Pagona Lagiou3, Ariadne Malamitsi
Puchner1
1
Department of Neonatology, Aretaieion Hospital,
University of Athens Medical School, Athens, Greece
2
Department of Child Health, National School of Public
Health, Athens, Greece
3
Department of Hygiene, Epidemiology and Medical
Statistics, University of Athens Medical School, Athens,
Greece
Background&Aims: Term and late preterm neonates
are considerably less prone to necrotizing enterocolitis
(NEC), which is the most common gastrointestinal
emergency in preterms. The aim of this study was to
investigate a cluster of late preterm and term neonates
(gestational age >= 34 weeks) with NEC symptomatology
in a maternity hospital during a period of 14 months.
Emrah Aydın
Bahcelievler State Hospital
Aim: To present diagnosis of desmoid tumor in a patient
with recurrent upper abdominal pain and mass with
normal ultrasound findings.
Necrotizing enterocolitis affecting late
preterm and term neonates
Methods: Medical records of neonates with modified
Bell stage >= IB NEC were reviewed, in addition
to microbiological and environmental investigation.
Variables collected included demographics, maternal/
delivery and neonatal factors, medications, procedures
and feeding practices.
Results: Out of 1841 late preterm and term neonates,
20 presented with NEC symptomatology at mean 4.6
days (2-8 days). Μean (± SD) birthweight was 2529.3
(493.04) grams and mean gestational age was 36.96
(1.48) weeks. Most (14/20) were born by caesarean
delivery. Nearly all (19/20) resulted from high risk
pregnancies (late preterm, twin, intrauterine growth
restriction, in vitro fertilization, prolonged rupture of
membranes, placenta praevia and maternal diseases
– preeclampsia, gestational diabetes, thyroid disease,
thrombophilia,
autoimmune
hepatitis,
epilepsy,
Turner syndrome and Hodgkin’s disease) and received
postpartum intermediate care. No neonate had congenital
cardiac disease and none required intubation, umbilical
artery catheterization or blood transfusion. Apgar scores
were equal or more than 71’ and 95’. All were formula fed
(exclusively or partly). Ten neonates with suspected NEC
(stage IB) had mild clinical course and were treated with
antibiotics for less than 10 days with gradual resumption
of feedings. Ten other neonates had stage >= II NEC,
eight of whom underwent surgery, with no fatality. NEC
lesions were mainly located in the colon. No pathogen
could be identified.
Conclusions: Late preterm and term neonates with
predisposing clinical conditions, requiring postpartum
intermediate care, are susceptible to NEC; feeding
with breast milk seems to be the strongest preventive
measure.
Keywords: necrotizing enterocolitis, late preterm,
term
Preference of the Surgeon: The patient or
the radiologist? Desmoid tumor
Case: Fifteen-years-old boy had been operated due
to acute appendicitis and intraabdominal abscess four
years ago was admitted to our clinic with complaints of
right upper quadrant pain and swelling for two years.
Many ultrasound were performed but nothing was
found. There was 6cm thickening and heterogeneity of
right rectus abdominis muscle with a suspicious mass
neighboring the liver at CT and MRI. He was operated
and pathology was reported as desmoid tumor.
Conclusion: Even basic studies doesn’t reveal any
pathology if the patient’s complaint goes on surgeon
must be aggressive for diagnosis in order not to miss
out malignancies.
Keywords: desmoid tumor, desmoid type fibromatosis,
aggressive fibromatosis
[P-067]
One Month Ongoing Intussusception
Emrah Aydın
Bahcelievler State Hospital
Aim: To present a case operated due to suspected
intussusception and diagnosed as lymphoma
Case: A six years old boy was admitted tour clinic
with a complaint of colicky abdominal pain and bilious
vomiting for the last month. No pathology was found
at his examinations in other clinics. He had tenderness
and defense at physical examination but no mass was
palpated. All biochemical parameters were normal
except leukocytosis. Abdominal X-ray was normal. 3cm
long intussusception was found at ileocecal part at
ultrasound. He had colicky abdominal pain at his follow
up and bilious content at nasogastric tube. Pneumotic
reduction was performed but no intussusception was
found. Air passage to the proximal part of intestine was
normal but there was a mass image at ileocecal valve.
Edematous and pseudomembranous intestinal segment
was found. There was multiple lymph nodes at mesenteric
area. Ileoascendostomy was performed. Pathology was
reported as Burkitt lymphoma. He received two cycles of
chemotherapy at post-operative period. He is still event
free for the last year.
Conclusion: One must remember that lymphoma can
be the lead point in intussusception.
Keywords: intussusception, lymphoma
- 45 -
[P-068]
Recurrent abdominal pain: Multiseptated gall
bladder
Emrah Aydın
Bahcelievler State Hospital
Aim: To present a patient was admitted to our clinic with
recurrent abdominal pain and diagnosed as multiseptated
gall bladder.
Case: Seven years old boy admitted to our department
with recurrent abdominal pain and diagnosed as multiple
septation in gall bladder. All laboratory values were
normal. There was no sludge in ultrasound. MRCP didn’t
show any congenital abnormality. He has been abdominal
pain only once in a time for the last year.
Conclusion: Although a rare condition in childhood,
patients with multiseptated gallbladder should be
followed unless without clinical symptoms.
Keywords: multiseptate gall bladder, abdominal pain
[P-069]
A rare cause of acute abdomen in
prematurity: Intussusception
Emrah Aydın
Bahcelievler State Hospital
Aim: To present a neonatal referred to our clinic due to
acute abdomen and diagnosed as intussusception.
Case: A newborn who didn’t have any prenatal problem
born at 34th gestational weeks with normal spontaneous
vaginal way, weighed 2500gr was monitored in intensive
care unit for 10 days. Except being intubated for one
day he was well on follow up and discharged to home.
One week after discharge he was referred tour clinic
with bilious vomiting and rectal bleeding. At physical
examination he had normoactive bowel sounds and
abdominal distension with tenderness on the right lower
quadrant without defense or rebounds. White blood
cell count was 22000/mm3 besides other values were
normal. There was no free air under the diaphragm
at abdominal radiography but abdominal ultrasound
revealed 2cm ileocolic intussusception in the right lower
quadrant. Operation decision were taken and ileocolic
intussusception was revealed. Manuel reduction was
performed and no more pathology was found. He was
fed 2 days after operation and discharged 8 days after.
He was free of any symptom for the last 6 months.
Conclusion: Intussusception should be remembered
in neonatal admitted with bilious vomiting and rectal
bleeding besides necrotizing enterocolitis.
Keywords: Intussusception, acute abdomen, newborn
[P-070]
To analyse effect of tracheal occlusion in
the development of lung in nitrofen induced
congential diaphragmatic hernia model
Emrah Aydın
Bahcelievler State Hospital
Aim: Congenital daphragmatic hernia is a congenital
abnormality with high morbidity and mortality. Lung
hypoplasia and pulmonary hypertension is the leading
cause of death. In our study we aim to analyse the
effect of tracheal occlusion in the development of lung in
nitrofen induced congenital diaphragmatic hernia.
Method: Same age group Wistar albino rats were
mated. Rats were included in the study whose vaginal
smear showed sperm in. It was zero day of pregnancy
in which sperm was shown in vaginal smear. At the 9th
day of pregnancy 100mg Nitrofen was introduced to
pregnant rats by gavage and at the 18thday of pregnancy
laparatomy and hysterotomy was performed. Tracheal
occlusion was performed one of two fetuses and the
other one was accepted as control group. At 22nd day of
pregnancy laparatomy and hysterotomy was performed
and lungs of fetuses were excised. Development of lungs
were analysed histopathologically.
Results: Ten (%59) of 17 fetuses had congenital
diaphragmatic hernia. Five of them were within control
group and 5 were within surgical group. Defect of
diaphragm was rigth sided in 5 of cases, left in 3 and
bilateral in 2. Liver had tendency to toracic cavity in all.
Development of lung was stopped in psödoglandüler
stage in 2 of cases, in psödoglandüler-early canalicular
stage in 2 and early canalicular in 1 of cases in control
group while late canalicular-early saccular in 2, saccular
in 1 and early canalicular in 2 of cases in surgical group.
Conclusion: Nitrofen induced congenital diaphragmatic
hernia is an efficient model but it cannot simulate
Bochdalek’s hernia. Tracheal occlusion in nitrofen induced
congenital diaphragmatic hernia model increases lung
development.
Keywords: Congenital diaphragmatic hernia, nitrofen,
tracheal oclusion [P-071]
Actual View to Diagnosis of Cystic Fibrosis
Emre Özer1, Ayşe Ayzıt Atabek2, Fatma Deniz Uslu
Aygün3, Haluk Çokuğraş2
1
Istanbul University, Cerrahpasa Medical Faculty,
Department of Pediatrics, Istanbul, TURKEY
2
Istanbul University Cerrahpasa Medical Faculty,
Department of Pediatrics Division of Pneumology,
Istanbul, Turkey
3
Istanbul University Cerrahpasa Medical Faculty,
Department of Pediatrics Division of Infectious
Diseases, Istanbul, Turkey
A six-weeks old girl who was treated for sepsis and
meconium ileus was admitted to our hospital with
symptoms of vomiting and cough. Physical examination
- 46 -
showed that she has sunken eyeballs, dry mouth, pale
appearance. Her breathing sounded rough, crackles were
present. Laboratory examination showed hyponatremic
and hypochloremic metabolic alkalosis and the posterior
anterior chest x-ray revealed the infiltration. The
patent, who had high chloride levels in sweat twice,
was diagnosed with cystic fibrosis and pneumonia. On
the 14th day of treatment, the patient’s CF mutation
analysis resulted as ∆F508 homozygosis, without any
infiltration on the chest x-ray were observed due to
the clinically significant improvement. Cystic fibrosis is
a genetic disease which shortens life expectancy and
impact the quality of life. This disease is characterized
by the mulfunction of mucous glands primarily in lung
and pancreas. The most significant issue for the patients
is the recurrent lung infections and irreversible lung
damage due to secretions which could not be removed,
and malabsorption developed due to pancreatitis.
Fundamental treatment of cystic fibrosis is the removal
of dense lung secretions, treatment of infections, and
the treatment of pancreatitis in additon to vitamin
and nutritional support. However, in the recent years,
the treatments, which remedy the failure of chloride
channel, were developed and those studies are still
ongoing. If the cystic fibrosis is diagnosed early and
treated accordingly, both the life expectancy gets longer
and the quality of life improves. In Turkey, it is aimed
to prevent the delay of diagnosis and treatment by the
newborn screening. This study aimes to emphasize the
importance of the diagnosis and screening of cyctic
fibosis and how the treatment should be implemented,
by considering a newborn patient with the symptoms
of meconium ileus and development of hypochloremic
hyponatremic dehydration at 6-weeks.
Keywords: Cystic fibrosis, immunoreactive
trypsinogen, sweat test
[P-072]
Veno-occlusive disease as a complication
of postoperative chemotherapy for Wilms
tumor, successful and safe treatment with
defibrotide
Emre Tasdemir1, Gurcan Dikme2, Dilara Ece Toprak1,
Emre Ozer1, Hande Kumkumoglu2, Begum Koc2, Ayse
Ucar3, Sebuh Kuruoglu3, Tulin Tiraje Celkan2
1
Istanbul University, Cerrahpasa Medical Faculty,
Department of Pediatrics
2
Istanbul University, Cerrahpasa Medical Faculty,
Department of Pediatric Hematology Oncology
3
Istanbul University, Cerrahpasa Medical Faculty,
Department of Pediatric Radiology
Veno-occlusive disease (VOD) is an obliterative venulitis
of the terminal hepatic venules. The clinical presentation
of VOD includes jaundice, development of right upperquadrant pain, tender hepatomegaly, ascites and
unexplained weight gain. VOD is a well-known early
complication after human stem cell transplantation
(HSCT), however a rare and severe complication of
chemotherapeutic drugs. We report a 8 months old
male infant with Wilms tumor stage 2 who developed
VOD after second course of chemotherapy ( actinomicin
and vincristine ). The patient presented with abdominal
distension, weight gain, hepatomegaly, ascites,
hyperbilirubinemia, severe thrombocytopenia, anemia,
increase of liver transaminases and abnormalities
of coagulation on the day of 10 days after second
administration of actinomicin D. Defibrotide on a
compassionate-use basis was started. The dosage was
15 mg/kg/day in 4 divided doses and VOD recovered
after defibrotide treatment especially clinically with
laboratory findings in 3th day.
Defibrotide (DF) is a polydeoxyribonucleotide that has
been found to have anti-thrombotic, anti-ischemic and
thrombolytic properties without causing significant
anticoagulation. There are limited reports on the use of
defibrotide outside the human stem cell transplantation
(HSCT) setting. Herein, we report a case with Wilms
tumor complicated with VOD after chemotherapy which
is rarely seen.
Keywords: veno-occlusive disease, wilms tumor,
defibrotide
[P-073]
Fibrinogen storage disease associated with
hypobetalipoproteinemia and hepatocellular
disease due to mutation in fibrinogen
Aguadilla in a Turkish child
Emrullah Arslan1, Tanyel Zubarioglu2, Gozde Yesil3,
Ertugrul Kiykim2, Birol Ozturk4, Ayse Cigdem Aktuglu
Zeybek2
1
Istanbul University, Cerrahpasa Medical Faculty,
Department of Pediatrics
2
Istanbul University Cerrahpasa Medical Faculty,
Department of Pediatrics Division of Nutrition and
Metabolism
3
Bezmialem Vakif University, Faculty of Medicine,
Department of Medical Genetics
4
Marmara University, Faculty of Medicine, Departement
of Pediatrics Division of Gastroenterology
Hepatic fibrinogen storage disease (HFSD) is a rare
cause of chronic hepatocellular disease that is caused
by mutations in the γ-chain of the fibrinogen (FGG)
gene. It is characterized by hypofibrinogenemia and
accumulation of abnormal fibrinogen within hepatocytes.
In addition, hypofibrinogenemia is associated with
hypobetalipoproteinemia in Aguadilla mutation. In this
report, 3 years old male patient with elevated liver
enzymes, hypolipoproteinemia and hypofibrinogenemia
who was diagnosed as hepatic fibrinogen disease is
reported.
A 3 years old male patient was referred to our outpatient
clinic with hepatomegaly. In his physical examination,
liver was palpated 5 cm below the costal margin. Initial
laboratory examinations revealed elevated transaminase
levels, hypofibrinogenemia and dyslipidemia. Lipid
electroforesis and apo B levels were compatible
with hypobetalipoproteinemia. Immunomorphologic
and electron microscopic studies showed selective
and exclusive accumulation of fibrinogen within the
endoplasmic reticulum in liver biopsy of the patient.
Depending to laboratory and histological findings of the
patient HFSD was considered in differential diagnosis of
hepatocellular disease. Molecular analysis of FGG gene
was performed and patient was found in heterozygote
state for p.Arg401Trp mutation (fibrinogen Aguadilla).
- 47 -
HFSD is characterized by hypofibrinogenemia and
accumulation of abnormal fibrinogen within hepatocytes.
Despite its rarity, HFSD should be considered in
differential diagnosis of chronic hepatocellular diseases
especially in cases without an unknown etiology.
Keywords: hepatic fibrinogen storage disease,
hypobetalipoproteinemia, hypofibrinogenemia
[P-074]
A Rare Disease Presented with Abdominal
Pain and Red Eye: Tubulointerstitial
Nephritis and Uveitis Syndrome (TINU)
Müferet Ergüven, Enise Avcı, Cengiz Candan, Pınar
Turhan, Emine Olcay Yasa, Gizem Höyük, Betül Aluç
Medeniyet University,Goztepe Education and Research
Hospital, Department of Pediatrics, Istanbul
Background&Aims:
Uveal
inflammation
can
rarely
accompany
tubulointerstitial
nephritis
that cause renal failure in children. This cooccurrence was first defined in 1975 and termed as
tubulointerstitial nephritis and uveitis syndrome; TINU.
Methods: A 16-year-old male patient has come with
the complaints of abdominal pain and red eye. There
was no history of chronic illness, drug use, arthritis, oral
and genital ulcers, skin eruption. He was diagnosed with
uveitis after membrane formation in the right eye was
detected His laboratory findings suggested mild renal
insufficiency. CBC was normal, ESH (64 mm/h ) and CRP
( 9,39 mg/dl) were increased. Serum urea (39 mg/dl)
was normal and kreatinin (2,19 mg/dl) was increased,
GFR was 63 ml/dk/1.73 m2. Urinalysis showed low urine
density (1007), normoglycemic glycosuria (+++), and
nonnephrotic proteinuria (+) In the 24-hour urinalysis,
protein level was nephritic (13,44 mg/m2/h) and beta
2 mikroglobulin level was increased (25 mg/day). In
further laboratory findings, ANA, Anti dsDNA, ANCA, RF
were found negative. C3, C4, TSH, PTH, ACE levels were
normal. The viral serology was unremarkable. PPD skin
test and Patergy test had shown no reaction. A renal
biopsy was performed and showed dense leukocytes in
the interstitium. The patient was diagnosed with TINU
syndrome. Both his uveitis and interstitial nephritis
responded to high-dose corticosteroid treatment.
Results: The definitive diagnosis of TINU syndrome is
nonspecific and is suggested by the combination of uveitis
and renal involvement, with renal biopsy consistent with
acute interstitial nephritis. Patients with TINU are typically
treated with prednisone at a dose of 1 mg/kg per day.
Conclusions: The differential diagnosis for interstitial
nephritis occurring in association with ocular findings is
broad. The differential diagnosis includes sarcoidosis,
Sjögren’s syndrome, systemic lupus erythematosus,
Wegener’s granulomatosis, Behçet’s disease, and
infectious diseases such as tuberculosis, brucellosis,
toxoplasmosis, and histoplasmosis.
Keywords: renal insufficiency, tubulointerstitial
nephritis, uveitis
[P-075]
The Prolonged Fever in Early Childhood,
Kawasaki Disease
Erkan Erfidan1, Gökçen Kamış1, Mustafa Çakan2, Nuray
Aktay Ayaz2, Helen Bornaun3, Hüseyin Aldemir1, Emel
Karaoğlan1, Seda Balkaya1
1
Department of Pediatrics, Kanuni Sultan Suleyman
Teaching Hospital, Istanbul, Turkey
2
Department of Pediatric Rheumatology Clinic, Kanuni
Sultan Suleyman Teaching Hospital, Istanbul, Turkey
3
Department of Pediatric Cardiology Clinic, Kanuni
Sultan Suleyman Teaching Hospital, Istanbul, Turkey
Introduction: Kawasaki Disease is one of the most
frequent causes of the acquired heart diseases affecting
children in developed countries. More than 50% of the
cases are between 6 months to 2 years old. Only 10%
of the patients are younger than 6 months old. The
incidence is very low through children younger than 3
months and older than 8 years old.
Case Presentation: A thirty eight days old female
is referred to our clinics with high grade fever and
vomiting. She is hospitalized with the diagnosis
of sepsis. Her physical examination findings are
unremarkable.
Laboratory
investigations
show
WBC:16x103/µL,
Plt:
351x103/µL,
CRP:58mg/L,
ESR:60mm/h. Urine analysis and chest X-ray are
normal. The peripheral blood smear shows neutrophilia
with toxic granulation, multiple platelet clumps. After
blood, urine and CSF culturing, the antibiotic treatment
is started as Ampicilina-Sulbactam and Cefotaxime.
During the hospitalization, maculopapular rash appears
in her cheeks,trunk and proximal extremities. erythema
and fissure in her lips(Figure 1). Due to suspicion of the
Kawasaki disease, Echocardiography is done, but shows
coronary arteries with normal diameter. Incomplete
Kawasaki disease cannot be ruled out, so IVIG (2 gr/
kg) is given and acetylsalicylic acid (4 mg/kg/day)
is added. After a few days, periungal desquamation
begins. In control echocardiography, right and left
coronary arteries are measured respectively 1.24mm
and 1.65mm (z-score:0.91). Second IVIG dose is given.
Methylprednisolone (2 mg/kg/day) is added due to high
fever. During the follow-up, the fever is reduced. So
methylprednisolone is gradually stopped.
Conclusion: Kawasaki disease should be remembered
in toddlers with resistant fever to the antipyretics and
antibiotics and significantly high acute phase reactants.
İncomplete form is seen more often in early periods of
the life. Early diagnosis and treatment may save the
coronary arteries.
Keywords: Kawasaki disease, early childhood,
prolonged fever
- 48 -
[P-076]
[P-077]
Erkan Erfidan1, Gökçen Kamış1, Mustafa Çakan2, Nuray
Aktay Akaz2, Hamide Sevinç Genç1, Sevgi Yavuz3, Aysel
Kıyak3, Gönül Aydoğan4
1
Department of Pediatrics, Kanuni Sultan Suleyman
Teaching Hospital, Istanbul, Turkey
2
Department of Pediatric Rheumatology Clinic, Kanuni
Sultan Suleyman Teaching Hospital, Istanbul, Turkey
3
Department of Pediatric Nephrology Clinic, Kanuni
Sultan Suleyman Teaching Hospital, Istanbul, Turkey
4
Department of Pediatric Hematology-Oncology Clinic,
Kanuni Sultan Suleyman Teaching Hospital, Istanbul,
Turkey
Introduction: SLE is a chronic autoimmune disease.
Presentation of a case may vary the mild disease with
skin and joint involvement to serious disease with
severe anemia, renal failure, convulsions. Hematologic
involvement is almost seen in every patient. Mild
to moderate anemia of chronic disease is the most
commonly seen hematologic involvement. Autoimmune
hemolytic anemia and thrombocytopenia is seen 2535%. Renal involvement is seen in half of the patients
and mostly as class 4 lupus nephritis. In renal, cerebral
and hematologic involvements, the treatment choice
is steroids and monthly cyclophosphamide infusions.
İf the patient is resistant to the classical treatment,
second choice is rituximab that is a monoclonal antibody
against the CD20 and cause apoptosis of B lymphocytes.
Case Presentation: The patient who has been followed up
for three years with SLE is admitted to the hospital with
malaise. Three years ago, she diagnosed as Type 4 Lupus
Nephritis and treated with six dose cyclophosphamide,
then low dose steroids, hydroxychloroquine treatment
was planned for maintenance. But she hasn’t used
her drugs for last six months. Laboratory examination
shows WBC:5300/mm3 Hb: 5.7gr/dL Hct:15.3%
Plt:253000/mm3 LDH:850 U/L BUN: 22mg/dL Kreatinin:
0.7mg/dL Direct and indirect Coombs:positive. Bone
marrow aspiration is normal. IVIG and hidh dose
methylprednisolon treatment is started. During the
treatment, platelet counts decreased and kidney
function
tests
deteriorated.
Cyclophosphamide
infusion is given to the patient. Two weeks after the
treatment, Hb:4.8 gr/dL PLT: 59000/mm3 BUN:36mg/
dL Kreatinin: 1.8mg/dL. The patient is resistant to
treatment,so Rituximabe (375 mg/m2) is given as
weekly for four weeks. After four dose, WBC:5000/mm3
Hb:11mg/dL Hct:37% Plt:189000/mm3 BUN:23mg/dL
Kreatinin:0.9mg/dL Direct and indiret Coombs: negative.
For maintenance of the treatment, low dose steroids,
Mycophenolate mofetil, hydroxychloroquine are planned.
Steroid and cyclophosphamide resistant SLE patients
with severe hematological and renal involvement may
respond to Rituximab treatment like in our case.
Keywords: Systemic Lupus Erythematosis, Rituximab,
Hematologic and Renal Involvement
Erkan Erfidan1, Gökçen Kamış1, Mustafa Çakan2, Nuray
Aktay Ayaz2, Hamide Sevinç Genç1, Zeynep Topkarcı3,
Ayşe Kavak3
1
Department of Pediatrics, Kanuni Sultan Suleyman
Teaching Hospital, Istanbul, Turkey
2
Department of Pediatric Rheumatology Clinic, Kanuni
Sultan Suleyman Teaching Hospital, Istanbul, Turkey
3
Department of Dermatology, Bakirkoy Dr Sadi Konuk
Teaching Hospital, Istanbul, Turkey
Introduction: JDM is an extremely rare disease
in childhood and primarily effects muscle and skin.
Diagnostic criteria of the disease are typical skin
lesions, symmetric proximal muscle weakness, high
muscle enzymes, EMG and muscle biopsy results. For
the diagnosis, three of the four criteria with typical skin
lesions are enough. Skin lesions are pathognomonic in
JDM and found 80-90% of the cases. Also heliotropic
rash, Gorton papules and periungal changes, edema
of the face and extremities are typical findings. Skin
ulcers are found in 6-23% of the cases. But extensive
and multiple skin necrosis like in our case, is reported
in limited numbers. Steroids and methotrexate is first
choice of the treatment in JDM. We discussed a case
who developed extensive necrotic skin lesions under
steroid and methotrexate and treated successfully with
cyclosporin and monthly IVIG infusion.
Rituximab Treatment of A Systemic Lupus
Erythematosis Case with Severe Hematologic
and Renal Involvement
A case of Juvenile Dermatomyositis with
Severe Skin Necrosis and Treatment with
Cyclosporin and IVIG
Case: A five years old female is admitted to the hospital
with unable to climb stairs, unable to stand up, rash
and edema on her eyelids and hands. Her physical
examination revealed the proximal muscle weakness,
periorbital heliotropic rash, edema in her face, Gorton
papulles on metacarpophalangeal and proximal
interphalangeal joints. İn capillaroscopy examination,
capillary dilatation, decrease of the capillaries and
micro bleedings are seen. Gastrointestinal system and
lungs don’t involved. There is a ulceration about 2x2
centimetres on left scapula. CMAS score is 17. EMG
examination shows myopathic changes. CK levels are
457 U/L (normal levels:30-155U/L), AST is 65 U/L. MR
imaging of lower extremities show extensive myositis of
all muscle groups. She is diagnosed as JDM and started
to the treatment with methotrexate (15 mg/m2/week)
and high dose methylprednisolon (30mg/m2/day).
Cyclosporin and IVIG treatment may be an option for
JDM patient with extensive necrotic skin lesions
Keywords: Juvenile Dermatomyositis, Skin necrosis,
Cyclosporin and IVIG
- 49 -
[P-078]
Clinical outcomes of myocardial non
compaction, is genetic testing necessary for
all patients?
Reyhan Dedeoğlu1, Funda Öztunç1, Erkan Erfidan3,
Helen Bornaun2, Sezen Atik1, Aida Koka1, Levent Saltık1
1
Department of Pediatric Cardiology, Istanbul
University, Cerrahpasa Medical Faculty, Istanbul, Turkey
2
Department of Pediatric Cardiology, Kanuni Sultan
Suleyman Teaching Hospital, Istanbul, Turkey
3
Department of Pediatrics, Kanuni Sultan Suleyman
Teaching Hospital, Istanbul, Turkey
Objective: Myocardial non-compaction (MNC) is a rare
type of cardiomyopathy, resulting from the failure of
myocardial development during embryogenesis.
Methods: We retrospectively investigated records of 13
patients diagnosed as non-compaction cardiomyopathy.
We used four echocardiographic characteristics of Jenni
criteria. We also had detailed biochemical and metabolic
analyses of each patient in addition to genetic testing.
Results: 13 patients who were diagnosed with MNC
were admitted to our clinic within the 2-years period. 8
patient had systolic dysfunction and suffered from heart
failure, 3 of these also had pulmonary hypertension, 5
patient had normal cardiac function (CF). We identified
two new genetic mutations for MNC but these patients
were died during the neonatal period. One patient
was diagnosed with Hallermann-Streiff Syndrome
which was the first reported case associated with
MNC. Genetic testing revealed MNC mutation in one
of the patients which had MNC (heterozygot c.481G>T
p.C161G mutation in ANKRD1 gene) but normal CF. All
patients were on follow-up.
Conclusions: This study demonstrates that mortality
rate of MNC in newborns can be higher. Genetic testing
should be suggested for each MNC patient even having
normal CF for counselling future prognosis.
Keywords: myocardial non compaction, genetic testing
[P-079]
Steroids secondary complication occuring in
the treatment of juvenile idiopathic arthritis:
ZONA ZOSTER
Müferet Ergüven, Olcay Yasa, Ersin Böcü, Elif Yıldız
İstanbul Medeniyet Üniversitesi Göztepe EAH
Introduction: Herpes zoster infection occurs in the
following trigeminal ganglia and it arises out of reactivated
VSV which remains as latent in sensory neurons in the
dorsal root. Generalized or disseminated herpes zoster
can be seen in the immunocompromised patients or in
cases of such secondary immunosuppresion of steroid
use.
Purpose: The case has been reported in order to
emphasise the possible emergence of zona zoster
infection caused by steroid used in the treatment of JIA
patients.
Case: 9-year-old female patient with the diagnosis of JIA
applied to the pediatric rhuematology policlinic by the
reason of itchy and painful vesicular lesions which began
3 days ago and spread from right lumbar region to dorsal.
The patient having vesicular lesions was hospitalized with
a diagnosis of zona zoster. Because of the JIA symptoms,
the patient had received 1 mg/kg/day metilprednizolon
and salazopyrin therapy during 3 weeks. In the physical
examination, there were no active arthritis symptoms in
the patient having normal height and weight percentile.
There were vesicular zona lesions along the right thoracic
dermatome. In the medical workup, WBC:14500 /mm3,
CRP: 6.51 mg/dl, Sedimentation: 47 mm/h, biochemical
values was fixed normal. 1500 mg/m2/day acyclovir IV,
acyclovir cream, B vitamin PO, antihistaminic PO and IV
hydration treatment was administered.
Methylpredinisolone dose was decreased gradually. On
the sixth day of treatment, pain and itching were at an
end, and lesions started to regress.Additional pathology
did not arise in the patient complated the treatment
in ten days. Prophylactic oral acyclovir was prescribed
to the patient, whose complains finished and lesions
started to obviously regress, and the patient was treated.
Conclusion: Steroid secondary zona zoster, which occurs
as a complication on the sidelines of JIA treatment, can
emerge. These patients follow-ups are important also for
this aspect.
Keywords: zona, steroid
[P-080]
Primary Spontaneous Pneumothorax Case at
an Early Age
Ayse Ayzit Atabek1, Esra Karabiyik3, Shiyar Hasan3,
Fatma Deniz Aygun2, Haluk Cokugras1
1
Pediatric Pulmonology, Cerrahpasa Faculty of Pediatric
Medicine, İstanbul University,İstanbul
2
Pediatric Infectious Disease, Cerrahpasa Faculty of
Pediatric Medicine, İstanbul University,İstanbul
3
Cerrahpasa Faculty of Pediatric Medicine, İstanbul
University, İstanbul
Pneumothorax is defined as the existence of air in the
pleural space between the lungs and chest wall. A 15-yearold female patient was admitted to the emergency unit
due to the sudden onset of chest pain. In her physical
examination, she looked pale and had tachypnea. When
she was auscultated, it was found that breathing sounds
was decreased in the left lung and cardiac sounds were
deeply. After thoracic tube was inserted to the patient
whose chest radiography revealed a pneumothorax line in
the left hemithorax, her tachypnea regressed and oxygen
saturation was elevated. In the tomography of the thorax
subpleural cysts were detected in the apical segment of
the upper lobe in the right lung and in the apicoposterior
segment of the upper lobe in the left lung. Pleurodesis/
VATS was not thought to be performed because the
patient did not have a history of previous pneumothorax
and pneumothorax did not persist. Then, the patient,
whose general state of health was good, was discharged
from the hospital by giving some recommendations.
Primary spontaneous pneumothorax(PSP) is the type
of pneumothorax that occurs in patients without any
- 50 -
underlying pulmonary disease. It mostly develops due
to the rupture of apical subpleural blebs. Patients are
generally young, tall and slim males. The most important
risk factor is smoking. The recurrence rate of this
disease is high. The goal of treatment in the first episode
is to decrease symptoms independently of the size of
pneumothorax. Aspiration is needed only in symptomatic
patients. On the other hand, talc pleurodosis or VATS
(video assisted thoracic surgery) are the techniques
that should be preferred in recurrent PSP.In this study,
it was aimed to present a PSP patient, who applied with
the complaint of chest pain, in order to re-evaluate the
etiology and treatment of the disease.
Keywords: Spontaneous Pneumothorax, VATS,
Pleurodosis
[P-081]
Right Aortic Arch with Right Ductus
Arteriosus
Esra Özpınar1, Helen Bornaun2, Kazım Öztarhan2, Merih
Çetinkaya3, Gökhan Büyükkale3, Seda Yılmaz Semerci3
1
Kanuni Sultan Suleyman Training and Research
Hospital,Department of Pediatrics, İstanbul, Turkey
2
Kanuni Sultan Suleyman Training and Research
Hospital,Department of Pediatric Cardiology, İstanbul,
Turkey
3
Kanuni Sultan Suleyman Training and Research
Hospital, Department of Neonatology, İstanbul, Turkey
The term right aortic arch (RAA) with right ductus
arteriosus (RDA) refers to a congenital abnormal
position of the aortic arch, that is, to the right of
the trachea, with or without an abnormal branching
pattern, and is one of the least frequently prenatally
diagnosed cardiac abnormalities. Right aortic arch
identified in prenatal period is associated frequently
with other cardiac/non-cardiac malformations, notably
tracheal or esophageal compression and microdeletions
22q11. Intrauterine and postnatal survey of the fetus
depends on these anomalies and their effects. The right
aortic arch and double aortic arch have been known
about for a long time although a right ductus arteriosus
has however only occasionally been described. We
describe here a case of RAA with a RDA in which with no
other cardiac or morphological abnormalities.
Keywords: right aortic arch, right ductus arteriosus
[P-082]
Trends and prevalence of overweight and
obesity in school children from 2005 to 2014
in Turkey
Mustafa Akcam1, Tugba Koca1, Ozgur Pirgon2, Selim
Dereci1, Esra Sesli3
1
Division of Pediatric Gastroenterology, Hepatology and
Nutrition, Department of Pediatrics, Suleyman Demirel
University Medical School, Isparta, Turkey
2
Division of Pediatric Endocrinology, Department of
Pediatrics, Suleyman Demirel University Medical School,
Isparta, Turkey
3
Department of Pediatrics, Suleyman Demirel University
Medical School, Isparta, Turkey
Aim: The aim of this study was to determine the prevalence
of overweight and obesity in school children and to
compare the results with previous studies conducted
by our clinic in the same region, in 2005 and 2009.
Methods: The study was conducted at 7 primary schools
and 3 high schools in Isparta, southern Turkey, which
had been randomly selected for a study on obesity, 5
and 9 years previously. The height and weight of the
students were measured in a standard way. Those in the
85-94 percent of BMI were classified as overweight and
those >=95 percent were classified as obese.
Results: The study comprised a total of 7116 students;
3445 (48.4%) females and with a mean age of 11.7±2.7
years (range, 5.8-18.9 years).
The total of the overweight+obesity prevalence is
determined as 23.5% for 2014 year. They were
23.8% and 23.5% for the years 2005 and 2009,
respectively. No statistically significant difference was
determined in the prevalence according to the years.
The total overweight frequency was determined as
13.6% for 2014 year. They were 12.2% and 11.1% for
the years 2005 and 2009, respectively. The total obesity
rate was determined as 9.9% in 2014. They were 11.6%
and 12.5% for the years 2005 and 2009, respectively.
When the current study results were compared with
those of previous studies, the overweight prevalence
was seen to have increased and the obesity rates had
decreased (p<0.05).
Conclusion:
Although
the
prevalence
of
overweight+obesity is still at a high level, it’s level shows
not in increasing trend in recent years, it pauses, and
even shows signs of a decrease in obesity, like in some
developed country. It seems that through the efforts of
recent years, there may be a promising trend towards
reduction rather than increase.
Keywords: obesity, children, overweight
[P-083]
Herpetic Whitlow in pediatric patient; a rare
localization of primary herpes infection
Esra Yücel1, Adem Karbuz2
1
SB Okmeydanı EAH Department of Pediatric Allergy
and Immunology
2
SB Okmeydanı EAH Department of Pediatric Infectious
Diseases
Background&Aims: Primary herpetic infections of
children are commonly localized in oral mucosa as
herpetic gingivostomatitis. Primary herpetic whitlow is a
rare condition in pediatric patients. Pediatricians should
distinguish this from other infectious diseases (e.g
bacterial whitlow) because of the different treatments
are required. Here we present a 9 month-old boy with
a three days history of painful swelling and multiple
pustules in right index finger.
Case: A 9 month-old boy is referred to our clinic because
of fever, erythema, swelling and multiple pustules in his
right index finger. He had been prescribed amoxicillinclavulonic, the lesion was diagnosed as bacterial whitlow.
Despite this antibiotherapy his complaints persisted. He
- 51 -
was born 3360 as the first child of non consanginous
parents. His neurologic, physical and social development
is normal. When he admitted to hospital his fever was
38.2 C, his vital signs were in normal limits. In laboratory
examination only mild leukocytosis was detected. His
family reported that his baby sitter has also herpes
labialis and the patient has finger sucking behavior for
two weeks. According to this clinical condition herpetic
whitlow was suggested, his antibiotherapy stopped and
oral acyclovir were prescribed for one week. The patient
lesions resolved within ten days.
Conclusions: Primary herpetic whitlow is a rare
condition generally caused by HSV 1. This is
usually seen in children co-occur with herpetic
gindivostomatitis or close contact to infected secretions
of daily care givers. The longer course and failure to
the antibiotherapy is definitive for herpetic whitlow.
Pediatricians should be aware of herpetic whitlow as
an occupational risk for themselves and also delayed
and failed treatment puts on the patients at risk of
superinfection and other complications.
Keywords: Primary, HSV 1, Whitlow
[P-084]
Leukocyte Adhesion Deficiency-1(LAD-1) in
a pediatric patient presenting with perirectal
abscess
Esra Yücel1, Saniye Berna Hamilçıkan2, Zeynep Gör2,
Yeşim Acar3, Ömer Faruk Beşer3
1
SB Okmeydanı EAH Department of Pediatric Allergy
and Immunology
2
SB Okmeydanı EAH Department of Pediatrics
3
SB Okmeydanı EAH Department of Pediatric
Gastroenterology
Leukocyte Adhesion Deficiency-1 (LAD-1) is a rare
autosomal recessive primary immune deficiency
characterised by the absence expression of the adhesion
molecules on leukocytes. LAD-1 is associated with
leukocytosis, recurrent bacterial infections, delayed
separation of umbilical cord and impaired healing of
wounds. Omphalitis, sepsis, gingivitis, periodontitis,
skin ulcers, perirectal abscess are the other common
clinical features of this disease. We present 3 year-old
girl admitted to our clinic because of fever, recurrent oral
ulcers and perirectal ulcer.
Case: She was the third child of consanguineous
parents. Her umbilical cord was separated at 17 days
of life. She was admitted to hospital for several times
because of pneumonia and also had recurrent oral ulcers.
She referred to our clinic for intractable fever, perirectal
ulcer and high leukocyte counts on CBC. In physical
examination periodontitis, oral ulcer was detected. Rectal
examination revealed perirectal ulcer with purulent
secretion. Laboratory data showed leukocytosis 74500
/mm3 with neutrophilia (90%). Serum immunoglobulin
(Ig) G, Ig M, Ig E, Ig A levels and Nitroblue tetrazolium
(NBT) test were normal. She had normal T,B and NK
cell numbers in peripheral blood flow cytometric analysis
but expression of adhesion molecules on leukocytes
were absent (CD 18; 0%, CD11a; 3%, CD 11b; 0%,
CD11c; 0%). 12*6 mm perirectal abscess was shown on
pelvic MRI. She was received broad-spectrum antibiotics
(sulperazone with metronidazole) for two weeks and
discharged after recovery. Prophylactic oral amoxicillin
was started.
Conclusion: Different clinical manifestations have been
reported in LAD-1 patients. Severity of symptoms is
releated to the degree of surface expression of CD18
and CD11. Curative treatment is only possible with until
hematopoietic stem cell transplantation (HSCT). Patients
should receive antibiotics prophylaxis for infections until
HSCT.
Keywords: Leukocyte adhesion, perirectal ulcer, LAD-1
[P-085]
Suspect hair tourniquet syndrome in crying
infants without an obvious reason
Engin Çarkçı1, Mehmet Esat Uygur2, Ethem Ayhan
Ünkar3, Zeynep Alp Ünkar4
1
Department of Orthopaedics and Traumatology,
Sefaköy Hospital, Medipol University, Istanbul, Turkey
2
Department of Orthopaedics and Traumatology, Vera
Hospital, Istanbul, Turkey
3
Department of Orthopaedics and Traumatology, Kartal
Yavuz Selim State Hospital, Istanbul,Turkey
4
Department of Pediatrics, Division of Neonatology,
Medical Faculty, Marmara University, Istanbul, Turkey
Background&Aims: Hair-tourniquet syndrome (HTS)
is a rare and painful condition occurring when hair or
thread pieces are tightly wrapped around toes, fingers
or other appendages, occluding blood flow and causing
strangulation or autoamputation. Our aim is to present a
HTS and discuss possible reasons of it.
Case: Four-month-old female infant was brought to
our emergency clinic because of inconsolable crying.
She was afebrile and without evident signs of systemic
infection. Detailed physical examination after removing
all clothing items revealed a hair thread circumferentially
wrapped around the left 3rd toe at the level of proximal
interphalangeal joint (Figures 1a and 1b). The distal
part of the toe was strangulated and congested.
The hair was removed under local anesthesia. Examination
of the patient’s clothes revealed long strands of hair in
her socks. Following removal of the strangulating hair,
pain was completely relieved within 12 hours; whereas,
recovery of the skin was prolonged up to 3 days.
Discussion: HTS which was first defined in 17th
century, is a rare entity not commonly known by both
pediatricians and orthopedic surgeons. Even though
child abuse should be kept in mind, the most common
reason is accidental knotting of mothers hair around the
infant’s toes. It is most commonly seen between the
2nd postnatal week and 6 months of age. The proposed
reason is telogen effluvium, a form of nonscarring
alopecia characterized by diffuse hair shedding. It
can be caused by medications or by a metabolic or
hormonal changes, especially in the postpartum period.
HTS is diagnosed by physical examination. Digital
infections can be ruled out by normal acute phase
reactants. It should be suspected in cases of crying
babies without an obvious reason, before diagnosing
- 52 -
infantile colic. Treatment is simple but if the oppurtunity
is missed, it can lead to finger/toe deformities or
amputation.
Keywords: hair-tourniquet, toe, infant
[P-086]
The use of vascularized fibular graft in
reconstruction of a long bone defect caused
by osteomyelitis in a pediatric case
Tolga Tüzüner1, Ahmet Şenel1, Ziya Demirci1, Ethem
Ayhan Ünkar2, Zeynep Alp Ünkar3
1
Department of Orthopaedics and Traumatology,
Istanbul Training and Research Hospital, Istanbul,
Turkey
2
Department of Orthopaedics and Traumatology, Kartal
Yavuz Selim State Hospital, Istanbul,Turkey
3
Department of Pediatrics, Medical Faculty, Marmara
University, Istanbul, Turkey
Introduction: Acute/subacute osteomyelitis causes
serious morbidity in children because of delays in diagnosis
and treatment. It may occur via hematogenous spread,
invasion from peripheral tissues, or contamination
from traumatic or surgical borders. Antibiotic regimens
according to antibiograms are an important part of
treatment along with surgery when necessary.
Case: 8-year-old boy was operated for displaced
subtrochanteric femur fracture. Osteosynthesis with
internal fixation was applied. On the 15th postoperative
day, discharge from a fistula on the operation region was
observed. Empirical cefazolin treatment was started and
wound culture was taken revealing Gram positive rods.
Radiological findings were consistent for osteomyelitis.
Plaque removal and debridement was performed.
Postoperatively he stayed in intensive care unit (ICU)
for one day. In the following days, a second debridement
was performed due to rising acute phase reactants
(APR). Debridement material culture was positive for
P.aeruginosa; thus, antibiotherapy was changed to
ceftazidime and amikacin. With decreasing APR, amikacin
was stopped on the 14th day and ceftazidime was
continued for another 4 weeks. At the end of treatment
period, patient was asymptomatic and APR were
negative. Fixation with a vascularized autologous fibular
graft was performed to the defective pseudoarthrosis
area caused by the early implant removal because of
infection on the initial fracture line. In follow-up visits,
healing in the graft area was observed and laboratory
findings were normal. After physiotherapy, patient
returned to his daily life.
Conclusion: Surgery for femur fractures in pediatric
age group is a crucial option with osteomyelitis as
a rare but important complication. Patient’s stay in
ICU might have caused contamination with resistant
bacterial species, complicating healing and necessitating
prolonged antibiotiotherapy. Biological reconstructive
interventions have significantly improved the clinical
outcome in pediatric patients. In literature, these kinds
of interventions were more commonly performed after
osteosarcoma resections but rarely in cases of defective
pseudoarthrosis.
Keywords: osteomyelitis, vascularized fibular graft,
pseudoarthrosis
[P-087]
Short term results of computer assisted
external fixator in treatment of neglected
clubfoot deformity
Tolga Tüzüner1, Ahmet Şenel1, Ziya Demirci1, Cenk
Ermutlu1, Ethem Ayhan Ünkar2, Atakan Telatar1,
Mustafa Güngör1
1
Istanbul Training and Research Hospital
2
Kartal Yavuz Selim State Hospital
Background&Aims: Treatment of neglected or
inadequately treated clubfoot deformity is quite
problematic. Several corrective surgical procedures have
been defined to gain painless plantigrad gait. Soft tissue
complications and risk of neurovascular insult have
made distraction osteogenesis and gradual correction
favorable over acute procedures.
We present the early results of 4 patients with
complex foot deformities treated with computer
assisted smart circular fixator in the last two years.
Methods: Lengthening of the Achilles tendon, plantar
fasciotomy and release of the posterior capsule was
performed in all cases. One patient had osteotomy
at the midtarsal level and another one had calcaneal
osteotomy. Functional outcome is assessed according to
criteria described by Ferreiara et al.
Results: Patients’ ages ranged between 12 and 16. Two
of the patients had congenital clubfoot whereas the other
2 cases had neurogenic etiology. One of the patients with
clubfoot deformity was a totally neglected case while the
other patient was initially treated in another country long
time ago. One patient had neurological sequel from head
trauma while the other case had meningomyelocele.
Mean follow-up was 8 months. In one case, gradual
correction had to be stopped and fixator was removed
because of metatarsal fracture and infection. The patient
has recovered from infection and is currently under
medication for severe osteopenia.Two patients had good
functional outcome while one patient had bad outcome.
Conclusions: Infinite hinge points principle of
computer assisted hexagonal external fixator devices
make them advantageous over conventional Ilizarov
designs for correcting neglected and complex foot
deformities. However, treatment of these deformities
is still problematic and prone to complications. Patient
compliance, surgeon’s experience and steep learning
curve should also be taken into consideration when
attempting these corrections.
Keywords: neglected, clubfoot, fixator
- 53 -
[P-088]
[P-089]
Reconstruction of radius and ulna following
infectious nonunion with segmental
bone loss in a pediatric case using non
vascularised fibular graft
Hemolytic Uremic Syndrome caused by coinfection of Salmonella Typhi and Escherichia
Coli
Tolga Tüzüner1, Ahmet Şenel1, Cenk Ermutlu1, Ziya
Demirci1, Ethem Ayhan Ünkar2, Alican Barış1, Enes
Kanay1
1
Istanbul Training and Research Hospital
2
Kartal Yavuz Selim State Hospital
Background&Aims: Infection after internal fixation
of fractures is rare in childhood. This complication is
even less frequent in fractures of the both bones of the
forearm where there is rich soft tissue vascularization.
We present a case with severe infection and resultant
bone destruction following plate fixation of Radius and
ulna.
Methods: Fourteen years old child with infective nonunion
and bone loss of radius and ulna was referred to our clinic.
He had external fixators applied to both bones. Patient
history revealed open reduction and plate fixation of both
bones 5 months prior to presentation. Plates had to be
removed because of early onset suppurative infection.
Laboratory was consistent with infection. He was
diagnosed with osteomyelitis with massive bone loss and
two stage reconstruction was planned. External fixators
were left in place, extensive debridement was carried
out and nonviable bone segments were resected en bloc.
Antibiotic impregnated spacer was placed along the bone
defect. Second stage of the treatment was begun after
6 weeks of parenteral antibiotics treatment following
return of inflammatory markers to normal levels. Bone
defects were reconstructed using nonvascularised fibular
graft fixed with compression plates.
Results: Early postoperative radiographs revealed that
original lengths of both bones were restored. Graft
incorporation was evident on plain x-rays at month 1 and
3. He had full wrist and elbow ROM at the final follow-up.
Conclusions: Osteomyelitis of the both bones of the
forearm in the pediatric population is extremely rare.
Simultaneous inoculation of both bones despite exposure
through two different incisions make hematogenous
spread more probable. Two staged treatment of implant
related infections is a widely accepted approach.
Reconstruction of massive bone loss following
infection is controversial. In pediatric patients, use of
nonvascularised fibular grafts is more suitable due to
technical simplicity and high rate of success.
Keywords: childhood, nonvascularized, reconstruciton
Ezgi Yalçın, Nilüfer Göknar, Ayşegül Doğan Demir, Faruk
Öktem
Bezmi Alem Vakıf Üniversitesi Tıp Fakültesi
Background&Aims:   Hemoltyic-uremic syndrome is one
of the most common cause of acute kidney injury in young
children.It is characterized by the triad of microangiopathic
hemolytic anemia,thrombocytopenia,and renal failure.
The most common form of HUS is diarrhea related type
and the etiologic factors are shigella toxin-producing
Escherichia Coli (STEC), Shigella dysenteriae and rarely
Salmonella Typhi.In this case we presented a patient
with HUS co-infected by Salmonella and STEC.
Methods: A previously healthy 7-year-old girl was
admitted to our emergency room with abdominal pain,
vomiting and darkening of the urine color.Laboratory
tests demonstrated anemia, thrombocytopenia and
elevated creatinine and BUN levels.A blood film revealed
fragmented erythrocytes. She was diagnosed as
Hemolytic Uremic Syndrome.After hospitalization she
received hemodialysis with a continuous monitoring of
hemoglobin levels and platelet counts. On second day
of administration she had fever as 39C with axillary
method. Salmonella Typhi was identified in the blood
culture.Clinical improvement was observed after the
start of ceftriaxone for salmonella.On follow up days,
patient had positive stool culture for enterohemorrhagic
E.coli.She was treated with intravenous ceftriaxone.On
fourth week of hospitalization she was out of dialysis
and the creatinine,hemoglobin and platelet levels slowly
turned back to normal limits.She was discharged and
received follow-up care as an outpatient.
Results: Salmonella is a microorganism involved in the
etiology of HUS but not very often encountered in clinical
practice.In our case firstly we isolated S.thyphi from
blood culture and then STEC from stool samples.Few
cases in the literature have been reported co-infected
by Salmonella and E.coli.According to Ardissino G et al.
STEC is often(35.6%–39.9%) co-infected with either
Salmonella and should be consider as STEC-associated
HUS with a co-infection
Conclusions: As a result,we consider that the Salmonella
coinfection in patients with Hemolytic Uremic Syndrome
also be kept in mind.This case was reported because of
a rare co-occurence and high morbidity associated with
the condition.
Keywords: Hemolytic Uremic Syndrome, co-infected
by Salmonella and E.coli
- 54 -
[P-090]
[P-091]
Fatih Varol1, Ayşe Ağbaş2, Beyhan Tüysüz3, Rumeysa
Yasemin Çiçek2, Firuze Erbek Alp4, Nur Canpolat2, Halit
Çam1
1
İstanbul University Cerrahpaşa Medical Faculty
Pediatric Emergency Department
2
İstanbul University Cerrahpaşa Medical Faculty
Pediatric Nephrology Department
3
İstanbul University Cerrahpaşa Medical Faculty
Pediatric Genetics Department
4
İstanbul University Cerrahpaşa Medical Faculty
Pediatrics Department
Osteopetrosis with renal tubular acidosis (RTA) is a rare
disease due to carbonic anhydrase (CA) II deficiency in
which findings of both proximal and distal renal tubular
dysfunction and extrarenal findings. We present a case
with hypokalemic paralysis who was diagnosed RTA and
osteopetrosis.
7 years old boy attended to emergency department
with muscle weakness. He had been diagnosed as
RTA with the findings of both proximal and distal renal
tubular dysfunction including a normal anion gap
metabolic acidosis with alkaline urine (urine pH as
7.0), hypouricemia, hypocitraturia, hypercalciuria, and
nephrocalcinosis at the age of 3 years. Serum potassium
level was in normal range during 4 years of follow up.
In the admission, he was lethargic, unable to hold his
head and walk. Muscle strength was reduced and he had
growth retardation and hepatosplenomegaly.
Serum potassium level was 1.4 mmol/L. There was
ST depression, flattening of T wave on ECG. His X-Ray
examination showed increased bone density without
corticomedullary differentiation, broadened metaphysis
and transverse fractures of tibia. CT scanning
demonstrated hydrocephalus, cerebral calcification,
crowded posterior fossa, hydrosyringomyelia, and
osteopetrosis. On the basis of these findings, CA II
deficiency was suspected. He was treated with potassium
citrate. His neuromuscular findings were gradually
resolved after potassium replacement.
Hypokalemia should be remembered for differential
diagnosis of muscle weakness and CA II deficiency should
be kept in mind when the patient has the combination of
RTA and osteopetrosis.
Keywords: Osteopetrosis, hypocalemıc paralysis, rta
Fatih Varol1, Fatih Aygün2, Reyhan Dedeoğlu3, Feyza
Aydın4, Sevinç Mertoğlu4, Halit Çam1
1
İstanbul University Cerrahpaşa Medical Faculty
Pediatric Emergency Department
2
İstanbul University Cerrahpaşa Medical Faculty
Pediatric İntensive Care Department
3
İstanbul University Cerrahpaşa Medical Faculty
Pediatric Cardiology Department
4
İstanbul University Cerrahpaşa Medical Faculty
Pediatrics Department
Hair-thread tourniquet syndrome is a rarely encountered
disease. It is seen primarily in extremities but other
body parts such as penis, clitoris and vulva can also be
involved. Hair-thread tourniquet syndrome occurs when
these body parts are strangulated with a strand of hair or
a piece of thread. Early diagnosis and treatment prevents
loss of involved sites. In this study we report a patient
who presented with irritability due to toe strangulation
with a strand of hair.
Four-month-old male patient presented to us with a twoday history of irritability along with redness and swelling
on his left toe. The past medical history and family history
were insignificant. In physical examination a circular
constriction line was observed on proximal phalanx of
the fifth toe of the left foot with a strand of hair hanging
from the constriction line. No other particularity was
noted in physical examination.
In the operation, which was performed by the plastic
and reconstructive surgery department, the strand
of hair was removed and circular sutures were put on
the constriction line. Due to the facts that the skin
and subcutaneous tissues were cut by the strand
of hair and the constriction line reached the bone,
the patient was put under parenteral antibiotics to
prevent a possible osteomyelitis. After a further
detailed history was taken it was understood that there
was no child abuse. The family was informed about
childcare and hygiene. The toe healed without sequela.
Hair-thread tourniquet syndrome as its name suggests,
is the condition in which a body part is strangulated with
a strand of hair or a piece of thread. Mostly in extremities
the fingers are involved; but other body parts such as
penis, clitoris and vulva can also be involved. In every
case, child abuse should also be considered.
Keywords: hair-theread tourniquet, strangulation
Hypocalemıc Paralysis In A Case With
Osteopetrosis With Renal Tubuler Acidosis
Hair-thread tourniquet syndrome in a fourmonth-old male patient
- 55 -
[P-092]
[P-093]
Tuberculous Meningitis Presenting With
Different Clinical Stages: Two Case Reports
Fatma Nur Öz1, Mehmet Yusuf Sarı2, Hatice Gamze
Poyrazoğlu3
1
Fırat University Medical Faculty, Ankara, Turkey,
Division of Pediatric Infectious Diseases
2
Fırat University Medical Faculty, Ankara, Turkey,
Division of Pediatric Intensive Care Unity
3
Fırat University Medical Faculty, Ankara, Turkey,
Division of Pediatric Neurology
It is estimated that one-third of the world’s population
has been infected with Mycobacterium tuberculosis, and
70,000 individuals have tuberculous meningitis (TM).
The clinical stage at the time of presentation is the single
most important predictor of clinical outcome.
Case 1: Fourteen years old male was referred to
our hospital diagnosis of meningoencephalitis and
hydrocephaly. His chilhood vaccination was absent. On
examination his conscious was lethargic with a Glasgow
coma score of 8/15 (E2V2M4). Cerebrospinal fluid (CSF)
examination showed white cell count 100/mm³ (%80
lymphocyte), CSF protein 238 mg/dL, CSF glucose 23 mg/
dL. CSF to blood glucose ratio was reduced. No acid-fast
bacilli (AFB) were seen in microscopy. CSF mycobacteria
PCR was positive. A brain CT showed dilatation of all
ventricles. Chest X-ray showed right middle zone
consolidation. The patient was started on antituberculous
drugs and intravenous steroids. His hydrocephaly was
worsened progresively and he underwent VP shunt but
his clinical condition deteriorated and he died on the
sixth day after admission.
Case 2: Sixteen yaers old female presented with
headache, vomiting lasting for 1 week. She had no
BCG vaccination. Her physical examination was normal
except the signs of meningeal irritation. CSF examination
showed 80/mm³ lymphocyte, CSF protein 60 mg/dL,
CSF glucose 50 mg/dL, CSF/Blood glucose ratio was
0,4. The CSF microscopy were negative but AFB were
seen. CSF mycobacteria PCR was positive. Chest X-ray
showed right lower zone consolidation. Kranial MR
showed mild hydrocephalus. The patient was started
on antituberculous drugs and intravenous steroid. She
made a remarkable clinical recovery and was discharged
home 4 weeks after admission.
Conclusion: We report two cases of TM, one of them
presented late and rapidly developed hydrocephalus the
other one has early presentation. This report is intended
to make clinicians aware of clinical stages at presentation
of TM.
Keywords: tuberculous meningitis, children,
hydrocephaly
Successful treatment of a multidrug
resistant Acinetobacter Baumannii wound
infection in a patient with ewing sarcoma
Fatma Özgüç Çömlek1, Nurhan Aruçi Kasap1, Özden
Türel2, Fatma Betül Çakır3
1
Bezmialem Vakıf University Medical Faculty,
Paediatrics, Turkey/Istanbul
2
Bezmialem Vakıf University Medical Faculty, Paediatric
Infections,Turkey/Istanbul
3
Bezmialem Vakıf University Medical Faculty, Paediatric
Hematology and Oncology,Turkey/Istanbul
Summary: Nowadays, the increasing resistance to
antibiotics that are used for treatment of Acinetobacter
baumannii infections became a serious health problem in
our country as well as all over the world. A. baumannii
causes serious nasocomial infections and outbreaks.
It quickly develops resistance therefore a combination
of drugs is required for treatment. Here we describe a
ewing sarkom patient with A. Baumannii wound infection
and discuss treatment options in A. Baumannii.
Case: A sixteen year old female patient diagnosed with
Ewings Sarcome came to us. She had a wound infection
in her primer tumor location. Laboratory findings
showed CRP 25,1 mg/dL, WBC 22,57. Wound drainage
was green and smelly. The treatment was continued by
using meropenem and vancomycin. Wound, urine and
blood materials were collected to make culture. After
surgical consultation, it was not considered any invasive
procedures only wound care was recommended with
epitelizan creams. Five days after hospitalization was
produced A. Baumannii which is multidrug-resistant,
colistin and tigecycline sensitive in the wound culture
and the treatment was replaced with colistin and
ampicillin-sulbactam. Daily wound dressing was made.
7th and 15th days after treatment were performed
wound cultures and the same bacteria was produced
again. Control culture was negative on 27th day after
treatment. Our patient has fully healed. The Patient was
discharged and was asked to carry on with the treatment
for the primer disease.
Discussion: Acinetobacter baumannii is a nonfermantative, gram-negative, non-moving, catalase
positive, oxidase negative microorganism. While these
bacteria microscopically are seen as gram-negative
bacilli(1)
Recently, It is extensively encountered with Acinetobacter
baumannii in nosocomial infections, such as meningitis,
pneumonia, VAP, infection related to central venous
catheter, urinary system infection, wound infection.
Carbapenems, polymyxin E and B, sulbactam, piperacillin/
tazobactam, tigecycline and aminoglycosides, which
are known effective against A. Baumannii, are being
preferred from antibiotic agents. (2)
Keywords: A.Baumannii, nosocomial infection
- 56 -
[P-094]
Spontaneouse pneumomediastinum with
subcutaneous emphysema in an adolescent
boy
Filiz Serdaroglu1, Tugba Koca2, Isa Döngel3, Fatma
Saltık1
1
Department of Pediatrics, Süleyman Demirel University
Faculty of Medicine, Isparta, Turkey
2
Department of Pediatrics, Division of Pediatric
Gastroenterology, Hepatology and Nutrition, Süleyman
Demirel University Faculty of Medicine, Isparta, Turkey,
3
Department of Thoracic Surgery, Süleyman Demirel
University Faculty of Medicine, Isparta, Turkey
Spontaneous pneumomediastinum, which generally
develops as a result of alveoli bursting under pressure,
is rarely seen in children and may cause families and
physicians to panic. Acute or chronic pulmonary
disease is expected in the etiology. However, it may
also be seen with exercises which increase pulmonary
pressure, without any facilitating factor. The case is here
presented of an adolescent with no known pulmonary
disease, who presented with chest pain that had started
during the normal activity of a game and the diagnosis
of spontaneous pneumomediastinum was missed in the
first physical examination.
Keywords: Children, spontaneous
pneumomediastinum, subcutaneous emphysema
[P-095]
Small bowel intussusception presenting as
seizure and lethargy
Fatouma Khalif Abdillahi1, Ufuk Erenberk2
1
Bezmialem Vakıf University, Department of Pediatrics
2
Bezmialem Vakıf University, Department of Pediatric
Emergency
Case Report: A previously healthy 1year-old boy is
brought to the emergency department with a suspected
seizure and letargy. The patient’s temperature was 37°C
rectally, pulse 125, respiration 35, and blood pressure
100/80 mmHg. There were no signs of meningitis or
intracranial hypertension, no abnormalities in sensibility
and strength, and reflexes were normal. Cardiopulmonary
examination revealed no heart murmurs, and breath
sounds were normal. The abdomen was not painful to
palpation and there were no masses. The laboratory
studies were normal, including a white blood cell (WBC)
count of 11,000,negative acute phase reactants,normal
serum electrolytes and blood lactate as well as ammonia.
Cranial MRI was planned. Air-fluid levels patern was
perceived in abdominal plain film. The transabdominal
ultrasound demonstrated a midabdominal targetshaped mass consistent with intussusception. Barium
enema confirmed the diagnosis of intussusception,
which was reducible in the radiology suite. The
patient spent discharged to home after three days.
Discussion: İntussusception is a common disease that
can present with nonspecific symptoms, and may mimic
a spectrum of serious illnesses. The findings of profound
lethargy and seizure may lead the clinician towards a
diagnosis of intracranial pathology. Fortunately, ED
ultrasonography provides quick and accurate assessment
of intraabdominal pathology that may be confounded
by another process, or masked by vague presenting
symptoms. The etiology of this alteration in status
mental is unknow. Some hypothesis are considered
to explain these clinical signs and symptoms by the
realese of cytokines produced during the disease.İn our
case, the child’s neurological state returned to normal
immediately after proper blood supply to the bowel had
been restored.The condition should be suspected in
all children presenting with the association of seizure
and lethargy if no cause is identified. Radiological or
tomographic study of abdomen should be performed
also in cases without intussusception typical symptoms.
Keywords: Intussusception, lethargy, seizure
[P-096]
A Case: Hypothyroidy In Pediatric Secondary
Pseudotumor Cerebri Syndrome
Fayize Maden, Selvinaz Edizer, Aycan Unalp, Gurkan
Gurbuz, Kazim Coban
Dr. Behcet Uz Children’s Hospital
Introduction: Idiopathic intracranial hypertension (IIH)
also known as pseudotumor cerebri is characterized by
signs and symptoms of increased intracranial pressure
in the absence of a space-occupying lesion. The causes
of IHH are numerous and include metabolic disorders
(galactosemia,hypoparathyroidism, hypophosphatasia,
prolonged corticosteroid therapy, possibly growth
hormon treatment, malnourished child, hypervitaminosis
A, severe vitamin A deficiency, obesity, menarche,
oral contraceptives), infections,drugs (doxycycline,
minocycline,tetracycline),
hemotologic
disorders
(polycythemia, hemolytic and iron-defiency anemias),
obstruction of intracranial drainage by venous thrombosis,
head injury, and obstruction of the superior vena cava.
Case Presentation: 15-year –old girl has had a headache
for 2 years. Migraine was diagnosed and analgesic
therapy was taken. The last 2 weeks raging and increased
frequency of headache, she has and papilledema with an
enlarged blind spot. Psoudotumor cerebri was diagnosed
and she was hospitalized. Physical examination, her
weight was over the 97 percentile and papilledema was
revealed. The other system examination was normal.
In order to diagnosis and treatment, lumbar puncture
was performed, CSF pressure measured 30 cmH2O.
Laborotary findings are; increased thyroid autoantibodies
and TSH, normal T3, T4 were tested and thyroid
ultrasound was interpreted in favor of thyroiditis. With
these findings, she was referred endocrinology and she
was started L-tyrosine treatment together acetazolamide.
Conclusion: IHH is a diagnosis of exclusion. This is
required to exclude all other causes of papiledema and
raised intracranial pressure. We advise considering
hypothyrodism as a potential cause of IHH. Also obesity
accompanies with IHH. We see a strong association
between IHH and recent weight gain. In thıs case, we
specified that obesity related to hypotroidsm, may cause
the pseudotumor cerebri.
Keywords: Pseudotumor cerebri, Hypothyrodism
- 57 -
[P-097]
It may not only acute pericarditis;
myopericarditis in 3 adolescent boy
Ferda Gürkan, Ali Yurtseven, Yasemin Özdemir Şahan,
Raziye Burcu Güven Bilgin, Eylem Ulaş Saz
Ege University School of Medicine, Department of
Pediatrics, Division of Emergency Medicine
Background&Aims: Acute pericarditis is inflammation
of the pericardium defined as at least 2 of the following
criteria: usually precordial or retrosternal chest pain
(sharp and pleuritic, improved by sitting up and leaning
forward), pericardial friction rub, suggestive ECG
changes (generalized elevation of the ST segment or
PR depressions), new or worsening pericardial effusion.
Changes in myocardial necrosis markers (CK-MB and
troponin) may occur for epicardial impairment and
should suggest a diagnosis of myopericarditis. In up to
70 % of pediatric patients a specific etiology cannot be
detected and myopericarditis is considered idiopathic.
Methods: The medical records of 3 consecutive patients
with acute pericarditis who presented to the emergency
department were reviewed retrospectively.
Results: Our patients were all male and had upper
respiratory tract infection with subfebrile temperature
at disease onset. According to their presentation time
patients were 15, 17 and 16 years old, respectively.
They all presented with retrosternal pleuritic chest pain
and had suggestive ECG changes which were identified
as following; patient 1 (elevation of the ST segment in
DI, DII), patient 2 and 3 (elevation of the ST segment in
V5,V6). The mean troponin,CRP, ESR level was 0.364ng/
ml (normal <0.014ng/ml), 3.6mg/dl and 30mm/h
respectively. Echocardiogram showed that entire patients
had minimal pericardial effusion. Patient 1 and 3 was
treated by Ibuprofen, IVIG and ibuprofen used for patient
2. Myopericarditis was idiopathic or viral in 100 % of cases.
Conclusions: Acute myopericarditis is most often either
idiopathic or viral. The diagnosis is based on clinical
criteria and laboratory testing. The evaluation and
treatment of patients with acute pericarditis can be
performed in the ambulatory setting unless the patient
has myopericarditis. In most patients, acute pericarditis
has a brief and benign course and an excellent response
to NSAIDs, alone or in combination with other agents.
Keywords: adolescent, myopericarditis
[P-098]
Dyskinesia and Kranial MRI changes caused
by vitamin B12 in a 1 year old child
Hüseyin Kılıç1, Feyza Aydın2, Sema Saltık1
1
Cerrahpaşa Tıp Fakültesi Çocuk Nöroloji Anabilim Dalı,
İstanbul
2
Cerrahpaşa Tıp Fakültesi Çocuk Sağlığı ve Hastalıkları
Anabilim Dalı, İstanbul
Introduction: B12 vitamin deficiency of first the years
of the life generally arises from malnutrition and depleted
maternal stores. Deficiency of this vitamin may cause to
hematologic, neurologic and psychiatric disorders.The
aim of this article is an evaluation of the development
of encephalopathy-like clinical symptoms in a 1 year old
child with B12 insufficiency and the cure of the clinical
and radiological signs of the dyskinesia-like complications
occurring after B12 vitamin replacement.
Case: 12 months old healthy girl was born as a second
child of healthy parents, with no consanguineous
marriage and familial metabolic disorders history was
admitted to the hospital due to somnolence, inability to
feeding. In cranial magnetic resonance imaging (MRI),
acute diffusion restriction and hyperintensity in the
T2 sequences were detected on the inferior aspect of
the bilateral lentiform nucleus. Very low level of B12
vitamin ( 30pg/ml) and anaemia was detected and B12
was replaced as a possible cause of these clinical signs.
Symptoms were resolved after replacement and the
patient was discharged.This patient was admitted to the
hospital after one month with complaints of dyskinetic
movements in her face and arms. There was no any other
pathology besides dyskinesia and these symptoms were
resolved after intravenous midazolam treatment. After
B12 replacement serum B12 level was 1496 pg/ml and
any pathologic finding was not found in metabolic and
toxic analysis and in EMG of this patient. Maternal serum
B12 level was low, as well (135 pg/ml). The patient was
discharged with clonazepam treatment. In her control
observation, dyskinetic symptoms were declined totally
and clonazepam treatment was ceased. MRI signs were
resolved in a control assessment after three months, as
well.
Conclusion: In a clinical scenario with a basal ganglionic
involvement in the first years of the life, B12 depletion
also should be taken into account.
Keywords: vitamib b12, diskinezia
[P-099]
Vitamin B12 deficiency in childhood
Sinan Yavuz, Ozgul Yigit, Meltem Erol, Zubeyde Kilinc
Yaprak, Dilara Fucucuoglu, Feyza Coskun
Bagcilar Training and Research Hospital, Pediatric
Department, Istanbul, Turkey
Background&Aims: Vitamin B12 deficiency is usually
evoked in presence of compatible hematologic or
clinical (usually neurologic) signs. Clinical findings
are usually characterized by anemia, leucopenia,
thrombocytopenia, anorexia, irritability, failure to
thrive, developmental delay or regression, cognitive
dysfunction and irreversible neurological damage.
However, many cases of deficiency are asymptomatic.
In this study we aimed to screen clinical and
hemotological findings of the children determined vitamin
B12 deficiency (incidentally or on clinical suspect).
Methods: Clinical and laboratory features screened from
medical automation system retrospectively. Clinically
features (age, gender, complaints) and laboratory
findings (hemoglobin, hematocrit, mean corpuscular
volume, ferritin, serum iron levels, total iron binding
capacity) are recorded and evaluated
Results: In our study 127 patient included. Of patients
%53 were female(n=67) %47 were male (n=60). The
- 58 -
main complaints of patients were paleness in %48, flu
like symptoms in %31, failure to thrive in %18. Vitamin
B12 deficiency is more frequently seen in groups aged
0-2 and aged 12-17. We determined mean vitamin
B12 values 168.1±34.1 pg/ml, mean hemoglobulin
values 12±1.9 g/dl, mean MCV values 78.4±8.1 fl. The
hemotological features were anemia in %38, bicytopenia
in %4, thrombocytopenia in %9 pancytopenia in %3,
macrocytosis in %2 of patients. There is no significant
difference in mean hemoglobin and RDW levels between
the patients with low iron levels and normal ones.
Conclusions: Vitamin B12 deficiency are primarily
investigated in patients with anemia and neurological
symptoms. Based on the knowledgement that vitamin
B12 deficiency can be asymptomatic we suggest to
investigate vitamin B12 levels especially in the ages of
increased growth rate.
Keywords: Childhood, Vitamin B12 deficiency
[P-100]
Hereditary Angioedema Case Report
Feyza Coşkun1, Hikmet Tekin Nacaroğlu1, Meltem Erol1,
Özgül Yiğit1, Raif Coşkun2
1
Ministry of Health Bağcılar Research and Training
Hospital, Pediatric Clinic
2
Istanbul University, Faculty of Medicine, İnternal
Disease, Department of Allergy and Immunology
Background&Aims:
Hereditary
angioedema
is
an autosomal dominant inherited disease which is
characterized by C1 inhibitor deficiency or dysfunction
and recurrent attacks of angioedema. Angioedema
most often seen in limbs, oropharynx and visceral
organs. Intestinal mucosal edema can cause temporary
bowel obstruction and severe abdominal pain that can
be confused with acute abdomen. Abdominal attacks
accompanied by 50% of all attacks. Before the diagnosis
of hereditary angioedema some of the patients may
undergo unnecessary appendectomy or exploratory
laparotomy. The most feared complication of hereditary
angioedema is laryngeal edema
Case: Twelve years old boy presented with swelling of the
face, eyes and limbs and recurrent abdominal pain since
he was 1 year old. It was learned that the attacks resolve
spontaneously within 48 hours and not accompanied by
pruritus or urticaria and his uncle has similar complaints.
When he was 9 years old, he has been appendectomy
for abdominal pain. Physical examination was normal.
C4:5,9 mg/dL (10-40) and C1 inhibitor: 8,1 mg/dL (2440) levels was found to be low. C1 inhibitor concentrate
is prescribed for use in attacks.
Conclusions: Hereditary angioedema is a rare autosomal
dominant inherited disease which is characterized by
C1 inhibitor deficiency or dysfunction and recurrent
attacks of angioedema. C4 levels are low in almost all
cases and it is used as a screening test. Corticosteroids,
antihistamines and epinephrine which are used in the
treatment of allergic angioedema has no place in the
treatment of hereditary angioedema. To replace the
missing C1 inhibitor for the treatment of attacks fresh
frozen plasma and recombinant C1 inhibitor (CetorR) are
used in our country.
We want to raise awareness among emergency medicine,
family and pediatric physicians about hereditary
angioedema, a rare but potentially lethal disease, with
this case report
Keywords: Hereditary, Angioedema
[P-101]
A rare cause of cardiac arrest:
catecholaminergic polymorphic ventricular
tachycardia
Firuze Erbek Alp1, Sezen Ugan Atik2, Reyhan Dedeoglu2,
Aida Koka2, Funda Oztunc2, Ayse Guler Eroglu2
1
Istanbul University Cerrahpasa Medical Faculty
Department of Pediatrics
2
Istanbul University Cerrahpasa Medical Faculty
Pediatric Cardiology Department
Introduction:
Catecholaminergic
polymorphic
ventricular tachycardia (CPVT) is characterized by
episodic syncope occurring during exercise or acute
emotional stress in children without structural cardiac
abnormalities. In people with CPVT, these episodes
typically begin in childhood. If CPVT is not recognized,
an episode of ventricular tachycardia may lead to cardiac
arrest and sudden death. Generally CPVT results from
mutations in the RyR2 gene. Here we will present a case
who was admitted to the emergency service with cardiac
arrest and diagnosed as CPVT.
Case: 2-year-old male patient presented to our
emergency department with cardiac arrest. Cardiac
arrest had developed after a syncope attack when he
was playing. The patient had a history of recurrent
syncope attacks and had been treated for epilepsy. It
stated that the syncope usually occurred after exertion.
Cardiopulmonary resuscitation (CPR) and intubation
was performed and the patient was brought back to
life. There were ventricular extrasystoles in the ECG.
Because of hypotension dobutamine and norepinephrine
treatment was started for positive inotropic support.
After starting the dobutamine treatment ventricular
tachycardia,
ventricular
fibrillation
and
cardiac
arrest developed, respectively. Dobutamine was
stopped immediately. Cardiopulmonary resuscitation
was performed and the patient returned to sinus
rhythm. Amiodarone was administered for ventricular
tachycardia. Echocardiography couldn’t detect structural
cardiac pathology. According to these findings, CPVT
was suspected and genetic testing was performed. The
genetic test revealed heterozygote missense mutation
(c.9110G>A(p.Gly3037Asp)) in exon 64 of RYR2 gene.
The patient did well under amiodaron treatment without
any ventricular tachycardia. Intracardiac defibrillator
implantation was recommended to patient’s family but
they didn’t accepted because of severe neurological
damage.
Conclusion: Catecholaminergic polymorphic ventricular
tachycardia is a rare cause of sudden cardiac death in
children.
Keywords: Catecholaminergic polymorphic ventricular
tachycardia, cardiac arrest
- 59 -
[P-102]
Prenatally diagnosed asymptomatic cardiac
rhabdomyoma
Firuze Erbek Alp1, Sezen Ugan Atik2, Aida Koka2,
Reyhan Dedeoğlu2, Levent Saltık2
1
Istanbul University, Cerrahpasa Medical Faculty,
Department of Pediatrics
2
Istanbul University, Cerrahpasa Medical Faculty,
Department of Pediatric Cardiology
Introduction: Rhabdomyoma is the most common
cardiac tumor in children often associated with tuberous
sclerosis (TS). Arrhythmia due to cardiac rhabdomyomas
may be the initial sign of TS. We report a case of
rhabdomyoma which diagnosed prenatally because of its
large dimensions and despite of its large dimensions it
was asymptomic during the postnatal course.
Case: We presented a case of a preterm male newborn
who was born to primigravida at 35 weeks of gestation.
After birth the baby’s physical examination was
unremarkable in the delivery room. The mass didn’t
cause arrhythmias or inflow-outflow obstructions after
birth. Fetal echocardiography at 31 weeks of gestation
had revealed a large mass in the interventricular septum
suggestive of rhabdomyoma. Postnatal echocardiogram
demonstrated a large cardiac mass, which had large
extensions (16x16 mm) along interventriculer septum.
There was no evidence of inflow or outflow tract
obstruction. No tachycardia was observed. There was
no need for treatment. Renal ultrasonography, cranial
Magnetic Resonance imaging, genetic evaluation for TS
weren’t investigated yet. Family members were also
examined for stigmas of TS and no significant clinical
findings were detected. A month after discharge from the
hospital, the child was doing well without any treatment.
Follow-up echocardiographic assessments were planned.
Our expectation was spontaneous regression of the mass
in the following echocardiographic assessments.
Discussion:
While
rhabdomyomas
may
be
asymptomatic, they can also cause arrhythmias,
inflow-outflow obstructions and even cardiac failure.
The symptoms may occur depending on the anatomic
location of the tumor.
Keywords: cardiac rhabdomyoma, tuberous sclerosis
[P-103]
When the Paediatric Intensive Care Unit
(ICU) plays the role of the Emergency
Department (ED)... A two-year retrospective
study
Foteini Zafeiropoulou, Maria Vasilopoulou, Olga
Giannouli, Zoi Beka, Leda Sianidou
Paediatric Intensive Care Unit, Penteli Children’s
Hospital, Athens, Greece
Background & Aims: The role of an ED in a tertiary
hospital is the emergency management and acute care
of severely ill and injured patients. An ICU undertakes
the continuous monitoring and specialized treatment of
unstable and critical patients. In many settings, due to
lack of organized EDs, patients in need of emergency
treatment get admitted in the ICU and that is a burden to
the patient as well as the health care system. This study
aims to underscore the fact that a percentage of ICU
admissions could have been managed in an organized
ED.
Methods: This is a retrospective observational study
based on the ICU register during the period September
2013 until September 2015. Population: ICU admissions
through the Outpatient Department.
Inclusion criteria: a. Patients who received emergency
treatment and got stable during the first few hours of
their stay in the ICU.
b. Patients who were nursed in the ICU
solely for monitoring of their vital signs
c. Patients who were admitted for interhospital transfer.
Results: Total ICU admissions: 107. Twelve of those
fulfilled the inclusion criteria representing 11,2% of the
total. In particular, there were:
a. 4 patients that were rapidly resuscitated after
anaphylactic reaction, asthma crisis, croup and
hypovolemia
b. 5 that only needed vital signs’ monitoring and
c. 3 stable patients with a new diagnosis of brain tumor
that were transferred to neurosurgical centers.
Conclusions: An ICU admission is an unpleasant
experience for little patients. It comes with a number of
tests and interventions and staying in an unhospitable
environment without their family. Additionally, the daily
cost of ICU stay climbs up to 700 €. It is self-evident
that we ought to do everything in our power to decrease
unnecessary ICU admissions. The development of
organized EDs will be a key factor in this endeavor.
Keywords: Emergency, Intensive, Paediatric
[P-104]
Protein loosing enteropathy in an infant with
concomitant rotavirus infection and Celiac
disease
Gamze Ozgurhan1, Gokten Ongel1, Meltem Yildirim1,
Lida Bulbul2, Zerrin Onal2, Bulent Hacihamdioglu1
1
Suleymaniye Maternity and Children’s Training and
Research Hospital, Department of Pediatrics, Istanbul,
Turkey
2
Bakirkoy Dr. Sadi Konuk Training and Research
Hospital,Department of Pediatrics, Istanbul, Turkey
Introduction: Protein-loosing enteropathy (PLE) is
a rare complication of a variety of intestinal disorders
characterized by an excessive loss of proteins into the
gastrointestinal tract due to impaired integrity of the
mucosa. The etiology of PLE is various. Rarely case of
Rotavirus gastroenteritis complicated by PLE has been
reported. It is known that Celiac disease is the reason of
severe form of PLE. Here in we report an infant who was
developed PLE after acute Rotavirus gastroenteritis and
diagnosed as Celiac disease during follow-up.
Case presentation: A 12-month-old boy presented
with vomiting, diarrhea and weight loss for 7 day
prior to admission. Subsequently, he was hospitalized
- 60 -
because of dehydration. The physical examinations
revealed that weight: 8200 gr(weight for height: 81%),
pretibial edema, and abdominal distention. Laboratory
examinations showed that hypoalbuminemia (2.1 g/dl)
with mild hypertransaminasemia. A stool examination
was positive for Rotavirus antigen. There was not
proteinuria in urinalyses. Abdominal ultrasound showed
bowel expansion and intestinal wall thickening with
a small amount of ascites. He diagnosed as Rotavirus
infection induced PLE but in follow-up his diarrhea
persisted more than 2 weeks. He was evaluated other
reason of chronic diarrhea and antibodies associated with
Celiac disease were detected positive. Intestinal biopsy
was confirmed Celiac disease diagnosis. His symptoms
were fully improved after gluten free diet.
reproduction was seen in hemoculture and BOS culture,
treatment was continued with cefotaxime. The patient
was discharged with minimal-mid level hydrocephalus
findings in cranial USG and MR following 21 days of
antibiotics treatment.
Conclusions: In clinical practice; if proteinuria is not
detected in children admitted with edema, PLE must
be considered. Rotavirus gastroenteritis is not always
self-limited and may due to PLE especially infant with
previous silent Celiac disease.
Keywords: Protein-loosing enteropathy, Rotavirus,
Celiac disease
[P-106]
[P-105]
E.coli meningitis after rotavirus
gastroenteritis in an infant
Gamze Ozgurhan1, Oznur Vermezoglu1, Didem Ocal
Topcu1, Adem Karbuz2, Bulent Hacihamdioglu1
1
Suleymaniye Maternity and Children’s Training and
Research Hospital, Department of Pediatrics, Istanbul,
Turkey.
2
Okmeydanı Training and Research Hospital,
Department of Pediatrics, Istanbul, Turkey
Introduction: Although Rotavirus gastroenteritis
is quite common in pediatric population, secondary
bacterial sepsis and/or meningitis following Rotavirus
infection are rare clinical entities.Gram-negative bacilli
are the fifth most common cause of meningitis in infants
but its rarely occur after gastroenteritis. Here in we
report an infant who developed E.coli meningitis after
acute Rotavirus gastroenteritis.
Case presentation: 2.5 month male infant with fever,
vomiting and watery diarrhea that started 1 day ago was
admitted to hospital. Past medical and family history
revealed that he was born with term delivery with no
special postnatal history. Apart from mild dehydration
and irritation, physical examination was normal. Initial
leukocyte count was 10.100/mm3 and C-reactive
protein was 19,3 mg/L (references; 0-8). Rotavirus
antigen in stool sample was positive. No pathogenic
bacteria reproduction was seen in stool culture. He was
hospitalized because of oral intolerance and dehydration
due to Rotavirus gastroenteritis. Fever measured at
second day of hospitalization was 39,5oC and he was
irritable on physical examination. Lumbar puncture was
done for suspicion of meningitis. In analysis of the CSF; a
neutrophilic pleocytosis (68% PMNL, 32% lymphocytes),
elevated protein (127 mg/dl), and reduced glucose
concentration (11 mg/dl, simultaneous blood glucose:
91 mg/dl) were found. Intravenous vancomycin and
cefotaxime were started empirically. Since E.coli
Conclusions: Rotavirus infection is not always
benign, pediatricians should be kept in mind bacterial
translocations and severe life-threating infections in
patients with Rotavirus infection.It is vital to start
prompt antibiotic treatment as soon as the diagnosis of
secondary bacterial infection is made because of high
mortality and morbidity rates.
Keywords: Rotavirus, bacterial sepsis
15-month case of septic arthritis without
swelling and rash
Gamze Sarıkaya Uzan1, Mustafa Uzan2, Funda Kökali
Atash1, Bahar Çalışkan1, Nilgün Selçuk Duru1
1
Haseki Education and Research Hospital, Department
of Pediatrics
2
Şişli Hamidiye Etfal Education and Research Hospital,
Family Medicine C
Introduction: Septic arthritis is the suppurative
inflamation of the joint often caused by bacteria.
Lifelong bone and joint damage affecting the childs life
can develop due to inadequate diagnosis and delayed
treatment. Clinical findings of the disease is mostly
seen under age of 2 years and may be weak in this age
group. Here we submit 15-month old septic arthritis
case presented with fever but no redness or swelling of
the knee joint.
Case: 15-month-old girl, was admitted to emergency
department with complaint of 38.4 ° C fever. At systemic
examination, patient had poor general status and
increased discomfort with pressure on non sweeling
and non redness left knee, and no other findings. Blood
samples showed that leukocyte: 19.1 10⁹/uL, C-Reactive
Protein: 438mg/l (0-5). Arthrocentesis sample obtained
from knee joint was cloudy, gray colur and high dansity
and leukocyte: 150,000/mm ³ erythrocyte: 200/
mm ³ in cell counting. Orthopaedic clinicians took
the patient to emergency operation. Vancomycin,
ceftriaxone and age-appropriate rehydration therapy
was started and postoperative 2. day acute phase
reactants were significantly decreased. Synovial fluid
cultures Staphylococcus aureus reproducing patient
was discharged after 4 weeks of intravenous antibiotic
treatment to child infection outpatient follow-up.
Result: Fever is a symptom that has to be clarify the
cause in children.Other symptoms and complaints
accompanied with fever must be examined thoroughly
and all systemic examination must be certainly done
with detailed medical history. Even if they do not have
any abnormality,locomotor system necessarily should be
examined, joint sensitivity should be explored. This case
is submitted to focus on detailed systemic examination
especially on infants when fever cause culdnt be cleared,
before invasive procedure and further screening.
Keywords: Fever, Physical examination, Septic arthritis
- 61 -
[P-107]
[P-108]
Gizem Pamuk1, Fatih Varol2, Gürcan Dikme3, Hande
Kumkumoğlu3, Duhan Demircioğlu1, Halit Çam2, Tülin
Tiraje Celkan3
1
Istanbul University Cerrahpasa Medical Faculty,
Department of Pediatrics, Istanbul
2
Istanbul University Cerrahpaşa Medical Faculty,
Department of Pediatrics, Pediatric Emergency, Istanbul
3
Istanbul University Cerrahpaşa Medical Faculty,
Department of Pediatrics, Pediatric HematologyOncology, Istanbul
Introduction: Hemophilia A is an X-linked,recessive
disorder caused by deficiency of functional plasma
clotting factor VIII.The development of inhibitory
antibodies to FVIII can result in acquired hemophilia A or
can complicate the treatment of genetic cases.Patients
with hemophilia A may bleed into tissues or from
damaged skin and mucosal surfaces.Bleeding from the
renal tract may not be recognized as a sign of a severe
bleeding disorder in patients who have not undergone
excretory urography.Awareness of other possible causes
of hematuria should prompt more frequent investigation
of the urinary tract in such patients.We report a case
of spontaneous acute renal hematoma in a 5-year-old
boy,who was diagnosed with hemophilia A.
Gizem Pamuk1, Fatih Varol2, Duhan Demircioğlu1, Duygu
Ülger1, Halit Çam2
1
Istanbul University Cerrahpasa Medical Faculty,
Department of Pediatrics, Istanbul
2
Istanbul University Cerrahpaşa Medical Faculty,
Department of Pediatrics, Pediatric Emergency, Istanbul
Introduction:
Cutis
marmorata
telangiectatica
congenita is a congenital, rare, usually benign, sporadic,
localized or generalized vascular lesion characterized
by a persistent purple or blue “marbled” or “fishnet”
appearance of the skin. In this report, we report a
preterm female newborn with CMTC at birth who showed
typical cutaneous features.
Spontaneous renal hematoma in a patient
with Hemophilia A
Case: A 5-year-old boy diagnosed with hemophilia A
was presented with painless gross haematuria that
had started 5 days before admission.There was no
history of trauma or fever.At the time of presentation
of hematuria,he was prescribed metilprednisolone.The
hematuria persisted on the 5th day of metilprednisolone
and he was referred to the pediatric emergency clinic.
At the admission,vital signs revealed blood pressure
110/70 mmHg,respiratory rate 23/min,pulse rate 96/min
and body temperature 36.7°C.The laboratory test results
were as follows:haematocrit 37,6%,haemoglobin 11,9 g/
dl,white blood cell 11,5×103/μl,platelet 792×103/
μl,prothrombin time 11,4 s, partial thromboplastin time
84,8 s,INR:0,9, serum albumin 4,2 g/dl, serum urea
22 mg/dl and creatinine 0,5 mg/dl.Urinalysis showed
many red blood cells without pyuria.Urine culture was
negative. Kidney ureter bladder radiography did not
reveal any radio opaque stone, while the initial abdominal
US imaging revealed a hyperechogenic image of 14x12
mm in the left renal pelvis.The doppler US revealed no
abnormality.Bed rest and iv hidration treatment was
ordered. He responded well to treatment: the haematuria
has gradually regressed.
Conclusion: Renal hematoma should be considered as
one of the possible causes of haematuria in hemofilia
patients.Awarness of this cause should prompt more
frequent investigation of the urinary tract in such
patients.
Keywords: Hemophilia A, hematuria, renal hematoma
Cutis Marmorata Telangiectatica Congenita
in a female newborn: A Case Report
Case: A female newborn was presented with persistent
blue marbled appearance of the skin since birth. She
was the first child of non-related parents, spontaneously
delivered at 33 weeks of gestational age. During first
hours of life, she was interned in a neonatal intensive
care unit due to respiratory distress with the diagnosis of
transient tachypnea of the newborn and discharged after
20 days. Since birth marbled bluish and deep purple
reticulated skin lesions involving the whole body were
noted. Physical examination showed reticulated bluish
purple skin changes all over the body including the face
and limb. The lesions were unvarying and permanent and
became more visible with crying and exposure to room
temperature. The baby had normal facies, with head
circumference of 33 cm and birth weight 2135 gr, without
other vascular anomalies or asymmetry of the growth
of the limbs. The systemic examination was normal.The
laboratory test showed haematocrit 29,4%,haemoglobin
9,8 g/dl,white blood cell 10,5×103/μl,platelet 410×103/
μl,serum urea 5 mg/dl and creatinine 0,4 mg/dl,serum
albumin 3,6 g/dl. Serum electrolytes, liver enzyme
tests and serum lactate level were normal. The
abdominal and cranial ultrasound imaging were normal.
Conclusion: Clinical characteristics and persistence
of cutis marmorata prompted the diagnosis of Cutis
Marmorata Telangiectatica Congenita.Vascular anomalies
as Sturge–Weber syndrome, Klippel–Trenaunay–Weber
syndrome have been associated with CMTC. The case
we reporte had a normal face with no other vascular
abnormality, and systemic examination was normal.
She had no asymmetry of the limbs.The disorder is
self limiting and treatment is not necessary unless
complicated with other associated anomalies.
Keywords: Cutis marmorata telangiectatica congenita,
cutis marmorata, congenital vascular anomaly
- 62 -
[P-109]
[P-110]
Diagnosis of Pseudohypoparathyroidism
during a routine care of an asthmatic child:
Do pediatricians aware enough about the
disease?
Gaucher disease in Russia - pediatric
experience
Gizem Sayar Bilgin1, Zehra Yavaş Abalı2, Muhammet Ali
Varkal1, İsmail Yıldız1, Firdevs Baş2, Emin Ünüvar1, Ayşe
Kılıç1
1
Istanbul University Istanbul Medical Faculty, Pediatrics,
General Pediatrics, Istanbul
2
Istanbul University Istanbul Medical Faculty, Pediatrics,
Pediattric Endocrinology, Istanbul
Background: Pseudohypoparathyroidism (PHP) is
characterized by hypocalcemia, hyperphosphatemia,
increased serum concentration of parathyroid hormone
(PTH), and insensitivity to the biologic activity of PTH.
We report a patient diagnosed with PHP incidentally
during her routine control.
Case: A seven year old girl presented with the history
of recurrent cough since she was one year of age. Two
years ago before the admission she had been incidentally
diagnosed as having mild low calcium values and she
had been treated with oral vitamin D. Vitamin D and
serum calcium levels were normal within 3 months of
treatment. The family didn’t took her to regular visits
for about two years. On her recent admission she
evaluated for spasms in her face. Her weight 30.6 kg
(1.71 SDS), height 135 cm (2.80 SDS), and body mass
index were 16.8 kg/m2 (0.6 SDS). Chvostec’s sign
was positive. Systemic examinations were normal and
there were no subcutaneous calcifications. Besides mild
round face, she had no AHO feature and no intellectual
disability. At first evaluation serum calcium:6.5 mg/
dl (8.8-10.2 mg/dl), phosphorus: 7.68 mg/dl (2.7-4.5
mg/dl), magnesium:1.77mg/dl (1.5-2.5 mg/dl) alkaline
phosphatase:421U/l (70-300 U/l), 25 hydroxy vitamin
D3:35.5 ng/ml (>20 ng/ml) and PTH were 1400pg/ml
(15-65 pg/ml). There were no renal pathologies and she
had hypocalciuria. After the treatment of symptomatic
hypocalcemia, oral calcium replacement and calcitriol
were added to the therapy. Symptoms resolved and
serum calcium gradually increased. Her 4th metacarpals
were short and brain MRI revealed no calcification.
Conclusion: In this patient with PHP we learned that as a
rare disease PHP must come to attention by pediatricians
when evaluating children with hypocalcemia. As it’s
already known, hypocalcemia may induce laryngospasm
and cough/respiratory problems in this patient’s history
may also misdiagnose hypocalcemic episodes.
Keywords: asymptomatic, Pseudohypoparathyroidism
Goar Movsisyan, Leyla Namazova Baranova, Kirill
Savost’anov, Olga Gundobina
FSBI “Scientific Center of Children’s Health” of the
Ministry of Health of the Russian Federation
Background&Aims: To describe our 15 years
experience in the management of patients with Gaucher
disease(GD) by multidisciplinary assessment.
Methods: An observational, retro- and prospective
study based on Russian pediatric GD registry data of
SCСH. The diagnosis was confirmed by biochemical and
molecular genetic research.
Results: Based on registry data 2000-2015 years the
total number of pediatric patients with GD in Russia was
90; the age was between 0 to 18 years; M:F 39:51;
Type1:Type2:Type3
78(87%):2(2%):10(11%).
The
mean age of a debut and diagnosis was less then 7
years. The most common signs and symptoms noted
were: growth retardation (25% in or below the third
percentile for weight and 34% in height; majority
between ages of 2 to 5 years), hepatosplenomegaly
(95%, 4 patients were splenectomized), anemia(90%),
thrombocytopenia(85%), skeletal involvement by
X-ray(85%), bone pain(30%), bone crises(10%),
hemorrhagic syndrome(65%), reduced bone mineral
density(60%), neurologic symptoms (11%). All patients
had low glucocerebrosidase activity. In molecular
analysis we identified 13 novel mutations; genotypes
N370S/L444P and N370S/W184R were most common
in type1, the most common genotype identified in
neuronopathic forms was L444P/L444P. The additional
important biochemical tests at diagnosis was high
chitotriosidase activity and ferritin level; low iron, serum
lipids and v.B12 levels. The 87 patients were treated by
imiglucerase, 3 patients - by velaglucerase alpha, the
dose of ERT in type1 was from 30 to 60 U/kg/2weeks,
in type3 - from 60 to 100 U/kg/2weeks. Most of our
patients presented the significant improvement of the
course of disease after 2-10 years of ERT.
Conclusions: Our study shows the wide clinical and
genetic variability of GD in pediatric patients. National
pediatric GD registry is a good model for other rare
diseases because it provides us the advanced knowledge
about certain pathology and helps to improve the
management and the life quality of these patients.
Keywords: Gaucher disease, multidisciplinary
assessment, children
- 63 -
[P-111]
[P-112]
Leukocyte Adhesion Deficiency
Gökçen Kamış1, Esin Aldemir2, Çiğdem Aydoğmuş3,
Sultan Kavuncuoğlu2
1
Kanuni Sultan Süleyman Training and Research
Hospital,Department of Pediatrics
2
Kanuni Sultan Süleyman Training and Research
Hospital,Neonatology Clinics
3
Kanuni Sultan Süleyman Training and Research
Hospital,Pediatric İmmunology Clinics
Introduction: Leukocyte adhesion deficiency(LAD) is a
rare primary immune deficiency that characterized by
inability of neutrophil migration to inflammatory area.
In our clinics, an infant who referred to the hospital with
malaise and high grade fever is diagnosed with LAD-1
severe form after further investigations.Because of the
rarity of this syndrome,the case is presented.
Case Presentation: A four days old male referred to
the our clinics with high grade fever, feeding difficulty
and grunting. Laboratory investigations showed
WBC:34x103/µL,ANC:27x103/µL,CRP:115
mg/L.
Chest radiography was normal. After blood, urine and
CSF culturing, the antibiotic treatment was started as
Ampicilina, Cefotaxime and Amikasin. Blood and CSF
culture result in growth of Gram(+) alpha hemolytic
streptococcus which is susceptible to the treatment.
During hospitalization, fever relapsed and he has
omphalitis and nonpurulent abscess formation in axillary
skin area. His laboratory findings showed WBC:36x103/
µL, ANC:30x103/µL, CRP:36,2mg/L, Procalcitonin:0,17.
Vancomycin and Cefoperazone+Sulbactam is started.
Blood immunoglobulin levels were found in normal by
age. In the day 26,his umbilical cord detached.Flow
cytometry shows the leukocyte CD18 levels of %0.He
was diagnosed with LAD-1 severe form.
Discussion: Migration of leukocytes from circulation to
inflammatory area has four steps:recruitment,rolling,firm
adhesion,extravasation. In LAD-1,leukocytes are unable
to perform firm adhesion to the blood vessel walls due to
the deficiency of ß2 integrin subunit,CD18. This molecule
is coded by ITGB2 gene,chromosome 21q22.3.Thus far
86 different mutations have been defined in the gene
The levels of CD18 determine the severity.
CD18 expression is below %2 in severe form.
Immune deficiency is seen in early infancy. Th symptoms are
delayed umbilical cord detachment,omphalitis,recurrent
and resistant infections,nonpurulent necrotizing infections
Laboratory
findings
are
remarkable
neutrophilia,leukocytosis,high inflammatory markers.
Conclusions: In the cases of severe neonatal sepsis
that has low response to antibiotics, severe leukocytosis
with neutrophilia,recurrent nonpurulan infections and
the delay of the umbilical cord detachment,LAD-1 should
be considered
Keywords: immunodeficiency, infant
Coexistence of Glanzmann’s thrombastenia
and Glucose 6 Phosphate Dehydrogenase
deficiency in one patient
Gözde Yücel1, Gürcan Dikme2, Hande Kızılocak
Kumkumoğlu2, Begüm Şirin Koç2, Gül Nihal Özdemir2,
Tülin Tiraje Celkan2
1
Istanbul University, Cerrahpaşa Medical Faculty,
Department of Pediatrics
2
Istanbul University, Cerrahpaşa Medical Faculty,
Department of Pediatric Hematology Oncology
Introduction: Glanzmann’s thrombastenia (GT) is a
genetic platelet surface receptor disorder of GPIIb/
IIIa which results in faulty platelet aggregation,
diminished clot retraction and it is more common in
ethnic groups that display higher rates of consanguinity.
Case: A 3 month old male patient was brought into
our emergency department with a widespread rash
of one week duration. The patient have parents with
consanguinity and a birth history of a term C/S delivery
without any significant complication. Postnatally he had
prolonged jaundice and after the etiologic assesment,
at 1 month of age, he was diagnosed with Glucose 6
Phosphate Dehydrogenase deficiency with an enzyme
level of 6mU/10 RBC.
The first physical examination revealed a petechial
rash that is widely distributed over the face; the whole
body of the child with many scattered purpuric lesions.
According to the parents the occurrence of these lesions
was spontaneous and unprovoked. There was not any
associated abnormal physical findings.
The complete blood count was entirely normal:
Hemoglobin level 9.7 g/dL, white blood cell count
10400/mm3, platelet count 350000/mm3. The PT (12.4
seconds) and aPTT (30.6 seconds) were also normal.
However, the bleeding time was prolonged; more than
10 minutes, which warranted further investigation.
For assessing platelet function we used light transmission
aggregometry (LTA); platelet aggregation failed to occur
with ADP and collagen, except ristocetin, where the
reaction was found to be preserved. For further analysis
we used flow cytometry. This analysis revealed that
CD41(GPIIb) and CD61 (GPIIIa) levels were absent.
Overall, our patient with G6PD deficiency was also
diagnosed with Glanzmann’s thrombastenia.
Conclusion: We have diagnosed 2 distinct hematologic
entities in one patient which are not related and have
not been observed coincidentally in our clinical practice
before.
Keywords: Glanzmann’s thrombastenia, glucose 6
phosphate dehydrogenase deficiency, purpura
- 64 -
[P-113]
Cayler Cardiofacial Syndrome: A rare
condition in newborns
Gunce Basarir1, Bedia Deniz Ekici1, Abdülhamit Tüten2,
Handan Hakyemez Toptan2, Nilgün Karadağ2, Gülsen
Akay1, Güner Karatekin2
1
Children’s Health Department, Zeynep Kamil
Gynecologic and Pediatric Training and Research
Hospital, Istanbul, Turkey
2
Newborn Intensive Care Unit, Zeynep Kamil
Gynecologic and Pediatric Training and Research
Hospital, Istanbul, Turkey
Cayler cardiofacial syndrome is a rare syndrome
characterised by asymmetric crying facies associated
with unilateral absence or hypoplasia of the depressor
anguli oris muscle, and congenital heart defects.
Asymmetric crying facies (ACF) is usually noticed when
a newborn cries and the affected angle of the mouth
is pulled downward while contralateral side is immobile.
The face seems symmetric during sleep. ACF can be seen
as an isolated minor event but the important aspect of
this abnormality is the association with other congenital
anomalies; mostly in the cardiovascular system.
Coexistance of unilateral hypoplasia/aplasia of the
depressor anguli oris muscle and various congenital heart
defects is called Cayler snyndrome and we report a case
of ACF with tricuspid atresia, ventricular septal defect
(perimembranous), atrial septal defect (secundum),
patent foramen ovale, and proximal stenosis of left
pulmonary artery. We emphasize the importance of being
aware that this minor facial anomaly may be associated
with other congenital anomalies; cardiac defects mostly.
Keywords: Cayler, cardiofacial
[P-114]
Late Diagnosis of DHPR Deficiency Treated
as PKU: A Case Report
Gurkan Tarcin1, Ertugrul Kiykim2, Tanyel Zubarioglu2,
Ayse Cigdem Aktuglu Zeybek2, Erdogan Soyucen3, Serif
Cansever4
1
Istanbul University, Cerrahpasa Medical Faculty,
Department of Pediatrics, Istanbul, TURKEY
2
Istanbul University Cerrahpasa Medical Faculty,
Department of Pediatrics Division of Nutrition and
Metabolism, Istanbul, TURKEY
3
Akdeniz University, Department of Pediatrics Division
of Nutrition and Metabolism, Antalya, TURKEY
4
Istanbul University, Cerrahpasa Medical Faculty Central
Laboratory, Istanbul, TURKEY
Dihydropteridinereductase (DHPR) deficiency is an
inborn defect of tetrahydrobiopterin (BH4) recycling that
leads to decrease in levels of dopamine, serotonin and
catecholamines in cerebrospinal fluid (CSF) and causes
neurological symptoms such as hypotonia, psychomotor
delay and seizures. Here, we present a case of a 27-monthsold male patient who is lately diagnosed as DHPR deficiency
after having been followed up with a misdiagnosis
of phenylketonuria (PKU) since 2 months of age.
A male infant was diagnosed as mild PKU according to the
results of newborn screening and was begun to receive
phenylalanine (phe) restricted diet. Phe levels were
monitored closely and found between normal ranges.
At 6 months of age, generalized hypotonia developed
and phe levels started to increase. Patient was referred
to our outpatient clinic for further evaluation and
treatment. His physical examination revealed profound
truncal hypotonia dominated with dystonic movement
disorder. BH4 metabolism disorders were considered
in differential diagnosis due to hyperphenilalaninemia
and neurological findings. Neurotransmitter levels
and pterin metabolites were performed in CSF sample
and found compatible with DHPR deficiency. L-Dopa,
5-hydroxytryptophan and folinic acid replacement
therapies and phe restricted diet were started immediately
and the patient achieved a good clinical response.
In our country, frequency of inherited metabolic diseases
is high due to consanguineous marriages. As a result,
all neonates should be screened for metabolic disorders.
In all patients who have hyperphenylalaninemia in
metabolic investigations, BH4 metabolism disorders
should be included in differential diagnosis.
Keywords: Dihydropteridinereductase deficiency,
DHPR deficiency
[P-115]
Osteogenesis imperfecta – experience of
Scientific Center of Children’s Health in
Moscow
Guzal Yakhyaeva, Tea Margieva, Kirill Savostyanov,
Alexander Pushkov, Natalia Jurkova, Konstantin Jerdev,
Nato Vashakmadze, Anait Gevorkyan, Alexander
Baranov, Leyla Namazova Baranova
Scientific Center of Children’s Health
Background&Aims: Osteogenesis imperfecta (OI)
is a hereditary genetic disorder of connective tissue,
characterized by bone fragility and recurrent fractures
due to the defects of collagen 1 type. We want to describe
our 4 year experience in the management of patients
with OI by multidisciplinary assessment.
Methods: An observational, retrospective study based
on data obtained from the analysis of the clinical files of
all patients with OI. The studied variables were: age at
diagnosis, familial history of OI, age at fracture, number
of fractures, molecular genetic mutation (by nextgeneration sequencing (NGS), medical/surgical therapy
Results: 30 pediatric patients (male=19; female=11,
mean age 6.1 years), by clinical and radiographic
evaluation registered n=12 type I, n=9 type III, n=8
type IV, n=1 type V. 8 patients had familial history of
OI. A causative variant in the COL1A1 gene was found
in 5 cases; in the COL1A2 gene was found in 3 cases.
A causative variant in the COL1A1 gene near the amino
(N)-proteinase cleavage site, which result an overlap OI/
Ehlers-Danlos syndrome was found in 1 case. In other 7
cases was found mutations in the COL5A1/2 gene (n=5),
in the COL3A1 gene (n=1). 19 patients underwent
medical treatment with pamidronate, starting at an
average of 4.16 years. In follow-up sample (n=18) there
was a decrease in the number of fractures after starting
treatment (average 9.1 to 5.8 fractures/patient).
- 65 -
Surgical treatment of bone deformities was performed in
10 patients (33,3%).
Conclusions: OI is disease with a wide clinical and
genetic variability with insignificant molecular-genetic
correlation. There are still no effective treatment,
however medical treatment with early intervention of
bisphosphonates seems to reduce the incidence of new
fractures and surgical treatment with intramedullary
rods, the last ones not registered in Russia, seems to
improve functional outcomes and decrease the level of
disability
Keywords: bone fragility, collagen 1 type, pamidronate
[P-116]
two brothers who were diagnosed with
hyper IgE syndrome on the basis of
Molluscum contagiosum
Mebrure Yazıcı1, Ayşegül Demir2, Gülsüm Güzel2,
Mustafa Atilla Nursoy1, Erkan Çakır3, Özlem Su4
1
Bezmialem Vakıf üniversitesi Çocuk Alerji Bilim Dalı
2
Bezmialem Vakıf üniversitesi Çocuk Sağlığı ve
Hastalıkları Bilim Dalı
3
Bezmialem Vakıf üniversitesi Çocuk Göğüs Hastalıkları
Bilim Dalı
4
Bezmialem Vakıf üniversitesi Dermatoloji Bilim Dalı
Introduction: OD hyper-IgE which is a type of
eczema, that is usually observed in recurrent skin and
lung infections with high serum IgE levels on various
skeletal systems, which is a rare primary immune
deficiency syndrome associated with connective tissue
and vascular anomalies. Hyper IgE syndrome is an
autosomal recessive severe eczema type with recurrent
bacterial and viral skin infections (such as Molluscum
contagiosum) which could be observed with neurological
symptoms in addition to sinopulmonary infection.
Molluscum contagiosum is a round and saddle-backed
DNA pox virus infection characterized by white papules,
which is commonly observed on the facial skin, the
eyelids, the neck and axilla.
Case: A 9 and a 3 years-old two brothers who are recurrent
lung infections, was admitted to our clinic with complaints
of cough and phlegm. The older brother had the recurrent
lung infections while his younger brother since 4 months
had similar infections and it was found out that on their
body along with these infections contain wart-like lesions.
During the whole body examination many saddle-backed
(middle sunken)white papules were detected. Molluscum
contagiosum infection was diagnosed with a skin biopsy.
We have noticed that there was also the big brother’s finger
clubbing. We have also observed on big brother’s chest CT
lesions and bilateral infiltrates with bronchiectasis after
seeing chronic changes and problems on chest X-ray.
Lymphocyte subsets, IgA, IgG and IgM levels were within
normal limits while it was thought there is an immune
deficiency during the investigation of the patients.
Younger brother’s IgE levels was found to be 530 IU /
mL while the older one’s was 2779 IU / ml. After these
findings the patients were diagnosed with hyper IgE
syndrome on the basis of Molluscum contagiosum.
infiltrations can be seen in hyper-IgE syndrome.
Keywords: Molluscum contagiosum, OD hyper-IgE
[P-117]
A Rare Infection in Children: Pelvic
Osteomyelitis
Halil Özdemir1, Tuğçe Tural Kara1, Adem Karbuz2, Anıl
Tapısız3, Suat Fitöz4, Ergin Çiftçi1, Erdal İnce1
1
Department of Pediatric Infectious Diseases, Ankara
University Medical School, Ankara, Turkey
2
Department of Pediatric Infectious Diseases,
Okmeydani Education and Research Hospital, İstanbul,
Turkey
3
Department of Pediatric Infectious Diseases, Gazi
University Medical School, Ankara, Turkey
4
Department of Pediatric Radiology, Ankara University
Medical School, Ankara, Turkey
Acute
hematogenous
osteomyelitis
is
typically
seen in long tubular bones in children. Pelvic bone
involvement is so rare and diagnosis is sometimes
delayed, due to deep localization of infection. Herein,
we report a pediatric patient with pelvic osteomyelitis.
A 15-year-old girl was admitted because of the left groin
pain without trauma and fever. The pain was continuing
approximately one month which was constant during rest
and movement. On physical examination, temperature
was 38.0 C⁰. She could not walk without support.
Besides she did not exactly stand on his left foot. Her hip
joint was limited in movement. Pelvic radiography did
not show any pathological findings. Magnetic resonance
imaging showed bone marrow and muscles were detected
with pathological signal changes during ischium-pubic
branch. We considered osteomyelitis and malignant
infiltrative process. Then, bone marrow aspiration was
done and it was found to be normal. Bone scintigraphy
determined symphysis pubic, lower part of the left
sacroiliac joint and left acetabular region had increased
uptake activity, left iskium bone revealed pathological
uptake. Biopsy and curetage of infiltrative lesion was
performed. Biopsy material culture was unremarkable.
Tuberculosis polymerase chain reaction, acid resistant
bacillus and culture were negative, but the diagnosis
of osteomyelitis was confirmed by pathology results.
Ceftriaxone treatment was given during one month. Also
oral ciprofloxacin and amoxicillin-clavulanate treatment
was received one month after discharge. At the end
of treatment the patient recovered without sequelae.
As a result; pelvic osteomyelitis is a rare infection in
children. Pelvic magnetic resonance and scintigraphy
are useful for diagnosis. However, diagnoses should be
confirmed with culture. Sometimes diagnosis delays due
to deep localization of site infection. If the diagnosis is
suspected, imaging techniques should be used.
Keywords: Acute hematogenous osteomyelitis,
children, pelvic osteomyelitis
Conclusion: Moloscum contagiosum and chronic lung
- 66 -
[P-118]
SPINAL TUBERCULOSIS (POTT’S DISEASE),
A CASE REPORT
Halise Zeynep Işcan1, Özgür Genç2, Özgecan Avcı1
1
Department of Pediatrics,Şişli Hamidiye Etfal Training
and Research Hospital,İstanbul,Turkey
2
Departmant of Radiology,Şişli Hamidiye Etfal Training
and Research Hospital,İstanbul,Turkey
Aim:
Tuberculosis
is
primarily
a
pulmonary
infection,extrapulmonary
manifestations
are
not
uncommon,especially
in
children.
The
classic
manifestation of tuberculous spondylitis is progression
to Pott’s disease,in which destruction of the vertebral
bodies.Early diagnosis and treatment is important to
avoid complications.We hereby present a case getting
spinal tuberculosis diagnosis which starts with a
complaint of the incapability to walk at the age of 2.
Case: A 2-year-old female patient had abrupt complaint
of incapability to walk,back pain.Thoracic intradural
mass was observed in spinal MR.By external centre,a
biopsy was conducted on it,resulted necrotizing
granulomatous inflammation.In physical examination
of the patient who was sent to us,there was plegia in
both lower extremities.Gastric fluid was taken 3 times,it
was ARB negative and there was no reproduction in
culture results.PPD was negative(8mm,there is one
BCG),she had a bidirectional chest scan,it was normal.
Her family scan was negative,Quantiferon was negative.
In spinal MR that she had before treatment, nodular
lesion covering total L2 corpus vertebrae and a lesion
including cystic expansion and septation covering in
spinal tract between D8-L2 were detected. 4(quad)
anti-tuberculosis treatment was started.Prednol(2mg/
kg/day) was started for pressure findings.Her left leg
movements improved on 4th day of prednol.Prednol
treatment was in exact dose during 4 weeks,in halfdose during 2 weeks,stopped at the end of 6 weeks.
After 2 months,treatment is changed to 2(dual) antituberculosis.She still receives 2(dual) anti-tuberculosis
treatment (3rd month of treatment),is followed by
Paediatric Infectious Disease Clinic. She does physical
treatment exercises and can put her feet on the ground.
Conclusion: Spinal tuberculosis(Pott’s Disease) is
a disease remaining to be diagnosed as its onset is
insidious,its radiological findings progress slowly.It
has early and late neurological findings,even if early
neurological findings go down by anti-tuberculosis
treatment, late neurological cases are mostly irreversible.
Prognosis is excellent with adequate medical treatment.
Keywords: Tuberculosis, Pott’s Disease, Neurological
Deficit
[P-119]
evaluation of nosocomial bloodstream
infections in our unit detected in a 3-year
period
Adil Umut Zübarioğlu, Ali Bülbül, Halise Zeynep Işcan,
Hasan Sinan Uslu, Evrim Kıray Baş, Duygu Besnili Acar
Department of Pediatrics,Şişli Hamidiye Etfal Training
and Research Hospital,İstanbul,Turkey
Introduction: Nosocomial blood stream infection is an
important cause of mortality and morbidity in neonatal
intensive care units (NICU). In this study, it was aimed
to determine blood culture positive nosocomial infections
in 3 year period(2011-2013).
Materials-Methods: Blood culture samples, taken
from the NBCU between 2011-2013, were examined.
Reproduction was determined.Organisms, isolated
from blood culture, association with the length of
stay, gestational age and birth weight of the newborn,
antibiotic susceptibility,mortality rates were determined.
Findings: Throughout the study period,121 blood
culture positive nosocomial infection episodes in 85
cases were evaluated. 55(64.7%)of the cases was
premature,38 (44.7%)was female,mean gestational age
was 31 weeks,mean birth weight was 1812gr.The most
common admission diagnoses were prematurity(n=55,
64.7%), congenital anomaly/syndromic infants(n=10,
11.8%),perinatal
asphyxia(n=7,
8.2%).Mean
hospitalization duration was 67 days.Distribution of the
microorganisms; gram positive bacteria 65.3%(n=79),
gram negative bacteria 26.4%(n=32),candida 8.3%
(n=10).While coagulase-negative staphylococci(n=69)
was seen most among gram positive bacteria, klebsiella
species of gram negative agents(n=12) were most
common. Number of the cases lost in the study group
was 17 (premature; n=10, congenital anomalies /
syndromic infants; n=5, asphyxia; n=2) and mortality
rate was 20%.The gestational weeks of the newborns
who were lost were significantly(p=0.025). Antibiotic
resistance of the gram positive factors were as follows;
penicillin 100%, clindamycin 63.3%, methicillin 89.8%
and vancomycin resistance 26.6%. As to gram negative
bacteria; gentamicin 65.6%, cefotaxime 78%, amikacin
28% and carbapenems 18.7%.
Result: In our study, it was revealed that in our NICU,
nosocomial infections occurred mostly due to gram
positive factors, mortality in nosocomial infections was
higher in those with low birth weeks, and antibiotic
resistance was high in all nosocomial infections.
Keywords: nosocomial bloodstream infections,
neonatal intensive care units
[P-120]
Hyperımmunglobulin E Syndrome: A Case
Report
Abdulkadir Bozaykut, Handan Ayhan Akoğlu, Rabia
Gönül Sezer, Nil Yazar Alpay
Zeynep Kamil Maternity and Childrens’ Diseases
Training and Research Hospital, Istanbul,Turkey
Introduction: Hyperimmunoglobulin E syndrome (HIE)
is a primary immunodeficiency disease characterized
by markedly high titers of serum immunoglobulin E,
chronic eczema, recurrent staphylococcal infections,
pneumatoceles, reduced neutrophil chemotaxis and
variable impaired T cell function. The main characteristic
laboratory abnormalities are highly elevated serum IgE
levels and eosinophilia. Here, we report a case with
hyperimmunglobulin E syndrome.
- 67 -
Case: A 45 days-old male infant presented with
fever,vomiting
and swelling
in left underarm,
hospitalized with the diagnosis of lymphadenitis. He was
the first child of a non-consanguineous marriage. He
had a history of hospitalization in the newborn period
because of hypoglycemia, ichthyosis, leukocytosis
and sepsis with MRSA and candida in blood cultures.
On examination he had coarse facial features, rough
skin (Figure 1 and 2), palpable axillary and inguinal
lymph nodes, other system examinations were normal.
Laboratory investigations revealed; Hg 7.5 g/dl, WBC
28900/mm3, absolute eosinophil count 550/mm3,
platelet 74000/mm3, CRP 5.2 mg/dl, serum IgE 978 IU/
ml (range:0-15 IU/ml). Bone marrow aspiration revealed
eosinophilia and increased eosinophil precursor cells
with no malignant infiltration. Hyperimmunoglobulin E
syndrome was suspected and further investigation was
scheduled but unfortunately patient was lost due to
sudden clinical deteriation with septic shock.
Conclusion: Hyperimmunoglobulin E syndrome is a
rare genetic disease characterized with coarse facies,
recurring cutaneous and visceral bacterial infections due
to staphylococci, severe generalized eczema, which may
be associated with osteoporosis. Biochemistry includes a
high level of total and specific IgE and blood eosinophilia.
Although the etiology of HIE syndrome is unknown,
there is evidence that patients with this syndrome have
abnormalities in cellular and humoral immune responses.
We present this case to remind that an abcess could
present first as a lympadenitis and in cases of suspected
immundeficiencies, prompt antibiotic treatment can be
life-saving.
Keywords: Hyperımmunglobilin E Syndrome,
Eozinofilia, Leukocytosis
[P-121]
Does a child need to go to a routine dental
visit: parents’view
Rabia Gönül Sezer, Abdulkadir Bozaykut, Handan Ayhan
Akoğlu
Zeynep Kamil Maternity and Childrens’ Diseases
Training and Research Hospital, Istanbul,Turkey
Aim: In Turkey, 84.9 % of children aged between 5-9
have dental caries which is an important health issue.
We aim to evaluate the views and knowledge of parents
about the need and the timing for the first dental visit of
their children.
Methods: Parents from pediatrics outpatient clinics were
invited to fill in a questionnaire. The survey included
questions on socio-demographic characteristics; parents
gender, age, number of children, parents education level,
opinion on the need for routine dental visits and the age
at which a child should attend the first dental visit. The
statistical analyses were made by SPSS version 17.
Results: Two hundred sixty-nine questionnaires were
returned Mean age of the parents was 30.6±6.9 years
(range: 18-62, median: 30). The participants had
amedian of 2 children (range: 1-8). The study population
consisted of 231 (85.9 %) mothers and 38 (14.1 %)
fathers and 33 (12.3 %) parents had a university degree.
Only 45 parents answered both of the two questions
correctly. 195 (72.5 %) of parents agreed that a child
should go routinely to a dentist and only 60 (22.3 %)
suggested the age for the first dental visit to be under
1-years of age. There was no significant relationship
between the gender of the parents, number of children,
educational status, age of the parents and the correct
answers (p>0.05).
Conclusion: Childrens’ oral health has a serious impact
on childrens’ general health. Parents knowledge and
attitudes about dental care are important issues for the
improvement of children’s oral health. Educational public
health programs may help improve the knowledge about
dental health, also pediatricians unique role in child care
should include parental education about dental care.
Keywords: dental care, children
[P-122]
Two siblings with congenital glucose
galactose malabsorption from the same
family
Hasret Ayyildiz Civan1, Tanyel Zubarioglu2, Ayse Cigdem
Aktuglu Zeybek2, Yair Anikster3, Ben Pode Shakked3,
Tufan Kutlu1
1
Istanbul University Cerrahpasa Medicine Faculty
Department of Pediatric Gastroenterology, Hepatology
and Nutrition
2
Istanbul University Cerrahpasa Medicine Faculty
Department of Pediatric Metabolism and Nutrition
3
Tel Aviv University Faculty of Medicine Department of
Medical Genetics
Background:
Congenital
glucose
galactose
malabsorption (cGGM) is a rare disease with an autosomal
recessive pattern of inheritance. The disorder leading to
osmotic diarrhea in early infancy is caused by mutations
in the Na+/glucose cotransporter gene SLC5A1 which
encodes SGLT1 protein, a sodium dependent transporter,
providing absorption of glucose and galactose from the
brush borders of small intestine.
Case Report: In this report we present two siblings
with tenacious diarrhea from the same family whose
parent had a second degree of kinship. Our first case
was a two-month-old male infant admitted with fever,
growth retardation, nephrolithiasis, acute renal failure,
hypoalbuminemia and diarrhea. As he did not recover
from renal failure with medical treatment, hemodialysis
was initiated. By the fourth day of treatment, he had
normal levels of creatinine and electrolytes. However,
diarrhea was present since his birth and continued.
After ruling out microbiological and biochemical causes,
breastfeeding was quitted and a formula containing
fructose as the main carbohydrate source was initiated
for a possible diagnosis of cGGM. After the formula,
frequency of defecation decreased immediately and
other biochemical markers improved. Our second case
was the sister of the previous patient. She admitted with
diarrhea and growth retardation when she was 7-monthold. As her elder brother was diagnosed with cGGM and
the current symtoms were mild form of that he suffered,
cGGM was considered as a possible diagnosis and
- 68 -
feeding with a fructose-based formula was tried. It was
also efficacious for her symptoms. Both of them gained
weight following the treatment and an homozygous
mutation of SLC5A1 gene was detected in their gene
analysis.
Conclusion: As the disease can progress with lethal
complications owing to lack of early diagnosis, it should
be included in differential diagnosis of patients with
chronic diarrhea in early infancy. Diet is useful for both
diagnosis and treatment.
Keywords: infantile diarrhea, malabsorption, SLC5A1
[P-123]
Corrosive esophagitis in a two days old
neonate
Hasret Ayyildiz Civan1, Sahin Hamilcikan2, Gulnaz
Sariyeva1, Didem Gulcu3, Tulay Erkan1
1
Istanbul University Cerrahpasa Medicine Faculty
Department of Pediatric Gastroenterology, Hepatology
and Nutrition
2
Istanbul Sisli Kolan Hospital, Neonatal Intensive Care
Unit
3
Adana Gynecologic, Obstetric and Paediatric Diseases
Hospital, Department of Paediatric Gastroenterology
Background: Benzalkonium chloride is a caustic agent
which is used in farms, homes and hospitals for cleaning
skin, mucous membranes and wounds as an antiseptic
solution. Caustic substances may lead to respiratory
and/or digestive system injuries in case of ingestion.
Such injuries are rare in neonates and emanate mostly
from erroneous practices of parents.
Case Report: We present a two-days-old newborn case
which was carried to the emergency unit with complaints
of poor breastfeeding, uneasiness and crying for 4-6
hours. Her physical examination was unremarkable
except mildly elevated intestinal sounds and weakness
of sucking reflex. When her mom was questioned
again, it was revealed that she had given a spoon of
10% BAC solution (approx.10-15cc) for her cough with
the intention of treatment and that she did not vomit
thereafter. Blood gases and laboratory blood tests
were in normal ranges. A gastroscopy performed in the
second hour of her admission revealed an hyperemic and
edematous mucosa in the middle third of esophagus and
a circumferential mucosal ulceration followed in the distal
portion. She was transferred to neonatal intensive care
unit and a conservative treatment including intravenous
fluid, total parenteral nutrition, H2 receptor blocker and
an antibiotherapy of cephasol and amicasin for 10 days
was administered. A control gastroscopy on the 10th
day of hospitalization demonstrated that the damage on
the mucosa was almost totally improved. She was the
youngest case with this etiology and successfully treated
with conservative approach.
Keywords: corrosive, benzalkonium chloride, neonate
[P-124]
Olanzapine-induced atypical neuroleptic
malignant syndrome in an adolescent male
with anorexia nervosa
Hasret Ayyildiz Civan1, Senol Turan2, Didem Gulcu1,
Cana Aksoy Poyraz2, Esra Pehlivanoglu3, Mehmet Kemal
Arikan2, Fugen Cullu Cokugras1
1
Istanbul University Cerrahpasa Medicine Faculty
Department of Paediatric Gastroenterology, Hepatology
and Nutrition, Istanbul-Turkey
2
Istanbul University Cerrahpasa Medicine Faculty
Department of Psychiatry
3
Istanbul University Cerrahpasa Medicine Faculty
Department of Paediatrics
Background: Anorexia nervosa (AN) is a serious mental
illness of adolescents and young adults which involves
fatal restriction of food intake and resulting physical,
cognitive and social deteriorations. Antipsychotics have
been especially used for weight gain and body focused
delusional thoughts of these patients and neuroleptic
malignant syndrome (NMS) is one of their rare fatal
complications. We report a case of AN who developed
NMS with a low dose of olanzapine.
Case Presentation: A 17-year-old male referred
by psychiatry department admitted with weight loss,
restriction of food intake and intermittent binge eating
followed by vomiting. On admission he had a BMI of 11.9
kg/m2. He achieved a weight gain of 10 kg with nutritional
and cognitive behavioral therapy within the first four
months. After a quarrel with his family, however, his
vomiting recurred. In order to increase his weight and to
prevent vomiting, olanzapine 5mg/day was initiated. On
the second day of olanzapine therapy, he developed high
fever (axillary 40ᴼC) unresponsive to antipyretics, muscle
rigidity in extremities and stupor without any sign of
meningeal irritation, infection and abnormal laboratory
test results. Despite discontinuation of olanzapine,
sporadic episodes of fever, muscle rigidity and elevated
creatine kinase levels were observed even thirty days
after the recovery, all of which ceased spontaneously
within following five days. No other explanation could
be made for his complaints at that time and he has been
followed up in pediatric outpatient clinic.
Conclusion: Although administration of olanzapine was
discontinued, NMS persisted up to a month after the
onset. Clinicians should bear in mind that the course of
NMS may be heterogeneous. In addition, one should be
careful while prescribing antipsychotic medications to
paediatric cases and patients with AN who are severely
underweight.
Keywords: anorexia nervosa, neuroleptic malignant
syndrome, olanzapine
Conclusion: Although the outcomes of corrosive
esophagitis with benzalkonium chloride may be fatal,
a conservative approach may occasionally be sufficient
owing to early diagnosis and neonatal wound healing
with minor sequela.
- 69 -
[P-125]
Intussusception in a newborn: a rare and
unusual cause for intestinal obstruction
Alper Aykanat1, Hatice Güllüelli1, Abdülhamit Tüten2, Ali
Çay3, Osman Hacıosmanoğlu4
1
Department of Pediatrics, Zeynep Kamil Maternity and
Children’s Training and Research Hospital, Uskudar,
Istanbul, Turkey
2
Department of Pediatrics, Division of Neonatology,
Zeynep Kamil Maternity and Children’s Training and
Research Hospital, Uskudar, Istanbul, Turkey
3
Department of Pediatric Surgery, Avicenna Hospital,
Esenler, Istanbul, Turkey
4
Department of Pediatrics, Avicenna Hospital, Esenler,
Istanbul, Turkey
Background&Aims: Intestinal obstruction is a
life threatening emergency in neonatal period
and is usually caused by necrotizing enterocolitis,
meconium ileus and surgical causes. Intussusception
is rarely reported in neonatal period as a cause of
intestinal obstruction. We report a newborn presented
with intussusception and wanted to underline
the importance of accurate and timely diagnosis.
Case: A 3350 g male baby was born to a G1P0 mother
after 38 weeks of gestation. After routine newborn care,
enteral feeding is started immediately. The newborn
presented with bilious vomiting and progressive
abdominal distension in first 8 hours of life and enteral
feeding is terminated. Abdominal radiograph revealed
dilated intestinal loops with air-fluid levels and raised
suspicion to a possible intestinal obstruction. Abdominal
ultrasound imaging diagnosed intussusception near
ileo-cecal junction. The newborn was operated within
postnatal 1st day. Intra-operative findings confirmed ileoileal intussusception, affected segment was resected and
end-to-end anastomosis was made. The newborn was
discharged on post-operative 10th day with full health.
Conclusions: Intussusception in neonatal period
is a rarely encountered entity and can easily be
misdiagnosed unless a high degree suspicion is present.
The etiology and pathophysiology of instussuseption
remains unrevealed in newborns. Physical examination,
laboratory tests and abdominal radiograph may not be
sufficient for accurate diagnosis. Surgical procedures can
be delayed due to late diagnosis. Ultrasound imaging is
proved to be helpful and gains time in diagnosis.
Keywords: Intestinal obstruction, Intussusception,
Newborn
[P-126]
Isoniazid intoxication: Case report
Hatice Öztürk, Arzu Aras, Aynur Bedel, Özlem Ketenci
Altıkardeşler, Çağatay Nuhoğlu
Haydarpaşa Numune Education and Research Hospital,
Department of Paediatry
Isoniazid (INH) is a bactericidal agent that is used for
treatment and prophylaxis of tuberculosis infection.
Exposure to INH in high doses may cause seizures and
mental changes.
A 16 years old male patient, was admitted to the
pediatric emergency department with seizure. In his first
examination, he was unconscious and had a generalized
tonic-clonic seizure. After intravenous (IV) diazepam
administration to the patient, seizure stopped, 15
minutes later unconsciousness relieved. In his medical
and family history, there wasn’t no special finding except
taking INH for the prophylaxis of tuberculosis for a
week. When the family was questioned, we learnt that
our patient was using ten pills once a day. We planned
to give intravenous pyridoxine (B6) to the patient for
the treatment. Since we had no intravenous pyridoxine
form in this hospital, we gave oral pyridoxine (250
mg capsule). Following this treatment, in the second
day of hospitalization, all of physical examination and
laboratory findings relieved.
All of the patients who are taken to the emergency
departments of the hospitals with seizure or
unconsciousness, should be evaluated for trauma
exposure or medicine ingestion. They should be kept in
mind for the etiology of seizures especially if the patients
are not epileptic. Early diagnosis and treatment of INH
intoxication has a great importance in order to decrease
mortality and morbidity.
Keywords: Isoniazid, seizure, pyridoxine
[P-127]
A Rare Infection In A Newborn:
Meningococcal Meningitis
Hatice Öztürk, Arzu Aras, Aynur Bedel, Umut Durak,
Dilşad Koca, Çağatay Nuhoğlu
Haydarpasa Numune Education and Research Hospital,
İstanbul,Turkey
Meningitis is inflammation of the arachnoid membrane
and pia surrounding the brain and spinal cord. Neonatal
period is a period which bacterial meningitis is more
commonly seen than other ages. Neisseria meningitidis
is an encapsulated gram-negative bacterium; there is no
reservoir in other aliens in nature. It can cause either
limited infection or septic shock syndromes. Although it
is seen in all age groups, it is more common in children.
After delivered from 22-year-old mother in expected
date with normal spontaneous vaginal route, the male
newborn was admitted to our clinic in 3730 g weight
and on postnatal day 22 with fever. The patient with
neonatal sepsis was hospitalized in intensive care unit
for further examination and treatment. In history, there
is no remarkable data except for a visit of patient’s
relative returning from a pilgrimage. Peripheral blood
smear supported sepsis. Following lumbar punction,
cerebrospinal fluid(CSF) examination revealed abundant
polymorphonuclear leucocytes. Antibiotic therapy was
started. Gram-negative diplococcic was isolated in CSF
culture. On the 7th day of treatment, control lumbar
puncture was performed, 25 cells were counted. Control
CSF cultures and blood cultures were evaluated. Visual
and hearing tests were planned for possible complications.
Invasive meningococcal disease can be seen in high
risk population especially in younger than 2 years of
children, 15-19 year-old adolescents, students and
young adults in dormitories and military recruitments in
army. Meningococcemia is still a systemic disease with
high morbidity and mortality. Under 5 years of age, it
- 70 -
is responsible for 9.5% of all mortality in our country.
By presenting this rarely seen infection in neonatal
period, we pointed out the importance of diagnosis and
treatment for meningococcal meningitis.
Keywords: Meningitis, newborn, Neisseria meningitidis
[P-128]
Group A Streptococcal Meningitis in a
Newborn: Transmission from Mother
Hatice Sınav Ütkü1, Şifa Şahin1, Gamze Demirel2, Ayhan
Taştekin2
1
Medipol University, Department of Pediatrics
2
Medipol University, Department of Neonatology
Introduction:
Group
A
Streptococci
(GAS;
Streptococcus Pyogenes) is an agent that may result
in bacteremia, sepsis, menengitis and deep soft tissue
infections and associated to 0,2-1 % of the meningitis
cases. Here we report a newborn who hospitalized for
meningitis and the etiological agent was GAS which
was also detected at the throat culture of the mother.
Case Report: A 22 days old term female neonate
presented with fever and poor sucking for six hours.
She was hospitalized for initial diagnosis of sepsis. In
laboratory test results, infection markers increased and
leukopenia was detected. We performed lumbar puncture
which is full of leukocytes on microscopic examination.
We started ampicillin and cefotaxime ampirically.
Because of the deterioration of clinical condition,
patient was intubated. We added inotropic agents
related to developing hypotension, sodium replacement
therapy was given for hyponatremia and phenobarbital
administered for the convulsion. GAS reproduced in the
cerebrospinal fluid and blood culture. We detected GAS in
her mother’s throat culture. There are bilateral hypodens
encephalomalacic areas especially in left cerebral
hemisphere, also multipl hyperdens haemorrhagic areas
detected in patients’ cranial computed tomography.
EEGs report was bioelectric silence. She was
discharged at 22th day of hospitalization. The patient
is on follow up, she is now on 6th month of age, the
neurologic examination is normal except microcephaly.
Conclusion: GAS meningitis is a serious disease rarely
seen in newborns. Parent and siblings of the patients
may constitude the source of the infection. Clinicians
should always consider GAS in the differential diagnosis
of neonatal sepsis and meningitis and appropriate
therapy should be given.
Keywords: group a streptoccocci, newborn, meningitis
[P-129]
asymptomatic case: 16 month-old duchenne
muscular distrophy
Haticenur Kirar1, Ihsan Kafadar1, Cavid Serdarzade1,
Attila Alp Gözübüyük1, Fatih Kirar2, Ozan Özkaya1
1
Department of Pediatrics, Okmeydanı Training &
Research Hospital, Istanbul, Turkey.
2
Department of Neurosurgery, Haseki Training &
Research Hospital, Istanbul, Turkey.
Duchenne muscular dystrophy (DMD) is one of the
most common neuromuscular disorders of childhood.
Progressive degeneration of muscle is responsible for
the clinic of patients. Symptoms begin at the age of
3–5 years. A case of a 16 month-old boy with DMD
who was initially admitted the emergency department
with febrile convulsion was reported. No symptoms and
family history was noted. In his physical examination,
bilateral stiffness of calf muscle(gastrocnemius) was
interpreted as pseudohypertrophy. Other organ-systems
were normal. Laboratory evaluation showed elevation
of alanine amino transferase (ALT), aspartate amino
transferase (AST), serum creatine kinase (CK) and
lactate dehydrogenase (LDH). His chest X ray was
normal. His genetic analysis revealed a deletion implying
at 45-51 exons of the dystrophin gene. A molecular
work-up of the parents showed his mother was the
carrier. Genetic consultancy  was given to the family.
 
DMD is characterized by the progressive muscle
weakness. It is caused by the mutation in dystrophin
gene located on x chromosomes that’s 
affecting 1
in 3600 live male births. It is the main cause of the
progressive muscle weakness occurring clinical of the
patients. Symptoms include trouble in walking, loss of
reflexes, difficulty in standing up, poor posture, bone
thinning, scoliosis, mild mental impairment, breathing
difficulties, swallowing problems, lung and heart
weakness. In our patient there are no specific symptoms
of DMD. He presented only elevation of amino transferase
(ALT and AST) which are markers of hepatocellular
injury but are highly concentrated in muscle cells.
We aim to report this case, asneuromuscular
disorders may be suspected in the patient who identified
only with high amino transferase. 
Keywords: duchenne muscular distrophy,
aminotransferase, asymptomatic
[P-130]
Effect of Cultural Differences of Nursing Care
Hazal Hazal Huzmeli1, Ayşe Ferda Ocakci2
1
Amerikan Hospital
2
Koç University School of Nursing
Summary: Earth; 21th century with the name “Big
Village”. In this age we live in a different nation every
moment, we can encounter people from different race or
ethnicity. One of the best examples are in the hospital.
Historical and cultural journeys, especially in “health
tourism” because of the spread of different cultures in
our confrontation is inevitable. Turkey said “We can not
ignore the role of health tourism. The historical building,
both with people we meet with many ethnic origins in
domestic and abroad because of advanced hospitals. As
- 71 -
well as the last century wars with globalization in almost
all regions of the world, ethnic conflicts, environmental
crises, repressive regimes, economic collapse situations
like many of the people in the country or as refugees
migrants to other countries, is caused to migrate
voluntarily or compulsory. In this case the world in
different cultures and subcultures of individuals are
emerging multicultural population structure composed
of families and groups. Nursing disease is not concerned
with the effects on the patient’s disease it is the most
affected by health disciplines to cultural differences. It
will adversely affect the rights of patients ignore their
culture because they are all with their own culture.
Objectives: Nursing Care of “cultural differences” in
creating awareness.
Objective: Use in nursing care plans of cultural
differences and to improve the quality of care.
Methods: Review Article
Keywords: cultural differences, nursing care, nursing
effects of cultural diversity
[P-131]
A rare benign condition that should be
remembered in differential diagnosis
of hyperamylasemia in children:
Macroamylasemia
Melike Emiroğlu1, Halil Haldun Emiroğlu2, Hikmet
Akbulut3
1
Department of Pediatric Infectious Disease,Faculty of
Medicine,Selcuk University, Konya, Turkey
2
Department of Pediatric Gastroenterology, Hepatology
and Nutrition,Faculty of Medicine,Selcuk University,
Konya, Turkey
3
Department of Pediatric, Faculty of Medicine,Selcuk
University, Konya, Turkey
Aim: Macroamylasemia is a benign hyperamylasemic
condition
caused
by
circulating
macroamylase
complexes of pancreatic or salivary amylase bound
to immunoglobulins (IgG, IgA), which can be filtered
very slowly from the blood by the renal glomeruli.
Although macroamylasemia accounts for 2.5% of
hyperamylasemic conditions and has been associated
with a variety of diseases including celiac disease,
autoimmune disorders, rheumatoid arthritis, ulcerative
colitis and lymphoma. Also, 1% of healthy subjects have
macroamylasemia and it requires no treatment, may be
transient. In macroamylasemia, the amylase/creatinine
clearance ratio described below is usually <1% (normal
1-5%), and the urine amylase is usually low. Because of
it is a rare condition in children with hyperamylasemia,
we report a healthy boy with macroamylasemia.
Case: A 1.5 year old boy was admitted to the
hospital for nausea and vomiting associated with
hyperamylasemia and suspected acute pancreatitis. His
physical examination and blood biochemistry analysis
were normal except high level of serum amylase.
Ultrasonographic examinations for pancreatitis and/or
salivary glands diseases (parotitis, etc.) were normal.
Screening for viral infections including mumps IgM was
also normal. There was no member with high level of
serum amylase in his family. We confirmed elevated
serum amylase, normal serum lipase, and very low
amylase clearance/creatinine clearance ratio (0.3%),
consistent with hyperamylasemia.
Conclusion: If macroamylasemia is kept in mind as
an etiology of amylase elevation in the children without
elevation of serum lipase level, unnecessary further
diagnostic tests will be precluded.
Keywords: macroamylasemia, pediatric
[P-132]
Is MUAC cut-off level must be specific for
each country in diagnosis of malnutrition?
Sedat Özdemir1, Melike Emiroğlu2, Fatih Kara3, Mehmet
Sinan İyisoy4, Hikmet Akbulut1, Seral Navdar1, Halil
Haldun Emiroğlu5
1
Department of Pediatric, Faculty of Medicine,Selcuk
University, Konya, Turkey
2
Department of Pediatric Infectious Disease,Faculty of
Medicine,Selcuk University, Konya, Turkey
3
Department of Publıc Health, Faculty of
Medicine,Selcuk University, Konya, Turkey
4
Head of Medical Education and Informatics
Department,Meram Faculty of Medicine,Necmettin
Erbakan University, Konya, Turkey
5
Department of Pediatric Gastroenterology, Hepatology
and Nutrition,Faculty of Medicine,Selcuk University,
Konya, Turkey
Objective: We aimed to assess the usability of midupper arm circumference (MUAC) measurement in
malnutrition diagnosis of children aged between 1 and 5
years-old and, its relationship with other classifications.
Material-Method: 1500 children between 1-5 yearsold, who admitted to the Selcuk University Faculty of
Medicine, polyclinics of the Department of Pediatrics,
during June 01, 2014/September 30, 2014 period,
were included by simple random sampling method.
Anthropometric and MUAC measurements recorded and
malnutrition levels have been determined, according to
the Gomez, Waterlow and WHO classifications. MUAC
cut-off values in respect of malnutrition were calculated
for Gomez, Waterlow and WHO classifications by the
means of ROC analysis. The sensitivity, specificity, PPVs,
and NPVs of the MUAC were determined according to
either malnutrition classifications.
Results: MUAC had better compatibility with Gomez
classification in respect of sensitivity and specificity. In
the diagnosis of malnutrition, cut-off point of MUAC for
Gomez and Waterlow classifications is determined to be
14.95 cm; for WHO weight for age z score to be 13.95
cm; for WHO height for age z score to be 14.55 cm; and
for all classifications to be 15.15 cm. When we compared
the compatibility of MUAC with other malnutrition
classifications, cut-off point value as 11.5 cm produced
better results than 11.0 cm in respect of sensitivity,
specificity, PPVs, and NPVs in the diagnosis of severe
malnutrition.
Conclusion: For the diagnosis of severe malnutrition,
11.0 cm value is used in some countries as a MUAC cutoff point, but we think that preferring 11.5 cm value
- 72 -
is more suitable for our country. Although 12.5 cm is
routinely used as MUAC cut-off value for the diagnosis
of malnutrition, 15.15 cm cut-off value may increase the
sensitivity of this method.
Keywords: Malnutrition, mid-upper arm
circumference(MUAC), malnutrition classifications
[P-133]
Is there a relationship between nutritional
characteristics and malnutrition among the
age group of 1–5 years old children?
Seral Navdar1, Melike Emiroğlu2, Fatih Kara3, Gülay
Önal3, Hikmet Akbulut1, Halil Haldun Emiroğlu4
1
Department of Pediatric, Faculty of Medicine,Selcuk
University, Konya, Turkey
2
Department of Pediatric Infectious Disease,Faculty of
Medicine,Selcuk University, Konya, Turkey
3
Department of Publıc Health, Faculty of
Medicine,Selcuk University, Konya, Turkey
4
Department of Pediatric Gastroenterology, Hepatology
and Nutrition,Faculty of Medicine,Selcuk University,
Konya, Turkey
Objective: Malnutrition is an important health problem.
The aim of this study was to investigate the relationship
between nutritional characteristics and malnutrition
among the age group of 1-5 years old children in Konya,
a city in the Central Anatolia of Turkey.
Material-Methods: A total of 435 children between 1-5
year-old who admitted to Pediatrics Outpatients Clinic of
Selcuk University Faculty of Medicine between January
2013 and 2015 were included in the study. The children
divided into two groups according to Waterlow or Gomez
Classifications: (1) malnutrition group (215 children); (2)
control group (220 children). Both groups were evaluated
with 51 item questionnaire that include sociodemografic
and socioeconomic features of families, nutritional
characteristics and eating behaviors of children,
nutrition knowledge and child feeding style of mothers.
Results: The main cause of malnutrition is the error
in the practice during additional foods transition period
subsequent to breastfeeding. During the transition period
to supplementary feeding, there were more mistakes in
the patients with malnutrition than the control group.
Eating behavior abnormalities and inappropriate feeding
styles of mothers were apparently high in malnutrition
group.
Conclusion:
The
training
of
mothers
about
supplementary feeding may reduce the incidence of
malnutrition.
Keywords: Breastfeeding, malnutrition prevalence,
nutrition education
[P-134]
Cyst hydatid case presenting with thorax
deformity
Yaşar Şen1, Hikmet Akbulut2, Emine Ayça Cimbek1,
Yavuz Köksal3, Orhan Özbek4
1
Department of Pediatric Endocrinology,Faculty of
Medicine,Selcuk University, Konya, Turkey
2
Department of Pediatric, Faculty of Medicine,Selcuk
University, Konya, Turkey
3
Department of Pediatric Oncology, Faculty of
Medicine,Selcuk University, Konya, Turkey
4
Department of Radiology, Meram Medical Faculty,
Necmettin Erbakan University, Konya, Turkey
Introduction: Hydatid cyst, endemic in many regions,
is a parasitic disease caused by echinococcosis
granulosus.According to the affected organ, different
clinical symptoms are evident. It has a prevalence of
50-400/100 000 and an incidence of 3.4/100 000 in
our country. Diagnosis is based on the cyst determined
during radiological examination.In the present case,
a 6 year old girl referring to our clinic due to thorax
deformity with distinct hepatomegaly determined during
physical examination and diagnosed with hydatid cyst is
presented.
Case: A 6 year old girl referred to our outpatient clinic
due to thorax deformity which has been recognised for
4 years. On physical examination, her height and weight
percentiles were normal. In the abdominal region, 5-6 cm
below the ribs, a palpable mass with well circumscribed
borders adjacent to the liver was determined. Moreover,
the patient had also a chest wall deformity. Other
systems were considered to be normal. Abdominal
ultrasonography (USG) and magnetic resonance imaging
(MRI) of the patient revealed a 151X113 mm cystic
structure, membranous lesion (hydatid cyst), pushing
the kidney inferior without well circumscribed liver
parenchyma border with tiny (mm) moving echogenities
(daughter vesicles) elongating papillary. Complete blood
count, biochemical markers were normal. Accompanied
with USG and fluoroscopy, puncture-aspiration-injectionre-aspiration (PAIR) was applied to the patient.
Discussion: In the present case, the detailed physical
examination of the patient referred to our clinic due to
chest wall deformity revealed hepatomegaly due to cyst
hydatid as the main diagnosis. We think that severe
hepatomegaly and abdominal distansion led to patient’s
thorax deformity present since early childhood to be
more prominent and thus to the referral to our clinic.
Liver hydatid cyst diagnosis was made based on the
careful examination and diagnostic tests made. Hereby,
the importance of detailed patient history and physical
examination is to be highlighted once again.
Keywords: Thorax deformity, hydatid cyst, children
- 73 -
[P-135]
Mosaic 47 XYY syndrome presenting with tall
stature and macrogynecomastia
Emine Ayça Cimbek1, Sevil Arı Yuca1, Yaşar Şen1, Nadir
Koçak4, Hikmet Akbulut2, Fuat Buğrul1, İlhan Çiftçi3,
İlter Paydur2
1
Department of Pediatric Endocrinology,Faculty of
Medicine,Selcuk University, Konya, Turkey
2
Department of Pediatric, Faculty of Medicine,Selcuk
University, Konya, Turkey
3
Department of Pediatric Surgery, Faculty of
Medicine,Selcuk University, Konya, Turkey
4
Department of Medical Genetics, Faculty of
Medicine,Selcuk University, Konya, Turkey
47,XYY syndrome is a chromosomal abnormality
associated with an increased risk of learning disabilities,
delayed development of speech, language and motor
skills, tremors or motor tics, behavioral and emotional
difficulties. These characteristics vary widely among
affected individuals. Boys with this condition may be
taller than average, have severe acne during adolescence
but gynecomastia is not a very common finding. Here we
present a boy diagnosed as mosaic 47 XYY syndrome
associated with tall stature and macrogynecomastia.
A 13-year-old male patient visited our hospital for
assessment of his tall stature and breast development. He
was born with a birth weight of 4,100 g. His family history
revealed members with very tall stature. His height and
weight were both above the 97th percentile. On physical
examination, he was found to have severe acne on his
face, a Tanner stage of 4 and bilateral macrogynecomastia.
His routine biochemistry, thyroid function tests, insulinlike growth factor Ievel and other pituitary hormone
levels were all within normal ranges. His bone age was
consistent with his chronological age, and his predicted
adult height was approximately 200 cm. Sella magnetic
resonance imaging showed a microadenoma 8x6 mm in
diameter. He was diagnosed with XYY syndrome with a
chromosome test result showing a 47,XYY (%93)/46XY
(%7) mosaism and referred to the pediatric surgery
department for the assessment of macrogynecomastia.
When evaluating boys with learning disabilities and
behavioral problems a chromosome study should be
carried out if they have additional physical abnormalities
such as tall stature (although it may be familial) and
gynecomastia. In that case XYY syndrome should be
ruled out keeping in mind that they are very responsive
to early intervention and supportive treatment.
Keywords: children, 47XYY, gynecomastia
[P-136]
A case report: Miller Fisher variant of Guillan
Barre Syndrome and anti GQ1b antibody
positivity
Hilal Fevziye Durmaz Şimşek, Tamay Gürbüz, Çiğdem
Sağ, Umut Durak, Narin Akıcı, Çağatay Nuhoğlu
depertment of pediatrics, Haydarpasa Numune
Hospital, Istanbul, Turkey
Guillan- Barre Syndrome is an acute post-infectious
demyelinating polyneuropathy with an acute onset
characterised by genereally fast progressive muscle
weakness and paraesthesia. GBS is diagnosed by
clinical, laboratory and neurophysiological findings.(1)
Gbs has different clinical forms characterised by the
different involvement of motor and sensory axons of
peripheral nerves and the autonomic nervous system.
(2) Miller Fisher syndrome is a subtype of GBS which
characterized by external ophthalmoplegia, ataxia and
muscle weakness with areflexia. Incomplete forms
include acute opthalmoplegia without ataxia and
acute ataxic neuropathy without ophthalmoplegia.
Cerebrospinal fluid findings and electrophysiologic
features are similar to those in protype of GBS.
Anti-gangliosid anti bodies, mostly ıg g type, in patient
serum support the diagnosis.Ig G anti-GQ! B antibody is
one of the best studied antibodies whichare specifically
associated with variant of Fisher Syndrome. AntiGQ1b monoclonal antibody specifically immunostains
paranodal myelin of human cranial nerves innervating
extraocular muscles and some large neurons in dorsal
root ganglia. (3) Thus, the anti GQ1b antibodies may
cause ophthalmoplegia and ataxia by binding to the
regions where GQ1b is densely localized.
Keywords: guiilan barre syndrome, miller fisher
syndrome, anti gq1b antibody
[P-137]
Bone marrow suppression in an adolescent
with toxoplasmosis
Huray Kok, Hatice Eren, Dilsad Koca, Zehra Esra Onal,
Cagatay Nuhoglu
Haydarpasa Numune Education and Research Hospital
Introduction: Toxoplasma gondii is an intra cellular
protozoan parasite. Infection in humans occurs through
the ingestion of raw or low cooked meal that contains
cysts, or congenitally tranplacentally from an infected
mother during pregnancy.
Case: Seventeen years old female was admitted
to our policlinics with nausea and vomiting. In
her history, she had been using corticositeroid,
pyrimethamine and thyrimthmine-sulfamethoxazol for
the diagnosis of chorioretinitis due to toxoplasmosis.
Her physical examination revealed jaundice of skin,
she had no splenomegaly or hepatomegaly and
lymphadenopathy.
Neurologic
examination
was
normal. Laboratory findings showed anemia and
thrombocytopenia with Hb:5,9 gr/dl, Plt: 109.000/ml.
Peripheral blood smear showed hypochromic erythrocytes
with hypersegmented neutrophils, there was no atypical
cell. Reticulocyte, hemoglobin electrophoresis, B12
- 74 -
levels, PT, ANA (Anti-nuclear anticore), RF (Romatoid
factor), EBV IgM-IgG (Epstein Barr virus) levels were
within normal limits. Serum folic acid level was 1,5 ng/
ml, lower than normal. Oral folic acid replacement was
used. Bone marrow aspiration evaluation was evoked
bone marrow due to steroid treatment. It was considered
as secondary bicytopenia caused by toxoplasmosis.
Following up the patient, since anemia and
thrombocytopenia got worse, the patient was transfused
with erythrocyte and thrombocyte suspensions.
Corticosteroid
treatment
stopped.
Her
second
bone marrow aspiration was within normal limits.
Discussion: The etiology of pancytopenia or bicytopenia
has a wide distribution in childhood. In children viral
infections are the main causes of bone marrow suppression,
but in can be the result of malign disorders as well. It can
also be due to drugs, chemotherapy and radiotherapy.
Pancytopenia or bictopenia are the clinical manifestations
of toxoplasmosis. Bone marrow suppression is the main
complication of treatment with pyrimethamine. But
we demonstrated serological tests and bone marrow
aspiration for differential diagnosis of etiology.
Keywords: toxoplasmosis, thrombocytopenia,
pyrimethamine
[P-138]
Pericardial effusion in diarrhea positive
atypical hemolytic uremic syndrome
Hüseyin Bardak1, Aylin Gençler2, Merve Korkut1, Havva
Evrengül2, Dolunay Gürses3, Selçuk Yüksel2
1
Department of Paediatrics, Pamukkale University
School of Medicine Hospital at Denizli, Turkiye
2
Department of Paediatrics, Division of Paediatric
Nephrology, Pamukkale University School of Medicine
Hospital at Denizli, Turkiye
3
Department of Paediatrics, Division of Paediatic
Cardiology, Pamukkale University School of Medicine
Hospital at Denizli, Turkiye
Introduction: Besides several extra-renal manifestations
in diarrhea positive (D+) hemolytic uremic syndrome
(HUS), cardiac involvement is a rare but significant
complication. We present a severe pericardial effusion in
a child with D+ atipical HUS.
Case: A previously healthy thirteen years old girl
presented with four day history of bloody diarrhea. At
presentation, she was unwell, blood pressure was 120/80
mmHg, has no edema. The initial blood tests revealed
white blood cells (WBC) 12610/mm3, haemoglobin 11,9
gr/dL, platelet 63000/mm3, urea 49 mg/dL, creatinine
1,41 mg/dL. Urinalysis showed proteinuria and
microscobic heamaturia. Abdominal ultrasound revealed
both kidneys with normal size and echogenity, widespread free fluid and diffuse mucosal edema on bowels.
Within 24 hours, she developed anuria. Control lab
results showed anemia, trombocytopenia and haemolysis
(Heamoglobin 7gr/dL, LDH 2600 IU/L, haptoglobuine <10
mg/dL). Blood smear showed fragmanted erythrocytes.
She was determined as D+ HUS. O157:H7 returned
negative, ADAMTS-13 activity was 15% (40-130).
Within four days, she needed haemodialysis due
to rapid urea and creatinine rise and diuretic
resistant anuria. She was instituted Eculizumab
therapy due to ongoing anuria and worsening renal
functions despite haemodialysis. Urine output was
observed on second day of Eculizumab infusion.
She encountered hypotension and troponine-T rise (0,016
ng/mL) on day 28. Echocardiography revealed pericardial
effusion without tamponate and left ventricle ejection
fraction 70%. She had undergone a pericardiosyntesis
right after we had seen progression on effusion with
control echocardiograpies. 280 ml of pericardial fluid
was drained. She was then observed with antibiotics
and anti-inflmatory treatment. Pericardial effusion was
diminished on day 32 of Eculizumab treatment and she
was discharged with plans of Eculizumab protocol.
Conclusion: Cardiac manifestations in D+ HUS cases are
rare. These should be considered and promptly evaluated
with echocardiograpyh when patients encounter abrupt
chest pain while renal functions are recovering.
Keywords: pericardial, effusion, hus
[P-139]
A Rare Type Of Situs Inversus: Isolated
Levocardia
Ibrahim Mert Erbaş, Ozge Demirel, Helen Bornaun,
Rengin Şiraneci, Nail Uzunlulu, Özlem Öner
Kanuni Sultan Süleyman Eğitim ve Araştırma Hastanesi,
İstanbul, Turkey
Background&Aims: Situs inversus is a congenital
condition in which the major visceral organs (within the
thorax and abdomen) are either reversed or mirrored
from their normal positions. When the heart remains
on the normal left side of the thorax in the presence of
abdominal situs inversus, the condition is known as situs
inversus with isolated levocardia. The estimated reported
incidence is 1 per 22,000 in the general population and
from 0.4% to 1.2% in all patients with congenital heart
disease. Severe forms of congenital heart defects are
almost always associated with this condition. Splenic
defects such as asplenia and polysplenia are examples of
this anomaly. Polysplenia is characterized by a tendency
for bilateral left-sidedness, including bilateral two-lobed
lungs, interruption of the inferior vena cava (IVC) with
azygos continuation, and complex cardiac anomalies.
The only information regarding the outcome of patients
with this anomaly has been that of case reports on
adults and children in whom the diagnosis was made
incidentally or after abdominal surgery, particularly for
a volvulus or bowel obstruction. Moreover, according to
published data, the outcome is exiguous in children and
adults. In only approximately 5% to 13% do patients
survive for more than 5 years, mainly because of the
severity of an associate cardiac abnormality.
Keywords: Situs, Isolated, Levocardia
- 75 -
[P-140]
A Cause Of Treatment Resistant
Bronchopneumonia: H Type Tracheooesophageal Fistula
Irem Ceren Arslan, Gözde Akın, Emel Karaoğlan,
Hüseyin Aldemir, Ibrahim Mert Erbaş
Kanuni Sultan Süleyman Eğitim ve Araştırma Hastanesi,
İstanbul, Turkey
Background&Aims:
Oesophageal
atresia
and
tracheo‐oesophageal fistula (OA/TOF) are common
life‐threatening malformations with an incidence of
approximately 1 in 3500 births. The etiology of OA/TOF is
unknown in the majority of cases. In approximately half
of the cases (syndromic oesophageal atresia), there are
other associated anomalies, with cardiac malformations
being the most common.1 It is hard to diagnose H type
fistula in neonatal period, which is a subtype of TOF
without oesophageal atresia.2 These patients can live
until adult ages, without any diagnose. Most of them
have recurrent respiratory system infections, cyanosis
and apirations during feeding.3 In our case, we reported
a 45-days-old male patient that had treatment resistant
bronchopneumonia, and the further studies showed us H
type tracheo‐oesophageal fistula.
Keywords: Tracheo-oesophageal fistula, h type,
bronchopneumonia
[P-141]
Rasmussen encephalitis: hard diagnosis,
harder therapy
Judit Szamosújvári1, Beáta Rosdy1, Katalin Kollár1, Judit
Móser1, Mónika Mellár1, Éva Kovács2, Gabriella Kiss3,
Gábor Rudas4, György Várallyay4
1
Neurology Department of Heim Pál Children Hospital,
Budapest, Hungary
2
Department of Radiology, Heim Pál Children Hospital,
Budapest, Hungary
3
ICU, Heim Pál Children Hospital, Budapest, Hungary
4
Semmelweis Medical School MR Research Institute
Introduction: Rasmussen encephalitis (RE) is an
extremely rare autoimmune chronic inflammatory
neurodegenerative disease affecting a single cerebral
hemisphere,
causing
progressive
neurological
deterioration and intractable seizures.
Antiepileptic and immunmodulatory treatment can be
tried, but surgery (hemisphaerotomy), if applicable is
the treatment of choice.
Case: 9-year-old boy presented to our department with
fever provoked repetitive grand mal seizures in sleep.
Later in the disease course, his seizures progressed
to epilepsia partialis continua (EPC) with right sided
hemiparesis and speech difficulties. EPC did not respond
to antiepileptic treatment. MRI of the brain showed
on T2/FLAIR hyperintensive signals in cortical and
subcortical region of the left temporal lobe, suggesting
the diagnosis of RE. Later cortical atrophy developed
in this region. PET-CT presented focal hypometabolic
activity in that region, as well. Because eloquent cortex
was affected, surgery could not be a solution. We started
immunmodulatory therapy using steroid pulse therapy
with plasmapheresis. Because of septic complications we
switched to intravenous immunoglobulin (1,2 g/kg/dose)
monthly, combined with long-term oral steroids. Seizure
freedom was achieved and hemiparesis disappeared.
The patient is on these medications even up to date. We
taper steroids.
Conclusion: Immunmodulatory treatment combined
with antiepileptic drugs should be considered in therapy
of RE. Presurgical evaluation must be performed. If
surgery is applicable, the timing of it is a critical point
in the treatment decision. Post-surgical neurological
deficits are frequently equivalent to those inevitably
resulting from RE.
Keywords: Rasmussen encephalitis, diagnosis, therapy
[P-142]
A Life-Threatening Retropharyngeal Abscess
Adem Binnetoğlu1, Yavuz Gündoğdu1, Kıymet Keçelioğlu
Binnetoğlu2, Ali Cemal Yumuşakhuylu1, Tekin Bağlam1,
Murat Sarı1
1
Marmara University Pendik Treaning and Research
Hospital, Department of Otorhinolaryngology Head and
Neck Surgery, Istanbul-TURKEY
2
Marmara University Pendik Treaning and Research
Hospital, Department of Pediatrics, Istanbul-TURKEY
The incidence of Retropharyngeal abscess (RPA) is
increased from 0.1 cases per 10,000 to 0.22 cases per
10,000 in 2000 to 2009 [1] The reasons are thought
to be due to bacterial resistance as well as improving
investigative modalities (some of RPAs were misdiagnosed and treated with the antibiotics) [1]. Children
with RPA are usually caused by abscess formation within
inflamed local lymph nodes during or after the upper
respiratory tract infections[2]. RPAs are potentially
life-threatening conditions in both paediatric patients
and adults. They can result in local, regional and
systemic complications, as follows airway obstruction,
mediastinitis, jugular vein thrombophlebitis, cranial
nerve dysfunction, cervical osteomyelitis, meningitis,
sepsis, disseminated intravascular coagulopathy (DIC),
and death [3,4]. These complications have become a
less likely due to appropriate antibiotic treatment [3,4].
In our case, the patient have cervical lymphadenopathy
and swelling, odynophagia, stertor and difficulty in neck
movement. His breathing sound gets more noisy and
WBC count and CRP levels were not decreased even
under intravenous (IV) antibiotic treatment. Radiological
evaluation revealed RPA with 60x30 mm in diameter. We
performed transoral abscess drainage. Post-operative
follow-up showed dramatical clinical improvement.
RPA is an increasingly common infection that leads to
admissions to pediatric and emergency departments. RPA
is a potentially serious illness that should allert physicians.
They have to consider RPA in the differencial diagnosis with
other diseases that show the same clinical manifestations.
Topic: Retropharyngeal abscess
Keywords: Life-threatening conditions, Antibiotheraphy,
Transoral Drainage
- 76 -
[P-144]
Keywords: Life-threatening conditions,
Antibiotheraphy, Transoral Drainage
Comparism between transcutaneous
and serum bilirubin levels of neonates at
Delivery Room
[P-143]
A Rare Disease: Subtelomeric Deletion of
Long arm of 1st Chromosome in 4 Year Old
Patient
Koray Hacıoğlu1, Helen Bornaun2, Tuğçe Kalaycı Oral1,
Zeynep Ocak3, Kazım Özatrhan2, Fatma Ekici3
1
Department of Pediatrics, Kanuni Sultan Süleyman
Reseach and Training Hospital,İstanbul,Turkey
2
Department of Pediatric Cardiology, Kanuni
Sultan Süleyman Reseach and Training
Hospital,İstanbul,Turkey
3
Department of Medical Genetic and Birth Defects,
Kanuni Sultan Süleyman Reseach and Training
Hospital,İstanbul,Turkey
Background&Aims: Methods: Subtelomeric FISH
analysis
Results: Subtelomeric deletion was detected in long
arm of 1st chromosome
Conclusions: Distal subtelomeric region of long arm
of 1st chromosome is associated with characteristic
facial appearance and accompanying malformations.
Characteristic findings are: round facies, short neck,
turned-down the corners of the mouth, epicanthic
fold, long upper lip and a short philtrum, low set ears,
micrognathia, microcephaly, abnormal hand and feet
structure, various cardiac and genital anomalies, mild or
severe mental-motor retardation, hypotonia, seizures,
epilepsy, skeletal anomalies, psychomotor retardation
and growth retardation. Deletions in distal subtelomeric
region of long arm of the 1st chromosome are very rarely
seen and firstly reported by Mankinen and friends. When
clinical findings of our 3 years and 6 months old patient,
who was under examination due to hypotonia and cardiac
murmur, are evaluated, and pathology in all cardiac
valves found at echocardiography, karyotype analysis is
planned with suspicion of possible genetic disease. Patient
is found to have normal karyotype analysis, performed
for the differential diagnosis of genetic diseases.
Subtelomeric FISH analysis has been performed when
detailed dysmorphic examination revealed possible
subtelomeric region changes. Subtelomeric deletion was
detected in long arm of 1st chromosome. With his case,
we would like to emphasize the importance of adding
subtelomeric region investigations to karyotype analysis
in hypotonic infants, since the latter may be insufficient.
Keywords: FISH analysis, hypotonia, subtelomeric
deletion
Mürşide Uysal, Yelda Türkmenoğlu, Kyaw Zin Latt,
Ensar Duras, Doygoameneh Ghare Mashggharavi, Ozan
Özkaya
SB Okmeydanı Eğitim ve Araştırma Hastanesi,Çocuk
Sağlığı ve Hastalıkları Kliniği, Istanbul
Background&Aims: More than 60% of newborns
develop clinical jaundice in the first week of life and
pathologic levels of bilirubin may lead to bilirubin
encephalopathy. Because of its non-invasiv procedure,
trancutaneous
bilirubin
measurement
becomes
more preferrable than serum bilirubin, but there are
discussions about reliability. The aim of this study is to
evaluate the compactibility between transcutaneous and
serum bilirubin levels of neonates at delivery room of
our hospital.
Methods: The study was done at Pediatrics Department
of Okmeydanı Research and Training Hospital.
Transcutaneous bilirubin levels were measured from
forehead and sternum of neonates of ages between 0-7
days, healthy, late preterm and term(above 35GW) by
Bili Check metre. The results were compared with venous
bilirubin levels. The same procedure was performed to
neonates who lost weight more than 5% of their birth
weight.
Results: Out of 38 neonates, 20 were male(52,6%)
and the birth weights vary from 2265 to 4270 grams
(median 3252.11±505.75 gram).Serum bilirubin levels
range between 6,7- 15,1 mg/dl. The study found
out statistically and significantly positive corelation
between serum bilirubin and transcutaneous sternum
bilirubin levels 78,4%, between serum bilirubin and
transcutaneous forehead bilirubin levels 73,4%,
and between transcutaneous sternum bilirubin and
transcutaneous forehead bilirubin levels 75,7%
(p:0.001; p<0.01). There was no statistics and
significant correlarions between serum bilirubin and
transcutaneous bilirubin levels (sternum and forehead)
bilirubin levels of the neonates who lost weight more
and less than 5% of their birth weight (p>0.05).Three
of neonates who had high bilirubin levels according to
bilirubin nanograms were carried out phototherapy.
Conclusions: Transcutaneous bilirubin measurement is a
cheap, non invasive and safe method to detect the bilirubin
levels of neonates before disharge from the hospital. But
in the cases with significant hyperbilirubinemia, serum
bilirubin level is more reliable for treatment protocols.
Keywords: neonate, transcutaneous bilirubin
- 77 -
[P-145]
A rare causes of cholestasis: Arthrogryposisrenal tubular dysfunction-cholestasis
syndrome
Yelda Türkmenoğlu1, Yeşim Acar1, Fatiih Cemal
Özdemir1, Kyaw Zin Latte1, Ralfi Singer2, Erdal Adal1
1
Department of Pediatrics,Ministry of Health Okmeydanı
Research and Training Hospital, Istanbul
2
Department of Dermatology,Ministry of Health
Okmeydanı Research and Training Hospital, Istanbul
Arthrogryposis- renal tubular dysfunction and cholestasis
(ARC ) syndrome, the association of arthrogryposis, renal
tubular dysfunction and cholestasis is a rare autosomal
recessive multisystem disorder. Additional features are
severe failure to thrive, ichthyosis, abnormal platelets,
diarrhoea, recurrent infections, mild dysmorphic signs,
nephrogenic diabetes insipidus, hypotonia, dysgenesis
of corpus callosum and nerve deafness.Gissen et al.
identified a mutation in VPS33B gene on chromosome
15q26.1. Most of patients die because of pneumonia,
septicemia, acidosis, bleeding or dehydratation in the
first year. Here we report a case of ARC syndrome from
Turkey diagnosed by genetic analysis instead of invasive
biopsies, but she died three years old age because of
septicemia.
Keywords: cholestasis, ichthyosis, renal tubular
dysfunction
[P-146]
Mediastinal tumor; as case report
Mahir Tıras, Ozlem Bostan Gayret, Ozgül Yiğit, Meltem
Erol, Leyla Besel
Bagcilar Treaning and Research Hospital, Pediatric
Department, Istanbul, Turkey
Background&Aims: Mediastinal masses in children
constitute a heterogeneous group of malignant and
benign neoplasms. Neurogenic tumors are reported
to be the second most common pediatric mediastinal
malignancy seen in 21%- 44% of cases. Neuroblastoma
is most common and occurs in children two years of age
or younger. In this report we present a case who applied
with abdominal pain and diagnosed neuroblastoma with
biopsy.
Case: A five years old girl, whose personal and family
medical history revealed no significant findings, admitted
to pediatric emergency clinic with abdominal pain. Her
heart rate, blood pressure and body temparature were
normal. In his physical examination, breathing sounds
were not heart in right hemithoraces. İn her chest
radiography, opacity was seen completely in right lung.
Her thorax tomography was compatible with mediastinal
mass. According to her mediastinal biopsy result, she was
diagnosed neuroblastoma. In her laboratory findings;
Hb:10.57 gr/dl, WBC: 22000/mm3, PLT: 317000/mm3,
LDH: 2786 U/L, uric acid: 2.6 mg/dl, CRP: 123 mg/L.
neuron-specific enolase level was 309 ngr/ml. Other
blood analysis were normal. She referred pediatric
oncology clinic.
neuroblastoma in differential diagnosis of pediatric
mediastinal mass.
Keywords: Mediastinal masses, neuroblastoma,
childhood
[P-147]
Presentation of Atypical Hemolytic Uremic
Syndrome
Maltam Baghirova, Zehra Esra Önal, Tamay Gürbüz,
Mustafa Behçet Şimşek, Çağatay Nuhoğlu
Department of Pediatrics, Haydarpasa Numune EAH,
Istanbul, Turkey
Background&Aims: Microangiopatic hemolytic anemia,
thrombocytopenia, fever, central nervous system
abnormalities and acute renal failure are five main
components of the Hemolytic Uremic Syndrome (HUS).
Atypical HUS is related to genetic mutations and may
occur without a gastrointestinal prodrome. Atypical HUS
generally leads to poor outcomes and may advance to
end-stage renal disease or permanent brain damage in
approximately 50% of the patients.
Methods: A four-year-old girl was admitted to our
hospital with complaints of abdominal pain and high
grade fever. She was given antibiotic medication. Edema
and petechial rashes occurred on her skin over time. Due
to these symptoms, we first evaluated the disease as
meningococcemia. However, her blood count showing
values as “HGB: 12.1, PLT: 70.000/uL, APTT: 19.4, PT:
1.61 and INR: 1.68”, the appearance of blood in the
urine (macroscopic hematuria) and absence of diarrhea
led us to give presumptive diagnosis as atypical HUS.
A further more detailed blood test resulted in following
Findings: microangiopatic hemolytic anemia with a
negative Coombs’ test and thrombocytopenia (<40000/
uL). Afterwards, plasmapheresis, eculizumab, freshfrozen plasma was administered at a dose of 10 ml per
kilogram on day in intensive care unit.
Results: She had normal level of blood pressure and
was fully conscious. Despite the therapy, her edema and
thrombocytopenia did not resolve.
Conclusions: This case underlines the limited
understanding of clinical features of atypical HUS. Since
it is a rarely seen disease, the diagnosis is difficult.
Even if the right diagnosis is given, genetic testing is
of great importance in deciding treatment options, but
these tests are not widely available. This case highlights
the need for high level of suspicion and more in-depth
research towards better treatment alternatives keeping
in mind the mortality it may cause.
Keywords: Hemolytic Uremic Syndrome, atypical HUS
Conclusions: This case presented to emphasize the
- 78 -
[P-148]
A case of empyema necessitatis in a child
with M. tuberculosis
Manolya Acar1, Murat Sutcu1, Ezgi Topyildiz1, Tansu
Salman2, Hacer Akturk1, Gonca Erkose Genc3, Bulent
Oguz Erol4, Nuran Salman1, Ayper Somer1
1
Istanbul University Istanbul Medical Faculty, Pediatric
Infectious Diseases
2
Istanbul University Istanbul Medical Faculty, Pediatric
Surgery
3
Istanbul University Istanbul Medical Faculty,
Microbiology and Clinical Microbiology
4
Istanbul University Istanbul Medical Faculty, Radiology
Introduction: Empyema may extravasate outside the
thorax cavity under pressure. This is known as empyema
necessitatis (EN). It rarely occurs in children. Herein we
report a child with EN due to pulmonary tuberculosis
(TB).
Case report: An 11-year-old boy presented with
recently occurrence of swelling on his chest wall. He
had been hospitalized for plevral empyema a year ago
and his father had been diagnosed with cavitary TB
at that time. Tube thoracentesis had been performed,
and acid resistant stain for mycobacterium reported as
negative from exudative material. Interferon gamma
releasing test (IP-10) and tuberculin skin test had
been positive. Anti tuberculous treatment (isoniazide,
rifampisin, pyrazinamide, ethambutol) was initiated
and he was discharged with recovery. After that he
did not come to regular follow-up. Retrospectively we
found, he had refused to take his medication and plevral
fluid culture had come positive for M. tuberculosis.
On admission, systemic examination was unremarkable
other than a nodular area on his chest. X- ray showed
small infiltration in left lung parenchyma without
effusion. Thorax ultrasound and CT revealed 5cm
diameter of intense material collection on thorax wall
communication with pleural cavity. Acid resistant stain
and PCR for M. tuberculosis were reported as positive.
He was restarted on anti tubercuosis treatment and the
abscess disappeared with time.
Discussion: Pediatric EN is very uncommon. Since chest
x-ray examinations are not always useful, high suspicion
and further evaluation may be necessary for diagnosis.
Keywords: Child, empyema necessitatis, M
tuberculosis
[P-149]
Wilms tumor with Neurofibromatosis; A rare
case report
Melih Er1, Elvan Çağlar Çıtak1, Erdem Ak1, Simge Kaya2
1
Mersin University Medicine Faculty, Department of
Pediatrics, Mersin, Turkey
2
Mersin Maternity and Child Health Hospital Department
of Pediatrics, Mersin, Turkey
Introduction And AİM: Wilms’tumoristhemostcommonprimaryrenaltumorinchildhood.80%ofthecasesareseenbetween15yearsofage.
Somecongenitalanomaliesandotherdiseasesseen-
moreofteninpatientswithWilmstumor:Neurofibromatosis1,BeckwithWidemannsyndrome,DanysDrashsyndrome,Perlmansyndrome,WAGRsyndromeetc.
TheriskofmalignancyisincreasedinpatientswithNF1thaninthegeneralpopulation.Themalignantperipheralnevresheathtumorsaremostcommon.
Meningioma,ependymoma,Wilms’tumor,rhabdomyosarcoma,neuroblastoma,medullary thyroid carcinoma
and melanoma can also seen in these patients.We are
present a case of Wilms tumor with a rare involvemet
of the posterior bladder in our patient diagnosed with
neurofibromatosis
Case Report: There was weakness and swelling in the
abdomen, especially the right side for 20 days in a patients
with neurofibromatosis. The abdominal ultrasound was
reported as it was observed a heterogeneous, hypoecoic
and hypervascular mass lesion that 12.5x10 cm in size,
including cystic degeneration in the middle and have
solid component. The multifocal lesions thought to cause
diffuse infiltration behind the right kidney and bladder
and solid nodular malignant mesenchymal tumors like
metastasis in the lung parenchyma has been identified
in thoracic and abdominal computer-tomography (CT)
scans. The findings obtained by biopsy samples from
different regions were reported as being primarily
compatible with Wilms’ tumor.The mass was trying to
removed as possible as by laparotomy performed by
pediatric surgeons, but it could not be removed because
there is a total invasion,biopsy was taken and processing
was ended. It was determined that the lesion defined
behind right kidney and bladder was shrank comparing
with older tests after the chemotherapy.
Discussion: Ito F. et al. Reported that a 2-year-old male
with neurofibromatosis who had a Wilms tumor of the right
kidney and an ipsilateral adrenal ganglioneuroblastoma
in 1997. Shvartsbeyn et al. reported a case report in
2011. The authors report the first case of a Wilms tumor
(WT) with diffuse anaplasia metastatic to the brain in
a 13-year-old girl with a history of neurofibromatosis
Type 1.As can be understood from these studies in the
literature, Neurofibromatosis can accompany each other
with Wilms tumor very infrequently.
Keywords: Neurofibromatosis, Wilms
[P-150]
Brucellosis case presented with arthritis on
elbow
Melike Irem Petan, Hatice Nilgun Selçuk Duru, Funda
Kökali
Haseki Eğitim ve Araştırma Hastanesi Çocuk Sağlığı ve
Hastalıkları Kliniği, İstanbul
Introduction: Brucellosis is an infectious disease can
spread by contact with infected animals, consuming fresh
milk and its products, inhalation of infected droplets.
Disease can occur as acute onset fever, splenomegaly,
night sweat, joint pain and also some variety of diseases
characterised by atypical signs and symptoms that mimic
insidious rheumatic or psychosocial tables.Locomotor
system complication in children is mostly seen as septic
and reactive arthritis.Especially knee and ankle joints are
- 79 -
involved.Sacroileitis is rare in children.Here we submit
a brucellosis case presented with swelling, redness and
movement restriction on elbow and initially thought to
be a rheumatologic disease or septic arthritis, to draw
attention to different involvement of the disease.
Case: 15 years old boy was admitted to emergency
department with redness, swelling and movement
restriction on left elbow.Detailed history showed that
he had complaint of migratory arthralgia for 2 months
and his family also had same complaints.They had
history of eating cheese fermented underground for 6
months in Bitlis.On physical examination there was no
other signs.Laboratory and screening tests results were,
crp 111 mg/l(0-3), esr 71 mm/hour, wbc 6,2(10e9/
ul), ana positive, brucella IgM positive(>23,6), brucella
IgG positive(>17.5), wright test positive in 1/1280
titre,liver size 170 mm(>95p), spleen size 155 mm
(>95p).Effusion in elbow joint detected in direct x-ray
and mrı and joint puncture was done by consulting
orthopedics.Puncture sample was PNL dominant.Patient
was diagnosed brucellosis and started doxocyxlin,
rifampicine,
streptomycin
treatment.Streptomycin
treatment completed to 14 days.Doxocyxline, rifampicine
treatment completed to 6 weeks.
Result: Arthritis is seen %10-40 in brucellosis
osteoarthricular involvement and especially knee, hip
and ankle joint involved.Our country is an endemic
region of brucellosis.It is not just animal husbandary
sector and also those who consume products derived
from the animals are at risk.Therefore arthritis etiology
and differential diagnosis of brucellosis should be
considered.
Keywords: brucellosis, arthritis, elbow
[P-151]
Bronchiolitis Obliterans In A Patient Require
Frequent Nebulizing Treatment and Early
Steroid Treatment
Melike Irem Petan, Hatice Nilgun Selçuk Duru, Funda
Kökali
Haseki Research and Training Hospital Pediatrics
Department, Istanbul, Turkey
Introduction: Bronchiolitis obliterans is characterized by
persistant symptoms of obstructive lung disease after an
acute bronchiolar injury. It is a rare disease in childhood
occurring as a complication of mostly viral(adenoviral)
lower respiratory tract infections.Most of misdiagnosed
patients require intensive care and mechanical
ventilation.Here we submit a bronciolitis obliterans case
to draw attention to importance of differential diagnosis
in patients who have recurrent bronchiolitis symptoms
and starting early treatment that affects prognosis of
the disease, number of hospitalization and requirement
of picu.
Case: 5 years old girl was admitted to emergency
department with respiratory distress and tachypnea.
She had history of such episodes and hospitalizations
for several times.On physical examination, there were
diffuse expirium elongation and sibilant rales but no other
findings.Laboratory tests were in normal ranges and no
significant signs in chest x-ray.On detailed anamnesis
she had an adenoviral respiratory infection.Thorax bt
imaging was done to patient who had no benefit from
inhalation treatment.Thorax bt showed mosaic pattern
and vascular attenuation, so the patient thought to be
bronchiolitis obliterans and systemic steroid treatment
started.After treatment respiratory signs became normal
and inhalation treatment requirement was decreased.
Patient was thought to be bronchiolitis obliterans
because of no benefit of 10 days lasting nebulizing
treatment and excluding of other differential diagnosis
such as tuberculosis, cystic fibrosis, infectious and
rheumatological diseases. Prophylactic steroid treatment
and vaccination for flu after externation, decrease
nebulizing treatment requirement.
Result: Bronchiolitis obliterans should be considered
in patient who has recurrent bronchiliotis symptoms,
hospitalizations and early steroid treatment should
be started.The use of clinical practice guidelines can
standardize care, reduce admissions, manage resources
better, and shorten the length of hospital stays without
increasing readmissions rate or decreasing family
satisfaction.
Keywords: bronchiolitis, bronchiolitis obliterans,
wheezing
[P-152]
Delirium due to cyclopentolate eye drop in a
child
Sinan Oğuz1, Melike Ocak2, Nilden Tuygun1, Emine
Polat2, Can Demir Karacan1
1
Ankara Dr. Sami Ulus Maternity, Children Health and
Diseases Training and Research Hospital, Pediatric
Emergency Department
2
Ankara Dr. Sami Ulus Maternity, Children Health and
Diseases Training and Research Hospital, Department of
Pediatrics
Background: Cyclopentolate is a muscarinic receptor
antagonist that is widely used topically in ophthalmology
for preoperative evaluation and clinical diagnosis because
of its mydriatic and cycloplegic activity. When systemic
drug absorption occurs central nervous system symptoms
may be seen. Here, a four-year-old girl with hallucinations
and experienced difficulty in walking after medication
of eye drop containing cyclopentolate was presented.
Case Report: A four-year-old girl presented with inability
to walk, unstable gait, hallucinations and feeling of fear
after fifteen minutes of instillation of cyclopentolate
for diagnostic eye examination. Physical examination
revealed ataxia and incoherent speech and fix dilated
pupils. The rest of the examination was unremarkable.
Based on these findings and history of cyclopentolate eye
drop usage, cyclopentolate toxicity was diagnosed. Her
symptoms gradually resolved over the next eight hours. She
was discharged uneventfully after 24 hours of follow-up.
Discussion and Conclusion: Physicians should be aware
of the uncommon systemic side effects of cyclopentolate
that is widely used for ophthalmological examinations.
Treatment is essentially symptomatic, and nasolacrimal
duct occlusion for 3-4 minutes following instillation of
- 80 -
the drug may reduce its absorption and systemic side
effects.
Keywords: Delirium, Cyclopentolate, Pediatric
Emergency
[P-153]
Intussusception Presenting with Shock
Sinan Oğuz1, Şakire Başer2, Burak Ardıçlı3, Nilden
Tuygun1, Melike Ocak2, Can Demir Karacan1
1
Ankara Dr. Sami Ulus Maternity, Children Health and
Diseases Training and Research Hospital, Pediatric
Emergency Department
2
Ankara Dr. Sami Ulus Maternity, Children Health and
Diseases Training and Research Hospital, Department of
Pediatrics
3
Ankara Dr. Sami Ulus Maternity, Children Health and
Diseases Training and Research Hospital, Pediatric
Surgery
Background: Shock is defined a dynamic and unstable
pathophysiologic state characterized by inadequate
tissue perfusion. Hypovolemic shock resulting from
gastroenteritis is the most common type of pediatric
shock. Early diagnosis and determining cause of
shock improves the success of treatment. Here an
intussusception case admitted to our pediatric emergency
department (PED) with shock state was presented.
Case Report: Two-years-old girl was admitted with
complaints of vomiting and weakness. Vomiting was
repeated many times during the day. After two days of
diarrhea she had no defecation for the last 24 hours.
She was lethargic with body temperature of 37.6 °
C, pulse 220/min, arterial blood pressure of 80/50
and oxygen saturation of 98%. She was diagnosed
and treated as compensated shock. Because of
abdominal tenderness and distention she underwent
ultrasonography. In the lower right quadrant ileoileal
intussusceptions was determined. Surgery reduction
was performed and she was discharged on the 5th day.
Discussion: Intussusception is an invagination of a part
of the intestine into itself. It is the most common cause
of intestinal obstruction in infancy. The classic triad of
pain, a palpable sausage-shaped abdominal mass, and
bloody or currant jelly stool is seen in under one third of
patients with intussusceptions. If the intussusception is
not reduced, the patient becomes progressively weaker
and lethargic. Finally, a shock-like state, with fever
and peritonitis, may develop. Reduction of an acute
intussusception is an emergency procedure and should
be performed immediately after diagnosis.
Conclusion: Children with intussusception can be
present with classic symptoms but also may be present as
an acutely ill child with severe abdominal pain, lethargy,
dehydration, and shock. Intussusception should be kept
in mind in patients presenting with these symptoms or
signs.
Keywords: Intussusception, Shock, Pediatric
Emergency
[P-154]
Anticholinergic poisoning due to Mangragora
Officinarum in a eight years old boy
Sinan Oğuz1, Nilden Tuygun1, Fatma Burçin Kurtipek2,
Selman Kesici3, Melike Ocak2, Can Demir Karacan1
1
Ankara Dr. Sami Ulus Maternity, Children Health and
Diseases Training and Research Hospital, Pediatric
Emergency Department
2
Ankara Dr. Sami Ulus Maternity, Children Health and
Diseases Training and Research Hospital, Department of
Pediatrics
3
Ankara Dr. Sami Ulus Maternity, Children Health and
Diseases Training and Research Hospital, Pediatric
Intensive Care Unit
Background: Poisoning is a significant fraction of
pediatric emergency admissions. The cause of poisoning
may not be determined in all cases. Here we present
a boy who admitted to our pediatric emergency
department (PED) with disorientation, bizarre behavior,
visual hallucinations, dilated pupils and blurry vision
after unknown plant ingestion.
Case Report: Eight years old boy was admitted to
PED with mumbling speech, dryness of mouth, blurred
vision, bizarre behavior and visual hallucinations
after ingestion of an unknown plant root. On physical
examination; he had normal vital sings with dilated
pupils, disorientation and unbalanced walking. Routine
laboratory tests and EKG were normal. The patient was
considered as anticholinergic poisoning. The plant had
a thick, fleshy, forked root and resembled a human
figure that is thought to Mangragora Officinarum and
had anticholinergic effects. He was hospitalized and
had supportive treatment. On follow-up bladder globe
developed so urethral catheter inserted. His signs
and symptoms improved after two days and he was
discharged.
Discussion: Ingestion of some plants can produce
anticholinergic toxicity because they contain significant
concentrations of belladonna alkaloids (hyoscyamine,
hyoscine, atropine and scopolamine). Clinical findings
of anticholinergic plant ingestion are similar to classic
anticholinergic (atropine-like) toxidrome. Flushed, hot,
dry skin, dilated pupils and blurry vision, disorientation,
bizarre behavior, paranoia, delirium, visual hallucination,
seizure, tachycardia, decreased bowel sounds and
urinary retention can be seen in anticholinergic toxicity.
Conclusion: Children are at risk being exposed to
potentially toxic plants, both in the home and outdoors.
The majority of these exposures do not lead to clinically
important poisoning. However, anticholinergic poisoning
should be kept in mind in patients presenting with plant
ingestions.
Keywords: Anticholinergic poisoning, Mangragora
Officinarum, Pediatric Emergency
- 81 -
[P-155]
An Infant Boy with Big Head
Merve Erdemir Kula1, Elif Begüm Kılıç1, Türkan Uygur
Şahin1, Emel Torun1, Akın Işcan1, Gözde Yeşil2
1
Department of Paediatrics, Faculty of Medicine,
Bezmialem Vakıf University, Istanbul, Turkey
2
Department of Medical Genetics, Faculty of Medicine,
Bezmialem Vakıf University, Istanbul, Turkey
Introduction: Macrocephaly may be the symptom of
an underlying congenital, genetic or acquired problem in
children. Here we present a ten month old patient who was
diagnosed with Vanishing White Matter Disease (VWMD),
a hereditary cause of megaloencephalic macrocephaly.
Case: Ten months old infant boy was admitted to our
outpatient clinic with large head size. His height was
74 cm (50 p), his weight was 9,3 kg (50 p) and his
occipitofrontal circumference(OFC) was 51 cm ( > 98
p) which was increased by five cm at last two months,
anterior fontanelle was 2 x 2 cm. The patient was
delivered by caesarean section with a 35.5 cm (50 p)
of head circumference, after a full-term pregnancy.
Prenatal history and neurological development of the
boy were normal. The baby was able to hold her head up
at two months and sit without support at seven months
old. He couldn’t walk yet. He was the second child of
unrelated parents whom their OFCs were normal.
To eveluate the etiology of our patient’s macrocephaly,
blood and urine samples were taken and analysed. There
were no abnormalities on his neurometabolic tests. His
electroencephalogram was normal. Cranial MRI, that was
consistent with VWMD, showed the local encephalopathy
which extending from periventricular grey matter to
subcortical white matter. The diagnose comfirmed
with revealing of MLC -1 mutation by genetic analysis.
Discussion: As macrocephaly could emerge hereditary,
it also could be the consequence of obstructive/
non-obstructive hydrocephalus, anatomic/metabolic
megaloencephalopaty or thickening of skull bones
(hyperphosphatemia,
osteopetrosis,
osteogenesis
imperfecta, rickets, dysostosis). One of the most
prevalent hereditary white matter diseases, VWMD also
known as childhood ataxia with central hypomyelination
(CACH). To diagnose VWMD, the mutation of MLC gene
should be revealed on the 22th cromosome.
Keywords: macrocephaly, infant, white matter
[P-156]
A rare cause of hypoglycemia in infancy:
growth hormone deficiency
Merve Hazal Yılmaz1, Tanyel Zübarioğlu1, Ertuğrul
Kıykım1, Aydilek Çakır2, Çiğdem Aktuğlu Zeybek1
1
Istanbul University Cerrahpaşa Medical Faculty
Department of Pediatrics Division of Nutrition and
Metabolism, Istanbul, Turkey
2
Istanbul University Cerrahpaşa Medical Faculty
Department of Pediatrics Division of Endocrinology,
Istanbul, Turket
Growth hormone deficiency is an autosomal recessive
inherited disorder characterized by growth retardation.
In the early infancy period the only symptom of growth
hormone deficiency can be refractory hypoglycemia.
It is important to consider growth hormone
deficiency in differential diagnosis of hypoglycemia
since untreated cases can be life threatening
Here a 4-month old female patient who is presented
with hypoglycemia and diagnosed as growth hormone
deficiency is reported.
4 month-old female patient was referred to our outpatient clinic because of refractory hypoglycemia
with a suspicion of fructose intolerance. Initial
metabolic investigations including plasma lactate,
ammonia, uric acid levels, liver and renal functions,
muscle enzymes were found normal. As the
laboratory findings of the patient did not support the
metabolic disorders and complaint of patient persisted
despite the fructose-free diet, initial diagnosis was
excluded. In the further investigations, tests for disorders
of carbohydrate, aminoacid, fatty acid metabolisms and
hyperinsulinemia results were normal. For the differential
diagnosis of endocrinologic disorders hormon profile
examinations were performed. As the basal growth
hormone levels were found to be low during hypoglycemia
attack; ACTH loading test and L-DOPA loading tests
were made. As sufficient growth hormone increase was
not observed, diagnosis of growth hormone deficiency
was made. After initiation of As growth hormone
replacement therapy, hypoglycemia attacks ceased.
Children with growth hormone deficiency may be short
at birth, but their lengths are usually within normal
limits especially during infancy. In the absence of growth
retardation refractory hypoglycemia must be an alerting
sign for the phisician. Growth hormone levels should
be assessed in any baby with severe or symptomatic
hypoglycaemia in whom another cause is not obvious.
Keywords: growth hormone, hypoglycemia, infant
[P-157]
A rare disorder seen in patients diagnosed
with cholestasis: Primary bile acid synthesis
disorders
Merve Nur Hepokur1, Banu Bal Çermik2, Selim Gökçe2
1
Bezmialem University Hospital, Clinics of Pediatrics,
İstanbul
2
Bezmialem University Hospital, Clinics of Pediatric
Gastroentelogy, İstanbul
Primary bile acid synthesis disorders (BASDs) are a group
of rare metabolic disorders characterized by defects in the
synthesis of bile acids. Overall prevalence is estimated
to be around 1-9/1.000.000 for overall BASDs. 3-betahydroxy-delta-5-c27-steroid dehydrogenase deficiency
is believed to be the most common form of BASDs. Its
deficiency is caused by mutations of the HSD3B7 gene
on short arm of chromosome 16 (16p11.2). Clinically;
cholestasis, hepatocellular damage, malabsorption,
lack of fat-soluble vitamins and cirrhosis can be seen.
It can be distinguished from other forms of cholestatic
liver diseases in childhood by normal GGT and serum
bile acids level and absence of itching. If untreated,
progressive liver disease occurs. Diagnosis is based on
urine and serum bile acid analysis. Herein, we presented
a 19 month-old choletatic male infant without itching.
- 82 -
He had normal GGT and serum bile acids. Primary bile
acid synthesis disorders was eventually diagnosed by
demonstration of atypical bile acids in urine.
Keywords: cholestasis, bile acids
[P-158]
Hipotalamic-Pituitary-Adrenal axis
supression in children with asthma
Merve Şakar1, Şebnem Özdoğan2, Ahmet Uçar3,
Ayşenur Kaya4, Sermin Özcan1
1
Department of Pediatrics, Sisli Hamidiye Etfal Search
and Training Hospital, Istanbul, Turkey
2
Department of Pediatric Pulmonolgy, Sisli Hamidiye
Etfal Search and Training Hospital, Istanbul, Turkey
3
Department of Pediatric Endocrinology, Sisli Hamidiye
Etfal Search and Training Hospital, Istanbul, Turkey
4
Department of Pediatric Allegy and Immunology, Sisli
Hamidiye Etfal Search and Training Hospital, Istanbul,
Turkey
Background&Aims:
Hypothalamic-pituitary-adrenal
axis (HPAS) suppression is among the most important
potential adverse effect of ICS. In this study we aim to
assess the prevalence and predictive factors for HPAS in
children with persistent asthma who has been on daily
ICS.
Methods: Clinical features of HPAS, steroid dose, and
symptom control were documented. Adrenal suppression
was measured by morning cortisol levels and confirmed
by low-dose adrenocorticotropic hormone stimulating
test.
Results: A total of 80 children aged 6-18 years old
participated. Mean age was 10.24±2.61 years. Two
children had HPA axis suppression. Both children with
HPA axis suppression were on medium dose ICS. None of
the children had clinical features of HPA axis suppression.
Conclusions: The clinical significance of effects of ICS
on adrenal function still remains to be established.
Assessment in large number of patients is ongoing to
determine the prevalence and predictive factors of HPAS
in children with asthma.
Keywords: adrenal supression, asthma, pediatric
[P-159]
A Patient with systemic involvement: Is
tuberculosis the mere cause?
Mine Yüksel1, Ahmet Hakan Gedik2, Erkan Çakır2,
Nurhan Aruçi Kasap1, Fatma Özgüç1
1
Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Bezmialem
Vakıf Üniversitesi, İstanbul, Türkiye
2
Çocuk Göğüs Hastalıkları Bilim Dalı, Bezmialem Vakıf
Üniversitesi, İstanbul, Türkiye
Background: Childhood tuberculosis (TB) can present
in various forms. A rare case of TB with systemic
involvements with no detected underlying condition is
being presented here.
antituberculosis treatment due to mediastinal 1.5 cm
lymphadenopathies seen in computed tomography (CT)
of the lungs and treatment resistant infiltration in the
upper right side seen in pulmonary x-ray when she
had presented with cough and sweating 7 years ago
(no TB contact, PPD:0 mm, microbiology negative).
Hematologic and rheumatologic examinations performed
for concomitant leucopenia yielded normal results.
Treatment lead to reduction in the lesion and the
treatment was discontinued at month 4 by the family.
With persisting leucopenia one year later, the lesion
returned to its previous sizes, as seen with the pulmonary
x-ray taken for night sweatings. With sedimentation
65 mm/h, PPD 15 mm and leucopenia (WBC:4100),
the patient was referred to our clinic. Bronchoscopy
demonstrated narrowing of apical segment at the upper
right segment and disturbed mucosal structure which was
accompanied by tracheal bronchus. With transthoracic
biopsy showing intraalveolar fibrosis and chronic
inflammation, TB organizing pneumonia was considered
and anti-tuberculosis and steroid treatment was initiated.
Her lesion remitted completely with treatment but she
had intermitted leucopenia episodes. 3 years later, she
had cough for which pulmonary x-ray was taken and
showed recurrence of the lesion in the same region. With
pulmonary CT, there was a mass blocking the tracheal
bronchus. Biopsy taken with flexible bronchoscopy
showed Mycobacterium tuberculosis complex and
the patient was started on anti-tb treatment and the
lesion disappeared completely at month 2 of treatment
Conclusions: Tuberculosis is still a major healthcare
problem in our country. In this case, the patient was
presented to you as she involved a rare presentation.
Keywords: Childhood tuberculosis, organizing
pneumonia, systemic involvement
[P-160]
Regulatory T cells and insulin resistance
within the triangle of obesity, asthma and
liver diseases
Mustafa Metin Donma1, Muhammet Demirkol1, Burcin
Nalbantoglu1, Orkide Donma2
1
Department of Paediatrics, Faculty of Medicine, Namik
Kemal University, Tekirdag, Turkey
2
Department of Medical Biochemistry, Cerrahpasa
Medical Faculty, Istanbul University, Istanbul, Turkey
Background&Aims: Obesity and associated chronic
diseases such as asthma and cancer are known with
their inflammatory aspects. The impairment of the ability
of regulatory T cells (Tregs) to suppress inflammatory
responses may lead to obesity-associated inflammation.
Obesity-associated diseases are critical risk factors for
liver diseases. Effects of obesity on respiratory system
appear to play roles in childhood asthma. Hepatotropic
viruses also affect lungs as well as respiratory system.
Methods: A flow cytometric analysis of T cell
subpopulations should be performed in peripheral blood
mononuclear cells. Homeostatic Model Assessment for
Insulin Resistance was used to determine insulin sensitivity.
Case: The 12-year-old female patient was started on
Results: Reduction in Tregs may initiate inflammatory
- 83 -
processes and cause obesity. Obesity in childhood
increases the risk of primary liver cancer in adults.
Significantly reduced Tregs were detected in obese,
asthmatic and obese asthmatic children. Tregs was also
reduced during acute hepatitis A. Decreased Tregs lead
to reduced suppressive activity and result in liver injury.
Inflammation may contribute to the development
of insulin resistance (IR). Hyperinsulinemia may
impair the ability of Tregs to suppress inflammatory
responses and may lead to the development of obesityassociated inflammation. Obese patients with IR
display significantly decreased Tregs. Tregs possess a
potential therapeutic value to improve IR by limiting
inflammation. Visceral obesity enhances hepatitis C
virus-induced IR and infection associated with obesity
promotes hepatocellular carcinoma development.
Conclusions: Interactions among hepatitis, obesity
and asthma characterized by chronic and systemic
inflammation are quite important. Many bidirectional
interactions might contribute to the progression to
obese state and then to steatohepatitis and eventually
fibrosis, which may be combined with asthma. Common
mechanisms and synergy among them are interesting
and quite complicated. This needs further investigation.
Keywords: Regulatory T cells, insulin resistance,
obesity
[P-161]
Gender difference in association with insulin
levels and parental body mass index in
underweight children
Mustafa Metin Donma1, Muhammet Demirkol1, Burçin
Nalbantoğlu1, Birol Topçu2, Orkide Donma3
1
Department of Paediatrics, Faculty of Medicine, Namik
Kemal University, Tekirdag, Turkey
2
Department of Biostatistics, Faculty of Medicine, Namik
Kemal University, Tekirdag, Turkey
3
Department of Medical Biochemistry, Cerrahpasa
Medical Faculty, Istanbul University, Istanbul, Turkey
Background&Aims: Malnutrition (MN) is one of the
most important causes of child mortality alone. The
investigations related to the evaluation of abnormal
glucose homeostasis, hyperinsulinemia and insulin
resistance in malnourished children are extremely
limited. The aim of this study is to investigate the
availability of the anthropometric measurements,
ratios based on them, and insulin sensitivity indices
for the evaluation of underweight (UW) children.
Methods: Based upon WHO-BMI criteria, of 119
children, 48 were underweight (UW) and 71 were
normal weight (NW). The children within 85th – 15th
percentiles were evaluated as NW and those below 15th
percentiles were included in UW groups. Study protocol
was approved by the Ethics Committee and informed
consent was taken from the parents prior to study.
Anthropometric measurements were recorded. Ratios
based upon them as well as insulin sensitivity indices
were calculated. The analyses of the body fat were
performed. PASW 18 was used for statistical analyses.
Results: Significant
differences
were
observed
in
height:2-to-waist circumference and height:2-to- hip
circumference ratios of the children (p<=0.05). Strong
correlations were observed between insulin levels and
maternal as well as paternal BMIs in UW girls and boys,
respectively (p<=0.01). There were strong correlations
between HOMA levels and maternal as well as paternal
BMIs in UW girls and boys, respectively (p<=0.01).
Conclusions: It was shown that togetherness of the
mother-her daughter and father-his son, socioeconomic
and cultural values shared within the family may all have
important effects on intergenerational continuity of the
poor eating habits. The important correlations found
between insulin as well as HOMA levels and parental BMIs
of UW children seem to contribute to the investigations
performed on intergenerational transmission.
Keywords: Gender difference, insulin levels,
underweight children
[P-162]
Evaluation of the insulin sensitivity indices in
accordance with the cut off points in morbid
obese boys
Mustafa Metin Donma1, Muhammet Demirkol1, Burçin
Nalbantoğlu1, Birol Topçu2, Orkide Donma3
1
Department of Paediatrics, Faculty of Medicine, Namik
Kemal University, Tekirdag, Turkey
2
Department of Biostatistics, Faculty of Medicine, Namik
Kemal University, Tekirdag, Turkey
3
Department of Medical Biochemistry, Cerrahpasa
Medical Faculty, Istanbul University, Istanbul, Turkey
Background&Aims: Obesity is a growing health problem
in the world. Especially increasing rate of morbid obesity
accounts for serious health problems during childhood.
The aim of this study is to evaluate anthropometric
measurements and their ratios along with the surrogate
indices of insulin sensitivity in morbid obese (MO) boys.
Methods: Seventy five MO boys, aged 10.1±2.2 years,
participated into the study. Those in the >99 percentile
were considered as MO based upon WHO criteria. Written
informed consent was obtained from the parents and the
study protocol was approved by the Ethics Committee.
Anthropometric measurements were carried out. Ratios
and insulin sensitivity index (ISI) values were calculated.
Children were grouped based on the frequently used cutoff points as well as medians and arithmetic means: The
values were designed as below and above 2.0, 2.43, 2.5
and 3.16 for HOMA-IR; 6.0, 7.0, 9.42 and 15.24 for FGIR;
0.328, 0.344, 0.356 and 0.357 for QUICKI. PASW 18
statistical package program was used to evaluate the data.
Results: In the evaluation of MO boys, head
Circumference (C) /neck C, HOMA/BMI, log HOMA, FMI,
Index I and Index II were statistically significant upon
evaluation of four different values for each ISI. Especially
head C/neck C, Index-I and Index-II were quite
significant in evaluation of these parameters according
to the values below and above 2.5, 15.24, 0.356 for
HOMA-IR, FGIR and QUICKI, respectively (p<=0.01).
Conclusions: Evaluation of laboratory criteria is an
important approach for upgrading the quality of life of
- 84 -
children. We concluded that head C/neck C, Index-I and
Index-II are important and three new valuable indices
for the evaluation of MO boys, when considered based
upon ISI indices.
Keywords: Insulin sensitivity indices, morbid obese
boys
[P-163]
The positive family history of hypertension
and coronary heart diseases among
grandparents as a risk factor for childhood
obesity
Mustafa Metin Donma1, Muhammet Demirkol1, Burçin
Nalbantoğlu1, Orkide Donma2
1
Department of Paediatrics, Faculty of Medicine, Namik
Kemal University, Tekirdag, Turkey
2
Department of Medical Biochemistry, Cerrahpasa
Medical Faculty, Istanbul University, Istanbul, Turkey
Background&Aims: Hypertension (HT) is one of the
risk factors of obesity. Its association with coronary heart
diseases (CHDs) is also well known. Living with chronic
HT can be stressful for those with the diagnosis and their
families. Obesity is rapidly increasing during childhood.
In this study, the association between HT as well as CHDs
in grandparents and obesity in children was investigated.
Methods: A total of 118 children, aged 2-14 years,
were included into the scope of the study. The body
mass indices (BMIs) of seventy-eight were above
95 percentile criteria defined by WHO. Another forty
children, whose BMIs are within normal ranges,
constituted the control group. The finding of being
hypertensive was investigated in grandparents [maternal
grandmother (MGM), paternal grandmother (PGM),
paternal grandfather (PGF), maternal grandfather
(MGF)]. SPSS was used for statistical analyses.
Results: In obese group, HT was observed in at
least one of the grandparents of 47 out of 78 children
(60%). In the control group, this ratio was 14 out of
40 (35%). A statistically significant difference was
observed between the groups (p<=0.05). In the obese
group, the percentages of hypertensive MGM, PGM,
PGF and MGF were 27.5%, 27.5%, 15% and 10%,
respectively. In the control group, these values were
found as 22.5%, 10%, 7.5% and 5%, respectively.
Out of 47 hypertensive grandparents, 39 suffered also
from CHDs in the obese group. The frequency of the
coincidence of both HT and CHDs was 83% and 57%
in obese and control group, respectively (p<=0.05).
Conclusions: The preponderance of the grandparental
female gender was noted in obese group (55% vs
32.5%). This finding may be confirmed by the future
genetic studies.
Keywords: Family history, hypertension, childhood
obesity
[P-164]
Application of surfactant in a child case with
acute respiratory distress syndrome due to
bronchopneumonia
Kazım Küçüktaşçı1, Duygu Taşkaldıran2, Nedim
Samancı2, Özgür Kızılca3, Muhammet Demirkol2, Nuriye
Ece Mintaş2, Hilal Balcı2, Zeynep Ersöz Güngör2
1
Clinic of Paediatrics, Tekirdag State Hospital, Tekirdag,
Turkey
2
Department of Paediatrics, Faculty of Medicine, Namik
Kemal University, Tekirdag, Turkey
3
Division of Paediatric Cardiology, Department
of Paediatrics, Faculty of Medicine, Namik Kemal
University, Tekirdag, Turkey
Introduction: Acute respiratory distress syndrome
(ARDS) has an incidence of 8.5-16 cases/1,000 pediatric
intensive care unit admissions. Primary lung disorders
that cause ARDS include pneumonia, aspiration,
inhalation injuries, near-drowning and contusions from
trauma. These disorders injure alveolar epithelium
and increase permeability of the alveolar-capillary
barrier. Activity of surfactant is impaired in ARDS due
to decreased production, recurrent alveolar collaps
during ventilation, and the last and the most importantly
due to the dysfunction caused by plasma proteins.
Surfactant therapy is aimed to improve gas exchange
and protect from the injury caused by the ventilator. In
this report, we presented a child case with ARDS due
to bronchopneumonia, who benefitted from surfactant
therapy.
Case: 11 month-old female patient who has neurological
sequelae due to meningitis in the newborn period was
hospitalized with the diagnosis of bronchopneumonia.
Ampicillin-sulbactam, cefotaxime, methylprednisolone,
salbutamol, fluid and oxygen with nasal mask were began
as treatment. Clinical status of the patient did not improve,
and fever occurred. On the 4th day, antibiotherapy was
changed to vancomycin and meropenem. On the 6th
day of admission, patient was intubated (P-SIMV mode,
PIP:28cmH2O, PEEP:7cmH2O, Rate:40/min, FiO2:1.00).
SaO2 was around 80-90%. pH:7.10, pCO2:113mmHg,
HCO3:35mmol/L, PaO2:67mmHg were detected in the
blood gas, and PaO2/FiO2 was 67. Chest radiograph
revealed bilateral edema. Echocardiography showed
normal pulmonary artery pressure, and there was no
heart failure. Patient with suspected primary ARDS due to
severe bronchopneumonia was treated with intratracheal
surfactant (survanta®) therapy in two doses of 4cc/kg
with an interval of 24 hours. After surfactant therapy,
SaO2 rised in a short time. Patient was intubated for
thirteen days. Antibiotherapy was stopped at 21st day. On
the 24th day of admission, she was discharged with cure.
Conclusion: We wanted to emphasize that application
of surfactant may be beneficial in patients with ARDS by
correcting oxygenation.
Keywords: Acute respiratory distress syndrome,
bronchopneumonia, surfactant
- 85 -
[P-165]
[P-166]
A child case of retropharyngeal abscess due
to Stenotrophomonas maltophilia after a
penetrating trauma
Two cases with dystonia due to risperidone
intake
Muhammet Demirkol1, Özgür Kızılca2, Zeynep Ersöz
Güngör1, Nedim Samancı1, Kazım Küçüktaşçı3, Nuriye
Ece Mintaş1, Duygu Taşkaldıran1, Hilal Balcı1
1
Department of Paediatrics, Faculty of Medicine, Namik
Kemal University, Tekirdag, Turkey
2
Division of Paediatric Cardiology, Department
of Paediatrics, Faculty of Medicine, Namik Kemal
University, Tekirdag, Turkey
3
Clinic of Paediatrics, Tekirdag State Hospital, Tekirdag,
Turkey
Introduction: Retropharyngeal abscess (RPA) is an acute
infection of the lymph nodes between the rear wall of the
pharynx and the prevertebral fascia. Upper respiratory
tract infections and local trauma take role in the etiology.
Here we presented a case with RPA developed after
a penetrating trauma to the oral mucosa with a pen.
Case: 17-month-old male patient was admitted with
complaints of fever lasting for 2 days, swelling in the neck,
and difficulty in swallowing. He had an history of an oral
trauma with a pen 3 days ago. In the first examination he
had restlessness, hypersalivation and mild hyperextension
of the neck. His AT:38.9°C, HR:137beats/min, RR:25/
min, BP:94/56mmHg. His neck was significantly swollen
and he had bilateral multipl lymphadenomegalies. Other
systems were normal. His Hb:8.9g/dL, Hct:29.3%,
MCV:76fl, WBC:25400/mm3, Plt:510000/mm3, Sed.
Rate:101mm/h, CRP:89.8mg/L. In peripheral blood
smear 75% PMNL and toxic granulation were determined.
Other laboratory tests were within normal limits.
Neck ultrasonography revealed bilateral cervical and
submandibular lymphadenomegalies the largest of which
is 27x11mm. Because patient had history of penetrating
trauma, he was consulted to ENT and hospitalized with
treatment of 100mg/kg/day iv ceftriaxone and 30mg/
kg/day iv clindamycin. Defervescence occurred and
lymphadenomegalies became smaller on the 4th day of
treatment. But on the 6th day, fever recurred and stridor
became evident. Antibiotherapy was changed to 40mg/
kg/day vancomycin and 90mg/kg/day meropenem. On
the 7th day, stridor increased and oxygen desaturation
occurred and patient was intubated. Neck CT revealed
RPA formation. Abscess was drained and 100.000 cfu/
ml Stenotrophomonas maltophilia with trimethoprim/
sulfametoxazol (TMP-SMX) sensitivity was isolated.
On the 3rd day of iv TMP/SMX, defervescence
occurred and patient was extubated on the 8th day.
On the 10th day, patient was discharged with cure.
Results: Retropharyngeal abscess is rarely seen in
childhood, but early diagnosis and treatment is of vital
importance.
Keywords: Penetrating trauma, retropharyngeal
abscess, stenotrophomonas maltophilia
Muhammet Demirkol1, Kazım Küçüktaşçı2, Nedim
Samancı1, Nuriye Ece Mintaş1, Özgür Kızılca3, Ayşin
Nalbantoğlu1, Hilal Balcı1, Duygu Taşkaldıran1, Zeynep
Ersöz Güngör1
1
Department of Paediatrics, Faculty of Medicine, Namik
Kemal University, Tekirdag, Turkey
2
Clinic of Paediatrics, Tekirdag State Hospital, Tekirdag,
Turkey
3
Division of Paediatric Cardiology, Department
of Paediatrics, Faculty of Medicine, Namik Kemal
University, Tekirdag, Turkey
Introduction: Dystonia is characterized by sustained
muscular contractions with twisting and repetitive
movements or abnormal postures. It is an extrapyramidal
adverse effect mostly seen after exposure to dopamine
receptor blockers, including anti-emetics, gastrointestinal
promotility agents, antipsychotic drugs. However, acute
dystonic reactions may be triggered with all drugs that
alter dopamine signaling. Clinical manifestations include
oculogyric crises, blepharospasm, complex cervical
dystonia, focal limb dystonia, and back arching. Acute
dystonic reactions respond dramatically to injections
of anticholinergic drugs. Biperidene, procyclidine,
benztropine, trihexyphenidyl or diphenhydramine may
be used to control reaction. Here, we presented 2
children with dystonic reaction due to risperidone intake.
Case-1: 15 years-old female patient was admitted to
emergency department due to suicidal attempt with 30
risperidone 1 mg tablets and 20 clomipramin 10 mg
dragees. Applications of gastric lavage and activated
charcoal were done in period of 60 minutes after the pills
had been taken. 12 hours later the pills had been taken,
she had an acute dystonic reaction in her upper and lower
limbs as semiflexion and extension movements. After 10
mg of intramuscular biperidene, the reaction was under
control. In her 96 hours of follow up there was no clinical and
laboratory finding. The patient was discharged with cure.
Case-2: 10 years-old female patient with known
epilepsy that is under control with levetiracetam and
risperidone was admitted to the emergency department
with complaints of vertical deviation in eyes, extension
of neck and uncontrolled flexion-extension movements
in upper extremities. With suspicion of dystonia, 5
mg dose of intramuscular biperidene was given to
the patient and reaction was under control. After 96
hours of follow up, patient was discharged with cure.
Conclusions: As a side effect of risperidone, dystonia
has to be kept in mind and families should be informed
about this effect.
Keywords: Dystonia, risperidone, side effect
- 86 -
[P-167]
[P-168]
Spondylodiscitis in an adolescent case
Nedim Samancı1, Muhammet Demirkol1, Kazım
Küçüktaşçı2, Nuriye Ece Mintaş1, Özgür Kızılca3, Hilal
Balcı1, Ayşin Nalbantoğlu1, Duygu Taşkaldıran1, Zeynep
Ersöz Güngör1
1
Department of Paediatrics, Faculty of Medicine, Namik
Kemal University, Tekirdag, Turkey
2
Clinic of Paediatrics, Tekirdag State Hospital, Tekirdag,
Turkey
3
Division of Paediatric Cardiology, Department
of Paediatrics, Faculty of Medicine, Namik Kemal
University, Tekirdag, Turkey
Introduction: Childhood spondylodiscitis represents a
process of infectious conditions of the spine, from discitis
to vertebral osteomyelitis with occasional associated
soft tissue abscess formation. It often presents as
backache, restricted spinal mobility, gait disturbance,
refusal to walk and sit, deteriorated general condition,
and a need to hold on to objects for support. While
the inflammation markers increase slight to moderate
degree, blood cultures are usually negative. Although
radiographs should show intervertebral disc height
narrowing if symptoms have exceeded 10 days, further
imaging with MRI is likely to confirm the diagnosis.
Because childhood spondylodiscitis is extremely
rare in childhood, diagnosis is often established
after an extensive delay. Treatment includes antiinflammatory agents, antibiotics and immobilization.
Here, we presented an adolescent girl with a diagnosis
of spondylodiscitis who was treated successfully.
Case: 14 years-old female patient was admitted to
outpatient clinic with complaints of fever and lower
backache. Physical examination was normal. Her
Wbc:7740/mcl
(72%
neutrophilia),
ESR:38mm/
hr, CRP:84mg/L (<10); ASO, RF, Rose Bengal, ANA,
Anti-dsDNA, PPD, QuantiferonTB, HLA-B27 were
negative.
Chest
radiograph,
abdominal-urinary
ultrasonography,
echocardiography
were
normal.
Contrast-enhanced lumbosacral MRI showed significant
contour derangement, T1-hypointense, T2-hyperintense
pathological signal difference and dense contrast
enhancement in inferior part of L2 and superior part
of L3 vertebrae corpora with narrowing of the distance
between both. 99mTc MDP whole body scintigraphy
supported the diagnosis of spondylodiscitis suggested in
MRI. Vankomisin intravenous, cefotaxime intravenous,
and ibuprofen po were started as therapy. On the 5th
day of therapy, complaints of the patient were relieved.
On the 21st day of therapy, the patient was discharged
with treatment of oral fusidic acid and ciprofloxacin in
order to be used for 3 weeks.
Eventration of the diaphragm as a rare cause
of cyanosis in infancy
Özgür Kızılca1, Kazım Küçüktaşçı2, Muhammet
Demirkol3, Nedim Samancı3, Zeynep Ersöz Güngör3,
Hilal Balcı3, Duygu Taşkaldıran3, Ayşin Nalbantoğlu3
1
Division of Paediatric Cardiology, Department
of Paediatrics, Faculty of Medicine, Namik Kemal
University, Tekirdag, Turkey
2
Clinic of Paediatrics, Tekirdag State Hospital, Tekirdag,
Turkey
3
Department of Paediatrics, Faculty of Medicine, Namik
Kemal University, Tekirdag, Turkey
Introduction: Eventration of the diaphragm is rare in
childhood. Birth trauma and cardiac surgery are the
most common causes. Right-side involvement is more
common. Elevated diaphragm with fields of atelectasis
in chest radiograph provides the diagnosis. Thorax
CT and fluoroscopy also clarify the status. Treatment
is support in the beginning. Surgical plication is the
treatment of choice in the patients with significant
respiratory distress or without spontaneous regression.
Here, we presented a patient with cyanosis in whom
we detected unilateral eventration of the diaphragm.
Case: 6 months-old male patient was admitted to
emergency department with diarrhea, vomiting and fever.
Cyanosis was realized and pO2 saturation was detected
82-84%. He was hospitalized for further evaluation.
His weight: 5,6 kg (<3p), height: 68 cm (10-25p), BP:
88/43 mmHg, RR: 45/min and fingers had clubbing. He
had abdominal breathing pattern and decreased breath
sounds on the base of right lung. Cardiovascular system
and other systems were normal. Routine laboratory
tests of CBC, CRP, KFT, LFT and electrolytes were
normal. In venous blood gas pH:7.36, PCO2: 48mmHg
and bicarbonate: 27mmol/L. Chest radiograph revealed
lung opacities on the base of right lung. ECG and
echocardiography showed no abnormality. Elevated right
diaphragm and basal atelectasis in the right lung were also
detected in the chest CT which is done in suspicion of a
mass. Nebulised salbutamol and fluticasone with postural
drainage were started to the patient as a treatment.
His paO2 saturation raised to 92% in room air with
treatment. Diaphragmatic paralysis was detected in the
fluoroscopic examination of the patient. Then the patient
was discharged with a plan of diaphragmatic plication.
Result: Eventration of the diaphragm, as a rare disorder,
has to be kept in mind in case of cyanosis.
Keywords: Cyanosis, diaphragmatic paralysis,
eventration of the diaphragm
Conclusion: Spondylodiscitis, as a rare disease of
childhood, should be kept in mind in differential diagnosis
of patients with complaints of fever and backache.
Keywords: Adolescent, spondylodiscitis
- 87 -
[P-169]
A case of central nerves system tuberculosis
diagnosed by biopsy
Murat Sutcu1, Manolya Acar1, Ayper Somer1, Aslı Toros1,
Nur Aydınlı2, Mine Çalıskan2, Cicek Bayındır3, Nuran
Salman1
1
Istanbul University Istanbul Medical Faculty, Pediatric
Infectious Diseases
2
Istanbul University Istanbul Medical Faculty, Pediatric
Neurology
3
Istanbul University Istanbul Medical Faculty, Pathology
Introduction: Central nervous system tuberculosis is
the most serious form of tuberculosis which can be mortal
unless treated effectively. Several forms of infectious
and non infectious diseases should be eveluated in
differential diagnosis. Surgical intervention may be
necessary for certain diagnosis in selected cases.
Case presentation: Six year old child with history
of herpes encephalitis, presented with headache,
vomiting and confusion. Physical examination and initial
laboratory evaluation were normal. Cranial MRI revealed
hypodense edematous lesion with shifting on right
temporoparietooccipital lobe. Necrotizing granulomatous
inflammation and acid resistant stain positive bacilli
were identified from biyopsy material. Antituberculous
therapy and corticosteroid treatment were initiated. After
18 months of antituberculous treatment, child recovered
well with normal neuromotor development.
Conclusion: In the cases when differential diagnosis is
impossible with clinical and radiological findings, surgical
intervention may be obligatory.
Keywords: Biopsy, child, central nervous system
tuberculosis
[P-170]
Electrical storm in an adolescent
with arrhythmogenic right ventricle
cardiomyopathy resulted in cardiac
transplantation
Hayrettin Hakan Aykan1, Mustafa Gülgün2, Ilker
Ertuğrul1, Tevfik Karagöz1
1
Hacettepe University, Pediatric Cardiology, Ankara/
Turkey
2
Gulhane Military Medical Academy, Pediatric
Cardiology, Ankara/Turkey
Arrhythmogenic right ventricle dysplasia/cardiomyopathy
(ARVD/C) is an inherited cardiomyopathy characterized by
fibrofatty replacement of the right and less frequently left
ventricle. Ventricular arrhythmias requiring implantable
cardioverter defibrillator (ICD) are common in patients
with ARVD/C. Electrical storm (>=3 life-threatening
ventricular arrhythmia within 24-hour period) resulting
in ICD discharges is a major cause of morbidity and
mortality. We had experience of a case who 15 year-old
boy presented with chest pain and decreased exercise
capasity. He had been undergone surgical closure for
atrial septal defect and complicated with ventricular
tachycardia/fibrillation (VT/VF) postoperatively at
11 years-old. No history of congenital heart defect in
family and syncope were found. Premature ventricular
contractions were determined occasionally in 24-hours
Holter monitoring. Echocardiography demonstrated
dilatation of right ventricle and right ventricle outflow
tract. The dilated infundibulum and increasing in the right
and left ventricular trabeculation were demonstrated
in the cardiac angiography and magnetic resonance
imaging. Non-sustained monomorphic VT (260 beats/
minute) was induced during electrophysiologic study.
He was diagnosed as ARVD/C and ICD was implanted
for primary prevention. Two years later, the episodes
of VT/VF was occurred 35 times within one month.
He was started on amiodarone and sotalol and the
radiofrequency ablation was performed. Two months
later, the electrial storm repeated again and the patient
was arrested in a short time. He was immediately
connected to the pump after cardiac resuscitation and
undergone the cardiac transplantation one day later. He
has been on follow up with no symptom for three year.
ICD seems indispensable and the ablation of VT can be
considered for reducing the side effects of the drugs and
prolonging a ICD battery life in ARVD/C currently. The
management of electrical storm should be individualized
for each patient and the treatment may indicate
extracorporal membrane oxygenation and cardiac
transplantation.
Keywords: electrical storm, cardiomyopathy,
transplantation
[P-171]
Kawasaki disease shock syndrome: Case
Report
Ayşegül Dogan Demir, Selçuk Uzuner, Ufuk Erenberk,
Mustafa Oğur, Özden Türel, Yılmaz Yozgat, Mebrure
Yazıcı, Nurettin Onur Kutlu
Pediatrics, Bezmialem Vakif University, Istanbul, Turkey
Background: Kawasaki disease is a rare self-limited
systemic pediatric vasculitis and results in coronary
artery aneurysms in 20% of untreated patient. During
acute phase of illness, hemodynamic instability and
shock is uncommon. Kawasaki disease shock syndrome
(KDSS) was recently described is associated with moresevere laboratory markers of inflammation and mitral
regurgitation, prolonged myocardial dysfunction and shock.
Case: We report twelve-year-old who presented with
mumps and a toxic shock-like syndrome was treated with
fluid resuscitation and vasoactive infusion. Finally our case
was diagnosis with Kawasaki disease shock syndrome
when right giant coronary artery aneurysms were
found on subsequently echocardiographic examination
Result: KDSS should be considered in all children
have prolonged fever, most- overt systolic and
diastolic dysfunction, and shock. pediatricians assess
for coronary artery abnormalities with subsequently
echocardiographic examination in all children have
severe myocardial dysfunction, prolonged fever and
shock syndrome.
Keywords: Kawasaki disease, echocardiographic
examination
- 88 -
[P-172]
[P-173]
Mustafa Taha Özkul1, Melis Köse2, Mehtap Kağnıcı2,
Cahit Barış Erdur1, Erhan Özbek1, Ferah Genel1
1
Pediatri Anabilim Dalı,Dr.Behcet Uz Cocuk Hastaliklari
Hastanesi Egitim ve Arastirma Hastanesi, Izmir, Turkiye
2
Cocuk Metabolizma Hastaliklari Bilim Dalı, Dr.Behcet
Uz Cocuk Hastaliklari Hastanesi Egitim ve Arastirma
Hastanesi, Izmir, Turkiye
Background&Aims: Citrin deficiency, which is encoded
by SLC25A13 gene, is a disorder with two phenotypes:
neonatal intrahepatic cholestasis caused by citrin
deficiency (NICCD), and adult onset type II citrullinaemia
(CTLN2). NICCD is an autosomal recessive metabolic
disorder characterized with intrahepatic cholestasis and
increased serum citrulline. Symptoms resolve within
the first year of life mostly without special treatment.
However, some patients may develop more severe
symptoms of CTLN2 like neuropsychiatric symptoms
and hyperammonemia on adulthood. The objective of
this study is to report the case of a 2.5 months old boy
with NICCD, focusing on its diagnosis and treatment.
Methods: A 40-week gestation male, with a birth weight
of 3200 grams, was born after an eventful pregnancy
with cesarean delivery, second child of healthy nonconsanguineous couple. He was exclusively breastfed.
He was admitted to hospital for prolonged jaundice. He
had jaundice, pallor and hepatomegaly. Acholic stool
was negative. Total bilirubin was 11.6 mg/dl, direct
bilirubin was 4.6 mg/dl, serum α-fetoprotein level was
51643.62 ng/mL. Infectious causes of neonatal jaundice
were negative. Abdominal ultrasonography was normal.
Metabolic evaluation disclosed elevation of blood lactate,
blood ammonia and citrulline, methionine, threonine,
arginine and lysine among plasma aminoacids. Serum
total galactose, free galactose and galactose 1-p
levels were found elevated with normal galactose 1-p
uridil transferase activity. These findings and mutation
analysis of SLC25A13 gene confirmed NICCD. Patient
was treated with high protein diet, fat-soluble vitamins,
ursodeoxycholic acid.
Mustafa Torehan Aslan1, Benhur Sirvan Cetin2,
Taylan Celik2, Enes Sali2, Solmaz Celebi2, Mustafa
Hacimustafaoglu2
1
Uludag University Medical Faculty Department of
Paediatrics, Bursa/TURKEY
2
Uludag University Medical Faculty Department of
Paediatric Infectious Diseases, Bursa/TURKEY
Introduction: Childhood pneumonia, especially in
developing nations is one of the most important morbidity
and mortality causes. Round pneumonia (RP), a subgroup
of pneumonia, is characterised by round increased
opacities in the chest X-Ray. Our case is presented
because RP accompanied by leukemoid reaction is rare
and the presence of malignity in differential diagnosis.
A rare cause of neonatal cholestasis;citrin
deficiency
Results: Patient has shown improvement in clinical
follow-up and observed the progressive normalization
of liver function tests and aminoacid profile with in few
months.
Conclusions: In neonatal cholestasis, early recognition
of NICCD is fundamental to apply specific diagnostic tools
and appropriate dietary treatment, giving the possibility
of a close monitoring of patients in follow-up care and
ways to prevent the development of CTLN2.
Keywords: Citrin deficiency, Citrullinemia, Neonatal
intrahepatic cholestasis
A Round Pneumonia Case with Leukemoid
Reaction Mimicking Lung Malignity
Case: A 3 year 7 months male patient with fever, cough
and difficulty in breathing complaints that started two
weeks ago and did not show improvement after oral
antibiotics prescription from a health center, fever
and cough increased over the last two days. Physical
examination; right mid-lower lung zone localized
decreased breathing sounds and widespread crackles,
bilateral rough lung sounds. Complete blood count;
leukocytosis (53,900/mm³), peripheral smear had 86%
neutrophilia, two positive toxic granulation with no blast
or atypic cell. Biochemical tests; C-Reactive Protein
9.87mg/dl,
Procalcitonin
2.37ng/mL,
Erythrocyte
Sedimentation Rate 26mm/hour. Chest X-Rays showed
right paracardiac increased consolidation and the right
lung lower zone had demarcated 24x15mm round,
contoured opasity increase. A Thorax Computed
Tomography taken for further investigation and differential
diagnosis revealed a consolidation region compatible
with RP in the lower lobe’s posterio-basal segment of
the right lung. Parenteral cefotaxime antibiotherapy was
initiated. After three days of parenteral antibiotherapy
clinical and laboratory findings showed significant
improvement and the patient was discharged with a 14
day oral sequential therapy (Amoxicilin-Clavulanic Asid).
After completing the treatment our patient came for
out-patient clinic control and showed full improvement
in his complaints, laboratory findings and chest X-Ray.
Conclusion: Other than RP being a well described
radiologic entity, RP accompanied by leukemoid
reaction is a rarely seen condition. Radiologically
solitary, demarcated mass lesion has malignities in
the differential diagnosis. Our case’s first complaints
resembled malignity but full improvent with appropriate
antibiotherapy left no need for further investigation for
differential diagnosis.
Keywords: Round Pneumonia, Child, Leukemoid
Reaction
- 89 -
[P-174]
A Case with Widespread Herpes Virus
Dermatitis and Conjunctivitis
Mustafa Torehan Aslan1, Sefika Elmas Bozdemir2,
Solmaz Celebi2, Benhur Sirvan Cetin2, Mustafa
Hacimustafaoglu2
1
Uludag University Medical Faculty Department of
Paediatrics, Bursa/TURKEY
2
Uludag University Medical Faculty Department of
Paediatric Infectious Diseases, Bursa/TURKEY
Introduction: Conjunctivitis in children is common in
pediatric emergency departments. It is characterised
with severe tear flow, conjunctival hyperemia, chemosis,
watering, itching and pain. Conjunctivitis due to herpes
simplex virus (HSV) may be primary infection or
recurrence, and is unilateral in 80% of the cases. This
report presents a child with bilateral HSV conjunctivitis
and dermatitis that showed full lesional improvement
after two weeks of systemic and local treatment.
Case: A 3 year old male patient, no history of known
systemic illness, presented with skin eruptions that started
a week ago, first on the eyelids then spread to the whole
eye area accompanied with erythema and eye discharge
that did not respond to local treatment prescribed by eye
specialists. Physical examination revealed widespread
vesicular skin lesions with hyperemic ground on the eye
area spreading towards the face and purulent flow from
both eyes. Other systemic examinations were normal.
Full blood count; leucocytes 8170/mm³, haemoglobin
11.6gr/dl, platelet 331000/mm³, C-Reactive Protein
0.47mg/dl. The case was evaluated by the departments
of Pediatric Infectious Diseases and Eye Diseases and
diagnosed with herpes virus dermatitis and herpes
virus conjunctivitis without corneal involvement;
intravenous acyclovir, cefuroxime, clindamycin and
local acyclovir eye ointment, tobramycin eye drops,
ofloxacin eye drops and biborate de soude eyewash
treatments were initiated. After 10 days of treatment
almost all the lesions dried up and conjunctivitis healed.
Conclusion and Discussion: The case was presented
in order to attract attention on the rarely seen pediatric
bilateral HSV conjunctivitis and dermatitis and the need
for systemic treatment.
Keywords: Herpes Virus Dermatitis, Child,
Conjunctivitis
[P-175]
a leishmaniasis case present with prolonged
fever and hepatosplenomegaly
Müferet Ergüven, Doruk Gül, Asena Pınar Sefer, Ismet
Düşmez
GOZTEPE RESEARCH HOSPITAL ISTANBUL/TURKEY
Introduction: Leishmaniasis, is a common name of a
clinical syndrome caused by Leishmania specieses which
is an obligatory intracellular parasite. Leishmaniasis is
an endemic disease in our country and our neighbour
countries. Leishmania specieses cause three main clinical
form of diseases which are cutenous,mucocutoneous and
visceral.Infected sand fly is the vector of leishmaniasis..
Systemic leishmaniasis is a progressive infectious
diseases which mainly affect reticuloendotelial system.
If the disease treated unproperly,it may cause death.
%80 of the cases are younger than 5 years old. Because
of the prolonged incubation period and unspecific
symptoms; Its hard to diagnose of the disease.
In this case, we described a systemic Leishmaniasis
present with prolonged fever and hepatosplenomegaly.
Case: A 2 years old girl was admitted to a hospital with
prolonged fever which was continues about 15 days
and unresponsive to antibiotic treatment.The fever was
relapsing 2-3 times in a day, reaching to 40 degrees and
decreased spontaneously
She hadn’t any complain other than fever
The physical examination of the patient;
she had hepatosplenomegaly about 4-5 cm.The other
findings were normal.
In laboratory; Hb:8.03g/
dl,Plt:156000,Wbc:4.1,Neu:770,Crp:0.33mg/dl
biochemical analysis:normal urinalyse:normal
Chest x-ray: normal
In abdominal USG;liver and spleen size increased
We started broad spectrum antibiotic.In the 72.hour
of the antibiotic;the patient still had fever and her
hepatosplenomegaly were increased.
The laboratory findings;Hb:5.8mg/dl
plt:130000,wbc:6700,neu:670,Crp:2.7mg/dl
Because of continued fever and pancytopenia; We
made bone marrow aspiration
Leishmania amastigotes were seen in her bone marrow
aspiration material.
The patient was diagnosed to Leishmaniases and
amphotericin b treatment was started.In the 48. hour
of treatment the fever was decreased dramaticaly and
the sizes of liver and spleen decreased.
At the 10th day of the treatment patient was fully
recover and externated from hospital.
Keywords: leishmaniasis, prolonged fever,
hepatosplenomegaly
[P-176]
The Etiology of Hypertransaminasemia in
Children
Filiz Serdaroglu1, Mustafa Akcam2, Tugba Koca2, Selim
Dereci2, Nagehan Aslan1
1
Department of Pediatrics, S. Demirel University,
Medical School, Isparta, TURKEY
2
Department of Pediatrics, Division of Pediatric
Gastroenterology, Hepatology and Nutrition, Faculty of
Medicine, S. Demirel University, Isparta, Turkey
Aim: To investigate the etiology and demographic
characteristics of children determined with high levels of
transaminase in our hospital over a period of two years.
Material-Method: Children aged 3 months – 8 years
who presented at our hospital between December 2012
and December 2014, either as in-patients or outpatients
and were determined with elevated transaminase
(ALT>45, AST>50 IU/L) were identified from the hospital
records system (MEDULA). Retrospective evaluation was
made of these children from the records in respect of
demographic characteristics, diagnosis and tools used
- 90 -
for diagnosis.
Results: The study included a total of 281 children who
met the study criteria. These comprised 125 (44.5%)
females and 156 (55.5%) males with a mean age of
7.01±5.24 years. The most common complaint was
weakness (53.4%) and 15.7% had no complaints. In
the physical examination the most common findings
were jaundice and hepatomegaly and 15% of cases
were completely normal. In the etiology, infectious
causes (34%) were most common and within these,
HAV infection (18.9%) was the leading cause. Drugsrelated causes were determined at a rate of 18.1% and
non-alcoholic fatty liver disease (NAFLD) at 11.1%.
Idiopathic cases were seen at the rate of 6.4% and all
were seen to have returned to normal levels at mean 95
days (range, 7 days- 14 months). The highest levels of
transaminase were determined to be caused associated
with HAV infection and slightly elevated levels were
caused by medications and NAFLD.
Conclusion: The results of this study showed that
elevated transaminase was caused most often
by infections, drug toxicity and NAFLD. Elevated
transaminase is often encountered in daily practice and
even though it indicates liver disease in the majority
of cases it may also be associated with a cause other
than the liver and may be seen in completely healthy
individuals.
Keywords: Children, hepatitis, transaminase
[P-177]
Early-onset chromhidrosis in an infant
Nagehan Aslan, Şenay Akbay, Ali Ayata
Department of Pediatrics, Suleyman Demirel University
Faculty of Medicine, Isparta, Turkey
Chromhidrosis is the production of colored sweat by eccrine
or apocrine sweat glands. There have been very few reports
of chromhidrosis in the pediatric age group. We report a
4 month old girl with this rare dermatological disorder.
A 4-month-old girl presented with a history of staining
of her undershirt, especially the axillary region. This
coloration noted by parents for last two month. She was
born after a normal pregnancy from non-consanguineous
parents. She had no history of drug use or illness. She was
fed with brest milk. On physical examination it was noticed
that her undershirt was bluish colored (Fig.1). The rest of
the physical examination was normal. Routine laboratory
investigations were normal. Urinary homogentisic acid
level was within normal limits. Microbiological examination
of her skin revealed normal skin flora. Clinical diagnosis
was established. Skin biopsy was not performed
Chromhidrosis refers to secretion of colored sweat
and was first reported in 1709 by Yonge of Plymouth
(1). It has been classified into apocrine, pseudoeccrine, and true eccrine chromhidrosis. Apocrine
chromhidrosis refers to the secretion of colored sweat.
Apocrine sweat glands are located in the axillae,
anogenital skin, areolae and over the skin of the
trunk, face and scalp. The color of chromhidrosis may
be yellow, green, blue, brown or black, depending on
the level of oxidation of lipofuscin secreted in sweat.
In conclusion, although apocrine chromhidrosis may
develop at any age, it is more often after puberty. The
age of the patients reported in the literature range
from 9 months [5] to 76 years. Carman et al. report a
9 month patient reported from Turkey. Our patient was
chromhidrotic for 2 months. To the best of our knowledge,
our 4-month old patient might be the youngest case in
the literature.
Keywords: Chromhidrosis, colored sweat, 4-month-old
[P-178]
A Rare Case: Congenital Asymmetric Crying
Face
Neslihan Özhan1, Ece Gültekin2, Güzide Turanlı2
1
Pediatrics, Faculty of Medicine, Medipol University,
Istanbul, Turkey
2
Pediatric Neurology, Faculty of Medicine, Medipol
University, Istanbul, Turkey
Introduction: Asymmetric crying face is an abnormality
caused by congenital hipoplasia or agenesis of unilateral
depressor anguli oris muscle. The major finding is the
absence or weakness in the outer or lower movement of
the corner of mouth during crying or smiling. The other
expressions of the facial muscles are normal and the face
is symmetric at rest. The major differential diagnosis
is central facial paralysis. To avoid invasive diagnostic
procedures and potential complications, pediatricians
should be consider this congenital abnormality.
Case Presentation: This is a newborn presented with
asymmetric movement of right corner of the lower lip
since birth. The facial asymmetry was more pronounced
when she cried. The other facial movements were
essentially symmetrical. The rest of the neurological
examination was normal including facial nerve function,
as determined by frowning forehead, wrinkling,
eye closure, nasolabial fold depthness and tearing.
Cranial ultrasound was normal and echocardiogram
did not reveal any cardiac abnormality. Auditory
brainstem response did not indicate any abnormality.
Conclusion: Congenital hypoplasia or agenesis of
depressor anguli oris muscle is a rare abnormality and it is
mainly diagnosed by neurological examination. Although
electromyelographic evaluation is not necessary to detect
the muscle weakness sometimes it could be beneficial
for differential diagnosis. The most common problem is
false diagnosis of central facial paralysis in a patient with
asymmetric crying face. Cervicofacial, musculoskeletal,
respiratory, genitourinary and central nervous system
anomalies could be associated with congenital unilateral
hypoplasia or agenesis of depressor anguli oris muscle.
In our case no other malformation was detected.
Combination of clinical suspicion and detailed physical
examination for abnormalities in other systems ensures
early diagnosis and so prevents from invasive diagnostic
procedures in children with asymmetric crying face.
Keywords: asymmetric crying face, agenesis of
depressor anguli oris muscle
- 91 -
[P-179]
A patient with tuberosclerozis who is
tachyarrhythmic without rhabdomyoma
Nihat Cem Çelebi1, Aslı Nur Ören1, Ali Oruç1, Leyla
Telhan2
1
Istanbul Medipol University, Faculty Of Medicine
Department Of Pediatrics
2
Istanbul Medipol University, Faculty Of Medicine
Department of Pediatric Intensive Care
Tuberosclerosis (TS) is an Autosomal Dominant
neurocutenous syndrome, characterised with multipl
hamartomatous lesions that are seen in many organs
such as skin, brain, eyes, kidneys and heart. This
syndrome frequently associated with cortical dysplasia,
subependimal nodules, giant cell astrocytoma, retinal
astrocystic hamartoma, facial angiofibroma, renal
and lung angiolipoma and cardiac rhabdomyoma.
Hamartomatous lesions are usually asymptomatic. For
this reason,these findings are guide for diagnosing of
cases without mental retardation and/or convulsions.
Pathognomonic skin findings of tuberous sclerosus
are
adenoma
sebaceum,
periungual
fibromas,
shagreen patches and ash-leaf macules. Cardiac
rhabdomyomas are seen in sixty percent of the cases
that under age of two. Supraventricular and ventricular
tachycardias, A-V blocks may also accompany for
cardiac rhabdomyomas. Tuberous sclerosis is usually
considered in cases of cardiac arrhytmias that are
determined during healthy children follw-up. But
however, characteristic skin lesions are generally missed.
In this case report, we try to emphasize the importance
of physical examination.
Keywords: tuberosclerozis, tachyarrhythmia,
rhabdomyoma
[P-180]
A case of hypocalsemic convulsion caused by
vit d deficiency
Nilay Kadakal1, Ayşegül Doğan Demir1, Ufuk Erenberk1,
İlker Tolga Özen2, Türkan Uygur Şahin3
1
Bezmialem Vakıf University,Department of
Pediatrics,Istanbul,Turkey
2
Bezmialem Vakıf University,Department of Pediatric
Endocrinology,Istanbul,Turkey
3
Bezmialem Vakıf University,Department of Pediatric
Neurology,Istanbul,Turkey
Background&Aims: Nutritional Rickets is the most
important result of vitamin d deficiency in childhood.
It typically presents between four months and three
years of age and associated with prolonged breastfeeding without vit d supplementation. Vitamin D deficiency
presents with medical conditions such as growth disturbance,bone deformities,muscle weakness,hypoplasia
of teeth, susceptibility to infections and also can cause
serious complications such as hypocalsemic convulsion
Nutritional vitamin D deficiency is diagnosed by clinical manifestations and typical laboratory findings such
as hypocalcemia,hypophosphatemia,hyperparathyroidism,and low level of 25 hydroxyvitamin D.
Methods: A previously healty 10 -months –old -boy
has applied to our hospital for generalized tonic –clonic
convulsions.The patient had not received any vitamin d
supplementation and adequate nutritional intake of calcium and phosphorus after the birth. Laboratuvary test
have shown that calcium:5,9 phosphorus:3,5 Alp:829
Pth:412(15-65) 25 OH vit D <4 ng /ml, indicating hypocalcemia,hypophosphatemia,hyperparathyroidism,and
low level of 25 hydroxyvitamin D.In physical examination;we have found open and large fontanel,caput
quadratum deformity and kraniotabes.Hand-wrist radiograph showed metaphyseal hyperplasia,The patient was
diagnosed with nutritional rickets.He was treated with
vit D and calcium lactat replacement.After 3 days of hospitalization,the patient was discharged with normal clinical manifestations and laboratory findings
Results: Vitamin D is an essential nutrient that plays an
important role in calcium homeostasis and bone health.
Vitamin D deficiency has been associated with rickets, a
disease in which the bone tissue doesn’t properly mineralize, leading to soft bones and skeletal deformities and
also can cause serious complications such as hypocalsemic convulsion tetany and larnygospazm.
Conclusions: As a result;we present hypocalsemic convulsion in a 10 months old boy with vit d deficiency.This
case is an example of serious complications of nutritional
rickets as an important public health problem despite
vitamin d supplemantation is common in our country.
Keywords: hypocalcemia, vit d deficiency, rickets
[P-181]
An Important Cause Of Persistant Vomiting
In Childhood: Superior Mesenteric Artery
Syndrome
Tufan Kutlu1, Nisa Pekcan2, Hasret Ayyıdız Civan1
1
İstanbul Üniversitesi Cerrahpaşa Tıp Fakültesi Çocuk
Gastroenteroloji
2
İstanbul Üiversitesi Cerrahpaşa Tıp Fakültesi Çocuk
Sağlığı ve Hastalıkları
Superior Mesenteric Artery Syndrome (also known as
Wilkie’s syndrome) has many causes including high
insertion of the duodenum at the ligament of Treitz
a congenitally low origin of the superior mesenteric
artery and compression of the duodenum due to
peritoneal adhesions as a result of duodenal malrotation.
However,the most common cause is thought to be
narrowing of the aorta-mesenteric angle causing
extrinsic compression of the third part of the duodenum.
Herein, a 13 year-old male patient presented with
persistant vomiting and 15-kg weight loss during last 8
months who is diagnosed as superior mesenteric artery
syndrome is reported.
The patient was admitted to our out-patient clinic with
severe vomiting and weight loss. The patient had a
history of obesity who started a diet programme on
his own and lost 15 kilograms of weight during last 8
months. He had complaint of severe vomiting after each
meal. His physical examination was unmarkable except
cachexia. He did not have abdominal pain or tenderness.
In scope of this history we suspected superior mesenteric
artery syndrome. We performed double contrast barium
passage graphy which showed compression of first,
- 92 -
second and third part of duedonum. CT-angiography
scan had revealed elongation and increase in volume of
stomach. Besides dilatation of the first, second and third
part of duedenum. Distance and angle between superior
mesenteric artery and abdominal aorta was measured as
2 mm and 18 degrees, respectively. These findings were
compatible with superior mesenteric artery syndrome.
We have recommended to insert a nasojejunal tube,
however patient refused this treatment.
Conclusion: In general, when signs and symptoms of
upper gastrointestinal obstruction are accompanied with
rapid weight loss, superior mesenteric artery syndrome
should be considered. The most useful diagnostic tool
to detect superior mesenteric artery syndome is CT
anjiography scanning.
Keywords: superior mesenteric artery syndrome,
Wilkie syndrome
[P-182]
Neuro-Behçet’s Disease in Childhood
Nurcan Yusufoğlu, Burcu Karadaş, Dilşad Koca, Zehra
Esra Önal, Çağatay Nuhoğlu
Department of Pediatrics, Haydarpaşa Numune Training
and Research Hospital, Istanbul, Turkey
Introduction: Behçet’s Disease is a multisystemic
disease that commonly presents dermal, ocular, vascular,
nervous and joint involvements. The disease course has
remissions and attacks, and usually involves recurrent
oral aphthous ulcers. Behcet’s disease is most commonly
diagnosed between the ages 20-40. It rarely can be
diagnosed on early ages.
Case: 13-year-old male patient was admitted with a
headache that has a duration for a month and decreased
visual acuity. The eye exam demonstrated that he had
panuveitis, neuroretinitis and bilateral papileodema.
He had recurrent oral aphthous ulcers on his history.
Right transverse sinus thrombosis had been seen on the
cranial Magnetic Resonance Imaging (MRI) and Magnetic
Resonance Venography. Antitrombin 3, Protein C, Protein
S, Factor V Leiden, Prothrombin 20210, Methylene
tetrahydrofolate reductase (MTHFR) mutations has
been searched, results were negative. Pathergy test
result was positive. HLA-B51 was found as a result of
histological evaluation. With the data in hand the patient
was diagnosed with ‘Behçet’s Disease’ and he was sent
to Children Rheumatology Department for treatment
and follow-up.
Discussion: Our patient had recurrent oral aphthous
ulcers in his history. In our case panuveitis and
neuroretinitis were found. Severity of ocular involvement
and loss of vision are related to the early onset of the
disease and the patient’s gender being male. Vascular
involvement may occur at any kind of vein (but venous
is the most) that is commonly seen in male patients.
Our patient had right transverse sinus thrombosis.
Our patient’s pathergy test was positive. Behçet’s
Disease has no specific laboratory findings. HLA-B5 and
HLA-B51 positivity can be demonstrated. Treatment is
symptomatic.
Conclusion: Prognosis for Behçet’s Disease is worse in
young ages and males. Diagnosis at early stages of the
disease and monitoring with multi-disciplinary approach
have a great importance for the prognosis of the disease.
Keywords: Neuroretinitis, Oral ulcers, Thrombosis
[P-183]
Fanconi Anemia with VACTERL association
Nurhan Aruçi Kasap1, Fatma Betül Çakır2, Gözde Yeşil3,
Fatma Özgüç1, Mine Yüksel1
1
Bezmialem Vakıf Üniversity Medical Faculty,
Paediatrics, Turkey/İstanbul
2
Bezmialem Vakıf Üniversity Medical Faculty, Paediatric
Hematology and Oncology, Turkey/İstanbul
3
Bezmialem Vakıf Üniversity Medical Faculty, Medical
Genetics, Turkey/İstanbul
Introduction: Fanconi aplastic anemia (FA) is a disease
that effects several systems. There is a risk for bone
marrow deficiency, physical abnormalities, organ defects
and some cancers. At least three of VACTERL syndrome
features
(vertebral
abnormalities,
anal
atresia,
cardiovascular abnormalities, tracheoesophageal fistula,
renal abnormalities, extremity defects) are seen in 5%
of these patients. An 8-year-old female patient who was
diagnosed with FA and determined to have VACTERL
association is being presented in this case report.
Case Report: The 8-year-old female patient, who
presented to our clinic with seizures, was examined and
found to have pancytopenia. With history of esophageal
atresia, operated tracheoesophageal fistula, operated
anal atresia, prenatal hydrocephalus-VP shunt, renal
agenesis, bilateral external auditory canal agenesis,
patient’s examination demonstrated absence of a thump,
clinodactilia, micrognathia, triangular-shaped face, lowset ear, toe abnormalities, labial synechia. Karyotype
analysis and DEB/MMC test were requested for suspected
FA. Result of the karyotype analysis was reported as normal
but DEB/MMC test was consistent with FA. Requiring
continuous transfusions, the patient was diagnosed with
transfusion-dependent FA and was referred to another
healthcare facility for bone marrow transplantation.
Discussion – Conclusion: VACTERL association was
found in our patient due to presence of anal atresia,
tracheoesophageal fistula, renal agenesis and extremity
defects. Thirty-three similar cases were found in the
literature. Our patient presented to our clinic with
pancytopenia when she was 8 years old. 24% of the
cases reported had also hematologic complications
during the first decade.
In the literature review who had FA diagnosis with
VACTERL association has 33% hypo-hyper-pigmentation,
76% renal abnormalities, 55% microphthalmiai/
dysmorphism, 24% genital abnormalities, 67%
growth/developmental
retardation,100%
extremity
abnormalities, 70% anal/duedenal atresia, 52% cardiac
defect, %49 tracheoesophageal fistula/atresia, %30
hydrocephallus which our patient also had.
In conclusion, we believe that it would be appropriate to
evaluate patients who had FA diagnosis about VACTERL
association.
- 93 -
Keywords: fanconi aplastic anemia, VACTERL
association
[P-184]
Experiences on Neonatal Care After Fetal
Surgery: First Cases of Turkey
Nursu Kara1, Huseyin Canaz2, Ibrahim Alatas2, Kerem
Ozel3, Ali Gedikbasi4
1
Department of Neonatology, Florence Nightingale
Hospital, Istanbul Bilim University, Istanbul, Turkey
2
Department of Neurosurgery, Florence Nightingale
Hospital, Istanbul Bilim University, Istanbul, Turkey
3
Department of Pediatric Surgery, Florence Nightingale
Hospital, Istanbul Bilim University, Istanbul, Turkey
4
Department of Perinatology, Kanuni Sultan Suleyman
Training and Education Hospital, Istanbul, Turkey
Myelomeningocele (MMC), one of the most common
congenital malformations, can result in severe lifelong
disabilities, including paraplegia, hydrocephalus, Chiari II
malformation, incontinence, sexual dysfunction, skeletal
deformations and mental impairment. MMC was the first
nonlethal anomaly to be treated by fetal surgery. Fetal
surgery was performed currently for several hundreds of
human fetuses in North America and also for some fetuses
in Brazil, Poland, Spain and Switzerland. Since preterm
labor is one of the most common complications of fetal
surgery, neonatologists must be aware of problems that
can be faced off in these neonates. Fetal surgery requires
a multidisciplinary team and neonatologist is one of the
most important members. Our aim is to present the first
two cases of Turkey and discuss the period in neonatal
intensive care unit (NICU). Procedures were performed at
respectively 25+2 and 25+4 weeks of gestation. Fetuses
were delivered at 31+1 and 31+3 weeks of gestation
and stayed 36 and 34 days respectively in NICU. Fetal
surgery which is currently performed only in six centers
in Europe, is a new procedure for our country. It is also
a new point of interest and challenge for neonatologists.
Keywords: Fetal surgery, myelomeningocele,
neonatology
[P-185]
Jarcho Levine Syndrome: Experience on
Neonatal Care
Nursu Kara1, Huseyin Canaz2, Sukran Yildirim1, Banu
Yazici3, Kerem Ozel4, Ibrahim Alatas2
1
Department of Neonatology, Florence Nightingale
Hospital, Istanbul Bilim University, Istanbul, Turkey
2
Department of Neurosurgery, Florence Nightingale
Hospital, Istanbul Bilim University, Istanbul, Turkey
3
Department of Pediatrics, Florence Nightingale
Hospital, Istanbul Bilim University, Istanbul, Turkey
4
Department of Pediatric Surgery, Florence Nightingale
Hospital, Istanbul Bilim University, Istanbul, Turkey
Jarcho-Levin
syndrome
(JLS)
is
a
segmental
costovertebral malformation. It is a congenital disorder
consisting of various vertebral and costal anomalies.
Incidence is 0,25/10.000. Its main clinical signs are
asymmetric chest wall, short neck-trunk stature
scoliosis, and multiple radiological vertebral and related
rib defects with mild thoracic restriction. It is composed
of two subgroups: Spondylothorasic and spondylocostal
dysostosis. Treatment requires earlier respiratory
support and later orthopedic or surgical management.
This syndrome is usually diagnosed in newborns. Spina
bifida appears to be a common finding in reported JLS
cases. The aim of this study is to report five cases with
severe spondylocostal dysostosis and myelomeningocele
and to discuss the complications in neonatal period. All
the patients underwent surgery for myelomeningocele
repair in the first 24 hours of life. Mean lenghth of stay in
neonative intensive care unit was 16 days. Two patients
died because of respiratory complications and one patient
died due to cardiac complications. Congenital heart
defects, abdominal wall malformations, urogenital and
anal abnormalities, multiple skeletal anomalies, upper
limb anomalies, spina bifida, and inguinal, umbilical and
diaphragmatic hernias can be seen as components of
JLS. Contents of JLS must be considered in neonates
with myelomeningocele and spondylocostal dysostosis.
Because JLS constitutes a challenge in neonatal care.
Keywords: Jarcho Levine Syndrome,
Myelomeningocele, Neonatal Care
[P-186]
A Newborn With Hemitruncus Arteriosus;
Case Report
Osen Ari1, Aras Tulunoglu1, Helen Bornaun2, Gökhan
Büyükkale3, Abdullah Yahya Heybeci1, Murat Şahin2,
Kazım Öztarhan2
1
Kanuni Sultan Suleyman Hospital, Pediatrics
Department,,Istanbul, Turkey
2
Kanuni Sultan Suleyman Hospital, Pediatrics
Cardiology, Istanbul, Turkey
3
Kanuni Sultan Suleyman Hospital, Neonatalogy,
Istanbul, Turkey
Introductıon: Wilson described the term Hemitruncus
Arteriosus in 1798 as a congenital heart disease
characterized by a single great artery that leaves the
base of the heart giving rise to the coronary, pulmonary
and systemic arteries. This term has fallen out of use, but
it refers to a rare anomaly in which one pulmoner artery
branch, usually the right, arises from the ascending aorta
just above the aortic sinuses while the main pulmoner
artery and the other pulmoner artery branch arise in
their normal positions.
Case Description: A 2-day-old male neonate delivered
at term was referred to us due to poor feeding. He was
tachypneic and tachycardic, his physical examination
revealed no cardiac murmur. Chest X-ray showed
cardiomegaly (cardiothoracic index was 0.60) with
increased pulmonary arterial circulation. ECG was
normal and right axis was dominant. Transthoracic
Echocardiography revealed right pulmonary artery
was arising from aorta approximately 1 cm from aortic
valve leaflet and main pulmonary artery was continuous
with the left pulmonary artery. Bifurcation was absent.
Echocardiography also showed 7 mm ASD secundum.
Discussion: In patient with anomalous origin of one
pulmonary artery from the aorta, the affected lung is
perfused at systemic pressure and remaining lung is
- 94 -
exposed to the entire output of the right ventricle. As the
pulmonary resistance decreases in the neonatal period,
the blood pressure and flow increases in the anomalous
pulmonary artery causing pulmonary over circulation
and pulmonary hypertension. As the mortality is very
high in the first year of life early repair is mandatory to
prevent development of irreversible pulmonary vascular
disease.
Conclusion: Hemitruncus arteriosus is an uncommon
congenital
cardiac
malformation
accounting
for
approximately %0.7 of all complex congenital heart
lesions. Early diagnosis and pre- operative planning with
management of pulmonary hypertension are necessary
for successful outcome.
Keywords: hemitruncus, congenital heart disease,
truncus arteriosus
drug on RBC surface and auto-antibody production
resulting in IgG and/orIgM (C3d comploment) positivity
in direct antiglobulin test. The most common causes
of drug induced HA are penicillin and cephalosporins,
non-steroidal anti-inflammatory and anti-neoplastics.
In reported cases ceftriaxone is the most common drug
which causes drug- iduced hemolytic anemia.
Conclusion: Ceftriaxone- induced hemolytic anemia is
rare but potentially fatal condition. In sick children who
hospitalized for ceftriaxone treatment, hematocrit values
and direct coombs testing must be evaluated closely for
early diagnosis of drug-induced hemolytic anemia.
Keywords: drug-induced hemolytic anemia,
ceftriaxone
[P-188]
Guillian Barre Syndrome with CMV IgM
Positivity
[P-187]
Ceftriaxone-Induced Immune Hemolyitic
Anemia; Case Report
Osen Ari1, Çağatay Günay1, Kenan Toprak1, Gökçen
Kamış1, Özlem Başoğlu Oner1, Gönül Aydoğan3, Rengin
Şiraneci2
1
Kanuni Sultan Suleyman Hospital, Pediatrics, İstanbul,
Turkey
2
Kanuni Sultan Suleyman Hospital, Pediatric Infectious
Disease Department, İstanbul, Turkey
3
Kanuni Sultan Suleyman Hospital, Pediatric
Hematology and Oncology İstanbul, Turkey
Introduction: Drug-induced hemolytic anemia (HA) is
very rare state in childhood. Second and third generation
of cephalosporins are the most common cause of drug
induced HA.
Case Description: A nine year–old boy with down
syndrome was treated with ceftriaxone (100 mg/kg/
day) for acute gastroenteritis and pneumonia. While
receiving ceftriaxone treatment, patient had developed
anemia signs and symptoms. Hemolytic transfusion
reaction to ceftriaxone was suspected because of the
marked dropped in hematocrit value in the abscence
of any overt clnical bleeding. The patient’s hematocrit
value was 14% when patient deterirorated. Laboratory
studies showed decrease in hemoglobin values from
14 gr/dl to 5 gr/dl, increase in LDH values to 476IU/L
and (++++) direct coombs testing. Further evaluation
revealed hemolysis on peripheral blood smear which
demonstrated spherocytosis and polichromasia. He was
treated with intravenous methyil-prednisolone 2mg/kg/
day, IVIG 1gr/kg for once, transfused with erytrocyte
suspension, ceftriaxone was discontinued and antibiotic
therapy was switch to vancomycin and clindamycin
intravenously. The patient was discharged on the 30th
day of the hospitalization with oral methtyl-prednisolone
therapy, hematocrit value was 36%, direct coombs was
evaluated (+) and C-reactive protein found negative.
Discussion: There are three widely recognized
mechanisms proposed to explain drug induced positive
direct antiglobulin tests (diect coombs testing) and
immune hemolytic anemia. Drug adsorption mechanism
(hapten induced), immuncomplex formation with the
Ozge Gucuyeter1, Tamay Gürbüz2, Behçet Şimşek3,
Narin Akıcı4, Çiğdem Sağ5, Çağatay Nuhoğlu6
1
Özge Gücüyeter
2
Tamay Gürbüz
3
Behçet Şimşek
4
Narin Akıcı
5
Çiğdem Sağ
6
Çağatay Nuhoğlu
Guillian-Barre Syndrome (GBS) is a postinfectious
polyneuropathy involving mainly motor nerves. The
initial diagnosis is based upon the clinical presentation
and confirmed with albuminocytologic dissociation in
cerebrospinal fluid and acute motor axonal polyneuropathy
on electromyography. Plasmapheresis and intravenous
immunoglobuline are the main treatment modalities.
We present a case of Guillian-Barre Syndrome treated
with both IVIG and plasmapheresis. A 16 year old female
patient admitted with pain and weakness of legs and
gait difficulty. On physical examination generalized
muscle weakness of lower extremities were noted. In
her blood tests CMV IgM was positive. Lumbar puncture
was performed and albuminocytologic dissociation
was detected. Acute motor axonal polyneuropathy
in lower extremities was demonstrated on EMG and
Guillian-Barre syndrome was diagnosed. IVIG was
administered 1 gr/kg for two days. On the follow up
the patient had progressive muscle weakness in spite
of IVIG therapy and plasmapheresis was performed.
Following seven sessions of plasmapheresis the patient
markedly recovered from muscle weakness and was
discharged on her twenty fourth day of hospitalisation.
Data from the available small open-label randomized
trials in children suggest that IVIG shortens the time
to recovery compared with supportive care alone. In a
meta-analysis of six trials and 649 patients with GBS
that included a small number of children who were all
>=10 years of age, treatment with plasma exchange
was superior to supportive care. In our case, the patient
had progressive muscle weakness and pain despite
IVIG therapy. We performed plasma exchange on the
fourteenth day of onset symptoms. After seven sessions
of plasma exchange, the patient had remarkably less
muscle weakness and pain. In conclusion, in the cases
of Guillian-Barre Syndrome which are nonresponsive to
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intravenous immunoglobulin therapy with progressive
muscle weakness, plasmapheresis can be performed as
a superior alternative choice of therapy.
Keywords: guillianbarre, plasmapheresis
[P-189]
Three cases of anorexia nervosa with
different presentations
Övgü Kul Çınar1, Çiğdem Oruç1, Yağmur Birsev1, Oya
Ercan2, Müjgan Alikaşifoğlu3
1
İ.Ü Cerrahpasa Medical Faculty Department of
Pediatrics, Istanbul, Turkey
2
İ.Ü Cerrahpasa Medical Faculty Department of Pediatric
Endocrinology, Istanbul, Turkey
3
İ.Ü Cerrahpasa Medical Faculty Department of
Pediatrics, Adolescent Health Care, Istanbul, Turkey
Background: Anorexia nervosa (AN) is a serious eating
disorder characterized by abnormal eating behaviour,
an intense fear of gaining weight, a distorted body
image and psychiatric comorbidities. Anorexia nervosa
often begins during the pre-teen or teen years or young
adulthood and it is estimated to occur ten times more
common in females.
Cases: We present here three cases of AN who had
long-term follow up in our adolescent health care clinic.
All of them were females and they were; case 1:17
years 5 months, case 2:15 years, case 3:17 years 6
months). Only one of the patients admitted to hospital
with a complaint of being extremely preoccupied with
her appearance. The two other patients’ complaints were
completely different at their admission. One of them
admitted with constipation and the other with bone pain
affecting both lower limbs. All three of the patients had
secondary amenorrhea (7 months, 6 months, 6 months
respectively), gastrointestinal symptoms as constipation
and dyspepsia.
While taking psychosocial history, we noticed that all
three patients had distorted body image, they were
on diet and perception of their weight had powerful
influence on their self-evaluation, so we considered AN
for diagnosis and consultated with psychiatry clinic.
Their body mass indexes which are important to show
the severity of disease were under 5 percentile. All the
patients had vitamin B12, vitamin D and folate deficiency.
One of the patients had decreased bone mineral density
(Z score: -2,6).
Results: For early diagnosis of AN, inquiring the body
perception and eating behaviours must be a part of the
routine evaluation of adolescents especially the ones
whose BMI is under 5 percentile, who is on a diet or use
other methods to lose weight, who has weight loss more
than ten percentile of their body weight.
Keywords: Anorexia nervosa, Distorted body image,
Psychosocial history
[P-190]
Coexistence of immune thrombocytopenic
purpura with cobalamin deficiency
Özden Aksu Sayman, Zehra Esra Önal, Burcu Karadaş,
Dilşad Koca
Haydarpasa numune education and research
hospital,istanbul,turkey
Immune
thrombocytpenia
(ITP)
of
childhood
is
characterized
by
isolated
immune-mediated
thrombocytopenia.
(peripheral
blood
platelet
count<100,000/microl).It is an acqired and most
commonly benign disorder.Nutritional megaloblastic
anemias were chacterized over one hundred years ago
with macroovalocytic red cells.The megaloblasts are
products of impared DNA formation,due to deficiencies of
cobalamin or folate. One year-aged male child was admitted
to our clinic with the symptoms of petechiae,purpura
and ecchymosis on the trunk.His physical examination
was normal except hepatosplenomegaly.Laboratory
studies; hemoglobulın:8.24g/dl, mean corpuscular
volüme:82,5fl platelet count: 28x103 /µl cobalamin:
83pg/ml.The other whole blood examination were
within normal limits.Examination of peripheral blood
smear revealed normochromic,macrocyctic erythrocyte
morphology,
hypersegmented
neutrophils
and
single-large platelets without clustered. We couldn’t
examine bone marrow aspiration (no family consent).
Thrombocytopenia related to immun thrombocytopenic
purpura responsed to the treatment of intravenosus
gamaglobulin,since cobalamin levels was lower than
normal.So related with megaloblastic anemia, coexisted
with ITP.We presented this case because of this
coincidental presence of these two disorders.
Keywords: cobalamin deficiency, ITP, megaloblastic
anemia
[P-191]
Pulse oximetry screening for critical
congenital heart disease in healty newborns
before discharge
Hacer Ergin, Özmert Muhammed Ali Özdemir, Özgün
Taban, Ceren Çıralı, Merve Korkut
Pamukkale University, Faculty of Medicine, Department
of Pediatrics, Division of Neonatology, Denizli, Turkey
Background and Aim: Congenital heart disease (CHD)
occurs in approximately 8 out of 1000 live births. About
1-1.8 babies per 1000 live births have a duct dependent
circulation, with a persistent ductus arteriosus being
necessary for survival. A sizeable proportion of critical
CHD (CCHD) is not detected by routine neonatal
examination prior to discharge. Standard and portable
echocardiograms are effective in identifying CCHD but
are too costly to be used as a routine screening method.
The aim of this study is to evaluate the use of pulse
oximetry for early detection of CCHD.
Methods: This study included neonates born in
Pamukkale University Hospital and followed in well
baby nurseries. Newborns, prenatally diagnosed with
duct dependent circulation and admitted to neonatal
intensive care unit, were excluded. Oxygen saturation
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screening was conducted preductally (palm of right
hand) and postductally (either foot) with identical pulse
oximetry. When both preductal and postductal oxygen
saturations were <95% or the difference between the two
measurements was >3%, the newborn was considered to
be screening positive. Newborns with abnormal cardiac
findings such as murmurs, tachypnea, overt cyanosis,
and had three repeated positive measurements were
supposed to have an echocardiogram.
Results: Of the 859 newborns eligible for the screening
study, 657 (76.4%) had complete data from both pulse
oximetry and physical examination. Of the 657 infants
with complete screening data, 8 (1.2%) had positive pulse
oximetry results, and 65 (9.8%) had abnormal cardiac
findings. While diagnostic echocardiogram showed
patent foramen ovale in 6 infants whose pulse oximetry
results were positive, none had ductus dependent CCHD.
Conclusion: Pulse oximetry screening is an effective,
noninvasive, and inexpensive tool allowing earlier
diagnosis of CCHD. Newborns with positive screen should
evaluate for diagnostic echocardiogram and pediatric
cardiology consultation.
Keywords: pulse oximetry, congenital heart disease,
newborn
[P-192]
An infant with Herpes Simplex Encephalitis:
A case report
Özlem Öçal Doğan1, Nihat Çelebi1, Pınar Yamaç Dilaver1,
Ece Gültekin2, Leyla Telhan3, Nalan Karabayır4, Güzide
Baysan Turanlı2
1
Medipol Univercity, Department of Pediatrics
2
Medipol Univercity, Department of Pediatric Neurology
3
Medipol Univercity, Department of Pediatric Intensive
Care Unit
4
Medipol Univercity, Department of Social Pediatrics
Background&Aims: Herpes simplex encephalitis (HSE)
is the most common cause of viral encephalitis in infants and
children and it is a devastating disease that can be difficult
to diagnose in its early stages. Treated or untreated, it can
be associated with considerable morbidity and mortality.
In this report, we present a case of herpes simplex
encephalitis, 4,5 month-old male patient, who admitted
to our emegency department with fever, diarrhea and
focal seizures. He had bulging fontanelle, focal tonicclonic seizures on the left arm, no eye contact. Other
physical examinations, blood count, biochemical and
stool tests were all normal. HSV was subsequently
detected by PCR in the cerebrospinal fluid (CSF).
MRI scan demonstrated features consistent with
meningoencephalitis on temporal lobe involvement.
Periodic lateralising epileptiform discharges in the
right temporal lobe and abnormal background activity
on the right hemisphere were EEG findings. High-dose
intravenous(IV) ceftriaksone and acyclovir were initiated
immediately. The antiepileptic treatment decreased
the frequency of seizures. The patient who completed
7 days of ceftriaksone and 21 days of antiviral therapy
were discharged on day 32. The patient is periodically
checking by neurology and psychological and educational
rehabilitation.
Conclusions: In conclusion, herpes virus infections
are often asymptomatic in the infancy period. An
infant who presents with fever, altered mental state
(encephalopathy) and focal seizures or focal neurological
abnormalities, we should also consider HSV encephalitis.
Earlier antiviral therapy is associated with a better
outcome, which is very important in reducing morbidity
and also mortality.
Keywords: fever, seizure, herpes simplex encephalitis
[P-193]
Orbital cellulitis in a forty-five day old baby
Yelda Türkmeoğlu1, Elif Arslan1, Pınar Bütün1, Sibel
Aşkın Perkin1, Vefik Arıca2, Gülşen Köse3
1
İstanbul Okmeydanı Eğitim ve Araştırma Hastanesi
Çocuk Sağlığı ve Hastalıkları
2
İstanbul Yeni Yüzyıl Üniversitesi Tıp Fakültesi
Gaziosmanpaşa Hastanesi Çocuk Sağlığı ve Hastalıkları
3
İstanbul Şişli Hamidiye Etfal Eğitim ve Araştırma
Hastanesi Çocuk Sağlığı ve Hastalıkları
Preseptal cellulitis is a mild infection of the structures
in front of orbital septum characterized by the edema,
tenderness and erythema of the eyelid. Whereas orbital
cellulitis is the infection of adnexial tissues composed of
the fat and muscle, behind the orbital septum, in addition
to the signs of preseptal cellulitis, there is proptosis
and limitation of ocular movements. In addition to loss
of vision, it has life- threating complications, such as
intracranial infections and cavernous sinus thrombosis.
For this reasons its urgent differential diagnosis from
preseptal cellulitis and treatment are crucial. Although
orbital cellulitis is more common in children than in
adults, it is very rare in babies younger than six - month.
In this article a case of orbital cellulitis, whom was
treated with appropriate antibiotic therapy without any
complication, in a 45-day old baby is reported.
Keywords: infant, newborn, orbital cellulitis
[P-194]
Successful Hyperbaric Oxygen Treatment of
Bilateral Refractory Gangrenous Lesions due
to Systemic Lupus Erythematosus
Pınar Özge Avar Aydın1, Çiğdem Aktuğlu Zeybek1,
Ertuğrul Kıykım1, Akın Savaş Toklu2, Ozgur Kasapçopur3
1
Department of Pediatric Nutrition and Metabolism,
Istanbul University Cerrahpasa Faculty of Medicine,
Istanbul, Turkey
2
Department of Underwater and Hyperbaric Medicine,
Istanbul University Faculty of Medicine, Istanbul, Turkey
3
Department of Pediatric Rheumatology, Istanbul
University Cerrahpasa Faculty of Medicine, Istanbul,
Turkey
Background&Aims: Necrotizing vasculopathy is a
relatively uncommon and life-threatening condition
in children. Raynaud phenomenon and digital
ischemia are the most common symptoms. Although
immunosuppressive treatments in combination with
vasodilators are the first chosen therapy methods
hyperbaric oxygen therapy is also used as primary or
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[P-196]
adjuvant therapy in some.
Case: We report a case of 15 years-old female patient
who presented with gangrenous lesions at her feet
phalanges and hyperglycemia had been diagnosed
as diabetes mellitus type 1 and systemic necrotizing
vasculitis due to systemic lupus erythematosus. Effective
treatment by hyperbaric oxygen therapy in combination
with immunosuppressive and vasodilator drugs with
supporting therapies led to significant improvement in
gangrenous lesions without amputation.
Conclusion: Adjunctive hyperbaric oxygen therapy
should be considered in cases with progressive
gangrenous lesions due to vasculopathies.
Keywords: Hyperbaric oxygen treatment, systemic
necrotizing vasculitis, systemic lupus erythematosus
[P-195]
Different Clinical Spectrum of Leptospirosis
Fatma Deniz Aygün, Pınar Özge Avar Aydın, Haluk
Çokuğraş, Yıldız Camcıoğlu
Division of Pediatric Infectious Diseases, Clinical
Immunology and Allergy, Istanbul University
Cerrahpasa Faculty of Medicine
Background&Aims: Leptospirosis is a prevalent
zoonotic disease. Human infection usually occurs through
exposure to environmental sources. Clinical course of
leptospirosis is variable.
Cases: We presented five patients, aged between 4-14,
having a history of contact with rodents and symptoms
7-10 days after contact. The first three cases were
relatives and had contact with dead rats after applying
insecticides to bakery opposite to their house. The first
case diagnosed as isolated meningitis, others as flulike illness. The fourth case had a contact history with
a rat inundate in the canalization and diagnosed as
acute hepatitis. The last case, living in a village with
poor sanitation, developed secondary hemophagocytic
syndrome. ELISA was performed for diagnosis. High
dose penicillin and additional immunsuppressive drugs
for the last case were used. All cases showed recovery
within 10 days.
Conclusion: Leptospirosis should be considered in any
patient presenting with an abrupt onset or prolonged
fever, myalgia, headache and jaundice.
Keywords: leptospirosis, meningitis, hemophagocytic
syndrome
Peripherally Inserted Intravenous Catheters
in Children: One Year Survelliance
Fatih Aygün1, Fatma Deniz Aygün2, Pınar Özge Avar
Aydın1, Halit Çam1
1
Istanbul University, Cerrahpasa Medical Faculty,
Pediatric Intensive Care Unit, Istanbul, Turkey
2
Istanbul University, Cerrahpasa Medical Faculty,
Department of Pediatric Infectious Diseases, Istanbul,
Turkey
Aims: Intravascular catheters are used with increasing
frequencies in the management of children. The aim
of this study is to evaluate the complications and risk
factors associated with intravascular catheterization.
Methods: Between the years 2014-2015 Augusts, in
Medical Faculty of Cerrahpasa, University of Istanbul,
Pediatric Intensive Care Unit, 105 patients and placed
171 intravascular catheters analyzed retrospectively by
the patients’ medical records.
Results: Average age of catheter insertion was
4,45±5,48 years, ranging between 3 days-228
months. 78 of the patients (45.6%) were female, 93
(54.4%) were male. One hundred and fifteen catheters
(67.3%) were used 7 days or more, the average
length of usage was 20.33±38.80. The most common
underlying disease was metabolic disease (n = 43,
25.1%). Catheters were inserted most often because
of the absence of a peripheral venous route (n = 77,
45%). The most common catheter types were central
venous catheter (n = 110, 64.3%) and hemodialysis
catheters (n = 51, 29.8%), respectively. Ninety eight
femoral (57.3%), 48 jugular (28.1%) and 22 subclavian
(12.9%) catheters were placed. Hundred and seven of
the catheters (62.6%) were used only in intensive care
unit, 30 (17.5%) in services after intensive care unit
and 34 catheters (19.9%) were placed for the usage in
services. Catheter-related infections were observed in
13 patients (7.6%). Noninfectious complications were
observed in 6 cases (3.5%) during or after insertion.
Ninety five catheters (55.6%) withdrew due to no more
remaining requirement. As catheter type, size and
location had no risk for catheter related infections (p>
0.05), increased time length for the usage of catheters
showed a significant increase in infection incidence (p
<0.05). The frequency of infection of catheters followed
in services was significantly higher (p <0.05).
Conclusions: The insertion and care of catheters by
skilled persons reduce complication rates.
Keywords: Children, intravascular catheter, catheter
complications
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[P-197]
[P-198]
Two Cases with Systemic Trombosis as a
Complication of Intraarterial Chemotherapy
for Retinoblastoma
Pınar Özge Avar Aydın1, Ahmet Sarıcı2, Osman Kızılkılıç3,
Tiraje Celkan1
1
Istanbul University, Cerrahpasa Medical Faculty,
Department of Pediatric Hematology-Oncology,
Istanbul, Turkey
2
Istanbul University, Cerrahpasa Medical Faculty,
Department of Ophthalmology, Istanbul, Turkey
3
Istanbul University, Cerrahpasa Medical Faculty,
Department of Radiology, Istanbul, Turkey
Background&Aims: Retinoblastoma (Rb) is the most
common intraocular tumor in childhood. Conventional
treatment methods include systemic chemotherapy,
enucleation, external beam radiotherapy and focal
therapies, such as cryotherapy, thermotherapy, laser
photocoagulation. Intraarterial chemotherapy (IAC)
for advanced and refractory Rb has become popular
recently in an attempt to avoid enucleation and systemic
complications. Common minor ocular side effects
occur in many cases and can resolve spontaneously
within a few months. Common systemic complications
include neutropenia, bronchospasm and allergy. Local
complications at femoral puncture side are possible.
Systemic vascular complications are rare. Herein,
we report two cases with Rb in which extraocular
complications after IAC with melphalan.
Cases:A 2,5 months old boy was diagnosed as bilateral
Rb. The patient underwent local cryotherapy and
systemic chemotherapy. Because of his refractory Rb
in his left eye IAC with melphalan was performed. Two
days after the first IAC, the patient was admitted with
confusion and emesis. On his physical examination, there
was right hemiplegia and hemiparesis. Cranial CT scan
and MRI were performed and revealed acute ischemic
lesion in the territory of the right middle cerebral artery.
He was given parenteral low-molecular-weight heparin
for 14 days. Hemiplegia didn’t resolve completely and
he was not given second cycle of IAC. Second case is
a 7 months old boy with unilateral Rb. He was given 3
cycles of IAC. After the third IAC, he was admitted with
a painful, blue left toe. He was given 14 days of both oral
aspirin and low-molecular-weight heparin due to blue
toe syndrome. After 2 weeks, the apperance of left foot
was totally normal.
Conclusions: IAC is milestone of the treatment of
refractory and advanced Rb in children. It may cause
systemic vascular complications which are rare but
serious. Patients may be analysed for tendency to
trombosis and systemic vascular complications should
be defined.
Keywords: retinoblastoma, intraarterial chemotherapy,
trombosis
Juvenile Dermatomyositis: Clinical Features,
Laboratory Findings, Treatment Modalities
and Disease Course (A Single Center
Experience From Turkey)
Özgür Kasapçopur1, Pınar Özge Avar Aydın2, Kenan Barut1,
Salim Çalışkan3, Lale Sever3, Nil Arısoy1
1
Department of Pediatric Rheumatology, Istanbul
University Cerrahpasa Medical Faculty, Istanbul, Turkey
2
Department of Pediatrics, Istanbul University
Cerrahpasa Medical Faculty, Istanbul, Turkey
3
Department of Pediatric Nephrology, Istanbul
University Cerrahpasa Medical Faculty, Istanbul, Turkey
Introduction:
Juvenile
dermatomyositis
(JDM)
is the most prevalent idiopathic inflammatory
myopathy of childhood and characterized by proximal
muscle weakness and typical skin involvement.
Objectives: To describe demographics, clinical features,
laboratory findings and treatment modalities of patients
with juvenile dermatomyositis (JDM) at a referral
pediatric rheumatology center in Turkey.
Methods: Retrospective review of forty three patients
meeting the Bohan and Peter criteria diagnosed as JDM
at the Pediatric Rheumatology Department of Istanbul
University Cerrahpasa Medical Faculty between the years
2003-2013.
Results: Forty three patients were identified; 30 of
them (69 %) were female. Mean age for the onset of
the disease was 6.3, mean age for the diagnosis was
6.9 years. Mean follow-up period was 48 months. As
the most common clinical presentations, heliotropic
rash and Gottron papules were seen in 42 patients (97
%), muscle weakness was seen in 39 patients (90 %),
erythroderma in 36 patients (87 %) and calcinosis in
16 patients (37 %). All patients had elevated muscle
enzymes at the diagnosis. Twenty seven patients (62
%) had anti-nuclear antibody (ANA) positivity. Muscle
biopsy was performed on 14 patients (32 %) and all of
them showed histopathological changes of inflammatory
myositis. Electromyography was performed on 25
patients (58 %) and all of them recorded myopathy.
All patients had been treated with corticosteroids at
different dosages. Methotrexate was used in 41 patients
(95 %), cyclosporine was used in 16 patients (37 %).
All patients except two cases, who were referred to
our clinic after a long duration of the disease and had
widespread calcinosis, achieved remission.
Conclusion: Our patients with JDM have similar
characteristic findings compared with previously
described cohorts. Awareness about JDM diagnosis and
treatment should be increased among pediatricians.
Keywords: Juvenile dermatomyositis, muscle
weakness, skin rashes
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[P-199]
[P-200]
Pınar Özge Avar Aydın1, Bahar Özcabı2, Aydilek
Dağdeviren Çakır2, Oya Ercan2, Olcay Evliyaoğlu2
1
Cerrahpasa Medical Faculty of Istanbul University,
Department of Pediatrics, Istanbul, Turkey
2
Cerrahpasa Medical Faculty of Istanbul University,
Department of Pediatric Endocrinology and Diabetes,
Istanbul, Turkey
Background&Aims: The evaluation of the patients
diagnosed with growth hormone deficiency and treated
with growth hormone for at least one year and the
analysis of the factors affecting growth rates.
Nalan Karabayir1, Dogukan Aktas2, Kazim Oztarhan3,
Emre Kelesoglu1, Abdullah Erdem2, Helen Bornau3, Pinar
Yamac Dilaver1
1
Pediatrics Department, Faculty of Medicine, Istanbul
Medipol University, Istanbul, Turkey
2
Pediatric Cardiology Department, Faculty of Medicine,
Istanbul Medipol University, Istanbul, Turkey
3
Pediatric Cardiology Department, Kanuni Sultan
Suleyman Training and Education Hospital, Istanbul,
Turkey
Dilated cardiomyopathy, which mostly has an
idiopathic etiology or is caused by genetic inheritance
or infection, may cause irreversible congestive heart
failure. Hypocalcemia is a rare etiology of reversible
dilated cardiomyopathy. Cardiogenic shock related to
hypocalcemic cardiomyopathy is a rare event. Here we
report two cases with congestive heart failure who was
diagnosed as having dilated cardiomyopathy secondary to
hypocalcemia. After calcium and vitamin D replacement
therapy, the patients showed improvement in left
ventricular function. Hypocalcemia should be included in
the differential diagnosis of dilated cardiomyopathy.
Keywords: cardiogenic shock, dilated cardiomyopathy,
vitamin D deficiency
Growth Hormone Therapy in Childhood: A
Single Center Experience
Methods: Patients diagnosed with growth hormone
deficiency at the Pediatric Endocrinology Department of
Cerrahpasa Medical Faculty of Istanbul University between
the years 2008-2014 and treated with growth hormone
for at least one year were analyzed retrospectively.
Results: One hundred and eight patients were identified;
55 of them (50,9%) were female. Mean age at the
admission was 9,72±3,50. Six patients were treated
with GH diagnosed with multiple hypophyseal hormone
deficiency, 9 patients with Turner Syndrome, 6 patients
with rheumatic diseases and 11 patients with other
diagnoses. Mean responses were 3,63±2,31 ng/ml on
levodopa and 4,91±2,58 ng/ml on clonidine tests. Forty
six patients (42,6%) were diagnosed as complete and 62
(57,4%) as partial GHD. Sella MRI was obtained normal
in 81,5% of the patients. Thirty one of the patients
(28,7%) were pubertal. The most commonly seen side
effects were as follows: hyperglycemia (14%), elevated
muscle enzymes (10,3%) and hyperinsulinemia (10,3%).
The highest height velocity was detected during the first
year (8,29±2,55 cm) and decreased gradually during
following years. Mean delta boy SDS was 0,90±0,89.
Genetic consistency was medium (-1,62±1,50) before
GH therapy while good (-0,68±1,16) after GH therapy,
respectively.
Conclusions: Growth hormone therapy is safely used
in children with increasing frequency. The highest height
velocity was detected at the first year of the therapy.
There are several factors affecting growth velocity and
the effectiveness of the therapy may show individual
differences. In this study, the most important factors
affecting growth response were age and height SDS
before the treatment. The importance of early diagnosis
and treatment were desired to be emphasized with these
results.
Keywords: Growth hormone, growth hormone
deficiency, children
Dilated Cardiomyopathy due to Vitamin D
Deficiency
[P-201]
Bilateral Uretheral Stones Mimicking
Hemolytic Uremic Syndrome
Cihangir Akgun1, Ayse Serap Akgun2, Mehmet Gunduz3,
Pinar Yamac Dilaver3, Vahit Guzelburc4, Meliha Aksoy3,
Mesut Okur3
1
Pediatric Nephrology Department, Faculty of Medicine,
Istanbul Medipol University, Istanbul, Turkey
2
Radiology Department, Faculty of Medicine, Istanbul
Medipol University, Istanbul, Turkey
3
Pediatrics Department, Faculty of Medicine, Istanbul
Medipol University, Istanbul, Turkey
4
Urology Department, Faculty of Medicine, Istanbul
Medipol University, Istanbul, Turkey
A four years old boy was admitted to our hospital with
total anuria and facial swelling which started one day ago.
Except mild facial edema, he had no pathologic findings on
his physical examination including costovertebral angle
tenderness. Bilateral grade 1 renal parenchymal disease,
grade 2 hydronephrosis have been reported and no stone
has been identified on his ultrasonographic evaluation
of urinary tract. Initially, the patient was diagnosed as
hemolytic uremic syndrome due to the history of bloody
diarrhea, acute renal failure, thrombocytopenia and
anemia so hemodialysis was performed immediately, by
the way laboratory examination was continued. There
was no sign for microangiopathic hemolytic anemia at
peripheral blood smear and other laboratory values were
normal. Then, for the possibility of obstructive pathology
computed tomography was performed and bilateral
uretheral stones were seen. After double j stent have
been placed to both renal pelvis due to fluoroscopy by
urologist, urine output was normalized and the patient
- 100 -
has been discharged. Bilateral uretheral obstruction due
to stone is a very rare condition in children, underlying
reasons have to identify. Non-contrast computed
tomography can be helpful especially in non-opaque
stone.
Keywords: bilateral renal stone, anuria, hemolytic
uremic syndrome
[P-202]
Nutcracker Syndrome in Siblings Without
Hematuria
Cihangir Akgun1, Ayse Serap Akgun2, Mehmet Gunduz3,
Gizem Ondalikoglu3, Pinar Yamac Dilaver3, Mesut Okur3,
Arda Ozyuksel4
1
Pediatric Nephrology Department, Faculty of Medicine,
Istanbul Medipol University, Istanbul, Turkey
2
Radiology Department, Faculty of Medicine, Istanbul
Medipol University, Istanbul, Turkey
3
Pediatrics Department, Faculty of Medicine, Istanbul
Medipol University, Istanbul, Turkey
4
Department of Cardiovascular Surgery, Faculty of
Medicine, Istanbul Medipol University, Istanbul, Turkey
Introduction: Nutcracker syndrome (NCS) refers to
compression of LRV (left renal vein) between aorta
and superior mesenteric artery (SMA). The patients
have symptoms such as hematuria, proteinuria, and
left flank pain. The following frequency of symptoms
was noted: 78.57% for haematuria, 38.39% for left
flank pain, 35.71% for varicocele in males, 30.36%
for proteinuria and 13.39% for anaemia. Isolated
hematuria was reported 33.3% in children with
NCS. Microhematuria is 4 times more common than
macrohematuria.Orthostatic proteinuria is another
common symptom in NCS. Here, we present two new
cases of NCS in a young male person and his sister.
Case 1: 15 years old female patient was admitted to
our outpatient clinic with proteinuria that detected while
swimming pool registery tests. Only proteinuria was found
on urinalysis. In the history of the patient, kidney biopsy
was performed for proteinuria before refferred to our
hospital and the result was normal. MR of the abdomen
was requested for further investigation of proteinuria.
A diagnosis of nutcracker syndrome was established.
Case 2: 18 year-old male patient whose sister was
diagnosed as NCS. His blood tests were normal and
only proteinuria was found on urinalysis. Bilateral renal
artery MR performed. MR eventually revealed pressed
left renal vein due to compression between the superior
mesenteric artery (SMA) and the abdominal aorta,
consistent with NCS. The patient had also left varicocele.
Discussion: Eventough, NCS is frequently associated
with hematuria,it may present with only proteinuria
without hematuria. NCS must be considered in different
diagnosis of isoleted proteinuria. We report two cases
of family members with NCS. It is important in terms of
being the first case that seen in siblings, according to
our knowledge.
Keywords: nutcracker, siblings, wıthout hematuria
[P-203]
Seizure and loss of consciousness in an
adolescent: mitochondrial disease
Püren Özüdoğru1, Özlem Sert1, Nilgün Erkek2
1
Akdeniz University Faculty of Medicine, Department of
Pediatrics, Antalya
2
Akdeniz University Faculty of Medicine, Division of
Pediatric Emergency, Antalya
Background&Aims: A seizure represents the clinical
expression of abnormal, excessive, synchronous
discharges of neurons, which makes transient changes
in behavior, movement and sensation for a short time.
The etiology of seizures is multifactorial, one of these
is thromboembolism. The aim of this case report is
to indicate that mitochondrial diseases can lead to
thromboembolic episodes, which can come up with
seizures and changes in consciousness.
Methods: A 17 year old boy with seizure and acute
loss of consciousness hospitalized in the department of
pediatrics. His glasgow coma scale was 8 and laboratory
investigations were normal. The neurological status
came to normal at the 12th hour of treatment, except
for the articulation deficit, which he had also before.
His cranial MR-screening showed restricted diffusion
at the right parietooccipitotemporal area, indicating
thrombembolism. His genetic screening for thrombophilia
was MTHFR 677- heterozygote mutant. MR spectroscopy
showed lactate changes at the area of thromboembolism,
suggesting MELAS. We performed genetic analysis for
mitochondrial diseases, in which %95 of the responsible
genes were studied and none of them were mutant.
Results: There are multiple risk factors for
thromboembolism in children (anatomic, genetic and
acquired). In our case we found it interesting that the
patient had MTHFR 677 gene heterozygote mutant and
he had lactate changes in MR spect.
Conclusions: This report highlights the role of
mitochondrial diseases in the etiology of thromboembolism
and seizure
Keywords: seizure, thromboembolism, MELAS
[P-204]
A severe case of neonatal hypotonia
Raffaella Panza1, Annalisa Resta1, Alessandro Nicola
Abbaticchio1, Donatella Capodiferro1, Nicoletta Resta2,
Lucrezia De Cosmo1, Nicola Laforgia1
1
Department of Neonatology and NICU, University of
Bari, Italy
2
Department of Human and Medical Genetics, University
of Bari, Italy
“Floppy infant” is an hypotonic newborn who may
present respiratory distress at birth, low Apgar score,
weak sucking and swallowing.
The diagnosis is difficult in the neonatal period as it may
be related to numerous conditions.
An accurate neurological examination, the assessment
of Precthl’s General Movements, the study of
spontaneous motility of passive and active muscle
- 101 -
tone and primitive reflexes, may help understand
if hypotonia is due to central or peripheral causes.
MRI, US, laboratory tests, specific tests such as
muscle biopsy, EMG, genetic tests are usually required.
A newborn, born at 42+4 weeks of GA by vaginal delivery,
was admitted to our UTIN because of perinatal asphyxia
and required therapeutic hypothermia and ventilatory
support for one month; the patient died on the 30th DOL.
She presented severe generalized hypotonia, no
spontaneous movements of the limbs, inappropriate
suck-swallow pattern, no deep tendon reflex.
The mother reported reduced fetal movements and
polyhydramnios.
Brain MRI was normal, Total Body Rx revealed multiple
spontaneous fractures of tibia and femur, EMG showed
absence of spontaneous and induced motility, and
muscle biopsy showed non-specific signs of myopathy.
Karyotype and genetic test for Prader-Willi were normal.
Molecular test for Spinal Muscular Atrophy (SMA)
revealed a non-funcional point mutation of the
SMN1 gene and only one copy of the SMN2 gene.
SMA I with neonatal onset, named SMA 0, always carries
a fatal prognosis. It is usually due to an omozygotic
mutation of the SMN1 gene. Moreover, copy-number
variation of the SMN2 gene seems to be linked to the
disease onset: the lower the number of copies (e.g. 1
or 2) the worst both clinical presentation and prognosis.
The association of a SMN1 gene non-functional mutation
with a single copy of the SMN2 gene could explain the
clinical severity and negative rapid outcome, similar to
SMA 0.
Keywords: floppy infant, hypotonia, SMA
[P-205]
Meningitis with a history of head trauma: a
case report
Emine Olcay Yasa, Refia Gözdenur Savcı, Müferet
Ergüven
İstanbul Medeniyet University Göztepe Training and Research
Hospital, Pediatrics Department, İstanbul, Turkey
Introduction: Meningitis is an inflamatory disease of
pie and arachnoid membranes surrounding the brain and
spinal cord. Meningitis is a medical emergancy and the
possibility of meningitis increases after head injury and
skull fractures. This may be recurrent and causing agent
is often S. pneumoniae.
Case: A 8 year old previosly healthy female child
presented with the complaints of fever, headache,
drowsiness, diplopia and altered consciousness.Her
mother told that she had stared at ceilling and hadn’t
responded to any stimulus. She was feverish and had
a poor general appearance. Neurologic examination
revealed nuchal rigidity. Because of her ongoing confusion
we had a cranial CT scan. It showed us the asymetry
of lateral venticles. In laboratory findings, WBC:33500/
mm3, %84 NEU, Hb:13,9 gr/dl, Hct:43%, PLT:251000/
mm3, CRP:7,8 mg/dl.There was contrast enhancement
in leptomeningeal area in cranial MRI. There was no
papilledema in fundus examination. We performed a
lumbar puncture and found that WBC: 12/mm3 ( %80
Lymph), Erythrocyte: 2/mm3, Glu: 53 gr/dl (SBG: 80
gr/dl), protein 47 mg/dl, Na: 140 mEq/lt. We started
Ceftriaxon for empirically and mannitol due to increased
intracranial pressure. In detailed history of the patient
we learned that she had fallen from the first floor and
had got an epidural hematoma and a skull fracture two
years ago. Therefore we added vancomycine to avoid s.
pneumoniae. The patient’s fever dropped, neurological
symptoms were improved, her antibiotics were
completed and she was discharged. In follow up visit we
found that she had a CSF fistula in MR cisternography.
We directed her to brain surgery department to eveluate
for operation.
Conclusion: Meningitis is the one of the most common
infectious disease of childhood. A detailed history would
help to eluciate the underlying cause and to avoid
recurrency.
Keywords: head trauma, skull fracture, meningitis
[P-206]
Perforated appendicitis: is it a challenging
diagnosis in children?
Gülser Esen Besli1, Refia Gözdenur Savcı1, Müferet
Ergüven1, Çiğdem Ulukaya Durakbaşa2
1
İstanbul Medeniyet University Göztepe Training and Research
Hospital, Pediatrics Department, İstanbul, Turkey
2
İstanbul Medeniyet University Göztepe Training and Reseach
Hospital, Pediatric Surgery Department, İstanbul, Turkey
Introduction: Acute appendicitis is the most common
non-traumatic emergency surgery in childhood. Its
mortality and morbidity are very low when a prompt
diagnosis and an early treatment are carried out.
Delayed diagnosis can cause severe complications. This
report highlights two cases of perforated appendicitis
with severe clinical presentations having malignancy
suspicion initially.
Case 1: A 16 year old male presented with a three
week history of abdominal pain and weight loss. He
was referred from another institution with diagnosis of
abdominal tumour. He looked unwell, apathetic, and
had fever and a tender pelvic mass. An abdominal
ultrasonography reported a solid mass lesion located
behind the bladder. An abdominal CT showed an abcess
which started supravesical pelvic region and extanding
rectovesical recess. He was operated on for perforated
appendicitis and a large pelvic appendiceal abscess was
drained.
Case 2: A ten year old girl had admitted to a hospital
with a four week history of nausea, vomitting, abdominal
pain, and respiratory distress. She referred to our hospital
with a suspicion of malignancy due to pleural effusion,
ascites, hepatomegaly and weight loss. She looked
unwell, cachectic, and had tachypnea, dyspnea, and
distended abdomen. His breath sounds were decreased
especially in the right hemithorax. She had diffuse
abdominal tenderness. An abdominal CT showed located
collections in perihepatic and perisplenic areas and a
fecalith in the appendix. There were bilateral atelectasis
and pleural effusion in the right lung on thorax CT. She
was operated on for perforated appendicitis, abdominal
absess and generalized peritonitis.
- 102 -
Discussion: Delayed diagnosis of acute appendicitis
can cause life-threatening conditions such as abdominal
abscess and sepsis. Extraabdominal complications like
pleural effusion and empyema may occur. It should
also be considered that childhood appendicitis may
mimic pelvic malignancy. We emphasize the importance
of early treatment for acute appendicitis and septic
complications.
Keywords: perforated appendicitis, abdominal absess,
malignancy
[P-207]
One of the major causes of pleural effusions:
Tuberculosis
Emine Olcay Yasa, Refia Gözdenur Savcı, Müferet
Ergüven, Şeyma Sönmez Şahin
İstanbul Medeniyet University Göztepe Training and Research
Hospital, Pediatrics Department, İstanbul, Turkey
Introduction: Tuberculosis(TB) is an infectious disease
caused by Mycobacterium tuberculosis Even if it is a
preventable and curable disease TB remains as one of the
most common causes of mortality and morbidity worldwide
Case: A 13-year-old female child presented with the
complaints of high grade fever, cought and back pain
for the last 3 weeks. She had received several courses
of antibiotics without any response. Thoracentesis
had been performed to her grandmother because of
pulmonary infection but she didn’t live in the same house.
She had showed good general state of health. Height and
weight were within limits compatible with the patient’s
age. She was feverish. Respiratory system eveluation
was suggestive of a right sided pleural effusion. A chest
radiograph and thorasic ultrasonography confirmed
the pleural effusion and atelectatic areas in the right
hemithorax. CBC and biochemical analysis were normal
but acute phase reactants were significantly higher.
Rheumotologic markers were evaluated to exclude
rhemotologic diseases. We started empirical antibiotic
therapy. Thorasentesis was performed by interventional
radiologists. In laboratory findings of samples; it was
pale yellow, cell count was 3440 Leukocyte/mm3 ( %90
Lymphocyte), 3600 Erytrocyte/mm3 and biochemical
analysis revealed that Glu: 93 mg/dl, Cl: 107 mEq/L,
protein 5,4 gr/dl, ADA 40,9 U/L. Microbiological
examination of fluid was normal. A Tuberculin skin
test (TST) performed with a markedly positive result
after 72 hours. All family members were screened in
tuberculosis dispansaries. Because of lack of clinical
benefit from antibiotic therapy in last 3 weeks, positive
TST, characteristics of pleural fluid examination, and
suspicious family history we diagnosed as pleural TB and
started antiTB treatment including İsoniazid, Rifampin
and Pyrazinamide. The patient’s fever dropped, her
respiratory symptoms were improved and she was
discharged. Periodic follow-up visits are ongoing.
Conclusion: TB should be kept in mind in expecially
feverish and antibiotic refractory cases.
Keywords: tuberculosis, pleural effusions
[P-208]
Multiple avascular necrosis in a pediatric
antiphospholipid syndrome secondary to
systemic lupus erythematosus - clinical case
Rodica Eremciuc, Prof. Ninel Revenco, Tatiana Macovei
Pediatric Department, State University of Medicine and
Pharmacy “Nicolae Testemitanu”, Chisinau, Republic of
Moldova
Background&Aims:
Avascular
necrosis
(AVN)
occurs in several chronic illnesses, including systemic
lupus erythematosus (SLE). The mechanism about
why and how AVN occurs isn`t unraveled yet. AVN
in the pediatric lupus population is understudied.
Methods: We report a case of a 15-year-old girl, who was
on record in the Rheumatology Clinic over the last year
with the diagnosis of SLE with multiorgan involvement
associated with secondary antiphospholipid syndrome
(immunologically confirmed by the presence of positive
ANA, anti-dsDNA, antiphospholipid and anticardiolipin
antibodies). The exacerbation of the articular
syndrome 9 months after onset of disease required a
bilateral CT scan of the coxofemoral and knee joints.
Results: Clinical examination of the child 9 months
after onset of disease showed the exacerbation of the
inflammatory articular syndrome expressed by manifest,
prolonged, treatment-resistant pain in the coxofemoral
(R>L), bilateral knee and left talocrural joints (8090 mm along VAS). Swelling of the both knees and
both talocrural joints was determined on the clinical
assessment. On paraclinical examination persisted
increased inflammation acute phase reactants. X-ray
revealed erosions and signs for lacunar osteoporosis in
the knees and coxofemoral joints. The CT-scan of the
coxofemoral joint showed a marginal fracture of the
right capitis femoris (the fragment size 16x28mm) with
dislocation of 2mm. Knee CT-scan presented bilateral
AVN of femoral condyles, with signs of pathologic fracture
at these levels. Further therapeutic management of this
case is being realized in a multidisciplinary team, that
consists of pediatric rheumatologists and orthopeds.
Conclusions: AVN can cause significant morbidity. No
single etiology is known to cause the interruption of
blood supply which is the common pathway for all AVN;
rather, AVN is likely the end result of a multifactorial
process. Considering the age of this patient, periodic
follow-ups with an orthopedic specialist are essential for
a good long-term outcome.
Keywords: Avascular necrosis, Secondary
antiphospholipid syndrome, Systemic lupus
erythematosus
[P-209]
Mixed Germ Cell Tumor In Down Syndrome
Ruhsar Erdoğmuş, Muhammed Nurullah Yakut, Süheyla
Ocakçı, Hilal Şen
İstanbul Okmeydanı Eğitim Araştırma Hastanesi
Introductıon: Down syndrome (trisomy 21) is the
most common and best known chromosomal disorder in
- 103 -
humans. Only a few cases of testicular germ cell tumor
in Down Syndrome (DS) children have been reported in
literature. We present here an adolescent boy with DS
and testicular mixed germ cell tumor.
Case: 15-years-old boy with DS referred to our outpatient
clinic due to a left testicular mass. He has not been on
regular follow up for DS. A painless lump on the left testis
has been first noticed about one month ago. He had no
history of criptorchidism. Physical examination revealed a
5x6 cm, painless, hard, well-defined left testicular mass.
Right testis was normal. Serum AFP was 752 ng/ml (N
< 7 ng/ml) and B-HCG was 275 miu/ml (N < 0,5 miu/
ml). Scrotal ultrasonography (USG) revelaed a 63x41
cm left testicular hypervascular mass with scattered
microcalcifications. Abdominal USG and chest x-ray were
normal. Left orchiectomy with high inguinal ligation was
performed. Pathologic examination was reported as
mixed germ cell tumor ( embriyonal carcinoma 50%,
yolk sac tumor 30%, immature teratoma 20%), with
negative surgical margins. Post-operative serum AFP,
B-HCG levels, scrotal USG, abdominopelvic MRI and
Thorax CT were all normal. He was considered as Stage
I, testicular mixed germ cell tumor. He was planned to
be on close follow up with no additional treatment.
Conclusions: Children with DS have a significantly (1020 fold) increased risk of hematologic malignancies. But
incidence of solid tumors in DS has been proposed to be
lower than the general population. Only a few testicular
solid tumors (mainly seminomas) were reported in DS
patients in older ages ( >16 years). As it is not clear
whether the incidence of testicular solid tumors are
different than the normal population, we aimed to report
this rare association.
Keywords: Down, Malıgnancies
[P-210]
Phenothypic Variations in Long QT Syndrome
Patients; Single Center Experience
Rukiye İrem Yekeler, Elif Neslihan Tuzcu, Celal Akdeniz,
Mehmet Karacan, Volkan Tuzcu
ISTANBUL MEDIPOL UNIVERCITY
Background&Aims: Long QT syndrome is the most
common hereditary arrhytmia syndrome that can cause
sudden cardiac death. With predominantly autosomal
dominant inheritance, not only index cases but also
asymptomatic members of the family have risk of sudden
cardiac death. Same genetic mutation can lead different
phenotypic presentation and QTc values, so detecting of
asymptomatic carriers with EKG-QTc analysis may be
incapable.
Methods: Study based on regarding index cases and
genetically diagnosed family members, between March
2013-November2015. With the aim of viewing phenotypic
variations clearly; Jervell Lange Nielsen cases, overlap
syndrome cases and LQTS cases that have more than
one type of mutations are not included. Median QTc,
minimum QTc, maximum QTc values are detected
between family members with the same mutation.
in genetically diagnosed 68 LQTS cases. OTc in index
cases was 426-700 (median 472), min and max QTc
values are 403-479 and 432-700 respectively. Nine of
these patients were symptomatic (7 of them were index
cases), 4 index cases had ICD implantation. %56.5 of
these cases were LQTS 1, %17.3 were LQTS 2, %21.7
were LQTS 3, %4.3 was LQTS 8.
Conclusions: Genetic test has a critical role in diagnose
of hereditary arrythmia sydnromes, especially screening
for asymptomatic family members. Existance of
phenotypic diversity in family members having the same
genotype; makes genetical test particularly important.
Keywords: Long QT syndrome, genetic test,
phenotypic variations
[P-211]
Spontaneous Pneumothorax in a Term
Neonate
Rukiye Taş, Tuğrul Atay, Emel Ataoğlu
Department of Pediatrics, Haseki Education ve Research
Hospital, ıstabul, Turkiye
Spontaneous pneumothorax is a recognised and rare
cause of respiratory distress in the neonatal period.
Here, we report a case of symptomatic spontaneous
pneumothorax associated with congenital lung disase
which needed intercostal drainage.
A 2760 gr, 18 days old male infant was admitted with
respiratuar distress. He was vaginally delivered at term.
Physical examination revealed tachypnea, subcostal
and intarcostal retractions. Patient had an incision
scar of a thorax tube on the left side of his chest. His
medical history revealed a hospitalisation for 15 days
due to spontaneous pneumothorax in neonatal period
An urgent Chest X ray was ordered which revealed a
left sided Pneumothorax with mediastinal shift to the
opposite side. ABG showed respiratory acidosis. (pH –
7,29 PCO2 – 58 mmHg). An intercostal drainage tube
was immediately placed on the left chest. Check X ray
confirmed the position of the tube which functioned well.
The patient was started on amphicillin and cephotaxime.
CT Thorax showed left sided pneumothorax with 4 lung
cyts two of which were ruptured. Since there is an
association between lung cycts and cardiac and renal
abnormalities, we performed echocardiography and
renal ultrasonography which were normal.
Antibiotics were stopped after 14 days and control
X-ray showed a normal lung paranchyme, except for
the 2 cysts. He has a normal growth and development
with no respiratory distress. He has been on a regular
follow up in chest surgery outpatient clinics since then.
In conclusion, although symptomatic spontaneous
pneumothorax is a rare condition in term newborns, it
should always be kept in mind while evaluating a new
born with severe respiratory distress for early recognition
and treatment is life saving.
Keywords: neonatal, Spontaneous Pneumothorax
Results: Twenty two different mutations were found
- 104 -
[P-212]
[P-213]
Fatma Betül Çakır1, Rumeysa Tuna2, Ayşegül Doğan
Demir2, Faruk Öktem3, Bülent Zülfikar4, Rejan Kebudi4
1
Bezmialem Vakif University, Department of Pediatric
Hematology-Oncology, Istanbul
2
Bezmialem Vakif University, Department of Pediatrics,
Istanbul
3
Bezmialem Vakif University, Department of Pediatric
Nephrology and Rheumatology, Istanbul
4
Istanbul University, Cerrahpaşa Medical Faculty and
Institute of Oncology, Pediatric Hematology-Oncology,
Istanbul
Background&Aims: Posttransplant lymphoproliferative
disorder (PTLD) is a group of disease that occur after
solid organ or hematopoietic stem cell transplantation
associated with immunosuppression and Epstein Barr
Virus (EBV) viral load, and characterized by atypical
lymphoid and plasmositik cell proliferation. We present a
successfully treated case about this rare complication of
organ transplantation results in high mortality.
Sairah Akbar, Hilary Pearce
Royal Hospital for Sick Children, Glasgow
Background&Aims: Croup is common, affecting up
to 3% of children.¹ Although there is little evidence
for using more than 0.15 mg/kg of dexamethasone, in
practice, higher doses are often used for patients who
remain symptomatic. In this audit, factors influencing
the decision to give more than a standard dose were
reviewed.
Lymphoproliferative disorders after kidney
transplant: A succesfully treated lymphoma
case
Methods: Six-year-old girl was diagnosed with chronic
kidney failure. Cadaveric kidney transplantation was
performed after two years. Recipient’s EBV viral load
was negative before transplantation. After a year from
transplantation under intensive immunosuppression, mass
was detected with routine abdominal ultrasonography.
Magnetic resonance examination showed great masses
in the right quadrant of the abdomen that have made
paraaortic and pelvic lymph node involvement. True-cut
biopsy from the mass resulted EBV positive diffuse large
B Cell Lymphoma.
Results: Reducing the immunosuppressive treatment
of the patient was only allowed to take steroids.
After six cycles Anti-CD20 monoclonal antibody
rituximab the mass decreased by 60%. Treatment
with low dose chemotherapy; six cycles Vincristine and
cyclophosphamide was added to therapy. At the end of
this treatment 80% answer was seen. After four more
cycles of rituximab complete remission was seen in
the PET taken. The transplanted kidney function has
remained normal.
Conclusions: Lymphoproliferative disease development
in pediatric patients after renal transplantation comes
after heart-lung, heart and liver transplant with 2.4%.
Early discontinuation of immunosuppressive therapy is
the most effective treatment. Conventional standarddose chemotherapy can be mortal and monoclonal
antibodies are used as first-line therapy. In patients with
slow response to that therapy low-dose conventional
chemotherapy can be used.
Keywords: Posttransplant lymphoproliferative disorder,
Renal transplantation, Epstein Barr Virus
High dose dexamethasone, to give or not
to give. A review of croup management in a
tertiary Accident and Emergency department
Methods: A retrospective audit was performed of
attendances with croup between October 2013 and
January 2014.
Results: In total 403 patients were identified. 64%
were defined as mild,² with signs present only when
upset or active. 34% had moderate croup (symptomatic
at rest) and the remaining 2% were moderate/severe.
Ninety four percent of patients received dexamethasone.
In total 19 (4.7%) were given more than 0.15 mg/kg of
dexamethasone. 83% were given an additional dose or
‘top up’; the remaining 17% were given higher doses
following initial assessment.
When topping up, 80% were given a total of 0.3 mg/kg,
13% were given 0.6 mg/kg and 7% were given >0.6
mg/kg. The commonest reason cited was persistence of
stridor in 40%; in 13% a further dose was given for
worsening recession and stridor. A clear reason was not
cited in the remainder. The average timing for a further
dose was 2.6 hours. On further analysis, all patients
given more than 0.15 mg/kg were classed as moderate
croup with none of those moderate/severe given higher
doses.
Twenty one patients (5.2%) were given adrenaline
nebulisers. Of those given nebulisers, 6 patients (29%)
were given more than a standard dose of dexamethasone:
83% were given 0.3 mg/kg and one patient was given
0.6 mg/kg.
Conclusions: This audit has shown variation in
management for patients with croup who remain
symptomatic. Further studies are recommended to
determine best practice.
Keywords: ‘Top up’ dose i.e. additional dose,
Additional dose i.e. further dose
- 105 -
[P-214]
[P-215]
A Case Of Disseminated Fasiolazis
Sare Betül Kaygusuz1, Ayşegül Doğan Demir1, Selçuk
Uzuner1, Issam Cheikh Ahmad2, Özden Türel3, Faruk
Öktem4
1
Department of Pediatrics,Bezmialem Vakıf University,
İstanbul,Turkey
2
Department of Radiology,Bezmialem Vakıf University,
İstanbul,Turkey
3
Department of Pediatric Infectious Disease,Bezmialem
Vakıf University, İstanbul,Turkey
4
Department of Pediatric Nephrology,Bezmialem Vakıf
University, İstanbul,Turkey
Background: Fascioliasis is a trematode infection caused
by Fasciola hepatica and commonly acquired by eating
metacercaria encysted on leaves. İt has been reported
frequently to be found from the bile ducts of patients
who were operated with the diagnosis of cholecystitis.
But it is often neglected despite its common occurrence
in endemic areas.Here, we report a case of fasciolosis
without gastrointestinal complaints who was presented
as urticaria and resulting in successful treatment without
any sequelae.
Case: A 6 year old boy complained of swelling and
redness in hands and feet for 3 months was referred
to us for the evaluation of rheumatic diseases.His
developmental history and physical examinations was
unremarkable. Complete blood count revealed marked
eosinophilia (11.760 μ/lt) which led to the suspicion of
a parasitic infection. Serological examination revealed
borderline titres for echinococcus.Stool exams were
negative for Fasciola ova. Abdominal ultrasonography
showed solid, hypoechoic lesions in the liver suspicious
for malignancy.Therefore magnetic resonance imaging
(MRI) of abdomen and brain were performed. Numerous
hyperintense lesions in the liver were suggestive of
fascioliasis.Cranial MRI revealed 4 mm subcortical
hyperintense lesion in the left parieto occipital area.
Fasciola antigen was found to be positive.Diagnosis
of fasciolasis was based on eosinophilia, serology and
mostly the characteristic findings on MRI. After the first
dose of triclabendazole the symptoms disappeared. MRI
scan of liver and brain done one month after therapy
showed complete resolution. On follow-up fasciola
antigen was found negative.
Conclusions: The diagnosis of fascioliasis consists
of detection of eggs in stool samples,serological tests
and imaging.If detected at early stage,can be treated
successfully without any sequelae. There for a high
index of suspicion is important, especially because of the
clinical manifestations are nonspecific and progression of
the disease can cause liver failure and even death.
Keywords:
fascioliasis,
eosinophilia,
radiological
diagnosis of parasitosis
Henoch Schoenlein purpura on the basis
of familial Mediterranean fever: Recurrent
intussusception requiring surgical correction
Seda Sönmez1, Seçil Arslansoyu Çamlar1, Alper Soylu1,
Osman Zeki Karakuş2, Mehmet Atilla Türkmen1, Salih
Kavukçu1
1
Department of Pediatrics, Medical Faculty, Dokuz Eylül
University, İzmir,Turkey
2
Department of Pediatric Surgery, Medical Faculty,
Dokuz Eylül University, İzmir, Turkey
Introduction: Henoch Schoenlein purpura (HSP) is
the most common vasculitis of childhood, and it mainly
affects skin, joints, gastrointestinal tract and the kidneys.
Familial Mediterranean fever (FMF) is a recessively
inherited periodic fever syndrome characterized by
recurrent polyserositis attacks. Several types of
vasculitis are associated with FMF; the most frequent
one is HSP, which occurs in about 5% of FMF patients.
Case Report: An 8-year old girl presented with
purpuric rash on the extensor surfaces of lower
extremities and on the buttocks, arthralgia in the left
knee and intermittent abdominal pain. She has been
using colchicine for 4 years due to FMF which was
diagnosed clinically (recurrent fever and abdominal
pain episodes) and genetically (heterozygous R202Q,
M680I, M694V mutations). The patient was diagnosed
as HSP and oral prednisolone treatment was prescribed
for abdominal pain in association with occult blood in
stool. Intussusception was suspected on the basis of
episodic vomiting attacks along with colicky abdominal
pain, and confirmed by plain abdominal radiography and
ultrasonography. Spontaneous resolution was followed
by two more recurrences. The third attack did not resolve
spontaneously and necessitated laparoscopic correction
of both ileoileal and jejunojejunal invaginations.
Discussion: Intestinal intussusception can occur in the
course of HSP and is attributed to edema and damage to
the vasculature of the gastrointestinal tract as a result
of the vasculitic process. Intussusception occurs in 0.7–
13.6% of children with HSP. Our patient had already
been diagnosed as FMF when she presented with clinical
findings of HSP and invagination. However, it has been
stated that the children of Mediterranean extraction who
have unusual and complicated clinical picture of HSP
should have genetic testing for FMF.
Keywords: Henoch- Shönlein Purpura,
Intussusception, Gastrointestinal İnvolvement
- 106 -
[P-216]
[P-217]
Mervan Bekdas1, Serkan Ozturk2, Buket Kara1, Seher
Acar1, Hulya Ozturk2
1
Department of Pediatrics, Abant Izzet Baysal University
Faculty of Medicine
2
Department of Pediatric Surgery, Abant Izzet Baysal
University Faculty of Medicine
Benzalkonium chloride (BAC) is a kind of skin and mucosal
membranes antiseptic. Concentration used for the skin
is harmful to the mucosal membranes, for this reason
it must be diluted. In this case study, an infant with
severe esophagitis after ingestion of BAC is reported.
A 2 months old male patient was admitted to hospital
for fever after vaccination. Paracetamol suspension was
proprosed, but the baby’s mother was administered her
baby with 5 ml of 10% BAC (zephiran TM) instead of
paracetamol accidentally. Agitation and vomiting began
after taking it. The baby was referred to our hospital.
On admission, he had a pulse rate of 168 bpm, blood
pressure of 80/58 mmHg, respiratory rate of 62/min and
body temperature of 37 °C. On physical examination
of the unrestlesness patient, hyperemia and increased
salivation were detected in oral mucosa and pharynx,
the other systems findings were unremarkable. The
hemogram revealed hemoglobin: 9.9 g/L, hematocrit:
27.5%, WBC: 9.9x109/L, and platelets:447x109/L.
Serum chemical values were within the reference range,
fecal occult blood was negative. There was no abnormal
finding on the chest radiography and arteriel blood
gas values. Because of the edematous epiglottis and
vocal cords, endotracheal tube was inserted, assisted
ventilation (PEEP:5, PİP:20) was initiated. An endoscopic
examination, mucosal edema, hyperemia and fibrin
plaques in esophagus were revealed. Enteral feeding
was stopped. Total parenteral nutrition, sulbactamampicillin 100 mg/kg, amikacin 15 mg/kg of amikacin,
metilprednisolone 2 mg/kg and ranitidine 3 mg/kg
was begun. He was extubated and enteral feeding was
started on the fifth day. All medications were stopped
on the eighth day and discharged in the same day.
Esophageal stenosis was not detected on contrast
radiography which made at the end of the third week.
Consequently, parents should be very careful while
giving medication to children.
Keywords: Benzalkonium, esophagus, child
Fatih Demircioglu1, Bengu Ulku Tarhan2, Mervan
Bekdas1, Mustafa Erkocoglu1, Sevil Bilir Goksugur1,
Mustafa Dilek1, Seher Acar1, Elifnur Ildes1
1
Department of Pediatrics, Abant Izzet Baysal University
Faculty of Medicine
2
Department of Pediatrics, Duzce University Faculty of
Medicine
Infectious mononucleosis (IM) is presented as
adenopathies in the neck, swallowing difficulty
and
fever.
Mild
thrombocytopenia
accompanies
approximately more than 50% of patients with IM.
Pseudothrombocytopenia (PT) is a phenomenon in which
a low platelet count is due to the presence of antiplatelet
antibodies in blood that cause platelet clumping. It may
be seen in normal individuals or some disorders when
their blood samples are anticoagulated in a medium
containing ethylenediaminetetraacetic acid (EDTA).
A 9 year old male patient was admitted to our
outpatient clinics for the assessment of fever and neck
adenopathies. His parents denied any bleeding disorder
like epistaxis, melena, hematuria and easy bruising. His
platelet count was 36.000 /mm3 by electronic counting
method. Microscopic examination of peripheral blood
smears showed adequate numbers of platelets and
reactive lymphocytes typical of IM. Then, the diagnosis
of IM was confirmed by the presence of IgM against
Ebstein barr virus. In addition, when the blood count
was processed in a medium containing citrate instead
of EDTA, the platelet count was 152.000/ mm3.
PT is a condition in which the risk of bleeding, stroke or
the need for platelet transfusion are not increased. It can
be associated with many diseases. EDTA dependent PT is
the most frequent cause of PT. The misdiagnosis of the
thrombocytopenia can be prevented with the microscopic
inspection of blood smear. The finding of PT prevents
unnecessary patient examinations and therapeutic
interventions. In our case, the cause of PT was IM. False
severe thrombocytopenia is seen in this case and the
possibility of these situations must be taken into account
to avoid misdiagnosis and mismanagements.
Keywords: Pseudothrombocytopenia, Infectious
Mononucleosis
A bad result of carelessness: Esophageal
necrosis due to benzalkonium chloride
ingestion
An Atypical Cause Of
Pseudothrombocytopenia; Infectious
Mononucleosis
[P-218]
A Rare Diagnosis In Neonatal Period:
Lipoprotein Lypase Deficiency
Selen Baran1, Ertuğrul Kıykım2, Tanyel Zübarioğlu2,
Ayşe Çiğdem Aktuğlu Zeybek2
1
Istanbul University, Cerrahpasa Medical Faculty,
Department of Pediatrics, Istanbul, TURKEY
2
Istanbul University Cerrahpasa Medical Faculty,
Department of Pediatrics Division of Nutrition and
Metabolism, Istanbul, TURKEY
Lipoprotein lypase deficiency ( hyperlipoproteinemia
type 1 ) is a rare disorder which is inherited in an
autosomal recessive fasion. The course of the disease
consists of hyperlipidemia, chylomicronemia and related
- 107 -
clinical symptoms. This condition is mostly diagnosed
during childhood period with hepatosplenomegaly
accompanied with abdominal pain, pancreatitis attacks
and xanthomas. It is rarely diagnosed during adulthood.
Here we present a 5 days-old neonate who was
referred to our out-patient clinic with neonatal
hyperbilirubinemia. Her blood samples were lypemic and
laborotory tests revealed triglyceride levels as 5540 mg/
dl and total cholesterol levels as 426 mg/dl. According
to the results apo-c2 deficiency and lipoprotein lypase
deficiency considered in the differential diagnosis.
We performed 5 days of plasma replacement therapy
which consist of apo-c2, however laboratory results did
not decrease. Afterwards, 5 days of diet resulted in a
marked decrease in triglyceride and total cholesterole
levels which revealed lipoprotein lypase deficiency.
Lipoprotein lypase deficiency is rarely diagnosed
in neonatal period because symptoms can usually
be obvious after infancy. Coincidental diagnosis of
lipoprotein lypase deficiency in this case can make our
patient have less secondary complications of the disease
because of the early treathment.
Keywords: Lipoprotein lypase deficiency,
hypertriglyceridemia
[P-219]
A Not Fully Vaccinated Case:Whooping
Cough
Sema Bozkaya Yilmaz1, Erdal Sari2, Feyza Mediha
Yildiz3, Rumeysa Citli4, Handan Akoglu5
1
Sema Bozkaya Yilmaz,Zeynep Kamil Maternity
And Childrens Diseases Training And Research
Hospital,İstanbul,Turkey
2
Erdal Sari,Zeynep Kamil Maternity And
Childrens Diseases Training And Research
Hospital,İstanbul,Turkey
3
Feyza Mediha Yildiz,Zeynep Kamil Maternity
And Childrens Diseases Training And Research
Hospital,İstanbul,Turkey
4
Rumeysa Citli,Zeynep Kamil Maternity And
Childrens Diseases Training And Research
Hospital,İstanbul,Turkey
5
Handan Akoglu,Zeynep Kamil Maternity And
Childrens Diseases Training And Research
Hospital,İstanbul,Turkey
Introduction: Whooping cough is caused by gram
negative pleomorphic bacillus,Bordatella Pertussis.It
is a highly contagious and potentially life-threatening
respiratory tract illness. The illness can be diagnosed by
culture or PCR on a properly collected nasopharyngeal
swab.Here, we present a case of pertussis who was
unvaccinated because of her special condition.
Case: A 2-year-old female patient admitted to our
pediatric clinic with persistant cough with paroxysyms
lasting for 2 weeks.The patient presented to another clinic
1 week ago with the same complaints and was treated
with salbutamole nebules.She was not fully vaccinated
because of her atopic dermatititis. She was admitted
to hospital;chlarithyromycin and nebulized budesonide
therapy was started.Blood and nasopharyngeal swab
samples were tested for pertussis.After seven days of
hospitalization,her symptoms resolved. Nasopharyngeal
swab was positive for Bordatella Pertussis.
Discussion: Isolation of B.Pertussis in culture is the
gold standard for diagnosis.Direct fluoroscent antibody
testing of potential isolates using specific antibody
against B.Pertussis and B. Parapertussis maximizes
detection rates. Polymerase chain reaction analysis to
test nasopharyngeal wash specimens has a sensitivity
similar to that of culture and averts difficulties on isolation.
Unvaccinated refugees and other special conditions
resulting with unvaccination or delaying vaccination may
precipitate rare diseases.We present this case to remind
you pertussis in case of chronic cough,which is a rare
condition in fully vaccinated communities.
Keywords: Pertussis, Vaccination
[P-220]
Intracranial Hemorraghe With Herpes
Simplex Virus Encephalitis: A Case Report
Sermin Özcan, Ihsan Kafadar, Haşim Gencer, Nazan
Dalgıç
Şişli Hamidiye Etfal Eğitim ve araştırma Hastanesi,
Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalı, İstanbul
Herpes Simplex Virus (HSV)is the main cause of sporadic
viral encephalitis. HSV is characterized by causing
necrotizing hemorrhagic encephalitis on medial temporal
and frontal lobes. It is documented that HSV cause
intracerebral hemorrhage in infants and also in early
childhood. Virus causes encephalitis by spreading to
brain via peripheral nerves like trigeminal and olfactory
nerves.
Symptoms like headache, fever, seizure, unconsciousness
develop within a few days. Early diagnosis and acyclovir
treatment may reduce the mortality to %19 whereas the
mortality rate is approximately %70 in untreated child
patients.
Herpes simplex encephalitis (HSE) is the most common
cause of fatal acute sporadic encephalitis in older than
6 months children and adults. HSV has a ratio of %2-19
in whole encephalitis cases wheras it has a ratio of %20
-75 in whole necrotizing encephalitis cases. We prepared
this case to emphasize the importance of thinking HSE
as diagnosis in febrile encephalopathy patients with
focal neurologic signs with the acknowledgment of early
diagnosis and treatment’s positive effect on mortality,
also to determine problems encountered and the points
that need attention at diagnosis of HSE.
Keywords: herpes simplex virus, encephalitis,
intracranial hemorrhage
[P-221]
Congenital Nephrogenic Diabetes Insiipidus
Sermin Özcan, Nurver Akıncı, Zeynep Civelek, Merve
Şakar
Şişli Hamidiye Etfal Eğitim ve araştırma Hastanesi,
Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalı, İstanbul
Introduction: Congenital (primary) Nephrogenic
Diabetes Insipidus is a rare inherited disorder
- 108 -
characterized by insensitivity of distal renal tubule to the
effect of antidiuretic hormone (ADH), which may cause
growth and mental retardation. The clinical symptoms
can be different and hard to diagnose in infants and
newborns relatively older children.
Case: A-32 days old boy was brought pediatric emergency
room with fever, feeding problems and irritability. In
anamnesis, polyuria and polydipsia were present in
mother and mother’s family. In physical examination
lack of affinity to allimentation and affinity to water
were observed. 38.2°C of body temperature, dry skin
and sunken eyes were all observed pathological findings.
Hypernatremia (Na:157mmol/l) and low density of urine
(d:1001) were determined in blood and urine tests.
After hydration due to the hypernatremic dehidration
prediagnosis, high blood sodium level and low urine
density were obtained. Hydration was cut, and baby
was started to feed with only alimentaion, without extra
water. However high sodium level, dehydration and the
low urine density continued. His urinary and cranial
imagings were checked and no pathological evidences
were found. Desmopressin 6mg/kg/day was given as a
treatment and a small increase was obtained in urine
density however hypernatremia and polyuria continued
and central cause of diabetes insipidus was put a side.
Indometasine 2mg/kg/day was started and labatory test
results bacame normal and polyuria stopped. It was
understood that the reason of diabetes insipidus was
nephrogenic causes.
Result: It is aimed with this case, Congenital Nephrogenic
Diabetes Insipidus should be the differential diagnosis of
the patients with hypernatremic dehidration.
Keywords: diabetes insipidus, hypernatremic
dehydration, congenital
[P-222]
Association Between Urinary Bisphenol A
Levels And Body Weight Among Children In
Our Clinic
Sevinç Gümüş1, Ayşe Kılıç1, Ismet Çok2, Dilek Battal3,
Şükran Poyrazoğlu4, Feyza Darendeliler4
1
Istanbul University, Istanbul Faculty of Medicine,
Department of Pediatrics, Istanbul, Turkey
2
Gazi University, Faculty of Pharmacy, Pharmaceutical
Toxicology, Ankara, Turkey
3
Mersin University, Faculty of Pharmacy, Pharmaceutical
Toxicology, Mersin, Turkey
4
Istanbul University, Istanbul Faculty of Medicine,
Department of Pediatric Endocrinology, Istanbul, Turkey
Background And Aims: Prevalence of childhood obesity
is rising in which endocrine disruptors chemicals play an
important role. Bisphenol a is one of them widely used in
food and drink packaging. It mimics estrogen that leads to
insuline resistance and obesity. We aimed to draw attention
on this topic and collect data about Turkish children.
Methods: Our study group is consisted of children
among patients followed up with overweight and obesity
in Istanbul Faculty of Medicine, Pediatric Endocrinology
outpatient clinic. Normal weight group is selected
among children applied to Istanbul Medical Faculty
General Pediatrics outpatient clinic with similar age and
sex distribution. Weight and height were measured by
trained personnel. Fresh spot urine samples preserved in
glass tubes at -20Cjavascript:addsym(‘%C2%B0’); until
laboratory testing. Urinary bisphenol A (BPA), bisphenol
A glucronid (BPAG) and creatinine levels are mesured
with liquid chromatography–tandem mass spectrometry.
Results: 172 participants were %47,7 (n=82) male,
%52,3 (n=90) female who aged between 4 and 18 (mean
11,68±3,25) years. Mean BPA and BPAG concentration
corrected with urine creatinin is 182,46±293,18
mg/g creatinin in general, 138,46±224,08 in obese,
105,91±174,95 in overweight, 104,36±127,80 in
normal weight group. BPA concentrations revealed
poor correlation with Body mass index (BMI) but no
statistically proven difference among the groups.
When participants were grouped by BPA concentration
persantile obesity prevalence did not show difference.
Conclusions: Factors related to obesity are not easy
to study as other multifactorial diseases. Although it
is not proven statistically we had a clue about relation
of BPA an childhood obesity. Endocrine disrupters is an
important topic needed to be focused on. We think that
this study is important to be the beginning point and
nationally representative study is needed to evalute the
effects of BPA in Turkish population.
Our study is financially supported by Istanbul University
Scientific Research Projects Coordination Unit.
Keywords: endocrine disruptors, bisphenol a,
childhood obesity
[P-223]
Unnecessary Antibiotic Use in Hand Foot and
Mouth Disease
Sule Nursah Ayata1, Burcu Karadaş2, Hatice Dülek1,
Hüray Kök2, Zeynep Emine Tuzcular Vural1, Çağatay
Nuhoğlu2
1
Department of Family Medicine, Haydarpasa Numune
Training And Research Hospital, Istanbul, Turkey.
2
Department of Pediatrics, Haydarpasa Numune
Training And Research Hospital, Istanbul, Turkey.
Background&Aims: Hand, foot, and mouth disease
(HFMD) mostly affects infant and young children and is
one of the major public health concerns in developing
countries. Characteristic clinical signs and symptoms are
usually sufficient to diagnose the disease and no specific
treatment other than relief of symptoms is necessary in
usual cases. High rate of unnecessary antibiotic use is
remarked in this case series.
Methods: Clinical data of 35 children were retrospectively
reviewed in Haydarpaşa Numune Training and research
Hospital, Department of Pediatrics. Hematological or
serologic investigation has not been performed and
diagnosis was made according to clinical and physical
examination of the patients, which are mild fever,
fatigue, odynophagia, rash on the hands and feet, and
blisters in the oral cavity.
Results: Among the 35 children, 23 (65.7%) were boys
- 109 -
and 12 (34.3 %) were girls, and their mean age was 20.4
± 14.0 months. Majority of the patients were between
3 and 20 months of age, which accounted for 60% (21
of 35) of the total patients. Rash was the most common
symptom (35 of 35 patients) and was the major reason
for parents to seek medical care. Five of the patients
(14.3%) were hospitalized overnight for treatment of
dehydration and rest of the patients were given outpatient
care. Before admission to our clinic 15 of patients (42.9
%) had been prescribed antibiotics by pediatricians or
primary care physicians due to misdiagnosis or fear from
superinfection.
Conclusion: In this case series almost half of the
patients had been treated with antibiotics for some
reason. In this report we remark the the importance of
being familiar with the specific clinical scene of HFMD for
pediatricians and primary care physicians to making the
correct diagnosis and avoiding unnecessary antibiotic
treatments.
Keywords: Rash, Hand Foot and Mouth Disease,
Antibiotic Treatment
[P-224]
Pediatric Mastocytosis: Report of 7 cases
Süheyla Ocak1, Esra Yücel2, Hilal Susam Şen1
1
Okmeydani Research and Training Hospital,
Department of Pediatric Oncology
2
Okmeydani Research and Training Hospital,
Department of Pediatric Allergy
Background&Aims: Pediatric mastocytosis can very
easily be mistaken for a variety of common rashes that
plague the pediatric population.The management of
children with mastocytosis possess a chalenge as due
to lack of evidence that reliably predict prognosis. Here
we presents 7 cases of mastocytosis diagnosed between
November 2014- June 2015.
Results: Of the 6 children with mastocytosis and 1 child
with mastocytoma 5 were male and 2 were female. The
mean age at first symptom and at diagnosis were 4
months (range 1-6 months) and 11,5 months ( range 6-22
months), respectively. Skin rash is the main complaint.
All patients were evaluated by a general pediatrician at
first admission, and the major diagnoses were allergic
eruption and dermatitis. The diagnosis was proved by
skin biopsy in all cases. On admission aside from the
diffuse,multiple, solmon or pink coloured eruption, all
patients had normal physical examination. Complete
blood counts with differential, peripheral blood smear,
renal and liver function tests, serum IgE and tryptase
levels were all normal. Abdominal ultrasonography did
not suggest systemic involvement. Oral antihistaminics
and mast-cell stabilizers were prescribed for symptomatic
patients. Anaphylaxis did not ocur in any patients.
Although the number and the site of the lesions have
changed with time, no spontaneous disappearance were
documented on mean follow-up period of 8 months
(range 4-12 months).
to differentiate from other common causes of skin
eruptions in infants and to provide symptomatic relief to
patients. Also informing the parents about the follow-up
and prognosis will prevent anxiety and the uncessary
visits to hospital.
Keywords: child, cutaneous mastocytosis
[P-225]
Pneumonia Is More Than Pneumonia
Şifa Şahin1, Tuba Koçkar1, Halil Suat Ayyıldız2, Sedat
Öktem1, Ömer Ceran1
1
Pediatrics, Faculty of Medicine, Medipol
University,Istanbul Turkey
2
Pediatric Surgery, Faculty of Medicine, Medipol
University,Istanbul Turkey
Introduction: Pneumonia is the most cause of morbidity
and mortality of childhood. Generally infiltrations of X-ray
make a sign infections of lung like pneumonia. Therefore
other reasons are disregarded for many times. Clinicians
have to consider other causes for differential diagnosis.
Case 1: The patient was 6 year old girl with a history
of fever, cough and persistant hemoptysis. And chest
X-ray infiltration was determined. She had treated with
antibiotics for three times as a pneumonia. Hemoptysis
persisted although treatment. The patient was
excamined again and properly limited round infiltrate
area was detected on right hemithorax pericardial area.
Serological tests were examined for hydatid cyst and
treated with albendasole. Hemoptysis stopped. Figure.1
Case2: 6 year old boy, had suddenly onset and
persistent cough with fever. An infiltration was noticed
on left hemithorax and the patient was treated with
broad spectrum antibiotics. But symptoms persisted.
After an evaluation of history and the image of chest,
we thought that it may be foreign body aspiration. We
performed bronchoscopy and peanut was detected left
main bronchus. Figure.2
Case 3: 3 month year old boy, the patient had fever,
cough, desaturation from the birth. There was no feeding
intolerance. And the patient was treated with antibiotics
for many times. He was unresponsive to treatment. We
saw an air density on right hemythorax on chest X-ray.
We performed esophagography and hiatal hernia was
detected. Figure.3
Case4: 3 year old girl who had neurological defect,
was treated as a pneumonia for many times. But we
considered another diagnosis for recurrent pulmonary
infections. So that we determined swallow disfunction.
Conclusion: All infiltrations are not always pneumonia.
Physicians should consider other reasons like cyst
hydatic, swallow disfunction, foreign body aspiration,
hiatal hernia.
Keywords: Pneumonia, cyst hydatic, hiatal hernia
Conclusions: Pediatric mastocytosis is a rare and
pediatricians is not familiar with the disease. Although it
is mostly limited to skin, early recognition is important
- 110 -
[P-226]
A Case Of CHARGE Syndrome With Recurrent
Respiratory Problems
Hatice Ütkü Sınav1, Şifa Şahin1, Leyla Telhan2, Fatma
Gamze Demirel3, Sedat Öktem4
1
Pediatrics, Faculty of Medicine, Medipol University,
Istanbul Turkey
2
Pediatric Critical Care Medicine,Faculty of Medicine,
Medipol University, Istanbul Turkey
3
Pediatrics Neonatalogy, Faculty of Medicine, Medipol
University, Istanbul Turkey
4
Pediatric Pulmonology, Faculty of Medicine, Medipol
University, Istanbul Turkey
Introduction: The estimated birth incidence of CHARGE
syndrome is 1 in 8.500 -12.000. This syndrome is
generally associated with coloboma, heart defect,
choanal atresia, retarded growth and development,
genital hypoplasia, ear anomalies/deafness. Children
with CHARGE syndrome require multidisciplinary
evaluation and some times need pediatric intensive care
follow-up.
Case: 26 week gestational age, 620 gr male baby
was born by cesarean section to a 24-year old female.
The infant had Apgar scores of 1 and 7 at first and
fifth minutes. He was intubated and transferred to
neonatal intensive care unit for assisted ventilation.
He had dismorphic face appearance, ear anomaly. We
could not pass through nasogastric tube on right side of
nasal cavity. Choanal atresia was determined on computed
tomography and treated surgically. Echocardiography
was performed and of bronchopulmonary dysplasia.
He was discharged with home ventilation. However
he hospitalized many times for aspiration pneumonia
outpatient follow up. We examined etiology of aspirations.
Gastroesophageal reflux determined. It was applied
Nissen fundoplication, gastrostomy and symptoms
decreased. Genetic tests did not result yet. Patients’
follow-up still continue in pediatric pulmonology.
Conclusion: CHARGE syndrome is a rare disease and
a clinical diagnosis. The etiology may be genetically as
with other syndromes. Clinicians should consider this
syndrome for patients that have multi system defects
and recurrent respiratory problems and aspirations.
Keywords: CHARGE syndrome, coloboma, choanal
atresia
[P-227]
Statistical analysis of the Syrian mothers
and their babies in our hospital
Tolga Besci1, Özge Besci1, Zeynep Alp Ünkar2, Aslı
Memişoğlu2, Hülya Özdemir2, Hülya Bilgen2, Eren Özek2
1
Derpartment of Pediatrics, Marmara University Medical
Faculty, İstanbul, Turkey
2
Division of Neonatology, Derpartment of Pediatrics,
Marmara University Medical Faculty, İstanbul, Turkey
Background & Aims: Immigration may result in
deaths, spreading contagious diseases, inclining poverty,
malfunctioning health issues related to mother and child,
antenatal care and family planning. Immigrant mothers’
difficulties to reach for antenatal care, mortality of the
fetuses, the incidence of low birth weight infants, the rate
of birth of the premature infants and the prevalence of
infectious diseases are discussed in different researches
worldwide. We intended to outline the demographical
characteristics and the health issues related to the
hospital stay of the Syrian mothers and their babies who
applied to our hospital in year 2014.
Methods: Demographical data, and problems related
to the hospital stay of the pregnant women and their
babies in our hospital in year 2014 were retrospectively
analyzed from the patients’ files.
Results: In year 2014 in our hospital, 72 (%2.6) of all
the 2753 live births were Syrian refugees. Only 57 of
72 mothers’ pregnancy history could be taken. Mean
mother age was 24.3±6.4 years. Adolescent pregnancy
rate was (<19years) %19.4. Average pregnancy number
was 2.6. Average gestational age of the babies was
37.5±3.2 and the average birth weight 3068±705 gr. Of
all babies %5 was preterm, %12.5 was late preterm, 7%
of the babies of small for gestational age. 7% of all the
babies needed resuscitation at birth. 12 % of the babies
were internalized in neonatal intensive care unit (NICU).
Conclusions: Communication difficulties with the
mothers create problems in defining the babies at risk.
Delay in diagnosis and/or treatment could be an inevitable
result of an inaccurate history. Despite the language
problem, fewer babies than expected were admitted to
NICU with the help of effort and good communication
skills of health care providers. Thus, in order to improve
the quality of health care services given to immigrants,
more translators are needed in hospitals.
Keywords: Syrian refugees, health problems,
maternity
[P-228]
A Case Report: Haemophagocytosis with
Respiratory Distress and Lymphangiomatosis
with Bleeding
Tuba Koçkar1, Pınar Yamaç2, Ebru Tuğrul Sarıbeyoğlu3,
Suat Ayyıldız4, Rıza Rızalar4, Sedat Öktem1
1
Pediatric Pulmonology, Faculty of Medicine, Medipol
University, Istanbul, Türkiye
2
Pediatrics, Faculty of Medicine, Medipol University,
Istanbul, Türkiye
3
Pediatric Haemotology, Faculty of Medicine, Medipol
University, Istanbul, Türkiye
4
Pediatric Surgery, Faculty of Medicine, Medipol
University, Istanbul, Türkiye
Background: Lymphangiomatosis is a syndrome in which
multiple organs are involved. Frequent anatomic sites of
involvement include the mediastinum, retroperitoneum,
axilla and neck. Lymphangiomatosis is usually fatal at
an early age.
Objection: We aimed to highlight a rare causes of
inspiratory stridor and respiratory distress in children
before age 2.
- 111 -
Case: A 21 months of girl was admitted with respiratory
distress, malaise and failure to thrive. She had story
of long stay at hospital in Georgia. She was performed
several laboratory test and examination but her diagnosis
were not named yet. Sweat chloride test result was
found intermediate levels and genetic analysis resulted
7T/7T polimorfism (which is nonsense mutation for cystic
fibrosis). So that she referred to our hospital suspicion of
cystic fibrosis. There was shortness of breath, inspiratory
stridor, several ecchymosis on trunk and extremity,
bilateral multipl servical lympadenomegaly, abdominal
distension, hepatomegaly and splenomegaly in her first
examination. Abdominal ultrasonography confirmed an
increase in size of the spleen and liver; the spleen size
was 120 mm and the liver size was 100 mm. Servical
ultrasonography confirmed in various size of servical and
submandibular multipl lympadenomegaly. Abdominal
computed tomography confirmed hepatosplenomegaly
and probably suggested diffuse lymphangiomatosis.
Oral bleeding and epistaxis started and progressively
increased then dissemine intravascular coagulation
occurred. She was transfused many times with erytrocyte
and thrombocyte suspensions, fresh frozen plasma. She
underwent palliative splenectomy. The pathologic findings
suggested that massive splenomegaly (the size of spleen
was from 6.5 cm to 16 cm. The pathologic diagnosis was
haemophagocytosis and lymphangiomatosis.
Conclusion: Diffuse lymphangiomatosis is a rare
condition in infancy. It presented various symptoms.
Pediatricians should be vigilant lymphangiomatosis for
in patients with inspiratory stridor beside servical and
mediastinal vascular mass especially in infancy and early
childhood.
Keywords: Infancy, Stridor, Lymphangiomatosis
[P-229]
An Alternative Treatment to Tracheostomy
in Pierre Robin Sequence: Mandibular
Distraction Osteogenesis
Tuba Koçkar1, Sedat Öktem1, Ismail Mithat Akan2,
Ayhan Taştekin3
1
Pediatric Pulmonology, Faculty of Medicine, Medipol
University, Istanbul, Turkey
2
Plastic Surgery, Faculty of Medicine, Medipol University,
Istanbul, Turkey
3
Neonatology, Faculty of Medicine, Medipol University,
Istanbul, Turkey
Background: The triad of glossoptosis, micrognathia,
and cleft palate characterizes the Pierre Robin sequence
(PRS). Mandibular distraction osteogenesis can be a
safe and effective way to avoid tracheostomy placement
in selected neonates with Pierre Robin sequence.
Case: A two month-old boy who was admitted to the
hospital with the complaints of cyanosis and feeding
difficulty and he was diagnosed as Pierre Robin sequence.
He had respiratory distress after a while deteriorated.
Firstly he followed with intermittent nasal continuous
positive airway pressure (CPAP) then underwent assisted
ventilation. He had difficulty in weaning from mechanical
ventilation so that flexible bronchoscopy was performed
for evaluation of respiratory tract. We established
severe laryngomalacia. Nasopharyngeal airway was
performed to the patient to ensure airway safety. But
respiratory distress did not improve, therefore we
decided to perform mandibular distraction osteogenesis
and tracheostomy. In follow up difficulty of feeding
was improved after 11th day of mandibular distraction
osteogenesis. Computerized tomography revealed an
adequate airway; therefore tracheostomy was closed
at 18th day. The distractors were removed two months
after ossification of callus formation. We did not observe
any complications during the surgery or post-operative
follow-up visits.
Conclusion: Mandibular distraction osteogenesis can
be used as an alternative treatment in Pierre Robin
syndrome patients who underwent tracheostomy. A
successful external mandibular distraction induces the
removal of the airway obstruction, allowing spontaneous
ventilation without devices.
Keywords: Pierre Robin, Mandibular Distraction
Osteogenesis
[P-230]
A Case of Pulmonary and Intracardiac
Thrombosis in Behcet Disease
Tugce Goksu Yilmaz, Fatih Mehmet Keleşoğlu, Gafur
Doğdu, Ayça Gürbüzer, Hakan Çakır, Sevinç Emre,
Rukiye Eker Ömeroğlu, Başak Saraçoğlu
Istanbul University, Istanbul Faculty of Medicine
Case Presentation: The present report is of a 4-year-old
male patient, admitted to the Istanbul University Istanbul
Faculty of Medicine Pediatric Emergency Department
with complaints of cough and hemoptysis. The patient
had been admitted to various centers with complaints of
chest pain for the past two months. Patient’s symptoms
including hemoptysis did not improve with treatment,
then we decided to do further work-up for diagnosis.
Thorax CT scan showed thrombi in both lungs and heart.
Thrombus in the right ventricle was demonstrated with
Echocardiography and Cardiac Magnetic Resonance
Imaging (Figure 1).
The patient was hospitalized for further work-up and
treatment. Department of Pediatric Hematology was
consulted. Bloodwork was ordered to investigate the
etiology of thrombosis and Low Molecular Weight
Heparin (LMWH) was started following the suggestions
of the Department of Pediatric Haematology. Further
anamnesis revealed history of recurrent oral aphtosis.
Due to the fact that Behçet’s disease could present with
trombi in similar locations, Pathergy test was ordered.
Pathergy test was negative but HLA B51 test was positive.
The patient was given intravenous methylprednisolone
and azathioprine therapy. Complaints of chest pain and
hemoptysis responded well to the therapy. Intravenous
methylprednisolone was later replaced with oral
prednisone which was tapered off. The patient is now
only on azathioprine and followed-up in the outpatient
clinic.
Keywords: Behcet’s, Disease, thrombosis
- 112 -
[P-231]
[P-232]
Tugce Kalayci Oral1, Nazli Kavcik1, Ilker Gonen1, Elif
Kirit1, Esin Aldemir2, Aysel Kiyak3, Sevgi Yavuz3
1
Department of Pediatrics, Kanuni Sultan Suleyman
Education and Research Hospital
2
Department of Neonatology, Kanuni Sultan Suleyman
Education and Research Hospital
3
Department of Pediatric Nephrology, Kanuni Sultan
Suleyman Education and Research Hospital
Introduction: Nephrogenic diabetes insipidus (NDI),
is a group of diseases caused by decreased urine
concentration capability due to antidiuretic hormone
(ADH) insusceptibility, resulting in polyuria and polydipsia.
Vesicoureteral reflux disease (VUD) and ureterovesical
junction (UVJ) obstruction are congenital urinary tract
abnormalities and coexistence of those is very rare. In
this paper, we would like to present a case, diagnosed
with nephrogenic diabetes insipidus secondary to stage
5 right VUD and left UVJ obstruction.
Tugce Kalayci Oral1, Sevgi Yavuz2, Aysel Kiyak2, Hamide
Sevinc Genc1, Mustafa Cakan3, Nuray Aktay Ayaz3
1
Department of Pediatrics, Kanuni Sultan Suleyman
Education and Research Hospital, Istanbul, Turkey
2
Department of Pediatric Nephrology, Kanuni Sultan
Suleyman Education and Research Hospital, Istanbul,
Turkey
3
Department of Pediatric Rheumatology, Kanuni Sultan
Suleyman Education and Research Hospital, Istanbul,
Turkey
Introduction: Cystinosis is an autosomal recessively
inherited disease characterized by accumulation of
cystine in tissues due to transporting defect from
lysozymes. It accumulates in cornea, bone marrow,
thyroid gland, pancreas and mainly in kidney and causes
organ dysfunction. Involvement of skeletal system is a
very rare entity. Until this date, only one patient with
cystinosis reported to have arthritis. We would like to
present a case, applying to our outpatient clinic with
arthritis and diagnosed with cystinosis.
Nephrogenic diabetes insipidus with the
background of vesicoureteral reflux disease
and ureterovesical junction obstruction
A Cystinosis Case Presenting With
Arthropathy
Case Presentation: A patient, 4 days old, applied to
emergency clinic with fever. He had antenatal diagnosis
of left renal hydronephrosis. Weight loss detected as
360 grams (%13). The patient was admitted to NICU
with pre-diagnosis of hypernatremic dehydration.
Even though adequate hydration was obtained; urea,
creatinine, Na ve Cl levels preceded as high and urine
density as low. Polyuria was constant. “Bilateral severe
hydronephrosis” was found in abdominal ultrasound.
Voiding cystourethrography showed stage 5 right
VUR. MAG-3 scintigraphy was concordant with left UVJ
obstruction. Patient diagnosed as nephrogenic diabetes
insipidus. Hydrochlorothiazide treatment started.
Result: NDI develops due to ADH insusceptibility
of kidneys. NDI patients are under risk for
neurodevelopmental retardation, due to hypernatremic
dehydration. In our patient, uncorrectable hypernatremia
by hydration, stage 5 VUD in right kidney and left
UVJ obstruction led us to diagnose him as secondary
nephrogenic diabetes insipidus and normal ADH values
helped us to differentiate this disease from central
diabetes insipidus. The main goals of the treatment
are replacing urinary water loss with appropriate fluids
and decreasing urine output. Urine output can be
decreased by medical treatment up to %30-70, daily.
As a result, screening renal pathologies in children
applying with hypernatremia, hypercholoremia and
dehydration; although rare, keeping in mind nephrogenic
diabetes insipidus as secondary to those pathologies is
important in decreasing treatable complications such as
growth and mental retardation.
Keywords: NDI, VUD, UVJ
Case Presentation: A 3 year old patient applied to our
outpatient clinic with the complaints of pain and swelling
of the right knee for 2 weeks. In her history, polyuria,
polydipsia and lack of weight gaining were present. The
parents did not describe a recent infection or fever. Weight
and height were found to be below 5 percentile, body
temperature was 36.5 C and blood pressure was 90/50
mmHg. Her physical examination revealed sensitivity,
swelling and lack of movement in the right knee. There
was no significant elevation of temperature. Other
system examinations were normal. In her laboratory
findings, full blood count and acute phase reactants were
in normal ranges but hypokalemia, hypophosphatemia,
mild metabolic acidosis, glycosuria, proteinuria and
hypercalciuria were detected. Increased joint fluid of the
right knee found in radiological evaluation. The patient
started on ibuprofen and arthritis findings are regressed
in two weeks. Acute phase reactants remained in normal
ranges. Her wrist x-ray revealed findings of rickets and
ophthalmologic examination showed cystine crystals.
Her blood leukocyte cystine levels found to be high, she
was diagnosed as cystinosis and started on cysteamine
treatment.
Discussion: This case emphasizes the importance of
careful evaluation of renal tubular functions in patients
with arthritis accompanied by growth retardation, rickets
and electrolyte imbalance; and keeping cystinosis in
mind in differential diagnoses
Keywords: cystinosis, arthritis, cystine
- 113 -
[P-233]
The Relationship Between Streptococcal
Tonsillopharyngitis and Erythema Nodosum
in a Child
[P-234]
Five Children with Leg Pain
Tuğçe Kalın1, Aysun Yahşi2, Tuğçe Tural Kara2, Döndü
Nilay Penezoğlu1, Osman Umur Başaran1, Çisem
Saygılı3, Tuğba Erat2, Halil Özdemir2, Ergin Çiftçi2, Erdal
İnce2
1
Department of Pediatrics, Ankara University Medical
School, Ankara, Turkey
2
Department of Pediatric Infectious Diseases, Ankara
University Medical School, Ankara, Turkey
3
Department of Family Medicine, Ankara University
Medical School, Ankara, Turkey
Erythema nodosum (EN) is a variant of septal panniculitis
with no vasculitis which occurs most probably due to
the antigenic stimuli by inciting factors like infections,
sarcoidosis, rheumatologic diseases, inflammatory
bowel diseases, medications, autoimmune disorders.
Streptococcal infections are the most frequently
causative factors in children; the cutaneous lesions
appear 2-3 weeks after the throat infection. Tissue defect
is localized on the anterior aspects of the legs which is
caused by reactive oxygen intermediates produced by
activated neutrophils and the subcutaneous fat are
infiltrated by inflammatory cells. EN recovers without
scarring and ulceration in 3-6 weeks spontaneously.
Bed rest, anti-inflammatory drugs, potassium iodide,
systemic corticosteroids, colchine, hydroxchloroquine
can be used for treatment. Herein, we reported a 10year old-girl who had EN secondary to streptococcal
tonsillopharyngitis. She was suffering from painful,
edematous and erythematous lesions localized on
bilateral anterior tibia. Her complaints started two weeks
ago. Fever and cough added at the same time. On the
physical examination, she had oropharynx hyperemia
and the typical eruption of EN that included symmetric,
tender, erythematous, warm and raised plaques located
on her knees. Group A β-hemolytic streptococcus
(GABHS) was isolated from the throat culture. Following
the laboratory examinations, hemoglobin: 11.1 g/dL,
leukocyte count: 19340/mm³, platelet count: 506000/
mm³, sedimentation: 74 mm/h, C reactive protein: 38.6
mg/L, antistreptolysin O titer: 2864 IU/mL. Biochemical
parameters and radiograph were normal. Additionally,
PPD test was negative. She was diagnosed as EN
secondary to GABHS. Oral paracetamol and intravenous
methylprednisolone were given for 3 days. As expected,
clinical findings were regressed, she was discharged
with oral methylprednisolone. As a result, EN, which is
an unusual cutaneous reactive process in children, may
be seen as a complication of streptococcal infections.
Because of this, GABHS should be considered as a
causative agent of EN.
Keywords: childhood, erythema nodosum,
streptococcal tonsillopharyngitis
Tuğçe Kalın1, Tuğçe Tural Kara2, Halil Özdemir2, Fatih
Günay1, Nisa Eda Çullas İlarslan1, Elif İnce3, Deniz
Tekin4, Zümrüt Uysal3, Emine Zeliha Suskan4
1
Department of Pediatrics, Ankara University Medical
School, Ankara, Turkey
2
Department of Pediatric Infectious Diseases, Ankara
University Medical School, Ankara, Turkey
3
Department of Pediatric Hematology and Oncology,
Ankara University Medical School, Ankara, Turkey
4
Department of Pediatric Emergency, Ankara University
Medical School, Ankara, Turkey
Leg pain, which has so many different causes, is a
common complaint in childhood. The pediatricians should
always be skeptical when evaluating the leg pain and take
a detailed history, make physical examination carefully.
When an underlying disease is suspected, laboratory
tests and imaging should be done. In this article, we
studied five cases with leg pain which were admitted to
Ankara University Medical School Hospital Departments
of Pediatrics. The aim of this study is to emphasize
that the leg pain might be a symptom of a serious
illness. All other pathologic causes should be eliminated
while diagnosing the growing pain or musculoskeletal
syndrome. First case was a 3-year-old girl who had left
leg pain for three months. Physical examination was
normal except from the 3/6 systolic murmur. There were
pancytopenia in complete blood count and bone marrow
aspiration contained remarkable amount of blast cells.
Then she was diagnosed as leukemia. The second case
was 10-year-old girl who had leg pain continuing for
four months. The tuberositas tibia area was sensitive
to palpation, piece ossification of the tibial tubercle was
detected on the X-ray so she was diagnosed as OsgoodSchlatter disease. The third case was 5-year-old girl
who had right leg pain and limping. The X-ray showed
a defective structure on the right femoral epiphysis
and hence she was diagnosed as Legg-Calves-Pertes
disease. The fourth case was 10-year-old boy who
suffered leg pain for twenty days. Acute phase reactants
were significantly high. X-ray detected lytic lesions
on femoral metaphysis. He was diagnosed as acute
osteomyelitis. In the fifth case, leg pain started after flu
symptoms, creatinine kinase levels were elevated. She
was diagnosed as acute viral myositis. In conclusion;
leg pain could be a sign of many diseases. Pediatricians
should be careful when evaluating the leg pain.
Keywords: childhood, leg pain, growing pain
- 114 -
[P-235]
A Rare Agent of Peritoneal Dialysis Related
Peritonitis in a Child: Campylobacter Jejuni
[P-236]
Tuğçe Tural Kara1, Songül Yılmaz2, Halil Özdemir1,
Zeynep Birsin Özçakar2, Ahmet Derya Aysev3, Ergin
Çiftçi1, Erdal İnce1
1
Department of Pediatric Infectious Diseases, Ankara
University Medical School, Ankara, Turkey
2
Department of Pediatric Nephrology, Ankara University
Medical School, Ankara, Turkey
3
Microbiology Laboratory, Ankara University Medical
School, Ankara, Turkey
Peritonitis is a serious problem in children receiving
peritoneal dialysis. The overwhelming majority of
peritonitis cases are caused by pathogenic bacteria
like
Staphylococcus
epidermidis,
Staphylococcus
aureus, Streptococcus spp., Enterococcus faecalis and
Escherichia coli. Peritoneal fluid culture is important,
because unusual microorganisms may be causative.
Campylobacter jejuni is a rare peritonitis agent in
children. We report a case of C. jejuni peritonitis in a
10-year-old boy who had end stage renal failure due to
atypical hemolytic uremic syndrome. He was admitted to
hospital with abdominal pain and fever. Peritoneal dialysis
fluid was cloudy and microscopic examination showed
abundant leukocytes. The diagnoses of peritonitis were
established. Then we started intraperitoneal cefepime for
empirical treatment. On the 3rd day of treatment clinical
and laboratory findings were not exactly recovered.
Patient’s temperature did not return to normal range
and peritoneal fluid did not become clear. Peritoneal
fluid culture revealed C. jejuni which was identificated
with Matriks Assisted Laser Desorption/Ionization Time of Flight (MALDITOF) technology, in two different
samples. Then oral clarithromycin treatment was
added for 10 days. Intraperitoneal cefepime treatment
was continued for 14 days. At the end of the therapy,
clinical findings recovered, peritoneal fluid culture
became negative. In addition, he did not have any
peritonitis attacs. In this case, we report a child with
C. jejuni peritonitis who was successfully treated.
Campylobacter peritonitis is an unusual infection in
children, which have been more reported in adult
patients in the literature. Oral clarithromycin is more
effective for treatment and clinical outcomes are very
good. As a result, unusual patogen microorganisms like
Campylobacter spp. must be remembered as a causative
agent of peritonitis in children who are not responsive to
empiric treatment.
Keywords: Campylobacter jejuni, continuous
ambulatory peritoneal dialysis, peritonitis
Petechial-Purpuric Rash and
Thrombocytopenia Associated with
Parvovirus B19 in a 10-year-old Child
Tuğçe Tural Kara, Aysun Yahşi, Tuğba Erat, Halil
Özdemir, Ergin Çiftçi, Erdal İnce
Department of Pediatric Infectious Diseases, Ankara
University Medical School, Ankara, Turkey
Parvovirus B19 is a common infection around the
world which can cause different skin rashs like
maculopapular, erythematous, petechial or purpuric
lesions. Fever, artralgia, myalgia and lymphanopathy
may also occur. Petechial and/or purpuric lesions may
be seen with edema and erythema. Papular-purpuric
gloves and socks syndrome and thrombocytopenia
are some clinic spectrums of Parvovirus B19.
We report a child who was admitted to hospital with
petechial rash on her upper and lower extremities.
The petechial rash occured especially in her hands and
feet, like glove and sock style. Fever was continued
during one week. On physical examination bilateral
submandibular and cervical lymphadenopathy which
were multiple, painful and the largest size 2x1 cm, were
localized. In addition aphthous lesions in the mouth,
petechiae on the hard palate and gingival hyperemia
were revealed. Petechial-purpuric rash occurred on
trunk, face and extremities especially on hands and
feet. Routine laboratory analysis revealed a hemoglobin
level of 12.7 g/dL, white blood cell count of 4700/mm3,
platelets count of 125000/mm³. Serologic testing of
parvovirus B19 IgM was determined positive and IgG
was found negative. Petechial rash began to decline
after third day of hospitalization. We believe that the
disease is primary Parvovirus infection. The patient was
treated with only antipyretic drugs and on follow up the
clinical and laboratory findings completely recovered.
As a result; Parvovirus B19 can cause some
different
skin
rashs
like
petechiae
and
purpura. It should be considered in the differential
diagnosis in patients with fever, thrombocytopenia
and petechial-purpuric lesions. In healthy children
symptomatic treatment is enough to improve the clinical
and laboratory signs.
Keywords: Parvovirus B19, rash, thrombocytopenia
[P-237]
Calcaneus Osteomyelitis: Secondary to
Guthrie Test
Tuğçe Tural Kara1, Tuğba Erat1, Halil Özdemir1, Aysun
Yahşi1, Suat Fitöz2, Ergin Çiftçi1, Erdal İnce1
1
Department of Pediatric Infectious Diseases, Ankara
University Medical School, Ankara, Turkey
2
Department of Pediatric Radiology, Ankara University
Medical School, Ankara, Turkey
Calcaneus osteomyelitis is an unusual infection in
children. It is seen about 3-10% of all pediatric
osteomyelitis. Invasive procedures, prematurity, low
birth weight are some causative factors. Clinical signs
may be mild. We report a neonate with calcaneous
osteomyelitis which was secondary to Guthrie test.
In our patient, Guthrie test was made with nozzle on
- 115 -
the 7th postnatal day. Then three days later swelling
occurred on her right heel. On physical examination,
5x5 cm swelling, redness, tenderness and increased
temperature was found on her right heel. Patients
laboratory test results were as follows; leukocyte
count: 16600/mm3, C-reactive protein: 5.1 mg/dl and
erythrocyte sedimentation rate: 40 mm/h. X-ray showed
osteolytic lesion on the calcaneus which was consistent
with osteomyelitis. Superficial tissue ultrasound showed
24x11 mm fluid collection with heavy content was
observed on the posterior of the right foot. Extremity
magnetic rezonans imaging showed tissue defect,
edema-inflammation and contrast enhancement in
right foot. All these findings supported osteomyelitis.
Puncture and drainage was performed and methicillinsusceptible Staphylococcus aureus was isolated from
drainage culture. Then intravenous sulbactam ampicillin
treatment was started. S. aureus was detected in
nasal swab culture of the patient and her mother.
Therefore, topical mupirocin was used during 10
days. Intravenous sulbactam-ampicillin treatment was
given for 3 weeks. After discharge, oral amoxicillinclavulanate treatment was received during 3 weeks.
At the end of treatment, the lesion was completely
resolved and acute phase reactants were normal. In
follow-up, the patients recovered without sequelae.
As a result, calcaneous osteomyelitis is an uncommon
infection in newborns which should be considered in the
differential diagnosis of swelling, redness, and increased
in temperature on the heel. We should not forget that
invasive procedures may cause osteomyelitis. Therefore
aseptic technique should be performed before the
invasive procedures.
Keywords: Calcaneus, Guthrie test, osteomyelitis
[P-238]
A Case Report of Congenital Lobar
Emphysema: A Rare Cause of Hypertension
Tuğçe Tural Kara1, Halil Özdemir1, Hüseyin Dindar2,
Ergin Çiftçi1, Erdal İnce1
1
Department of Pediatric Infectious Diseases, Ankara
University Medical School, Ankara, Turkey
2
Department of Pediatric Surgery, Ankara University
Medical School, Ankara, Turkey
Congenital lober emphysema is a rare disease which
affects especially newborns and infants. It is characterized
by pulmoner hyperinflation. Depending on the degree of
bronchial obstruction, the clinical presentation is variable
like tachypnea, tachycardia and retractions.
We report a rare case of congenital lober emphysema in
a 38-day-old male infant who admitted to our hospital
with tachypnea, tachycardia and retractions. On physical
examination he was found to be tachypneic at the rate
of 65/min, heart rate of 185/min and blood pressure
of 130/70 mmHg (normal range 103/56 mmHg-78/37
mmHg). His oxygen saturation was 85% at room air. In
addition, subcostal and intercostal retractions, decreased
air intake and bronchospasm were found. Increased
aeration of left lung and significant mediastinal shift to
the right one were observed on the chest X-ray.
Emphysematous changes were seen in the upper lobe of
the left lung on the thorax computed tomography which
was consistent with congenital lobar emphysema.
Then the patient was successfully operated. During
the postoperative period, the patient’s blood pressures
remained normal. Chest X-ray demonstrated the left lung
ventilation decreased and right one shifted at the left side.
We thought that due to increased from left to right shift,
the renal blood flow reduced by compressing main blood
vessels. Reduction in renal blood supply activated the
renin angiotensin aldosterone system which caused
hypertension by increasing the involvement of water and
salt. After left upper lobectomy, the patient’s symptoms
significantly decreased furthermore hypertension was
not seen again.
As a result; congenital lober emphysema is a rare disease
which may cause lots of clinical signs like respiratory
distress, tachypnea, tachycardia. As well as it can
also cause hypertension. The clinicians should think
congenital lobar emphysema in the differential diagnosis
of respiratory distress and hypertension.
Keywords: Congenital lober emphysema,
hypertension, respiratory distress
[P-239]
Bone Marrow Aspiration Complications:
Iliopsoas Abscess and Sacroiliac
Osteomyelitis
Tuğçe Tural Kara1, Halil Özdemir1, Tuğba Erat1, Aysun
Yahşi1, Songül Yılmaz2, Ergin Çiftçi1, Suat Fitöz3, Fatoş
Yalçınkaya2
1
Department of Pediatric Infectious Diseases, Ankara
University Medical School, Ankara, Turkey
2
Department of Pediatric Nephrology, Ankara University
Medical School, Ankara, Turkey
3
Department of Pediatric Radiology, Ankara University
Medical School, Ankara, Turkey
Iliopsoas abscess and sacroliliac osteomyelitis are rare
disease in children. History of pelvic trauma may be
described in some cases. Bone marrow aspiration may
cause some rare complications. We present a boy with
iliopsoas abscess and sacroiliac osteomyelitis which are
unusual complications after bone marrow aspiration.
An 18-month-old boy was admitted to hospital because
of fever that was not responsive to antipiretic drugs.
Homozygous M694V mutation was identified that
confirmed the diagnosis of Familial Mediterranean Fever
and oral colchicine was started. However fever continued
despite colchicine. Then bone marrow aspiration was
performed which was found to be normal. Three months
after bone marrow aspiration, he had pain on his left leg.
On physical examination, limited movement and pain
were found on the left leg. Acute phase reactants were
significantly elevated on laboratory examination. Pelvic
computed tomography showed left iliopsoas abscess
near the muscle with many enlarged lymph nodes and
lytic destructive changes in sacrum and iliac bone.
Sacroiliac joint magnetic resonance imaging showed T2
signal enhancement near the bone structure and left
sacroiliac joint, contrast enhancement areas, cortical
thickening and periosteal reaction in the sacroiliac bone.
Percutaneous abscess drainage was performed and
methicillin sensitive Staphylococcus aureus was isolated
from drainage culture. Intravenous ampicillin-sulbactam
and intravenous clindamycin were given for 6 weeks. Follow
up was excellent; fever disappeared, clinical findings
- 116 -
improved, left foot pain reduced and he started to stand on
his foot. Levels of acute phase reactans decreased. After
he was discharged from the hospital, oral amoxicillinclavulanic acid treatment was continued for 2 weeks.
On follow up the patient did not have any complaints.
As a result; bone marrow aspiration may cause some rare
complications. If patients have unusual complaints after
aspiration, psoas abscess and sacroiliac osteomyelitis
should be considered as a complication.
Keywords: Bone marrow aspiration, iliopsoas abscess,
sacroiliac osteomyelitis
[P-240]
Local Complications of Bacille CalmetteGuérin Vaccine at The Injection Site
Tuğçe Tural Kara, Halil Özdemir, Tuğba Erat, Aysun
Yahşi, Ergin Çiftçi
Department of Pediatric Infectious Diseases, Ankara
University Medical School, Ankara, Turkey
Background&Aims: Bacille Calmette-Guérin (BCG)
vaccine can cause some local reactions at the injection
site. Although some of these are normal vaccine
reactions, unexpected complications may be seen. In
literature clinical features, treatment modalities and
outcomes of these complications have not been clearly
defined. We aimed to determine the incidence of local
complications after BCG vaccination, to evaluate the
treatment and to compare this result with the literature.
Methods: Patients, who admitted to our Pediatric
Infectious Diseases Department between January 2010
and June 2015 and developed local complications after
BCG vaccination, were retrospectively included in our
study. Demographic and clinical informations, laboratory
findings, treatment modalities and outcomes were
noted from database. Patients who were diagnosed as
congenital or acquired immunodeficiency or disseminated
BCG infection were excluded from the study.
Results: Eight patients are diagnosed as local cutaneous
complications after BCG vaccination. The mean age was
7.0±2.23 months. Abscess formation (38.5%), localized
inflammation signs like hyperemia, swelling (38.5%),
lesion with purulent discharge (12.5%) and eczematous
lesions (12.5%) were found at the injection site. Patients
were treated with antituberculosis drugs, amoxicillinclavulanic acid, ampicillin-sulbactam and intermittent
drainage. In addition two patients were followed without
treatment. On follow up all lesions regressed without
complications.
Conclusions: There is no still consensus about treatment
of local complication of BCG vaccination. In our study,
some of these complications improved with conservative
treatment, but localized abscess formation required
drainage. In addition, anti-tuberculosis treatment and
antibiotics were given for some indications. As a result
treatment of complications should be decided to each
patient’s clinical symptoms and underlying disease.
Keywords: BCG vaccine, complications, injection site
[P-241]
Fonsecaea Pedrosoi: A Rare Cause of
Maxillary Osteomyelitis and Dental Infection
in a Child with Hematological Malignancy
Tuğçe Tural Kara1, Halil Özdemir1, Aysun Yahşi1, Tuğba
Erat1, Elif İnce2, Ergin Çiftçi1
1
Department of Pediatric Infectious Diseases, Ankara
University Medical School, Ankara, Turkey
2
Department of Pediatric Hematology-Oncology,Ankara
University Medical School, Ankara, Turkey
Fonsecaea pedrosoi is a dermatiaceous fungus which can
cause skin and subcutaneous infections. Oral manifestations
are commonly seen in children with malignancy. However
F. pedrosoi associated dental infection and maxillary
osteomyelitis has not been reported in literature.
Herein, we report a child with F. pedrosoi infections.
A 6-year-old boy with a diagnosis of standart risk precursor
B cell acute lymphoblastic leukemia presented with fever
on the 43rd day of late intensification chemotherapy.
On physical examination black-brown coloured areas
were found near the upper left molar tooth with edema
of the gingiva. On laboratory evaluation, white blood
count was 200/mm3, absolute neutrophil count was 0/
mm3. Paranasal sinus computed tomography showed
hyperdense areas and mucosal thickening in the left
maxillary sinus and dentin loss with central soft tissue on
the upper left second premolar tooth, and osteomyelitis
of maxillary bone. Fungal infection was thought to be
the cause of these findings. Upon this, tooth was pulled
by dentist. Histopathological examination showed
fungal hyphae and conidia on the hematoxylin-eosin
stained section. Grocott’s Methenamine Silver staining
demonstrated the hyphae and conidia of F. pedrosoi. In
addition, F. pedrosoi was isolated from the tissue culture.
The patient received intravenous liposomal amphotericin
B therapy for 5 weeks and he was discharged on oral
voriconazole. His complaints decreased markedly.
He remains on oral voriconazole therapy because
of the possibility of prolonged neutropenia during
chemotherapy. Currently, the patient is at the seventh
month of treatment without any adverse side-effects.
As a result, oral lesions may be seen in patients with ALL
in neutropenic period. F. pedrosoi is an unusual causative
agent of dental infection and maxiller osteomyelitis.
However, it should be considered in the differential
diagnosis of black-brown coloured lesions in neutropenic
patients with malignancy.
Keywords: Dental infection, Fonsecaea pedrosoi,
osteomyelitis
[P-242]
Complete clinical improvement of acute
disseminated encephalomyelitis due to
Mycoplasma Pneumania infection
Ulkem Colak, Emine Tekin, Omer Faruk Aydın
Ondokuz Mayis University, Faculty of Medicine,
Department of Pediatrics
Background&Aims: Mycoplasma pneumania is a
common pathogen for respitatory track infections.
Mycoplasma
infections
can
affect
hematologic,
- 117 -
cardiovasculary,
genitourinary,
gastriontestinal
and central nervous system. Acute Disseminated
Encephalomyelitis (ADEM) can be seen as a central
nervous system manifestation of Mycoplasma and seem
to have poor prognosis. In this paper we present an
eight year old child diagnosed as ADEM assosiated with
Mycoplasma Pneumania infection who developed full
clinical recovery in three months. Our aim is to show to
the clinicians that ADEM due to Mycoplasma Pneumania
may have better outcome.
Case: An 8 year old boy presented with alteration
in consciousness, paraplegia and urinary retention.
He had ten days history of fever, cough and general
muscle pain. His cerebrospinal fluid (CSF) protein was
slightly increased, glucose were normal. MRI showed
hyperintense areas at the left capsula interna, right
talamus, mesencephalon, pons and left dentate nucleus.
The lesions showed increased signal intensity on diffusionweighted images. Acute Disseminated Encephalomyelitis
has been diagnosed. The child was treated with i.v.
metilprednisolone (Prednol) 30 mg/kg/day for five days
and continued with a tappering dose. X-ray of the chest
showed bilateral perihilar and peribronchial infiltration,
Mycoplasma IgM antibodies were positive at serum so
Mycoplasma pneumania infection was diagnosed. The
patient was treated with clarithromycin 14 mg/kg/
day for 10 days. He had clinical improvement on day
5, gained normal limb power and developed complete
remission in 3 months.
Conclusions: To the best of our knowledge this is the
first presentation of ADEM case due to Mycoplasma
infection recovered fully. We suggest that clinicans can
predict a better outcome for the patients who were
diagnosed with ADEM due to Mycoplasma Pneumania.
Keywords: ADEM, child, mycoplasma
[P-243]
Central Venous Catheterization Experince in
Neonatal Intensive Care Unit
Adil Umut Zübarioğlu1, Ali Bülbül1, Ümmühan Öncül1,
Pınar Karadeniz1, Taliha Öner2, Evrim Kıray Baş1, Duygu
Besnili Acar1, Sinan Uslu1
1
Sisli Hamidiye Etfal Hospital, Neonatal Intnesive Care
Unit, Istanbul, Turkey
2
Sisli Hamidiye Etfal Hospital, Pediatric Cardiology
Department, Istanbul, Turkey
Background&Aims: In this study, we aimed to present
our experience about central venous catheterization and
evaluate the utility of the central venous catheterization
in newborns over last 3 years followed-up at our
institution.
Methods: The charts of patients were reviewed
retrospectively between January 2012 and December
2014. Demographic statistics, catheterizations and
laboratory test results investigated for each patient.
All of the catheterizations performed by pediatric
cardiologist and neonatologist. We present access sites,
duration of stay and complications of catheterizations.
C-reactive protein (CRP),BUN, creatinin, ALT, AST,
Hemoglobin, platelet and white blood cell (WBC) counts
were measured before catheterization and after 7 days
catheterization.
Results: In this time period we performed 30 central
vein catheterizations to 23 patients. 27 catheters were
implanted successfully. Of the 23 patients, 11 were
female (%47,8); they had a mean gestational age of
34,2+4,7 weeks and mean body weight was 3225+1733
grams. Mean catheter implantation day was postnatal
26,2+13,4 days (12-80). Of the 23 patients, 5 (%21,7)
were extremely low birth weight infants, 3(%13) were
with multiple anomalies, 4(%17,4) were pneumonia
and 5(%21,7) werecongenital heart disease. Site of the
catheters; 11 (%40,7) right subclavian, 10(%37) left
subclavian, 3 (%11,1) left femoral and 3 (%11,1) right
femoral veins have been used. In total, 8,3+4,0 (5-18)
duration of catheter days were analyzed. There wasn’t
significant difference between duration of catheter
days and catheter insertion site(p=0.315). There was
2(%7,4) significant catheter related complication;
venous thrombosis and pneumothorax. There wasn’t
significant difference between catheter complication and
catheter insertion site(p=0,947). There wasn’t significant
difference between 1st and 7th day of laboratory tests.
Conclusions: In this study life threatening complications
due to central venous catheterization hasn’t been
determined. Therefore this kind of catheterization
should be helpful for painful treatments and decreasing
workloads.
Keywords: central vein catheterization, newborn
[P-244]
Heterotopic gastric mucosal patch in the
distal esophagus – case report
Vasile Valeriu Lupu1, Ancuta Ignat1, Gabriela Paduraru1,
Angelica Cristina Marin1, Anamaria Ciubara2, Marin
Burlea1
1
Pediatrics Department, University of Medicine and
Pharmacy “Gr. T. Popa”, Iasi, Romania
2
Psychiatry Department, University of Medicine and
Pharmacy “Gr. T. Popa”, Iasi, Romania
Heterotopic gastric mucosa (HGM) represents a congenital
anomaly consisting of abnormally placed gastric mucosa
outside of the stomach. It can be found almost anywhere
within the gastrointestinal tract. HGM is most commonly
found in the upper esophagus. The diagnosis of HGM
is confirmed via endoscopy with biopsy. On endoscopy,
HGM is clearly distinct from the esophageal squamous
mucosa. HGM located in the distal esophagus needs
differentiation from Barrett’s esophagus. Barrett’s
esophagus is a well-known premalignant injury for
adenocarcinoma of the esophagus. Despite the benign
nature of HGM, important complications have been
reported. Malignant progression of HGM occurs in a
stepwise pattern, following the metaplasia–dysplasia–
adenocarcinoma sequence.
We present a rare case of a teenage girl with HGM
located in the distal esophagus, associated with chronic
gastritis and biliary duodenogastric reflux. Endoscopy
combined with biopsies is a mandatory method in clinical
evaluation of metaplastic and nonmetaplastic changes
within HGM of the esophagus.
- 118 -
Keywords: heterotopic gastric mucosa, teenager,
Barrett’s esophagus
[P-245]
Osteogenesis imperfecta – case report
Gabriela Paduraru, Marin Burlea, Ancuta Ignat, Angelica
Cristina Marin, Nadia Al Namat, Vasile Valeriu Lupu
Pediatrics Department, University of Medicine and
Pharmacy “Gr. T. Popa”, Iasi, Romania
Osteogenesis imperfecta (OI), a secondary cause of
osteoporosis, mainly manifests as bone fragility. It
is an inherited disorder of connective tissue integrity;
it affects up to one in 10 000 persons. Diagnosis of
mild OI is challenging, due to its variable phenotypic
expression and inconstant course. Family physicians
must maintain a high index of suspicion, as diagnosis,
along with proper follow up and counseling, so
many complications of this disorder can be avoided.
We report the case of an 11-year-old female presented
with OI Type IV. From the past medical history, we
mention more than twenty fractures, shoulder contusion,
proximal bilateral radio-cubital synostosis, dextroconvex
dorsal scoliosis, tuberosity of left anterior tibia and
pectus excavatum, delay in language development,
polymorphic dyslalia, minor right bundle branch block,
nocturnal enuresis, psychomotor retardation, mental
deficiency, weight loss.
OI most often presents after infancy and should be
considered, whenever children or adults have recurrent
fractures. Early diagnosis of this disease by family
physicians will enable initiation of therapy, as well as
patient education regarding management of modifiable
risk factors linked with osteoporosis (e.g. diet, smoking,
alcohol). For many, disease detection can prevent the
trauma of separation of parents and children, when OI
is misdiagnosed as child abuse. Genetic counseling and
family screening could also be offered. The treatment
plan should maximize mobility and function, increase
peak bone mass and muscle strength, and employ as
much exercise and physical activity as possible.
Keywords: osteogenesis imperfecta, fractures, child
[P-246]
A Patient With Atypical Kawasaki Disease:
Presented With Arthritis And Hydrops Of
Gallbladder
Yasemin Ataman, Ayşegül Doğan Demir, Nilüfer Göknar,
Dilek Karacanoğlu
Bezmialem Vakıf Üniversitesi Tıp Fakültesi Hastanesi,
Çocuk Sağlığı ve Hastalıkları Anabilim Dalı
Introduction: Kawasaki disease (KD) is an acute
systemic vasculitis of unknown etiology that primarily
affects infants and young children. The diagnosis is
based on five of six findings; fever persisting for more
than 5 days, conjunctuvitis, stomatitis, indurative
dermatitis in the extremities, erythematous rash and
lymphadenopathy. The most common complications are
coronary arteritis or coronary aneurysm or thrombotic
occlusion of the coronary vessels. There are some
patients, who do not fulfill the classic diagnostic criteria
for KD and have atypical clinical presentations, called
atypical (incomplete) KD.
In this case we present a patient with atypical KD,
presented with arthritis and hydrops of gallbladder.
Case: A 3-year-old boy had been referred with complaints
of fever, jaundice, pain and swelling in his knees and
proximal and distal interphalangeal joints. Physical
examination revealed jaundice, bulbar conjunctival
injection, erythematous lips and arthritis of the bilateral
knees and proximal and distal interphalangeal joints
of upper and lower extremities. In laboratory tests,
the white blood cell account (WBC), the erythrocyte
sedimentation ratio (ESR), serum bilirubin levels and
liver function tests were found higher. Abdominal
ultrasound showed hydrops of the gallbladder. On the
fifth day of the fever, an echocardiogram was performed,
coronary artery dilatation was revealed. The patient was
diagnosed with KD and intravenous immunoglobulin
(2 gr/kg) and aspirin (100 mg/kg/day) were instutied.
After IVIG therapy his clinical conditions and laboratory
results became better and he was discharged on the
10th day of hospitalization.
Discussion: It is important to recognize and treat
atypical KD because these patients carry a risk of
coronary artery aberrations similar to complete KD. For
this reason, physicians should suspect and diagnose
patients with KD prematurely, even though they do not
meet the complete diagnostic criteria for preventing the
potentially life threatening complications of coronary
aneurysm.
Keywords: Arthritis, Atypical Kawasaki Disease,
Hydrops of Gallbladder
[P-247]
Oksipital lokalized congenital tufted angioma
Yasemin Ersözlü1, Ayşe Bozkurt Turhan2, Murat Vural3,
Deniz Arık4, Tuğba Barsan Kaya5, Özcan Bör6, Neslihan
Tekin5
1
Eskişehir Osmangazi Üniversitesi Tıp Fakültesi
Hastanesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı
Eskişehir
2
İstanbul Medeniyet Üniversitesi Göztepe Eğitim
Araştırma Hastanesi Çocuk Sağlığı ve Hastalıkları
Anabilim Dalı Çocuk Hematoloji Bilim Dalı istanbul
3
Eskişehir Osmangazi Üniversitesi Tıp Fakültesi
Hastanesi Beyin Cerrahisi Anabilim Dalı Eskişehir
4
Eskişehir Osmangazi Üniversitesi Tıp Fakültesi
Hastanesi Patoloji Anabilim Dalı Eskişehir
5
Eskişehir Osmangazi Üniversitesi Tıp Fakültesi
Hastanesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı
Yenidoğan Bilim Dalı Eskişehir
6
Eskişehir Osmangazi Üniversitesi Tıp Fakültesi
Hastanesi Çocuk Sağlığı ve Hastalıkları Anabilim Dalı
Çocuk Hematoloji ve Onkoloji Bilim Dalı Eskişehir
Background&Aims: Tufted angioma is a rare cutaneous
angiomatous tumor usually occurring between the ages
of one and five years, and involving both sexes equally.
Congenital onset and diagnosis at birth is a rare condition.
In this case report we presented a newborn who had
tufted hemangioma located on the occipital region of the
head. Diagnosis was confirmed pathologically showing
presence of vessels grouped in glomerulus-like patterns
- 119 -
with the aspect of a “cannon bullet”.
Case: A male newborn was delivered at 381/7 weeks
with C-section who had tumoral formation localized at the
occipital region antenatally. Fetal MRI in 26.GW reported
that the tumor was 33x20 mm and hemanjiomatous
feature. The baby was followed in the Neonatal Intensive
Care Unit for diagnosis and treatment. It was reported
as vascular in postnatal doppler USG. Cerebral MRI
revealed a hypervascular mass with 4x2x4,5 cm in size.
The patient was consulted with Pediatric Neurosurgery
and Oncology and operated on postnatal 13th day. The
mass was confirmed as tufted angioma by pathological
examination.
Discussion: Tufted angioma is a benign angiomatous
formation
may
interfere
with
kaposiform
hemangioendothelioma. Both seen in children showing
similar immunophenotype and histological examination
shows focal glomerular structure and lymphoid infiltrating
nodules. Several treatment options are available which
has a high recurrence after surgical excision. Regression
with systemic corticosteroids, immunosuppressive
therapy and subcutaneous interferon alfa have been
shown. Follow up is important for tufted hemangioma
cases.
Keywords: tufted, hemangioma
[P-248]
RASopaties: two case reports
Yasemin Kendir Demirkol1, Zeynep Alp Ünkar2, Hülya
Özdemir2, Hülya Selva Bilgen2, Huriye Nursel Elçioğlu1
1
Division of Pediatric Genetics, Derpartment of
Pediatrics, Marmara University Medical Faculty,
İstanbul, Turkey
2
Division of Neonatology, Derpartment of Pediatrics,
Marmara University Medical Faculty, İstanbul, Turkey
Introduction: The RASopathies are a relatively common
group of phenotypically similar and genetically related
autosomal dominant genetic syndromes including Noonan
syndrome (NS) and Noonan syndrome with multiple
lentigines (NSML, formerly LEOPARD syndrome). NS and
NSML can be difficult to differentiate during infancy, but
the presence of multiple lentigines, cafe au lait spots,
and specific cardiac defects facilitate the diagnosis.
Case 1: A 17-day-old male infant was referred for
neonatal intensive care unit because of jaundice. The
patient was born via caesarian section with 2270gr
weight to a 38-year-old gravida 4, parity 4 mother.
The parents are not consanguineous but they are from
same village. On physical examination his weight was
3220gr (<3rd centile) his length 50cm (-3,-4 SD), and
his occipito-frontal diameter 36cm (-3,-4 SD). He had
characteristic features of NS along with mild pulmonary
valve stenosis (PS) and atrial septal defect (ASD). His
karyotype analysis was 46,XY. In exon 13 of the PTPN11
gene p.GLN506Pro(c.1517A>C) a heterozygote mutation
was found, classified as NS.
Case 2: A 5-month-old female was referred for
thromboytopenia, splenomegalia and dismorfic feature.
The parents were nonconsanguineous. On physical
examination her weight was 4360gr (<3rd centile),
height 56cm (-4 SDS), and head circumference 39cm
(-2,-3SDS). She had low-set ears, short neck, bilateral
mild ptosis, 3/6 systolic murmur, 2cm hepatomegaly and
4cm splenomegaly. Echocardiography revealed ASD and
PS. In exon 3 of the PTPN11 gene p.Thr7211e(c.218C>T)
heterozygote mutation was found, identified as NS.
Discussion: The RASopathies are disorders with an
extremely wide spectrum of clinical manifestations and a
heterogenous moleculer pathogenesis. Long-term follow
up of patients is required to evaluate the evolution of
the clinical disease expression and confirm the initial
diagnosis. Despite significant advances in moleculer
diagnostics, there are still problems with finding the
correct clinical diagnosis of the diseases from the group
of the RASopathies.
Keywords: RASopathies, Noonan Syndrome
[P-249]
A case of Split Notochord Syndrome:
presenting with respiratory failure
Yeşim Coşkun1, İpek Akman1, Mustafa Kemal Demir2,
Özlem Yapıcıer3, Salih Somuncu4
1
Pediatrics Department, Bahcesehir University School of
Medicine, Istanbul, Turkey
2
Radiology Department, Bahcesehir University School of
Medicine, Istanbul, Turkey
3
Pathology Department, Bahcesehir University School of
Medicine, Istanbul, Turkey
4
Pediatric Surgery Department, Bahcesehir University
School of Medicine, Istanbul, Turkey
Split notochord syndrome (SNS) is a very rare congenital
anomaly. In this report we described a male newborn
with a neuroenteric cyst in the posterior mediastinum
and multiple vertebrae anomalies presenting with
respiratory failure and pulmonary hypertension.
A 12 days old full term boy, weighing 2920 gram, was
referred to the Neonatal Intensive Care Unit of Bahcesehir
University School of Medicine Goztepe Medicalpark
Hospital with respiratory failure. At birth, he was
hospitalised because of dyspnea and tachypnea. The first
diagnosis was congenital pneumonia until he was unable
to be weaned from the mechanical ventilation and the
cyst in the right lung was seen in a chest computerized
tomography.
Physical examination showed severe respiratory failure,
decreased breath sounds on the right lung. The AP
chest radiograph showed a large, well-defined mass
in the right hemitorax, displacing the mediastinum to
the right, and cervico-thoracic vertebral anomalies. The
abdominal magnetic resonance imaging findings showed
a homogeneous unilocular high signal cystic mass in
the right prevertebral region without extension to the
spinal canal. Vancomycin and meropenem were the
antibiotic treatment. For the pulmonary hypertension
sildenafil was given. He was sent pediatric surgery for
the operation of the cyst in his right lung. The mass was
in the posterior mediastinum and it was removed totally.
The histopathological finding revealed as Neuroenteric
cyst.
He was discharged from the hospital at 53 days of age.
Since SNS is seen very rarely, the management of SNS
- 120 -
must be tailored on the different anomalies present in
each case. In the present case excision of cyst resulted
in improvement of respiratory symptoms. Early diagnosis
and treatment improves prognosis.
Keywords: split notochord syndrome, neuroenteric
cyst, respiratory failure
[P-250]
A rare cause of vomiting: Annular pancreas
Yasin Şahin1, Yusuf Can Doğan2, Hasret Ayyıldız Cıvan1,
Şenol Emre3, Osman Uzunlu3, Gonca Topuzlu Tekant3,
Hatice Arıöz Habibi4, İbrahim Adaletli4, Fügen Çullu
Çokuğraş1, Tufan Kutlu1, Tülay Erkan1
1
Istanbul University Cerrahpasa Faculty of Medicine,
Depatment of Pediatrics, Division of Gastroenterology,
Istanbul, Turkey
2
Istanbul University Cerrahpasa Faculty of Medicine,
Depatment of Pediatrics, Istanbul, Turkey
3
Istanbul University Cerrahpasa Faculty of Medicine,
Depatment of Pediatric Surgery, Istanbul, Turkey
4
Istanbul University Cerrahpasa Faculty of Medicine,
Division of Pediatric Radiology, Istanbul, Turkey
Background & Aim: Anular pancreas is a rare congenital
anomaly which consists of a ring of pancreatic tisue partially
or completely encircling the second part of duodenum. It
may affect anyone from the neonatal period to the adult.
It is difficult to diagnose because it can present in a wide
range of clinical conditions. Although the cases are also
reported in adults, the symptomatic cases often refer
in infancy or early childhood. Here, we report a case of
duodenal obstruction associated with anular pancreas,
which has rarely been reported in late adolescent period.
Case: The 17 years female patient has had a non- bilious
vomiting accompanied with abdominal pain especially
5-10 minutes after meals for seven years. She has lost
7.5 kg in last 2 months. Esophagogastroduodenoscopy
was performed for etiological evaluation. The endoscopic
appearance of upper esophagus was dirty yellow color
and biopsy was taken. The microbiological analysis of
the tissue example showed candida infection. The distal
part of duodenum after bulbus could not be visualised,
referring to obstruction. The contrast X-ray imaging of
the upper gastrointestinal tract showed no passage of
contrast material through the bulbus. These findings of
obstruction were aproved by contrast-enhanced CT-scan.
The diagnosis of annular pancreas was considered. Then,
this diagnosis was approved by operation. ’’Diamond
shaped duodeno-duodenostomy’’ was carried out.
Conclusion: Annular pancreas which may be seen at
any age by taking into account, should be considered
in the diagnosis of patients with non-bilious vomiting
particularly after meals for a long time.
Keywords: vomiting, annular pancreas, child
[P-251]
Raoultella terrigena, a rare pathogen in
neonatal intensive care unit
Zeynep Alp Ünkar1, Gülşen Akkoç2, Ebru Eren3, Hülya
Özdemir1, Aslı Memişoğlu1, Nurver Toprak3, Ahmet
Soysal2, Hülya Selva Bilgen1, Eren Özek1
1
Marmara University Faculty of Medicine, Department of
Pediatrics, Division of Neonatology
2
Marmara University Faculty of Medicine, Department of
Pediatrics, Division of Pediatric Infectious Diseases
3
Marmara University Faculty of Medicine, Department of
Microbiology
Raoultella terrigena (formerly Klebsiella terrigena) is a
gram-negative, oxidase-negative bacillus, belonging to
the family Enterobacteriaceae. First isolated in 1981
from soil and water, it’s a rare pathogen in human
hosts. There are only two adult cases in the literature
– one endocarditis and one sepsis – reported so far and
a congress abstract of neonatal sepsis in the neonatal
intensive care unit (NICU) in Gaza, reporting its incidence
as 25.5% among Klebsiella species. We present the
first neonatal case of R. terrigena sepsis from Turkey.
The first-born of thirty gestational-week-old dizygotic twin
boys was intubated and given surfactant in the delivery
room. He was transferred to NICU where umbilical
venous catheter was inserted and empiric ampicillin
gentamycin therapy was started. He was extubated in
18 hours and didn’t need any form of assisted ventilation
later on. Enteral feeding was initiated on the 2nd day of
life (DOL). On DOL 8 he presented with increased gastric
residuals, hyperglycemia, cutis marmoratus and apnea
attacks for which he was intubated. Sepsis screening
was positive with increased C-reactive protein (CRP)
and procalcitonin (PCT) levels. Cerebrospinal fluid (CSF)
analysis was normal. Blood, urine and CSF cultures were
taken. Considering the unit’s flora, empirical vancomycin
and meropenem were started. Blood culture revealed
R. terrigena resistant only to ampicillin and sensitive
to amoxicillin-clavulanate, gentamycin, cefuroxime,
cefuroxime-axetil and trimethoprim-sulfamethoxazol.
Meropenem was switched to cefepime. CRP and PCT
decreased gradually and the baby improved clinically.
Patient was extubated on the third day of antibiotics
and whole therapy was completed to 21 days.
Clinical significance and pathogenesis of R. terrigena
is still uncertain. Despite occurrence in human clinical
samples and colonization of feeding tubes, there are few
case reports about human infections. Considering that
feeding tubes are commonly used in NICU, attention
should be paid to this seemingly-rare gram-negative
pathogen.
Keywords: Raoultella terrigena, neonatal sepsis, NICU
- 121 -
[P-252]
Double aneuploidy: Down - Klinefelter
Syndrome
Zeynep Alp Ünkar1, Yasemin Kendir Demirkol2, Aslı
Memişoğlu1, Huriye Nursel Elçioğlu2, Eren Özek1
1
Division of Neonatology, Derpartment of Pediatrics,
Marmara University Medical Faculty, İstanbul, Turkey
2
Division of Pediatric Genetics, Derpartment of
Pediatrics, Marmara University Medical Faculty,
İstanbul, Turkey
Background: Trisomy 21, also named Down syndrome
(DS), is caused by the presence of an additional
autosome, affecting 1/700 live births. Klinefelter
Syndrome (KS) is a genetic condition in which humans
have an extra X chromosome, resulting in a 47,XXY
karyotype. Double Aneuploidy involving both autosomal
and sex chromosomes is rarely described. Presence of
coincidental two meiotic non-disjunctional events, can
lead to double aneuploidy which called as trisomy of two
different chromosomes.
Case: A 3-month-old male, born to non-consanguineous
parents referred to our polyclinic because of dysmorphic
features. Mother’s age was 30 years, and father age
was 28. Physical examination showed flat occiput,
hypertelorism, low-set ears, high narrow palate and
hypotonia. Chromosomal analysis showed a karyotype
48, XXY+21, a double aneuploidy of DS and KS.
Conclusion: In conclusion, Down-Klinefelter syndrome
is a rare occurrence. It presents trisomy 21 characteristics
early in life and klinefelter syndrome features after 10
months of age.
Keywords: Down Syndrome, Klinefelter Syndrome,
double aneuploidy
[P-253]
Can Mycoplasma pneumoniae be a trigger for
Type 1 Diabetes?
Zeynep Hızlı1, Zehra Yavaş Abalı2, Hacer Aktürk3, Ahmet
Uçar2, Şükran Poyrazoğlu2, Firdevs Baş2, Ali Ağaçfidan4,
Rüveyde Bundak2, Feyza Darendeliler2, Ayper Somer3
1
Istanbul University Faculty of Medicine, Istanbul,
Turkey
2
Department of Endocrinology, Istanbul University
Faculty of Medicine, Istanbul, Turkey
3
Department of Infection, Istanbul University Faculty of
Medicine, Istanbul, Turkey
4
Department of Microbiology, Istanbul University Faculty
of Medicine, Istanbul, Turkey
Background: Type 1 diabetes mellitus (T1DM) is a
multi-factorial disease. One of the possible theory
for disease is various viruses do play a role in the
pathogenesis in individuals with genetic susceptibility.
Other environmental factors were reported in many
studies. Bacterial pathogens also suggested as a trigger.
In this report, we presented a child with T1DM presenting
with Mycoplasma pneumoniae infection.
Case: A 10-year-old boy was referred by an
otolaryngologist because of weight loss, after diagnosis
of right acute otitis media and left tympanic membrane
perforation. He had polydipsia, polyuria and enuresis
nocturne. His height was 135.5 cm (-0.20 SDS), weight
40.6 kg (1.33 SDS) and body mass index 22.2 kg/m2
(1.68 SDS). He had tachycardia (125/min), tachypnea
(36/min) and his blood pressure was 95/60 mmHg.
He had no fever. He had hyperglycemia (408 mg/dL),
ketonuria and metabolic acidosis (pH: 7.09 HCO3: 5.1
mmol/L). Basal insulin and C-peptide levels were 2.2
μU/mL and 0.3 ng/mL, respectively. HbA1c was 12.8%.
Ketoacidosis was corrected in 18 hours and subsequently
treated with subcutaneous insulin. Specific serum tests
for diabetes; glutamic acid decarboxylase antibody –
GADA 26.9 U/mL (<1.0), islet antibody-IA 6.13 U/
mL (<1.0) and HLA- DRB1-DR3&DR4 genotypes were
positive. At the same time his nasopharyngeal swab for
M. pneumoniae DNA was detected positive by polymerase
chain reaction (PCR). Also positive M. pneumoniae IgM
and IgG antibodies identified by enzyme-linked immune
assay (EIA). These findings suggest that acute otitis
media caused by M.pneumoniae which is a rare pathogen
for this infection, triggered diabetic ketoasidosis.
Conclusion: As far as we know, this is the first report
of an association between DKA and M. pneumoniae
infection. We suggest that infection should be kept
in mind developing of T1DM. Further study is needed
to elucidate the relationship between T1DM and M.
pneumoniae infection.
Keywords: M. pneumoniae, Type 1 diabetes
[P-254]
Results of the Hearing Screening Tests of
Newborn Children for 3 years
Adil Umut Zübarioğlu, Sinan Uslu, Zeynep Işcan, Ali
Bülbül, Duygu Besnili Acar, Evrim Kıray Baş
Department of Neonatology, Sisli Hamidiye Etfal
Education and Research Hospital, Istanbul, Turkey
Background&Aims: To share data which showed up
at the end of the hearing screening tests on newborn
children at our hospital.
Methods: The work was carried out on 4438 newborn
children, who were born at our hospital and near hospitals,
at Şişli Hamidiye Etfal Hospital between July 2012 and
July 2015. The newborns who were not responsive to
unilateral and bilateral emissions were invited to control
for 15 days later. At first control, newborns who passed
TEOAE(Transient Evoked Otoacoustic Emission) test
for both sides of ears are determined as they passed
the hearing test. The newborns who failed TEOAE test
at second control were called for third control and
performed ABR(Auditory Brainstem Response) on them.
Results: TEOAE test was performed for 8876 newborn
children which was the 91 percent of all 9754 newborns.
969 (10.9%) newborns who failed the test were called
for checking. 311 newborn (3.2%) of recalled ones failed
the second test and they were called for the ABR test.
At the end of the ABR test, 71(0.8%) newborns were
dispatched on the suspicion of hearing loss. It’s important
to say that 52(0.6%) newborns are detected as patients
of unilateral hearing loss and 19(0.2%) newborns are
detected as patients of bilateral hearing loss.
- 122 -
Conclusions: The hearing screening tests of newborn
children are noninvasive and quick tests. Thanks to
these tests, cases of hearing loss which can be detected
at at least 2-3 ages of children, can be determinated
and started treatment at first 3 months of newborns.
However, these tests must become widespread and must
be performed on all newborn children.
Keywords: hearing loss, screening
[P-255]
Transient Pseudohypoaldestronism in an
infant with urinary tract infection associated
with urinary tract malformation
Zeynep Karakaya, Gamze Ozgurhan, Didem Ocal Topcu,
Bulent Hacihamdioglu
Suleymaniye Maternity and Children’s Training and
Research Hospital-Department of Pediatrics -Istanbul,
Turkey
Introduction:
Uncommon
presentation
of
hyperkalemia,hyponatremia and dehydration may be
life threatening in infancy and therefore need immediate
investigation to found out possible causes particularly
congenital adrenal hyperplasia (CAH) and other causes of
hypoaldestronism. Transient pseudo-hypoaldosteronism
(PHA) is a rare condition characterized by renal resistance
to the action of aldosterone; patients exhibit salt wasting
and hyperkalemia associated with high levels of plasma
aldosterone. Here we report a case with transient PHA
caused by urinary tract obstruction associated with
urinary tract infection (UTI).
Case Report: Patient was 4-month-old previously healthy
male,with uncomplicated birth history. There was a history
of hydronephrosis, which was detected on antenatal
screening. He was admitted to our pediatric emergency
department with complaints of fever, poor sucking and
restlessness. Physical examination revealed moderate
dehydration and fever with 38.5oC, other systemic
examinations were unremarkable. Initial blood samples
revealed hyponatremia with sodium level 122 mmol/L
and hyperkalemia with potassium level 6.6 mmol/L.
Urinalysis revealed pyuria with nitrite and urine culture
was positive for Klebsiella pneumonia.Renal sonography
demonstrated bilateral hydronephrosis with megaureters.
Serum sodium and potassium level normalized within 24
hour after intravenous fluids and antibiotic therapy. The
diagnosis of pseudohypoaldosteronism became apparent
when serum aldosterone level obtained on admission was
markedly elevated to 664 ng/dl (reference range 3.7-43).
Discussion: Transient PHA is a condition that presents
with hyponatremia and hyperkalaemia. The initial
biochemical picture is similar to adrenal hypoplasia, CAH
or secondary PHA. UTI with or without any underlying
malformation may also cause transient renal tubular
resistance to aldosterone, manifested by low sodium and
high potassium level. This condition should be kept in mind
when infants present with salt wasting or hyperkalemia.
Urinalysis and renal ultrasonography should always be
performed in order to allow early recognition of transient
PHA to avoid unnecessary treatment.
Keywords: Transient Pseudohypoaldestronism, Urinary
Tract Infection
[P-256]
A child with methamphetamine Intoxication
Asli Kibris1, Zeynep Karakaya1, Gamze Ozgurhan1,
Oznur Vermezoglu1, Suray Aliskan2
1
Suleymaniye Maternity and Children’s Training and
Research Hospital,Department of Pediatrics,Istanbul
Turkey
2
Beylikduzu Medilife Hospital, Pediatric ICU, Istanbul
Turkey
Introduction: Acute altered level of consciousness
(LOC) is the common presenting complaint to pediatric
emergency department.It is usually emerged by toxic,
metabolic and structural causes. Methamphetamine
being a rare cause of acute altered LOC in intoxicated
cases,may cause hypertension, tachycardia, severe
agitation and psychosis. It is a synthetic psycho stimulant
and hallucinogenic agent that can be easily manufactured
with low cost via readily available chemicals at meth
labs prepared at home.Here we report a child evaluated
by altered LOC and diagnosed as methamphetamine
Intoxication.
Case Report: 26-month-old female child, admitted
to pediatric emergency department with presenting
complaints of acute onset of lethargy and agitation.Past
medical history of patient was unremarkable.On physical
examination;patient was lethargic and pupils were
dilated. Her blood pressure was 95/59 mmHg, heart
rate was 140/min, capillary filling time was prolonged
and axillary temperature was 36.5 C. Other systemic
examinations were unremarkable. Urine toxicology
screen was positive for methamphetamine and she was
diagnosed as methamphetamine intoxication. Other
laboratory tests were found insignificant and cranial BT
was found normal. She was followed in pediatric intensive
care unit as intubated because of apneic breathing and
then discharged after three days with full recovery.
Conclusion: With the growing prevalence of
methamphetamine use and production in home
laboratories, children are at high risk of injuries resulting
from living in a drug-endangered environment. We
present a case of a child harmed by the production of
methamphetamine at home. Acute alteration of LOC
in otherwise known healthy patients,without history of
previous trauma, belonging conflicting and contradictory
parents are subjected to be investigated for intoxication.
Although methamphetamine intoxication is rare but
should be kept in mind by pediatricians.
Keywords: Methamphetamine Intoxication, Stimulant
Toxicty in Children
- 123 -
[P-257]
And thrombus formation in dilated atrium is also a rare
complication. Herein we present a patient with restrictive
cardiomyopathy.
A patient with pseudo vitamin D-deficiency
rickets
Gamze Ozgurhan, Zeynep Karakaya, Pınar Selimoglu,
Bulent Hacıhamdioglu
Suleymaniye Maternity and Children’s Training and
Research Hospital -Department of Pediatrics-Istanbul
Turkey
Pseudo vitamin D-deficiency rickets (PDDR) (Vitamin
D-dependent rickets Type I) is an autosomal recessive
disorder characterized by hypocalcemia, rickets and low
or undetectable level of serum 1, 25-dihydroxyvitamin
D [1, 25(OH) 2D3]. This disorder results from a defect
in the renal 25-hydroxyvitamin D 1α-hydroxylase, the
key enzyme in the pathway of vitamin D metabolism.
We reported an infant who diagnosed as PDDR.
The 15-month-old female patient presented to our
pediatric endocrinology department with inability to
walk. She used adequate amount of vitamin D3; 400
IU/day till 1-years-of age. There was a history of using
total 600.000 units of oral vitamin D3 just after 1 years
of age due to difficulty in inability to walk. She is the
child of first-cousin marriage, with no known family
history of metabolic bone disease. Physical examination
revealed short stature, frontal bossing, mildly reduced
muscle tone, normal teeth development and widening of
the wrist. There was no rachitic rosary, Harrison’s sulcus
or prominent bone deformity. There was no evidence of
latent tetany. Initial investigations resulted as: low serum
calcium (5.9 mg/dl, normal 8.5-10.5) and phosphorus
(3.4 mg/dl, normal 4.2-6.4), high serum parathyroid
hormone (PTH) (925 pg/ml, normal 15-65), high serum
alkaline phosphatase level (2531 IU/l, normal 124-332
U/L), a normal serum 25OHD3 (41 ng/ml), and very low
level serum 1,25(OH)2D3 (<3.67 ng/ml, normal 25-153
ng/ml) suggesting 1α-hydroxylation defect. Radiological
workup showed findings of generalized fraying and
cupping at the metaphysis. Calcitriol and oral calcium
treatment was initiated with a clinical diagnosis of PDDR.
Children with inability to walk should be investigated
for rickets by pediatricians. PDDR usually presents with
rachitic changes nonresponsive to Vitamin D3 treatment
with low circulating levels of 1, 25-Dihydroxyvitamin
D3. It must be distinguished from nutritional rickets and
vitamin D-dependent rickets Type II in order to plan
appropriate therapy.
Keywords: Pseudo vitamin D-deficiency rickets,
Vitamin D-dependent rickets Type I
[P-258]
A Rare Complication of Restrictive
Cardiomyopathy in Pediatric Age Group
Sertac Hanedan Onan, Ozgul Yigit, Zubeyde Kilinc
Yaprak, Sultan Bent, Leyla Besel, Erkut Ozturk, Alper
Guzeltas
Bagcilar Training and Research Hospital, Pediatric
Department, Istanbul, Turkey
Background&Aims:
Idiopathic
restrictive
cardiomyopathy is a rare disease in children and is
characterized by ventricular diastolic dysfunction with
dilated atria and normal ventricular size and thickness.
Case: A 39 months-old Syrian refugee girl, with previous
history of restrictive cardiomyopathy and three siblings’
death by heart disease, presented to the emergency room
complaints with swelling of legs and difficulty to breath.
On physical examination, she appeared orthopneic and
dispneic. BPM: 45/min, HR:150/dk, BP: 105/55 mmHg.
There was 3/6 pansystolic murmur on the apex and left
lower sternal border. She had a distanded abdomen and
a massive hepatomegaly with liver edge 8 cm below the
right costal margin. Her legs are cold and pitting edema
was determined. In telecardiography cardiothoracic
index was increased. ECG showed sinus tachicardia, low
voltage, P-mitrale and P-pulmunale. In Echocardography
biventricular restriction,biatrial dilatation, severe mitral
and tricuspid regurgitation were demonstrated. There
was also a giant solid mass detected in the anterior
side of right atrium. Fractional shortening was%39. In
laboratory studies hematocrit and leucocyte count were
normal, platelet count:134.000/mm3 fibrinogen:117mg/
dl(normal values:150-400mg/dl), D-dimer: 9,60ug/
L(normal values:0-0.5ug/L). These findings suggested
right atrial thrombus and low moleculer weight heparin
therapy was started. She has been using furosemide,
captopril and spironolactone for fifteen months till the
first diagnosis. And there was no history of dysrhythmia.
Anticoagulant therapy was not used because of difficulties
in close monitorization.
Conclusions: We report this case to discuss the
management of atrial thrombus formation in restrictive
cardiomyopathy and emphasize the thrombus formation
can be seen in pediatric age group even during sinus
rythym.
Keywords: Restrictive Cardiomyopathy, thrombus
[P-259]
Vitamin D deficiency in newborns and
mothers
Abdullah Sert, Ozgul Yigit, Gamze Yildirim, Meltem Erol,
Ozlem Bostan Gayret, Zubeyde Kilinc Yaprak
Bagcilar Training and Research Hospital, Pediatric
Department, Istanbul, Turkey
Background&Aims: Vitamin D status at birth and
during early infancy, when breast milk is the predominant
source of nutrition, is determined by maternal vitamin
D status. The aim of the study is to determine the
parameters effecting the maternal vitamin D levels and
the correlation between maternal and newborns’ vitamin
D levels.
Methods: Between January 2015 and March 2015,
108 neonates, who were born in Bağcılar Training and
Resourch Hospital obstetrics clinic, and their mothers
were included to the study. We questioned the women
about dressing style(covered vs uncovered), exposure to
sunlight, vitamin supplements usage and consumption
of fish and milk products. And blood samples for Vitamin
D levels were obtained both women and their babies.
The levels of 25-(OH) D were classified in four groups (<
- 124 -
5ng/mL, 5-10ng/mL, 10-15ng/mL, >15ng/mL).
Results: We include 108 mother and newborn couple
to study. de women were dressed covered (%94),
exposure to sunlight was less than 2 hour in %88 of the
women. The usage of vitamin D supplements was %64
(n=69) and the consumption of fish and milk pruducts
were %76, %87 respectively. The 25-(OH)D levels was
<5ng/mL in %81 of women, and %75 of newborns. Only
significant correlation determined between mother and
newborn 25-(OH)D levels(p<0.05)
Conclusions: Newborn’s vitamin d levels reflects their
mother’s levels. Adequate vitamin d stores in women
would prevent diseases caused by vitamin d deficiency
in early infancy.
Keywords: Vitamin d, mother, infant
[P-260]
Gastritis in Childhood
Ancuta Ignat
Pediatrics Department, University of Medicine and
Pharmacy “Gr. T. Popa”, Iasi, Romania
Gastritis is acute or chronic, diffuse or focal inflammation
of gastric mucosa. Gastroduodenal ulcer, as well as
gastritis is a multifactorial disease characterized by
limited loss of substance to the mucosa, submucosa
and muscle caused by imbalance of mucosal protection
mechanisms and aggressive factors. Bacterial infection,
nonsteroidal anti-inflammatory drugs, bile reflux,
autoimmunity or allergic response are pathogenic links
in children with gastroduodenal diseases. H. pylori
gastritis is a primary infection of the stomach and is
the most frequent cause of chronic gastritis. Chronic
reactive chemical gastritis is associated with long-term
intake of aspirin or NSAIDs. Noninfectious diseases are
the usual cause of gastric granulomas; they include
Crohn disease, sarcoidosis, and isolated granulomatous
gastritis. Eosinophilic gastritis can be part of the spectrum
of eosinophilic gastroenteritis. Radiation gastritis usually
occurs 2-9 months after initial radiotherapy. Ischemic
gastritis is believed to result from atherosclerotic
thrombi arising from the celiac and superior mesenteric
arteries. Clinical data, especially the increasing quality
and accuracy of paraclinical diagnosis, result in a low
rate of complications of these disorders, through the use
of constantly improved multimodal treatment strategies.
loss of weight and stature. Also, H. pylori is involved in
diabetic patients’ status and food allergies. Children differ
from adults with respect to H. pylori infection in terms
of the prevalence of the infection, the complication rate,
the near-absence of gastric malignancies, age-specific
problems with diagnostic tests and drugs, and a higher
rate of antibiotic resistance. Increasing the quality and
accuracy of detection of infection with H. pylori causes a
low rate of complications of these diseases by applying
constantly improved treatment strategies.
Keywords: Helicobacter pylori, digestive disease,
extradigestive diseases, child
[P-262]
Particularities of the Gastroesophageal
Reflux Disease in Childhood
Vasile Valeriu Lupu
Pediatrics Department, University of Medicine and
Pharmacy “Gr. T. Popa”, Iasi, Romania
Gastroesophageal reflux (GERD) is a frequent entity in
the pediatrics that is still underdiagnosed (“the third
millennium’s disease”). The gastroesophageal reflux
disease represents the intermittent or permanent
passing of the stomach content into the esophagus,
which triggers a whole set of digestive, respiratory
and/or neurobehavioral symptoms or the absence of
any symptoms. GERD occurs in approximately 1 in 300
infants. Clinical polymorphic symptoms are not only
dominated by post-food vomiting, but, in many cases,
by respiratory symptoms (night coughing at episodes
of apnea, recurrent wheezing, aspiration pneumonia
and even sudden death syndrome). Frequent, effortless
regurgitation of feeds is common during early infancy.
GERD has triple therapy: general measures, surgical and
pharmacological therapy. The initial drugs (prokinetics
agents) were replaced in the last period with proton
pump inhibitors (PPI), because they are able to cause
the disappearance of the symptoms of reflux in most
cases.
Key words: gastroesophageal reflux, respiratory
manifestations, child
[P-263]
The demographic features and treatment
complications of acute leukemia patients
followed by hematology clinic between
2004-2008
Adem Karbuz1, Neşe Yaralı2, Pamir Işık2, Ali Bay3,
Abdurrahman Kara2, Bahattin Tunç2
1
Pediatric Infectious Disease, Okmeydanı Education and
Research Hospital, İstanbul, Turkey
2
Ankara Children’s Hematology Oncology Training and
Research Hospital, Ankara, Turkey
3
Gaziantep University Faculty of Medicine Hospital,
Gaziantep, Turkey
Key words: gastritis, gastroduodenal ulcer, child
[P-261]
Helicobacter Pylori Infection in Children
Angelica Cristina Marin
Pediatrics Department, University of Medicine and
Pharmacy “Gr. T. Popa”, Iasi, Romania
Helicobacter pylori (H. pylori) is a gram-negative bacillus
responsible for one of the most common infections
found in humans worldwide. The prevalence is high in
developing countries and the infection is acquired at a
young age. Infection with H. pylori is a cofactor in the
development of three important upper gastrointestinal
diseases: duodenal or gastric ulcers, gastric cancer,
and gastric mucosa-associated lymphoid-tissue (MALT)
lymphoma. Recent research argues and the involvement
of H. pylori in the occurrence of extradigestive diseases.
Chronic infection with H. pylori is associated with anemia,
ABSTRACT
Aim: Our aim was to analyze the patients diagnosed
with acute leukemia and received chemotherapy in
our hospital during five years, retrospectively. Their
epidemiological, clinical and laboratory features were
determined. Also, all the systemic complications of
these patients were reviewed in order to contribute to
therapeutic approaches of such patients.
Methods: A total of 97 patients who were followed
by the pediatric hematology department between
- 125 -
January 2004 and December 2008, were recruited
to our study. Their demographic, clinical, laboratory,
radiological and echocardiographic characteristics were
documented. Finally, 94 of these patients could be
evaluated in terms of all their systemic complications.
Results: The mean age of cases was 71.9 + 5 months
and the age interval was 2-201 months. 65% of the cases
were male and 35% of them were female. The diagnoses
were acute lymphoblastic leukemia in 84.5% and acute
myeloid leukemia in % 15.5 of the patients. While the
most common manifestations were fever (63.9%) and
fatigue (57.7%), the most common physical examination
findings were hepatomegaly (77.3%) and pallor (68%)
at the diagnosis. The complications observed during
chemotherapy in the patients were at least one episode
of febrile neutropenic attack in 90%, five-fold increase in
hepatic enzymes in 80.8%, mucositis in 43.6%, allergy
to l-asparaganiase in 28.7%, and compliance problem
in 9.5%.
Conclusions: The childhood leukemia has a potential
to cause complications in many organ systems due to
the both disease itself and chemotherapy medications
used for the treatment. Multidisciplinary management
and follow up are prerequisites.
[P-263]
Collectıng And Observıng The Growths Of
The Cases Wıth Cleft Lıp And Cleft Palate
Avidan Kızılelma Yiğit1, Şerife Suna Oğuz2
1
Division of Neonatology, Okmeydanı Education and
Research Hospital, Istanbul, Turkey.
2
Division of Neonatology, Zekai Tahir Burak Maternity
Teaching Hospital, Ankara, Turkey.
Objective: Cleft lips and palates are frequent congenital
malformations of the head-neck area. There are no
sufficient data on its frequency in our country. The
purpose of this study is determining the incidence of
cleft lips and palates in the infants born in Zekai Tahir
Burak Maternity Teaching Hospital and evaluating the
growth results of these patients.
Material and Methods: The infants born in Zekai Tahir
Burak Maternity Teaching Hospital in 2010 diagnosed
with cleft lips and/or palates in physical examinations by
the pediatrist were included in the study. By considering
the number of the infants born in the hospital in the
same year, an approximate incidence was determined.
All of the patients were provided with prosthetic nutrition
plates, which were prepared specially for the patients.
They were discharged to be followed in the relevant
departments. The body heights and weights of the
patients who could be reached at the age of four were
recorded and their growth were evaluated.
Results: 17990 live births were observed during the
study; and in 19 cases, cleft lips and palates were
determined. The frequency of the disease was found as 1
infant in every 947 live births. 9 patients could be reached
at the age of 4. Their body heights and weights were
recorded. It was observed according to the percentile
curves formed by Olcay Neyzi et al. that the body height
and weight of 1 patient was below 3 percentile, and the
body heights and weights of the other 8 patients were
below 3-50 percentile.
Conclusion: The frequency of cleft lips and palates
in Zekai Tahir Burak Maternity Teaching Hospital is in
accordance with the literature. The proper treatment
for these frequent malformations in the right time is
necessary for a normal growth.
[P-264]
Successful management of a complicated
orbital cellulitis with ampicillin-sulbactam
monotherapy
Bilge Aldemir-Kocabaş1, Bülent Çekiç2, İclal Erdem
Toslak2
1
Pediatric Infectious Disease, Antalya Education and
Research Hospital, Antalya, Turkey
2
Clinic of Radiology, Antalya Education and Research
Hospital, Antalya, Turkey
Orbital infections require prompt diagnosis and treatment
because of the risk of severe complications such as
subperiosteal and epidural abscesses. Orbital cellulitis
is an infection that concerns soft tissue behind the
orbital septum. Ampicillin-sulbactam is an appropriate
treatment for etiologic agents such as S. aureus, S.
pyogenes, H. influenzae, S. pneumoniae and anaerobs
responsible from orbital cellulitis.
An 8.5 years old girl was hospitalized at an outpatient clinic
for 5 days with complaint of high fever. Subsequently, she
was referred to our clinic wherefore proptosis, swelling,
erythema and warmth of left eyelid occurred. The eyelid
was completely closed. There was no limitation of eye
movements or findings of ophtalmoplegia at presentation.
Laboratory findings were as following: Hemoglobin:
9,9 g/dL, leukocyte: 22,800/mm3, platelets: 890,000/
mm3, sedimentation rate: 118 mm/h, CRP: 28,8 mg/L.
Ampicillin-sulbactam (200 mg/kg/day) and ibuprofen (10
mg/kg/dose, q8hr) were initiated for treatment of orbital
cellulitis. Additionally, orbital CT revealed a subperiosteal
abscess (22X14 mm in size) and epidural abscess. At
the third day of treatment proptosis was started to
resolve and the acute phase reactants regressed to
normal ranges at the end of first week. The duration of
treatment completed to eight weeks because of epidural
involvement. Magnetic resonance imaging revealed a
regression of the subperiosteal abscess enlargement to
4x2 mm in size and disappearing of the epidural abscess.
She discharged from the hospital with oral amoxicillinclavulonate for four weeks. All complications were fully
recovered at the end of 12th of the treatment.
Effective monoterapy is very important for both drug
adverse effects and convenience of use. So, if clinical,
laboratory findings and fever resolve with monoterapy it
is not necessary to change the antibiotic regimen.
Key Words: Orbital cellulitis, epidural abscess,
ampicillin-sulbactam
[P-265]
Wilson Disease
Dr. Kyaw Zin LATT and Dr. Zeynep GÖR
Introduction: Wilson disease is an autosomal recessive
disorder which is related to the copper metabolism and
characterized by degenerative changes in the liver,
brain, cornea and the other tissues. Presented is a 3
year old male patient who admitted to the hospital due
to the icterus of the cornea and skin, who was evaluated
for jaundice and whose serum ceruloplasmin level was
found to be lower than normal.
Case Presentation: A 3-year old male patient was
admitted to the hospital due to the icteric appearance
which started about a week ago. The liver function
tests were found to be elevated (AST: 2949 IU/L ALT:
1762 IU/L ALP 190 IU/L GGT 52 IU/L). The serum total
- 126 -
bilirubin level is 11.63 mg/dL and the direct bilirubin level
was 9.9 mg/dL. The PT was slightly prolonged (16.59
sec.) and INR was 1.46. His peripheric blood film showed
no signs of hemolysis. The abdomen USG revealed
hepatomegaly; liver (134 mm) and the spleen (106.5
mm). The viral hepatitis markers were all negative. The
hepatic portal Doppler USG showed no abnormality. IV
contrasted Abdominal CT revealed slight elevated liver
size (154 mm) and generalized oedema in the periportal
area of the liver. The spleen was slightly enlarged
(102 mm). The alpha 1 antitrypsin level was 170 U/L,
excluding alpha 1 antitrypsin deficiency. Autoimmune
hepatitis markers such as ANA, ASMA and LKM antibody
levels were all negative. Celiac disease antibodies
levels were also negative. Ophthalmologic examination
showed no abnormal findings. The serum ceruloplasmin
level was 15.6mg/dL in favour of the diagnosis of Wilson
Disease. Copper level in the 24 hour urine collection was
163.5mcg/g (high). Liver biopsy was performed and the
hepatic copper content was 586 ug/g (normally <10
ug/g dry weight).
Discussion: Wilson Disease is one of the causes of
hyperbilirubinemia with the incidence of 1/ 100.000
to 1/500.000 birth. However It should be always kept
in the physician’s minds when evaluating a patient
with hyperbilirubinemia, together with hematologic
pathologies, autoimmune and viral hepatitis and the
other causes, considering the possibility that the disease
progress can be controlled by the right diagnosis and
treatment.
[P-266]
A case report: Progressive familial
intrahepatic cholestasis type 2 with liver
transplantation
Introduction:
Progressive
familial
intrahepatik
cholestasis (PFIC) type 2 was previosly known as
Beyler’s syndrome (1) and is a resault of muatation
in the ABCB 11(ATP binding cassette (ABC) familiy
B,member 11) encoding BSEP ,located on chromosome
2(2q24)(2).BSEP is transporter protein,expressed at
the canalicular membrane of hepatocyte (3).İt is the
main exporter of bile acids from hepatocyte of to caniluli
against a concertration gradient.Early recognition of
PFIC -2 which can had to hepatic failure in generally frist
decade contribute to the therapy of discase.Our purpose
is to present a PFIC case in which hepatic transplantation
was needid because of hepatic failure devoleped in early
period.
5 month control
*ALT: 121 IU
*AST; 184 IU
*T.PTROTEİN: 5.0 MG/DL
*ALBUMİN :3.4 MG/DL
*T.BİL:6.3 MG/DL
*D.BİL :4.8 MG/DL
*HB 10.1 G/DL
*WBC 9.7X10.000 /Ul
*plt 313.000/Ul
*ALT 121 IU
*T.BİL 6.3 MG/DL
*ALBUMİN :3.4 MG/DL
*D.BİL 4.8 MG/DL
*HB 10.1 G/DL
*WBC 9.7X10000 /Ul
*PT 20 SN (INR 1.9)
*PLT 313.000 /Ul
*AST 184 IU
*cmv dna pcr <
100copya/ml
*T.PROTEİN 5,0 MG/DL
Child history- no fauture
Family history-no fauture
Phisical examination
*skin and scleras are icteric
*erruption+
*liver 3/1 sm (rigid,smooth)
*spleen 2 sm
*expitium was long,rales
*grade2 ascides
*feces dilute
*weight: 8100 gr p (50-75) length79sm p(50-75)
APPLİCATİON TESTS
*ALT 218IU
*AST 188IU
*ALP 312IU
*GGT 59U/L
*T.PROTEİN
4.8MG/DL
*ALBUMİN 2.8
MG/DL
*T.BİL 9.7 MG/
DL
*D.BİL 7.1 MG/
DL
*HB9.1 G/DL
*WBC
6.2X10000/Ul
*plt 195.000/ul
*PT 22 SN(INR
2.1)
*AMMONİA 45
MG/DL
Gulnara Heydarova
Haydarpaşa Numune Egitim-Araştırma Hastanesi
Çocuk Saglıgı Ve Hastalıklıgı Klinigi
Case: A Seven month old girl patient
Presentinhg complaint: jaundice, acidic feces, skin
erruptions.
Historiy: jaundice noticed by the familiyin the second
month
6 month control
ABDOMEN USG:LIVER 109 mm,parankyme
heterogenous, spleen 86 mm,gallbladdereyes is
present
Advanced test
*alfa-1-antitrypsine 199 mg/dl
*alfafetoprotein 5600ng/ml
*eye eximination .pathology(-)
*TORCH-S IG M(-)
*CMV DNA PCR<100 copy/mı
*sweat test :32 meg/l
*serum bile acid :293mmol/l
*Urinary reducin agent (-)
*Urinary succininyl aciton(-)
*tandem-MS pataloji(-)
*urinary organic acids(-)
*blood aminoacids tyrosine and methionin are
minimaly high
*liver biops can not be made (inr is high)
AT 10 TH DAY ,EXİMİNATİON –suction is weak,lethargic
*PT 15 SN(INR:1.5)
*TORCH IG M(CMV IgM+)
- 127 -
CONTROL TESTS
*ALT 331 IU
*AST 217 IU
*ALP 288 U/L
*GGT 48U/L
*T.PROTEİN 4.7
MG/DL
*ALBUMİN 2.2
MG/DL
*T.BİL 12.5MG/DL
*D.BİL 9.3MG/DL
*HB 8.8G/DL
*WBC 7.3X10000/
Ul
*plt 217/Ul
*PT32SN(INR2.9)
*AMMONİA 184/
ug/DL
AFTER K VİT AND TDP THERE İS NO İMPROVOMENT İN
PT LEVELS
FACTOR V LEVEL:%28
İn the 8 th month the patient was sent tol iver
transplantation by the was of patient transport surgery.
Allogenic left lateral lobe live transplantation was
performed
After 2 month of transplantation the patient is being
abserved withaut complaint.
İn ABCB 11 GEN EXONS C 3084A>G(A1028A) (rs497692)
mutation was observedhomologus.
The fact that mutation leads to PFIC2 discase is recorded
in HUMAN GENE MUTATİON DETABASE.
- 128 -
INDEX
- 129 -
A
Abdülhamit Tüten 65, 70
Abdulkadir Bozaykut 67, 68
Abdullah Barıs Akcan 40
Abdullah Erdem 100
Abdullah Erdil 44
Abdullah Kocabaş 18
Abdullah Sert 124
Abdullah Yahya Heybeci 94
Abdurrahman Kara 125
Ada Bulut Sinoplu 19
Ádám Schmidt 19
Adem Binnetoğlu 76
Adem Karbuz 19, 29, 30, 51, 61, 66, 125
Adil Umut Zübarioğlu 20, 67, 118, 122
Ágnes Rita Martonosi 20
Ahmet Hakan Gedik 83
Ahmet Naci Emecen 40
Ahmet Sarıcı 99
Ahmet Şenel 37, 53, 54
Ahmet Soysal 121
Ahmet Uçar 83, 122
Aida Koka 50, 59, 60
A.ishak Işık 18
Akın İşcan 26, 28, 82
Akın Savaş Toklu 97
Akl Fahed 23
Alessandro Nicola Abbaticchio 101
Alexander Baranov 65
Alexander Pushkov 65
Ali Adil Fouad 30
Ali Ağaçfidan 122
Ali Alkan 42
Ali Ayata 91
Ali Bay 125
Ali Bülbül 20, 67, 118, 122
Alican Barış 54
Ali Çay 70
Ali Cemal Yumuşakhuylu 76
Ali Gedikbasi 94
Ali Oruç 92
Ali Yurtseven 58
Alkim Beyhan 21
Alper Aykanat 70
Alper Guzeltas 124
Alper Kaçar 19
Alper Soylu 106
Amra Adrovic 19
Anait Gevorkyan 65
Anamaria Ciubara 118
Ancuta Ignat 21, 118, 119, 125
Andrea Párniczky 22
Andrea Szentesi 22
Angelica Cristina Marin 21, 118, 119, 125
Anıl Tapısız 29, 66
Annalisa Resta 101
Anna Tóth 22
A. N. Surkov 18
A. O. Anushenko 18
Aras Tulunoglu 23, 94
Arda Ozyuksel 101
Ariadne Malamitsi Puchner 45
Arif Kut 25
Arzu Aras 24, 70
Asena Pınar Sefer 24, 43, 90
Asli Kibris 123
Aslı Memişoğlu 111, 121, 122
Aslı Nur Ören 92
Aslı Toros 88
Asuman Kıral 24
Atakan Telatar 53
Attila Alp Gözübüyük 71
Avidan Kızılelma Yiğit 41, 126
A. V. Nikitin 18
Avniye Kübra Baskın 25
Ayça Gürbüzer 112
Aycan Unalp 57
Ayça Sözen 25
Aydilek Çakır 82
Aydilek Dağdeviren Çakır 100
Ayhan Taştekin 71, 112
Ayla Balcı 25
Aylin Gençler 75
Aylin Öztarhan 23
Aynur Bedel 24, 26, 70
Ayper Somer 33, 34, 79, 88, 122
Ayşe Ağbaş 55
Ayse Ayzit Atabek 50
Ayşe Bozkurt Turhan 119
Ayşe Çiğdem Aktuğlu Zeybek 37, 47, 65, 68, 107
Ayşe Ferda Ocakci 71
Ayşegül Demir 66
Ayşegül Doğan Demir 26, 54, 88, 92, 105, 106, 119
Ayse Guler Eroglu 59
Ayşe Kalkancı 30
Ayşe Kavak 49
Ayşe Kılıç 63, 109
Aysel Kıyak 49, 113
Aysel Vehapoğlu 26, 27, 28
Ayşenur Kaya 83
Ayse Serap Akgun 100, 101
Ayse Ucar 47
Ayşin Nalbantoğlu 86, 87
Aysun Yahşi 114, 115, 116, 117
B
Bahar Çalışkan 61
Bahar Caran 36
Bahar Özcabı 100
Bahattin Tunç 125
Balázs Csaba Németh 22
Banu Bal Çermik 82
Banu Çermik 27
Banu Yazici 28, 94
Başak Koç 33
Başak Saraçoğlu 112
Beáta Rosdy 31, 76
Bedia Deniz Ekici 65
Begum Koc 47
Begüm Şirin Koç 64
Behçet Şimşek 78, 95
Bekir Yiğit Develi 41
Bendavid Matthieu 28
Bengu Ulku Tarhan 107
Benhur Sirvan Cetin 89, 90
Ben Pode Shakked 68
Berna Gümüşoğlu Akşahin 41
Betül Aluç 48
Betül Çınar 29
Beyhan Tüysüz 55
Bidet Philippe 28
Bilge Aldemir Kocabaş 29, 30, 126
Birgül Kırel 39
Birol Ozturk 47
Birol Topçu 84
Bogdan Ciubara 21
Boglárka Fehér 22
Bonacorsi Stéphane 28
Borbala Zsigmond 31
Buket Kara 107
Bülent Çekiç 126
Bulent Hacıhamdioğlu 36, 60, 61, 123, 124
Bulent Oguz Erol 79
Bülent Zülfikar 105
Burak Ardıçlı 81
Burak Deliloğlu 31
Burak Tatlı 34
Burçin Nalbantoğlu 83, 84, 85
Burcu Karadaş 31, 93, 96, 109
Burcu Karakayalı 20, 31
Burcu Karakol 31
Busra Duzyol 28
C
Çağatay Günay 95
Çağatay Nuhoğlu 24, 26, 31, 70, 74, 78, 93, 95, 109
- 130 -
Cahit Barış Erdur 89
Cana Aksoy Poyraz 69
Canan Aygün 44
Can Demir Karacan 80, 81
Can Polat 38, 39
Caseris Marion 28
Cavid Serdarzade 71
Celal Akdeniz 104
Cengiz Candan 48
Cenk Ermutlu 53, 54
Ceren Çıralı 96
Cicek Bayındır 88
Çiğdem Aktuğlu Zeybek 37, 82, 97, 107
Çiğdem Aydoğmuş 25, 64
Çiğdem Oruç 96
Çiğdem Sağ 74, 95
Çiğdem Ulukaya Durakbaşa 102
Cihangir Akgun 100, 101
Çisem Saygılı 114
Civan Işlak 29
Cointe Aurélie 28
Coşkun Armağan 32
Csilla Andorka 22
D
David Terence Thomas 25
Dénes Molnár 22
Deniz Arık 119
Deniz Aygun 32, 33, 34, 35, 50
Deniz Tekin 114
Derya Aysev 30, 115
Derya Büyükkayhan 41, 42, 43
Derya Gültekin Başboğa 35, 36
Didar Arslan 36
Didem Gulcu 69
Didem Ocal Topcu 36, 61, 123
Dilara Ece Toprak 37, 47
Dilara Fucucuoglu 37, 38, 39, 58
Dildar Bahar Genç 40
Dilek Battal 109
Dilek Karacanoğlu 119
Dilşad Koca 26, 70, 74, 93, 96
Dimitris Papamichail 45
Dogukan Aktas 100
Dolunay Gürses 75
Donatella Capodiferro 101
Döndü Nilay Penezoğlu 114
Dóra Mosztbacher 22
Doruk Gül 24, 43, 90
Doygoameneh Ghare Mashggharavi 77
Duhan Demircioğlu 62
Duhan Hopurcuoglu 32
Durdugül Ayyıldız Emecen 40
Duygu Besnili Acar 20, 67, 118, 122
Duygu Övünç Hacıhamdioğlu 25
Duygu Taşkaldıran 85, 86, 87
Duygu Ülger 62
E
Ebru Atas Aslan 40
Ebru Eren 121
Ebru Tuğrul Sarıbeyoğlu 111
Ebru Türkoğlu Ünal 20
Ece Gültekin 91, 97
Elif Arslan 41, 97
Elif Begüm Kılıç 82
Elif İnce 114, 117
Elif Kirit 113
Elif Neslihan Tuzcu 104
Elifnur Ildes 107
Elif Yıldız 50
Elif Yılmaz Güleç 23
E. L. Semikina 18
Elvan Çağlar Çıtak 79
Emel Akıncı Ataoğlu 41, 42, 43
Emel Ataoğlu 104
Emel Karaoğlan 48, 76
Emel Torun 82
Emine Ayça Cimbek 73, 74
Emine Erdogan Kutanis 37
Emine Olcay Yasa 43, 48, 102, 103
Emine Polat 80
Emine Tekin 117
Emine Türkkan 41
Emine Yurdakul Ertürk 44
Emine Zeliha Suskan 114
Emin Ünüvar 63
Emmanouela Sdona 45
Emrah Aydın 45, 46
Emre Gök 33
Emre Kelesoglu 100
Emre Ozer 47
Emre Tasdemir 47
Emrullah Arslan 47
Enes Kanay 54
Enes Sali 89
Engin Çarkçı 52
Enise Avcı 48
Ensar Duras 77
Erdal İnce 29, 30, 66, 114, 115, 116
Erdal Sari 108
Erdem Ak 79
Erdogan Soyucen 65
Eren Özek 111, 121, 122
Ergin Çiftçi 29, 30, 66, 114, 115, 116, 117
Erhan Özbek 89
Erkan Çakır 66, 83
Erkan Erfidan 48, 49, 50
Erkut Ozturk 124
Ersin Böcü 50
Ersin Ulu 29
Ertuğrul Kıykım 37, 47, 65, 82, 97, 107
Esin Aldemir 64, 113
Esra Karabiyik 50
Esra Ozcan 38
Esra Özpınar 51
Esra Pehlivanoglu 69
Esra Sesli 51
Esra Yücel 51, 52, 110
Ethem Ayhan Ünkar 52, 53, 54
Éva Kovács 31, 76
Éva Németh 20
Evrim Çeliker Yapıcı 41
Evrim Kıray Baş 20, 67, 118, 122
Eylem Ulaş Saz 58
Ezgi Topyildiz 79
Ezgi Yalçın 54
F
Fanni Zsoldos 22
Faruk Öktem 54, 105, 106
Fatih Aygün 55, 98
Fatih Çelmeli 18, 30
Fatih Cemal Özdemir 78
Fatih Demircioglu 107
Fatih Günay 114
Fatih Kara 72, 73
Fatih Kirar 71
Fatih Mehmet Keleşoğlu 112
Fatih Varol 32, 55, 62
Fatma Betül Çakır 56, 93, 105
Fatma Burçin Kurtipek 81
Fatma Deniz Aygün 98
Fatma Ekici 77
Fatma Gamze Demirel 111
Fatma Nur Öz 56
Fatma Özgüç 83, 93
Fatma Özgüç Çömlek 56
Fatma Saltık 57
Fatoş Yalçınkaya 116
Fatouma Khalif Abdillahi 57
Fayize Maden 57
Ferah Genel 89
Ferda Gürkan 58
Feyza Aydın 55, 58
Feyza Coskun 58
Feyza Coşkun 59
Feyza Darendeliler 109, 122
Feyza Mediha Yildiz 108
Feyza Mediha Yıldız 35, 36
Feyza Ustabas 27
Filiz Serdaroglu 57, 90
Firdevs Baş 63, 122
Firuze Erbek Alp 55, 59, 60
Fırat Beğde 30
Foteini Zafeiropoulou 60
Fuat Buğrul 74
Fügen Çullu Çokuğraş 69, 121
Funda Çipe 25
Funda Kökali 61, 79, 80
Funda Öztunç 50, 59
G
Gábor Rudas 76
Gábor Veres 22
Gabriela Paduraru 21, 118, 119
Gabriella Kiss 76
Gafur Doğdu 112
Gamze Demirel 71, 111
Gamze Ozgurhan 36, 60, 61, 123, 124
Gamze Sarıkaya Uzan 61
Gamze Yildirim 124
Gizem Höyük 48
Gizem Ondalikoglu 101
Gizem Pamuk 19, 62
Gizem Sayar Bilgin 63
Goar Movsisyan 63
Gökçen Kamış 48, 49, 64, 95
Gökhan Büyükkale 51, 94
Gokten Ongel 60
Gonca Erkose Genc 79
Gonca Keskindemirci 25
Gonca Topuzlu Tekant 34, 121
Gönül Aydoğan 49, 95
Gözde Akın 76
Gözde Yeşil 47, 82, 93
Gözde Yücel 19, 64
Gülay Önal 73
Gulnaz Sariyeva 69
Gül Nihal Özdemir 64
Gülperi Yağar Keskin 40
Gülsen Akay 65
Gülşen Akkoç 121
Gülşen Köse 40, 97
Gülser Esen Besli 102
Gülsüm Güzel 66
Gulnara Heydarova 127
Gulsum Karakus 38
Gunce Basarir 65
Güner Karatekin 65
Gürcan Dikme 47, 62, 64
Gurkan Gurbuz 57
Gurkan Tarcin 65
Guzal Yakhyaeva 65
Güzide Baysan Turanlı 97
Güzide Turanlı 91
G. V. Volynets 18
György Várallyay 31, 76
H
Hacer Aktürk 79, 122
Hacer Ergin 96
Hakan Çakır 112
Halil Haldun Emiroğlu 72, 73
Halil Özdemir 29, 66, 114, 115, 116, 117
Halil Suat Ayyıldız 110
Halise Zeynep Işcan 67
Halit Çam 55, 62, 98
Haluk Çokuğraş 32, 33, 34, 35, 50, 46, 98
Haluk Güriz 29
Hamdi Ograg 40
Hamide Sevinç Genç 49, 113
Handan Akoglu 108
Handan Ayhan Akoğlu 67, 68
- 131 -
Handan Hakyemez Toptan 65
Hande Kızılocak Kumkumoğlu 35, 47, 62, 64
Haşim Gencer 108
Hasret Ayyıdız Civan 68, 69, 92, 121
Hatice Arıöz Habibi 121
Hatice Dülek 109
Hatice Eren 74
Hatice Gamze Poyrazoğlu 56
Hatice Güllüelli 70
Hatice Nilgun Selçuk Duru 79, 80
Haticenur Kirar 71
Hatice Öztürk 24, 26, 70
Hatice Sınav Ütkü 71
Hatice Ütkü Sınav 111
Havva Evrengül 75
Hayrettin Hakan Aykan 88
Hazal Hazal Huzmeli 71
Helen Bornaun 23, 48, 50, 51, 75, 77, 94, 100
Heves Kırmızıbekmez 36
Hikmet Akbulut 72, 73, 74
Hikmet Tekin Nacaroğlu 59
Hilal Balcı 85, 86, 87
Hilal Fevziye Durmaz Şimşek 74
Hilal Şen 103
Hilal Susam Şen 110
Hilary Pearce 105
Hilmi Apak 35
Hülya Bilgen 111
Hülya Özdemir 111, 120, 121
Hulya Ozturk 107
Hülya Selva Bilgen 120, 121
Huray Kok 74
Hüray Kök 109
Huriye Nursel Elçioğlu 40, 120, 122
Hüseyin Aldemir 48, 76
Hüseyin Aslan 39
Hüseyin Bardak 75
Huseyin Canaz 28, 94
Hüseyin Dindar 116
Huseyin Kaya 38
Hüseyin Kılıç 58
I
İbrahim Adaletli 121
Ibrahim Alatas 28, 94
Ibrahim Mert Erbaş 75, 76
İclal Erdem Toslak 126
Ihsan Kafadar 71, 108
Ildikó Guthy 22
İlhan Çiftçi 74
Ilker Ertuğrul 88
Ilker Gonen 113
İlker Tolga Özgen 27, 92
İlter Paydur 74
İpek Akman 120
Irem Ceren Arslan 76
Isa Döngel 57
Ismail Göçmen 25
İsmail İşlek 31
Ismail Mithat Akan 112
İsmail Taşkıran 44
İsmail Yıldız 63
Ismet Çok 109
İsmet Düşmez 24, 43, 90
Issam Cheikh Ahmad 106
István Tokodi 22
J
Judit Czelecz 22
Judit Móser 76
Judit Szamosújvári 76
K
Kaan Boztug 25
Károly Bakó 22
Katalin Kollár 31, 76
Kazim Coban 57
Lale Sever 99
László Szabó 20
Leda Sianidou 60
Levent Saltık 50, 60
Leyla Besel 78, 124
Leyla Namazova Baranova 63, 65
Leyla Telhan 92, 97, 111
Lida Bulbul 60
Lucrezia De Cosmo 101
Muhammet Ali Varkal 63
Muhammet Demirkol 83, 84, 85, 86, 87
Muhammet Nurullah Yakut 41
Müjgan Alikaşifoğlu 96
Munevver Kaynak Turkmen 40
Murat Elevli 41, 42, 43
Murat Şahin 23, 94
Murat Sarı 76
Murat Sutcu 79, 88
Murat Vural 119
Mürşide Uysal 77
Mustafa Akcam 51, 90
Mustafa Atilla Nursoy 66
Mustafa Behçet Şimşek 78
Mustafa Çakan 48, 49, 113
Mustafa Dilek 107
Mustafa Gülgün 88
Mustafa Güngör 53
Mustafa Hacimustafaoglu 89, 90
Mustafa Kemal Demir 120
Mustafa Kemal Özdemir 19, 41
Mustafa Metin Donma 83, 84, 85
Mustafa Oğur 88
Mustafa Taha Özkul 89
Mustafa Torehan Aslan 89, 90
Mustafa Uzan 61
M
N
Mahir Tıras 78
Mahmut Can Kızıl 39
Mahmut Doğru 35
Maisam Abu El Haija 22
Maltam Baghirova 78
Manolya Acar 79, 88
Margit Lörincz 19
Mariani Patricia 28
Maria Vasilopoulou 60
Marin Burlea 21, 118, 119
Marton Hernadi 31
Mebrure Yazıcı 66, 88
Mehmet Ali Erceylan 43
Mehmet Atilla Türkmen 106
Mehmet Esat Uygur 52
Mehmet Gunduz 100, 101
Mehmet Karacan 104
Mehmet Kemal Arikan 69
Mehmet Şerif Cansever 37
Mehmet Sinan İyisoy 72
Mehmet Vural 29
Mehmet Yusuf Sarı 56
Mehtap Kağnıcı 89
Mehtap Sak 35
Meliha Aksoy 100
Meliha Demiral 39
Melih Er 79
Melike Emiroğlu 72, 73
Melike Irem Petan 79, 80
Melike Ocak 80, 81
Melis Köse 89
Meltem Erol 37, 38, 39, 58, 59, 78, 124
Meltem Yildirim 60
Merih Çetinkaya 51
Mervan Bekdas 107
Merve Erdemir Kula 82
Merve Hazal Yılmaz 82
Merve Korkut 75, 96
Merve Nur Hepokur 82
Merve Şakar 83, 108
Mesut Dursun 20
Mesut Okur 100, 101
Mesut Parlak 18
Mine Aslan 37
Mine Çalıskan 88
Mine Kucuk 33, 34
Mine Özdil 29
Mine Yüksel 83, 93
Mónika Mellár 76
Müferet Ergüven 48, 50, 90, 102, 103
Muhammed Nurullah Yakut 103
Nadia Al Namat 119
Nadir Koçak 74
Nagehan Aslan 90, 91
Nail Uzunlulu 75
Nalan Karabayır 97, 100
Narin Akıcı 74, 95
Natalia Jurkova 65
Natália Lásztity 22
Nato Vashakmadze 65
Nazan Dalgıç 108
Nazli Kavcik 113
Nedim Samancı 85, 86, 87
Neşe Yaralı 125
Neslihan Özhan 91
Neslihan Tekin 119
Nevin Uzuner 32
Nicola Laforgia 101
Nicoletta Resta 101
Nihat Cem Çelebi 92, 97
Nil Arısoy 99
Nilay Kadakal 92
Nilden Tuygun 80, 81
Nilgün Erkek 101
Nilgün Karadağ 65
Nilgün Selçuk 61
Nilüfer Göknar 27, 54, 119
Nil Yazar Alpay 67
Ninel Revenco 103
Nisa Eda Çullas İlarslan 114
Nisa Pekcan 92
Noémi Scheuring 20
Noémi Vass 22
Nuran Salman 33, 34, 79, 88
Nuray Aktay Ayaz 48, 49, 113
Nur Aydınlı 88
Nur Canpolat 55
Nurcan Yusufoğlu 31, 93
Nurettin Onur Kutlu 28, 88
Nurhan Aruçi Kasap 56, 83, 93
Nuriye Ece Mintaş 85, 86, 87
Nurşen Belet 29
Nursu Kara 28, 94
Nurver Toprak 121
Kazim Oztarhan 100
Kazım Küçüktaşçı 85, 86, 87
Kazım Özatrhan 77
Kazım Öztarhan 23, 51, 94
Kemal Baysal 44
Kemal Gökkuş 30
Kenan Barut 19, 99
Kenan Toprak 95
Kerem Ozel 94
Kirill Savostyanov 63, 65
Kıymet Keçelioğlu Binnetoğlu 76
Konstantin Jerdev 65
Koray Hacıoğlu 77
Kyaw Zin Latt 77, 126
L
O
Olcay Evliyaoğlu 100
Olcay Yasa 43, 48, 50, 102, 103
Olga Giannouli 60
Olga Gundobina 63
Ömer Bek 30
- 132 -
Ömer Ceran 110
Omer Faruk Aydın 117
Ömer Faruk Beşer 41, 52
Ömer Faruk Özer 27
Orhan Özbek 73
Orkide Donma 83, 84, 85
Osen Ari 23, 94, 95
Osman Hacıosmanoğlu 70
Osman Kızılkılıç 99
Osman Umur Başaran 114
Osman Uzunlu 121
Osman Zeki Karakuş 106
Övgü Kul Çınar 96
Oya Ercan 96, 100
Ozan Özkaya 41, 71, 77
Özcan Bör 119
Özden Aksu Sayman 96
Özden Türel 28, 56, 88, 106
Özge Aydemir 39
Özge Besci 111
Özgecan Avcı 67
Ozge Demirel 75
Ozge Gucuyeter 95
Ozgul Yigit 37, 38, 39, 58, 59, 78, 124
Özgün Taban 96
Özgür Genç 67
Özgür Kasapçopur 19, 99, 97
Özgür Kızılca 85, 86, 87
Ozgur Pirgon 51
Özlem Başoğlu Oner 95
Ozlem Bostan Gayret 37, 38, 39, 78, 124
Özlem Erdede 35, 36
Özlem Ketenci Altıkardeşler 70
Özlem Kosvalı 42
Özlem Öçal Doğan 97
Özlem Öner 75
Özlem Özdemir 44
Özlem Sert 101
Özlem Su 66
Özlem Temel 35, 36
Özlem Yapıcıer 120
Özmert Muhammed Ali Özdemir 96
Öznur Vermezoğlu 41, 42, 61, 123
P
Pagona Lagiou 45
Pamir Işık 125
Péter Hegyi 22
Pierron Charlotte 28
Pınar Bütün 97
Pınar Karadeniz 118
Pınar Özge Avar Aydın 97, 98, 99, 100
Pınar Selimoglu 124
Pınar Turhan 48
Pınar Yamaç Dilaver 97, 100, 101, 111
Püren Özüdoğru 101
R
Rabia Gönül Sezer 67, 68
Raffaella Panza 101
Raif Coşkun 59
Raziye Burcu Güven Bilgin 58
Refia Gözdenur Savcı 102, 103
Rejan Kebudi 105
Rengin Şiraneci 75, 95
Reyhan Dedeoğlu 50, 55, 59, 60
Rıza Rızalar 111
Rodica Eremciuc 103
Royala Babayeva 29
Ruhsar Erdoğmuş 103
Rukiye Eker Ömeroğlu 112
Rukiye İrem Yekeler 104
Rukiye Taş 104
Rumeysa Citli 108
Rumeysa Tuna 105
Rumeysa Yasemin Çiçek 55
Rüveyde Bundak 122
S
T
Sahin Hamilcıkan 39, 69
Sairah Akbar 105
Şakire Başer 81
Salih Kavukçu 106
Salih Somuncu 120
Salim Çalışkan 99
Saniye Berna Hamilçıkan 41, 52
Sare Betül Kaygusuz 106
Şebnem Özdoğan 83
Sebuh Kuruoglu 47
Seçil Arslansoyu Çamlar 106
Seda Balkaya 48
Seda Şirin Köse 32
Seda Sönmez 106
Sedat Öktem 110, 111, 112
Sedat Özdemir 72
Seda Yılmaz Semerci 51
Sefika Elmas Bozdemir 90
Seher Acar 107
Selçuk Uzuner 88, 106
Selçuk Yüksel 75
Selen Baran 107
Selim Dereci 51, 90
Selim Gökçe 82
Selman Kesici 81
Selver Ametoglou 30
Selvinaz Edizer 57
Sema Bozkaya Yilmaz 108
Sema Saltık 58
Şenay Akbay 91
Şenol Emre 121
Senol Turan 69
Seral Navdar 72, 73
Serdar Ceylaner 18
Serdar Türkmen 26, 27
Serif Cansever 65
Şerife Suna Oğuz 126
Serkan Ozturk 107
Sermin Özcan 40, 83, 108
Serpil Değirmenci 35, 36
Sertac Hanedan Onan 124
Sevgi Yavuz 49, 113
Sevil Arı Yuca 74
Sevil Bilir Goksugur 107
Sevinç Emre 112
Sevinç Gümüş 109
Sevinç Mertoğlu 55
Şeyma Sönmez Şahin 103
Sezen Atik 50
Sezen Ugan Atik 59, 60
Sezgin Şahin 19
Shiyar Hasan 50
Sibel Aşkın Perkin 97
Şifa Şahin 71, 110, 111
Simge Kaya 79
Sinan Oğuz 80, 81
Sinan Uslu 20, 67, 118, 122
Sinan Yavuz 58
Şirin Güven 31
Solmaz Celebi 89, 90
Soner Sazak 41
Songül Yılmaz 115, 116
Suat Ayyıldız 110, 111
Suat Fitöz 66, 115, 116
Süheyla Ocak 110
Süheyla Ocakçı 103
Şükran Poyrazoğlu 109, 122
Sukran Yildirim 94
Şükrü Candan 23
Şükrü Küçüködük 44
Sule Nursah Ayata 31, 109
Şule Terzioğlu 26, 27
Sultan Bent 124
Sultan Kavuncuoğlu 64
Suna Asilsoy 32
Suray Aliskan 123
Suzan Sahin 40
Takis Panagiotopoulos 45
Taliha Öner 118
Tamay Gürbüz 26, 74, 78, 95
Taner Yavuz 31
Tansu Salman 79
Tanyel Zübarioğlu 37, 47, 65, 68, 82, 107
T. A. Skvortsova 18
Tatiana Macovei 103
Taylan Celik 89
Tea Margieva 65
Tekin Bağlam 76
Tevfik Karagöz 88
Tolga Besci 111
Tolga Tüzüner 53, 54
Tuba Koçkar 110, 111, 112
Tufan Kutlu 68, 92, 121
Tuğba Barsan Kaya 39, 119
Tuğba Erat 114, 115, 116, 117
Tuğba Erener 23
Tugba Koca 51, 57, 90
Tugce Goksu Yilmaz 112
Tuğçe Kalaycı Oral 77, 113
Tuğçe Kalın 114
Tuğçe Tural Kara 66, 114, 115, 116, 117
Tuğrul Atay 104
Tülay Erkan 69, 121
Tülin Tiraje Celkan 35, 47, 62, 64, 99
Türkan Uygur Şahin 26, 28, 82, 92
Turkay Rzayev 32, 35
U
Ufuk Erenberk 57, 88, 92
Ulkem Colak 117
Ümmühan Öncül 118
Umut Durak 70, 74
V
Vahit Guzelburc 100
Vasfi Onur Vermezoğlu 41, 42
Vasile Valeriu Lupu 21, 118, 119, 125
Vefik Arıca 19, 97
Veronika Ila 22
Volkan Tuzcu 104
Y
Yağmur Birsev 96
Yair Anikster 68
Yaşar Şen 73, 74
Yasemin Ataman 119
Yasemin Ersözlü 119
Yasemin Kendir Demirkol 120, 122
Yasemin Özdemir Şahan 58
Yasin Şahin 121
Yavuz Gündoğdu 76
Yavuz Köksal 73
Yelda Türkmenoğlu 41, 77, 78
Yelda Türkmeoğlu 97
Yeşim Acar 41, 52, 78
Yeşim Coşkun 120
Yıldız Camcıoglu 34
Yıldız Camcıoğlu 98
Yıldız Perk 29
Yılmaz Yozgat 88
Yusuf Can Doğan 34, 121
Z
Zehra Esra Önal 24, 31, 74, 78, 93, 96
Zehra Yavas 33
Zehra Yavaş Abalı 63, 122
Zerrin Onal 60
Zeynep Alp Ünkar 52, 53, 111, 120, 121, 122
Zeynep Birsin Özçakar 115
Zeynep Ceren Karahan 30
Zeynep Emine Tuzcular Vural 109
Zeynep Ersöz Güngör 85, 86, 87
- 133 -
Zeynep Gör 52, 126
Zeynep Hızlı 122
Zeynep Işcan 67, 122
Zeynep Karakaya 123, 124
Zeynep Ocak 23, 77
Zeynep Topkarcı 49
Zeynep Yıldız Yıldırmak 40
Ziya Demirci 53, 54
Zoi Beka 60
Zubeyde Kilinc Yaprak 58, 124
Zümrüt Uysal 114
NOTES
- 134 -
NOTES
- 135 -
NOTES
- 136 -
Outlook to Istanbul
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