Department of Paediatrics The Hospital for Sick Children 2006-07 ANNUAL REPORT
Transcription
Department of Paediatrics The Hospital for Sick Children 2006-07 ANNUAL REPORT
Department of Paediatrics The Hospital for Sick Children ANNUAL REPORT 2006-07 Developing new health strategies CONTENTS 02 Chair’s Report Divisions 04 Adolescent Medicine 08 Cardiology 18 Clinical and Metabolic Genetics 24 Clinical Pharmacology and Toxicology 29 Developmental Paediatrics 32 Endocrinology 36 Gastroenterology, Hepatology and Nutrition 44 Haematology/Oncology 56 Immunology/Allergy 61 Infectious Diseases 66 Neonatology 74 Nephrology 78 Neurology 86 Paediatric Emergency Medicine 94 Paediatric Medicine 102 Respiratory Medicine 107 Rheumatology 113 Bloorview Kids Rehab 115 116 118 122 PAMOT Paediatric Housestaff Paediatric Grand Rounds Promotions/Retirements/ Service Recognition 124 Faculty Awards 126 Resident and Fellow Awards and Achievements Introduction Dedicated to improving children’s health through advances in clinical care and research and the education of future caregivers. DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 1 Chair’s Report On July 1, 2006, Hugh O’Brodovich stepped down after 10 outstanding years of leadership of the Department of Paediatrics at The Hospital for Sick Children (SickKids) and the University of Toronto (U of T). The transition has been smooth, in large part because of the commitment of Hugh and his team to academic excellence and fiscal responsibility. The 2006-07 academic year was one of ongoing high achievement in all aspects of academic paediatrics. This annual report attests to the magnificent nature of the Department’s accomplishments. What are the metrics of success? In research, they are The challenges of the past year have included recruitment relatively easily identified: more than 700 papers published of new faculty to fill vacancies due to retirement, departures annually, frequent invitations to faculty for plenary lectures and new opportunities. So far, about 20 new faculty at prestigious meetings or visiting professorships, members have joined the Department across nearly all of increasing grant funding (the vast majority of our faculty the divisions. Furthermore, a number of Division Heads has some funding to support their research) and increasing (Chaim Roifman, Immunology, Andrew Redington, Cardiology numbers of research trainees. and Denis Geary, Nephrology) have been reappointed In education, we completed the Royal College of Physicians and Surgeons of Canada external review with overall good success, attracted a superb cohort of core paediatric residents and an ever increasing number of subspecialty and a new one appointed (Mark Palmert, Endocrinology). Our new recruits represent an enormously accomplished and talented group of people from all around the world. We have great expectations! trainees. Last year, there were about 60 residents, 60 Another challenge has been the protracted, but eventually Royal College approved trainees in subspecialty programs, successful, renegotiation of our Alternate Funding Plan with and 209 other trainees in both clinical and research the Ministry of Health and Long-Term Care. The new plan, programs. Increasing numbers of our faculty have taken although relatively short in duration, allows us to maintain education as their career path either doing a Masters in Education or the Education Scholars Program at the Centre fiscal stability and remain competitive. for Faculty Development at the University of Toronto. Finally, clinically, we continue to provide outstanding tertiary and quaternary care to the children of Ontario, partner with the paediatric academic health science centres in Ontario (as part of the relatively new Provincial Council for Children’s Health, PCCH) and community programs (in the context of the Child Health Network of the GTA). 2 DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 The major changes in leadership at the level of the Paediatric Executive have been the move of Susan Tallett to Director of the SickKids Learning Institute and her replacement by Rayfel Schneider, and the addition of Ben Vozzolo as Director of Administrative Services in the Department of Paediatrics. Rayfel’s mandate is to oversee all of the educational activities of the Department and ensure that we remain at the cutting edge of educational activities. Rayfel’s team includes new Program Directors in both Postgraduate (Adelle Atkinson) and Undergraduate (Stacey Bernstein) Medical Education, as well as Suzan Schneeweiss, Director of Continuing Education. New directions in education can be expected from this highly talented and enthusiastic group. Ben has more than ably taken over where Rich Gerber left off, overseeing the department’s financial well-being and supporting the administrative staff across all of the divisions. The next steps for our department include: completion of the reevaluation of our Career Development and Compensation Program, and development of our strategic goals with alignment with those of SickKids and the University of Toronto. This has begun and will culminate later this year in transparent processes and plans. In the meantime, the Department of Paediatrics remains highly accomplished and productive, though not without its stresses and strains. I am greatly indebted to all members of the department for their hard work this past year and for the patience and encouragement they have all offered me. Special thanks to the Paediatric Executive, Daune MacGregor, Norman Rosenblum, Sue Tallett and Ben Vozzolo, and to the hospital administration, Mary Jo Haddad, CEO and President, Seonag Macrae, VP Child Health Services Portfolio, and Ron Laxer, VP Education and Quality, and Dean Cathy Whiteside of the University of Toronto’s Faculty of Medicine for their enormous support. DENIS DANEMAN, MB, BCh, FRCPC Chair of Paediatrics University of Toronto Paediatrician-in-Chief, The Hospital for Sick Children R.S. McLaughlin Chair in Paediatrics at The Hospital for Sick Children THE PAEDIATRIC EXECUTIVE COMMITTEE Left to right: Ms. Seonag Macrae, Dr. Daune MacGregor, Mr. Ben Vozzolo, Dr. Denis Daneman, Dr. Norman Rosenblum, Dr. Susan Tallett Division Head Dr. Debra K. Katzman Full-Time Dr. Eudice Goldberg Dr. Miriam Kaufman Dr. Karen Leslie Dr. Cathleen Steinegger Part-Time Dr. Michael Falk Dr. Miriam Rossi Dr. Rahul Saxena Clinical Fellows Dr. Fadia Al-Buhairan Dr. Rosawan Areemit Dr. Boonying Manaboriboon Dr. Ellie Vyver Administrative Staff Sawsan Bakhit Ursula Pajak Burnett Wint Adolescent Medicine DIVISIONAL OVERVIEW Drs. Karen Leslie and Miriam Kaufman with Adolescent Medicine Team The Division of Adolescent Medicine continues to focus its clinical activities in five program areas – Eating Disorders, The Good 2 Go Transition Program, Substance Abuse, Young Families, and Complex Adolescent Problems. The Eating Disorders Program at The Hospital for Sick Children continues to be the provincial centre for child and adolescent eating disorders providing a variety of integrated clinical services (inpatient, day treatment, outpatient and consultation) to adolescents and their families. Over the past year, the Ministry of Health and Long-Term Care (MOHLTC) granted new funding to enhance intensive inpatient services for children and adolescents with eating disorders at SickKids. In addition, other new initiatives include the integration of multi-family group therapy at SickKids and throughout the province. Over the past year, these year-long groups were completed in three provincial centers. In the fall of 2007, SickKids will lead in providing multifamily group therapy training to professionals from several centres and will continue to provide groups to families at the hospital. The collaborative Ontario Community Outreach Program (SickKids, University Health Network and the MOHLTC) continues to train health care professionals around the province in the assessment and treatment of eating disorders and has been an extremely successful educational initiative. A smooth transition from paediatric to adult health care is a priority for all SickKids patients. The Good 2 Go Transition Team, consisting of a Nurse Practitioner, Psychologist, Social Worker and Paediatrician, has made tremendous strides since its inception in August 2006. The team runs a weekly transition clinic in Adolescent Medicine, as well as being involved with transition clinics in other parts of the hospital. There have been meetings with over 15 clinical programs, most of which have already adopted new transition activities. Launched in January 2007, the Good 2 Go website offers a constantly expanding transition bibliography, a transition menu, and links to various resources. MyHealth Passport is a wallet-sized card with health information that is created by a teen with a health care provider, the process combining patient education and empowerment with a tool for sharing health information. A comprehensive transition intervention and research study is being developed with the Division of Cardiology. The Adolescent Substance Abuse Outreach Program and the Substance Abuse Day Treatment Program continue to provide exemplary clinical care to adolescents and their families with substance abuse problems. In-depth psychotherapy, brief counseling, group work and family support are fundamental services offered to our patients. As well, all patients receive regular medical assessments to assess for medical sequelae of their substance use and other medical concerns that result from a high-risk lifestyle. The program recently completed a sixmonth pilot project with the Department of Psychiatry, providing substance abuse training for child-track residents and receiving psychiatric assessment and treatment for co-morbid psychiatric diagnoses for adolescents in the Program. Due to the success of the pilot project, a second six-month program is underway. The Day Treatment Program expanded their services this year to include a summer program, combining treatment, academic and medical support to day treatment patients. All Substance Abuse Program staff work collaboratively with many other organizations to provide comprehensive care to at-risk youth. Staff members also provide extensive education at many levels including trainees from a variety of disciplines, staff in other areas of the hospital, health care professionals and other professionals in the community as well as adolescents and their caregivers. Expanded training initiatives have been implemented for Adolescent Medicine fellows and for staff in the Emergency Department. As well, enhanced training in substance use and abuse for paediatric residents will be initiated this academic year. In addition to the many outreach educational activities of the Program, staff presented at DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 5 international conferences in New Zealand and Australia at the invitation of local health care professionals. Members of our staff are involved in many aspects of service development, both at local and regional levels, and provide expertise in child and youth substance use and abuse to various committees dealing with prevention and treatment issues at the municipal, provincial, and national levels. The Complex Adolescent Patient Program (CAPP) continues to focus on adolescents with a chronic illness or disability. These services are provided in the Teen Clinic as well as in collaboration with renal, cardiac and lung transplant clinics, HIV clinic, SLE clinic and through inpatient consultations. This past year, a combined CAPP/SCAN clinic has been established to collaboratively assess and provide education and support for adolescents identified as engaging in highrisk sexual behaviours. The CAPP clinic has formalized an advocacy, outreach partnership with St. Stephen’s Community House to enable youth in the downtown core to access health information and necessary health services otherwise unavailable to them. The goal of the clinic is to reduce high-risk health behaviours, as well as to prevent pregnancy, sexual assault and sexually transmitted infections by promoting healthy sexuality and safe sexual relationships. Public education is an important part of CAPP and is delivered through talks at schools and to parent groups, media interviews and publications. The Young Families Program (YFP) continues to focus on providing health care, advocacy, support and education to highrisk adolescent parents and their children. The YFP collaborates with several agencies that provide services to high-risk adolescent parents including prenatal homes, health services and child protection agencies. In Particular the YFP provides a consultation clinic at “Jessie’s Centre for Teenagers” and provides educational, supportive and policy recommendations. In partnership with the SCAN team the “High Risk Project” has been operational since 2004 when external funding was established to standardize clinical care for adolescent parents and their children. Assessment and intervention in the areas of growth and development, infant and adolescent mental health, 6 DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 maltreatment, and social determinants of health provide the basis for research in adolescent parenting. Inter-disciplinary team members continue to disseminate information and provide education to health professionals in a variety of settings through workshops, poster presentations, and clinical practice guidelines. Research activities are an integral part of the activities of the faculty and trainees of the Division. Each of our clinical areas provides the substrate for our clinical research endeavours in the Division. As such, research activities include the medical complications of eating disorders with specific emphasis on osteoporosis, epidemiology of early-onset eating disorders, evaluation of family-based treatment for adolescents with eating disorders, the evaluation of the use of information technology for health promotion with youth, treatment outcomes in adolescent substance use, HIV educational interventions with incarcerated youth, and health values in adolescents with chronic illnesses. Members of the Division continue to make significant educational contributions locally, provincially, nationally and internationally. Members of our division hold leadership roles in the SickKids Summer Student Program, co-ordination of the Mentorship Program for Clinician-Teachers and -Educators at the Centre for Faculty Development, and the organization of the international annual scientific meeting on adolescent health at the Society for Adolescent Medicine. Division members play a leading role in national and international clinical, education and research committees including the Infant Mental Health Promotion Project, Canadian Paediatric Society, American Academy of Pediatrics, Society for Adolescent Medicine, Canadian Association for Medical Education and Academy for Eating Disorders. Over the past three years, the Division has taken the lead in pursuing paediatric subspecialty certification for Adolescent Medicine through the Royal College of Physicians and Surgeons of Canada (RCPSC). Adolescent Medicine was endorsed at the RCPSC’s Council in May 2007, and will now become the 63rd recognized discipline of the RCPSC as a subspecialty of Paediatrics. HONOURS AND AWARDS Katzman DK: Elected Treasurer for the Academy for Eating Disorders, 2007-2010. Katzman DK: Chair, Adolescent Medicine Subspecialty Working Group Committee, Royal College of Physicians and Surgeons of Canada PUBLICATIONS Adolescent Health Committee (Leslie K, et al.): Adolescent pregnancy – CPS Position Paper. Paediatrics & Child Health 2006: 11: pp 243-246. American Academy of Pediatrics, Committee on Adolescence, American College of Obstetricians and Gynecologists, and Committee on Adolescent Health Care: Menstruation in girls and adolescents: Using the menstrual cycle as a vital sign. Pediatrics 2006: 118: pp 2245-2250. Blythe MJ and the Committee on Adolescence: Emergency contraception. Pediatrics 2005: 116: pp 1026-1035. Bell M: Book Review: Protecting children from domestic violence: Strategies for community intervention. Canadian Journal of Community Mental Health 2007: 26: pp 1-4. Committee on Injury, Violence, and Poison Prevention and Committee on Adolescence: The teen driver. Pediatrics 2006: 118: pp 2570-2581. Goldberg MB, Katzman DK, Woodside B, Baker GI: Do eating disorders and chronic facial pain coexist? A preliminary study. J Can Dent Assoc. 2006: 72: p 51. Katzman DK, Walker LR, Rickert VI, Lock J: Invited commentary: The Year 2004: A depressing year for the treatment of depression adolescence. Journal of Adolescent Health 2005: 36: pp 454-456. Kaufman M: Transition of cognitively delayed adolescent organ transplant recipients to adult care Pediatric Transplantation 2006: 10: pp 413-417. Korczak D, MacArthur C, Katzman DK: Canadian paediatric residents’ experience and level of comfort with adolescent gynecological health care. Journal of Adolescent Health. 2006: 38: pp 57-59. Korczak D, Connolly B, Baron T, Katzman DK, Bernstein S: Safety and efficacy of image-guided gastrostomy (G) and gastrojejunostomy (GJ) tubes in children and adolescents with primary psychiatric illness. International Journal of Eating Disorders June 21, 2007 [Epub ahead of print]. Misra M, Miller KK, Cord J, Prabhakaran R, Goldstein M, Katzman DK, Herzog DB, Klibanski A: Relationships between serum adipokines, insulin levels and bone density in girls with anorexia nervosa. Journal of Clinical Endocrinology and Metabolism March 13, 2007 [Epub ahead of print]. Kirsh G, McVey G, Tweed S, Katzman DK: Psychosocial profiles of young adolescent females seeking treatment for an eating disorder. Journal of Adolescent Health 2007: 40: pp 351-356. Kohn M, Rees JM, Brill S, Fonseca H, Jacobson M, Katzman DK, Loghmani ES, Neumark-Sztainer D, Schneider M: Preventing and treating adolescent obesity: Position paper of the Society for Adolescent Medicine. Journal of Adolescent Health 2006: 38: pp 784-787. Pinhas L, Steinegger C, Katzman DK: Clinical assessment and physical complications. In: Eating Disorders in Childhood and Adolescence 3rd Edition. Lask B, Bryant-Waugh R (ed). London, Brunner-Routledge, 2006. AUDIO EDUCATIONAL PROGRAM Katzman DK, Golden NH, Kreipe RE: Eating disorders in adolescents. In: Critical Conversations with Pediatric Experts (Metzl K, ed). PrepAudio Vol 2: No 2: American Academy of Pediatrics, 2007. FUNDING Norris M, Boydell KM, Pinhas L, Katzman DK: Ana and the internet: A review of pro-anorexia websites. International Journal of Eating Disorders 2006: 39: pp 443-447. Pawelski J, Perrin E, Foy J, Allen C, Crawford J, Del Monte M, Kaufman M, Klein J, Smith K, Springer S, Tanner J, Vickers D: The effects of marriage, civil union, and domestic partnership laws on the health and well-being of children. Pediatrics 2006: 118: pp 349-364. Richardson D, Glover Takahashi S, Nasmith L, Leslie K, Bandiera G, Paterson M, Power B, Oandason I, Frank JR: The CanMEDS Ttrain-theTrainer Collaborator Faculty Development Program. Ottawa: The Royal College of Physicians and Surgeons of Canada, 2007. Richard P. Millman and the Working Group on Sleepiness in Adolescents/Young Adults; and AAP Committee on Adolescence. Excessive sleepiness in adolescents and young adults: Causes, consequences, and treatment strategies. Pediatrics 2005: 115: pp 1774-1786 BOOKS AND BOOK CHAPTERS Kaufman M: Role of adolescent development in the transition process. Progress in transplantation. 2006: 16: pp 286-290. Pinhas L, Katzman DK, Dimitropoulos G, Woodside B: Binging and bulimia nervosa in children and adolescents. In: Child and Adolescent Eating Disorders (Jaffa T, McDermott B, eds). Cambridge: Cambridge University Press, 2007. Wraith JE, Clarke JTR: The mucopolysaccharidoses. In: Physician’s Guide to the Treatment and Follow-up of Metabolic Diseases (Blau N, Hoffmann GF, Leonard J, Clarke JTR, eds). Springer-Verlag, Berlin, 2006. Adolescent Health Care: A Practical Guide, Fifth Edition (Neinstein L, Gordon C, Katzman DK, Rosen D, Woods E, eds). Philadelphia: Lippincott Williams & Wilkins, 2007. Effects of Anorexia Nervosa on Peak Bone Mass. Klibanski A, Katzman DK, Misra M, Herzog D, Levitsky L. National Institute of Health/National Institute of Diabetes and Digestive & Kidney Diseases ($3,801,660 2003-2008) Family therapy and fluoxetine in the treatment of adolescent anorexia nervosa. Agras S, Brandt H, Halmi K, Johnson C, Kaye W, Wilfley D, Woodside B, Pinhas L, Katzman DK. National Institute of Mental Health (TGH and SickKids site: $675,000 funds released with REB approval for 5 years; Total: $6,250,000 over five years) Positive Youth Project Capacity Building Grant. Canadian AIDS Treatment Information Exchange. Flicker S, Read S, Goldberg E, Skinner H, Veinot T, Taylor D & McClellan A. Ontario HIV Treatment Network ($25,000 2006-2007) Leadership: “Creating Communities of Practice for Interprofessional Collaboration (IPC) in Toronto” Kwan D, Leslie K, Lieff S, Nasmith L, Oandasan I, Reeves S, Silver I. Central project for a larger initiative funded by: Ministry of Health and LongTerm Care Interprofessional, Mentorship, Preceptorship, Leadership and Coaching Fund ($3,400,000 2007) A Longitudinal Impact Evaluation of the Centre for Faculty Development’s Stepping Stones Certificate Program: Educator Identity and Professional Networks. Richardson D, Wagner S, Dionne A, Leslie K, Reeves S. University of Toronto Continuing Education Research and Development Fund ($5,000 2006) Katzman DK, Golden NE: Eating disorders. In: Adolescent Health Care: A Practical Guide, Fifth Edition (Neinstein L, Gordon C, Katzman DK, Rosen D, Woods E, eds). Philadelphia: Lippincott Williams & Wilkins, 2007. DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 7 Cardiology DIVISIONAL OVERVIEW Over the past decade, the Divisional activities, in close collaboration with surgical and intensive care colleagues, have contributed to our markedly improved preoperative diagnosis and postoperative outcomes for children undergoing cardiac surgery, led the field in development of therapeutic catheter techniques, made important contributions to our understanding of fetal cardiac medicine, established one of the largest paediatric cardiac transplantation centres worldwide, and has won international recognition for its contribution in the field of epidemiology of acquired and congenital heart disease and surgical outcomes. Each of these areas has provided the substrate for the large clinical research output of the Division which is underpinned by our world-class laboratory science program. One of the most exciting clinical developments has been the recent commissioning of an integrated suite of catheter laboratories and magnetic resonance scanning facility. This unique fusion will allow ‘simultaneous’ multi-mode investigation and treatment, setting the scene for MRI-guided interventional procedures in children, and providing for unique physiologic insights into cardiovascular disease. This philosophy of clinical, academic and administrative integration led to formation of the Labatt Family Heart Centre in 2007, with major new financial support for the formation of chairs and fellowships, development of infrastructure, and the endowment of an innovation fund providing internal revenue for seed-funding and Heart Centresponsored thematic research. Division Head Dr. Andrew Redington Full-Time Dr. Lee Benson Dr. Tim Bradley Dr. Rajiv Chaturvedi Dr. Anne Dipchand Dr. Mark Friedberg Dr. Fraser Golding Dr. Gil Gross Dr. Robert Hamilton Dr. Edgar Jaeggi Dr. Paul Kantor Dr. Joel Kirsh Dr. Kyong-Jin Lee Dr. Brian McCrindle Dr. Seema Mital Dr. Jennifer Russell Dr. Elizabeth Stephenson The cardiovascular research focus within the Research Institute has made fundamental contributions to our understanding of the cellular biology and molecular genetics of pulmonary vascular bed, with recent diversification to include the study of the fetal myocardial development, and developmental genetics, detailed study of the intercellular structure of the heart and vessels, electrophysiology of the developing heart and immunology of cardiac transplantation. While undergoing a change in leadership, it is expected that high quality basic and translational research, with an emphasis on direct clinical applicability, will continue to form the bedrock of our academic program. Finally, the Royal College accredited Paediatric Cardiology Training Program, is the largest in Canada and one of the largest and most sought after in North America. As such, its fellows have populated cardiology groups throughout the world, many going on to become leaders of our specialty. Part-Time Dr. Tiscar CavalleGarrido Dr. Jack Colman Dr. Rejane Dillenburg Dr. Alexandra Jevremovic Dr. Anilkumar Menon Dr. Mark Friedberg The Division of Cardiology was formally established in the 1940’s and is now one of the largest and most successful in the world. Its philosophy has remained unchanged since its inception over sixty years ago; to provide the highest quality care in an evidence-based environment of clinical innovation and scientific discovery. Dr. Norman Musewe Dr. Lynne Nield Dr. Rachel Wald Dr. Roxanne Walker Adjunct Dr. Fred Keeley (Research Scientist) Cross-Apointees Dr. Tilman Humpl Dr. Steven Schwartz Dr. Ben Sivarajan Dr. Shi-Joon Yoo Clinical Fellows Dr. Abdulmajeed Al-Otay Dr. Merna Atiyah Dr. José Castro-Sancho Dr. Robin Clegg Dr. Gaute Døhlen Dr. Scott Fox Dr. Brian Grant Dr. Lars GrosseWortmann Dr. Ra Han Dr. Nasser Moiduddin Dr. Masaki Osaki Dr. Lucy Roche Dr. Imran Sajan Dr. Mark Walsh Dr. Roland Weber Departmental Clinical Fellows Dr. Alex Gooi Dr. Dragos Predescu Research Fellows Dr. Kentaro Asoh Dr. June Huh Dr. Maria Ilina Dr. Jon Lee Research Associates Dr. Jia Li Dr. Jing Li Subspecialty Residents Dr. Matthew Crystal Dr. Alejandro Floh Dr. Deborah Fruitman Paula Capela Dr. Derek Wong Esther Delea Dr. Marko Vezmar Karen Hodgson Susan Jessop Administrative Staff Shelley Marks Iris Biberstein Fran Matthews Theresa Braggs Shloka Mirchandaney Sandra Brisebois Andrea Morris Eleanor Paulino Rooma Vaidya Denise Vanek Linda Vorano Shirley Wang HONOURS AND AWARDS Dr. Anne Dipchand: James C. Fallis Teaching Award (Invited Speaker), Division of Paediatric Emergency Medicine, The Hospital for Sick Children, 2006. Dr. Paul Kantor: Second Prize, Oral Abstract Presentation. “Resting cardiac index, ejection fraction ad exercise capacity in a pediatric heart failure clinic population”. The Second International Conference on Heart Failure in Children and Young Adults, Laguna Niguel, CA, 2006. Dr. Paul Kantor: Third Prize, Oral Abstract Presentation. “Effect of medical therapy and transplantation on the natural history of pediatric dilated cardiomyopathy: A 25 year retrospective study”. The Second International Conference on Heart Failure in Children and Young Adults, Laguna Niguel, CA. 2006. Dr. Dragos Predescu: Third Prize, abstract poster annual resident research day: “Resting cardiac index, ejection fraction ad exercise capacity in a pediatric heart failure clinic population”. The Second International Conference on Heart Failure in Children and Young Adults, Laguna Niguel, CA. 2006 PUBLICATIONS Akikusa JD, Feldman BM, Gross GJ, Silverman ED, and Schneider R: Sinus bradycardia following intravenous ‘pulse’ methylprednisolone. Pediatrics 2007: 119: pp e778-e782. Aleem IS, Karamlou T, Benson LN, McCrindle BW: Transcatheter device versus surgical closure: A clinical decision analysis. Catheterization and Cardiovascular Interventions 2006: 67: pp 630-636. Alpert B, McCrindle B, Daniels S, Dennison B, Hayman L, Jacobson M, Mahoney L, Rocchini A, Steinberger J, Urbina E, Williams R: Recommendations for the blood pressure measurement in human and experimental animals: Part 1: Blood pressure measurement in humans (Letter) Circulation 2006 48: p e3. Dr. Fraser Golding and trainees Al-Radi OO, Caldarone CA, McCrindle BW, Jacobs JP, Williams G, Williams WG, Van Arsdell GS: Case complexity scores in congenital heart surgery: A comparative validation study of the Aristotle Basic Complexity score and the Risk Adjusted Congenital Heart Surgery (RACHS-1) scoring system. Journal of Thoracic and Cardiovascular Surgery 2007 133: pp 865-875. Alsenaidi K, Gurofsky R, Karamlou T, Williams WG, McCrindle BW: Management and outcomes of double aortic arch in 81 patients. Pediatrics 2006: 118: pp e1336-e1341. Alsoufi B, Karamlou T, Bradley TJ, Williams WG, Van Arsdell GS, Coles JG, Smallhorn JF, Nii M, Guerra V, Caldarone CA: Midterm Results of Aortic Valve Cusp Extension in the Treatment of Children with Aortic Insufficiency. Annals of Thoracic Surgery 2006: 82: pp 1292-1299. Alsoufi B, Karamlou T, McCrindle BW, Caldarone CA: Decision making in critical left ventricular outflow tract obstruction in neonates and infants. European Journal of Cardio-Thoracic Surgery 2007 31: pp 1013-1021. Alsoufi B, Karamlou T, Osaki M, Badiwala MV, Ching CC, Dipchand A, McCrindle BW, Coles JG, Caldarone CA, Williams WG, Van Arsdell GS: Surgical repair of multiple ventricular septal defects: the role of re-endocardialization strategy. Journal of Thoracic and Cardiovascular Surgery 2006: 132: pp 1072-1080. Anand N, McCrindle BW, Chiu-Man C, Hamilton RM, Kirsh JA, Stephenson E, and Gross GJ: Chronotropic incompetence in young patients with late postoperative atrial flutter: A case-control study. European Heart Journal 2006: 27: pp 2069-2073. Bernasconi A, Yoo SJ, Golding G, Langer JC, Jaeggi ET: Etiology and outcome of prenatally detected paracardial cystic lesions. Ultrasound in Obstetrics and Gynecology 2007: 29: pp 388-394. Bradley TJ, Alman B, Levin A, Chitayat D: Marfan syndrome. Parkhurst Exchange 2006: 14: pp 88-93. Bronzetti G, Chiu C, Stephenson E, Hamilton R, Gross G, Gao D, Neilson A, Kirsh JA: Simultaneous wide and narrow QRS complex tachycardias in a child: what is the mechanism? Journal of Cardiovascular Electrophysiology 2006: 17: pp 560-582. Crystal M, Benson L, Mikailian H: Catheter-based completion of the Fontan circuit: A non-surgical approach. Circulation 2006: 114: pp e5-e6. Dong-Sheng G, Wei-yi F, Chiu-Man C, Kirsh J, Gross G, Hamilton RM: QT hysteresis in long-QT syndrome children with exercise testing. Chinese Medical Journal 2007: 120: pp 179-182. Fernandez-Martorell P, Yoo S-J, Benson LN: An unusual form of anomalous systemic arterial supply to the left lung. Cardiology in the Young 2006: 16: pp 305-307. Friedberg MK, Bernheimer J, Chan FP, Silverman NH: Echocardiographic diagnosis of persistent fifth aortic arch. echocardiography 2007: 24: pp 258-262. Friedberg MK, Chang I, Silverman NH, Ramamoorthy C, Chan FP: Images in cardiovascular medicine. Near sudden death in an adolescent from a cardiac lipoma. Circulation 2006: 113: pp e778-e779. Friedberg MK, Feinstein JA, Rosenthal DN: A novel echo-doppler method for estimation of pulmonary arterial pressures. Journal of the American Society of Echocardiography 2006: 19: pp 559-562. Friedberg MK, Feinstein JA, Rosenthal DN: Noninvasive assessment of pulmonary arterial capacitance by echocardiography. Journal of the American Society of Echocardiography 2007: 20: pp 186-190. Friedberg MK, Silverman NH: Cardiac ventricular diastolic and dystolic duration in children with heart failure secondary to idiopathic dilated cardiomyopathy. American Journal of Cardiology 2006: 97: pp 101-105. Chaturvedi RC, Redington AN: Pulmonary regurgitation in congenital heart disease. Heart 2007: 93: pp 880-889. Friedberg MK, Silverman NH: The systolic to diastolic duration ratio in children with hypoplastic left heart syndrome: A novel doppler index of right ventricular function. Journal of the American Society of Echocardiography 2007: 20: pp 749-755. Chiu CC, Hamilton RM, Gow RM, Kirsh JA, McCrindle BW: Evaluation of computerized interpretation of the paediatric electrocardiogram. Journal of Electrocardiology 2007: 40: pp 139-143. Friedberg MK, Silverman NH, Dubin AM, Rosenthal DN: Mechanical dyssynchrony in children with systolic dysfunction secondary to cardiomyopath: A Tissue. Journal of the American Society of Echocardiography 2007: 20: pp 756-763. Cho JY, Min JY, Yoo SJ, McCrindle BW, Hornberger LK, Lee YH: Normal thymus diameter at fetal ultrasound. Ultrasound in Obstetrics and Gynecology 2007: 29: pp 634-638. Gao DS, Fang WY, Chiu-Man C, Kirsh J, Gross G, Hamilton RM: QT hysteresis in long-QT syndrome children with exercise testing. Chinese Medical Journal (England) 2007: 120: pp 179-182. Collins KK, Rhee EK, Kirsh JA, Cannon BA, Fish F, Dubin A, Van Hare GF: Cryoablation of accessory pathways in the coronary sinus in young patients: A multicenter study from the Pediatric and Congenital Electrophysiology Society’s Working Group on Cryoablation. Journal of Cardiovascular Electrophysiology 2007: 18: pp 592-597. Gross GJ: IK1: The long and the short QT of it. Heart Rhythm 2006: 3: pp 336-338. Gross GJ: Bradycardia-mediated ventricular electrical remodeling. Journal of Cardiovascular Electrophysiology 2006: 17: pp S178-S182. DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 11 Karamlou T, McCrindle BW, Williams WG: Surgery insight: Late complications following repair of tetralogy of Fallot and related surgical strategies for management. Nature Clinical Practice Cardiovascular Medicine 2006: 3: pp 611-622. Grosse-Wortmann L, Al-Otay A, Woo Goo H, Macgowan CK, Coles JG, Benson LN, Redington AN, Yoo SJ: Anatomical and functional evaluation of pulmonary veins in children by magnetic resonance imaging. Journal of the American College of Cardiology 2007: 49: pp 993-1002. Kavey RW, Allada V, Daniels SR, Hayman LL, McCrindle BW, Newburger JW, Parekh RS, Steinberger J: Cardiovascular risk reduction in high risk paediatric patients: A Scientific Statement from the American Heart Association Expert Panel on Population and Prevention Science; the Councils on Cardiovascular Disease in the Young, Epidemiology and Prevention, Nutrition, Physical Activity and Metabolism, High Blood Pressure Research, Cardiovascular Nursing, and the Kidney in Heart Disease; and the Interdisciplinary Working Group on Quality of Care and Outcomes Research. Journal of Cardiovascular Nursing 2007: 22: pp 218-253. Harris KC, Potts JE, Fournier A, Gross GJ, Kantoch MJ, Cote J-M, Sanatani S: A multicenter study of right ventricular outflow tract tachycardia in children. Journal of Pediatrics 2006: 149: pp 822-826. Herron TJ, Rostkova E, Kunst G, Chaturvedi RR, Gautel M, Kentish JC: Activation of myocardial contraction by the N-terminal domains of myosin binding protein-C. Circulation Research 2006: 98: pp 1290-1298. Horenstein MS, Idriss SF, Hamilton RM, Kanter RJ, Webster PA Jr, Karpawich PP: Efficacy of signal-averaged electrocardiography in the young orthotopic heart transplant patient to detect allograft rejection. Pediatric Cardiology 2006: 27: pp 589-593. Ilina MV, Jaeggi ET, Lee K-J: Neonatal rhabdomyoma causing right ventricular inflow obstruction with duct-dependent pulmonary blood flow: successful stenting of PDA. Catheterization and Cardiovascular Interventions 2007: 69: pp 881-885. Kantor PF, Slorach C: Clinical considerations for Heart Rhythm allied professionals: Understanding heart failure in congenital heart disease (CHD) patients. Heart Rhythm Journal 2007: 4: pp 248-250. Karamlou T, Blackstone EH, Hawkins JW, Jacobs ML, Kanter KR, Brown JW, Mavroudis C, Caldarone CA, Williams WG, McCrindle BW: Can pulmonary conduit dysfunction and failure be reduced in infants and children less than age two years at initial implantation? Journal of Thoracic and Cardiovascular Surgery 2006: 132: pp 829-838. Karamlou T, Gurofsky R, Al Sukhni E, Coles JG, Williams WG, Caldarone CA, Van Arsdell GS, McCrindle BW: Independent factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection. Circulation 2007: 115: pp 1591-1598. Kavey RW, Allada V, Daniels SR, Hayman LL, McCrindle BW, Newburger JW, Parekh RS, Steinberger J: Cardiovascular risk reduction in high risk paediatric patients: A Scientific Statement from the American Heart Association Expert Panel on Population and Prevention Science; the Councils on Cardiovascular Disease in the Young, Epidemiology and Prevention, Nutrition, Physical Activity and Metabolism, High Blood Pressure Research, Cardiovascular Nursing, and the Kidney in Heart Disease; and the Interdisciplinary Working Group on Quality of Care and Outcomes Research. Circulation 2006: 114: pp 2710-2738. Kharbanda RK, Li J, Konstantinov I, Cheung M, White P, Frndova H, Stokoe J, Cox P, Vogel M, Van Arsdell G, Macallister R, Redington AN: Remote ischemic preconditioning protects against cardiopulmonary bypass induced tissue injury – A preclinical study. Heart 2006: 92: pp 1506-1511. Kim N, Friedberg MK, Silverman NH: Diagnosis and prognosis of fetuses with double outlet right ventricle. Prenatal Diagnosis 2006: 26: pp 740-745. Kirsh JA, Stephenson EA, Redington AN: Recovery of left ventricular systolic function after biventricular resynchronization pacing in a child with repaired tetralogy of Fallot and severe biventricular dysfunction. Circulation 2006: 113: pp 691-692. Li J, Konstantinov IE, Cai S, Shimizu M, Redington AN: Systemic and myocardial oxygen transport responses to brain death in pigs. Transplantation Proceedings 2007: 133: pp 21-26. Li J, Zhang G, Holtby H, Humpl T, Caldarone CA, Van Arsdell GS, Redington AN: Adverse effects of dopamine on systemic hemodynamic status and oxygen transport in neonates after the Norwood procedure. Journal of the American College of Cardiology 2006: 48: pp 1859-1864. Li J, Zhang G, McCrindle BW, Holtby H, Humpl T, Cai S, Caldarone CA, Redington AN, Van Arsdell GS: Profiles of hemodynamics and oxygen transport derived by using continuous measured oxygen consumption after the Norwood procedure. Journal of Thoracic and Cardiovascular Surgery 2007: 133: pp 441-448. Mahadevan VS, Horlick EM, Benson LN, McLaughlin PR: Transcatheter closure of aortic sinus to left atrial fistula caused by erosion of Amplatzer septal occluder. Catheterization and Cardiovascular Interventions 2006: 68: pp 749-753. Maimon MS, Benson LN, Kirsh JK, Do A, Wilson GJ, Ratnapalan S: Cardiac perforation as a late complication of percutaneous atrial septal defect repair using Amplatzer septal occluder. Pediatrics 2006: 118: pp 1572-1575. Maimon MS, Ratnapalan S, Do A, Kirsh JA, Wilson G, Benson L: Cardiac perforation six weeks after percutaneous atrial septal defect repair using Amplatzer septal occluder. Pediatrics 2006: 118: pp e1572-e1575. McDonald CM, Karamlou T, Wengle JG, Gibson J, McCrindle BW: Nutritional environment available for outpatient, visitors and staff of children’s hospitals in Canada and the United States. Archives of Pediatrics & Adolescent Medicine 2006: 160: pp 900-905. McCrindle B, Karamlou T, Wong H, Gangam N, Trivedi K, Lee JK, Benson L: Presentation, management and outcomes of thrombosis for children with dilated cardiomyopathy. Canadian Journal of Cardiology 2006: 22: pp 685-690. McCrindle BW: Cardiovascular consequences of paediatric obesity: Will there be a future epidemic of premature cardiovascular disease? Paediatrics and Child Health 2007: 12: pp 175-177. McCrindle BW, Urbina EM, Dennison BA, Jacobson MS, Steinberger J, Rocchini AP, Hayman LL, Daniels SR, from the Atherosclerosis, Hypertension and Obesity in Youth Committee, Council of Cardiovascular Disease in the Young, American Heart Association: Drug therapy of high risk lipid abnormalities in children and adolescents. Circulation 2007: 115: pp 1948-1967. McCrindle BW, Urbina EM, Dennison BA, Jacobson MS, Steinberger J, Rocchini AP, Hayman LL, Daniels SR: Summary of the American Heart Association’s Scientific Statement on drug therapy of high risk lipid abnormalities in children and adolescents. Arteriosclerosis, Thrombosis, and Vascular Biology 2007: 27: pp 982-985. Dr. Seema Mital and lab staff Gross GJ, Chiu CC, Hamilton RM, Kirsh JA, Stephenson EA: Natural history of postoperative heart block in congenital heart disease: Implications for pacing intervention. Heart Rhythm 2006: 3: pp 601-604. McCrindle BW, Williams RV, Mital S, Clark BJ, Russell JL, Klein G, Eisenmann JC: Decreased levels of physical activity in pediatric patients after the Fontan procedure. Archives of Disease in Childhood 2007: 92: pp 509-514. McLaughlin P, Benson L, Horlick E: The role of cardiac catheterization in adult congenital heart disease. Cardiology Clinics 2006: 24: pp. 531-556. Nalli N, Stewart-Teixeira L, Dipchand AI: Amiodarone-sirolimus/tacrolimus interaction in a pediatric heart transplant patient. Pediatric Transplantation 2006: 10: pp 736-739. Newburger JW, Sleeper LA, McCrindle BW, Minich LL, Gersony W, Vetter VL, Atz AM, Li JS, Takahashi M, Baker AL, Colan SD, Mitchell PD, Klein GL, Sundel RP, for the Pediatric Heart Network Investigators: Randomized trial of pulse steroid therapy for primary treatment of Kawasaki disease. The New England Journal of Medicine 2007: 356: pp 663-675. Nielsen JM, Kristiansen SB, Ringgaard S, Nielsen TT, Flyvbjerg A, Redington AN, Botker HE: Left ventricular volume measurement in mice by conductance catheter. Evaluation and optimization of calibration. American Journal of Physiology – Heart and Circulatory Physiology March 23, 2007 [Epub ahead of print]. Nii M, Hamilton RM, Fenwick L, Kingdon JC, Roman KS, Jaeggi ET: Assessment of fetal atrioventricular time intervals by tissue Doppler and pulse Doppler echocardiography: normal values and correlation with fetal electrocardiography. Heart 2006: 92: pp1831-1837. Nii M, Roman KS, Kingdom J, Redington AN, Jaeggi ET: Assessment of the evolution of normal fetal diastolic function during mid and late gestation by spectral Doppler tissue echocardiography. Journal of the American Society of Echocardiography 2006: 19: pp 1431-1437. Ozawa A, Chaturvedi R, Lee K-J, Benson LN: Femoral vein hemostasis in children using a suture-mediated closure device. Journal of Interventional Cardiology 2007: 20: pp 1-5. Peirone AR, Lee KJ, Golding IF, Marks S, Russell J, Benson LN: Exercise performance and blood pressure responses in children after stenting of aortic coarctation. Progress in Pediatric Cardiology 2006: 22: pp 161-164. Dr. Bob Hamilton Pihkala J, Yazaki S, Metha R, Lee K-J, Chaturvedi R, McCrindle BW, Van Arsdell G, Benson LN: Feasibility and clinical impact of Tran catheter closure of interatrial communications after a fenestrated Fontan procedure: medium-term outcomes. Catheterization and Cardiovascular Interventions 2007: 69: pp 1007-1014. Pollock BarZiv SM, McCrindle BW, West LJ, Edgell D, Coles JG, Van Arsdell GS, Bohn D, Perez R, Campbell A, Dipchand AI: Outcomes of pediatric patients bridged to heart transplantation from extracorporeal membrane oxygenation (ECMO) support. American Society for Artificial Internal Organs 2007: 53: pp 97-102. Redington AN: Determinants and assessment of pulmonary regurgitation in tetralogy of Fallot: Practice and pitfalls. Cardiology Clinics 2006: 24: pp 631-639. Reid GJ, Webb GD, Barzel M, McCrindle BW, Irvine MJ, Siu SC: Estimates of life expectancy by adolescents and young adults with congenital heart defects. Journal of the American College of Cardiology 2006: 48: pp 349-355. Roman KS; Fouron JC; Nii M; Smallhorn JF; Chaturvedi RR; Jaeggi ET: Determinanats of outcome in fetal pulmonary valve stenosis or atresia with intact ventricular septum. American Journal of Cardiology 2007: 99: pp 699-703. Sarkissian T, Beyenne J, Feldman B, McCrindle BW, Silverman ED: Longitudinal examination of lipid profiles in pediatric systemic lupus erythematosus. Arthritis & Rheumatism 2007: 56: pp 631-638. Schmidt MR, Smerup M, Konstantinov IE, Shimizu M, Li J, Cheung M, White PA, Kristiansen SB, Sorensen KE, Dzavik V, Redington AN, Kharbanda RK: Intermittent peripheral tissue ischemia during coronary ischemia reduces myocardial infarction: Ffirst demonstration of remote ischemic preconditioning. American Journal of Physiology – Heart and Circulatory Physiology 2007: 292: pp H1883-H1890. Shimizu M, Konstantinov IE, Kharbanda RK, Cheung MH, Redington AN: Effects of intermittent lower limb ischaemia on coronary blood flow and coronary resistance in pigs. Acta Physiologica 2007: 190: pp 103-109. Sleeper LA, Anderson P, Hsu DT, Mahony L, McCrindle BW, Roth SJ, Saul JP, Williams RV, Geva T, Colan SD, Clark BJ: Design of a large crosssectional study to facilitate future clinical trials in children with the Fontan palliation. American Heart Journal 2006: 152: pp 427-433. Stephenson EA, Kaltman JR: Current state of the art for use of pacemakers and defibrillators in patients with congenital cardiac malformations. Cardiology in the Young 2006: 16: pp 151-156. Stoica SC, Satchithananda DK, White PA, Sharples L, Parameshwar J, Redington AN, Large SR: Brain death leads to abnormal contractile properties of the human donor right ventricle. Journal of Thoracic and Cardiovascular Surgery 2006: 132: pp 116-123. Suto F, Zhu W, Chan A, and Gross GJ: IKr and IKs remodeling differentially affects QT interval prolongation and dynamic adaptation to heart rate acceleration in bradycardic rabbits. American Journal of Physiology – Heart and Circulatory Physiology 2007: 292: pp H1782-H1788. Takahashi K, Guerra V, Roman KS Nii M, Redington AN, Smallhorn JF: Three-dimensional echocardiography improves the understanding of the mechanisms and site of left atrioventricular valve regurgitation in atrioventricular septal defect. Journal of the American Society of Echocardiography 2006: 19: pp 1502-1510. Thavendrianathan P, Jones E, Han RK, CullenDean G, Helden E, Conner WT, McCrindle BW: Association between physical activity, adiposity and lipid abnormalities for children with familial hyperlipidemia. European Journal of Cardiovascular Prevention & Rehabilitation 2007: 14: pp 59-64. Wald RM, Tham EB, McCrindle BW, Goff DA, McAuliffe FM, Golding F, Jaeggi ET, Hornberger LK, Tworetzky W, Nield LE: Outcome after prenatal diagnosis of tricuspid atresia: A multicenter experience. American Heart Journal.2007: 153: pp 772-778. Walsh MA, Lee K-J, Chaturvedi R, Van Arsdell GS, Benson LN: Radiofrequency perforation of right ventricular outflow tract as a palliative strategy for pulmonary atresia with ventricular septal defect. Catheterization and Cardiovascular Interventions 2007: 69: pp 1015-1020. Zhou YQ, Bishay R, Feintuch A, Tao K, Golding F, Zhu W, West LJ, Henkelman RM: Morphological and functional evaluation of murine heterotopic cardiac grafts using ultrasound biomicroscopy. Ultrasound in Medicine and Biology 2007: 33: pp 870-879. Zhou YQ, Bishay R, Feintuch A, Tao K, Golding F, Zhu W, West LJ, Henkelman RM: Morphological and functional observations of murine heterotopic cardiac isografts using ultrasound biomicroscopy. Journal of the American Society of Echocardiography 2006: 19: p 696. BOOK CHAPTERS Cheung M, Redington AR: Systolic and diastolic dysfunction. In: Heart Failure in Children and Young Adults: From Molecular Mechanisms to Medical and Surgical Strategies (Chang AC, Towbin JA, eds). Saunders Elsevier, Philadelphia, 2006. Ilina M, Stephenson EA: Conduction defects: Atrioventricular and bundle branch block. In: Electrocardiograms for the Family Physician (Yadava, OP, ed). Pushpanjali Medical Publication, Delhi, India, 2007. FUNDING A randomized, double-blind, multi-center study of antiarrhythmic medications in the treatment of infants with Supraventricular Tachycardia (SAMIS Trial). Sanatani S, McCrindle, BW, Stephenson E, Potts J, Etheridge S. The Heart and Stroke Foundation of Canada ($111,420 2006-2007; $444,060 four years to 2010) A transatlantic network to define the mechanisms, mediators and clinical applications of remote ischemic preconditioning. Redington AN, Callahan J, Wilson G, Kharbanda R, Toftegaard Nielsen T, Botker HE, McCrindle, BW. The Leducq Foundation (US$6,523,095 2006-2011) Characterization of circulating effectors and temporal profiling of remote ischaemic preconditioning. Redington AN, Callahan J, Gross GJ, Kharbanda R. Canadian Institutes of Health Research ($324,951 2006-2009) Clinical and genomic responses to remote ischemic preconditioning. McCrindle BW, Redington AN, Arab S, Coles J, Holtby H, Liu P, Schwartz S, Van Arsdell G. The National Heart Lung and Blood Institute/National Institutes of Health (US$432,091 2006-2007; US$2,047,004 five years to 2011) Clinical and laboratory evaluation of acute rejection, myocyte growth, repair and oxidative stress following de novo cardiac transplant: A comparison between Tacrolimus- and Cyclosporine-based immunprophylactic regimens with mycophenolic acid therapeutic drug monitoring. Dipchand AI, Pollock BarZiv S, AllainRooney T, Kantor PF. Astellas Pharma Canada Inc. ($65,000 2006-2008) Contractile and electrophysiologic adaptations to constrained heart rate conditions. Gross GJ, Redington AN. The Heart and Stroke Foundation of Ontario ($209,097 2006-2008) Contribution of 3-dimensional echocardiography to surgery for left ventricular outflow tract obstruction in children. Friedberg MK, Van Arsdell G, Calderone C, Coles J, Golding F, Bradley T, and Gooi A. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($9,819 20072008) Cyclosporine C2 Monitoring in paediatric heart transplant recipients: A pilot study. Dipchand AI, Allain-Rooney T. Novartis Pharmaceuticals Inc. ($17,223 2006-2007) 16 DEPARTMENT OF PAEDIATRICS Effects of remote ischemic preconditioning on the clinical investigation and genomic responses to open heart surgery in children. Redington AN, McCrindle BW. Canadian Institutes of Health Research ($100,000 2007-2008) Optimizing heart health for children after the fontan procedure through physical activity. McCrindle BW, Longmuir P, Russell J. The Heart and Stroke Foundation of Canada ($62,184 2006-2007; $119,967 two years to 2008) Effects of right ventricular intra-ventricular mechanical dyssynchrony on right and left ventricular dysfunction in tetralogy of fallot. Friedberg MK, Redington AN, Yoo S-J, Stephenson E, Russell J, Kantor P, Roche L. National Grants Program, The Hospital for Sick Children ($108,766 2007-2008) Perioperative use of thymoglobulin in paediatric heart transplant recipients. Dipchand AI, PollockBarZiv SM, Allain-Rooney T, Kantor PF. Genzyme Canada ($5,000 2006-2007) HLA Sensitization: Prevalence and impact of alloantibodies in paediatric heart transplant recipients. Dipchand AI, Pollock-Barziv S, McCrindle BM. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($10,000 2007-2008) Improving paediatric cardiac care in Ontario: Leveraging information to enhance patient outcomes, provide accountability and system monitoring. Kantor PF, Syed N, MacDonald S. Ontario Ministry of Health and Long-Term Care ($250,000 2006-2007) Individualized heparin and protamine management in infants undergoing cardiopulmonary bypass for cardiac surgery reduces bleeding and blood transfusion requirements. Gruenwald C, McCrindle BW, Chan A, Van Arsdell G, Holtby H. The Heart and Stroke Foundation of Canada ($57,063 2006-2007; $108,566 two years to 2008) Inflammation and atherosclerosis in paediatric rheumatic disease. Bradley TJ, Beyene J, Schneider R, Feldman B, Silverman ED. The Labatt Family Heart Centre – Innovation Fund ($24,000 2007-2008) Intrauterine growth retardation associated with and without placental insufficiency: Impact on myocardial function and on arterial compliance. Jaeggi ET, Bradley TJ, Keating SJ, Kingdom JCP. The Heart and Stroke Foundation of Ontario ($77,255 2006-2008) Late functional status in a cohort of survivors with pulmonary atresia intact ventricular septum from the Congenital Heart Surgeons Society Study. McCrindle BW, Caldarone C. The Children’s Heart Foundation (US$86,308 2006-2007; US$89,190 2007-2008) Molecular expression and physiology in developing atrioventricular nodal cells. Hamilton RM, Gross GJ, Silverman ED. Canadian Institutes of Health Research ($370,500 2006-2009) ANNUAL REPORT 2006-07 Pilot study of carotid intima medial thickness in association with second trimester uterine artery Doppler sonography. Lausman A, Bradley TJ, Kingdom JCP, Ray J. The Physicians’ Services Incorporated Foundation ($13,500, 2006-2007) Role of chronic inflammation and systemic lupus erythematosus-related factors in the development of premature atherosclerosis in paediatric SLE. Silverman ED, Bargman JM, Beyene J, Bradley TJ. The Heart and Stroke Foundation of Ontario ($304,826 2006-2009) Temporal profiling and circulating effectors of remote ischemic preconditioning. Redington AN, Callahan J. Canadian Institutes of Health Research ($284,000 2005-2008) The effect of remote ischemic preconditioning on clinical and genetic responses to open heart surgery in children. McCrindle BW, Redington AN. The Canadian Institutes of Health Research ($97,360 2007-2008) The SAMIS Trial: Multicenter study of antiarrhythmic medications for treatment of infants with supraventricular tachycardia. Sanatani, S, Stephenson EA, Potts J, McCrindle BM, Etheridge S. The Heart and Stroke Foundation of British Columbia and Yukon ($440,060 2006-2010) Validation of accelerometry as a measure of physical activity and inactivity in children with chronic disease. Feldman B, McCrindle BW, Stephens S, Takken T, Wright V, Tremblay M, Eslinger D, Beyene J, Walker-Schneiderman J, Biggar W, Longmuir P, Vandernet J. The Canadian Institutes of Health Research ($65,000 2007-2008) Ventricular force and vascular stiffness in the evaluation of paediatric heart failure. Kantor PF, Bradley T (co-PI), Roche SL, Slorach C. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($9,100 1997) Ventricular repolarization in bradycardic electrical remodeling. Gross GJ. The Heart and Stroke Foundation of Ontario ($268,447 2007-2010) Ventricular repolarization in complete heart block. Gross GJ. The Heart and Stroke Foundation of Ontario ($208,406 2004-2007) Dr. Lee Benson Vogel M, Redington AN: Assessment of contractile function by Doppler myocardial imaging: isovolumic versus ejection phase indices. In: Doppler Myocardial Imaging (Sutherland GR, Hatle L, Claus P, D’hooge J, Bijnens BH, eds). BSWK, Hasselt, Belgium, 2006. Division Head Dr. Rosanna Weksberg Dr. Stephen Meyn Dr. Ahmad Teebi Full-Time Dr. David Chitayat Dr. Joe Clarke Dr. Annette Feigenbaum Part-Time Dr. Alan Berzen Dr. Karen Chong Dr. Leona Fishman Dr. Chumei Li Clinical Fellows Dr. Gustavo Maegawa Dr. Marianne Rohrbach Dr. David Skidmore Dr. Hanna Faghfoury Dr. Sharan Goobie Dr. Linda Kim Dr. Lynette Penney Residents Dr. Fathiya Al-Murshedi Dr. Tawfeg Ben-Omran MSc Students Martha Balicki Mireille Cloutier Miranda Galvin Seema Jamal Laura Palma Christina Paradiso Sari Zelenietz Administrative Staff Michael Delaney Susan Dukhedin-Lalla Jennifer Ewing Delizia Ferri Laurie Furtado Sandy Maksimowska Hamali Maru Ardian Shtylla Rozmin Visrama Clinical and Metabolic Genetics DIVISIONAL OVERVIEW The Division of Clinical and Metabolic Genetics is recognized nationally and internationally for its contributions to clinical genetics, genetics research and education. One recent example of such leadership included the convening of key individuals from across the province for a retreat aimed at integrating negotiations with the Ontario Ministry of Health for resourcing clinical and laboratory genetic services. This highly successful provincial retreat was sponsored by the Department of Paediatrics and a second meeting is planned for 2008. New initiatives include the establishment of the DNA Resource Centre dedicated to facilitating genetic testing that is not available at SickKids. The DNA Resource Centre provides up-todate information regarding genetic testing available nationally and internationally, ensuring that all patients at SickKids have access to cutting-edge clinical and research testing options. Expansion of provincial newborn screening has led to the development of an independent Newborn Screening Program which is staffed by two nurses, a genetic counsellor, a dietician, a social worker, an information coordinator and a metabolic geneticist. The Newborn Screening Program involves multiple subspecialties at SickKids, including Genetics, Endocrinology, Haematology and Respirology. Research endeavours in the Division are a vital part of our program and currently focus on the translation of new genomic information for patients and family members. A rapid increase in the use of microarrays for genetic testing has expanded the need for clinical evaluation of new data being generated so that appropriate and relevant information can be provided to patients and their families. There were two awards for the Sixth Annual Starbucks Clinical Research Summer Students Competition. The recipients were Katie Marchington for a study on autism and epigenetics and Margarita Kaliazina for a study of the resources required to transition paediatric genetic metabolic patients to adult hospitals. The Division of Clinical and Metabolic Genetics has developed and participated in numerous educational activities for different training programs within the hospital. The Division provides excellent teaching opportunities with important links to the Research Institute and the Department of Laboratory Medicine. The Division directs Fellowship and Residency training programs in genetic medicine as well as the MSc Program in Genetic Counselling which was reaccredited this past year by the Ontario Council of Graduate Studies. Dr. Andreas Schulze The reorganization of the Neurofibromatosis type1 and Skeletal Dysplasia Clinics highlight the Division’s commitment to enhance patient services through interdisciplinary care. The recent establishment of the Neurogenetics Clinic also demonstrates our focus on providing excellent genetic care to our patients and their families. DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 19 Al-Hassnan ZN, Teebi AS: Craniofacial anomalies, humero-radial synostosis, rhizomelic limb shortness: Previously unrecognized autosomal recessive syndrome. American Journal of Medical Genetics 2007: 143: pp 521-5277. Alkazaleh F, Chaddha V, Viero S, Malik A, Anastasiades C, Sroka H, Chitayat D, Toi A, Windrim RC, Kingdom JC: Second-trimester prediction of severe placental complications in women with combined elevations in alphafetoprotein and human chorionic gonadotrophin. American Journal of Obstetrics and Gynecology 2006: 194: pp 821-827. Bader R, Hornberg LK, Nijmeh LJ, Al-Kazaleh F, Ryan G, Toi A, Viero S, Butany J, Chitayat D: Fetal pericardial teratoma: Presentation of two cases and review of literature. American Journal of Perinatology 2006: 23: pp 53-58. Bassett A, Caluseriu O, Weksberg R, Young D, Chow E: Catechol-O-methyl transferase and expression of schizophrenia in 73 adults with 22q11 Deletion Syndrom. Biological Psychiatry 2007: 61: pp 1135-1140. Beatty L, Weksberg R, Sadowski P: Detailed analysis of the methylation patterns of the KvDMR1 imprinting control region of human chromosome 11. Genomics 2006: 87: pp 46-56. Ben-Neriah Z, Withers S, Thomas M, Toi A, Chong K, Pai A, Velscher L, Vero S, Keating S, Taylor G, Chitayat D: OEIS complex: Prenatal ultrasound and autopsy findings. Ultrasound in Obstetrics and Gynecology 2007: 29: pp 170-177. Ben-Omran T, Wong H, Blaser S, Feigenbaum A: Late-onset Cobalamin-C disorder: Challenging diagnosis. American Journal of Medical Genetics (Part A) 2007: 143: pp 979-984. Blaser S, Propst E, James A, Feigenbaum A, Martin D, Shannon P, Papsin B: Inner ear dysplasia is common in children with Down syndrome (trisomy 21). Laryngoscope 2006: 116: pp 2113-2119. 20 DEPARTMENT OF PAEDIATRICS Cameron JM, Levandovskiy V, MacKay N, Raiman J, Clarke JTR, Feigenbaum A, Elpeleg O, Robinson BH: Novel mutations in lipoamide dehydrogenase deficiency in two cousins with borderline-normal PDH complex activity. American Journal of Medical Genetics 2006: 140: pp 1542-1552. Tolmie JL, Tomlin P, Voit T, Weschke B, Woods CG, Lebon P, Bonthron DT, Ponting CP, Jackson AP: Mutations in genes encoding ribonuclease H2 subunits cause Aicardi-Goutieres syndrome and mimic congenital viral brain infection. Nature Genetics 2006: 38: pp 910-916. Cameron JM, Levandovskiy V, MacKay N, Raiman J, Renaud DL, Clarke JTR, Feigenbaum A, Elpeleg O, Robinson BH: Novel mutations in lipoamide dehydrogenase deficiency in two cousins with borderline-normal PDH complex activity. American Journal of Medical Genetics 2006: 140: p 14. Feigenbaum A, Doherty E, Bai R, Kwon H, Tan D, Sloane A, Robinson B, Wong LJ: Novel mitochondrial DNA mutations associated with myopathy, cardiomyopathy, renal failure and deafness. American Journal of Medical Genetics 2006: 140: pp 2216-2222. Castagna A, Addis J, McInnes RR, Clarke JTR, Ashby P, Blaser S, Robinson BH: Late onset Leigh syndrome and ataxia due to a T to C mutation at bp 9,185 of mitochondrial DNA. American Journal of Medical Genetics (Part A) 2007: 143: pp 808-816. Ferrentino R, Bassi MT, Chitayat D, Tabolacci E, Meroni G: MID1 mutation screening in a large cohort of Opitz G/BBB syndrome patients: Twentynine novel mutations identified. Human Genetics 2007: 28: pp 206-207. Chitayat D, Sroka H, Keating S, Colby RS, Ryan G, Toi A, Blaser S, Viero S, Devisme L, BouteBenejean O, Manouvrier-Hanu S, Mortier G, Loeys B, Rauch A, Bitoun P: The PDAC syndrome (pulmonary hypoplasia/agenesis, diaphragmatic hernia/eventration, anophthalmia/microphthalmia, and cardiac defect) (Spear syndrome, MatthewWood syndrome): Report of eight cases including a living child and further evidence for autosomal recessive inheritance. American Journal of Medical Genetics (Part A) 2007: 143: pp 1268-1281. Chitayat D: First trimester ultrasound diagnosis of lethal multiple pterygium syndrome. Fetal Diagnosis and Therapy 2006: 21: pp 466-470. Clarke JTR: Fabry disease. Annals of Internal Medicine 2007: 146: pp 425-433. Crow YJ, Leitch A, Hayward BE, Garner A, Parmar R, Griffith E, Ali M, Semple C, Aicardi J, Babul-Hirji R, Baumann C, Baxter P, Bertini E, Chandler KE, Chitayat D, Cau D, Dery C, Fazzi E, Goizet C, King MD, Klepper J, Lacombe D, Lanzi G, Lyall H, Martinez-Frias ML, Mathieu M, McKeown C, Monier A, Oade Y, Quarrell OW, Rittey CD, Rogers RC, Sanchis A, Stephenson JB, Tacke U, Till M, ANNUAL REPORT 2006-07 Fluss J, Blaser S, Chitayat D, Akoury H, Glanc P, Skidmore M, Raybaud C: Molar tooth sign in fetal brain magnetic resonance imaging leading to the prenatal diagnosis of Joubert syndrome and related disorders. Journal of Child Neurology 2006: 21: pp 320-324. Georgiou DM, Nicolaou P, Chitayat D, Koutsou P, Babul-Hirji R, Vajsar J, Murphy J, Christodoulou K: A novel GDAP1 mutation 439delA is associated with autosomal recessive CMT disease. The Canadian Journal of Neurological Sciences 2006: 33: pp 311-316. Ghai S, Fong KW, Toi A, Chitayat D, Pantazi S, Blaser S: Prenatal US and MR Imaging Findings of Lissencephaly: Review of fetal cerebral sulcal development. Radiographics 2006: 26: pp 389-405. Gibas AL, Klatt R, Johnson J, Clarke JTR, Katz J: Pain in males and females with Fabry disease: A pilot study of the pain experienced by males and females with Fabry disease. Pain Research and Management 2006: 11: pp 185-192. Dr. Julian Raiman PUBLICATIONS Girirajan S, Mendoza-Londono R, Vlangos CN, Dupuis L, Nowak NJ, Bunyan DJ, Hatchwell E, Elsea SH: Smith-Magenis syndrome and moyamoya disease in a patient with del(17) (p11.2p13.1). American Journal of Medical Genetics 2007: 143: pp 999-1008. Gissen P, Tee L, Johnson CA, Genin E, Caliebe A, Chitayat D, Clericuzio C, Denecke J, Di Rocco M, Fischler B, Fitzpatrick D, Garcia-Cazorla A, Guyot D, Jacquemont S, Koletzko S, Leheup B, Mandel H, Sanseverino MT, Houwen RH, McKiernan PJ, Kelly DA, Maher ER: Clinical and molecular genetic features of ARC syndrome. Human Genetics 2006: 120: pp 396-409. Glanc P, Umranikar S, Koff D, Tomlinson G, Chitayat D: Fetal sex assignment by sonographic evaluation of the pelvic organs in the second and third trimesters of pregnancy. Journal of Ultrasound in Medicine 2007: 26: pp 563-569. Gundogan M, Fong K, Keating S, Pierre-Louis J, Chitayat D: First trimester ultrasound diagnosis of lethal multiple pterygium syndrome. Fetal Diagnosis and Therapy 2006: 21: pp 466-470. Hamamy HA, Teebi AS, Oudjhane K, Shegem NN, Ajlouni KM: Severe hypertelorism, midface prominence, prominent/simple ears, severe myopia, borderline intelligence, and bone fragility in two brothers: New syndrome? American Journal of Medical Genetics 2007: 143: pp 229-234. Kolomietz E, Ben-Omran T, Chitayat D, Mah M: Array-based genomic delineation of a familial duplication 11q14.1-q22.1 associated with recurrent depression. American Journal of Medical Genetics 2006: 141: pp. 214-219. Koren G, Cairns J, Chitayat D, Gaedigk A, Leeder SJ: Pharmacogenetics of morphine poisoning in a breastfed neonate of a codeine-prescribed mother. Lancet 2006: 368: p 704. Lenane P, Cammisuli S, Chitayat D, Krafchik B: What syndrome is this? KID syndrome (keratitis, ichthyosis, deafness). Pediatric Dermatology 2006: 23: pp 81-83. Maegawa G, Chitayat D, Blaser S, Whyte H, Thomas M, Kim P, Kim J , Taylor G, McNamara PJ: Duodenal and biliary atresia associated with facial, thyroid and auditory apparatus abnormalities. A new mandibulofacial dysostosis syndrome? Journal of Clinical Dysmorphology 2006: 15: pp 191-196. Maegawa GHB, Stockley T, Tropak M, Banwell B, Blaser S, Kok F, Giugliani R, Mahuran D, Clarke JTR: The natural history of juvenile or subacute GM2 gangliosidosis-21 new cases and literature review of 134 previously reported. Pediatrics 2006: 118: pp e1550-e1562. Maegawa GHB, Tropak M, Butner J, Stockley T, Kok F, Clarke JTR, Mahuran DJ: Pyrimethamine as a potential pharmacological chaperone for lateonset forms of GM2 gangliosidosis. Journal of Biological Chemistry 2007: 282: pp 9150-9161. Mahant S, Feigenbaum A: A child with an underrecognized form of metal delay: A congenital disorder of glycosylation. Canadian Medical Association Journal 2006: 175: pp 1369. Mallory SB, Krafchik BR, Lenane P, Cammisuli S, Chitayat D: What syndrome is this? Pediatric Dermatology 2006: 23: pp 81-83. Milic A, Blaser S, Robinson A, Viero S, Halliday W, Winsor E, Toi A, Thomas M, Chitayat D: Prenatal detection of microtia by MRI in a fetus with trisomy 22. Pediatric Radiology 2006: 36: pp 706-710. Pastores GM, Arn P, Beck M, Clarke JTR, Guffon N, Kaplan P, Muenzer J, Norato DY, Shapiro E, Thomas J, Viskochil D, Wraith JE: The MPS I registry-design, methodology, and early findings of a global disease registry for monitoring patients with Mucopolysaccharidosis Type I. Molecular Genetics and Metabolism 2007: 91: pp 37-47. Scaglia F, Hsu CH, Kwon H, Bai RK, Perng CL, Dai P, Chang HM, O’Brian Smith E, Whiteman DAH, Feigenbaum A, Gropman A, Wong LJC: Molecular bases of hearing loss in multisystemic mitochondrial cytopathy. Genetics in Medicine 2006: 8: pp 641-652. Seifert W, Holder-Espinasse M, Spranger S, Hoeltzenbein M, Rossier E, Dollfus H, Lacombe D, Verloes A, Chrzanowska KH, Maegawa GH, Chitayat D, Kotzot D, Huhle D, Meinecke P, Albrecht B, Mathijssen I, Leheup B, Raile K, Hennies HC, Horn D: Mutational spectrum of COH1 and clinical heterogeneity in Cohen syndrome. Journal of Medical Genetics 2006: 43: p e22. Shah PS, Siriwardena K, Taylor G, Steele L, Ray P, Blaser S, Chitayat D: Sudden infant death in a patient with FGFR3 P250R mutation. American Journal of Medicinal Genetics 2006: 140: pp 2794-2796. Shah PS, Weksberg R, Chitayat D: Overgrowth with severe developmental delay following IVF/ICSI: A newly recognized syndrome? American Journal of Medical Genetics 2006: 140: pp 1312-1315. Pasutto F, Sticht H, Hammersen G, GillessenKaesbach G, Fitzpatrick DR, Nurnberg G, Brasch F, Schirmer-Zimmermann H, Tolmie JL, Chitayat D, Houge G, Fernandez-Martinez L, Keating S, Mortier G, Hennekam RC, von der Wense A, Slavotinek A, Meinecke P, Bitoun P, Becker C, Nurnberg P, Reis A, Rauch A: Mutations in STRA6 Cause a broad spectrum of malformations including anophthalmia, congenital heart defects, diaphragmatic hernia, alveolar capillary dysplasia, lung hypoplasia, and mental retardation. American Journal of Human Genetics 2007: 80: pp 550-560. Shuman C, Smith AC, Steele L, Ray PN, Clericuzio C, Zackai E, Parisi MA, Meadows AT, Kelly T, Tichauer D, Squire JA, Sadowski P, Weksberg R: Constitutional UPD for Chromosome 11p15 in individuals with isolated hemihyperplasia is associated with high tumor risk and occurs following assisted reproductive technologies. American Journal of Medical Genetics 2006: 140: pp 1497-1503. Patel MS, Becker LE, Toi A, Armstrong DL, Chitayat D: Severe, fetal-onset form of olivopontocerebellar hypoplasia in three sibs: PCH type 5? American Journal of Medical Genetics 2006: 140: pp 594-603. Simchen MJ, Toi A, Silver M, Smith CR, Hornberger LK, Taylor G, Chitayat D: Fetal cardiac calcifications: Report of four prenatally diagnosed cases and review of the literature. Ultrasound in Obstetrics and Gynecology 2006: 27: pp 325-330. Potocki L, Bi W, Treadwell-Deering D, Carvalho CM, Eifert A, Friedman EM, Glaze D, Krull K, Lee JA, Lewis RA, Mendoza-Londono R, RobbinsFurman P, Shaw C, Shi X, Weissenberger G, Withers M, Yatsenko SA, Zackai EH, Stankiewicz P, Lupski JR: Characterization of Potocki-Lupski syndrome (dup(17)(p11.2p11.2)) and delineation of a dosage-sensitive critical interval that can convey an autism phenotype. American Journal of Human Genetics 2007: 80: pp 633-649. Sirrs SM, Clarke JTR: Agalsidase alfa therapy for Fabry disease. Expert Review of Endocrinology and Metabolism 2007: 2: pp 147-154. Ries M, Clarke JTR, Whybra C, Timmons M, Robinson C, Schlaggar BL, Pastores G, Lien YH, Kampmann C, Brady RO, Beck M, Schiffmann R: Enzyme replacement therapy with agalsidase alfa in children with Fabry disease. Pediatrics 2006: 118: pp 924-932. Rohrbach M, Chitayat D, Drake J, Velsher L, Sirkin WL, Blaser S: Prenatal diagnosis of fetal exencephaly associated with amniotic band sequence at 17 weeks of gestation by fetal magnetic resonance imaging. Fetal Diagnosis and Therapy 2006: 22: pp 112-115. Smith AC, Choufani S, Ferreira JC, Weksberg R: Growth regulation, imprinted genes and Chromosome 11p15.5. Annual Review Pediatrics Research 2007: 61: pp 43R-47R. Smith AC, Rubin T, Shuman C, Estabrooks L, Aylsworth AS, McDonald MT, Steele L, Ray PN, Weksberg R: New chromosome 11p15 epigenotypes identified in male monozygotic twins with Beckwith-Wiedemann syndrome. Cytogenetic and Genome Research 2006: 113: pp 313-317. Umranikar S, Glanc P, Unger S, Keating S, Fong K, Trevors CD, Myles-Reid D, Chitayat D: X-Linked dominant chondrodysplasia punctata: Prenatal diagnosis and autopsy findings. Prenatal Diagnosis 2006: 26: pp 1235-1240. Vincent J, Horike S, Choufani S, Paterson A, Roberts W, Szatmari P, Weksberg R, Fernandez B, Scherer S: An inversion inv (4) (p12-p15.3) in autistic sibling implicates the 4p GABA receptor gene cluster. American Journal of Medical Genetics 2006: 43: pp 429-434. Weksberg R, Shuman C, Wilkins-Haug L, Mann M, Stewart D, Croughan M, Rakowsky C, Leader A, Hall J, Friedman JM, Simpson JL, Holmes L, InfanteRivard C: Workshop report: Evaluation of genetic and epigenetic risks associated with assisted reproductive technologies and infertility. Elsevier Journal of Fertility and Sterility 2007: 88: pp 27-31. Weksberg, R, Stachon AC, Squire JA, Moldovan L, Bayani J, Meyn MS, Chow E, Bassett AS: Molecular characterization of deletion breakpoints in adults with 22q11 deletion syndrome. Human Genetics 2007: 120: pp 837-845. Yoon G, Kramer J. Zanko A, Guzijan M. Lin S, FosterBarber A, Boxer AL: Speech and language delay are early manifestations of Juvenile-Onset Huntington Disease. Neurology 2006: 67: pp 1265-1267. Yoon G, Oberoi S, Tristani-Firouzi M, Etheridge SP, Quitania L, Kramer JH, Miller BL, Fu YH, Ptácek LJ: Andersen-Tawil Syndrome. Prospective cohort analysis and expansion of the phenotype. American Journal of Medical Genetics 2006: 140: pp 312-321. Yoon G, Quitania L, Kramer JH, Fu YH, Miller BL, Ptácek LJ: Andersen-Tawil Syndrome. Definition of a neurocognitive phenotype. Neurology 2006: 66: pp 1703-1710. Zampino G, Pantaleoni F, Carta C, Cobellis G, Vasta I, Neri C, Pogna E, De Feo E, Delogu A, Sarkozy A, Atzeri F, Selicorni A, Rauen K, Cytrynbaum C, Weksberg R, Dallapiccola B, Ballabio A, Gelb B, Neri G, Tartaglia M: Diversity, parental germline origin, and phenotypic spectrum of de nova HRAS missense changes in Costello syndrome. Human Mutation 2007: 28: pp 265-272. BOOKS AND BOOK CHAPTERS Chitayat D, Babul-Hirji R: Preconceptional genetic counselling. In: Preventive Genetics (Gogate S, ed). Jaypee, New Delhi, India, 2006. MacRae AR, Chitayat D: Prenatal screening. In: Genetic Testing, Care, Consent, and Liability (Shart NF, Carter RF, Wiley-Liss, eds). Hoboken, New Jersey, USA. Chitayat D, Hall JG: Genetic aspects of twinning. In: Multiple Pregnancies (Kilby M, Baker P, Critchley H, Field D, eds). RCOG Press, London, England, 2006. Chitayat D, Koren G: Fetal malformations associated with drugs and chemicals: Visualization by sonography. Fetal MRI. In: Medication Safety in Pregnancy and Breastfeeding (Koren G, ed). McGraw-Hill, New York, 2007. Blau N, Hoffmann GF, Leonard J, Clarke JTR: Physician’s Guide to the Treatment and Follow-up of Metabolic diseases. Springer-Verlag, Berlin, 2006. Hoffmann GF, Clarke JTR, Leonard J: The management of metabolic emergencies. In: Physician’s Guide to the Treatment and Follow-up of Metabolic Diseases (Blau N, Hoffmann GF, Leonard J, Clarke JTR eds). Springer-Verlag, Berlin, 2006. Wraith JE, Clarke JTR: The mucopolysaccharidoses. In: Physician’s Guide to the Treatment and Followup of Metabolic Diseases (Blau N, Hoffmann GF, Leonard J, Clarke JTR eds). Springer-Verlag, Berlin, 2006. Phase III clinical trial of Coenzyme Q10 in mitochondrial disease. Multi-center double blind placebo controlled randomized crossover trial. Feigenbaum A. National Institutes of Health – Office of Rare Diseases (US$154,000 2007) Clarke JTR. Other storage disorders. In: Physician’s Guide to the Treatment and Follow-up of Metabolic Diseases (Blau N, Hoffmann GF, Leonard J, Clarke JTR eds). Springer-Verlag, Berlin, 2006. Stacpoole P, Feigenbaum A, Robinson B. National Institutes of Health, Department of Health and Human Services, Office of Orphan Products Development ($1,000,000 2006-2009) Clarke JTR. A clinical guide to inherited metabolic diseases. 3rd Edition. Cambridge University Press, Cambridge, 2006. PKU 006: Multidose trial of Phenoptin in PKU. Study approved and funded but closed by sponsor before enrollment. Feigenbaum A. Biomarin Pharmaceuticals ($16,485 2006) Mendoza-Londono R, Lee B: Cleidocranial dysplasia. In: GeneReviews at GeneTests: Medical Genetics Information Resource [database online]. Copyright, University of Washington, Seattle, www.genetests.org, 2006. Baskin B, Teebi AS: Cherubism. In: Gene Reviews at Gene Tests: Medical Genetics Information Resource [database online]. Copyright, University of Washington, Seattle, www.genetests.org, 2007. Teebi AS, Kennedy S, Chitayat D, Teshima I, Unger S, Babul-Hirji R, Shuman C, Weksberg R: Clinical genetics. In: Hospital for Sick Children Atlas of Paediatrics (Laxer R, Lee Ford Jones E, Friedman J, Gerstle T, eds). Current Medicine LLC, Philadelphia, 2006. Weksberg R, Sadowski P, Smith AC, Tycko B: Epigenetics. In: Emery & Rimoin’s Principles and Practice of Medical Genetics (Rimoin DL, Connor M, Pyeritz RE, Korf BR, eds). Churchill Livingstone, 5th Edition, Toronto, 2006. Geschwind MD, Yoon G, Goldman J: Adult-onset genetic disorders involving the frontal lobes. In: The Human Frontal Lobes, Functions and Disorders (Miller BL and Cummings JL, ed). Guilford Publications Inc, New York, 2006. FUNDING Canadian Molecular Cytogenetics Platform. Friedman JM, Chitayat D: Canadian Foundation for Innovation ($4,512,925 2005-2010) Rare diseases clinical research centre for urea cycle disorders. Clarke JTR, Feigenbaum A. Canadian Institutes of Health Research ($25,000 2006) Canadian Fabry disease initiative. Clarke JTR, multiple co-investigators across Canada. Canadian Institutes of Health Research ($1,200,000 2006) PKU 08: Phenoptin in PKU. Open label extension study. Feigenbaum A. Biomarin Pharmaceuticals ($25,500 2006-2009) Dynamics of human double-strand break repair. Meyn MS. Canadian Institutes of Health Research ($123,000 2004-2008) Canadian Molecular Cytogenetics Platform. Meyn MS. Canadian Foundation for Innovation ($4,800,000 2004-2009) Collaborative graduate training for program in molecular medicine. Meyn MS. Canadian Institutes of Health Research ($300,000 2002-2008) The National Genetic and Birth Defects Registry. Teebi AS. Prince Salman Institute for Handicap, Saudi Arabia ($750,000 2003-2008) Canadian Molecular Cytogenetic Platform. Friedman J, Weksberg R. Canadian Foundation for Innovation ($4,512,925 2004-2008) Integrative genomics for health research. Scherer S, Weksberg R. Canadian Foundation for Innovation ($3,600,262 2004-2008) Analysis of imprinting control in a tumour predisposing region on human chromosome 11p15. Weksberg R, Squire J. Canadian Institutes of Health Research ($359,565 2004-2007) Potential predictor of schizophrenia in a genetic high risk sample. Ontario project. Chow E, Bassett A, Weksberg R, Zipursky R, Mikulis D. Ontario Mental Health Foundation (Bill Jefferies Schizophrenia Endowment Fund) ($148,188 2002-2008) Gene expression of neuronal progenitors in a 1q22-linked familial subtype of schizophrenia. McCurdy R, Weksberg R. Canadian Psychiatric Research Foundation ($80,000 2005-2007) Hunter Outcome Survey (HOS). Clarke JTR. TKT/Shire ($16,000 2006) Computer assisted teaching software project. Clarke JTR, Al-Jasmi F. TKT/Shire ($60,000 2006) Autism: Searching for epigenetics errors. Weksberg R. SickKids Foundation New Ideas Grant ($50,000 2007-2008) Phenoptin in PKU PKU 004. Phase III multi-center open label variable dose clinical trial. Feigenbaum A. Biomarin Pharmaceuticals ($41,000 2006-2007) Pregnancy and development outcomes. Weksberg R, Goobie S, Kim L, Roberts W, Croughan M. The Physicians’ Services Incorporated Foundation ($20,000 2007-2008) DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 23 Clinical Pharmacology and Toxicology DIVISIONAL OVERVIEW The division is one of the two major training sites of the Royal College-accredited Clinical Pharmacology Residency program of the University of Toronto. Our medical residency training program spearheaded the recent change of the specialty residency training structure to add Emergency Medicine to the existing four entry disciplines, which include Paediatrics. This change enables us to train residents with emergency medicine background in the clinical toxicology stream of our education program. Our research programs focus on drug metabolizing enzymes and their regulation, drug transporters, placental and mammary gland drug transport and metabolism, and reproductive toxicology and teratology, including fetal alcohol syndrome research. The breadth and depth of these research programs are shown in the publication and funding records. In 2006 Dr. Anna Taddio received the Piafsky Young Investigator Award and Dr. Gideon Koren received the Education Award at the Canadian Society of Clinical Pharmacology. Dr. Shinya Ito The Division of Clinical Pharmacology and Toxicology focuses on a wide range of drug-related issues in paediatrics. The division is one of the largest and the most active paediatric pharmacology programs internationally. Our major clinical activities include a consultation service, the Motherisk Program, the Ontario Regional Poison Information Centre, and DART (Drug Allergy, Reactions and Toxicity) clinic, collaborating with the Division of Allergy and Immunology to provide specialized outpatient follow-up and management for those with various drug/toxin-related problems. The two toxicology information centres annually receive about 35,000 (Motherisk) and 75,000 (Poison Information Centre) inquiries, which have reached the limit of the response capacity of the systems. The Motherisk Program directed by Dr. Gideon Koren continues disseminating new knowledge in the field of drug safety for fetus and breastfeeding babies. The Ontario Regional Poison Information Centre led by Dr. Margaret Thompson has now become a hub for medical toxicology training, attracting many residents and fellows nationally and internationally. The Palliative and Bereavement Care Service directed by Dr. Christine Newman has recently joined the division that provides expertise specifically on drug therapy for terminally ill patients. 24 DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 Division Head Dr. Shinya Ito Scientific Staff Dr. Patricia Harper Full-Time Dr. Gideon Koren Dr. Christine Newman Dr. Irena Nulman Dr. Margaret Thompson Cross-Appointees/ Clinical Dr. Sylvain Baruchel Dr. Ran Goldman Dr. Sheila Jacobson Dr. Prashant Joshi Dr. Christopher Parshuram Part-Time Dr. David Juurlink Dr. Denis Scolnik Dr. Neil Shear Dr. Marco Sivilotti Cross-Appointees/ Scientific Dr. Lidia Derewlany Dr. Tom Einarson Dr. Anna Taddio Dr. Bonnie Stevens Clinical Fellows Dr. Radinka Boskovic Dr. Saskia de-Wildt Dr. Tali Finkelstein Dr. Yaron Finkelstein Dr. Facundo Garcia Bournissen Dr. Miho Inoue Dr. Dan Rhodes Dr. Arthur Staroselsky Dr. Nobuko Taguchi Dr. Toshihiro Tanaka Dr. Parvaneh Yazdanibrojeni Residents Dr. Lisa Thurgur (Emergency Medicine, Clinical Pharmacology) Research Fellows Dr. Alejandro A. Nava-Ocampo Dr. Shin-ichiro Takahashi Administrative Staff Ms. Niki Balamatsis Ms. Sarah Bondy Ms. Hila Halshtok Ms. Leah Moscato Ms. Donna Tedesco Research Associate Katarina Aleksa HONOURS AND AWARDS Koren G: Canadian Society for Clinical Pharmacology, Education Award, 2006. Koren G: Spirit of the Community Humanitarian Award, 2007. Taddio A: Piafsky Young Investigators Award, 2006. PUBLICATIONS Ahn E, Pairaudeau N, Pairaudeau N Jr, Cerat Y, Couturier B, Fortier A, Paradis E, Koren G: A randomized cross over trial of tolerability and compliance of a micronutrient supplement with low iron separated from calcium vs high iron combined with calcium in pregnant women. BioMed Central Pregnancy Childbirth 2006: 6: p 10. Aleksa K, Ito S, Koren G: Enantioselective metabolism of ifosfamide by the kidney. Chirality 2006: 18: pp 398-405. Avner M, Henning P, Koren G, Nulman I: Validation of the facial photographic method in fetal alcohol spectrum disorder screening and diagnosis. Journal of Fetal Alcohol Syndrome International 2006: 4: p e20. Cohen-Kerem R, Nulman I, Abramow-Newerly M, Medina D, Mazel R, Brent RL, Koren G: Diagnostic radiation in pregnancy: Perception versus true risks. Journal of Obstetrics and Gynecology 2006: 28: pp 43-48. Cohen-Kerem R, Ratnapalan S, Djulus J, Duan X, Chandra RV, Ito S: The attitude of physicians towards cold remedies for upper respiratory infection in infants and children: A questionnaire survey. Clinical Pediatrics 2006: 45: pp 828-834. Dugoua JJ, Seely D, Perri D, Koren G, Mills E: Safety and efficacy of black cohosh (Cimicifuga racemosa) during pregnancy and lactation. Canadian Journal of Clinical Pharmacology 2006: 13: pp e257-e261. Dugoua JJ, Mills E, Perri D, Koren G: Safety and efficacy of St. John’s wort (hypericum) during pregnancy and lactation. Canadian Journal of Clinical Pharmacology 2006: 13: pp e268-e276. Dugoua JJ, Mills E, Perri D, Koren G: Safety and efficacy of ginkgo (Ginkgo biloba) during pregnancy and lactation. Canadian Journal of Clinical Pharmacology 2006: 13: pp e277-e284. Einarson A, Koren G: Defective alleles may not have contributed to adverse effects. Therapeutic Drug Monitoring 2006: 28: pp 142-143. Einarson A, Koren G: A survey of women’s attitudes concerning healthy lifestyle changes prior to pregnancy. Journal of Fetal Alcohol Syndrome International 2006: 4: p e2. Kozer E, Scolnik D, Jarvis AD, Koren G: The effect of detection approaches on the reported incidence of tenfold errors. Drug Safety 2006: 29: pp 169-174. Garcia-Bournissen F, Shrim A, Koren G: Safety of gadolinium during pregnancy. Canadian Family Physician 2006: 52: pp 309-310. Kraemer J, Klein J, Lubetsky A, Koren G: Perfusion studies of glyburide transfer across the human placenta: Implications for fetal safety. The American Journal of Obstetrics and Gynecology 2006: 195: pp 270-274. Gareri J, Klein J, Koren G: Drugs of abuse testing in meconium. Clinical Chima Acta 2006: 366: pp 101-111. Gedeon C, Behravan J, Koren G, Piquette-Miller M: Transport of glyburide by placental ABC transporters: Implications in fetal drug exposure. Placenta 2006: 27: pp 1096-1102. Houghton LA, Sherwood KL, Pawlosky R, Ito S, O’Connor DL. [6S]-5-Methyltetrahydrofolate is at least as effective as folic acid in preventing a decline in blood folate concentrations during lactation. American Journal of Clinical Nutrition 2006: 83: pp 842-850. Koren G: A new indication for therapeutic drug monitoring in the neonate. Therapeutic Drug Monitoring 2006: 28: p 1. Koren G: Paul Lemoine, French pioneer in FAS research dies. Journal of Fetal Alcohol Syndrome International 2006: 4: p e6. Koren G, Florescu A, Costei AM, Boskovic R, Moretti ME: Nonsteroidal anti-inflammatory drugs during third trimester and the risk of premature closure of the ductus arteriosus: a meta-analysis. Annals of Pharmacotherapy 2006: 40: pp 824-829. Koren G, Gilbert C, Valois M: Metformin use during the first trimester of pregnancy. Is it safe? Canadian Family Physician 2006: 52: pp 171-172. Koren G, Nava-Ocampo AA, Moretti ME, Sussman R, Nulman I: Major malformations with valproic acid. Canadian Family Physician 2006: 52: pp 441-447. Koren G, Soldin O: Therapeutic drug monitoring of antithyroid drugs in pregnancy: The knowledge gaps. Therapeutic Drug Monitoring 2006: 28: pp 12-13. Kosuge K, Chuang AI, Uematsu S, Tan KP, Ohashi K, Ko BC, Ito S. Discovery of Osmo-sensitive transcriptional regulation of human cytochrome P450 3As (CYP3As) by the tonicity-responsive enhancer binding protein (TonEBP/NFAT5). Molecular Pharmacology June 28, 2007 (Epub ahead of print). Kozer E, Berkovitch M, Koren G: Medication errors in children. Pediatric Clinics of North America 2006: 53: pp 1155-1168. Kwok B, Yamauchi A, Rajesan R, Chen L, Dhillon U, Gao W, Xu H, Wang B, Takahashi S, Semple J, Tamai I, Nezu J, Tsuji A, Harper P, Ito S: Carnitine/xenobiotics transporters in the human mammary gland epithelia, MCF12A. American Journal of Physiology: Regulatory, Integrative and Comparative Physiology 2006: 290: pp 793-802. Many A, Koren G: Toxoplasmosis during pregnancy. Canadian Family Physician 2006: 52: pp 29-30. Moretti ME, Verjee Z, Ito S, Koren G: Breastfeeding during maternal use of azathioprine. Annals of Pharmacotherapy 2006: 40: pp 2269-2272. Nash K, Rovet J, Greenbaum R, Fantus E, Nulman I, Koren G: Identifying the behavioural phenotype in Fetal Alcohol Spectrum Disorder: Sensitivity, specificity and screening potential. Archive of Women’s Mental Health 2006: 9: pp 181-186. Parshuram CS, Dupuis LL, To T, Weitzman SS, Koren G, Laupacis A: Occurrence and impact of unanticipated variation in intravenous methotrexate dosing. Annals of Pharmacotherapy 2006: 40: pp 805-811. Perri D, Dugoua JJ, Mills E, Koren G: Safety and efficacy of Echinacea (Echinacea angustafolia, e. purpurea and e. pallida) during pregnancy and lactation. Canadian Journal of Clinical Pharmacology 2006: 13: pp e262-e267. Shrim A, Boskovic R, Maltepe C, Navioz Y, GarciaBournissen F, Koren G: Pregnancy outcome following use of large doses of vitamin B6 in the first trimester. Journal of Obstetrics and Gynaecology 2006: 26: pp 749-751. Shrim A, Garcia-Bournissen F, Koren G: Pharmaceutical agents and pregnancy in urology practice. Urologic Clinics of North America 2007: 34: pp 27-33. Stade B, Ungar WJ, Stevens B, Beyene J, Koren G: The burden of prenatal exposure to alcohol: Measurement of cost. Journal of Fetal Alcohol Syndrome International 2006: 4: p e5. Stempak D, Gammon J, Halton J, Moghrabi A, Koren G, Baruchel S: A pilot pharmacokinetic and antiangiogenic biomarker study of celecoxib and low-dose metronomic vinblastine or cyclophosphamide in pediatric recurrent solid tumors. Journal of Pediatric Hematology/Oncology 2006: 28: pp 720-728. Sung L, Tomlinson GA, Greenberg ML, Koren G, Judd P, Ota S, Feldman BM: Validation of the oral mucositis assessment scale in pediatric cancer. Pediatric Blood Cancer 2007: 49: pp 149-153. BOOKS AND BOOK CHAPTERS Addis A, Koren G: Safety of fluoxetine during the first trimester of pregnancy: A meta-analytical review of epidemiological studies. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Addis A, Moretti EM, Syed AF, Einarson RT, Koren G: Fetal effects of cocaine: An updated metaanalysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Bar-Oz B, Hackman R, Einarson RT, Koren G: Pregnancy outcome after cyclosporine therapy during pregnancy: A meta-analysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Bar-Oz B, Moretti EM, Mareels G, Van Tittelboom T, Koren G: Reporting bias in retrospective ascertainment of drug-induced embryopathy. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Ben David S, Einarson RT, Ben David Y, Nulman I, Pastuszak A, Koren G: The Safety of nitrofurantoin during the first trimester of pregnancy: A metaanalysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Bennett AH, Einarson A, Taddio A, Koren G, Einarson RT: Prevalence of depression during pregnancy: Systematic review. In: The EvidenceBased, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Bentur Y, Koren G: Occupational exposures known to be human reproductive poisons. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part I. McGraw Hill, New York, 2006. Bentur Y, Koren G: The common occupational exposures encountered by pregnant women. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part I. McGraw Hill, New York, 2006. Bonari L, Pinto N, Ahn E, Einarson A, Steiner M, Koren G: Perinatal risks of untreated depression during pregnancy. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Burtin P, Taddio A, Ariburnu O, Einarson RT, Koren G: Safety of metronidazole in pregnancy: A metaanalysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Gilbert C, Mazzotta P, Loebstein R, Koren G: Fetal Safety of drugs used in the treatment of allergic rhinitis: A critical review. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Cohen-Kerem R, Railton C, Oren D, Lishner M, Koren G: Pregnancy outcome following nonobstetric surgical intervention. In: The EvidenceBased, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Gilbert C, Valois M, Koren G: Pregnancy outcome after first-trimester exposure to metformin: A meta-analysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Diav-Citrin Or, Koren G: Direct drug toxicity to the fetus. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part I. McGraw Hill, New York, 2006. Goh YI, Bollano E, Einarson RT, Koren G: Prenatal multivitamin supplementation and rates of congenital anomalies: A meta-analysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Dolovich RL, Addis A, Vaillancourt JMR, Power JDB, Koren G, Einarson, RT: Benzodiazepine use in pregnancy and major malformations or oral cleft: meta-analysis of cohort and casecontrol studies. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Goh YI, Koren G: Folic acid and congenital birth defects: A review. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part I. In: The EvidenceBased, A To Z Clinician’s Pocket Guide, Part I. McGraw Hill, New York, 2006. Einarson RT, Leeder JS, Koren G: A method for meta-analysis of epidemiological studies. In: The Evidence-Based, A To Z Clinician’s Pocket Guide de. Part II. McGraw Hill, New York, 2006. Gutzin JS, Kozer E, Magee AL, Feig SD, Koren G: The safety of oral hypoglycemic agents in the first trimester of pregnancy: A meta-analysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Einarson RT, Koren G, Mattice D, Schechter-Tsafriri C: Maternal spermicide use and adverse reproductive outcome: A meta-analysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Hard LM, Einarson RT, Koren G: The role of acetaldehyde in pregnancy outcome after prenatal alcohol exposure. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Fernandes O, Sabharwal M, Smiley T, Pastuszak A, Koren G, Einarson T: Moderate to heavy caffeine consumption during pregnancy and relationship to spontaneous abortion and abnormal fetal growth: A meta-analysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, Canada, 2007. Hemels EHM, Einarson A, Koren G, Lanctot LK, Einarson RT: Antidepressant use during pregnancy and the rates of spontaneous abortions: A metaanalysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Fried S, Kozer E, Nulman I, Einarson RT, Koren G: Malformation Rates in children of women with untreated epilepsy: A meta-analysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Gareri J, Klein J, Koren G: Determination of prenatal exposures: In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part I. McGraw Hill, New York, 2006. Gedeon C, Koren G: Gestational changes in drug disposition in the maternal-fetal unit. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part I. McGraw Hill, New York, 2006. Koren G: Medication in pregnancy and breastfeeding. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part I. McGraw Hill, New York, 2006. Koren G: Risk of varicella infection during late pregnancy. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Koren G: Drugs in pregnancy: Acknowledging challenges-finding solutions. In: The EvidenceBased, A To Z Clinician’s Pocket Guide, Part I. McGraw Hill, New York, 2006. Koren G: Bias against the null hypothesis in maternal-fetal pharmacology and toxicology. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 27 Koren G, Nava-Ocampo A A, Moretti EM, Sussman R, Nulman I: Major malformations with valproic acid. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II, McGraw Hill, New York, 2006. Kozer E, Nikfar S, Costei A, Boskovic R, Nulman I, Koren G. Aspirin consumption during the first trimester of pregnancy and congenital anomalies: A meta-analysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Magee AL, Elran E, Bull BS, Logan A, Koren G. Risks and benefits of beta-receptor blockers for pregnancy hypertension: Overview of the randomized trials. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Magee AL, Mazzotta P, Koren G. Evidence-based view of safety and effectiveness of pharmacologic therapy for nausea and vomiting of pregnancy (NVP). In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Makarechian N, Agro K, Devline J, Trepanier E, Koren G, Einarson RT. Association between moderate alcohol consumption during pregnancy and spontaneous abortion, stillbirth and premature birth: A meta-analysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, McGraw Hill, New York, 2006. McMartin IK, Chu M, Kopecky E, Einarson RT, Koren G. Pregnancy outcome following maternal organic solvent exposure: A meta-analysis of epidemiologic studies. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Moretti EM, Bar-Oz B, Fried S, Koren G. Maternal hyperthermia and the risk for neural tube defects in offspring: systematic review and meta-analysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Nava-Ocampo AA, Talaie H, Koren G: Systematic Review of randomized controlled trials and cohort studies on pregnancy outcome following antiretroviral treatment in pregnancy. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Nikfar S, Abdollahi M, Moretti EM, Magee AL, Koren G: Use of proton pump inhibitors during pregnancy and rates of major malformations: A meta-analysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. 28 DEPARTMENT OF PAEDIATRICS Nulman I, Izmaylov Y, Staroselsky A, Koren G: Teratogenic drugs and chemicals in humans. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part I. McGraw Hill, New York, 2006. Nulman I, Knittel-Kerem D, Koren G: Fetal Alcohol Spectrum Disorder: The central nervous system tragedy. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part I. McGraw Hill, New York, 2006. Nulman I, Koren G: Epilepsy and pregnancy. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part I. McGraw Hill, New York, 2006. Nulman I, Laslo D, Fried S, Uleryk E, Lishner M, Koren G: Neurodevelopment of children exposed in utero to treatment of maternal malignancy. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Nulman I, Sussman R, Farber N, Chitayat D, Koren G: Fetal malformations associated with drugs and chemicals: Visualization by sonography/fetal MRI. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part I. McGraw Hill, New York, 2006. Park-Wyllie L, Mazotta P, Pastuszak, A, Moretti EM, Beique L, Hunnisett L, Friesen HM, Jacobson S, Kasapinovic S, Chang D, Diav-Citrin O, Chitayat D, Nulman I, Einarson RT, Koren G: Birth defects after maternal exposure to corticosteroids: Prospective cohort study and meta-analysis of epidemiological studies. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Polygenis D, Wharton S, Malmberg C, Sherman N, Kennedy D, Koren G, Einarson RT: Moderate alcohol consumption during pregnancy and the incidence of fetal malformations: A meta-analysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Raman-Wilms L, Tseng LAl, Wighardt S, Einarson RT, Koren G: Fetal genital effects of first-trimester sex hormone exposure: a meta-analysis. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, Canada, 2006. Seto A, Einarson RT, Koren G: Pregnancy outcome following first trimester exposure to antihistamines: A meta-analysis. In: The EvidenceBased, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Seto A, Einarson RT, Koren G: Evaluation of brompheniramine safety in pregnancy. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. Sivojelezova A, Gallo M, Bollano E, Boom H, Koren G: The use of natural health products in pregnancy and lactation. In: The Evidence-Based, A To Z Clinician’s Pocket Guide, Part I. McGraw Hill, New York, 2006. ANNUAL REPORT 2006-07 von Dadelszen P, Ornstein, MP, Bull, SB, Logan AG, Koren, G, Magee AL: Fall in mean arterial pressure and fetal growth restriction in pregnancy hypertension: A meta-analysis. In: The EvidenceBased, A To Z Clinician’s Pocket Guide, Part II. McGraw Hill, New York, 2006. FUNDING Child neurodevelopment and pharmacokinetic correlations following treatment with supratherapeutic doses of Diclectin for nausea and vomiting of pregnancy. Nulman I. Canadian Institutes of Health Research ($487,600 and $68,267 2006) Child neurodevelopment following in utero exposure to maternal azathioprine. Nulman I. Physicians’ Services Incorporated Foundation ($146,000 2006) Determinants of renal handling of drugs during development. Koren G, Ito S. Canadian Institutes of Health Research ($160,000 2003-2008) Determinants of renal handling of drugs during development. Koren G. Canadian Institutes of Health Research ($583,800 2003-2008) Drugs and human milk. Ito S. Canadian Institutes of Health Research ($645,998 2002-2007) Genotype-specific approaches in therapeutics in children. Carlton B, Hayden M, Ito S. UBC ($105,000 2005-2007) Hair analysis for cortisol and testosterone. VanUm S, Koren G. Physicians’ Services Incorporated Foundation ($130,000 2006-2008) Improving prenatal vitamins to women. Koren G. Duchesnay Incorporated ($400,000 2004-2007) New Emerging Team (NET) grant. Koren G, Brien J. Canadian Institutes for Health Research ($1,250,000 2002-2007) Neurodevelopment after fetal exposure to venlafaxine. Nulman I, Koren G. Wyeth Incorporated ($120,000 2004-2006) Neurodevelopment after fetal exposure to supradose diclectin. Koren G, Nulman I. Duchesnay Incorporated ($480,000 2005-2008) Novel transcriptional control of human CYP3A. Ito S. Canadian Institutes of Health Research ($340,540 2006-2009) Pharmacogenetics of adverse drug reactions in children. Carlton B, Hayden M, Ito S, Koren G. Genome Canada ($350,000 2005-2008) The role of placenta in fetal toxicology. Koren G, Derewlany L, Einarson T. Canadian Institutes of Health Research ($584,309 2002-2006) Developmental Paediatrics DIVISIONAL OVERVIEW The Division of Developmental Paediatrics was created within the Faculty of Medicine’s Department of Paediatrics effective March 1, 2005 in response to recent major advances in this important area of paediatrics. The Royal College of Physicians and Surgeons of Canada has recognized that developmental paediatrics represents a defined body of knowledge meriting recognition as a separate subspecialty area within paediatrics. The training program at the University of Toronto was granted “full accredited with certification” status by the College. SickKids and Bloorview Kids Rehab (formerly Bloorview MacMillan Children’s Centre) have created an enhanced child development program which provides a single access point and triage system for families and consolidates resources. As well, leaders at SickKids and Bloorview Kids Rehab research institutes are working closely together to augment research activities related to developmental disorders. HONOURS AND AWARDS Jethwa A: The Roszel Developmental Paediatric Research Award, 2007 PUBLICATIONS Biggar WD: Duchenne muscular dystrophy. Pediatrics in Review 2006: 27: pp 83-87. Biggar WD, Harris VA, Eliasoph L, Alman B: Longterm benefits of delfazacort treatment for boys with Duchenne muscular dystrophy in their second decade. Neuromuscular Disorders 2006: 16: pp 249-255. Bryson SE, Zwaigenbaum L, Brian JA, Roberts SW, Szatmari P, Rombough V, McDermott C: A prospective case series of high-risk infants who developed autism. Journal of Autism and Developmental Disorders 2007: 37: pp 12-24. Doja A, Roberts SW: Immunizations and autism. Canadian Journal of Neurological Sciences 2006: 33: pp 341-346. Dorota A, Kwasnicka-Crawford, Roberts W, Scherer SW: Characterization of an autism-associated segmental maternal heterodisomy of the chromosome 15q11-13 region. Journal of Autism Developmental Disorders 2006: 37: pp 694-702. Division Head Dr. Golda Milo-Manson (2006-2007) Dr. Darcy Fehlings (2007-present) Physicians Dr. Douglas Biggar Dr. Darcy Fehlings Dr. Cynthia Goldfarb Dr. Elizabeth Jimenez Dr. Tamarah Kagan-Kushnir Dosman C, Brian J, Drmic I, Senthilselvan A, Harford M, Smith R, Sharieff W, Moldofsky H, Zlotkin S, Roberts W: Children with autism: Effect of iron supplementation on sleep and ferritin. Pediatric Neurology 2007: 36: pp 152-158. Dosman C, Drmic I, Brian J, Senthilselvan A, Harford M, Smith R, Roberts W: Ferritin as an indicator of suspected iron deficiency in children with autism spectrum disorder: Prevalance of low serum ferritin concentration. Developmental Medicine and Child Neurology 2006: 48: pp 1008-1009. Feuk L, Kalervo A, Lipsanen-Nyman M, Skaug J, Kazuhiko N, Finucane B, Hartung D, Innes M, Kerem B, Nowaczyk MJ, Rivlin J, Roberts W, Senman L, Summers A, Szatmari P, Wong V, Vincent JB, Zeesman S, Osborne LR, Oram Cardy J, Kere J, Scherer SW, Hannula-Jouppi K: Absence of a paternally-inherited FOXP2 gene in developmental verbal dyspraxia. American Journal of Human Genetics 2006: 79: pp 965-972. Georgiades S, Szatmari P, Zwaigenbaum L, Duku E, Bryson S, Roberts W, Goldberg J, Mahoney W: The structure of the autism symptom phenotype: A proposed multidimensional model. Journal of the American Academy of Child and Adolescent Psychiatry 2007: 46: pp 188-196. Dr. Anne Kawamura Dr. Tara Kennedy Dr. Peggy Kirkpatrick Dr. Alvin Loh Dr. Golda Milo-Manson Dr. Wendy Roberts Dr. Jay Rosenfield Fellows Dr. Christakis Christofi Dr. Anita Jethwa Dr. Kassia Johnson Dr. Angelina Orsino Dr. Jonathan Ponesse Dr. Lyn Sonnenberg Ickowicz A, Feng Y, Wigg K, Quist J, Pathare T, Roberts W, Malone M, Schachar R, Tannock R, Kennedy JL, Barr CL: The serotonin receptor HTR1B: Gene polymorphisms in attention deficit hyperactivity disorder. Neuropsychiatric Genetics 2007: 144: pp 121-125. Kawamura A, Campbell K, Lam-Damji S., Fehlings D: A randomized controlled trial comparing botulinum toxin A dosage in the upper extremity of children with spasticity. Developmental Medicine and Child Neurology 2007: 49: pp 331-337. Loh A, Soman T, Brian J, Bryson SE, Roberts W, Szatmari P, Smith I, Zwaigenbaum L: Stereotyped motor behaviors associated with autism in highrisk infants: A pilot videotape analysis of a sibling sample. Journal of Autism and Developmental Disabilities 2007: 37: pp 25-36. Mitchell S, Brian J, Zwaigenbaum L, Roberts W, Szatmari P, Smith I, Bryson S: Early language and communication development of infants later diagnosed with autism spectrum disorder. Journal of Developmental and Behavioral Pediatrics 2006: 27: pp S69-S78. Administrative Staff at SickKids Maureen Hodge Barbara Zimnowodzki Administrative Staff at Bloorview Kids Rehab Romana Karnetova Narayanan U, Fehlings D, Weir S, Knights S, Kiran S, Campbelll K: Initial development and validation of the caregiver priorities and Child Health Index of Life with Disabilities (CPCHILD). Developmental Medicine and Child Neurology 2006: 48: pp 804-812. Rubinstein S, Malone MA, Roberts W, Logan WJ: Placebo-controlled study examining effects of selegiline in children with attention deficit hyperactivity disorder. Journal of Child & Adolescent Psychopharmacology 2006: 16(4): pp 404-415. Sanger T, Chen D, Delgado M, Gaebler-Spira D, Hallett M, Mink J, and the Taskforce on Childhood Motor Disorders (Fehlings D, Member): Definition and classification of negative motor signs in childhood. Pediatrics 2006: 118: pp 2159-2167. Szatmari P, Georgiades S, Bryson S, Zwaigenbaum L, Roberts W, Mahoney W, Goldberg J, Tuff L: Investigating the structure of the restricted, repetitive behaviours and interests domain of autism. Journal of Child Psychology & Psychiatry 2006: 47: pp 582-590. Scherer SW, Szatmari P, Roberts W (The Autism Genome Project Consortium): Mapping autism risk loci using genetic linkage and chromosomal rearrangements. Nature Genetics 2007: 39: pp 319-328. Scherer SW, Vincent JB, Horike S, Choufani S, Paterson AD, Roberts W, Szatmari P, Weksberg R, Fernandez BA: An inversion in autistic siblings implicates the 4p GABA receptor gene cluster. Journal of Medical Genetics 2006: 43: pp 429-434. Vajsar J, Zhang W, Dobyns WB, Biggar WD, Holden KR, Hawkins C, Ray P, Olney AH, Burson CM, Srivastava AK, Schachter H: Carriers and patients with muscle-eye-brain disease can be rapidly diagnosed by enzymatic analysis of fibroblasts and lymphoblasts. Neuromuscular Disorders 2006: 16: pp 132-136. Young N, Gilbert T, McCormick A, Ayling-Campos A, Law M, Fehlings D, Mukherjee S, Wedge J, Rumney P, Williams JI: Youth and young adults with cerebral palsy: Their use of physician and hospital services. Archives of Physical Medicine and Rehabilitation 2007: 88: pp 696-702. Young N, McCormick A, Mills W, Barden W, Boydell K, Wedge J, Fehlings D, Mukherjee S, Rumney P, Williams J: The transition study: A look at youth and adults with cerebral palsy, spina bifida and acquired brain injury. Physical and Occupational Therapy in Pediatrics 2006: 26: pp 25-45. 30 DEPARTMENT OF PAEDIATRICS BOOKS AND BOOK CHAPTERS Fehlings D, Hunt C, Rosenbaum P: Cerebral palsy. In: A Comprehensive Guide to Intellectual & Developmental Disabilities (Brown I, Percy M, eds). Paul H. Brookes Publishing Inc., Baltimore, 2007. Weiss S, Milo-Manson G, MacFarlane J. Better Sleep for Your Baby and Child. Robert Rose Publishers, Toronto, 2006. FUNDING A joint Canadian and Hong Kong, China initiative in the study of autism spectrum disorders. Scherer SW, Szatmari P, Roberts W. Canadian Institutes of Health Research ($24,720 2005-2006) Autism genome project. Scherer SW, Szatmari P, Paterson AD, Vincent JB, Zwaigenbaum L, Roberts W, Wellington SR, Fernandez BA, Miller FA, Hallmayer JF. Genome Canada ($15,611,534 2005-2009) Autism spectrum disorders: Pathways to better outcomes. Szatmari P, Bryson SE, Fombonne E, Zwaigenbaum L, Smith I, Waddell C, Vaillancourt T, Mirenda P, Roberts W, Volden J. Canadian Institutes of Health Research ($2,125,415 2004-2009) Development and reliability testing of a new observational gait assessment for children with cerebral palsy. Wright V, Fehlings D. Bloorview Research Institute Seed Grant ($24,952 2006-2007) Early developmental trajectories in autism. Zwaigenbaum L, Bryson SE, Roberts SW, Smith I, Szatmari, Vaillancourt T. Canadian Institutes of Health Research ($932,585 2006-2011) Enhancing inter-subjectivity in infants at high-risk for autism. Bryson S, Brian J, Smith I, Zwaigenbaum L, Roberts SW. Autism Speaks ($638,006 2007-2010) Evaluating outcomes associated with multidisciplinary care for children with cerebral palsy using clinical practice improvement (CPI) methodology. Damiano D, Chambers H, Revivo G, Brunstrom J, Horn S, Gassaway J, Krach L, Gormley M, Novacheck T, Matthews D, Chang F, Stevenson R, Abel M, McLaughlin J, Bjornson K, Rathjen K, Delgado M, Dabrowski E, Gaebler-Spira D, Dias L, Narayanan U, Fehlings D, Graham K, Koman A. American Academy for Cerebral Palsy and Developmental Medicine Clinical Research Planning Grant ($29,000 2006-2007) Evaluation of the reliability, validity and responsiveness of the Kinematic Dystonia Measure in children with upper extremity dystonia. Kawamura A, Fehlings D, Morris A, Campbell K. Bloorview Research Institute Seed Grant ($16,652 2007) ANNUAL REPORT 2006-07 Evaluation with a message: Interprofessional assessment of professionalism. Milo-Manson G, Kennedy T. Royal College of Physicians & Surgeons of Canada Medical Education Grant ($11,125 2006) Functional outcomes following orthopaedic surgery based on gait laboratory versus observational gait analysis in ambulatory children with cerebral palsy: A randomized controlled trial. Narayanan U, Fehlings D, Weir S. Pediatric Orthopaedic Society of North America (POSNA) ($31,000 2006-2008) Functional outcomes following orthopaedic surgery based on gait laboratory versus observational gait analysis in ambulatory children with cerebral palsy: A pilot randomized controlled trial. Narayanan U, Fehlings D, Weir S. Bloorview Research Institute Seed Grant Competition ($25,015 2006-2007) Genetic epidemiology of autism: Family and molecular studies. Szatmari P, Bryson S, Goldberg J, Patterson A, Mahoney W, Merette C, Roberts W, Zwaigenbaum L, Scherer S. Canadian Institutes of Health Research ($725,000 2003-2008) Identifying early markers of autism: A prospective study of infant siblings. Zwaigenbaum L, Roberts SW, Smith I, Szatmari P, Bryson SE. Canadian Institutes of Health Research ($331,650 20032006) Interprofessional Disaster/ Emergency Action Studies (‘IDEAS’): Promoting interprofessional collaboration and team building to deliver safe and effective casualty and patient-centred practice in disasters/emergencies and pandemics. Rosenfield J (Member of Steering Committee). Health Canada Interprofessional Education for Collaborative Patient-Centred Practice (IECPCP) ($792,011 2006-2007) Neuroimaging and frontal lobe function in children with autism. Taylor M, Roberts SW, Donner EJ, Bayless S, Shroff M. National Alliance for Autism Research ($115,907 2006-2008) The development and evaluation of the HAT-D: A discriminative tool differentiating types of hypertonia in children. Fehlings D, Jethwa A, Macarthur C. The Bloorview Research Institute ($9,606 2006-2007) The efficacy of an intelligent prompting system to develop self-care activities in children with autism. Mihailidis A, Roberts SW, Polatajko H, Chau T. SickKids Foundation and Canadian Institutes of Health Research ($129,470 2007-2009) Training program in autism research. Fombonne E, Bryson S, Paterson A, Scherer S, Roberts W, Szatmari P, Smith I, Moore C. Canadian Institutes of Health Research, National Alliance for Autism Research and Fonds de la Recherche en Sante du Quebec ($1,440,000 2003-2009) Dr. Darcy Fehlings Murphy AM, Fehlings D, Milo-Manson G, Best A, Campbell K: An evaluation of modafanil on spasticity reduction, function and quality of life in children with CP. Developmental Medicine & Child Neurology 2006: 48: p 12. Acting Division Head Dr. Diane Wherrett Full-Time Dr. Denis Daneman Dr. Jill Hamilton Dr. Kusiel Perlman Dr. Etienne Sochett Part-Time Dr. Jacqueline Curtis Dr. Susan Kirsch Dr. Stacey Urbach Gynaecology Dr. Lisa Allen Dr. Nicolette Caccia Dr. Sari Kives Dr. Melanie Ornstein Honorary Dr. Robert Ehrlich Dr. Dror Koltin Dr. Clodagh O’Gorman Gynaecology Fellows Dr. Rachel Spitzer Subspecialty Residents/ Clinical Fellows Dr. Abdulla Aljunaibi Dr. Bojana Babic Dr. Louise Conwell Dr. Ara Healey Research Fellows Dr. Shazhan Amed Dr. David Cherney Dr. Meranda Nakhla Cross-Appointees Dr. Amira Klip Dr. Joanne Rovet Administrative Staff Mira Milne Joyce Robinson Minet Smith Zarine Tilak Endocrinology DIVISIONAL OVERVIEW 2006-2007 continued to be a highly productive time for the Division of Endocrinology with ongoing development of existing programs and new innovations. We have had eight trainees in our subspecialty training program: four Canadian, one Israeli, one Australian, one Irish and one from the UAE. It has also been a year of changes, as Dr. Denis Daneman is now Chair of Paediatrics and Paediatrician in Chief at The Hospital for Sick Children. Dr. Diane Wherrett has been acting division head for the past year. Dr. Mark Palmert will be joining the division as the new head in September 2007. Our research efforts have targeted the following areas: (i) prediction and prevention of type 1 diabetes: our group is involved in the multinational TrialNet and TRIGR study groups; (ii) implications of insulin resistance and obesity in children and adolescents and the evolution of them in young children; (iii) study of the factors affecting metabolic control and the early complications of diabetes; (iv) psychosocial and psychological outcomes in diabetes and other endocrine disorders; and (v) evaluation of disorders of calcium and bone mineral metabolism in paediatrics. Dr. Diane Wherrett with clinic staff Our areas of clinical focus continue to be: (i) Diabetes: our comprehensive program provides ongoing care to nearly 1,000 children and teens with diabetes, including a program for comprehensive assessment and treatment for adolescents with chronically poor diabetes control; (ii) General Endocrinology emphasizing the areas of growth, thyroid disease, intersex conditions and reproductive health; (iii) Calcium: the Calcium-Bone and Osteoporosis Clinics assess children with abnormal bone health from a wide variety of causes; (iv) Gynaecology section: provides care to pre-teen and adolescent girls across a wide spectrum of disorders. Recent initiatives include the development of pathways for the care of children with thyroid cancer in collaboration with Oncology, Otolaryngology and Nuclear Medicine. DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 33 HONOURS AND AWARDS Aggarwal L, Thomas M, Spitzer R, Stephens D, Kives S, Allen L: Methotrexate in the management of adolescents with ectopic pregnancies: A physician survey. SOGC Poster Gynaecology Junior Members Award, 2007 Hamilton J: NovoNordisk Young Investigator Career Development Award, 2006-2007. Hamilton J: The Hospital for Sick Children, Department of Paediatrics Richard Rowe Junior Faculty Award for Excellence in Clinical Medicine – Runner Up, 2006. Klip A: Editor-in-Chief, The American Journal of Physiology – Endocrinology and Metabolism Physiology, 2007. Hoey H, McGee HM, Fitzgerald M, Mortensen HB, Hougaard P, Lynggaard H, Skovlund SE, Aanstoot HJ, Chiarelli F, Daneman D, Danne T, Dorchy H, Garandeau P, Greene S, Holl R, Kaprio E, Kocova M, Martul P, Matsuura N, Robertson K, Schoenle E, Sovik O, Swift P, Tsou RM, Vanelli M, Aman J; for the Hvidore Study Group on Childhood Diabetes: Parent and health professional perspectives in the management of adolescents with diabetes: Development of assessment instruments for international studies. Quality of Life Research 2006: 15: pp 1033-1042. Nash K, Rovet J, Greenbaum R, Fantus E, Koren G: Defining the behavioural phenotype in children with Fetal Alcohol Spectrum Disorder (FASD): Sensitivity, specificity and screening potential. Archives of Women’s Mental Health 2006: 9: pp 181-186 Huang C, Ezzat S, Asa S, Hamilton J: Dopaminergic resistant prolactinomas in the peripubertal population. Journal of Pediatric Endocrinology and Metabolism 2006: 19: pp 951-954. Ong G, Hamilton J, Sermer M, Connelly PW, Maguire G, Zinman B, Hanley AJ, Retnakaran R: Maternal serum adiponectin and infant birthweight: The role of adiponectin isoform distribution. Clinical Endocrinology April 27, 2007 [Epub ahead of print]. PUBLICATIONS Al-Jenaidi F, Makitie O, Grubenbaum E, Sochett E: Hypoparathyroidism in 22q11.2 deletion syndrome; follow up and relationship to associated clinical features. Hormone Research 2006: 67: pp 117-122. Ishikura S, Bilan PJ, Klip A: Rabs 8A and 14 are targets of the insulin-regulated Rab-GAP AS160 regulating GLUT4 traffic in muscle cells. Biochemical and Biophysical Research Communications 2007: 353: pp 1074-1079. Becker DV, Braverman LE, Delange F, Franklyn JA, Hollowell JG, Lamm SH, Mitchell ML, Pearce E, Robbins J, Rovet J: Iodine supplementation for pregnancy and lactation – United States and Canada: Recommendations of the American Thyroid Association. Thyroid 2006: 16: pp 949-951. JeBailey L, Wanono O, Niu W, Roessler J, Rudich A, Klip A: Ceramide- and oxidant-induced insulin resistance involve loss of insulin-dependent Rac-Activation and Actin remodeling in muscle cells. Diabetes 2007: 56: pp 394-403. Caprara D, Nash K, Greenbaum R, Rovet J, Koren G: Novel approaches to the diagnosis of Fetal Alcohol Spectrum Disorder. Neuroscience & Biobehavioral Reviews 2007: 31: pp 254-260. Dunger D, Schwarze CP, Cooper JD, Widmer B, Neil HA, Shield J, Edge JA, Jones TW, Daneman D, Dalton RN: Can we identify adolescents at high risk for nephropathy before the development of microalbuminuria? Diabetic Medicine 2007: 24: pp 131-136. Greenberg ML, Urbach SL: Preserving the fertility of children with cancer. Medical Journal of Australia 2006: 185: pp 532-533. Hamilton J, Skrivarhaug T: ISPAD 2006 – Highlights from the 32nd Annual Scientific Meeting, Cambridge, UK. Paediatric Diabetes 2006: 7: pp 336-340. Kives S: The IUCD – Is it an acceptable option for the adolescent? Journal of Pediatric and Adolescent Gynecology 2006: 19: p 301. Klip A, Vranic M: Muscle, liver, and pancreas: Three Musketeers fighting to control glycemia. American Journal of Physiology – Endocrinology and Metabolism 2006: 291: pp e1141-e1143. Lacy J, Brennand E, Ornstein M, Allen L: Vaginal laceration from a high-pressure water jet in a prepubescent girl. Pediatric Emergency Care 2007: 23: pp 112-114. Lacy J, Capra M, Allen L: Endodermal sinus tumor of the infant vagina treated exclusively with chemotherapy. Journal of Pediatric Hematology/Oncology 2006: 28: pp 768-771. Lam C, Tjon J, Hamilton J, Ahmet A: Recurrent anaphylaxis associated with gonadotropin releasing hormone analogues: Case report and literature review. Pharmacotherapy 2006: 26: pp 1811-1815. Milic A, Asch MR, Hawrlyshyn PA, Allen LM, Colgan TJ, Kachura JR, Hayeems EB: Laparoscopic ultrasound guided radiofrequency ablation of uterine fibroids. Cardiovascular & Interventional Radiology 2006: 29: pp 694-698. Nathan PC, Jovcevska V, D’Agostino N, Staneland P, Urbach SL, Barron M, Barrera M, Greenberg ML: The prevalence of overweight and obesity in pediatric survivors of childhood cancer. Journal of Pediatrics 2006: 149: pp 518-525. Rachmiel M, Charron M, Gupta A, Hamilton J, Wherrett D, Forte V, Daneman D: Evidence-based review of treatment and follow-up of pediatric patients with differentiated thyroid cancer. Journal of Pediatric Endocrinology and Metabolism 2006: 19: pp 1377-1394. Rachmiel M, Rota V, Atenafu E, Daneman D, Hamilton J: Final height in children with idiopathic growth hormone deficiency treated with a fixed dose of recombinant growth hormone. Hormone Research 2007: 68: pp 236-243. Thong F, Bilan PJ, Klip A: The Rab GTPaseactivating protein AS160 integrates Akt, protein kinase C, and AMP-activated protein kinase signals regulating GLUT4 traffic. Diabetes 2007: 56: pp 414-443. Wolfsdorf J, Craig ME, Daneman D, Dunger D, Edge J, Warren LW, Rosenbloom A, Sperling MA, Hanas R: Diabetic ketoacidosis. Pediatric Diabetes 2007: 8: pp 28-43. Zdravkovic V, Hamilton J, Daneman D, Cummings E: Pioglitazone as adjunctive therapy in adolescents with Type 1 diabetes. Journal of Pediatrics 2006: 149: pp 845-849. BOOKS AND BOOK CHAPTERS Perlman K. Management of Diabetes Mellitus in Children and Adolescents in Approach to the Management of Diabetes Mellitus. Banting and Best Diabetes Centre, Toronto, 2006. Till C, Koren G, Rovet J. Effects of organic solvents on reproductive outcome and offspring neurodevelopment. In: Human Developmental Neurotoxicology (Bellinger DC, ed). Taylor & Francis, New York, 2006. FUNDING Bridging the paediatric and adult health care environment: Promoting successful transitions for survivors of paediatric cancer. Greenberg M, D’Agostino N, Guger S, Staneland P, Lough M, Laperriere N, Urbach SL, Nathan PC. Princess Margaret Hospital Psychosocial Oncology and Palliative Care Grant Competition ($5,986 2006-2007) Bypassing insulin resistance of muscle cells caused by ceramide, oxidative stress and macrophage-co-culture. Klip A. Canadian Diabetes Association ($300,000 2006-2009) Combined diazoxide and metformin therapy in children with hypothalamic obesity secondary to craniopharyngioma: A Pilot Study. Hamilton J, Daneman D, Ahmet A. Canadian Institutes of Health Research ($49,500 2006-2008) Comparison of continuous versus cyclic use of a 30-MCG ethinyl estradiol 0.15 mg desogestrel oral contraceptive pill in adolescents. Allen L, Brown C. Azko Nobel – Organon Canada ($24,500 2006-2008) Comparison of continuous versus cyclic use of a 30-MCG ethinyl estradiol 0.15 mg desogestrel oral contraceptive pill in adolescents. Allen L, Brown C. Physicians’ Services Incorporated Foundation ($75,000 2006-2008) Cross-sectional study of overweight in paediatric survivors of acute lymphoblastic leukemia. Nathan PC, Urbach SL, Barrera M, Barron M, D’Agostino N, Staneland P, Charron, Greenberg ML. The University of Toronto, Faculty of Medicine Dean’s Fund ($9,998 2007) Evaluation of hepatic function and autoimmunity in girls with Turner Syndrome. Hamilton J, McAssey K, Milkiewicz P, Heathcote J, Norman G. Hoffman-La Roche Canada ($23,980 2006-2007) Fetal alcohol syndrome: Oxidative stress and innovative therapies: (Aiii) Structural manifestations of FAS/ARND brain injury. Rovet J. Canadian Institutes of Health Research New Emerging Team Grant ($50,000 2002-2007) Growth hormone therapy and bone quality in paediatric osteoporosis. Sochett E, Grynpas M. Eli Lilly Canada ($125,000 2006-2009) Hemodynamic modulation in type 1 diabetes. Miller J, Sochett E, Cherney D. Canadian Institutes of Health Research ($105,000 2007-2012) Post marketing surveillance program for Protropin and Nutropin. Hamilton J. Primary Investigator SickKids site. Genentech National Cooperative Growth Study (NCGS) ($35,300 2001-2007) The comprehensive care clinic for children with central tumours at The Hospital for Sick Children: Evaluating the efficacy of an integrated, multidisciplinary model of care for children with hypothalamic dysfunction. Hamilton J, Barrera M, Guger S, Popov T, Schulte F. b.r.a.i.n.child, The Hospital for Sick Children ($27,931.36 2006-2008) The genetics and neuroendocrinology of short stature. Hamilton J. Primary Investigator SickKids site. Lilly International Growth Study (GeNeSIS) ($58,542 2002-2007) Hormonal regulation of p38MAPK and its implications for glucose transport. Klip A. Canadian Institutes of Health Research ($643,800 2002-2007) Insulin resistance and beta cell dysfunction in early childhood: The role of maternal and infant metabolic risk factors. Hamilton J, Hanley A, Retnakaran R, Zinman B. Canadian Institutes of Health Research ($341,706 2005-2008) Insulin sensitization by muscle contraction pathways: implications for insulin resistance. Klip A. Canadian Institutes of Health Research/ National Natural Science Foundation of China – Canada Joint Health Research Initiative – Grants Program ($90,000 2006-2009) Intracellular compartment plasticity and signal transmission in insulin action. Klip A. Canadian Institutes of Health Research ($759,560 2005-2010) The GH Monitor registration. Hamilton J, Daneman D, Geary D, Perlman K, Hebert D, Sochett E, Langlois V, Wherrett D, Perl R. Serono Canada Incorporated ($1,200 2005-2008) Thyroid hormone insufficiencies and visual perceptual deficits in preterm infants. Rovet J, Westall C, Asztalos E, Perlman K, Heon E. March of Dimes (US$253,083 2004-2007) TrialNet Clinical Center (Study group for prevention of type 1 diabetes). Wherrett DK, Mahon J, Daneman D. National Institutes of Health (US$1,187,072 2001-2008) Type 1 Diabetes Genetics Consortium. Wherrett DK. Benaroya Research Institute (US$15,000 2004-ongoing) MRI analyses of visuospatial processing deficits in children born preterm or with congenital hypothyroidism. Rovet J, McAndrews MP, Desrocher M, Sheard E, Asztalos E. March of Dimes (US$339,945 2007-2008) Multiple courses of antenatal corticosteroids for preterm birth study: A five-year follow-up (MACS-5). Asztalos E, Rovet J. Canadian Institutes of Health Research ($3,585,161 2005-2012) National surveillance of non-type 1 diabetes mellitus in Canadian children. Hamilton J, Dean H, Amed S, Sellers E, Dannenbaum D, Laubscher T, Booth G. Canadian Diabetes Association ($97,939 2006-2007) Neuroimaging studies of hippocampal formation in children with pre- and postnatal thyroid hormone insufficiencies. Rovet J, Desrocher M, McAndrews MP, Sheard E. Canadian Institutes of Health Research ($728,640 2006-2011) DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 35 Gastroenterology, Hepatology and Nutrition DIVISIONAL OVERVIEW The Division of Gastroenterology, Hepatology and Nutrition is recognized nationally and internationally for its contributions to clinical care, research and education in the field of gastroenterology, including hepatology, nutrition and transplantation. It is one of the only programs in North America offering a full spectrum of paediatric ‘GI-coverage’ including clinical nutrition, gastroenterology, hepatology and transplantation. The division has a fully accredited three-year Royal College of Physicians and Surgeons of Canada Fellowship training program with three to four first-year fellows. Over the past year, four of our current research fellows (out of a total of nine) are combining a formal degree-granting program (MSc or PhD) with their research fellowship years. With the retirement this year of Dr. Eve Roberts, the division hosts nine full-time staff, four part-time and two cross-appointed members. The main clinical programs in the division include inflammatory bowel disease, cystic fibrosis, luminal GI disorders, nutritional disorders, liver diseases and liver and small bowel transplantation. Our research spans the spectrum from basic and translational to patient-based in such areas as inflammatory bowel disease, pancreatic diseases, ulcer disease, liver diseases, GI microbiology and immunology, nutritional metabolism and nutrient requirements. This year, once again, fellows in the division have been honoured with multiple awards, and fellowships. The staff members of the division have published over 90 peer-reviewed publications, 25 books or book chapters and have received well over $5 million of grant funding for projects spanning the next five years. HONOURS AND AWARDS Zlotkin S: International Ashoka Fellowship Award, 2007. Zlotkin S: Canadian Institutes of Health Research National Knowledge Translation Award, 2006. Zlotkin S: Member of the Order of Canada, 2007. Benchimol E: Travel Award from the First International Gastrointestinal Eosinophil Research Symposium. Gana J: Presentation Award at the Digestive Diseases Week of the American Gastroenterology Association, 2007. Guttman O: CASL/Schering Canada Fellowship Award, 2007. 36 DEPARTMENT OF PAEDIATRICS Muise A: Canadian Society for Clinical Investigators (CSCI)/Canadian Institutes for Health Research Award for Excellence in Resident Research, 2007. Muise A: Starr Medal for Outstanding Scholarship, Post-Graduate Medical Education, Faculty of Medicine, University of Toronto, 2006. Muise A: Paediatric Research Award, Senior Trainee – Basic Science, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, 2007. Muise A: Elizabeth Arbuthnot Dyson Fellowship: Awarded upon Recommendation of the Chair of Paediatrics The Hospital for Sick Children and Joseph M. West Family Memorial Fund, Faculty of Medicine, University of Toronto, 2006. Muise A: Posdoctoral Fellow, Canadian Child Health Clinician Scientist Program, Canadian Institute Health Research – Strategic Training Initiatives in Health Research Program Award, 2006. ANNUAL REPORT 2006-07 Turner D: American Physician Fellowship Award, 2006. Turner D: Postgraduate Research Award, Joseph M. West Family Memorial Fund and the Chisholm Memorial Fellowship, University of Toronto, 2006. Turner D: Levy/Thompson Award for Outstanding Oral Presentation, Canadian Digestive Disease Week (CDDW)-GRIT, 2007. Turner D: Canadian Institute Health Research/ Canadian Association of Gastroenterology/ Crohn’s Colitis Foundation of Canada (Canadian Institutes of Health Research/CAG/CCFC) Fellowship Award, 2006. Turner D: American Gastroenterology Association (AGA) Fellow Abstract Prize, 2007. Turner D: CanMeds Competencies Award, The Hospital for Sick Children, University of Toronto, 2007. Dr. Vicky Ng and GI team members Pencharz P: University of Toronto, Department of Paediatics, Physician Research Award for Career Excellence, 2007. Division Head Dr. Stanley Zlotkin Full-Time Dr. Peter Durie Dr. Nicola Jones Dr. Anne-Marie Griffiths Dr. Simon Ling Dr. Vicky Ng Dr. Paul Pencharz Dr. Eve Roberts Dr. Philip Sherman Dr. Mary Zachos Part-Time Dr. Glenn Berall Dr. Carol Durno Dr. Paul Kortan Dr. Margaret Marcon Dr. Gary May Dr. Mary Sherlock Dr. Catharine Walsh Cross-Appointed Dr. Mary Corey Research Fellows Dr. Eric Benchimol Dr. Juan Gana Dr. Tanja Gonska Dr. Aleixo Muise Dr. Khalid Noli Clinical Fellows Dr. Orlee Guttman Dr. Seamus Hussey Dr. Dan Turner Dr. Thomas Walters Dr. Eytan Wine Dr. Jason Yap Subspecialty Residents Dr. Ewurabena Simpson Administrative Staff Chona Callejo Roxanne Francis Margaret Johnson Sherry Joy Parasto Nejatie Lilatool Shakur Suzanne Shek Turner D: Chisholm Memorial Fellowship, the Elizabeth Arbuthnot Dayson Fellowship, the Nellie L. Farthing Fellowship, the William S. Fenwick Fellowship, the Miriam Neveren Memorial Award and the Joseph M. West Family Memorial Fund Research Award, 2007. Wine E: Canadian Institute of Health Research/ Canadian Association of Gastroenterology/ Industry (Astra Zeneca) Research Initiative Award, 2006. Wine E: University of Toronto Faculty of Medicine, Postgraduate Award, 2006. PUBLICATIONS Achkar JP, Dassopoulos T, Silverberg MS, Tuvlin JA, Duerr RH, Brant SR, Siminovitch K, Reddy D, Datta LW, Bayless TM, Zhang L, Barmada MM, Rioux JD, Steinhart JH, McLeod RS, Griffiths AM, Cohen Z, Yang H, Bromfield JP, Schumm P, Hanauer SB, Cho JH, Nicolae D and the NIDDK IBD Genetics Consortium: Phenotype-stratified genetic linkage study demonstrates that IBD2 is an extensive ulcerative colitis locus. American Journal of Gastroenterol 2006: 101: pp 572-580. Aspelund G, Ling SC, Ng V, Kim PCW: A role for laparoscopic approach in the treatment of biliary atresia and choledochal cysts. Journal of Pediatric Surgery 2007: 42: pp 869-872. Atkinson C, Morris SK, Ng V, Friedman JN: A child with fever, hip pain and limp. Canadian Medical Association Journal 2006: 174: pp 924. Avitzur Y, Ngan BY, Lao M, Fecteau A, Ng VL: Prospective evaluation of the prevalence and clinical significance of positive autoantibodies after pediatric liver transplantation. Journal of Pediatric Gastroenterology and Nutrition 2007: 45: pp 222-227. Ball RO, Courtney-Martin G, Pencharz PB: The in vivo sparing effect of cysteine on methionine requirements in animal models and adult humans. Journal of Nutrition 2006: 136: pp 1682-1693. 38 DEPARTMENT OF PAEDIATRICS Barron M, Doyle J, Zlotkin S: Vitamin K deficiency in children pre-bone marrow transplantation. Bone Marrow Transplantation 2006: 37: pp 151-154. Beharry SA, Ackerely C, Corey M, Kent G, Heng YM, Christensen H, Luk C, Yantiss RK, Nasser IA, Zaman M, Freedman SD, Durie PR: Long-term docosahexaenoic acid therapy in a congenic murine model of cystic fibrosis. American Journal of Physiology – Gastrointestinal Liver Physiology 2007: 292: pp 839-848. Bousvaros A, Antonioli D, Colletti R, Dubinsky M, Glickman J, Gold B, Griffiths A, JEvon G, Higuchi L, Hyams J, Kirschner B, Kugathasan S, Baldassano R, Russo P: Differentiating ulcerative colitis from Crohn’s disease in children and young adults: A report of a working group of the North American Society of Pediatric Gastroenterology Hepatology and Nutrition, and the Crohn’s and Colitis Foundation of America. Journal of Pediatric Gastroenterology and Nutrition 2007: 44: pp 653-674. Brunton J, Shoveller AK, Pencharz PB, Ball RO: Identification of limiting amino acids in two parenteral nutrition solutions by indicator amino acid oxidation. Journal of Nutrition 2007: 137: pp 1253-1259. Cantin AM, Hanrahan JW, Bilodeau G, Ellis L, Dupuis A, Liao J, Zielenski J, Durie P. Cystic Fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers. American Journal of Respiratory and Critical Care Medicine 2006: 173: pp 1139-1144. Clarke M, Berall G, Pencharz P, Locker D, Kenny DJ: Malnourishment in a population of young children with severe early childhood caries attending a hospital dental clinic for oral rehabilitation with general anesthesia. Pediatric Dentistry 2006: 28: pp 254-259. Cooper M, Ungar W, Zlotkin S. An assessment of inter-rater agreement of the literature filtering process in the development of evidence-based dietary guidelines. Public Health Nutrition 2006: 9: pp 494-500. ANNUAL REPORT 2006-07 Dasgupta R, Roberts E, Superina RA, Kim PC: Effectiveness of Rex shunt in the treatment of portal hypertension. Journal of Pediatric Surgery 2006: 41: pp 108-112. Dassopoulos T, Nguyen GC, Sitton A, Bromfiled GP, Schumm LP, Wu Y, Riguiero M, Siemanowski B, Griffiths A, Brant S, Rious JD, Yang H, Duerr RH, Silverberg M, Cho JH, Steinhart H: Assessment of reliability and validity of BID phenotyping within the NIDDK IBD Genetics Consortium. Inflammatory Bowel Disease 2007: 13: pp 975-983. De Pee S, Moench-Pfanner R, Martini E, Zlotkin S, Darton-Hill I, Bloem MW: Home-fortification in emergency response and transition programming: Experiences in Aceh and Nias, Indonesia. Food and Nutrition Bulletin 2007: 28: pp 189-197. Diamond IR, Fecteau A, Millis JM, Losanoff JE, Ng V, Anand R, Song C and the SPLIT Research Group: Impact of graft type on outcome in pediatric liver transplantation. Annals of Surgery 2007: 246: pp 301-310. Diamond IR, de Silva N, Pencharz PB, Kim JH, Wales PW: Neonatal short bowel syndrome outcomes after the establishment of the first Canadian multidisciplinary intestinal rehabilitation program: preliminary experience. Journal of Pediatric Surgery 2007: 42: pp 806-811. Dosman C, Brian J, Drmic I, Senthilselvan A, Harford M, Smith R, Sharieff W, Zlotkin S, Moldofsky H, Roberts W: Children with autism: Effect of iron supplementation on sleep and ferritin. Pediatric Neurology 2007: 36: pp152-158. Griffiths AM: Enteral feeding in inflammatory bowel disease. Current Opinion in Clinical Nutrition and Metabolic Care 2006: 9: pp 314-318. Griffiths AM: Interventions for growth failure in pediatric IBD. Journal of Pediatric Gastroenterology and Nutrition 2006: 43: pp S17-S18. Hirve S, Bhave S, Bavdekar A, Naik S, Pandit A, Schauer C, Christofides C, Hyder Z, Zlotkin S: Low dose ‘Sprinkles’ – An innovative approach to treat iron deficiency anemia in infants and young children. Indian Pediatrics 2007: 44: pp 91-100. Ho W, Cheretakis C, Durie P, Kulkarni G, Glogauer M: Prevalence of oral diseases in ShwachmanDiamond syndrome. Special Care in Dentistry 2007: 27: pp 52-58. Hsu JWC, Ball RO, Pencharz PB: Evidence that phenylalanine may not provide the full needs for aromatic amino acid needs in children. Pediatric Research 2007: 61: pp 361-365. Hyams J, Markowitz J, Lerer T, Griffiths AM, Mack D, LeLeiko N, and the Pediatric IBD Registry: The natural history of corticosteroid therapy for ulcerative colitis in children. Clinical Gastroenterology and Hepatology 2006:4: pp 1118-1123. Hyams JS, Crandall W, Kugathasan S, Griffiths AM, Olson A, Baldassano R, and the REACH Study Group: Induction and maintenance infliximab therapy for the treatment of moderate-to-severe Crohn’s disease in children. Gastroenterology 2007: 132: pp 863-873. Hyder Z, Zlotkin S: Effect of daily versus once weekly home fortification with sprinkles on haematological and iron status among young children in rural Bangladesh. Food and Nutrition Bulletin 2007: 28: pp 156-164. Jandu N, Shen S, Wickham M, Prajapati R, Finlay B, Karmali M, Sherman P: Multiple seropathotypes of Verotoxin-producing Escherichia coli disrupt interferon-gamma-induced tyrosine phosphorylation of signal transducer and activation of transcription-1. Microbial Pathogenesis 2007: 42: pp 62-71. Rioux JD, Xavier RX, Taylor KD, Silverberg MS, Goyette P, Huett A, Green T, Kuballa P, Barmada MM, Datta LW, Shugart YY, Griffiths AM, Targan SR, Ippoliti AF, Bernard E-J, Mei L, Nicolae DL, Regueiro M, Schumm LP, Steinhart AH, Rotter JI, Duerr RJ, Cho JH, Daly MJ, Brant SR: Genomewide association study identifies new susceptibility loci for Crohn’s disease and implicates autophagy in disease pathogenesis. Nature Genetics 2007: 39: pp 596-604. Johnson-Henry K, Hagen K, Gordonpour M, Tompkins T, Sherman P: Surface-layer protein extracts from Lactobacillus helveticus inhibit enterohaemorrhagic Escherichia coli O157:H7 adhesion to epithelial cells. Cellular Microbiology 2007: 9: pp 356-367. Kalnins D, Durie P, Pencharz P: Nutritional management of Cystic Fibrosis patients. Current Opinion in Clinical Nutrition and Metabolic Care 2007: 10: pp 348-354. Kalnins D, Ellis L, Corey M, Pencharz PB, Stewart C, Tullis E, Durie, PR: Enteric-coated pancreatic enzyme with bicarbonate is equal to standard enteric-coated enzyme in treating malabsorption in cystic fibrosis. Journal of Pediatric Gastroenterology and Nutrition 2006: 42: pp 256-261. Kappelman M, Bousvaros A, Hyams J, Markowitz J, Griffiths AM, and the Pediatric IBD Registry Group: Variation in the initial management of children with Crohn’s disease. Inflammatory Bowel Disease 2007: 13: pp 890-895. Khan E, Hyder SMZ, Tondeur MC, Raza S, Khan NA, Zlotkin S: Home fortification with sprinkles to reduce childhood anaemia: Lessons learned in North West Frontier Province, Pakistan. Pakistan Journal of Medical Research 2006: 45: pp 35-40. Khurana R, Fishbach L, Chiba N, Van Zanten S, Sherman P, George B, Goodman K, Gold B: Metaanalysis: Helicobacter pylori eradication treatment efficacy in children. Alimentary Pharmacology and Therapeutics 2007: 25: pp 523-536. Kirsch R, Bokhary R, Marcon MA, Cutz E: Activated mucosal mast cells differentiate eosinophilic (allergic) esophagitis from gastroesophageal reflux disease. Journal of Pediatric Gastroenterology and Nutrition 2007: 44: pp 20-26. Kulkarni PP, She Y-M, Smith SD, Roberts EA, Sarkar B: Proteomics of metal-transport and metal-associated diseases. Chemistry 2006: 12: pp 2410-2422. Law GK, Bertolo RF, Adiri-Awere A, Pencharz PB, Ball RO: Adequate oral threonine is critical for mucin production and gut function in neonatal piglets. American Journal of Physiology 2007: 292: pp G1293-G1301. Mack D, Griffiths AM, Markowitz J, Bousvaros A, Rosh J, Mezoff A, Moyer S, Keljo D, Hyams JS for the Pediatric IBD Registry: 2006-1865.R2 laboratory values of children newly diagnosed with Inflammatory Bowel Disease. Pediatrics 2007: 119: pp 1113-1119. Mager D, McGee P, Furuya KN, Roberts EA: Prevalence of vitamin K deficiency in children with chronic liver disease. Journal of Pediatric Gastroenterology and Nutrition 2006: 42: pp 71-76. Mager D, Roberts EA: Nonalcoholic fatty liver disease in children. Clinics in Liver Disease 2006: 10: pp 109-131. Mager DR, Wykes LJ, Roberts EA, Ball RO, Pencharz PB: Effect of orthotopic liver transplantation on branched chain amino acid requirement in children. Pediatric Research 2006: 59: pp 829-834. Mager DR, Wykes LJ, Roberts EA, Ball RO, Pencharz PB: Leucine oxidation in children with mild to moderate chronic cholestatic liver disease. Journal of Nutrition 2006: 136: pp 965-70. Markowitz J, Hyams J, Mack D, LeLeiko N, Griffiths AM, and the Pediatric IBD Registry: Corticosteroid therapy in the age of infliximab: acute and 1 year outcomes in newly diagnosed children with Crohn’s disease. Clinical Gastroenterology and Hepatology 2006: 4: pp 1124-1129. Muise AM, Turner D, Kim P, Marcon M, Ling SCL: Biliary atresia associated with choledochal cyst: implications for classification. Clinical Gastroenterology and Hepatology 2006: 4: pp 1411-1414. Muise AM, Walters TD, Wine E, Griffiths AM, Turner D, Duerr RH, Regueiro MD, Ngan BY, Xu W, Sherman PM, Silverberg MS, Rotin D: Protein Tyrosine Phosphatase Sigma is associated with ulcerative colitis. Current Biology 2007: 17: pp 1212-1218. Nicholas DB, Munk M, Otley A, Avolio J, Griffiths AM: Challenges and strategies of children and adolescents with Inflammatory Bowel Disease: A qualitative examination. Health and Quality of Life Outcomes 2007: 5: p 28. Norton SP, Jacobson K, Moroz SP, Culham G, Ng V, Turner J, John P: The congenital intrahepatic arterioportal fistula syndrome: elucidation and proposed classification. Journal of Pediatric Gastroenterology and Nutrition 2006: 43: pp 248-255. Nguyen GC, Torres EA, Regueiro M, Bitton A, Dassopoulos T, Griffiths AM, Hanauer SB, Kane SV, Lahaie R, Oliva-Hemker M, Pare P, Wild GE, Rioux JD, Yang H, Duerr RH, Cho JH, Steinhart AH, Brant SR, Silverberg MS: Inflammatory Bowel Disease characteristics among African Americans, Hispanics, and non-Hispanic Whites: Characterization of a large North American cohort. American Journal of Gastroenterology 2006: 101: pp 1012-1023. O’Shea A-M, Wilson G, Ling SC, Minassian B, Cutz E: Lafora-like ground glass inclusions in hepatocytes of paediatric patients – Report of two cases. Pediatric and Developmental Pathology March 22, 2007 [Epub ahead of print]. Otley AR, Griffiths AM, Kugathasan S, Pfefferkorn M, Mezoff A, Rosh J, Tolia V, Markowitz J, Mack D, Oliva-Hemker M, Wyllie R, Rothbaum, Bousvaros A, Hyams JS: Health-related quality of life in the first year following a diagnosis of pediatric inflammatory bowel disease. Inflammatory Bowel Disease 2006: 12: pp 684-691. Pencharz PB: Drug dosing in children – Special considerations. Haemophilia 2006: 12: pp 30-32. DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 39 Roberts EA, Yap J: Nonalcoholic fatty liver disease (NAFLD): Approach in the adolescent patient. Current Treatment Options in Gastroenterology 2006: 9: pp 423-431. Roberts EA: Nonalcoholic steatohepatitis (NASH) in children. Clinics in Liver Disease 2007: 11: pp 155-172. Roberts EA: Pediatric NAFLD – A “growing” problem? Journal of Hepatology 2007: 46: pp 1133-1142. Schibli S, Corey M, Gaskin KJ, Ellis L, Durie PR: Towards the ideal quantitative pancreatic function test: Analysis of test variables that influence validity. Clinical Gastroenterology and Hepatology 2006: 4: pp 90-97. Sharieff W, Horton SE, Zlotkin S: Economic gains of a home fortification program: Evaluation of “Sprinkles” from the provider’s perspective. Canadian Journal of Public Health 2006: 97: pp 20-23. Sharieff W, Yin S, Wu M, Yang Q, Schauer C, Tomlinson G, Zlotkin S: Daily or weekly administration of micronutrient sprinkles in school has high compliance and does not cause iron overload in Chinese children: A cluster-randomized trial. Public Health Nutrition 2006: 9: pp 336-344. Sharieff W, Zlotkin S, Tondeur M, Feldman B, Tomlinson G. Physiologic mechanisms can predict hematologic responses to iron supplements in growing children: a computer simulation model. American Journal of Clinical Nutrition 2006: 83: pp 681-687. Soon G, Saunders N, Ipp M, Sherman P, Macarthur C: Community-based case-control study of childhood chronic abdominal pain: role of selected laboratory investigations. Journal of Pediatric Gastroenterology and Nutrition 2007: 44: pp 524-526. Squires RH, Shneider BL, Bucuvalas B, Alonso E, Sokol RJ, Narkewicz M, Dhawan A, Rosenthal P, Rodriguez-Baez N, Murray KF, Horslen S, Martin MG, Lopez MJ, Soriano H, McGuire BM, Jonas M, Yazigi N, Shepherd RW, Schwarz K, Lobritto S, Thomas DW, Lavine JE, Karpen S, Ng V, Kelly D, Simonds N, Hynan LS: Acute liver failure in children: The first 348 patients in the pediatric acute liver failure (PALF) study group. Journal of Pediatrics 2006: 148: pp 652-658. Sweezey NB, Smith D, Corey M, Ellis L, Carpenter S, Tullis DE, Durie P, O’Brodovich H: Amilorideinsensitive nasal potential difference varies with the menstrual cycle in cystic fibrosis. Pediatric Pulmonology 2007: 42: pp 519-524. Sylvester FA, Wyzka N, Hyams JS, Lerer T, Vance K, Hawker G, Griffiths AM: natural history of bone metabolism and bone mineral density in children with Inflammatory Bowel Disease. Inflammatory Bowel Disease 2007: 13: pp 42-50. Terebiznik MR, Vazquez CL, Banks D, Colombo MI, Jones NL: Helicobacter pylori VacA toxin promotes bacterial intracellular survival in gastric epithelial cells. Infection and Immunity 2006: 74: pp 6599-6614. Turner D, Otley AR, de Bruijne J, Mack D, Uusoue K, Walters T, Zachos M, Mamula P, Hyams J, Griffiths AM: Development and validation of a pediatric ulcerative colitis activity index. A prospective multicenter study. Gastroenterology 2007: 133: pp 423-432. Turner D, Schreiber R, Grant D, Hebert D, Sherman P: Hemolytic uremic syndrome after pediatric liver transplantation. Journal of Pediatric Gastroenterology and Nutrition 2006: 43: pp 109-112. Shneider B, González-Peralta R, Roberts EA: Controversies in management pediatric liver disease: hepatitis B, C and NAFLD: Summary of single topic conference. Hepatology 2006:44: pp 1344-1354. Turner D, Walsh C, Steinhart AH, Griffiths AM: Response to corticosteroids in severe ulcerative colitis: a systematic review of the literature and a meta-regression. Clinical Gastroenterology and Hepatology 2007: 5: pp 103-110. Skinn A, Vergnolle N, Cellars L, Sherman P, MacNaughton W: Combined challenge of mice with Citrobacter rodentium and ionizing radiation promotes bacterial translocation. International Journal of Radiation Biology 2007: 83: pp 375-382. Turner D, Zlotkin SH, Shah P, Griffiths A: Omega 3 Fatty Acids (fish oil) for Maintenance of Remission in Crohn’s Disease. Cochrane Database of Systematic Reviews 2007: 2: pp 1469-1493. Skinn A, Vergnolle N, Zamuner S, Wallace J, Cellar L, MacNaughton W, Sherman P: Citrobacter rodentium causes iNOS-independent intestinal epithelial dysfunction in mice. Canadian Journal of Physiology and Pharmacology 2006: 84: pp 1301-1312. 40 DEPARTMENT OF PAEDIATRICS Urschel KL, Evans AR, Pencharz PB, Ball RO: Infusion of glucagon-like peptide 2 with an arginine deficient diet increases endogenous arginine synthesis from proline in parenterallyfed neonatal piglets. Livestock Science 2007: 108: pp 41-44. ANNUAL REPORT 2006-07 Urschel KL, Evans AR, Wilkinson CW, Pencharz PB, Ball RO: Parenterally-fed neonatal piglets have a low level of endogenous arginine synthesis from circulating proline. Journal of Nutrition 2007: 137: pp 601-606. Urschel KL, Pencharz PB, Ball RO: Ornithine metabolism, but not arginine synthesis, is affected by the addition of ornithine to an arginine-deficient diet in enterally-fed piglets. Livestock Science 2007:108: pp 137-141. Urschel KL, Shoveller AK, Uwiera RRE, Pencharz PB, Ball RO: Citrulline is an effective arginine precursor in enterally-fed neonatal piglets. Journal of Nutrition 2006: 136: pp 1806-1813. Urschel KL, Wilkinson CW, Pencharz PB, Ball RO: Coadministration of ornithine alpha-ketoglutarate is no more effective than ornithine as an arginine precursor in piglets enterally fed an argininedeficient diet. Journal of Nutrition 2007: 137: pp 55-62. Vogt JA, Ishii-Schrade KB, Pencharz PB, Jones PJH, Wolever TMS: L-rhamnose and lactulose decrease serum triacylglycerols and their rate of synthesis, but do not affect serum cholesterol concentration in men. Journal of Nutrition 2006: 136: pp 2160-2166. Walters TD, Gilman AR, Griffiths AM: Linear growth improves during infliximab therapy in children with chronically active severe Crohn’s disease. Inflammatory Bowel Disease 2007: 13: pp 424-430. Wilschanski M, Dupuis A, Ellis L, Jarvi K, Zielenski J, Tullis E, Martin S, Corey M, Tsui L-C, Durie P: Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials. American Journal of Respiratory and Critical Care Medicine 2006: 174: pp 787-794. Yea C, Bukh J, Ayers M, Roberts E, Krajden M, Tellier R: Monitoring of HCV quasispecies in chronic infection by MALDI-TOF mass spectrometry mutation detection. Journal of Clinical Microbiology 2007: 45: pp 1053-1057. Zachos M, Tondeur M, Griffiths AM: Enteral nutritional therapy for inducing remission in Crohn’s disease. Cochrane Database of Systematic Reviews 2007: 24: CD000542. Zareie M, Johnson-Henry K, Jury J, Yang P, Ngan B, McKay D, Soderholm J, Perdue M, Sherman P: Probiotics prevent bacterial translocation and improve intestinal barrier function in rats following chronic psychological stress. Gut 2006: 55: pp 1553-1560. Zlotkin S: Priorities in Nutritional Rehabilitation. Editorial. Journal of Pediatric Gastroenterology and Nutrition 2006: 43: pp S66-S71. Dr. Eve Roberts Duerr RH, Taylor KD, Brant SR, Rioux JD, Silverberg MS, Daly MJ, Steinhart AH, Abraham C, Regueiro M, Griffiths AM, Dassopoulos T, Bitton A, Yang H, Targan S, Datta LW, Kistner EP, Schumm LP, Lee A, Gregersen PK, Barmada MM, Rotter JI, Nicolae DL, Cho JH: A genome-wide association study identifies IL23R as an Inflammatory Bowel Disease gene. Science 2006: 314: pp 1461-1463. Zlotkin S: A critical assessment of upper intake levels for Infants and Children. Journal of Nutrition 2006: 36: pp 1S-5S. Zlotkin SH, Schauer S, Owusu-Agyie S, Wolfson J, Tondeur MC, Asante KP, Newton S, Serfass RE, Sharieff W: Demonstrating zinc and iron bioavailability from intrinsically labeled microencapsulated ferrous fumarate and zinc gluconate sprinkles in young children. Journal of Nutrition 2006: 136: pp 920-925. BOOKS AND BOOK CHAPTERS Ahmed NN, Durie PR: Nonpulmonary manifestations of Cystic Fibrosis. In: Kendig’s Disorders of the Respiratory Tract in Children, 7th Edition (Chernick V, Boat TF, Wilmott RW, Bush A, eds). Saunders Elsevier, Philadelphia, 2006. Chan V, Sherman P, Bourke B: Evolution and origin of virulence isolates. In: Bacterial Genomes and Infectious Diseases (Chan V, Sherman P, Bourke B, eds). Humana Press, Totowa, NJ, 2006. Chan V, Sherman P, Bourke B: Genomic approach to understanding infectious diseases mechanisms. In: Bacterial Genomes and Infectious Diseases (Chan V, Sherman P, Bourke B, eds). Humana Press, Totowa, NJ, 2006. Cox DW, Roberts EA: Wilson disease. In: Gastrointestinal and Liver Disease (Feldman M, Scharschmidt BF, Sleisenger MH, eds). SaundersElsevier, Philadelphia, 2006. Griffiths AM: Nutrition and inflammatory bowel disease. In: Modern Nutrition in Health and Disease, 10th Edition (Shils ME, ed). Lipincott, Williams & Wilkinson, Baltimore, 2006. Mager D, Pencharz PB: Nutritional considerations in children with developmental disabilities. In: A Comprehensive Guide to Intellectual and Developmental Disabilities (Brown I, Percy M, eds). Brookes Publishing Company, Baltimore, 2007. Ng VL: Laboratory assessment of liver function and injury. In: Liver Disease in Children, 3rd Edition. (Suchy FJ, Sokol RJ, Balistreri WF, eds). Cambridge University Press, New York 2007. Ng VL: Neonatal hepatitis. In: Pediatric Gastrointestinal and Liver Disease, 3rd edition, (Wyllie R, Hyams JS, eds). Elsevier, Amsterdam, 2006. Pencharz PB, Ball RO: Amino acid requirements of infants and children. In: Nestle Nutrition Workshop Series, Volume 58 (Rigo J, Ziegler EE, eds). Karger, Switzerland, 2006. Pencharz PB, Young VR: Protein and amino acids. In: Present Knowledge in Nutrition, 9th Edition (Bowman BA, Russell RM, eds). ILSI Press, Washington, 2006. Portmann B, Roberts EA: Developmental abnormalities and liver disease in childhood. In: MacSween’s Pathology of the Liver, 5th Edition (Burt A, Portmann B, Ishak K, Ferrell LD, eds). Elsevier, Edinburgh, 2007. Portmann B, Thompson RJ, Roberts EA, Paterson AC: Genetic-metabolic disorders and the liver. In: MacSween’s Pathology of the Liver, 5th edition (Burt A, Portmann B, Ishak K, Ferrell LD, eds). Elsevier, Edinburgh, 2007. Roberts EA, Cox DW: Wilson’s disease. In: Hepatology (Boyer T, Manns M, Wright T, eds). Elsevier, Philadelphia, 2006. Roberts EA: Drug-induced liver disease. In: Liver Disease in Children, 3rd Edition (Suchy FJ, Sokol RJ, Balistreri WF, eds). Cambridge University Press, New York, 2007. Roberts EA: NASH in children. In: Nonalcoholic Steatohepatitis (Bataller R, ed). Permanyer, Barcelona, 2007. FUNDING A longterm multicenter open-label follow-up study of safety of infliximab as maintenance therapy of paediatric Crohn’s disease. Griffiths AM. Centocor, Inc. ($10,000/year 2003-2008) A multi-centre study of the safety and efficacy of N-acetylcysteine in the treatment of acute liver failure in paediatric patients not caused by acetaminophen. Ng VL, Fecteau A. National Institutes of Health/National Institute of Diabetes and Digestive and Kidney Diseases (US$69,000 2005-2010) A novel candidate susceptibility gene at the IBD6 locus, Rotin D, Griffiths A. Crohn’s and Colitis Foundation of Canada Innovations Grant. ($50,000 2007) Arginine and CF. Grasemann H, Ratjen F, Pencharz P. Canadian Cystic Fibrosis Foundation ($140,000 2007-2010) Assessment of activity and prediction of outcome in paediatric ulcerative colitis. Turner D, Steinhart H, Griffiths A. Canadian Association of Gastroenterology/Canadian Institutes of Health Research ($65,000/year 2006-2008) Cell signalling in mucosal inflammation and pain. Ellen R, Sherman P. Canadian Institutes for Health Research ($300,000/year 2002-2008) Roberts EA: Wilson’s disease. In: Medicine (Liver II) (Chapman R, Gilmore I, eds). Oxford, UK, 2007. Cell signals: Cell signaling in mucosal inflammation and pain. Ellen R, Jones N. Canadian Institutes of Health Research ($300,000 2002-2008) Secker D, Zlotkin S: Infant feeding. In: Essentials of Human Nutrition, 3rd Edition (Mann J, Stewart T, eds) Oxford University Press, Inc., New York, 2006. Centre for the study of complex childhood diseases. Post M, Henkelman M, Ratjen F, Snead C, Pencharz P. Canadian Foundation for Innovation ($8,228,769 2006) Sherman P, Bourke B, Chan V: Future directions of infectious disease research. In: Bacterial Genomes and Infectious Diseases (Chan V, Sherman P, Bourke B, eds). Humana Press, Totowa, NJ, 2006. Clinical research facilitation award: Understanding CFTR folding, trafficking and membrane stability and applying this knowledge towards development of drug discovery assays. Durie PR. Cystic Fibrosis Foundation Therapeutics, Inc. (US$108,280 2007) Sonies BC, Marcon MA Imundo I: Swallowing and other digestive problems. In: Myositis and Me: A Complete Family Guide to Juvenile Dermatomyositis and Other Inflammatory Myopathies. Myositis Association of America, Washington, 2006. Clinician scientist training program in clinical nutrition research. Pencharz P. Canadian Institutes of Health Research ($265,000 2002-2008) Walters TR, Griffiths AM: Growth failure in childhood IBD. In: Challenges in IBD, 2nd Edition (Jewell D, Mortensen N, Steinhart H, Pemberton J, Warren B, eds). Blackwell Publishing Ltd., Oxford, UK, 2006 Zlotkin S, Tondeur MC: Successful approachessprinkles. In: Nutritional Anemia, 1st Edition (Kraemer K, Zimmermann M, eds) Sight and Life Press, Switzerland, 2007. Comparative pathogenesis and public health significance of Verotoxin-producing E. coli serotypes. Karmali M, Sherman P. Canadian Institutes of Health Research Safe Food and Water Initiative ($897,000 2003-2006) Determining the threshold of CFTR function that causes CF disease. Durie PR. Canadian Cystic Fibrosis Foundation ($110,000/year 2005-2008) Development and validation of a disease-specific health-related quality of life questionnaire for children after liver transplantation. Otley AR, Ng VL, Nicholas DB, Thompson J, Gilmour S, Watters C. Canadian Institutes of Health Research (US $280,576 2005-2008) Immune regulation and biomarker development in paediatric and adult onset human autoimmune diseases. Bar-Or A, Banwell B, Piccirillo CA, Polychronakos C, Sherman P. Canadian Institutes of Health Research Team Grant ($2,018,445 2007-2112) Development of a novel microfluidic biochip system for the detection of pathogenic bacteria. Liu D, Sherman P. National Science and Engineering Research Council of Canada ($362,260 2003-2006) Innovative strategies for optimizing management of infants with biliary atresia. Guttman O (CoSupervisor: Roberts EA) Canadian Association for the study of Liver Diseasse/Canadian Institutes of Health Research/Industry Fellowship ($55,000 2007-2008) Disordered hepatocellular cu handling in the Tx-J mouse, a model of Wilson Disease. Roberts EA. Canadian Institutes of Health Research ($73,640 2004-2007) Epidemiology of eosinophilic esophagitis in children and adolescents in Canada. Marcon, MA, Dourin EJ. Campbell Fund ($62,966 2006-2008) Food intake control in children. Anderson GH, Berall GB, Goode RC, Pencharz PB. Canadian Institutes of Health Research ($104,219 2007-2010) Functional outcomes after paediatric liver transplantation. Ng VL, Fecteau A. National Institutes of Health/NIDDK (US$6,000 2005-2007) Hepatocellular mitochondrial function in a novel murine model of Wilson Disease. Sarkar B, Roberts EA. Canadian Liver Foundation ($59,986 2004-2006) Homocysteine clearance by hemodialysis. Darling P, Goldstein M, McFarlane P, Pencharz P. Kidney Foundation ($28,350 2006) Host epithelial cell responses to attaching-effacing gastrointestinal pathogens. Sherman P. Canadian Institutes of Health Research ($679,517 2002-2007) Host immune response to Helicobacter pylori infection. Jones NL. Canadian Institute of Health Research ($311,073 2004-2007) IBD research database support. Griffiths AM. Schering Canada ($20,000/year 2005-ongoing) Identification of IBD susceptibility genes. Silverberg M, Siminovitch K, Steinhart H, Griffiths AM, Cohen Z. National Institutes of Health/National Institute of Diabetes and Digestive and Kidney Diseases ($38,000/year 2002-2007) International collaboration project. Zlotkin S. Canadian Institutes of Health Research ($25,000 2006-2006) International opportunities program development/planning. Zlotkin S. Canadian Institutes of Health Research ($25,000 2007-2008) Predictors of response to intravenous corticosteroids therapy in paediatric patients with severe ulcerative colitis: A multi-center study. Griffiths AM. Schering Canada ($150,000 2007) Role of probiotics in the management of experimental inflammatory bowel disease. Sherman PM. Crohn’s and Colitis Foundation of Canada “Fay Shapiro Cutler Grant-In-Aid Research” ($375,000 2007-2010) SBDS protein expression in peripheral blood leukocytes. Durie PR, Rommens JM, Shimamura A. Shwachman Diamond Syndrome Foundation (US$30,000 2006-2007) Sprinkles project support. Zlotkin S. HJ Heinz Company Foundation ($600,000 2006-2007) Investigation of Helicobater pylori survival in host cells. Jones NL. Early Researcher Award, Ontario Ministry of Research and Innovation ($150,000 2006-2011) Knowledge translation award. Zlotkin S. Canadian Institutes of Health Research ($100,000 2006-2007) Molecular genetics, SBDS function and deficiency in SDS. Rommens JM, Durie PR. National Institutes of Health ($1,250,000 2004-2009) Molecular mechanisms associated with H. pylori survival in macrophages. Jones NL. Canadian Association of Gastroenterology/Canadian Institutes of Health Research ($60,000 2004-2006) Multi-site trial of azathioprine dosing in Crohn’s disease. Griffiths A, Hanauer S. National Institutes of Health/National Institute of Diabetes and Digestive and Kidney Diseases ($20,000 per 2005-2007) Neonatal protein and energy metabolism. Pencharz P. Medical Research Council/Canadian Institutes of Health Research ($90,470 2005 and $136,817 2006-2011) NOD2/CARD15 genotypes in the prediction of time to post-operative recurrence following resection for ileal Crohn’s disease. Walters T, Griffiths AM, Silverberg M. Canadian Association of Gastroenterology/Canadian Institutes of Health Research ($65,000/year 2005-2007) Pico-Salax versus Poly-Ethylene Glycol for bowel cleanout before colonoscopy in children – A single blinded randomized controlled trial. Ling SC. Ferring Inc. ($24,000 2006-2007) Studies of paediatric liver transplantation (SPLIT). Ng V, Fecteau A. National Institutes of Health/ National Institute of Diabetes and Digestive and Kidney Diseases (US$75,000 2005-2010) Studies of primary sclerosing cholangitis “STOPSC”. Ling SC. The Morgan Foundation ($2,500 2006-2007) Targeted small molecule therapies for Cystic Fibrosis. Bear CE, Deber CM, Durie PR, Lingwood C, Rotin D, Ratjen F, Yeger H. Canadian Cystic Fibrosis Foundation – BREATHE Program ($480,000/year 2004-2009) The contribution of genetic modulators of disease severity in cystic fibrosis to other diseases with similarities of clinical phenotype. Durie PR, Zielenski J. Genome Canada ($5,787,662 2005-2009) The long-term effect of ALL and its treatment on bone mineralization. Pencharz P. Paediatric Oncology Group of Ontario ($30,310 2006) The use of indicator amino acid oxidation to determine amino acid requirement. Pencharz P, Ball R. Medical Research Council/Canadian Institutes of Health Research ($152,967 2005-2010) Therapeutic interventions for paediatric nonalcoholic fatty liver disease (NAFLD) Yap J. (Co-supervisor: Roberts EA) Canadian Institutes of Health Research/Strategic Training Initiative in Health Research ($40,000 2006-2008) DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 43 Division Head Dr. Victor Blanchette Full-Time Dr. Oussama Abla Dr. Ute Bartels Dr. Sylvain Baruchel Dr. Eric Bouffet Dr. Leonardo Brandão Dr. Michael Capra Dr. Manuel Carcao Dr. Helen Chan Dr. John Doyle Dr. Yigal Dror Dr. Adam Gassas Dr. Ron Grant Dr. Abha Gupta Dr. Hans Hitzler Dr. Annie Huang Dr. Meredith Irwin Dr. Walter Kahr Dr. Melanie Kirby Dr. David Malkin Dr. Ahmed Naqvi Dr. Paul Nathan Dr. Isaac Odame Dr. Lillian Sung Dr. Uri Tabori Dr. Sheila Weitzman Dr. Suzan Williams Cross-Appointed/ Part-Time Dr. Sean Bulley Dr. Anthony Chan Dr. Stephen Comay Dr. Lee Ann Gallant Dr. Mark Greenberg Dr. David Hodgson Dr. Nicole Inch Dr. Normand Laperriere Dr. Barbara-Ann Millar Dr. Aubrey Maze Dr. Fred Saunders Dr. Ben Saxon Dr. Jill Solomon Haematology/Oncology DIVISIONAL OVERVIEW Dr. Abha Gupta The Division of Haematology/Oncology provides a diagnostic service and specialist care for children <18 years of age with cancer (paediatric oncology), non-malignant blood disorders (paediatric hematology) and haematopoietic stem cell (blood and marrow) transplantation. The division is the largest of its kind in Canada and among the largest of such programs worldwide. Approximately 300 new cancer cases are registered in the oncology program each year, and The Hospital for Sick Children is the designated site for haematopoietic stem cell transplantation for patients <18 years of age in the Province of Ontario. Dr. Gupta holds a cross-appointment in the Department of Medical Oncology at the Princess Margaret Hospital where she will pursue her career interest in sarcomas and bone tumours in adolescents and young adults. Reflecting the size and diversity of its clinical services, the division is functionally organized into six sections and three programs: the leukaemia/lymphoma section (Section Head, Dr. Sheila Weitzman); the neurooncology (brain tumour) section (Section Head, Dr. Eric Bouffet); the solid tumour section (Section Head, Dr. Ron Grant); the blood and marrow transplantation section (Section Head, Dr. John Doyle); the haematology section (Section Head, Dr. Victor Blanchette); the inpatient services section (Section Head, Dr. Ahmed Naqvi); the aftercare (long-term follow up) program (Program Head, Dr. Mark Greenberg); the new agents and experimental therapies program (Program Head, Dr. Sylvain Baruchel); and the cancer genetics program (Program Co-Heads, Drs. David Malkin and Rosanna Weksberg). The haematology section has welldefined large programs in paediatric thrombosis and haemostasis, the paediatric haemoglobinopathies and the paediatric marrow failure syndromes. The division is an active member of the Children’s Oncology Group (COG), the largest paediatric clinical trials group in North America (SickKids Principal Investigator, Dr. David Malkin), and is committed to the improvement in outcomes of children with cancer through participation in ethics approved, prospective clinical trials. The division is a member of the paediatric oncology phase I Consortium of COG (Principal Investigator, Dr. Sylvain Baruchel), and is an active participant in activities of the Société Internationale d’Oncologie Pédiatrique (SIOP), the Histiocyte Society and the Berlin Frankfurt Munster (BFM) paediatric leukaemia clinical trials group. The division is the largest Royal College of Physicians and Surgeons of Canada accredited training program for the specialty paediatric haematology/oncology in Canada (Training Program Director, Dr. Ahmed Naqvi). The education program in the division also includes a very large international training program in paediatric haematology/ oncology and offers subspecialty training opportunities in areas such as paediatric neurooncology, cancer genetics, blood and marrow transplant and paediatric haemostasis. Research in the division spans clinical to basic science with a special focus on translational research. Currently, 14 of the division’s 27 full-time faculty have appointments as clinician-scientists or clinicianinvestigators in the Department of Paediatrics with parallel appointments in the Research Institute. Foci of research include studies in the paediatric leukaemias (Dr. Hans Hitzler), solid tumours (Dr. Meredith Irwin), brain tumors (Drs. Annie Huang, Uri Tabori), retinoblastoma (Dr. Helen Chan), neuroblastoma (Drs. Meredith Irwin, David Kaplan, Sylvain Baruchel), genetics of childhood cancer (Dr. David Malkin), supportive care of children with cancer (Dr. Lillian Sung), adverse effects in survivors of childhood cancer (Drs. Mark Greenberg, Paul Nathan), experimental cancer therapies and angiogenesis (Dr. Sylvain Baruchel), paediatric haemoglobinopathies (Drs. Isaac Odame, Melanie Kirby), paediatric marrow failure syndromes (Dr. Yigal Dror), platelet granule biology (Dr. Walter Kahr), the inherited and acquired bleeding disorders of childhood including the haemophilias (Drs. Victor Blanchette, Manuel Carcao) and paediatric thrombosis (Drs. Leo Brandao, Suzan Williams). Research in the Division is multidisciplinary, including health care professionals with relevant expertise from a variety of areas (e.g., nursing, pharmacy, psychology, social work). The division is committed to facilitating knowledge exchange through international partnerships with a particular focus on countries lacking the health care resources available in Canada. During the past year two full-time physicians were added to the division: Dr. Isaac Odame and Dr. Abha Gupta. Dr. Odame was appointed as Co-Director of the Division’s Haemoglobinopathy Program and Dr. Gupta joins the solid tumour section in the division. The administrative team for the division includes Dr. Victor Blanchette (Division Chief), Dr. Sheila Weitzman (Associate Director, Clinical), Judy Van Clieaf (Director, Child Health Services) and Lucy Holford (Team Leader, Administrative and Support Services). Clinical and Research Fellows Dr. S. Abdullah Dr. I. Abosoudah Dr. S. Afzal Dr. T. Al-Asaad Dr. A. Al-Rawas Dr. M. Al-Shahrani Dr. A. Al-Yamani Dr. A. Al-Zahrani Dr. M. Alharbi Dr. M. Ali Dr. S. Bartakke Dr. T. Biss Dr. E. Blanco Dr. V. Breakey Dr. M. Cada Dr. T. Finkelstein-Schechter Dr. A. Gupta Dr. M. Ishaqi Dr. L. Lau Dr. A. Lee Chong Dr. J. Moppett Dr. B. Pongtanakul Dr. M. Rojas Dr. K. Scheinemann Dr. C. Segbefia Dr. C. Traivaree Dr. T. Truong Dr. B. Williams Dr. P. Wong Dr. M. Zolaly Administrative Staff Bibi Ali Ingrid Argiropoulos Barbara Black Sandra Carbone-Walker Carol Ellis Connie Grillo Lucy Holford Shanaz Karim Philippa McCaffrey Christal Malcolm Sylvia Muir Annabel Sousa Laurel Willmott Arlene Zaldivar Baruchel S: National Order of Merit, France, 2006. Blanchette V: Dr. Cecil Harris Award, Canadian Haemophilia Society. 2006. Bouffet E: Claus Wirsig Humanitarian Award, 2007. Bouffet E: Ontario Institute of Cancer Research, 2007. Bouffet E: The Richard Rowe Award for Clinical Excellence in Paediatric Medical Care, 2007. Dror Y: Canadian Institutes of Health Research Clinical Investigator Award, 2006. Huang A: LIMA International Award for Excellence in Pediatric Brain Tumor Research, Children’s Brain Tumor Foundation, 2006. Kahr W: Phase II Clinician Scientist Award, The Heart and Stroke Foundation of Ontario, 2006. Malkin D: Physician Researcher Award for Scientific Accomplishment, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, 2006. Odame I: Holland Memorial Award for Professional Excellence, 2007. Tabori U: 18th Annual Paediatric Resident/Fellow Research Competition, First prize, 2006. Tabori U: Young Investigators Award, The Canadian Neuro-Oncology Society, 2006. Tabori U: 3rd Prize, Department of Paediatrics Resident and Fellows Research Day Competition, The Hospital for Sick Children, 2006. Tabori U: Merit Award, American Society of Clinical Oncology Annual Meeting, 2006. Weitzman S: Richard Rowe Award for Clinical Excellence in the Department of Paediatrics, 2006. PUBLICATIONS Abdelhaleem M, Beimnet K, Kirby-Allen M, Naqvi A, Hitzler J, Shago M: High incidence of CALMAF10 fusion and the identification of a novel fusion transcript in acute megakaryoblastic leukemia in children without Down’s syndrome. Leukemia 2007: 21: pp 352-353. Abdelhaleem M, Shago M, Abla O: TEL-AML1 fusion in acute lymphoblastic leukemia with L3 morphology. Pediatric Blood Cancer 2007: 49: p 217. Abdelhaleem M, Yi Q, Beimnet K, Grant RM: A novel t(4;11)(q21;q23) MLL-AF4 fusion transcript in infant leukemia. American Journal of Hematology 2007: 82: p 247. 46 DEPARTMENT OF PAEDIATRICS Abdelhaleem M, Yi Q, Beimnet K, Hitzler J: A novel TEL-AML1 fusion transcript involving the proapoptotic gene BCL-G in pediatric precursor B acute lymphoblastic leukemia. Leukemia 2006:20: p 1294. Bartels U, Hawkins C, Ma J, Ray A, Dirks P, Rutka J, Stephens D, Bouffet E: Vascularity and angiogenesis as predictors of growth in optic pathway/hypothalamic gliomas. Journal of NeuroSurgery 2006: 104: pp 314-320. Abla O, Sandlund J, Sung L, Brock P, Corbett R, Kirov I, Griffin T, Blaser S, Weitzman S: A case series of pediatric primary central nervous system lymphoma- favourable outcome without cranial irradiation. Pediatric Blood Cancer 2006: 47: pp 880-885. Bartels U, Shroff M, Sung L, Dag-Ellams U, Laperierre N, Rutka J, Bouffet E: The role of spinal MRI in the follow-up of children treated for medulloblastoma. Cancer 2006: 15: pp 1340-1347. Abla O, Weitzman S: Primary central nervous system lymphoma in children. Neurosurgery Focus 2006: 21: p e8. Abla O, Ye CC: Acute lymphoblastic leukemia with massive myelofibrosis. Journal of Pediatric Hematology Oncology 2006: 28: pp 633-634. Al-Faris N, Al-Harbi T, Boia G, Pappo A, Doyle J, Gassas A: Does consolidation with autologous bone marrow transplantation improve the outcome of children with metastatic or relapsed Ewing’s sarcoma? Pediatric Blood and Cancer 2007: 49: pp 190-195. Anderson D, Ali K, Blanchette V, Brouwers M, Couban S, Radmoor P, Huebsch L, Hume H, McLeod A, Meyer R, Molzan C, Nahirniak S, Nantel S, Pineo G, Rock G: Guidelines on the use of intravenous immune globulin for hematologic conditions. Transfusion Medicine 2007: 2: pp S9-s56. Arnold DM, Julian JA, Walker IR for the Association of Hemophilia Clinic Directors of Canada (AHCDC): Mortality rates and causes of death among all HIV-positive individuals with hemophilia in Canada over twenty-one years of follow-up. Blood 2006: 108: pp 460-464. Barlow JW, Wiley JC, Mous M, Narendran A, Gee MFW, Goldberg M, Sexsmith E, Malkin D: Differentiation of rhabdomyosarcoma cell lines using retinoic acid. Pediatric Blood and Cancer 2006; 47: pp 773-784. Barnes C, Blanchette V, Lillicrap D, Mann K, Stain AM, Leggo J, Hilliard P, Carcao M: Different clinical phenotype in triplets with haemophilia A. Haemophilia 2007: 13: pp 202-205. Barnes C, Lillicrap D, Pazmino-Canizares J, Blanchette VS, Stain AM, Clark D, Hensmen C, Carcao M: Pharmacokinetics of recombinant factor VIII (Kogenate-FS) in children and causes of inter-patient pharmacokinetic variability. Haemophilia 2006: 12: pp 40-49. Barr RD, Barrera M, Greenberg ML: Walking two worlds- Adolescent and Young Adult Oncology. Proceedings of the POGO Conference 2003. Cancer 2006: 107: p 1643. ANNUAL REPORT 2006-07 Baruchel S: Low-Dose Metronomic Chemotherapy: Myth or Truth? Onkologie 2006: 29: pp 305-307. Baruchel S, Diezi M, Hargrave D, Stempak D, Gammon J, Moghrabi A, Coppes MJ, Fernandez CV, Bouffet E: Safety and pharmacokinetics of temozolomide using a dose-escalation, metronomic schedule in recurrent paediatric brain tumours. European Journal of Cancer 2006: 42: pp 2335-2342. Belletrutti M, Ali K, Barnard D, Blanchette V, Chan A, David, M, Luke B, Price V, Ritchie B, Wu J, Canadian Pediatric Chronic ITP Working Group: Canadian Pediatric Thrombosis and Hemostasis Network (CPTHN): Chronic immune thrombocytopenic purpura in children: a survey of the Canadian experience. Journal of Pediatric Hematology/Oncology 2007: 29: pp 95-100. Berntorp E, Abshire T, Carcao M for the von Willebrand Disease Prophylaxis Network: Letter to the Editor: The von Willebrand disease prophylaxis network: exploring a treatment concept. Journal of Thrombosis and Haemostasis 2006: 4: pp 2511-2512. Berntorp E, Shapiro A, Astermark J, Blanchette VS, Collins PW, Dimichele D, Escuriola C, Hay CRM, Hoots WK, Leissinger CA, Negrier C, Oldenburg J, Peerlinck K, Reding MT, Hart C: Inhibitor treatment in haemophilias A and B: Summary statement for the 2006 International Consensus Conference. Haemophilia 2006: 12: pp 1-7. Bertolini F, Baruchel S, Stempak D, Crump M, Taylor D, Zhang L, Man S, Kerbel R, Shaked Y, Foden C, Lee C, Maya R: High dose celecoxib and metronomic ‘low dose’ cyclophosphamide is an effective and safe therapy in patients with relapsed and refractory aggressive histology NHL. Clinical Cancer Research 2006: 12: pp 5190-5198. Bradley CS, Bullinger M, McCusker PJ, Wakefield CD, Blanchette VS, Young NL: Comparing two measures of quality of life for children with haemophilia: the CHO-KLAT and the Haemo-QoL. Haemophilia 2006: 12: pp 643-653. Carcao MD, Aledort LM: Prophylaxis in the haemophilia population – optimizing therapy. Haemophilia 2007: 13: pp 227-232. Drs. Isaac Odame and Melanie Kirby and the Haemoglobinopathy Team HONOURS AND AWARDS 48 DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 Carret AS, Tabori U, Crooks B, Hukin J, Odame I, Johnston DL, Keene DL, Freeman C, Bouffet E: Outcome of secondary high-grade glioma in children previously treated for a malignant condition: A study of the Canadian pediatric brain tumour consortium. International Journal of Radiation Oncology, Biology, Physics 2006: 81: pp 33-38. Evans AJ, Losada OR, Russell RC, Burry TN, Fish JE, Maynard MA, Gervais ML, Sufan RI, Roberts AM, Vandewalle C, Berx G, Marsden PA, Irwin MS, Teh BT, Jewett MAS, Ohh M: VHL promotes ECadherin transactivation via HIF-dependent regulation of SIP1 and snail: implications for renal cell carcinoma. Molecular and Cellular Biology 2007: 27: pp 157-169. Hyder DJ, Sung L, Pollack IF, Gilles FH, Yates AJ, Davis RL, Boyett JM, Finlay JL: Anaplastic mixed gliomas and anaplastic oligodendroglioma in children: results from the CCG 945 experience. Journal of Neurooncology 2007: 83: pp 1-8. Cheretakis C, Leung R, Sun CX, Dror Y, Glogauer M: Timing of neutrophil tissue repopulation predicts restoration of innate immune protection in a murine bone marrow transplant model. Blood 2006: 108: pp 2821-2826. Gassas A, Evans AW, Armstrong C, Doyle JJ: Open wound chronic skin graft-vs.-host disease. Are these wounds ischemic? Pediatric Transplantation 2007: 11: pp 101-104. Keene D, Johnston D, Strother D, Rrossard J, Carret AS, Crooks B, Eisenstat D, Fryer F, Mpofu C, Moghrabi A, Odame I, Zelcer S, Silva S, Wilson B, Samson Y, Hand J, Bouffet E: Epidemiological survey of central nervous system germ cell tumours in Canadian Children. Journal of NeuroOncology 2006: 82: pp 289-295. Cheretakis C, Locker D, Dror Y, Glogauer M: Oral health-related quality of life of children with neutropenia. Special Care in Dentistry 2007: 27: pp 6-11. Chow LML, Nathan PC, Hodgson DC, Jenkin D, Weitzman S, Grant RM, Manson D, Bross A, Doyle JJ, Danjoux C, Greenberg M: Survival and late effects in children with Hodgkin’s lymphoma treated with MOPP/ABV and low-dose extendedfield irradiation. Journal of Clinical Oncology 2006: 24: pp 5735-5741. Das PK, Carcao M, Hitzler J: Use of recombinant factor VIIa in patients with acute leukemia and abnormal coagulation screen prior to lumbar puncture. Pediatric Blood and Cancer 2006: 47: pp 206-209. Das PK, Wherrett D, Dror Y: Remission of severe aplastic anemia induced by treatment of Grave’s disease in a pediatric patient. Pediatric Blood and Cancer 2006: 49: pp 210-212. Dr. Victor Blanchette and the Comprehensive Care Haemophilia Team Dekker A, Bulley S, Beyene J, Dupuis LL, Doyle JJ, Sung L: Meta-analysis of randomized controlled trials of prophylactic granulocyte colonystimulating factor and granulocyte-macrophage colony-stimulating factor after autologus and allongeneic stem cell transplantation. Journal of Clinical Oncology 2006: 24: pp 5207-5215. Diaz RJ, Bouffet E: New frontiers in paediatric medulloblastoma. University of Toronto Medical Journal 2006: 84: pp 26-31. DiPaola J, Aledort L, Britton H, Carcao M, Grabowski E, Hutter J, Journeycake J, Kempton C, Leissinger C: Application of current knowledge to the management of bleeding events during immune tolerance induction. Haemophilia 2006: 12: pp 591-597. Dutta S, Price VE, Blanchette V, Langer JC: A laparoscopic approach to partial splenectomy for children with hereditary spherocytosis. Surgical Endoscopy 2006: 20: pp 1719-1724. Gassas A, Sung L, Saunders EF, Doyle JJ: Comparative outcome of hematopoietic stem cell transplantation for pediatric acute lymphoblastic leukemia following cyclophosphamide and total body irradiation or VP16 and total body irradiation conditioning regimens. Bone Marrow Transplantation 2006: 38: pp 739-743. Gharib MI, Bulley S, Doyle JJ, Wynn RF: Venous occlusive disease in children. Thrombosis Research 2006: 118: pp 27-38. Gong GWK, Barrera M, Beyene J, Bhat S, Carcao M, Lord S, Narayanan UG, Sung L, Young NL, Feldman BM: The Gap Study (GapS) Interview – developing a process to determine the meaning and determinants of quality of life in children with arthritis and rheumatic disease. Clinical and Experimental Rheumatology 2007: 25: pp 149-155. Greenberg ML, Urbach SL: Preserving the fertility in children with cancer. Medical Journal of Australia 2006: 185: pp 532-533. Gupta A, Kumar A, Walters S, Chait P, Irwin MS, Gerstle JT: Analysis of needle versus open biopsy for the diagnosis of advanced stage pediatric neuroblastoma. Pediatric Blood and Cancer 2006: 47: pp 875-879. Gupta AA, Pappo AS: New Drugs for the treatment of metastatic or refractory soft tissue sarcomas in children. Future Oncology 2006: 2: pp 675-685. Hendershot E, Pappo A, Malkin D, Sung L: Tumor necrosis in pediatric osteosarcoma: Impact of modern therapies. Journal of Pediatric Oncology Nursing 2006 23: pp 176-181. Hernandez de Mezerville M, Tellier R, Richardson S, Hebert D, Doyle J, Allen U: Adenoviral infections in pediatric transplant recipients. A hospital-based study. Pediatric Infectious Disease Journal 2006: 25: pp 815-818. Hukin J, Steinbok P, Lafay-Cousin L, Hendson G, Strother D, Mercier C, Samson Y, Howes W, Bouffet E: Intracystic bleomycin therapy for craniopharyngioma in children: the Canadian experience. Cancer 2007: 109: pp 2124-2131. Irwin MS: _Np73-misunderstood protein? Cancer Biology and Therapy 2006: 5: 804-807. Khanani F, Al Ahmari A, Tellier R, Allen U, Doyle J, Richardson S, Gassas A: HHV7 infection in pediatric stem cell transplantation. Pediatric Blood and Cancer 2007: 48: pp 567-570. Klein-Geltink J, Pogany L, Mery LS, Barr RD, Greenberg ML: Impact of age and diagnosis on waiting times between important health care events among children 0 to 19 years cared for in paediatric units: The Canadian Childhood Cancer Surveillance and Control Program. Journal of Pediatric Hematology/Oncology 2006: 28: pp 433-439. Komvilaisak P, Blanchette V: Pharmacokinetic studies of coagulation factors: relevance of plasma and extracellular volume and body weight. Haemophilia 2006: 12: pp 33-39. Komvilaisak P, Connolly B, Naqvi A, Blanchette V: Overview of the use of implantable venus access devices in the management of children with inherited bleeding disorders. Haemophilia 2006: 12: pp 87-93. Lafay-Cousin, Millar BA, Mabbott DJ, Spiegler B, Drake J, Bartels U, Huang A, Bouffet E: Limited field radiation for bifocal germinoma. International Journal of Radiation Oncology, Biology, Physics 2006: 65: pp 486-492. Larouche V, Capra M, Huang A, Bartels U, Bouffet E: Supratentorial primitive neuroectodermal tumors in young children. Journal Clinical Oncology 2006: 24: pp 5609-5610. Lau L, Hansford L, Cheng LS, Hang M, Baruchel S, Kaplan Dr, Irwin MS: Cyclooxygenase inhibitors modulate the p53/HDM2 pathway and enhance chemotherapy-induced apoptosis in neuroblastoma. Oncogene 2007: 26: pp 1920-1931. Levine DS, Navarro OM, Chaudry G, Doyle JJ, Blaser SI: Imagining the complications of bone marrow transplantation in children. RadioGraphics 2007: 27: pp 307-324. Mabbott DJ, Noseworthy M, Bouffet E, Laughlin S, Rockel C: White matter growth as a mechanism of cognitive development in children. Neurolmage 2006: 15: pp 936-946. Mannucci PM, Abshire T, Dimichele D, Santagostino E, Blanchette V: Inhibitor development, immune tolerance and prophylaxis in haemophilia A: The need for an evidence-based approach. Haemophilia 2006: 12: pp 429-434. Mansky P, Arai A, Stratton P, Bernstein D, Long L, Reynolds J, Chen D, Steinberg SM, Lavende N, Hoffman K, Nathan PC, Parks R, Augustine E, Chaudhry U, Derdak J, Wiener L, Gerber L, Mackall C: Treatment late effects in long-term survivors of pediatric sarcoma. Pediatric Blood & Cancer 2007: 48: pp 192-199. Nathan PC, Jovcevska V, Ness KK, D’Agostino NM, Staneland P, Urbach SL, Barron M, Barrera M, Greenberg ML: The prevalence of overweight and obesity in pediatric survivors of cancer. Journal of Pediatrics 2006: 149: pp 518-525. Nathan PC, Whitcomb T, Wolters PL, Steinberg SM, Balis FM, Brouwers P, Hunsberger S, Bleyer WA, Feusner J, Sather H, Miser J, Odom LF, Poplack D, Reaman G: Very high-dose methotrexate (33.6g/m2)) as CNS preventive therapy for childhood acute lymphoblastic leukemia: Results of National Cancer Institute/Children’s Cancer Group trials CCG-191P, CCG-134P and CCG-144P. Leukemia & Lymphoma 2006: 47: pp 2488-2504. Ota S, McLimont M, Carcao MD, Blanchette VS, Graham N, Paradis E, Feldman BM: Definitions for haemophilia prophylaxis and its outcomes: The Canadian consensus study. Haemophilia 2007: 13: pp 12-20. Parshuram C, Dupuis L, To T, Weitzman S, Koren G, Laupacis A: Occurrence and impact of unanticipated variation in intravenous methotrexate dosing. The Annals of Pharmacotherapy 2006: 40: pp 805-811. Pope E, Krafchik BR, Macarthur C, Stempak D, Stephens D, Weinstein M, Ho N, Baruchel S: Oral versus high-dose pulse corticosteriods in problematic infantile hemangiomas: A randomized controlled trial. Pediatrics 2007: 119: pp 1239-1247. Qaddoumi I, Mansour A, Musharbash A, Drake J, Swaidan M, Tihan T, Bouffet E: Impact of telemedicine on pediatric neurooncology in a developing country: The Jordanian-Canadian experience. Pediatric Blood Cancer 2007: 48: pp 39-43. Rachmiel M, Charron M, Gupta A, Hamilton J, Wherrett D, Forte V, Daneman D: Evidence-based review of treatment and follow-up of pediatric patients with differentiated thyroid carcinoma. Journal of Pediatric Endocrinology and Metabolism 2006: 19: pp 1377-1393. 50 DEPARTMENT OF PAEDIATRICS Ramphal R, Pappo AS, Zielenska M, Pereira C, Grant RM, Ngan BY: Pediatric renal cell carcinoma: Clinical, pathological and molecular abnormalities associated with the members of the Mit transcription factor family. American Journal of Clinical Pathology 2006: 126: pp 332-334. Rujkijyanont P, Beyene J, Wei K, Kahn F, Dror Y: Leukaemia-related gene expression in bone marrow cells from patients with the pre-leukaemic disorder Shwachman-Diamond syndrome. British Journal of Haematology 2007: 137: pp 537-544. Sahgal A, Millar B-A, Michaels H, Jaywant S, Chan HSL, Heon E, Gallie BL, Laperriere M: Focal stereotactic external beam radiotherapy as visionsparing modality for treatment of peripapillary and perimacular retinoblastoma: Preliminary results. Clinical Oncology 2006 18: pp 628-634. Schechter T, Finkelstein Y, Doyle J, Verjee Z, Moretti M, Koren G, Dupuis LL: Pharmacokinetic disposition and clinical outcomes in infants and children receiving intravenous busulfan for allogeneic hematopoietic stem cell transplantation. Biology of Blood and Marrow Transplantation 2007: 13: pp 307-314. Schmugge M, Bang KWA, Blanchette VS, Albisetti M, Connolly BL, Freedman J, Rand ML: Von Willebrand factor binding to platelets and platelet activation in newborn infants with central venous lines. Acta Haematology 2006: 117: pp 145-148. Seely D, Stempak D, Baruchel S: A strategy for controlling potential interaction between natural health products and chemotherapy: A review in pediatric oncology. Journal of Pediatric Hematology Oncology 2007: 29: pp 32-47. Spiegler B, Kennedy K, Maze R, Greenberg ML, Weitzman S, Hitzler JK, Nathan PC: Comparison of long-term neurocognitive outcomes in young children with ALL treated with cranial radiation or high-dose or very high-dose intravenous methotrexate. Journal of Clinical Oncology 2006: 24: pp 3858-3864. Steele JM, Sung L, Klaassen R, Fernandez CV, Yanofsky R, Wu J, Odame I, Silva M., Champagne J, Ali, K, Brossard J, Samson Y, Abish S, Le D, Jardine L, Hand JP, Lipton JH, Charpentier K, Stephens D, Freedman M, Dror Y: Disease progression in recently diagnosed patients with inherited marrow failure syndromes: A Canadian Inherited Marrow Failure Registry (CIMFR) Report. Pediatric Blood and Cancer 2006: 47: pp 918-925. Stempak D, Gammon J, Halton J, Moghrabi A, Koren G, Baruchel S: A pilot pharmacokinetic and antiangiogenic biomarker study of celecoxib and low-dose metronomic vinblastine or cyclophosphamide in pediatric recurrent solid tumors. Journal of Pediatrics Hematology Oncology 2006: 28: pp 720-728. ANNUAL REPORT 2006-07 Stempak D, Seely D, Baruchel S: Metronomic dosing of chemotherapy: applications in pediatric oncology. Cancer Investigation 2006: 24: pp 432-443. Stevens B, McKeever P, Law MP, Booth M, Greenberg ML, Daub S, Gafni A, Gammon J, Yamada J, Epstein I: Children receiving chemotherapy at home: Perceptions of children and parents. Journal of Pediatric Oncology Nursing 2006: 23: pp 276-285. Strahm B, Malkin D: Hereditary cancer predisposition in children: molecular basis and clinical implications. International Journal of Cancer 2006: 119: pp 2001-2006. Sun Y, Boyd K, Xu W, Ma J, Jackson CW, Fu A, Shillingford JM, Robinson GW, Henninghausen L, Hitzler JK, Ma Z, Morris SW: Acute myeloid leukemia-associated Mkl1 (Mrtf-a) is a key regulator of mammary gland function. Molecular Cell Biology 2006: 26: pp 5809-5826. Sung L, Dror Y: Clinical applications of granulocyte-colony stimulating factor. Frontiers in Bioscience 2007: 12: pp 1988-2002. Sung L, Johnston D: Approach to febrile neutropenia in the general paediatric setting. Paediatric and Child Health 2007: 11: pp 19-21. Sung L, Tomlinson GA, Greenberg ML, Koren G, Judd P, Ota S, Feldman BM: Serial controlled N-of-1 trials of topical vitamin E as prophylaxis for chemotherapy-induced oral mucositis in paediatric patients. European Journal of Cancer 2007: 43: pp 1269-1275. Sung L, Tomlinson GA, Greenberg M, Koren G, Judd P, Ota S, Feldman BM: Validation of the oral mucositis assessment scale in paediatric cancer. Pediatric Blood & Cancer 2007: 49: pp 149-153. Tabori U, Dome J: Telomere biology of pediatric cancer. Cancer Investigation 2007: 25: pp 197-208. Tabori U, Jones H, Malkin D: Low risk of complications in severely neutropenic children with cancer at an overnight recreational summer camp. Pediatric Blood and Cancer 2007: 48: pp 148-151. Tabori U, Nanda S, Lees J, Malkin D: Younger age of cancer initiation is associated with shorter telomere length in Li- Fraumeni syndrome. Cancer Research 2007: 67: pp 1415-1418. Tabori U, Revach G, Nathan P, Strahm B, Rachlis A, Shago A, Grant R, Doyle J, Malkin D: Toxicity and outcome of children with treatment related acute myeloid leukemia. Pediatric Blood and Cancer January 24, 2007 [Epub ahead of print]. Dr. Oussama Abla Mabbott DJ, Noseworthy D, Bouffet E, Rockel C, Laughlin S: White matter and IQ after radiation for paediatric medulloblastoma: a diffusion tensor imaging study. Neuro-Oncology 2006: 8: pp 244-252. Tomlinson D, Capra M, Gammon J, Volpe J, Barrera M, Hinds PS, Bouffet E, Greenberg ML, Baruchel S, Llewellyn-Thomas HA, Sung L: Parental decision making in pediatric cancer endof-life care: using focus group methodology as a pre-phase to seek participant design input. European Journal of Oncology Nursing 2006: 10: pp 198-206. Valentino LA, Santagostino E, Blanchette V, van den Berg M: Managing the pediatric patient and the adolescent/adult transition. Seminars in Thrombosis and Hemostasis 2006: 32: pp 28-31. van den Berg HM, Dunn A, Fischer K, Blanchette VS: Prevention and treatment of musculoskeletal disease in the haemophilia population: Role of prophylaxis and synovectomy. Haemophilia 2006: 12: pp 159-168. Watson IR, Blanch A, Lin DCC, Ohh M, Irwin MS: MDM-2 mediated Nedd 8 modification of TA p73 regulates its transactivation function. Journal of Biological Chemistry 2006: 281: pp 34096-34103. Watson IR, Irwin MS: Regulation of p53 family proteins by ubiquitin and ubiquitin-like molecules. Neoplasia 2006: 8: pp 655-666. West AN, Neale GA, Pounds S, Figueredo BC, Galindo CR, Pianovski MAD, Filho AGO, Malkin D, Lalli E, Ribeiro R, Zambetti GP: Gene expression profiling of childhood adrenocortical tumors. Cancer Research 2007: 67: pp 600-608. Young NL, Bradley CS, Wakefield CD, Barnard D, Blanchette VS, McCusker PJ: How well does the Canadian Haemophilia Outcomes-Kids’ Life Assessment Tool (CHO-KLAT) measure the quality of life of boys with haemophilia? Pediatric Blood and Cancer 2006: 47: pp 305-311. Young NL, Wakefield CD, Bradley CS, Blanchette VS, Barnard D, McCusker P: Returning the focus to the quality of life of boys with haemophilia. Pediatric Blood and Cancer 2007: March 2, 2007 [Epub ahead of print]. Zhang L, Yeger H, Das B, Irwin MS, Baruchel S: Tissue microenvironment modulates CXCR4 expression and tumor metastasis in neuroblastoma. Neoplasia 2007: 9: pp 36-46. Zukotynski K, Jarrin J, Babyn P, Carcao M, Pazmino-Canizares J, Stain AM, Doria AS: Sonography for assessment of haemophilic arthropathy in children: A systematic protocol. Haemophilia 2007: 13: pp 293-304. BOOKS AND BOOK CHAPTERS Bouffet E, Hodgson DC, Koh ES: Pediatric cancers. In: Prognostic Factors in Cancer Third Edition (Gospodarowicz MK, O’Sullivan B, Sobin LH, eds). John Wiley & Sons Publishing, New Jersey, 2006. Irwin MS, Malkin D: Li Fraumeni Syndrome and the p53 tumour suppressor pathway. In: Russell and Rubenstein’s Pathology of Tumours of the Nervous System (McLendon RF, Rosenblum M & Bigner DD, eds). Hodder and Stoughton Ltd., London, 2006. Acute Megakaryocytic Leukemia in Down syndrome. Hitzler J. National Cancer Institute of Canada ($137,484 2005-2009) Biochemical and genetic analyses of JPO2, a novel of c-Myc oncogene interacting protein. Huang A. National Cancer Institute of Canada ($440,082 2006-2009) Biochemical and genetic analyses of JPO2, a novel c-Myc oncogene interacting protein (Equipment). Huang A. National Cancer Institute of Canada ($29,479 2006-2007) Jouvet A, Fauchon F, Bouffet E, Saint-Pierre G, Champier J, Montange MF: Tumors of pineal parenchymal and glial cells. In: Russell & Rubinstein’s Pathology of Tumors of the Nervous System (McLendon RF, Rosenblum M & Bigner DD, eds). Hodder and Stoughton Limited, London, 2006. Bridging the paediatric and adult health care environments: Promoting successful transitions for survivors of paediatric cancer. Greenberg M, D’Agostino N, Guger S, Staneland P, Lough M, Laperriere N, Urbach S, Nathan P. Princess Margaret Hospital Psychosocial Oncology and Palliative Care Grant ($5,986 2007) Khetan V, Chan HSL, Wang L, Gallie BL: Retinoblastoma. In: Prognostic Factors in Cancer, Third Edition (Gospodarowicz MK, O’Sullivan B, Sobin LH, eds). John Wiley & Sons Inc., New Jersey, 2006. Canadian Centre for Applied Cancer Genetics. Malkin D, Weksberg R, Meyn S, Ray P, Becker L, Rutka J, Yeger H, Zielenska M, Alman B. Canada Foundation for Innovation 40%, OIT 40%, and Ballard Foundation 20% of total ($1,811,000 2002-2007; additional $216,000 operating funds through OIF 2005-2007) Ronghe M, Bouffet E: Diffuse intrinsic pontine gliomas 28. In: Neuro-Oncology of CNS Tumours. (Tonn JC, Grossman S, Rutka J, Westphal M, eds). Springer Verlag, New York, 2006. Valenzuela A, Chan HSL, Heon E, Gallie BL: A language for retinoblastoma: Guidelines through standard operating procedures. In: Pediatric Retina. (Reynold J, ed). Taylor and Francis, New York, 2006. FUNDING Cancer and chemosensitivity. Irwin MS. Premier’s Research Excellence Award ($150,000 2004-2009) Careful transitions: developing and integrated service delivery model for paediatric palliative care. Antle B, Newman C, Beaune L, Rugg M, Wheat A, Bouffet E, Laxer R, Frager G, Wagner F. SickKids Foundation ($129,900 2006-2008) A cross-sectional study of overweight in paediatric survivors of acute lymphoblastic leukemia. Nathan P, Barrera M, Barron M, Urbach S, D’Agostino N, Staneland P, Charron M, Greenberg M. Faculty of Medicine Dean’s Fund ($10,000 2007) Celecoxib enhances chemosensitivity of neuroblastoma: Molecular pathways and therapeutic applications. Irwin MS. James Birrell Neuroblastoma Research Fund ($29,700 2004-2007) A group wide phase II study of ET-743 in recurrent paediatric sarcoma. Baruchel S. Children’s Oncology Group ($75,000 2007-2010) Characterization of a novel family of Myc protein interactors and their role in medulloblastoma transformation. Huang A. Eli-Lilly/Cancer Care Ontario/Canadian Institutes of Health Research ($675,000 2004-2007) A phase I study of Sorafenib in recurrent solid tumors. Reference laboratory. Wideman B, Baruchel S. Children’s Oncology Group ($32,000 2007-2008) A phase II study of vinblastine in chemotherapy naive children with progessive low grade gliomas. Bouffet E, Tabori U, Hawkins C, Keene D. Ontario Institute of Cancer Research ($430,197 2007-2010) A randomized study of IVIG vs IVIG with high dose methylprednisolone in rapidly augmenting platelet counts in childhood ITP. Carcao M, Blanchette V. Bayer (Canada) Incorporated ($145,065 2004-2006) Characterization of JPO2, a novel c-Myc cotransforming protein. Rodrigues T and Huang A. Canadian Institute of Health Research Doctoral Research Awards ($63, 000 2007-2010) Children with posterior fossa brain tumours: quality of life and predictors of outcome. Kulkarni A, Bouffet E, Pakulis J, Ellerton C, Shams I. b.r.a.i.n.child, The Hospital for Sick Children ($42,685 2006-2008) Clinical Trial Consortium. Pappo A, Bouffet E, Baruchel S. Ontario Cancer Research Network ($1,000,000 2005-2008) Comparative effectiveness of conventional and informatics-assisted clinical management and clinical trial participation. Gallie BL, Chan HSL, Eysenbach G, Kushniruk A, Heon E. Ontario Institute for Cancer Research ($446,066 20032006 with 2-year no cost extension 2007-2008) Cross-cultural validation of the CHO-KLAT (Canadian Haemophilia Outcomes – Kids’ Life Assessment Tool). Blanchette V, Young N, McCusker P. Bayer HealthCare Canada ($260,000 2006-2008) Defining the clinical phenotype of inherited marrow failure syndromes and the risk factors of severe haematological complications and cancer by the Canadian Inherited Marrow Failure Registry (CIMFR). Dror Y. Council of Canadian Paediatric Haematology/Oncology Directors Research Network ($83,000 2005-2007) Determining the potential for drug interactions between Natural Health Products (NHPs) and chemotherapy in a paediatric onocology population. Baruchel S, Mills E, Guyatt G, Koren G, Seely D, Stempak D, Zelunka E. The Hospital for Sick Children ($130,000 2005-2006) Development and validation of comparative genomic hybridization arrays for clinical use in cancer. Horsman D, Law W, Huang A, Squire J, Rutka J. Genome Canada ($4,600,000 2004-2007) Development of a feasible tool to measure oral mucositis in children with cancer. Sung L, Judd P, Tomlinson D, Doyle JJ, Hendershot EI, Maloney AM, Feldman BM. Pediatric Oncology Group of Ontario ($13,334 2007-2008) Educational Grant to review 1) usage patterns of rFVIIa; 2) impact of acquired coagulopathies in leukaemia patients; and 3) inhibitor development in mild haemophilia all at The Hospital for Sick Children. Carcao MD. Novo Nordisk Inc. ($34,750 2006-2007) Establishing surrogate markers of angiogenesis in paediatric oncology phase I and II clinical trials: A Children’s Oncology Group Study. Baruchel S, Stempak D, Bertolini F, Entz-Werle N. Alex’s Lemonade Stand Foundation ($198,417 2007-2009) Functional analysis of p73: Roles in chemosensitivity and apoptosis. Irwin MS. National Cancer Institute of Canada ($479,941 2003-2007) Functional analysis of the p53 family members p73 and p63 roles in apoptosis, development and cancer. Irwin MS. Canada Research Chair; Tier II ($500,000 2003-2008) Genetic epidemiology of osteosarcoma. Spector L, Malkin D, Ross, J, Sklar C, Nagarajan R. National Institutes of Health ($1,000,000 2006-2011) Genetic variation and risk of infection in children with primary acute myeloid leukaemia. Sung, L. Early Research Award – Ministry of Research and Innovation ($100,000 2007-2011) Infrastructure Grant Paediatric Consortium. Blanchette VS, Devine S (Co-PI), Site PI’s: Weitzman S, Portwine C, Halton J, Jardine L, Silva M. Ontario Cancer Research Network (OCRN) ($990,404 2006-2011) Genetics, genomic research and consent: attitudes of paediatric cancer survivors. Patenaude A, Malkin D. National Institutes of Health ($100,000 2006-2008) International Prophylaxis Study Group. Blanchette VS. Bayer, Baxter BioScience, Aventis and Wyeth/GI ($750,000 2002-2006) Genomic analyses of paediatric supratentorial primitive neuroectodermal tumours. Huang A. Children’s Brain Tumour Foundation ($150,000 2005-2007) In vivo anti-tumour and anti-metastatic activity of administration of CXCR4 antagonist and antiCTLA4 in neuroblastoma. Baruchel S. Pfizer Canada Inc. ($45,000 2007-2008) Genomic analyses of paediatric supratentorial primitive neuroectodermal tumours. Huang A, Li M, Rutka J. American Brain Tumour Association Basic Science Research Award ($70,000 2006-2008) In vivio anti-tumour and anti-metastatic activity of administration of Sutent and low-dose chemotherapy in preclinical neuroblastoma mouse model. Baruchel S., Zhang L. Pfizer Canada Inc. ($50,000 2007-2008) Genomic analyses of sPNET. Huang A. b.r.a.i.n.child, The Hospital for Sick Children ($84,300 2005-2007) Measurement properties of ultrasound for assessment of haemophilic arthropathy. MRI correlation. Doria, A, Srivastava A, Gibikote S, Keshava SN, Poonnoose P, Blanchette V, Babyn P, Hilliard, P. Bayer HealthCare Canada ($170,000 2006-2007) Group Chair Grant, Children’s Oncology Group. Institutional Principal Investigator: Malkin D. National Cancer Institute (US$900,000 2003-2008) Hope: The complex journey of parents of children with cancer. Antle B, Barrera M, Beaune L, Capra M, D’Agostino N, Maloney AM, Nicholas D, Volpe J. Canadian Institutes of Health Research ($291,067 2005-2008) and b.r.a.i.n.child, The Hospital for Sick Children ($25,000 2005-2008) Identification of genes involved in marrow failure and malignant myeloid transformation by gene expression analysis of bone marrows from patients with inherited marrow failure syndromes. Dror Y, Beyene J. Anemia Institute of Research and Education ($60,000 2004-2006) Identification of genetic pathways that regulate survival and development of cancer and cancer stem cells. Guidos C, Hitzler J. Genome Canada ($330,000 2005-2009) Identification of interstitial germline deletions in children with complex clinical syndromes that include medulloblastoma. Taylor M, Bouffet E. Pediatric Brain Tumour Foundation Institute Award ($100,000 2006-2008) Improving communication during the palliative care of children with brain tumours – learning from experience. Bouffet E, Chau T, Beal D, Van Halderen C, Brownstone D, Eaton C. b.r.a.i.n.child, The Hospital for Sick Children ($49,592 2005-2007) Improving the clinical management of paediatric severe haemophilia A patients via laboratory assessments of haemostasis. Kahr WHA, Blanchette V. The Bayer-Canadian Blood Services Partnership Fund ($148,420 2005-2007) Mechanism of pre-B cell leukemogenesis. Subversion of developmental and survival programs in leukenogenesis. Guidos C, Hitzler J. Specialized Center of Research; Leukemia Lymphoma Society ($750,000 2004-2009) Moderate term musculoskeletal outcomes with escalating dose prophylaxis: The Canadian Haemophilia Prophylaxis Follow-Up Study (CHPS-3). Feldman BM, Blanchette VS. Bayer (Canada) Incorporated ($742,500 2006-2009) Molecular and pharmacologic correlates of acute myeloid leukemia in Down syndrome. Taub J, Hitzler J. National Institutes of Health ($2,189,783 2007-2011) Molecular basis of CNS leukemia. Subversion of developmental and survival programs in leukemogenesis. Danska J, Hitzler J. Specialized Center of Research; Leukemia Lymphoma Society ($750,000 2004-2009) Molecular characterization of novel genes involved in sPNET pathogenesis. Lee K and Huang A. The Hospital for Sick Children Research Training Competition Award ($19,000 2006-2007) Molecular determinants of tumour formation in Li-Fraumeni Syndrome. Malkin D. National Cancer Institute of Canada ($387,000 2004-2007) Molecular determinants of tumour formation in Li-Fraumeni Syndrome. Malkin D. National Cancer Institute of Canada ($423,000 2007-2010) Neuroimaging and cognitive function in children with brain tumours. Mabbott D, Bouffet E, Noseworthy M, Laughlin S, Hyder D. SickKids Foundation ($122,395 2003-2006) DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 53 New surrogate markers of angiogenesis in paediatric brain tumours. Baruchel S, Bartels U, Hawkins C, Stempak D, Bouffet E, Abdelhaleem M, Doyle J. b.r.a.i.n.child, The Hospital for Sick Children ($97,446 2006-2008) Orphan late effect in paediatric brain tumour survivors. Lafay-Cousin L, Sockett E, Bouffet E. b.r.a.i.n.child, The Hospital for Sick Children ($30,000 2005-2007) Paediatric Phase I Consortium. Baruchel S, Pappo A, Gammon J. National Cancer Institute/Children’s Oncology Group ($250,000 2002-2007) Pain in child health: an innovative, transdisciplinary, cross-Canada research training consortium. McGrath PJ, Barr R, Baruchel S, Brown S, Camfield C, Chambers C, Connolly J, Craig K, Dube L, Feldman B, Finley G, Frager G, Gibbins S, Grunau R, Harman K, Hetherington C, Howlett A, Johnston C, Kenny N, Koren G, Langer J, Murray M, Oberlander T, Ohlsson A, Otley A, Rathwell T, Salter M, Schellinck H, Stevens B, Taddio A, Unruh A, Von Baeyer C, Walker C. Canadian Institutes of Health Research ($1,800,000 2002-2008) Palliative chemotherapy versus supportive care alone for end-of-life. Sung L, Hinds P, Llewwllyn-Thomas H, Volpe J, Capra M, Gammon J, Baruchel S, Bouffet E, Barrera M, Tomlinson D, Greenberg ML. National Cancer Institute of Canada ($131,626 2005-2008) Parents of children with cancer. Klaassen A, Sung L, Dix D, Pritchard S, Yanofsky R. National Cancer Institute of Canada ($225,000 2004-2006) Phase II study of high dose chemotherapy with cyclosporin for vision-threatening intraocular retinoblastoma. Chan HSL, Gallie BL, Heon E. Ontario Institute for Cancer Research ($551,723 2003-2006 with 2-year no cost extension 2007-2008) Pilot study of combination therapy of temozolomide and radiation therapy in brainstem glioma. Barucel S, Bouffet E, Tariq N, Hargrave D. b.r.a.i.n.child, The Hospital for Sick Children ($44,980 2001-2007) 54 DEPARTMENT OF PAEDIATRICS Prospective cohort study of genetic variation and risk of infection for Canadian children with newly diagnosed acute myeloid leukemia. Sung L, Beyene J, Allen U. Childhood Cancer Foundation ($17,000 2005-2006) The prevalence of symptomatic von Willebrand disease in the paediatric population. James PD, Blanchette VS, Lillicrap D, Silva M, Rand ML, Brandao L. Canadian Hemophilia Society ($139,800 2007-2009) Prospective cohort study of genetic variation and risk of infection in Canadian children with newly diagnosed acute myeloid leukemia. Sung L, Beyene J, Allen U. National Cancer Institute of Canada ($444,424 2005-2008) The role of SBDS in granulopoiesis. Dror Y. SickKids Foundation ($64,480 2006-2008) Role of p53 and VEGF signaling in paediatric sarcomas. Malkin D. SickKids Foundation ($200,000 2002-2007) Salary support for chairing a group-wide prospective study of ototoxicity in children receiving cisplatin chemotherapy. Sung L. Children’s Oncology Group ($50,000 2005-2007) Sibling and parent bereavement from childhood cancer. Gerhardt C, Barrera M, Capra M. National Cancer Institute of Canada, The Hospital for Sick Children ($92,000 2005-2010) Subversion of developmental and survival programs in leukemogenesis. Project 1: identification of leukemogenic genes that convert a normal human hematopoietic cell into a leukemic stem cell. Mak T, Dick J, Hitzler J. Leukemia Lymphoma Society ($748,575 2004-2009) Subversion of developmental and survival programs in leukemogenesis. Project 2: mechanism of pre B cell leukemogenesis. Guidos C, Hitzler J. Leukemia Lymphoma Society ($750,000 2004-2009) Subversion of developmental and survival programs in leukemogenesis. Project 3: Molecular basis of CNS leukemia. Danska J, Hitzler J. Leukemia Lymphoma Society ($750,000 2004-2009) Telomere maintenance in the biologic behaviour of low-grade gliomas. Malkin D, Tabori U. b.r.a.i.n.child, The Hospital for Sick Children ($60,000 2004-2007) Thalassemia Clinical Research Network Registry. Kirby M. National Institutes of Health ($45,696 2005-2010) The MAST project: Magnesium for Sickle Cell Trial. Goldman RD, Mounstephen W, Kirby-Allen M, Friedman JN. Physicians’ Services Incorporated Foundation ($163,846 2005-2007) The Ontario Pediatric Oncology Clinical Trials Research Network. Blanchette V, Malkin D, Devine S, Baruchel S, Weitzman, S, Bouffet E. Ontario Cancer Research Network ($1.2 million 2006-2009) ANNUAL REPORT 2006-07 The role of SBDS in promoting cell survival. Dror Y. Canadian Institutes of Health Research ($250,000 2006-2011) The Role of SBDS in promoting cell survival. Dror Y. SDS Project Incorporated ($30,000 2006-2007) The role of SBDS in protection from apoptosis. Watanabe K, Dror Y. The Hospital for Sick Children Research Training Competition ($105,000 2004-2007) The role of VPS33B in platelet alpha granule formation. Kahr WHA. Dean’s Fund New Staff Grant ($10,000 2006) The role of VPS33B in platelet alpha granule formation. Kahr WHA. The Heart and Stroke Foundation of Ontario ($210,750 2006-2009) The role of VPS33B in platelet alpha granule formation. Kahr WHA. Canadian Institutes of Health Research Canada ($441,912 2006-2010) To improve infrastructure for paediatric cancer clinical trials. Pappo A, Malkin D, Devine S, Hitzler H, Silva M, Barr R, Halton J, Jardine L, Baruchel S, Bouffet E. Ontario Cancer Research Network ($990,404 2005-2010) Toronto Angiogenesis Research Centre. Kerbel R, Baruchel S, Burns P, Dumont D, Hemkelman M. Canada Foundation for Innovation 40%, OIT 40%, and partner institutions 20% of total award ($11,630,155 2002-2006) Treatment choices for children with typical acute immune thrombocytopenic purpura: development of a decision aid. Beck C, Parkin P, Birken C, Blanchette V, Boydell K, Llewellyn-Thomas H. Dean’s Fund New Staff Competition ($10,000 2006-2011) Tumour volume as measurement of response to chemotherapy in paediatric low grade gliomas. Bartels U, Laughlin S, Mabbott D, Bouffet E. b.r.a.i.n.child, The Hospital for Sick Children ($13,775 2006) Validation of a new paediatric scoring system from the International Hemophilia Prophylaxis Study Group: Validity and reliability of the combined hemophilia joint health score (HJHS). Feldman B, Blanchette VS, Funk S, Bergstrom BM, Zourikian, Hilliard P, van der Net JJ, Engelbert R, Petrini P, van den Berg HM, Manco-Johnson M, Rivard G. Bayer Healthcare LLC, Biological Products Division ($195,000 2005-2007) Dr. Uri Tabori and lab staff Neuro-imaging of white matter and neuro-cognitive outcome following cranial radiation for paediatric brain tumours. Mabbot DJ, Bouffet E, Noseworthy M, Suzanne M, Kulkarni A, Norman L, Michael S, Montour-Proulx I, Daniel K, Strother D, Witol A, Wilson B, Chafe S, McConnel D, Hukin J, Freyer C. C17 Research Network ($95,000 2006-2008) Division Head Dr. Chaim Roifman Full-Time Dr. Adelle Atkinson Dr. Eyal Grunebaum Part-Time Dr. Milton Gold Dr. David Hummel Dr. Sasson Lavi Dr. Maria Triassi Clinical Fellows Dr. Maryam Al-Helale Dr. Daifulah Alzahrani Dr. Maitham Hussain Dr. Fotini Kavadas Dr. YewHon Lai Dr. Elana Lavine Dr. Raz Somech Clinical Assistant Dr. Sean Bulley Immunology Nurse Brenda Reid, CNS Allergy Nurses Kimberley Seaban, CNS Elisabeth White, CNS Senior Scientists Amos Cohen, PhD Sergio Grinstein Research Associate Nigel Sharfe, PhD Research Technologists Lorand Cimpeon Tom Grunberger Yiping Gu Vivien Lee Weixian Min Murugesapillai Mylvaganam JunYan Zhang Medical Lab Technologists Andrea Bates Harjit Dadi, PhD Administrative Staff Lucy Casamatta Sandra Mendonca Linda Quintal Immunology/Allergy DIVISIONAL OVERVIEW The Division of Immunology/Allergy continues as a leader in patient care, research and education in immunology. This year marks the launch of the Canadian Jeffrey Modell Foundation (JMF) Immunodeficiency Network and the Primary Immunodeficiency (PID) Network in Israel. Under Dr. Roifman’s leadership, these national programs are facilitating early recognition, diagnosis, research and effective treatment of primary immune deficiencies. Upcoming collaborations with the Canadian Jeffrey Modell Network members truly illustrate our vision of providing timely and optimal care to patients with PID worldwide. Dr. Adelle Atkinson The Primary Immunodeficiency program at The Hospital for Sick Children is the largest centre of its kind in Canada. Medical services are provided to over 2000 outpatients referred from physicians and hospitals nationally. This number has steadily increased by thirty percent over the last 10 years. To diminish waiting time in the Immunology clinic we have recently established the Complex Immunodeficiency Clinic. This addition has improved evaluation of our complex patients by providing extensive time and attention to their conditions. The Hypogammaglobulinemia Program at SickKids is recognized globally for its innovative approach and long-term follow-up of over 150 patients. We transformed the therapy of these patients from multiple hospital based admissions, to visiting the short stay unit and subsequently receiving treatment in their community. The proficiency of our team coupled with convenient care at a medical centre close to home, provides our patients with the personalized treatment they require. To further enhance this program, we are now implementing home therapy as the ideal model of treatment. Our recent symposium for a Canadian consensus on home treatment has resulted in funding by the Canadian Immunodeficiency Society (CI Society) and a publication in the Journal of Clinical Immunology. Innovative protocols created by members of the division have elevated the profile of the Bone Marrow Transplantation (BMT) program as one of the best in the world for survival of PID patients. In 1990 we made history with implementing matched unrelated donor transplants for PID. Today we demonstrate the impact of our procedures with a recently published study in JAMA. This analysis illustrates an 80% survival rate for SickKids patients utilizing our protocol. In contrast only a 50% survival rate is achieved by a widely used standard protocol exercised in most other countries in Europe and the US. Our protocol has become the preferred modality for treatment of severe combined immunodeficiency when full HLA matched related donor is not available. Our scientific and clinical achievements lead to much recognition. This includes membership by Dr. Roifman in the World Health Organization Scientific Committee for Clinical Immunology, which defines the classification of PIDs. Similarly we contributed to the Manual of Clinical Laboratory Immunology detailing the process of diagnosing a primary immunodeficiency. Research remains a major focus of activity in the division. Dr. Roifman’s laboratory continues to innovate anti-cancer treatments. Novel compounds were designed against targets in solid tumours which have lead to significant tumor shrinkage in vitro and in vivo. Moreover, since Dr. Roifman’s lab synthesized CR4, another novel compound capable of killing acute lymphocytic leukaemia (ALL) and acute myeloid leukaemia (Blood 2003), it is currently being given to patients with ALL in a phase one clinical study conducted by LymphoSign. The role of Eph receptors in the immune system is gaining recognition with our recent publication in the Journal of Immunology. Meanwhile the discovery of a genetic aberration in the RNA component of RMRP has initiated testing of patients for this defect (JACI 2006). DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 57 Dr. Grunebaum and his group have successfully developed and described a novel method to efficiently deliver full length enzymes such as Purine Nucleoside Phosphorylase (PNP) into cells. (J Clin Invest, 2006). To further this endeavour his laboratory continues to develop safer methods of inserting genes into bone marrow cells. This will facilitate therapy for diseases such as PNP and Adenosine Deaminase (ADA) deficiency, while improving the substandard outcomes for these patients. In view of his academic achievements and his multiple contributions to clinical immunology in Canada the CI Society created the Chaim Roifman Scholar Award. Launching in 2007, the recipients of this research grant will study the clinical, cellular and molecular characteristics of genetically determined immunodeficiency diseases, discover improved diagnostic tools for PID, research novel therapeutic approaches for PID or advance education of patients and medical professionals on PIDs. Education remains an important activity of every member of the division. The tireless dedication of our staff to teaching has recently been fully recognized by the full approval of reviewers from the Royal College of Physicians and Surgeons. Beyond this program we continue to attract more than 25 outstanding candidates every year from across the world. International trainees have the opportunity to participate in a flexible program which can be predominantly research oriented. Our commitment to education goes beyond the training program. Dr. Roifman organized and chaired the JMF anniversary scientific program and together with Dr. Grunebaum organized the CI Society/Canadian Society of Allergy and Clinical Immunology conference in Edmonton. In addition Dr. Adelle Atkinson was granted the CI Society physician’s awareness award for a third consecutive year. Similarly Brenda Reid was presented with a nursing education grant by the CI Society for her leadership in the teaching of PID care to nurses globally. From the common cold to cancer, the clinical applications and research potential of immunology converge with almost every aspect of patient care. Thanks to the support of The Donald & Audrey Campbell Chair, the Jeffrey Modell Foundation, the CI Society, Canadian Institute of Health Research, March of Dimes, and the Canadian Centre for Primary Immunodeficiency, children in our division will receive the best care possible, as soon as possible. HONOURS AND AWARDS Atkinson A: Physicians Awareness Initiative, Canadian Immunodeficiency Society, 2006. Reid B: Nurses Awareness Initiative, Canadian Immunodeficiency Society, 2006. Roifman CM: Schwartz-Reisman Israel Scholar, 2007. PUBLICATIONS Alexander RT, Grinstein S: Na+/H+ exchangers and the regulation of volume. Acta Physiologica 2006: 187: pp 159-167. Alexander RT, Malevanets A, Durkan AM, Kocinsky HM, Aronson PS, Orlowski J, Grinstein S: Membrane curvature alters the activation kinetics of the epithelial Na+/H+ exchanger, NHE3. The Journal of Biological Chemistry 2007: 282: pp 7376-7384. 58 DEPARTMENT OF PAEDIATRICS AL-Jenaidi F,Outi M’ekitie O, Grunebaum E, Sochett E: Parathyroid gland dysfunction in 22q11.2 deletion syndrome. Hormone Research 2007: 67: pp 117-122. AL-Zahrani, Grunebaum E, Roifman CM: Safety and efficacy of measles-mumps-rubella vaccine in patients with DiGeorge/22q11 microdeletion syndrome. Pediatric Academic Society 2007: 61: p e6293.14 Aoki CA, Roifman CM, Lian ZX, Bowlus CL, Norman GL, Shoenfeld Y, Mackay IR, Gershwin E: IL-2 receptor alpha deficiency and features of primary biliary cirrhosis. Journal of Autoimmunity 2006: 27: pp 50-53. ANNUAL REPORT 2006-07 Atkinson A, Zhang J, Quintal L, Williams B, Grunebaum E, Roifman CM: Novel RAG1 mutation in a case of severe combined immunodeficiency. Allergy, Asthma and Clinical Immunology 2006: 2: p 164. Candotti F, Roifman CM, Puck JM: Injecting some safety into SCID gene therapy. Gene Therapy 2006: 13: pp 741-743. Carcao M, St Louis J, Poon MC, Grunebaum E, Lacroix S, Stain AM, Blanchette VS, Rivard GE: Rituximab for congenital haemophiliacs with inhibitors: a Canadian experience. Haemophilia 2006: 12: pp 7-18. Corbett-Nelson EF, Marshall JG, Grinstein S: Signalling-dependent immobilization of acylated proteins in the inner monolayer of the plasma membrane. The Journal of Cell Biology 2006: 174: pp 255-265. Freywald A, Sharfe N, Miller CDE, Rashotte C, Roifman CM: EphA receptors inhibit anti CD3 induced apoptosis in thymocytes. The Journal of Immunology 2006: 176: pp 4066-4074. Grunebaum E, Atkinson A, Reid B, Roifman CM: Bone marrow transplantation from unrelated donors for severe combined immune deficiency. Allergy, Asthma and Clinical Immunology 2006: 2: p 164. Husain M, Grunebaum E, Naqvi A, Atkinson A, Ngan BY, Aiuti A, Roifman CM: Burkitt lymphoma in adenosine deaminase deficient-severe combined immunodeficiency treated with polyethylene glycol-adenosine deaminase. Journal of Pediatrics 151: pp 93-95 Huynh KK, Eskelinen EL, Scott CC, Malevanets A, Saftig P, Grinstein S: LAMP proteins are required for fusion of lysosomes with phagosomes. The EMBO Journal 2007: 26: pp 313-324. Grunebaum E, Atkinson A, Reid B, Roifman CM: Bone marrow transplantation from matched unrelated donors for patients with severe combined immune deficiency. Biology of Blood and Marrow Transplantation 2006: 12 S1: p 122. Kavadas F, Somech R, Pollanen M, Roifman CM: Hemophagocytic lymphocytosis in a patient with T-B+NK severe combined immunodeficiency. Pediatric Academic Society 2007: 61: p e6286.9. Grunebaum E, Mazzolari E, Porta F, Dallera D, Atkinson A, Reid B, Notarangelo LD, Roifman CM: Bone marrow transplant for severe combined immune deficiency. JAMA 2006: 295: pp 508-518. Lai YH, Gruneabum E, Atkinson A, Cutz E, Roifman CM: Pulmonary and hepatic disease in a patient with partial Adenosine Deaminase (ADA) deficiency. Pediatric Academic Society 2007: 61: p e6286.5. Grunebaum E, Roifman CM: Clinical and immunological features of patients with WHIM syndrome (Warts, Hypogammaglobulinemia, Infections and Myelokathexis) with or without mutations in the CXCR4 gene. Pediatric Academic Society 2007: 61: p e6286.1. Lamothe J, Huynh KK, Grinstein S, Valvano MA: Intracellular survival of Burkholderia cenocepacia in macrophages is associated with a delay in the maturation of bacteria-containing vacuoles. Cellular Microbiology 2007: 9: pp 40-53. Grunebaum E, Sharfe N, Roifman CM: Human T cell Immunodeficiency: When signal transduction goes wrong. Immunologic Research 2006: 35: pp 117-125. Grunebaum E, Somech R, Reid B, Atkinson A, Roifman CM: Survival and immune function in patients with severe combined immune deficiency after bone marrow transplantation from matched unrelated donors. Clinical Immunology 2006: 119S: p S37. Guggenheim R, Somech R, Grunebaum E, Atkinson A, Roifman CM: Bone Marrow transplantation for Cartilage-hair-hypoplasia. Bone Marrow Transplantation 2006: 38: pp 751-756. Husain M, Aiuti A, Atkinson A, Grunebaum E, Roifman CM: Long-term enzyme replacement for adenosine deaminase-defiecient patient – immune function and outcome. Clinical Immunology 2006: 119S: p S133. Husain M, Grunebaum E, Cutz A, Roifman CM: Respiratory distress and lymphopenia in a neonate – presenting manifestation of severe combined immunodeficiency. Clinical Immunology 2006: S119: p e12a. Husain M, Grunebaum E, Naqvi A, Atkinson A, Ngan B, Aiuti A, Roifman CM: Burkitt lymphoma in adenosine deaminase deficient severe combined immunodeficiency treated with polyethylene glycolADA. Clinical Immunology 2006: S119: p e12b. Lavine E, Picard C, Casanova JL, Roifman CM: Cellular and humoral aberrations in a kindred with IL-1 receptor–associated kinase 4 deficiency. Journal of Allergy and Clinical Immunology. 2007: Jun4: pp e1-e3. Lavine E, Roifman CM: Clinical course and immunological features of siblings with IRAK-4 deficiency. Pediatric Academic Society 2007: 61: p e6286.6. Lee WL, Cosio G, Ireton K, Grinstein S: Role of CrkII in Fc gamma receptor-mediated phagocytosis. The Journal of Biological Chemistry 2007: 282: pp 1135-1143. Liao P, Grunebaum E: Gene therapy for purine nucleoside phosphorylase deficiency. Federationof-Clinical-Immunology-Societies, San Francisco. Clinical Immunology 2006: 119: pp S134-135. Martin-Orozco N, Touret N, Zaharik ML, Park E, Kopelman R, Miller S, Finlay BB, Gros P, Grinstein S: Visualization of vacuolar acidification-induced transcription of genes of pathogens inside macrophages. Molecular Biology of the Cell 2006: 1: pp 498-510. Mason D, Mallo GV, Terebiznik MR, Payrastre B, Finlay BB, Brumell JH, Grinstein S: Alteration of epithelial structure and function associated with PtdIns(4,5)P2 degradation by a bacterial phosphatase. The Journal of General Physiology 2007: 129: pp 267-283. Mero P, Huang ZY, Kim MK, Schreiber AD, Grinstein S, Booth JW: Phosphorylationindependent ubiquitylation and endocytosis of Fc gamma RIIA. The Journal of Biological Chemistry 2006: 281: pp 33242-33249. Moraes TJ, Plumb J, Martin R, Vachon E, Cherepanov V, Koh A, Loeve C, Jongstra-Bilen J, Burrows LL, Grinstein S, Downey GP: Abnormalities in the pulmonary innate immune system in cystic fibrosis. American Journal of Respiratory Cell and Molecular Biology 2006: 34: pp 364-374. Nicolay U, Kiessling P, Berger M, Gupta S, Roifman CM, Gardulf A, Eichmann F, Haag S, Massion C, Ochs H: Health-related quality of life in patients with primary immunodeficiencies in North America receiving subcutaneous IgG self-infusions at home. Journal of Clinical Immunology 2006: 26: pp 65-72. Notarangelo L, Casanova JL, Conley ME, Chapel H, Fischer A, Puck J, Roifman CM, Seger R, Geha RS: Primary immunodeficiency diseases: An update from the International Union of Immunological Societies primary immunodeficiency diseases classification committee meeting in Budapest, 2005. Journal of Allergy and Clinical Immunology 2006: 117: pp 883-896. Roifman CM, Gu Y, Cohen A: Mutations in the RNA component of Rnase MRP cause Omenn Syndrome. Journal of Allergy and Clinical Immunology 2006: 117: pp 897-903. Smith AC, Heo WD, Braun V, Jiang X, Macrae C, Casanova JE, Scidmore MA, Grinstein S, Myer T, Brumell JH: A network of Rab GTPases controls phagosome maturation and is modulated by Salmonella enterica serovar typhimurium. The Journal of Cell Biology 2007: 176: pp 263-268. Somech R, Grunebaum E, Roifman CM: Short lived antibody response as a manifestation of primary immunodeficiency. Pediatric Academic Society 2007: 61: p e6286.2. Somech R, Roifman CM: A novel syndrome of severe immunodeficiency, gonadal dysgenesis and cardiac defect. Clinical Immunology 2006: S119: p e9. Somech R, Roifman CM: Different phenotypes of profound T cell immunodeficiency associated with Rnase MRP mutations in cartilage hair hypoplasia patients. Clinical Immunology 2006: 119S: p S137. Steinberg BE, Scott CC, Grinstein S: Highthroughput assays of phagocytosis, phagosome maturation and bacterial invasion. American Journal of Physiology Cell Physiology 2007: 292: pp 945-952. DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 59 Steinberg BE, Touret N, Vargas-Caballero M, Grinstein S: In situ measurement of the electrical potential across the phagosomal membrane using FRET. Contribution to the proton-motive force. Proceedings of the National Academy of Sciences of the United States of America 2007: 104: pp 9523-9528. Suh CI, Stull ND, Li XJ, Tian W, Price MO, Grinstein S, Yaffe MB, Atkinson S, Dinauer MC: The phosphoinositide-binding protein p40phox activated the NADPH oxidase during Fcg IIA receptor-induced phagocytosis. The Journal of Experimental Medicine 2006: 203: pp 1915-1925. Tabeta K, Hoebe K, Janssen EM, Du X, Georgel P, Crozat K, Mudd S, Mann N, Sovath S, Goode J, Shamel L, Herskovits AA, Portnoy DA, Cooke M, Tarantino LM, Wiltshire T, Steinberg B, Grinstein S, Beutler B: 3d, a missense mutation that disrupts exogenous antigen presentation and signaling via Toll-like receptors 3, 7, and 9. Nature Immunology 2006: 7: pp 156-164. Tan T, Lee W, Alexander DC, Grinstein S, Liu J: The ESAT-6/CFP-10 secretion system of Mycobacterium marinum modulates phagosome maturation. Cellular Microbiology 2006: 8: pp 1417-1429. Toro A, Grunebaum E: TAT-mediated intracellular delivery of purine nucleoside phosphorylase corrects its deficiency in mice. Journal of Clinical Investigation 2006: 116: pp 2717-2726. Toro A, Paiva M, Ackerley C, Grunebaum E: Intracellular delivery of purine nucleoside phosphorylase (PNP) fused to protein transduction domain corrects PNP deficiency in vitro. Cellular Immunology 2006: 240: pp 107-113. Touret N, Grinstein S: Dynamic and functional characteristics of the phagosomal membrane. Medecine Sciences Paris 2006: 5: pp 457-458. Vachon E, Martin R, Plumb J, Kwok V, Vandivier W, Glogauer M, Kapus A, Chow CW, Grinstein S, Downey GP: CD44 is a phagocytic receptor. Blood 2006: 107: pp 4149-4158. Yan M, Di Ciano-Oliveira C, Grinstein S, Trimble WS: Coronin function is required for chemotaxis and phagocytosis in human neutrophils. The Journal of Immunology 2007: 178: pp 5769-5778 Yeung T, Ozdamar B, Paroutis P, Grinstein S: Lipid metabolism and dynamics during phagocytosis. Current Opinion in Cell Biology 2006: 4: pp 429-437. Yeung T, Terebiznik M, Yu L, Silvius J, Abidi WM, Philips M, Levine T, Kapus A, Grinstein S: Receptor activation alters inner surface potential during phagocytosis. Science 2006: 313: pp 347-351. 60 DEPARTMENT OF PAEDIATRICS BOOK CHAPTERS AND PROCEEDINGS Alexander RT, Grinstein S: Activation of kinases upon volume changes: role in cellular homeostasis. In: Cell Volume Regulation: Contributions to Nephrology (Lang F, ed). Karger Press, Basel, Switzerland, 2006. Dalal I, Roifman CM: The immunity of the newborn. In: UpToDate (Rose BD, ed). UpToDate, Waltham, MA, 2006. Roifman CM: Approach to the Diagnosis of Severe combined immunodeficiency. In: Manual of Molecular and Clinical Laboratory Immunology, 7th edition (Detrick B, Hamilton RG, Folds JD, eds). ASM Press, Washington, DC, 2006. Roifman CM: Human Interleukin-2 Receptor alpha Deficiency. In: Primary Immunodeficiency diseases: a molecular and genetic approach (Ochs HD, Smith CIE, Puck JM, eds). Oxford University Press, New York, NY, 2007. FUNDING A Clincial Study of the Pharmacokinetics. Efficacy & Safety of Immune Globulin Intravenous (Human) Omr-IgG-am IGIV in Subjects with Primaty Immune Deficiency Disease. Roifman CM. Leading Investigator, Site Principal investigators: Roberts R, Melamed IR, Moy J, Grunebaum E, Sussman GL, Chouksey A, Stein M, Wasserman RL, Levy R, McNeil D, Suez D. FFF Enterprises ($550,000 2006-2008) Analysis of the Role of Coronin in Neutrophil Chemotaxis. Grinstein S. Canadian Institute of Health Research ($549,490 2004-2009) Canadian Centre for Primary Immunodeficiency. Roifman CM. Canadian Centre for Primary Immunodeficiency ($410,000 2003-2007) Chair of Immunology. Roifman CM. Donald & Audrey Campbell ($586,000 1998-2006) CI Society SCID Picnic. Roifman CM. World Immunodeficiency Society ($4,586 2006) Correcting purine nucleoside phosphorylase deficiency by prolonged intracellular enzyme replacement therapy. Grunebaum E. March of Dimes ($390,000 2007-2010) Correcting PNP deficiency by intracellular delivery of PNP fused to protein transduction domains. Grunebaum E. US Immunodeficiency Network ($330,000 2006-2008) Correcting purine nucleoside phosphorylase using lenti-virus mediated human PNP gene delivery into haematopoietic stem cells. Grunebaum E. SickKids Foundation and the Institute of Human Development, Child and Youth Health, Canadian Institute of Health Research ($130,000 2006-2008) ANNUAL REPORT 2006-07 Dual Role of CD36 in Macrophages: Atherosclerosis and Immune Responsiveness. Grinstein S. The Heart and Stroke Foundation ($323,804 2006-2010) Fellowship in Immunology. Roifman CM. Canadian Friends of Tel Aviv University ($55,000 2006-2007) Genetics of Primary Immunodeficiency. Roifman CM. Canadian Immunodeficiency Society ($100,000 2007-2010) Home Immunoglobulin Therapy. Roifman CM. Canadian Immunodeficiency Society ($430,000 2007-2011) Human Blood Monocyte Receptor Expression Modulation. Grinstein S. National Institutes of Health ($500,000 2006-2011) Imaging Facility Multi-User Maintenance. Grinstein S. Canadian Institutes of Health Research ($464,225 2005-2010) Immunology and Transplantation. Roifman CM. McMillan Binch Barristers and Solicitors ($75,000 2002-2007) Jeffrey Modell Research Laboratories for the diagnosis of primary immunodeficiencies. Roifman CM. Jeffrey Modell Foundation ($321,000 2001-2009) Molecular basis of primary immunodeficiency. Roifman CM. Jeffrey Modell Foundation ($250,000 2006-2011) Molecular Mechanisms of Phagocyte Activation. Grinstein S. Canadian Institutes of Health Research ($828,800 2006-2011) Novel synthetic styryl-acrylonitrile inhibitors of tumour angiogenesis: In vivo translation. Roifman CM. Ontario Cancer Research Network ($512,179 2004-2007) Pathogenesis of Immune Malignancies. Roifman CM. Audey Stanleigh Leukemia Research Foundation ($52,500 2005-2008) Primary Immunodeficiency (PID) Registry. Roifman CM. Canadian Blood Services/Bayer Inc. ($194,150 2004-2008) Retrovirus gene therapy for primary immunodeficiency diseases. Grunebaum E. Jeffrey Modell Foundation ($11,000 2006-2007) Role of Eph receptors in the immune system. Roifman CM. Canadian Institutes of Health Research – 2nd renewal ($350,000 2004-2007) Targeting and Regulation of Ion Transporters. Grinstein S. Canadian Institutes of Health Research ($804,500 2004-2009) Infectious Diseases DIVISIONAL OVERVIEW The Division of Infectious Diseases is a leader in clinical, educational and research activities locally, nationally and internationally. The division has active inpatient as well as ambulatory consultant services that include teams that focus on general infectious diseases and infectious diseases in immunocompromised hosts, respectively. We are a resource for issues related to infectious disease outbreaks, including new and emerging infectious, infection control and the specialized areas of HIV, congenital infections, management of infectious complications or organ transplantation, and the ever-increasing problem of multi-drug resistant tuberculosis. Division members have a leading role in national and international epidemiological, clinical, research advisory and related committees (e.g., the Infectious Diseases Society of America, the Canadian Paediatric Society, Health Canada, the American Society for Transplantation, the Government of the Bahamas and the HIV/AIDS program in Russia). Our research initiatives are structured around themes that include host susceptibility and related factors and innovative antimicrobial and associated therapies. Specific research initiatives include multicentered paediatric HIV studies related to maternal-to-child transmission and studies on new antiretrovirals, vaccine studies and research into the mechanisms of host susceptibility to viral infections. These infections include viral myocarditis, influenza illness, HIV, EBV-related post-transplant lympoproliferative disease (PTLD) and encephalitis. Multicentered trials related to the diagnosis and prevention of infections in transplant patients are ongoing, as well as participation in national surveillance studies Division Head Dr. Upton D. Allen Dr. Dat Tran Dr. Valerie Waters Full-Time Dr. Sean Ari Bitnun Dr. E. Lee Ford-Jones Dr. Anne G. Matlow Dr. Anne Opavsky Dr. Stanley E. Read Part-Time Dr. Ian Kitai Dr. Alana Rosenthal Dr. Anupma Wadhwa Cross-Appointees Dr. Susan Richardson Dr. Elaine Wang Honorary Dr. Ronald Gold Dr. Susan King Clinical Fellows Dr. Mohammed Al-Ayed Dr. Kahlid Al Hinai Dr. Walaa Al-Mansouri Dr. Abdul Al-Odayani (IMPACT) and national collaborative paediatric studies through the Paediatric Investigators Collaborative Network on Infections in Canada (PICNIC). Our research in infection control includes the clinical and molecular epidemiology of paediatric nosocomial infections. The division has continued to have a leadership role in structuring the approach to the management of major emerging and resurgent infectious disease threats. In this regard, the division has been very actively involved in influenza pandemic planning efforts at SickKids as well as provincially and nationally. In the area of education, representatives from our division continue to play a key role in activities of the Royal College of Physicians and Surgeons of Canada, and in many areas of undergraduate, graduate and post-graduate and continuing medical education activities. Faculty members continue to hold editorships, visiting professorships and related academic honours nationally and internationally. In addition, one of our faculty members, Dr. FordJones received the Claus Wirsig Humanitarian Award in 2007. Dr. Mohammed Al-Shehri Dr. Shalini Desai Dr. Spenta Kakalia Dr. Kaeda Ota Dr. Wanatpreeya Phongsmart Dr. Ashley Roberts Research Fellows Dr. Abdulkader Al-Rezqi Dr. Jason Brophy Dr. Michelle Barton-Forbes Dr. Michael Hawkes Helen Heurter Sharon Li Brenda O’Doherty Jennifer Ponsonby Asad Sabah Kim Simpson Laura-Lee Tannis Research Staff Nasser Khodai Booran Clinical Nurses Cathy Den Hollander Debra Louch Georgina MacDougall Pat Malloy Administrative Staff Brenda Bishop Toula Myriklis Zita Rotar Bebi Wali Sue Weller HONOURS AND AWARDS Allen U: Visiting Professor and External Examiner, University of West Indies, Jamaica (with site visits to Jamaica, Bahamas, Barbados and Trinidad), 2007. Allen U: Full Professorship, Departments of Paediatrics and Health Policy Management and Evaluation, University of Toronto, 2007. Allen U: Plenary Symposium Chair, Infections after transplantation, International Pediatric Transplant Association, Cancun, Mexico, March 2007. Allen U: Symposium Chair, New Antimicrobial Therapeutics, Paediatric Academic Societies, Toronto, May 2007, Bitnun A: PERLs Resident Teaching Award, 2007. Ford-Jones L: Proposal and initiation of the Royal College of Physicians and Surgeons of Canada CanMEDS Competencies Award for SickKids Residents and Subspecialty Trainees, 2007. Ford-Jones L: Claus Wirsig Humanitarian Award, The Hospital for Sick Children, 2007. Ford-Jones L: Convocation Address, George Brown College, School of Early Childhood Education and School of Nursing, 2007. Read SE: Professor Emeritus, University of Toronto, 2006. Read SE: Inaugural Professorial Lecture, University of the West Indies, Nassau Campus, 2007. PUBLICATIONS Allen U: Public health Implications of MRSA in Canada. Canadian Medical Association Journal 2006: 175: p 161. Drs. Ari Bitnun and Ian Kitai with trainees and nursing staff Allen U and the Infectious Diseases and Immunization Committee, Canadian Paediatric Society (CPS): Antimicrobial products in the home: The evolving problem of antibiotic resistance. Paediatrics & Child Health 2006: 11: pp 69-173. Allen U and the Infectious Diseases and Immunization Committee, Canadian Paediatric Society (CPS): Current Management of herpes simplex virus infection in pregnant women and their newborn infants. Paediatrics and Child Health 2006: 11: pp 363-365. Allen U, Aoki F, Stiver G: Influenza chemoprophylaxis and therapy: Recommended Guidelines for Practitioners. Paediatrics and Child Health 2006: 11: pp 520-522. Allen U, Aoki F, Stiver G: Influenza chemoprophylaxis and therapy: Recommended Guidelines for Practitioners. The Canadian Journal of Infectious Diseases and Medical Microbiology 2006: 17: pp 273-284. Allen U and the Infectious Diseases and Immunization Committee, Canadian Paediatric Society (CPS): Pandemic influenza and Canada’s children. Paediatrics & Child Health 2006: 11: pp 335-337. Allen U and the Infectious Diseases and Immunization Committee, Canadian Paediatric Society (CPS): Control and treatment of methicillin-resistant Staphylococcus aureus in Canadian paediatric healthcare institutions. Paediatrics & Child Health 2006: 11: pp 163-165. Allen U and the Infectious Diseases and Immunization Committee, Canadian Paediatric Society (CPS): Community-associated methicillinresistant Staphylococcus aureus: Implications for the care of children. Paediatrics & Child Health 2007: 12: pp 323-324. Allen U and the Infectious Diseases and Immunization Committee, Canadian Paediatric Society (CPS): Maternal infectious diseases, antimicrobial therapy or immunizations: Very few contraindications to breastfeeding. Paediatrics & Child Health 2006: 11: pp 490-491. Barton-Forbes M, Hawkes M, Moore D, Conly J, Nicolle L, Allen U, Boyd N, Embree J, Van Horne L, LeSaux N, Richardson S, Moore A, Tran D, Waters V, Vearncomve M, Katz K, Weese JS, Embil J, Ofner-Agnostini M, Ford-Jones EL: The Writing Group of Canadian Infectious Disease, Infection Prevention and Control; and Public Health Specialists, Guidelines for the prevention and management of community-associated methicillinresistant Staphylococcus aureus (CA-MRSA): A perspective for Canadian Health Care Practitioners. Canadian Journal Infectious Diseases and Microbiology 2006: 17: pp 4C-24C. Bitnun A, Roberts A, McGeer A, Tran D, Yau Y, Simpson K, Green K, Lowe D, Ford-Jones E and the Toronto Invasive Bacterial Diseases Network: Laboratory-confirmed influenza-associated requiring hospitalizations among children in the metropolitan Toronto and Peel region by active surveillance, 2004-2005. Canada Communicable Disease Report 2006: 32: p 19. Brophy J, Yau Y, Cox P, Katz K, Bitnun A: A sentinel fatal paediatric case of community-associated methicillin-resistant Staphylococcus aureus in Canada. Paediatrics and Child Health 2007: 12: pp 319-322. Doja A, Bitnun A, Ford-Jones EL, Richardson S, Tellier R, Petric M, Heurter H, MacGregor D: Pediatric Epstein-Barr encephalitis; a 10 review. Journal of Child Neurology 2006: 21: pp 385-391. Elbers J, Bitnun A, Richardson SE, Ford-Jones L, Tellier R, Wald R, Petric M, Kolski H, Heurter H, MacGregor D: Childhood herpes simplex encephalitis: A 12-year prospective study of childhood herpes simplex encephalitis: Is there a broader spectrum of disease? Paediatrics 2007: 119: p e399. Flicker SE, Skinner HR, McClelland A, Saulnier P, Read S, Goldberg E: Supposed to make you better but it doesn’t really: HIV-positive youths’ perceptions of HIV treatment. Journal of Adolescent Health 2006: 38: pp 261-267. Hawkes M, Barton-Forbes M, Conly J, Nicolle L, Barry C, Ford-Jones EL: Superbug at our doorstep. Canadian Medical Association Journal 2007: 176: pp 54-56. Hernandez De Mezerville M, Tellier R, Herbert D, Doyle J, Allen U: Adenovirus in paediatric transplant recipients: A hospital-based study. Paediatric Infectious Diseases Journal 2006: 25: pp 815-818. Kam A, Ford-Jones EL, Malloy P, Kitai I: Adolescent tuberculosis: Multi-focal involvement and delay in diagnosis. A 5 year experience from The Hospital for Sick Children, Toronto, Canada, 1999-2004. Paediatric Infectious Diseases Journal 2007: 26: pp 355-356. Kylat RI, Kelly EN, Ford-Jones EL: Clinical findings and adverse outcome in neonates with congenital CMV infection. European Journal of Paediatrics 2006: 165: pp 773-778 Maclean JE, Mahant S, Bitnun A, Read S, Capra M, Solomon M: Worlds apart – the different faces of childhood HIV. Paediatrics & Child Health 2006: 42: pp 739-742. Maclean JE, Mahant S, Bitnun A, Read S, Capra M, Solomon M: Family matters: A twelve-year-old male with respiratory symptoms, cachexia and clubbing. Paediatrics & Child Health 2006: 42: pp 739-742. Maekawa Y, Ouzounian M, Opavsky A, Liu PP: Connecting the missing link between dilated cardiomyopathy and viral myocarditis: Virus Cystoskeleton and Innate Immunity-Circulation 2007: 115: pp 5-8. DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 63 Matlow AG, Wishen A, Read SE, Raboud JM: A study of provider-caregiver communication in paediatric ambulatory care. Paediatrics & Child Health 2006: 11: pp 217-221. Moore DL, Vandry W, Scheifele DW, Halperin SA, Dery P, Ford-Jones E, Law BJ. Lebel M, Le Saux N, Grimsrud K, Tam T: Surveillance for influenza admissions among children hospitalized in Canadian immunization monitoring program active centres, 2003-2004. Paediatrics 2006: 118: pp e610-e611. Roberts A, Bitnun A, McGeer A, Tran D, Yau Y, Simpson K, Green K, Lowe D, Kawa B, Ford-Jones EL, and the Toronto Invasive Bacterial Disease Network: Laboratory-confirmed influenzaassociated hospitalizations among children in the metropolitan Toronto and Peel region by active surveillance. Canadian Communicable Disease Report 2006: 32: pp 203-207. Rosenthal A, White D, Churilla S, Brodie S, Katz K: Surveillance culture sites in newborns to optimize detection of methicillin-resistant Staphylococcus aureus. Journal of Clinical Microbiology 2006: 44: pp 4234-4236. Salter R, King SM, Smith ML, Bitnun A, Brophy J, Fernandes-Penney A, Lefebvre A, Louch D, MacDougall G, Moore W, Read SE: Disclosing HIV diagnosis to infected children: A health care team’s approach. Vulnerable Children & Youth Studies Journal 2007: 2: pp 12-16. Saunders A, Panaro L, McGeer A, Rosenthal A, White D, Willey BM, Gravel D, Bontovics E, Yaffe B. Katz K: An outbreak of methicillin-resistant staphylococcus aureus (MRSA) among healthy newborns and their mothers. Canadian Journal of Infectious Diseases and Medical Microbiology 2007: 18: pp 128-132. 64 DEPARTMENT OF PAEDIATRICS Schuh S, Lalani A, Allen U, Manson D, Babyn P, Stephens D, MacPhee S, Mokanski M, Kahikin S, Dick P.: Evaluation of the utility of radiography in acute bronchiolitis. Journal of Pediatrics 2007: 150: pp 429-433. Tjon J, Lam W, Seto W, Dekker A, Wong C, Atenafu E, Bitnun A, Waters V, Yau Y, Solomon M, Ratjen F: Pharmacokinetic modeling of a once-daily dosing regimen for intravenous tobramycin in paediatric cystic fibrosis patients. Journal of Antimicrobiology and Chemotherapy 2007: 59: pp 1135-1140. Walsh CM, Morris SK, Brophy J, Hiraki L, Richardson S, Allen U: Disseminated blastomycosis in an infant: A case report and review of the literature. Pediatric Infectious Diseases Journal 2006: 25: pp 656-658. Waters VJ, Gomez MI, Soong G, Amin S, Ernst R, Prince A: Immunostimulatory properties of the emerging pathogen Stenotrophomonas maltophilia. Infectious Immunology 2007: 75: pp 1698-1703. Waters VJ, Ratjen F: Multidrug-resistant organisms in cystic fibrosis: Management and infection control issues. Expert Review Anti-Infective Therapy 2006: 4: pp 807-819. Waters VJ, Sokol S, Reddy B, Soong G, Chun J, Prince A: The effect of cyclosporine on airway cell proinflammatory signaling and pneumonia. American Journal of Respiratory, Cellular and Molecular Biology 2006: 33: pp 138-144. Wadhwa A, Lingard L: A qualitative study examining tensions in interdictor telephone consultations. Medical Education 2006: 40: pp 759-767. ANNUAL REPORT 2006-07 BOOKS AND BOOK CHAPTERS Liu P, Fuse K, Chu G, Liu Y, Opavsky A: Recent insights into the role of host innate and acquired immunity responses. In: Ernst Schering Research Foundation Symposium Proceedings (Schultheiss H-P, Kapp J-F, Grötzbach G, eds). Springer, Berlin, 2006. Lynk DA, Kernested DL, Ford-Jones EL: Young infants in child care: In: Infection Control in the Child Care Centre and Pre-School, 7th Edition (Donowitz L, ed). Williams and Wilkins, Philadelphia, 2006. FUNDING A 48-week, phase II non-comparative, open-label multicentre study to evaluate the safety, tolerability, pharmacokinetics and antiviral activity of GW-433908/Ritnovir BID in HIV-infected children. Read SE, Bitnun A. GlaxoSmith Kline ($40,000 2004-2006) A multicenter, double-blind, randomized, placebocontrolled, dose-ranging trial evaluating the safety and efficacy of RI-001 (RespImmune/RSV-IVIG) in respiratory syncytial virus (RSV) infected patients at risk of severe RSV illness. Allen U and ADMA 001 Study Group (Allen U, PI for international study) ADMA Biologics (estimated $500,000 2007 internationally) A phase II, open-label trial, to investigate pharmacokinetics, safety, tolerability and antiviral activity of TMC 114/rtv b.i.d. in treatmentexperienced HIV-1 infected children and adolescents. Bitnun A, Read S. Tibotec ($6,675 2006-2007) A pilot study of genetic determinants of severe influenza in children. Tran D, Paterson A, Beyene J, Schuh S, Richardson S. SickKids Research Institute ($33,800 2006-2007) A pilot study of genetic determinants of severe influenza in children. Tran D, Paterson A, Beyene J, Schuh S, Richardson S. Dean’s Fund New Staff Grant, University of Toronto ($10,000 2007-2008) IMPACT Meningococcal Surveillance Program (January 1, 2007-December 31, 2007) Scheifele D, Halperin S, Ford-Jones L. Public Health Agency Canada Canadian Paediatric Society ($11,662 2007) Case control study of a pertussis outbreak in Toronto from October 2005-March 2006. Waters V, Ford-Jones L, Richardson S, Finkelstein M, Halperin S. Sanofi Pasteur ($80,000 2006-2007) Immunization Monitoring Practices Active (IMPACT) Program. Scheifele D, Halperin S, Ford-Jones L. Public Health Agency of Canada/Canadian Paediatric Society ($55,193 2007) Case control study of a pertussis outbreak in Toronto from October 2005-March 2006. Waters V, Ford-Jones L, Richardson S, Finkelstein M, Halperin S. Ontario Ministry of Health and Long-Term Care ($23,924 2006) Defining the role of Stenotrophomonas maltophilia in CF patients. Waters V, Yau Y. Bickell Foundation ($64,976 2007) Defining the role of Stenotrophomonas maltophilia in CF patients. Waters V, Yau Y. SickKids Summer Research Program ($2,000 2007) Defining the role of Stenotrophomonas maltophilia in CF patients. Waters V, Yau Y. SickKids Research Institute ($15,000 2006-2007) Heart failure and the Coxsackie-Adenovirus Receptor (CAR): Insights through a novel heart targeted CAR transgenic mouse. Opavsky A. Heart and Stroke Foundation of Ontario ($278,151 2007-2010) Mitochondrial DNA Damage in Infants exposed to HIV Antiretroviral Drugs in Utero. Cote H. Forbes J, Money D, Burdge D, Harrigan R, Bitnun A, Read S. Canadian Institutes of Health Research ($267,915 2006-2008) Multidose, open label randomized safety and pharmacokinetic study of Tipranivir in combination with low-dose Ritnovir in HIV infected paediatric patients. Read S, Bitnun A. Boehringer Ingelheim ($30,000 2004-2006) Night float: A qualitative evaluation of residents’ perceptions. Hall S, Ladhani M, Wadhwa A. McMaster Children’s Hospital Paediatric Residency Research Grant ($2,380 July 2006) Omega 3 fatty acid supplementation on hypertriglyceridemia in HIV-infected children taking Highly Active Antiretroviral Therapy (HAART): A randomized cross-over study. Read S, Bitnun A, Brophy J. Physicians’ Services Incorporated Foundation ($32,000 2007) Immunogenicity of the 7-valent conjugate penumococcal vaccine in HIV-infected children. Bitnun A, Alllen U, Read S. Physicians’ Services Incorporated Foundation ($16,000 2007-2008) Positive youth project capacity building grant. Canadian AIDS treatment information exchange. Flicker S, Read S, Goldberg E, Skinner H, Veinot T, Taylor D, McClellan A. Ontario HIV Treatment Network ($25,000 2006-2007) Impact of oximetry on disposition in acute bronchiolitis. Schuh S, Freedman S, Coates A, Parkin P, Allen U, Willan A, Ungar W, DaSilva Z. Physicians’ Services Incorporated Foundations ($163,000 2007-2009) Prospective cohort study of genetic variation and risk of infection in Canadian children with primary acute myeloid leukemia. Sung L, Beyene J, Allen U. National Cancer Institutes of Canada ($444,424 2005-2008) IMPACT program including pneumococcal surveillance. Scheifele D, Halperin S, Ford-Jones L. IMPACT centres, Canada (12 children’s hospitals): Public Health Agency Canada administered through Canadian Paediatric Society ($54,110 2006-2007) Retrospective study of caspofungin in organ transplant recipients. Allen U. Merck Canada ($21,000 2006-2007) RSV Immunoprophylaxis: graduate students funding support. Allen U, O’Brien K, Simmons B. Abbott Laboratories ($35,000 2005-2006) RSV nurse coordinator program. Allen U. Abbott Laboratories ($65,000 2006-2007) The Coxsackie-Adenovirus Receptor in heart infection and heart failure. Opavsky A. Heart and Stroke Foundation of Ontario ($166,500 2007-2010) The Coxsackie-Adenovirus Receptor: Function meets infection in viral myocarditis and dilated cardiomyopathy. Opavsky A. Heart and Stroke Foundation of Ontario ($172,014 2005-2007) The health and economic impact of rotavirus infection in young children: Retrospective and prospective study by the Immunization Monitoring Practices Active (IMPACT) Program. Lesaux N, Halperin S, Scheifele D, Bettinger, Ford-Jones L, Waters V, deWals P. IMPACT: Public Health Agency of Canada ($100,000 2006-2007) Study of effectiveness of the influenza vaccine to prevent hospitalization in children 6-23 months of age during the influenza season 2006-2007. DeSerres G, Ford-Jones L. Public Health Agency of Canada ($10,000 2006-2007) Tuberculosis immunity in childhood. Lewishon D, Kitai I, Loeffler A. Oregon Health Sciences University/National Institutes of Health ($11,000 2005-2008) What makes an excellent clinician in an Academic Health Sciences Centre? Mahant S, Wadhwa A. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($8,300 2007) Division Head Dr. Keith Tanswell SICKKIDS Full-Time Dr. Jacques Belik Dr. Jonathan Hellmann Dr. Andrew James Dr. Kyong-Soon Lee Dr. Patrick McNamara Dr. Aideen Moore Dr. Hilary Whyte Part-Time Dr. Rosemary Moodie Cross Appointees Dr. Bonnie Stevens Adjunct Dr. Xiaoping Luo Honorary Faculty Dr. Max Perlman Dr. Paul Swyer MOUNT SINAI HOSPITAL Dr. Yenge Diambomba Dr. Ann Jefferies Dr. Edmond Kelly Dr. Karel O’Brien Dr. Arne Ohlsson Dr. Prakesh Shah Dr. Vibhuti Shah Dr. Sharon Unger Dr. Kin Fan Young Tai ST. MICHAEL’S HOSPITAL Dr. Tony Barozzino Dr. Douglas Campbell Dr. Michael Sgro SUNNYBROOK HEALTH SCIENCES CENTRE Full-Time Dr. Elizabeth Asztalos Dr. Michael Dunn Dr. Robert Jankov Dr. Andrew Shennan Neonatology DIVISIONAL OVERVIEW The Division of Neonatology welcomes Dr. Adrian Ziino to the Faculty at the Sunnybrook Health Sciences Centre (Women’s College Hospital site). The division continues to be well supported by major funding agencies for its various foci at the three sites, including lung biology, epidemiology, outcomes-based research and clinical trials. Dr. Jonathan Hellmann and NICU staff The three level III NICUs at The Hospital for Sick Children, Mount Sinai Hospital and at the Women’s College Hospital site continue to suffer from a shortage of sufficient level III NICU beds to adequately meet the maternal and neonatal needs of our catchment area, which has resulted in regrettable deferrals of patients out of region, province and country. The Ministry of Health and Long-Term Care commissioned a Neonatology Task Force, with province-wide representation including representatives from the three Toronto NICUs, to address the issue of current level III NICU bed requirements across the province, and the predicted increase in requirements over the next 10-15 years. The report has been delivered to the Ministry and a response is awaited. The division’s Neonatal-Perinatal Fellowship Training Programme was reviewed this year, and received full approval. Dr. Martin Skidmore, the Programme Director, is to be congratulated on his dedication and effort. We continue to train the largest number of subspecialty trainees in Neonatology of any centre in Canada. Dr. Brian Simmons Dr. Martin Skidmore Dr. Adrian Ziino Part-time Dr. Shaheen Doctor Dr. Eugene Ng Clinical Fellows Dr. Mudaffer Al-Mudaffer Dr. Suhair Al-Shanteer Dr. Ijaz Aman Dr. Taiba Balkhair Dr. Rudaina Banihani Dr. Donna Capili Dr. Blondel Crosdale Dr. Amr El Shahed Dr. Walid El-Naggar Dr. Emer Finan Dr. Claudia Garcia Gelvez Dr. David Gryn Dr. Omer Hamud Dr. Doriana Kriguer Dr. Patricia Lewis Dr. Zhihau Li Dr. Pat Mateo Dr. Askok Mittal Dr. Marcelo Panzenhagen Dr. Shilpa Saxena Dr. Arvind Sehgal Dr. Sandesh Shivananda Dr. Niranjan Thomas Dr. Chris Tomlinson Dr. Connie Williams Dr. Jennifer Young Dr. Meijian Zhao Research Fellows Dr. Ben Hur Johnson Dr. Mandy Lau Dr. Azar Masoud Dr. Natasha O’Reilly Dr. Josh Silver Dr. Man Yi Dr. Adrian Ziino Administrative Staff Sonia Dos Santos Ana Granic Catherine Martin Jewel Randolph Jankov RP: Career Development Award, Canadian Child Health Clinician Scientist Program/Canadian Institutes of Health Research, 2004-2008. McNamara PJ: Teacher of the Year Award, Division of Neonatology, June 2007. Post M: Canadian Research Chair (Tier 1) in Fetal, Neonatal & Maternal Health, 2000-2007. Tanswell AK: Women’s Auxiliary Chair in Neonatology, 2007. PUBLICATIONS Al Nabhani D, Shivananda S, Holtby H, Forte V, McNamara PJ: The hazardous course of an end tidal CO2 catheter in a premature infant. Anesthesiology 2007: 107: pp 180-181. Aher S, Ohlsson A: Late administration of erythropoietin for preventing red blood cell transfusion in preterm and/or low birth weight infants. The Cochrane Database of Systematic Reviews 2006: 3: CD004868. Aher SM, Ohlsson A: Early versus late erythropoietin for preventing red blood cell transfusion in preterm and/or low birth weight infants.The Cochrane Database of Systematic Reviews 2006: Issue 3: CD004865. Al-Faleh K, Lee K-S, Ramsay J, Nowaczyk M: Genetic considerations in recurrent congenital diaphragmatic hernia in two siblings. Annals of Saudi Medicine 2006: 26: pp 391-394. Barton M, Hawkes M, Moore D, Conly J, Nicolle L, Allen U, Boyd N, Embree J, Van Horne L, Le Saux N, Richardson S, Moore AM, Tran D, Waters V, Vearncombe M, Katz K, Weese JS, Embil J, OfnerAgostini M, Lee Ford Jones E: The Writing Group of the Expert Panel of Canadian Infectious Disease, Infection Prevention & Control, & Public Health: Guidelines for the prevention and management of community-associated methicillinresistant Staphylococcus aureus: A perspective for Canadian health care practitioners. Canadian Journal of Infectious Disease & Medical Microbiology 2006: 17: pp 4C-24C. Belik J, Kerc E, Pato MD: Rat pulmonary arterial smooth muscle myosin light chain kinase and phosphatase activities decrease with age. American Journal of Physiology – Lung Cellular and Molecular Physiology 2006: 290: pp L509-L516. 68 DEPARTMENT OF PAEDIATRICS Belik J, Hehne N, Pan J, Behrens A: Soluble guanylate cyclase-dependent relaxation is reduced in the adult bronchial smooth muscle. American Journal of Physiology – Lung Cellular and Molecular Physiology 2007: 292: pp L699-L703. Breau LM, McGrath PJ, Stevens B, Beyene J, Camfield CS, Finley GA, Franck L, Gibbins S, Howlett A, McKeever P, O’Brien K, Ohlsson A: Judgments of pain in the neonatal intensive care setting: A survey of direct care staffs’ perceptions of pain in infants at risk for neurological impairment. The Clinical Journal of Pain 2006: 22: pp 122-129. Campbell D, Shah PS, Shah V, Kelly E: Nasal continuous positive airway pressure delivered by high flow cannula versus infant flow system for preterm infants – A randomized pilot trial. Journal of Perinatology 2006: 26: pp 546-549. Chan A, Jayasuriya K, Berry L, Roth-Kleiner M, Post M, Belik J: Volutrauma activates the clotting cascade in the newborn but not adult rat. American Journal of Physiology – Lung Cellular and Molecular Physiology 2006: 290: pp L754-L760. Das A, Shah PS: Octreotide for the treatment of chylothorax in neonates (Protocol). Cochrane Database Systematic Reviews 2007: 2: CD006388. Davey C, Moore AM: Necrotizing fascitis of the scalp in the newborn. Obstetrics and Gynecology 2006: 107: pp 461-463. Deimling J, Thompson K, Tseu I, Wang J, Keijzer R, Tanswell AK, Post M: Mesenchymal maintenance of distal epithelial cell phenotype during late fetal lung development. American Journal of Physiology 2007: 292: L725-L741. El-Sayed M, Taddio A, Fallah S, de Silva N, Moore AM: Safety profile of morphine following surgery in neonates. Journal of Perinatology 2007: 27: pp 444-447. Epelman M, Daneman A, Navarro OM, Morag I, Morre AM, Kim JH, Feingold R, Taylor G, Gerstle T: Necrotizing enterocolitis (NEC): A review of stateof-the-art imaging findings with pathologic correlation. Radiographics 2007: 37: pp 285-305. ANNUAL REPORT 2006-07 Fluss J, Blaser S, Chitayat D, Akoury H, Glanc P, Skidmore M, Raybaud C: Molar tooth sign in fetal brain magnetic resonance imaging leading to the prenatal diagnosis of Joubert syndrome and related disorders. Child Neurology 2006: 21: pp 320-324. Godambe S, Newby B, Shah V, Shah PS: Effect of Indomethacin on Closure of Ductus Arteriosus in VLBW Neonates after early surfactant. Acta Paediatrica 2006: 95: pp 1389-1393. Jankov RP, Kantores C, Belcastro R, Yi M, Tanswell AK: Endothelin-1 inhibits apoptosis of pulmonary arterial smooth muscle in the neonatal rat. Paediatric Research 2006: 60: pp 245-251. Kantores C, McNamara PJ, Engelberts D, Kavanagh BP, Jankov RP: Therapeutic hypercapnia prevents chronic hypoxia-induced pulmonary hypertension in the newborn rat. American Journal Physiology – Lung Cellular and Molecular Physiology 2006: 291: pp L912-L922. Kaylat RI, Kelly EN, Ford-Jones EL: Clinical findings and adverse outcome in neonates with symptomatic congenital cytomegalovirus (SCCMV) infection. European Journal of Paediatrics 2006: 165: pp 773-778. Kaylat R, Ohlsson A: Recombinant human activated protein C for severe sepsis in neonates. Cochrane Database Systematic Reviews 2006: 2: CD005385. Koehler DR, Martin B, Corey M, Palmer D, Ng P, Tanswell AK, Hu J: Readministration of helper-dependent adenovirus to mouse lung. Gene Therapy 2006: 13: pp 773-780. Liao L, Ning Q, Li Y, Wang W, Wang A, Wei W, Liu X, Auten RL, Tanswell AK, Luo X: CXCR2 blockade reduces radical formation in hyperoxia-exposed newborn rat lung. Paediatric Research 2006: 60: pp 299-303. Ly LG, Hawes J, Whyte HE, Teixiera LS, McNamara PJ: The hemodynamically significant ductus arteriosus in critically ill full-term neonates. Biology Neonate 2007: 91: pp 206-265. Magee LA, von Dadelszen P, Chan S, Gafni A, Gruslin A, Helewa M, Hewson S, Kavuma E, Lee SK, Logan AG, McKay D, Moutquin J-M, Ohlsson A, Rey E, Ross S, Singer J, Willan AR, Hannah ME: The CHIPS (Control of Hypertension in Pregnancy Study) pilot trial. British Journal of Obstetrics and Gynaecology 2007: 114: p 770. Dr. Aideen Moore and NICU team HONOURS AND AWARDS Maegawa G, Chitayat D, Blaser S, Whyte H, Thomas M, Kim P, Kim J, Taylor G, McNamara PJ: Bilateral microtia with absence of external auditory meati, fused ossicles and Mondini dysplasia, duodenal atresia, anal anomalies, thyroid hemiplasia and bilary atresia. A new mandibulofacial dysostosis syndrome? Clinical Dysmorphology 2006: 15: pp 191-196. Mark H, Yudin MH, Shah V, Ohlsson A, Farine D: Are we winning the fight against GBS infection? Obstetrics and Gynaecology 2006: 28: pp 499-500. McCaul C, McNamara P, Engleberts D, Slorach C, Hornberger L, Kavanagh B: Epinephrine increases mortality after brief asphyxial cardiac arrest in an in vivo rat model. Anesthesia and Analgesia 2006: 102: pp 542-548. McCaul C, McNamara PJ, Engleberts D, Slorach C, Hornberger L, Kavanagh B: The effect of global hypoxia on myocardial function after successful cardiopulmonary resuscitation in a laboratory model. Resuscitation 2006: 68: pp 267-275. Nadeau K, Jankov RP, Oyewumi L, Tanswell AK, Sweezey NB, Kaplan F: Lgl1 is suppressed in oxygen toxicity models of bronchopulmonary dysplasia and normalizes during recovery in air. Pediatric Research 2006: 59: pp 389-395. Shah P, Beyene J, To T, Ohlsson A, Perlman M: Postasphyxial hypoxic-ischemic encephalopathy in neonates: Outcome prediction rule within 4 hours of birth. Archives of Pediatrics & Adolescent Medicine. 2006: 160: pp 729-736. Newby B, Cuddy M, Shah V: No infantile hupertrophic pyloric stenosis among neonates who received erythromycin for postexposure prophylaxis against pertussis. Canadian Journal of Hospital Pharmacy 2007: 60: pp 35-39. Shah S, Ohlsson A: Ibuprofen for the prevention of patent ductus arteriosus in preterm and/or low birth weight infants. Cochrane Database Systematic Reviews 2006: 1: CD004213. Oepkes D, Seaward PG, Frank PHA, Vandenbussche FPHA, Windrim R, Kingdom J, Beyene J, Kanhai HHH, Ohlsson A, Ryan G, for the DIAMOND Study Group: Doppler ultrasonography versus amniocentesis to predict fetal anemia. New England Journal of Medicine 2006: 355: pp 156-164. Ohlsson A, Jacob S: Meta-regression can indicate if further NIDCAP studies are justified. Lakartidningen 2007: 104: pp 134-137. McDonald S, Walker M, Perkins S, Beyene J, Murphy K, Gibb W, Ohlsson A: The effect of tobacco exposure on the fetal hypothalamicpituitary-adrenal axis. British Journal of Obstetrics and Gynaecology 2006: 113: pp 1289-1295. Palencia R, Gafni A, Hannah M, Willian A, Hewson S, McKay D, Hannah W, Whyte H, Amankwah K, Cheung M, Guselle P, Helewa M, Hodnett E, Kung R, Saigal S: The costs of planned caesarean versus planned vaginal births in the Term Breech Trial. Canadian Medical Association Journal 2006: pp 1109-1113. McNamara PJ: Non-conventional use of amplitude integrated electroencephalography (aEEG): Lessons from a tertiary NICU. Neonatology Today 2007: 2: pp 1-9. Parvez O, Voss AM, de Kok M, Roth-Kleiner M, Belik J: Bronchial muscle peristaltic activity in the fetal rat. Pediatric Research 2006: 59: pp 756-761. Moore AM, O’Brien K: Follow-up issues with multiples. Paediatrics and Child Health 2006: 11: pp 283-286. Schmidt B, Roberts RS, Davis P, Doyle LW, Barrington KJ, Ohlsson A, Solimano A, Tin W, for the Caffeine for Apnea of Prematurity Trial Group: Methylxanthine therapy for apnea of prematurity. New England Journal of Medicine 2006: 354: pp 212-221. Morag I, Epelman M, Daneman A, Moineddin R, Parvez B, Shechter T, Hellmann J: Portal vien thrombosis in the neonate: Risk factors, course and outcome. Journal of Pediatrics 2006: 148: pp 735-739. 70 DEPARTMENT OF PAEDIATRICS Sgro M, Campbell CM, Shah V: Incidence and causes of severe neonatal hyperbilirubinemia in Canada. Canadian Medical Association Journal 2006: 175: pp 587-590. ANNUAL REPORT 2006-07 Shah PS, Shah N: Heparin bonded catheters for prolonging the patency of central venous catheters in children. Cochrane Database Systematic Reviews 2006: 2: CD005983. Shah PS, Kalyn A, Satodia P, Dunn M, Parvez B, Daneman A, Salem S, Glanc P, Ohlsson A, Shah V: A randomized controlled trial of heparin versus placebo infusion to prolong the usability of peripherally placed percutasneous central venous catheters (PCVCs) in neonates. Paediatrics 2007: 119: pp 284-291. Shah PS, Siriwardena K, Taylor G, Steele L, Ray P, Blaser S, Chitayat D: Sudden infant death in a patient with FGFR3 P250R mutation. American Journal of Medicine Genetics 2006: 140: pp 2794-2796. Shah PS, Shah N: Low molecular weight heparin for prevention of central venous catheterization related thrombosis in children. Cochrane Database of Systematic Reviews 2006: 2: CD005982. Shah PS, Weksberg R, Chitayat D: Overgrowth with severe developmental delay following IVF/ICSI – A new syndrome? American Journal of Medical Genetics 2006: 140: pp 1312-1315. Shah PS, Wong KY, Merko S, Bishara R, Dunn MS, Asztalos E, Darling PB: Postnatal growth failure in preterm infants ≤ 28 weeks at birth: A measure of ascertainment that is associated with outcome at 18-24 months. Journal of Perinatal Medicine 2006: 34: pp 484-489. Shah PS, Aliwalas LLD, Shah V: Breastfeeding or breast milk for procedural pain in neonates (review), Cochrane Database of Systematic Reviews 2006: 3: CD004950. Silva CT, Daneman A, Navarro OM, Moore AM, Moineddin R, Gerstle JT, Mittal A, Brindle M, Epelman M: Correlation of sonographic findings and outcome in necrotizing enterocolitis. Pediatric Radiology 2007: 37: pp 274-282. Shah V, Farine D, Perlman M: Postnatal laboratory timers of antenatal hyposcemic-ischemic brain damage. Journal of Perinatology 2006: 26: p 204. Shah PS, Kaufman D: Antistaphylococcal immunoglobulins to prevent staphylococcal infection in very low birth weight infants. Cochrane Database of Systematic Reviews 2007: 2: CD006449. Smith GN, Walker MC, Ohlsson A, O’Brien K, Windrim R, for the Canadian Preterm Labour Nitroglycerin Trial Group: Randomized double-blind placebo-controlled trial of transdermal nitroglycerin for preterm labor. American Journal of Obstetrics and Gynecology 2007: 196: pp 37-38. Smith RW, Shah V, Goldman RD, Taddio A: Caregivers’ responses to pain in their children in the emergency department. Archives of Pediatrics and Adolescent Medicine 2007: 161: pp 578-582. Stevens B, Guerriere D, McKeever P, Croxford R, Miller K-L, Watson-MacDonell J, Gibbins S, Dunn M, Ohlsson A, Ray K, Coyte P: Economics of home vs. hospital breastfeeding support for newborns. Journal of Advanced Nursing 2006: 52: pp 233-243. Stevens VJ, McGrath P, Yamada J, Gibbins S, Beyene J, Breau L, Camfield C, Finley A, Franck L, Howlett A, Johnston C, McKeever P, O’Brien K, Ohlsson A: Identification of pain indicators for infants at risk for neurological impairment: A Delphi consensus study. BMCC Pediatrics 2006: 6: p 1. Stevens B, McGrath P, Gibbins S, Beyene J, Breau L, Camfield C, Finley A, Franck L, Howlett A, Johnston C, McKeever P, O’Brien K, Ohlsson A, Yamada J: Determining behavioural and physiological responses to pain in infants at risk for neurological impairment. Pain 2007: 127: pp 94-102. Synnes AR, MacNab Y, Qui Z, Ohlsson A, Gustafson P, Dean CB, Lee SK, and the Canadian Neonatal Network: Neonatal intensive care unit characteristics affect the incidence of severe intraventricular hemorrhage. Medical Care 2006: 44: pp 754-759. Taddio A, Lee C, Yip A, Parvez B, McNamara PJ, Shah V: Intravenous morphine and topical tetracaine for treatment of pain in neonates undergoing central line placement. Journal of the American Medical Association 2006: 295: pp 793-800. Hellmann J: Commentary on ‘Comparison of mothers’ and counsellors’ perceptions of predelivery counselling for extremely premature infants. In: 2006 Yearbook of Neonatal and Perinatal Medicine (Fanaroff AA, Maisels MJ, Stevenson DK, eds). Mosby, St. Louis, 2006. Taddio A, Lee C, Yip A, Parvez B, Shah V: Contact dermatitis and bradycardia in a preterm infant given tetracaine 4% gel. Therapeutic Drug Monitoring 2006: 26: pp 546-549. Hellmann J: Commentary on ‘Medical staff guidelines for periviability pregnancy counselling and medical treatment of extremely premature infants’. In: 2006 Yearbook of Neonatal and Perinatal Medicine (Fanaroff AA, Maisels MJ, Stevenson DK, eds). Mosby, St. Louis, 2006. Teixeira L, McNamara PJ: Enhanced intensive care for the neonatal ductus arteriosus. Acta Paediatrica 2006: 95: pp 394-403. Turner D, Zlotkin SH, Shah PS, Griffiths AM: Omega 3 fatty acids (fish oil) for maintenance of remission in Crohn’s Disease. Cochrane Database of Systematic Reviews 2007: 2: pp CD006320. Vemgal P, Ohlsson A: Interventions for non-oliguric hyperkalaemia in preterm neonates. The Cochrane Database Systematic Reviews 2007: 2: p CD005257. Walsh MC, Yao Q, Horbar JC, Carpenter JH, Lee SK, Ohlsson A, for the NICHD Neonatal Network (NRN), Vermont Oxford Network (VON) and Canadian Neonatal Network (CNN): Paediatrics 2006: 118: pp e1328-e1335. Yi M, Belcastro R, Shek S, Luo D, Post M, Tanswell AK: Fibroblast growth factor-2 and receptor-1_(IIIc) regulate postnatal rat lung cell apoptosis. American Journal of Respiratory Critical Care Medicine 2006: 174: pp 581-589. Yi SL, Kantores C, Belcastro R, Cabacungan J, Tanswell AK, Jankov RP: 8-isoprostane-induced endothelin-1 production by infant rat pulmonary artery smooth muscle cells is mediated by Rhokinase. Free Radical Biology Medicine 2006: 41: pp 942-949. Yudin MH, Shah V, Ohlsson A, Farine D: Are we using the optimal strategy for GBS management in pregnancy? Are we winning the fight against GBS infection? Journal of Obstetrics and Gynaecology of Canada 2006: 28: pp 499-503. BOOKS AND BOOK CHAPTERS Hellmann J, Baylis F: Ethics in perinatal and neonatal medicine. In: Neonatal-Perinatal Medicine: Diseases of the Fetus and Infant, 8th Edition (Fanaroff AA, Martin RJ, eds). Mosby, St. Louis, 2006. Hellmann J: Commentary on ‘Prenatal consultation practices at the border of viability: A regional survey’. In: 2006 Yearbook of Neonatal and Perinatal Medicine (Fanaroff AA, Maisels MJ, Stevenson DK, eds). Mosby, St. Louis, 2006. Hellmann J: Commentary on ‘The ethics of neonatal resuscitation at the margins of viability: Informed consent and outcomes’. In: 2006 Yearbook of Neonatal and Perinatal Medicine (Fanaroff AA, Maisels MJ, Stevenson DK, eds). Mosby, St. Louis, 2006. Hellmann J: Ethical issues in the Neonatal Intensive Care Unit (NICU). In: Resident’s Handbook of Neonatology, 3rd Edition (Kirpalani H, Moore A, Perlman M, eds). B.C. Decker, Hamilton, 2007. Hellmann J: Commentary on ‘Matters of spirituality at the end of life in the pediatric intensive care unit’. In: 2007 Yearbook of Neonatal and Perinatal Medicine (Fanaroff AA, Maisels MJ, Stevenson DK, eds). Mosby, St. Louis, 2007. Hellmann J: Commentary on ‘Parents’ experiences of sharing neonatal information and decisions: Consent, cost and risk’. In: 2007 Yearbook of Neonatal and Perinatal Medicine (Fanaroff AA, Maisels MJ, Stevenson DK, eds). Mosby, St. Louis, 2007. Hellmann J: Commentary on ‘Pediatric resident education in palliative care: A needs assessment’. In: 2007 Yearbook of Neonatal and Perinatal Medicine (Fanaroff AA, Maisels MJ, Stevenson DK, eds). Mosby, St. Louis, 2007. Hellmann J: Commentary on ‘Restriction of ongoing intensive care in neonates: A prospective study’. In: 2007 Yearbook of Neonatal and Perinatal Medicine (Fanaroff AA, Maisels MJ, Stevenson DK, eds). Mosby, St. Louis, 2007. Lodha M, Moore AM: Gastroenterologic disorders. In: Resident’s Handbook of Neonatology, 3rd Edition (Kirpalani H, Moore AM, Perlman M, eds). B.C. Decker, Hamilton, 2007. McNamara PJ, Whyte HE: Principles of neonatal transport. In: Resident’s Handbook of Neonatology, 3rd Edition (Kirpalani H, Moore A, Perlman M, eds). B.C. Decker, Hamilton, 2006. McNamara PJ: The ductus arteriosus. In: Neonatology at a Glance (Lissauer T, ed). Blackwell Publishing, Massachusetts, 2006. McNamara PJ: Cardiac disorders. In: Neonatology at a Glance (Lissauer T, ed.). Blackwell Publishing, Massachusetts, 2006. McNamara PJ: Echocardiography for the neonatologist. In: Neonatology at a Glance (Lissauer T, ed). Blackwell Publishing, Massachusetts, 2006. FUNDING Airway modulation of pulmonary vascular tone during development and following pulmonary hypertension. Belik J. Canadian Institutes of Health Research ($110,108 2004-2009) An integrated, shared data management application for the Toronto Tri-Hospital Respiratory Syncyticial Virus Prophylaxis (RSVP) Program. O’Brien K, Bracht M, Barton P, James AG. Abbott Laboratories Limited ($50,000 2005-2007) Moore AM: Commentary on ‘Institutional Review Board Guidance on Pediatric Research: Missed opportunities’. In: 2006 Yearbook of Neonatal and Perinatal Medicine. Mosby, St. Louis, 2006. An outcome indicator of morbidity in medically vulnerable infants. Dick P, Whyte HE, Guerriere ND, Guttmann A, O’Brien K, To T. Canadian Institutes of Health Research ($216,976 2003-2006) Moore AM: Commentary on ‘Quantifying the Federal Minimal Risk Standard. Implications for pediatric research without a prospect of direct benefit’. Mosby, St. Louis, 2006. Bone mass and growth following necrotizing enterocolitis. Moore AM, Ward WE. SickKids Foundation ($83,236 2004-2006) Moore AM: Editor, Resident’s Handbook of Neonatology, 3rd Edition. (Kirpalani H, Moore AM, Perlman M, eds). B.C. Decker, Hamilton, 2006. CAP follow up at 5 years. Schmidt B, Roberts R, Barrington K, Ohlsson A, Solimano A, Davis P, Doyle L, Tin W. Canadian Institutes of Health Research ($1,744,740 2005-2009) Ohlsson A, Shah V: Evidence-based practice as means for clinical decision making. In: Pain in Neonates and Infants (Anand KJS, Stevens B, McGrath PJ, eds). Elsevier, Philadelphia, 2007. Shah P, Perlman M: Birth asphyxia and multiorgan dysfunction. In: Recent Advances in Paediatrics. (David T, ed). The Royal Society of Medicine Press, UK, 2006. Shah P, Perlman M: Neurology of newborn. In: Handbook of Neonatology, 3rd Edition. (Kirpalani H, Moore AM, Perlman M, eds). B.C. Decker, Hamilton, 2007. Shah P: Classification of newborn. In: Handbook of Neonatology, 3rd Edition. (Kirpalani H, Moore AM, Perlman M, eds). B.C. Decker, Hamilton, 2007. Taddio A, Shah V: Pain management. In: Handbook of Neonatology (Kirpalani H, Moore AM, Perlman M, eds). B.C. Decker, Hamilton, 2007. Young Tai KF: Infection control in the neonatal intensive care unit. In: Handbook of Neonatology, 3rd Edition. (Kirpalani H, Moore AM, Perlman M, eds). B.C. Decker, Hamilton, 2007. Comparison of multi-media computer disk versus face-to-face teaching for inhaled nitric oxide set up in neonates, a randomized controlled trial. Lee K-S, Gibbins S, Whyte H. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($5,000 2006) Continuous infusion of Heparin to prolong the patency of peripherally placed percutaneous central venous catheters (PCVC): A randomized controlled trial. Shah V, Shah PS, Parvez B, Dunn M, Kalyn A, Daneman A, Ohlsson A. SickKids Foundation ($129,960 2002-2006) Early external cephalic version-2 trial. Hutton, EK, Willan AR, Hannah ME, Gafni A, Ohlsson A, Barrett J, Ross SJ, Windrim R, Delisle MF, Carson GD, Dunn MS, Pollard JK, Natale R. Canadian Institutes of Health Research ($2,853,717 2003-2007) Effectiveness of sucrose analgesia in reducing pain responses in infants born to diabetic and non-diabetic mothers: A randomized controlled trial. Taddio A, Ohlsson A, Beyene J, Shah V, Stevens B, Katz J. Canadian Institutes of Health Research ($455,198 2003-2006) Efficacy and safety of targeting lower arterial oxygen saturations to reduce oxygen toxicity and oxidative stress in very preterm infants: Canadian Oxygen Trial (COT). Schmidt B., Roberts R, Whyte R, Azstalos E, Solimano A, Bairam, Chiu A, Clarke M, Harrold J, Kumar M, Moddemann D, Peliowski A, Rabi Y, Roukema H, Sauve R, Seshia M, Shah PS. Canadian Institutes of Health Research ($3,884,860 2006-2011) Evaluation of topical liposomal lidocaine and oral sucrose for treatment of pain in newborn infants undergoing venipuncture: A randomized controlled trial. Taddio A, Shah V, Katz J, Stevens D, Koren G. Canadian Institutes of Health Research ($397,450 2007-2009) Evidence-based practice identification and change (EPIC). Lee SK, Synnes A, Singhal N, Walker CR, Aziz K, Ohlsson A, Baker GR, Barrington KJ, Cronin CM, Hoube J, Langley J, Lee D, Liston R, Macnab YC, Martin C, Matthew JD, Moehr JR, Newman CJ, Pellowski A, Platt RW, Pope S, Sankaran K, Seshia M, Stevens B, Thiessen PN, von Dadelszen P, Whynot E, Whyte RK. Canadian Institutes of Health Research ($717,252 2002-2006) Functional MR Imaging for early detection and physiologic characterization of inflammatory arthritis in a rabbit model. Doria SA, Belik, J, Kassner A, Cheng H-L, Crawley A, Yeung R, Tomlinson G, Gahunia H. RSNA Research & Education Foundation ($29,964 2006-2007) Genome-environment interactions in Type 1 Diabetes. Danska J, MacPherson A, Moore AM. Genome Canada ($94,127 2005-2009) Group in lung development. Tanswell AK, Belik J, Chan A, O’Brodovich H, Post M, Rotin D. Cutz E, Hu J, Kavanagh B, Otulakowski LG, Yeger H. Canadian Institutes of Health Research ($1,377,695 2004-2009) Infant flow biphasic NCPAP vs. infant flow NCPAP for facilitation of extubation in infants less than 1250 gms: A randomized controlled trial. O’Brien K, Shah V, Campbell C. Physicians’ Services Incorporated Foundation ($73,000 2006-2008) Inhibition of apoptosis as a major component of chronic neonatal lung injury. Tanswell AK, Jankov RP. Canadian Institutes of Health Research ($430,045 2007-2012) INTAPP (International trial of antioxidants for the prevention of preeclampsia). Fraser W, Xiong X, Julien P, Thomas N, Magee LA, Ohlsson A. Canadian Institutes of Health Research ($4,923,447 2002-2007) Multiple vs. single courses of antenatal corticosteroids for preterm birth. Murphy KE, Hannah ME, Ohlsson A, Saigal S, Kelly EN, Amankwah KS, Matthews SG, Aghajafari F, Willan A, Dhindsa BL, Gafni A, Hewson S. Medical Research Council of Canada ($4,799,770 2000-2006) Multiple courses of steroids – 5 year follow-up. Asztalos E, Gafni A, Hannah M, Murphy K, Ohlsson A, ross S, Saigal S, Willan A, Matthews S, rovet J, Lee S, Amankwah K, Kelly EN, Lee SK, Delisle M-F, Guselle P, Sananes R. Canadian Institutes of Health Research ($3,585,161 2005-2012) Multicentre randomized trial of HeLP (heat loss prevention) in the delivery room. Vohra S, Reilly MC, Dunn M, Vincer M, Wiebe N, Zayack D, Wimmer J. Canadian Institutes of Health Research ($640,465 2004-2008) Nasal ventilation in premature infants. Kirpalani H, Chiu A, Lemyre B, Millar D, Roberts R, Vaillancourt K, Alvaro R, Connelly R, Howlett A, McNamara P, Peliowski A, Solimano A, Shah P, Aziz K, Zupancic J, Meade M. Canadian Institutes of Health Research ($2,233,100 2006-2009) National health partnership for reducing infections in NICU. Lee SK, Aziz K, Baker GR, Barrington K, Clarke MCW, Cronin CM, Dunn MS, James A, Langley J, Lee DS, Lefebvre F, Merchant P, Ohlsson A, Orrbine E, Peliowski A, Sankaran K, Seshia M, Shaw NT, Synnes AR, Tan-DY CR, Walker CR, Whyte RK. Canadian Institutes of Health Research ($99,999 2005-2008) Novel therapeutic interventions for bronchopulmonary dysplasia: Targeting oxidants and growth factors in the newborn rat lung. Robert P Jankov. Canada Foundation for Innovation New Opportunities Fund/Ontario Research Fund ($297,340 2004-2009) Pharmacology of milrinone in persistent pulmonary hypertension of the newborn. McNamara P, Taddio A. The Hospital for Sick Children – New Ideas Grant ($39,349 2006-2008) Predicting neurobehavioural outcome in preterm infants. Taylor M, Whyte HE, Moore AM. Canadian Institutes of Health Research ($929,910 2007-2012). Protocol for cerebral function monitoring in the neonatal intensive care unit (Protocol). McNamara PJ. Olympic Medical Incorporated ($2,875 2006) Pulmonetics LTV 1200 mechanical ventilator use in neonates less than 5 kg. Belik J, Whyte HE. Pulmonetic Systems Incorporated ($5140.45 2006). Quality of care ICE (QC-ICE) team in neonatalperinatal care. Lee KS, Baker GR, Langley JM, MacNab YC, Moehr JR, Ohlsson A, Stevens B, Magee L, Sauve R, Keller P, Michalos A, Klassen A. Canadian Institutes of Health Research ($935,000 2003-2007) Randomized study of Vasopressin vs Epinephrine in a neonatal model of asphyxial cardiac arrest. McNamara PJ, Kavanagh BP. American Academy of Pediatrics, Neonatal Resuscitation Grant ($28,752 2006-2008) Reactive nitrogen species and smooth muscle growth regulators in the pathogenesis of newborn pulmonary hypertension. Jankov RP. Career Development Award, Canadian Child Health Clinician Scientist Program/Canadian Institutes of Health Research ($280,000 2004-2008) Reactive nitrogen species and macrophages as mediators of pulmonary hypertension in the newborn rat. Jancov RP. The Physicians’ Services Incorporated Foundation ($154,000 2005-2006) Thyroid hormone and infant visual processing development. Rovet J, Westall C, Asztalos E. Canadian Institutes of Health Research ($616,540 2001-2006) Regulation of normal and oxygen-mediated aberrant postnatal lung growth by growth factors. Tanswell K. Canadian Institutes of Health Research ($799,686 2005-2010) Thyroid hormone insufficiencies and visual perception deficits in preterm infants. Rovet J, Asztalos E, Westall C, Heon E. March of Dimes ($253,083 2004-2007) Rho-kinase inhibitor as therapy for chronic neonatal pulmonary hypertension. Jankov RP, McNamara PJ. Canadian Institutes of Health Research Operating Grant ($323,592 2007-2010) The developing lung: Cellular and molecular physiology. Tanswell AK. Medical Research Council/Canadian Institutes of Health Research Group Grant ($1,285,443 2004-2009) Role of peroxynitrite and macrophages in newborn pulmonary vascular remodeling. Jankov RP. Canadian Institutes of Health Research ($285,888 2005-2008) The twin birth study: A multicentre randomised controlled trial comparing planned caesarean section with planned vaginal birth for twins at 32-38 weeks gestation. Barrett J, Hannah M, Willan A, Ross S, Hutton E, Allen A, Joseph KS, Armson A, Farrell S, Ohlsson A, Asztalos E, Gafni A, Okun N, Leduc L. Canadian Institutes of Health Research ($8,608,045 2003-2011) Role of reactive oxygen/nitrogen species in normal and aberrant postnatal lung growth. Tanswell AK. Canadian Institutes of Health Research ($708,400 2004-2009) Steroids in immune mediated congenital complete atrio-ventricular heart block (CAVB): Do they affect brain development? Kelly EN, Sananes R, Jaeggi ET. The Physicians’ Services Incorporated Foundation ($42,000 2005-2006) Strategic training initiative in research in reproductive health sciences. Fraser WD, Campbell M, Collet J, Davidge S, De Koninck M, Dodds L, Dodin S, Fisher W, Gagnon R, Grant A, HatemAsmar M, Heaman M, Joseph K, Klein M, Labrecque M, Lambert R, Lee S, Letourneau L, Leung P, Lye S, McCoshen J, Moutquin J, Nisker J, Ohlsson A, Pierson R, Sauve R, Smith G, Von Dadelszen P, Walker M, Wood S. Canadian Institutes of Health Research ($1,800,000 2002-2008) Surveillance of kernicterus in Canada. Sgro M, Campbell D, Shah V. Ontario Federation for Cerebral Palsy ($25,100 2007-2008) Survey of the attitudes and practices of physicians and mothers regarding analgesia during routine immunization in childhood. Taddio A, Shah V, Ipp M, Sgro M. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($5,050 2005-2006) Survey of the attitudes and practices of physicians and mothers regarding analgesia during routine immunization in childhood. Taddio A, Shah V, Ipp M, Sgro M. Canadian Pain Society ($1,000 2005-2006) Survey of mode of delivery and maternal and perinatal outcomes in Canada. Lee S, Armson A, Allen V, Andruschuk J, Attenborough R, Bottomley J, da Silva O, Frick C, Heaman M, Murphy P, Pasquier J-C, Bujold E, Crane J, Dumont A, Janssen P, Klein M, Liu S, Moutquin J-M, O’Brien B, Ohlsson A, Platt R, Sauve R, von Dadelszen P, Walker M. Canadian Institutes of Health Research/World Health Organization ($250,000 2007-2009) Using telemedicine strategies to prevent avoidable blindness in retinopathy of prematurity. Kertes P, Gibbins S, Asztalos E, Ells A, Maddelena P, Brent M, Lam WC, Devenyi R, Jefferies A. EA Baker Foundation ($40,000 2004-2006) Using telemedicine strategies to prevent avoidable blindness in retinopathy of prematurity: Reliability and measures of pain. Kertes P, Gibbins S, James AG. EA Baker Foundation of the National Institute of the Blind ($40,000 2006-2008) Ventilator-induced lung injury: Linking gene expression, physiology and outcome. Kavanagh B, Post M, Belik J. Canadian Institutes of Health Research Operating Grant ($102,530 2003-2006) Vasopressin in paediatric shock. Choong K, Bohn D, Joffe A, Menum K, McNamara P, Litalien C. Canadian Intensive Care Foundation ($20,000 2005-2006) Vasopressin in paediatric shock. Choong K, Bohn D, Joffe A, Menum K, McNamara P, Litalien C. Laerdal Society ($12,730 2005-2006) Vasopressin in paediatric shock. Choong K, Bohn D, Joffe A, Menum K, McNamara P, Litalien C. Canadian Institutes of Health Research ($136,792 2006-2007) Vasopressin in paediatric shock. Choong K, Bohn D, Joffe A, Menum K, McNamara P, Litalien C. Heart and Stroke Foundation of Canada ($61,623 2006-2007) DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 73 Nephrology DIVISIONAL OVERVIEW The Nephrology Division in the past year has been very ably led by Drs. Rosenblum and Langlois, while Dr. Geary was on sabbatical leave. The leadership change has not resulted in any change in the divisional activity, and clinical care and basic research continue as beacons of our success. There is also a commitment to increase clinical research productivity with specific focus on Nephrotic Syndrome in Children, Prevention of the Complications of Chronic Kidney Disease in Children, and Management of Congenital Renal Malformations. The training program was superbly led by Dr. Harvey, and culminated in successful accreditation for another seven years. Dr. Lisa Robinson was instrumental in development of the “Kids Science” program designed to provide at-risk youth in Ontario with exposure to careers in science and technology. The program is funded by YSTOP (Youth Science and Technology Outreach Program) through the Ministry of Research and Innovation, Government of Ontario, with a grant for $150,000 over 3 years (2006-2009). One of the components of the program was a two day science symposium (Science Extravaganza) which included state-of-the-art lectures, a luncheon hosted by various scientific and health-care staff, a speech by the Premier of Ontario, and an evening banquet. A 10-year review of the division was laudatory, and Dr. Geary was reappointed as Division Chief for another five-year term. HONOURS AND AWARDS Licht C: Associate Scientist, Cell Biology Program, Research Institute, The Hospital for Sick Children. The Interface of the Complement and the Coagulation System in Patients with aHUS, 2006. Rosenblum ND: Canada Research Chair in Developmental Nephrology (Tier 1), Canadian Institutes of Health Research, 2005-2012. Rosenblum ND: Outstanding Science Award, American Pediatric Society, 2006. PUBLICATIONS Dr. Denis Geary Alexander RT, Langlois V, Tellier R, Robinson L, Hebert D: The prevalence of BK viremia and urinary viral shedding in a pediatric renal transplant population: A single-center retrospective analysis. Pediatric Transplanation 2006: 10: pp 586-592. Cook A, Khoury A, Kader K, Hebert D, Navarro O, Pippi-Salle J, Farhat W: Does peak systolic velocity correlate with renal artery stenosis in a pediatric renal transplant population? Pediatric Transplantation 2006: 10: pp 608-612. Fluss JE, Deveber G, Geary DF: Cerebral sinovenous thrombosis and idiopathic nephrotic syndrome in childhood: Report of four new cases and review of the literature. European Journal of Pediatrics 2006: 165: pp 709-716. de Mezerville MH, Tellier R, Richardson S, Hebert D, Doyle J, Allen U: Adenoviral infections in pediatric transplant recipients: A hospital-based study. Pediatric Infectious Diseases 2006: 9: pp 815-818. Gill PS, Rosenblum ND: Control of murine kidney development by Sonic Hedgehog and its GLI effectors. Cell Cycle 2006: 5: pp 1426-1430. Durkan A, Alexander RT, Liu GY, Rui M, Fernia G, Robinson L: Expression and targeting of CX3CL1 (Fractalkine) in renal tubular epithelial cells. Journal of the American Society of Nephrology 2007: 18: pp 74-83. Durkan A, Keating L, Vigneux A, Geary D: The use of darbepoetin in infants with chronic renal impairment. Pediatr Nephrol. 2006: 21: pp 694-697. Division Head Dr. Denis F. Geary Dr. Lisa A. Robinson Honorary Dr. Norman D. Rosenblum Dr. Gerald S. Arbus Full-Time Dr. Elizabeth A. Harvey Dr. Diane Hébert Dr. Valerie Langlois Dr. Christoph Licht Dr. Tino D. Piscione Part-Time Dr. Marion Cummings Dr. Rachel Pearl Adjunct Dr. Leo Levin Dr. Meeta Mallik Dr. Rola Saqan Dr. Aoife Waters Clinical Fellows Dr. Mohamed Al Maghrabi Research Fellow Dr. Thoraya Al Maslamani Dr. Todd Alexander Dr. Indra Ganesan Subspecialty Residents Dr. Rajesh Krishnan Dr. Dana Al-Nabhani Hothi DK, St George-Hyslop C, Geary D, Bohn D, Harvey E: Continuous renal replacement therapy (CRRT) in children using the AQUARIUS. Nephrology Dialysis Transplantation 2006: 21: pp 2296-2300. Hothi KD, Geary DF, Fisher L, Chan CT: Short-term effects of nocturnal haemodialysis on carnitine metabolism. Nephrology Dialysis Transplantation 2006: 21: pp 2637-2641. Dr. Susan Koshy Dr. Katherine Martin Dr. Lindsay Teskey Clinical Research Associates Michelle Frieling Laura Keating Administrative Staff Dana Debernyj Juliet Rodrigo Bernard Singh Marisa Tuzi Humar A, Hebert D, Davies HD, Humar A, Stephens D, O’Doherty B, Allen U: A randomized trial of ganciclovir versus ganciclovir plus immune globulin for prophylaxis against Epstein-Barr virus related posttransplant lymphoproliferative disorder. Transplantation 2006: 81: pp 856-861. Jozsi M, Heinen S, Hartmann A, Ostrowicz C, Halbich S, Richter H, Kunert A, Licht C, Saunders RE, Perkins SJ, Zipfel P, Skerka C: C-terminal mutations of the immune regulatory factor H as described in patients with atypical hemolytic uremic syndrome. Journal of the American Society of Nephrology 2006: 17: pp 170-177. Licht C, Heinen S, Jozsi M, Loschmann I, Saunders RE, Perkins SJ, Waldherr R, Skerka C, Kirschfink M, Hoppe B, Zipfel PF: Deletion of a single amin acid (K224) in the regulatory domain of the immune inhibitor factor H reveals a novel pathomechanism for membranoproliferative glomerulonephritis type II. Kidney International 2006: 70: pp 42-50. McKenna AM, Keating LE, Vigneux A, Stevens S, Williams A, Geary DF: Quality of life in children with chronic kidney disease-patient and caregiver assessments. Nephrology Dialysis Transplantation 2006: 21: pp 1899-1905. Sidhu G, Beyene J, Rosenblum ND: Outcome of isolated antenatal hydronephrosis: A systematic review and meta-analysis. Pediatric Nephrology 2006: 21: pp 218-224. Sweeney C, Geary DF, Hebert D, Robinson L, Langlois V: Outpatient pediatric renal transplant biopsy – Is it safe? Pediatric Transplantation 2006: 10: pp 159-161. Turner D, Schreiber R, Grant D, Hebert D, Sherman PM: Hemolytic uremic syndrome after pediatric liver transplantation. Journal of Pediatric Gastroenterology and Nutrition 2006: 43: pp 109-112. 76 DEPARTMENT OF PAEDIATRICS von Schnakenburg C, Feneberg R, Plank C, Zimmering M, Arbeiter K, Bald M, Fehrenbach H, Griebel M, Licht C, Konrad M, Timmermann K, Kemper MJ for the APN (Arbeitsgemeinschaft Paediatrische Nephrologie): Percutaneous endoscopic (PEG) or open gastrostomy in 30 children on peritoneal dialysis. Peritoneal Dialysis International 2006: 26: pp 69-77. Wong H, Laberge R, Harvey E, Filler G: Preventing sensitization with mycophenolate mofetil in a pediatric kidney recipient. Pediatric Transplantation 2006: 10: pp 367-370. Wong H, Hadi M, Geary D, Khoury T, Rubin B: Management of severe hypertension in a child with tuberous sclerosis-related major vascular abnormalities. J Hypertens 2006 Mar: 24(3): pp 597-599. Zipfel PF, Misselwitz J, Licht C, Skerka C: The role of defective complement control in hemolytic uremicsyndrome. Seminars in Thrombosis and Hemostasis 2006: 32: pp 146-154. FUNDING Canada Research Chair in Developmental Nephrology, Tier I. Rosenblum ND. Canada Research Chairs Program ($200,000/year 2005-2012) Canadian Child Health Clinician-Scientist Program Rosenblum ND. Canadian Institutes of Health Research ($300,000/year 2002-2008) Canadian Paediatric Clinician Scientist Program. Rosenblum ND. SickKids Foundation ($300,000/year 2002-2008) Canadian Paediatric Clinician Scientist Program. Rosenblum ND. B.C. Research Institute for Children’s and Women’s Health ($150,000/year 2002-2008) Canadian Paediatric Clinician-Scientist Program. Rosenblum ND. B.C. Children’s Hospital Foundation ($150,000/year 2002-2008) Cellular regulation of Fractalkine. Lisa Robinson: The Heart and Stroke Foundation of Ontario, Grant-in-Aid ($171,944 2005-2007) Control of kidney morphogenesis by sonic hedgehog and its signaling effectors. Rosenblum ND. Canadian Institutes of Health Research ($134,419/year 2006-2009) Evaluation of photograph database use in children. Geary DF. Genzyme Inc. ($13,000 2006-2008) Kids Science. Robinson L. Ministry of Research and Innovation, Youth Science and Technology Outreach Program ($150,000 2006-2009) Molecular mechanisms that control branching morphogenesis in the normal and dysplastic kidney. Rosenblum ND. Canadian Institutes of Health Research ($148,700/year 2003-2008) Role of the Notch pathway in kidney development. Piscione TD. SickKids Research Institute ($101,500 2006-2008) Role of the Notch pathway in kidney development. Piscione TD. Department of Paediatrics, The Hospital for Sick Children ($37,500 July 2006) Steroid-induced Osteoporosis in the Paediatric Population – Canadian Incidence Study (The STOPP Study). Ward L, Alos N, Atkinson SA, Barr RD, Chanoine J-P, Clarson CL, Clifford TJ, Couch, RM, Cummings EL, Feber J, Filler G, Gaboury I, Glorieux FH, Grant RM, Halton JM, Hay JA, Huber AM, Lang BA, Leblanc CM, Matzinger MAE, Moher D, Rauch F, Rodd CJ, Stephure DK, Taback SP, Webber CE, Hebert D. Canadian Institutes of Health Research ($598,026 2003-2008) The role of Notch1 in kidney development. Piscione TD. The Kidney Foundation of Canada ($149,610 2004-2007) The role of slit/robo signaling in renal Inflammation. Robinson L. Canada Research Chairs, Tier 2 ($500,000 2004-2009) The role of slit/robo signaling pathways in kidney inflammation. Robinson L. Early Researcher Award, Government of Ontario ($100,000 2006-2008) Control of branching morphogenesis in the normal and dysplastic kidney. Rosenblum ND. Canadian Institutes of Health Research ($147,000/year 2003-2008) ANNUAL REPORT 2006-07 Dr. Christoph Licht Hu MC, Mo R, Bhella S, Wilson CW, Chuang P-T, Hui C-C, Rosenblum ND: GLI3-dependent transcriptional repression of Gli1 and Gli2 and kidney patterning genes disrupts renal morphogenesis. Development 2006: 133: pp 569-578. Division Head Dr. O. Carter Snead III Full-Time Dr. Rand Askalan Dr. Brenda Banwell Dr. Gabrielle deVeber Dr. Elizabeth Donner Dr. Cecil Hahn Dr. Daune MacGregor Dr. Berge Minassian Dr. Teesta Soman Dr. Ingrid Tein Dr. Jiri Vajsar Dr. Shelly Weiss Part-Time Dr. David Berbrayer Dr. Joseph Bruni Dr. Pamela Cooper Dr. Jonathan Gladstone Dr. Jeffrey Kobayashi Dr. Betty Koo Dr. Wayne Langburt Dr. Shanna Lindsay Dr. William Logan Dr. John Lovering Dr. Gilbert Miller Dr. Peter Rumney Honorary Dr. Douglas McGreal Dr. E. Gordon Murphy Dr. Diann Superina-Bell Scientific Staff Dr. Miguel Cortez Dr. Maureen Lovett Dr. James MacFarlane Dr. Hiroshi Otsubo Dr. Elizabeth Pang Dr. Mary Lou Smith Dr. Margot Taylor Dr. Jose Luis Perez Velazquez Neurology DIVISIONAL OVERVIEW The major foci of the clinical activities of the Division of Neurology are epilepsy, stroke, headache, neurometabolic disease, neuromuscular disease, sleep disorders, movement disorders, multiple sclerosis, functional neuroimaging and neurointensive care. Dr. Rand Askalan and team members The Division of Neurology has a Royal College accredited training program in Child Neurology as well as postdoctoral training programs in epilepsy and stroke. In addition, there are a number of graduate students and postdoctoral fellows in clinical neurophysiology and molecular and cellular neurobiology. The research foci of the Division of Neurology is epilepsy (both clinical and basic research), magnetoencephalography, stroke, dyslexia, burden of disease and quality of life, muscular dystrophy, multiple sclerosis, fatty acid oxidation disorders, and molecular and cellular neurobiology. Trainees Dr. Kristen Wentlandt Dr. Reem Al-Khater Dr. Naila Makhani Dr. Jorina Elbers Dr. Emily Tam Dr. David Callen Dr. Rand Askalan Departmental Fellows Epilepsy Fellows Dr. Juan Pablo Appendino Dr. Derrick Chan Dr. Cristina Go Dr. Klaus Werner Dr. Ismail Mohamed Stroke Fellows Dr. Erick Sell Dr. Adam Kirton Dr. Mahendranath Moharir Dr. M. Tan Research Fellows Dr. Katsumi Imai Dr. Ayataka Fujimoto Self-Funded Fellow Dr. Terrence Thomas Administrative Staff Erin Crawford Maureen Hodge Fatima Ledo Michelle Lo Marilyn McLaughlin Susy O’Neill Wendy Ricketts Laura Toth Barbara Zimnowodzki Appendino JP: William J. Logan Award for Excellence in Clinical Teaching, Division of Neurology, The Hospital for Sick Children, 2007. Banwell B: Canada’s Top 40 Under 40 Recipient, 2006. Banwell B: American Academy of Neurology Leadership Development Program (Selected), 2006. Banwell B: Women Against Multiple Sclerosis Award – Multiple Sclerosis Society of Canada, 2007. Cortez M: William A. Hawke Award for Excellence in Clinical Teaching, Division of Neurology, The Hospital for Sick Children, 2007. deVeber G: Canadian Paediatric Society “Geoffrey C. Robinson Award”, June 2007. Kirton A: 2007 Prichard Day Prize Winner, “Motor Outcome and Reorganization Prediction in Childhood Stroke Using Diffusion MR and TMS”. MacGregor DL: 7 Year Volunteer Long Service Award, Bloorview Children’s Hospital Foundation, 2007. Minassian B: Canada Research Chair in Paediatric Neurogenetics, 2006. Minassian B: Dreifuss-Penry Epilepsy Award, American Academy of Neurology, 2007. Tein I: Elected Member, International Child Neurology Association Executive Board. Tein I: Appointed Chair, Scientific Program Committee, XIth International Child Neurology Congress, Cairo, Egypt, 2010. PUBLICATIONS Ackerley CA, Minassian BA: Animal models of Lafora Disease. Microscopy and Microanalysis 2006: 12: pp 12-13. Akiyama T, Otsubo H, Ochi A, Galicia EZ, Weiss SK, Donner EJ, Rutka JT, Snead OC III: Topographic movie of ictal high-frequency oscillations on the brain surface using subdural EEG in neocortical epilepsy. Epilepsia 2006: 47: pp 1953-1957. Andrade DM, Scherer SW, Minassian BA: Protein therapy for Unverricht-Lundborg disease using cystatin B transduction by TAT-PTD. Is it that simple? Epilepsy Research 2006: 72: pp 75-79. 80 DEPARTMENT OF PAEDIATRICS Askalan R, deVeber G, Ho M, Ma J, Hawkins C: Astrocytic-inducible nitric oxide synthase in the ischemic developing human brain. Pediatric Research 2006: 60: pp 439-445. Bercovici E, Cortez MA, Snead OC III: 5-HT2 modulation of AY-9944 induced atypical absence seizures. Neuroscience Letters 2007: 418: pp 13-17. Aviv RI, Benseler SM, deVeber G, Silverman ED, Tyrrell PN, Tsang LM. Armstrong D: Angiography of primary central nervous system angiitis of childhood: Conventional angiography versus magnetic resonance angiography at presentation. American Journal of Neuroradiology 2007: 28: pp 9-15. Braun KP, Kappelle LJ, Kirkham FJ, deVeber G: Diagnostic pitfalls in paediatric ischaemic stroke. Developmental Medicine and Child Neurology 2006: 48: pp 985-990. Banwell B, Shroff M, Ness JM, Jeffrey D, Schwid S, Weinstock-Guttman B: RI features of pediatric multiple sclerosis. Neurology 2007: 68: pp S46-S53. Barcelo-Coblijn G, Murphy EJ Mills K, Winchester B, Jakobs C, Snead OC, Gibson KM: Lipid abnormalities in succinate semialdehyde dehydrogenase (Aldh5a1(-/-)) dehydrogenase mouse brain provide additional evidence for myelin alterations. Biochimica et Biophysica ACTA/General Subjects (Amsterdam) 2007: 1772: pp: 556-562. Bayless SJ, Gaetz W, Cheyne DO, Taylor MJ: Spatial and temporal localization of feedback processing using MEG and event-related synthetic aperture magnetometry. Neuroscience Letters 2006: 410: pp 31-36. Belman AL, Chitnis T, Renoux C, Waubant E, Banwell B: Challenges in the classification of pediatric multiple sclerosis and future directions. Neurology 2007: 68: pp S70-S74. Benifla M, Otsubo H, Ochi A, Snead OC III, Rutka JT. Multiple subpial transactions in pediatric epilepsy: Indications and outcomes. Child’s Nervous System 2006: 22: pp 992-998. Benifla M, Otsubo H, Ochi A, Weiss S, Donner E, Shroff M, Chuang S, Hawkins C, Drake JM, Elliott I, Smith ML, Snead OC III, Rutka JT: Temporal lobe surgery for intractable epilepsy in children: An analysis of outcomes in 126 children. Neurosurgery 2006: 59: pp 1203-1214. Benifla M, Rutka JT, Logan WJ, Donner EJ: Vagal nerve stimulation for refractory epilepsy in children: Indications and experience at The Hospital for Sick Children. Child’s Nervous System 2006: 22: pp 1018-1026. Bentin S, Taylor MJ, Rousselet GA, Itier RJ, Caldara R, Schyns PG, Jacques C, Rossion: Much ado about nothing: Controlling interstimulus perceptual variance does not abolish N170 face sensitivity. Nature Neuroscience 2007: 10: pp 801-802. ANNUAL REPORT 2006-07 Braun KP, Rafay MF, Uiterwaal CS, Pontigon AM, deVeber G: Mode of onset predicts etiological diagnosis of arterial ischemic stroke in children. Stroke 2007: 38: pp 298-302. Elbers JM, Bitnun A, Richardson SE, Ford-Jones EL, Tellier R, Wald RM, Petric M, Kolski H, Heurter H, MacGregor D: A 12-year prospective study of childhood herpes simplex encephalitis: Is there a broader spectrum of disease? Pediatrics 2007: 119: pp 399-407. Feasby T, Banwell B, Benstead T, Bril V, Brouwers M, Freedman M: Guidelines on the use of intravenous immune globulin for neurologic conditions. Transfusion Medicine Reviews 2007: 21: pp S57-S107. Fluss J, Geary D, deVeber G: Cerebral sinovenous thrombosis and idiopathic nephritic syndrome in childhood: Report of 4 new cases and review of literature. European Journal of Pediatrics 2006: 165: pp 709-716. Gallagher S, Weiss SK, Oram Cardy J, Humphries T, Harman KE, Menascu S: Efficacy of very high dose steroid treatment in a case of LandauKleffner syndrome. Developmental Medicine and Child Neurology 2006: 48: pp 766-769. Georgiou D-M, Nicholaou P, Chitayat D, Koutsou P, Babul-Hirji R, Vajsar J, Murphy J, Christodoulou K: A novel GDAP1 mutation 439delA is associated with autosomal recessive CMT disease. Canadian Journal of Neurological Sciences 2006: 33: pp 311-316. Gladstone JP: An approach to the patient with painful ophthalmoplegia – focus on Tolosa-Hunt syndrome. Current Pain and Headache Reports 2007: 11: pp 317-325. Grodin R, Chuang S, Otsubo H, Holowka S, Snead OC III, Raybaud C, Rutka JT: The role of magnetoencephalography in pediatric epilepsy surgery. Child’s Nervous System 2006: 22: pp 779-785. Hahn JS, Pohl D, Rensel M, Rao S, Banwell B: Differential diagnosis and evaluation in pediatric multiple sclerosis. Neurology 2007: 68: pp S13-S22. Dr. Cecil Hahn with Epilepsy fellows and staff HONOURS AND AWARDS Harvey CG, Menon SD, Stachowiak B, Noor A, Proctor A, Mensah AK, Mnatzakanian GN, Alfred SE, Guo R, Scherer SW, Kennedy JL, Roberts W, Srivastava AK, Minassian BA, Vincent JB: Sequence variants within exon 1 of MECP2 occur in females with mental retardation. American Journal of Medical Genetics Part B: Neuropsychiatric Genetics 2007: 144: pp 355-360. Hashizume A, Iida K, Shirozu H, Hanaya R, Kiura Y, Kurisu K, Otsubo H: Gradient magneticfield topography for dynamic changes of epileptic discharges. Brain Research 2007: 1144: pp 175-179. Hune SY, deVeber GA, MacGregor DL, Dirks PB, Yu HW, Soman TB, Allen A, Golomb MR: Nursing issues in caring for children with moyamoya vasculopathy and their families. Journal of Neuroscience Nursing 2007: 39: pp 33-39. Itier RJ, Herdman AT, George N, Cheyne DO, Taylor MJ: Inversion and contrast-reversal effects on face encoding and recognition assessed by MEG. Brain Research 2006: 1115: pp 108-120. Kadis D, Iida K, Kerr EN, Logan WJ, McAndrews MP, Ochi A, Otsubo H, Rutka J, Snead OC III, Weiss SK, Smith ML: Intrahemispheric reorganization of language in children with medically intractable epilepsy of the left hemisphere. Journal of the International Neuropsychological Society 2007: 13: pp 505-516. Kirton A, deVeber G: Ischemic stroke complicating pediatric cardiovascular disease. Nature Clinical Practice. Cardiovascular Medicine 2007: 4: pp 163-166. Kirton A, deVeber G: Perinatal ischemic stroke. Stroke Review 2006: 10: pp 38-47. Kirton A, Hill MD, deVeber G: Arterial ischemic strokes in neonates and children: Review and current issues. Current Pediatric Reviews 2006: 2: pp 301-314. Kirton A, Schechter T, Brandao L, Chan A, Williams S, MacGregor D, Pontigon A, deVeber G: Hemorrhagic transformation of arterial ischemic strokes in children. Stroke 2007: 38: pp 581. 82 DEPARTMENT OF PAEDIATRICS Kirton A, Shroff M, Visvanathan T, deVeber G: Quantified corticospinal tract diffusion restriction predicts neonatal stroke outcome. Stroke 2007: 38: pp 974-980. Kossoff EH, Pyzik PL, Rubenstein JE, Bergqvist AGC, Buchhalter JR, Donner EJ, Nordi Jr DR, Wheless JW: Combined ketogenic diet and vagus nerve stimulation: Rational polytherapy? Epilepsia 2007: 48: pp 77-81. Krupp LB, Banwell B, Tenembaum S: Consensus definitions proposed for pediatric multiple sclerosis and related disorders. Neurology 2007: 68: pp S7-S12. Kuban KC, O’Shea M, Allred E, Leviton A, Gilmore H, DuPlessis A, Krishnamoorthy K, Hahn C, Soul J, O’Connor SE, Miller K, Church PT, Keller C, Bream R, Adair R, Miller A, Romano E, Bassan H, Kerkering K, Engelke S, Marshall D, Milowic K, Wereszczak J, Hubbard C, Washburn L, Dillard R, Heller C, Burdo-Hartman W, Fagerman L, Sutton D, Karna P, Olomu N, Caldarelli L, Oca M, Lohr K, Scheiner A: Video and CD-ROM as a training tool for performing neurologic examinations of 1-yearold children in a multicenter epidemiologic study. Journal of Child Neurology 2006: 20: pp 829-831. Lamhonwah AM, Hawkins C, Mai L, Tein I: Expression patterns of organic cation/carnitine transporter family in adult murine brain. Brain and Development. June 16, 2007 [Epub ahead of print]. Lamhonwah AM, Tein I. Novel localization of OCTN1, an organic cation/carnitine transporter, to mammalian mitochondria. Biochemical & Biophysical Research Communications 2006: 345: pp 1315-1325. Latinus M, Taylor MJ: Face processing stages: Impact of difficulty and the separation of effects. Brain Research 2006: 1123: pp 179-187. Leshchenko Y, Likhodii S, Yue W, Burnham WM, Perez Velazquez JL: Carbenoxolone does not cross the blood brain barrier: An HPLC study. BMC Neuroscience 2006: 7: p 3. Loh A, Soman T, Brian J, Bryson SE, Roberts W, Szatmari P, Smith IM, Zwaigenbaum L: Stereotyped motor behaviours associated with autism in highrisk infants: A pilot videotape analysis of a sibling sample. Journal of Autism and Developmental Disorders 2007: 37: pp 25-36. ANNUAL REPORT 2006-07 Lohi H, Turnbull J, Zhao XC, Pullenayegum S, Ianzano L, Yahyaoui M, Mikati MA, Franceschetti S, Zara F, Minassian BA: Genetic diagnosis in Lafora Disease: Genotype-phenotype correlations and diagnostic pitfalls. Neurology 2007: 68: pp 996-1001. MacAllister WS, Boyd JR, Holland NJ, Milazzo MS, Krupp LB for the International Pediatric MS Study Group: The psychosocial consequences of pediatric multiple sclerosis. Neurology 2007: 68: pp S66-S69. MacGregor D, Tallett S, MacMillan S, Gerber R, O’Brodovich H: Clinical and education workload measurements using personal digital assistant based software. Pediatrics 2006: 118: pp e985-991. Maegawa G, Stockley T, Tropak M, Banwell B, Blaser S, Kok K, Giugliani R, Mahuran D, Clarke J: The natural history of juvenile or subacute GM2 gangliosidosis: 21 new cases and literature review of 134 previously reported. Pediatrics 2007: 118: pp 1550-1562. Mohamed I, Otsubo H, Pang E, Chuang SH, Rutka JT, Dirks P, Weiss SK, Snead OC III: Magnetoencephalographic spike sources associated with auditory auras in pediatric localization-related epilepsy. Journal of Neurology, Neurosurgery and Psychiatry 2006: 77: pp 1256-1261. Mohamed IS, Otsubo H, Donner EJ, Ochi A, Sharma R, Drake J, Rutka JT, Chuang SH, Holowka S, Snead III OC: Magnetoencephalography for surgical treatment of refractory status epilepticus. Acta Neurologica Scandivavica 2007: 115: pp 29-36. Mohamed IS, Otsubo H, Shroff M, Donner E, Drake J, Snead III OC: Magnetoencephalography and diffusion tensor imaging in gelastic seizures secondary to a cingulate gyrus lesion. Clinical Neurology and Neurosurgery 2007: 109: pp 182-187. Ness JM, Chabas D, Sadovnick AD, Pohl D, Banwell B, Weinstock-Guttman B: Clinical features of children and adolescents with multiple sclerosis. Neurology 2007: 68: pp S37-S45. O’Brodovich H, Beyene J, Tallett S, MacGregor D, Rosenblum ND: Performance of a career development and compensation program at an academic health science centre. Pediatrics 2007: 119: pp e791-e797. Ochi A, Otsubo H, Donner E, Elliott I, Iwata R, Funaki T, Akizuki Y, Akiyama T, Rutka JT, Snead OC: Dynamic changes of ictal high-frequency oscillations in neocortical epilepsy: Using multiple band frequency analysis. Epilepsia 2007: 48: pp 286-296. O’Connor K, McLaughlin KA, De Jager PL, Chitnis T, Bettelli E, Xu C, Robinson WH, Cherry SV, Bar-Or A, Banwell B, Fukaura H, Tenembaum S, Wong SJ, Tavakoli NP, Idrissova Z, Viglietta V, Rostasy K, Pohl D, Dale RC, Freedman M, Steinman L, Buckle GJ, Kuchroo VK, Hafler DA, Wucherpfennig KW: Self-assembling antigen tetramers permit discrimination of autoantibodies to folded and denatured self-antigens in demyelinating diseases. Nature Medicine 2007: 13: pp 211-217. Oishi M, Otsubo H, Iida K, Suyama Y, Ochi A, Weiss SK, Xiang J, Gaetz W, Cheyne D, Chuang SH, Rutka JT, Snead OC: Presurgical stimulation of intracerebral epileptiform discharges. Synthetic aperture magetometry virtual sensor analysis of interictal magnetoencephalography data. Journal of Neurosurgery 2006: 105: pp 41-49. Payne ET, Rutka JT, Ho TK, Halliday WC, Banwell B: Treatment leading to dramatic recovery in acute hemorrhagic leukoencephalitis. Journal of Child Neurology 2007: 22: pp 109-113. Perez Velazquez, JL: Brain research: a perspective from the coupled oscillators field NeuroQuantology 2006: 4: pp 155-165. Perez Velazquez JL: Functional contribution of specific brain areas to absence seizures: Role of thalamic gap-junctional coupling. European Journal of Neuroscience 2006: 23: pp 489-496. Perez Velazquez JL, Garcia Dominguez L, Wennberg R: Complex phase synchronization in epileptic seizures: Evidence for a devil’s staircase. Physical Review E: Statistical, Non-linear & Soft Matter Physics 2007: 75: p 011922 [Epub]. Perez Velazquez JL, Kokarovtseva L, Sarbaziha R, Jeyapalan Z, Leschenko Y: Role of gap junctional coupling in astrocytic networks in the determination of global ischaemia-induced oxidative stress and hippocampal damage European Journal of Neuroscience 2006: 23: pp 1-10. Petel-Galil Y, Benteer B, Galil YP, Zeev BB, Greenbaum I, Vecsler M, Goldman B, Lohi H, Minassian BA, Gak E: Comprehensive diagnosis of Rett’s syndrome relying on genetic, epigenetic and expression evidence of deficiency of the methyl-CpG-binding protein 2 gene: Study of a cohort of Israeli patients. Journal of Medical Genetics 2006: 43: p e56. Picton, TW, Taylor MJ: Electrophysiological evaluation of human brain development. Developmental Neuropsychology 2007: 31: pp 251-280. Pohl D, Waubant B, Chabas D, Chitnis T, Weinstock-Guttman B, Tenembaum S: Treatment of pediatric multiple sclerosis and variants. Neurology 2007: 68: pp S54-S65. RamachandranNair R, Otsubo H, Shroff MM, Ochi A, Weiss SK, Rutka JT, Snead OC III: MEG predicts outcome following surgery for intractable epilepsy in children with normal or non-focal MRI findings. Epilepsia 2007: 48: pp 149-157. Rodan LH, Aviv RI, Sahlas DJ, Murray BJ, Gladstone JP, Gladstone DJ. Seizures during stroke thrombolysis heralding dramatic neurologic recovery. Neurology 2006: 67: pp 2048-2049. Rubenstein S, Malone MA, Roberts W, Logan WJ: Placebo-controlled study examining effects of selegiline in children with attention deficit hyperactivity disorder. Journal of Child and Adolescent Psychopharmacology 2006: 16: pp 404-415. Saengpattrachai M, Ray PN, Hawkins CE, Berzen A, Banwell B: Grandpa and I have dystrophinopathy?: Approach to asymptomatic hyperCKemia. Pediatric Neurology 2006: 35: pp 145-149. Saengpattrachai M, Sharma R, Hunjan A, Shroff M, Ochi A, Otsubo H, Cortez MA, Snead OC: Nonconvulsive seizures in the pediatric intensive care unit. Etiology, EEG, and brain imaging findings. Epilepsia 2006: 47: pp 1510-1518. Salter R, King SM, Smith ML, Bitnun A, Brophy J, Fernandes-Penney A, Lefebvre A, Louch D, MacDougall G, Moore W, Read SE: Disclosing HIV diagnosis to infected children: A health care team’s approach. Vulnerable Children and Youth Studies Journal 2007: 2: pp 12-16. Shemie SD, Doig C, Dickens B, Byrne P, Wheelock B, Rocker G, Baker A, Seland TP, Guest C, Cass D, Jefferson R, Young K, Teitelbaum J on behalf of the Pediatric Reference Group and the Neonatal Reference Group (Hahn C, Member): Brain injury to neurological determination of death: Canadian Council for Donation and Transplantation Forum Recommendations. Canadian Medical Association Journal 2006: 14: pp S1-S13. Smirnov D, Dikanev T, Wennberg R, Perez Velazquez JL, Bezruchko B: Dynamical nonstationarity in electroencephalograms for temporal lobe epilepsy. Biomedical Technologies and Radio Electronics, 2006: 12: pp 26-32. Smith ML, Elliott IM, Lach, LM. Memory outcome after pediatric epilepsy surgery: Objective and subjective perspectives. Child Neuropsychology 2006: 12: pp 151-160. Stewart L, Persinger MA, Cortez MA, Snead OC III: Chronobiometry of behavioural activity in the Ts65Dn model of Down syndrome. Behaviour Genetics 2006: 37: pp 388-398. Stewart LS, Shukla R, Serbanescu I, Persad V, Mistry N, Bercovici E, Cortez MA, Snead OC: Daily rhythms of seizure activity and behavior in a model of atypical absence epilepsy. Epilepsy and Behavior 2006: 9: pp 564-572. Sy K, Mahant S, Taback N, Vajsar J, Chait PG, Friedman JN: Enterostomy tube placement in children with spinal muscular atrophy type 1. Journal of Pediatrics 2006: 149: pp 837-839. Tenembaum S, Chitnis T, Ness J, Hahn JS, Banwell B: Acute disseminated encephalomyelitis. Neurology 2007: 68: pp S23-S36. Tetreault M, Duquette A, Thiffault I, Bherer C, Jarry J, Loisel L, Banwell, B, D’Anjou G, Mathieu J, Robitaille Y, Karpati G, Vanasse M, Brais B: A new form of congenital muscular dystrophy with joint hyperlaxity maps to 3p23-21. Brain 2006: 129: pp 2077-2084. BOOKS AND BOOK CHAPTERS FUNDING Gladstone JP, Brandes JL: Diagnosis and classification of primary headache disorders. In: Standards of Care for Headache Diagnosis and Treatment (Freitag FG, Cady RK, eds). National Headache Foundation. Atypical absence epilepsy: GABA B receptormediated mechanisms. Snead OC III, Francis J, Cortez MA. Canadian Institutes of Health Research ($315,376 2000-2008) Vajsar J, Schachter H: Walker-Warburg syndrome. Orphanet Journal of Rare Disorders 2006: 1: p 29. Hahn CD, du Plessis AJ: Neurologic disorders associated with cardiac disease. In: Pediatric Neurology: Principles & Practice 4th Edition (Ferreiro DM, Swaiman KF, Ashwal S, eds). Mosby, St. Louis, 2006. Vajsar J: MRI findings in congenital muscular dystrophies associated with brain abnormalities. Future Neurology 2006: 1: pp 765-773. Wang W, Lohi H, Skurat AV, Depaoli-Roach AA, Minassian BA, Roach PJ: Glycogen metabolism in tissues from a mouse model of Lafora disease. Archives of Biochemistry and Biophysics 2007: 457: pp 264-269. Wieser HG, Jones-Gotman M, Smith ML, Zumsteg D, Mueller S, Buck A, Regard M, Yonekawa Y, Valavanis A. Selective temporal lobe amobarbital memory test. Epileptogologia 2007: 15: pp 85-106. Wilejto M, Buncic JR, Kennedy J, Banwell B: The clinical features, MRI findings, and outcome of optic neuritis in children. Neurology 2006: 67: pp 258-262. Wise JC, Sevcik RA, Morris RD, Lovett MW, Wolf M: The growth of phonological awareness by children with reading disabilities: A result of semantic knowledge or knowledge of graphemephoneme correspondences? Scientific Studies of Reading 2007: 11: pp 151-164. Wu Y, Chan K, Eubanks J, Wong CG, Cortez MA, Shen L, Liu CC, Perez Velazquez JL, Wang YT, Jia Z, Snead OC: Transgenic mice over-expressing GABABR1a receptors acquire an atypical absence epilepsy-like phenotype. Neurobiology of Disease 2007: 26: pp 439-451. MacGregor DL, Kulkarni A, Dirks P, Rumney P, (eds): Head Injury in Children and Adolescents. International Child Neurology Review Series. MacKeith Press, London, UK, 2007. Menascu S, MacGregor DL: Mild closed head injury. In: Head Injury in Children and Adolescents; International Child Neurology Review Series (MacGregor DL, Kulkarni A, Dirks P, Rumney P, eds). MacKeith Press, London, UK, 2007. Perez-Velazquez J-L, Bertram EH, Snead OC: Idiopathic generalized epilepsy. In: Neurobiology of Disease (Gilman S, ed). Elsevier, New York, 2006. Rumney P: Comprehensive Multidisciplinary Rehabilitation. In: Head Injury in Children and Adolescents; International Child Neurology Review Series (MacGregor DL, Kulkarni A, Dirks P, Rumney P, eds). MacKeith Press, London, UK, 2007. Rumney P: Overview of Outcomes. In: Head Injury in Children and Adolescents; International Child Neurology Review Series (MacGregor DL, Kulkarni A, Dirks P, Rumney P, eds). MacKeith Press, London, UK, 2007. Tein I: Primary carnitine transporter defect. In: Neurobase, 13th Edition (Gilman N, Goldstein GW, Waxman SG, eds). Arbor Publishing Corp, La Jolla, CA, 2007. Vajsar J. Congenital muscular dystrophies with structural brain abnormalities: The role of brain MRI. In: Medimond International Proceedings of the 10th International Child Neurology Congress (International Child Neurology Congress, ed). Medimond Proceedings, Montreal, 2006. Weiss SK: Better Sleep for Your Baby & Child: A Parent’s Step-by-Step Guide to Healthy Sleep Habits (Bob Hilderley, ed). Robert Rose Inc., Toronto, 2006. Weiss S, RamachandranNair R, MacGregor DL: Sleep and metabolism. In: Head Injury in Children and Adolescents; International Child Neurology Review Series (MacGregor DL, Kulkarni A, Dirks P, Rumney P, eds). MacKeith Press, London, UK, 2007. Atypical absence epilepsy: GABABR-mediated mechanisms. Snead OC III. Canadian Institutes of Health Research ($705,000 2003-2008) Carnitine-responsive cardiomyopathy & the different-affinity carnitine transporters. Tein I. The Heart and Stroke Foundation of Ontario ($267,144 2004-2008) Centre for the Investigation of Neuroplasticity and Developmental Disorders Snead OC, Taylor MJ, Salter M, Josselyn S, Westall C, Dennis M, Tannock R, Sled J, Cheyne D, Campbell M. Canadian Foundation for Innovation ($15,709,086 2006-2011) Characterization of factors and mechanisms that explain the association between physical activity and lung health in cystic fibrosis: Does muscle function play a role? Ratjen F, Coates A, Tein I. Canadian Cystic Fibrosis Foundation ($158,000 2006-2008) Childhood stroke project: Ontario needs assessment for childhood stroke. deVeber G. Ontario Ministry of Health and Long-Term Care ($185,000 2005-2007) Cognitive behavioural therapy as a novel approach to the management of dystonia in children: A pilot study. Soman T, Logan W, Chen R, O’Driscoll H. SickKids Foundation ($4,500 2007-2008) Evaluating research-based early intervention for children at risk for reading and academic failure. Lovett MW, De Palma M, Frijters JC. Provincial Centre of Excellence for Child and Youth Mental Health at CHEO ($148,500 2005-2007) Genetics of reading disabilities. Barr CL, Tannock R, Lovett MW, Humphries TW. Canadian Institutes of Health Research ($898,643 2002-2007) Health-related quality of life in children with epilepsy: the first two years after diagnosis through parents’ eyes. Smith ML, NixonSpeechley K, Camfield C, Wiebe S, Zou G, Levin S. Canadian Institutes of Health Research ($393,692 2003-08) Iatrogenic Effects of Stimulant medication on sleep in children with ADHD. Corkum P, Weiss S. Canadian Institutes of Health Research ($295,000 2006) ICES paediatric linkage project. deVeber G, To T, Zak M. Ontario Ministry of Health and Long-Term Care, Integrated Policy and Planning Research Unit ($30,000 2006-2007) Identification of genetic causes of paediatric neurological conditions and using these to uncover the cellular pathways involved and to determine the ways in which they are disturbed. Minassian BA. Canada Research Chairs Program ($478,585 2006-2011) Impact of non-invasive ventilation on children and adolescents with neuromuscular conditions. MacLusky I, Katz S, Banwell B, Nicholas D, Vajsar J, Keilty-Lau K, Mitchell M, Boyd J, Anderson P, Balthazor M. SickKids Foundation ($70,000 2002-2007) Measurement and modulation of brain plasticity in paediatric stroke using transcranial magnetic stimulation. deVeber G, Kirton A. Canadian Stroke Consortium Research Committee ($26,600 2006-2007) MEB Diseases. Vajsar J. SickKids Foundation ($37,000 2006-2007) Molecular basis of Lafora bodies (starch formation in the brain ) and Lofora’s progressive myoclonus epilepsy. Minassian BA, Ackerley CA, Scherer SW, Lohi H. Canadian Institutes of Health Research ($1,146,830 2006-2011) Multimodal neuroimaging of frontal lobe cognitive function in children. Taylor MJ, Donner EW, Pang EW. Canadian Institutes of Health Research ($466,335 2006-2011) Multiple-component remediation for struggling middle school readers. Morris R, Lovett MW, Sevcik R, Frijters JC. Institute of Education Sciences (US$2,882,630 2006-2010) Multi-site pediatric network for fMRI mapping in children. Gaillard WD, Donner EJ. American Epilepsy Society ($78,000 2005-2007) Murine knockout model of 4-hydroxybutyric aciduria, Snead OC III. National Institutes of Health ($587,500 USD 2006-2011) Systemic oxygen transport and neurologic outcomes after Norwood. Arsdell G, Otsubo, H. The Heart and Stroke Foundation ($118,000 2006-2008) Murine succinate semialdehyde Dehydrogenase deficiency: An epileptic disorder with elevated CNS GABA. Snead OC. Epilepsy Foundation/ Partnership for Pediatric Epilepsy Research ($100,000 2003-2006) Neuroimaging the development of frontal lobe cognitive function in autism. Taylor MJ, Roberts W, Donner EJ, Shroff MM, Bayless SJ. National Alliance for Autism Research (US$115,907 2006-2008) Neuroimaging. Snead OC III. Ontario Research Development Challenge Fund ($800,000 2001-2006) Neuromagnetic imaging methods for realistic models of brain activity. Cheyne D, Otsubo H, Taylor MJ. Canadian Institutes of Health Research ($114,806 2006) Physical activity, sport and health competition. Schneiderman-Walker J, Longmuir P, Banwell B, Beyenne J. Canadian Institutes of Health Research, Institute of Musculoskeletal Health and Arthritis – Seed Grant ($64,985 2007-2008) The practice of paediatric movement disorders amongst paediatricians. Soman T, MacGregor D. SickKids Foundation ($500 2006-2008) The role of carnitine transporters in sperm motility and maturation and male fertility. Tein I. Physicians’ Services Incorporated Foundation ($166,000 2003-2006) The Ts65Dn mice model. Cortez MA. SickKids Foundation, Down Syndrome Fund ($5,000 2005-2007) Towards targeted intervention for specific cognitive deficits in children with attention deficit hyperactivity disorder and comorbid reading disorder. Tannock R, Swanson JM, Lovett MW, Benson NJ, Ickowicz A. National Institute of Mental Health (US$450,600 2003-2006) Towards the correction of Rett-like behaviour in MeCP2-deficient mice by exogenous gene delivery. Snead OC III. Canadian Institutes of Health Research ($585,685 2004-2009) Restracom training fellowship, post-doctoral fellowship stipend for Greg Wells PhD. Tein I, Coates A. SickKids Foundation ($73,000 2005-2007) Treating Lafora disease with the ketogenic diet. INMHA Fellowship Award for post-doctoral fellow Miles Thompson. Minassian BA. Epilepsy Canada/Canadian Institutes of Health Research ($120,000 2005-2008) Role of the carnitine/organic cation transporters in the mammary gland and implications for the suckling infant Tein I. Mead-Johnson Grant ($28,500 2007-2008) Unravelling the pathogenesis of the autophagic vacuolar myopathies. Minassian BA, Ackerley C, Manolson MF. Canadian Institutes of Health Research ($779,395 2007-2012) Seed grant funding for multidisciplinary research projects. deVeber G, Taylor, M, Shroff M. Department of Medical Imaging, University of Toronto ($70,000 2006-2008) Validation of accelerometry as a measure of physical activity and inactivity in children with chronic disease. Feldman B, Stephens S, Takken T, Van der Net J, Tremblay M, Eslinger D, Bigger W, Wright V, Validation of the NIH Stroke Scale in Children. deVeber G, Ichord R. NINDS ($45,000 2006-2009) DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 85 Division Head Dr. Ran Goldman Dr. William Mounstephen Dr. Shauna Jain Dr. Anna Jarvis Full-Time Dr. Kelly Keogh Dr. Susanna Benseler Dr. Luba Komar Dr. Kathy Boutis Dr. Amina Lalani Dr. Kevin Chan Dr. Angelo Mikrogianakis Dr. Stephen Freedman Dr. Bruce Minnes Dr. Jonathan Pirie Dr. Savithiri Ratnapalan Dr. Susan Schneeweiss Dr. Suzanne Schuh Dr. Dennis Scolnik Dr. Jennifer Thull-Freedman Part-Time Dr. Sherry Baine Dr. Carolyn Calpin Dr. Joseph Copeland Dr. Anh Do Dr. Michael Falk Dr. Janine Flanagan Dr. John Freedman Dr. Claudio Fregonas Dr. Sabine George Dr. Cara Gilman Dr. Peter Glazer Dr. Donna Goldenberg Dr. Massoud Grouhi Dr. Shuja Hafiz Dr. Mark Handley-Derry Dr. Leah Harrington Dr. Nicole Inch Dr. Ronik Kanani Dr. Eddy Lau Dr. Yen Le Dr. Patricia Linders Dr. Trent Mizzi Dr. Sanjay Mehta Paediatric Emergency Medicine DIVISIONAL OVERVIEW The division has an active three-year academic fellowship program. The Royal College of Physicians and Surgeons has fully accredited our fellowship program. In addition, we have a number of clinical departmental fellows from around the world spending a year with us further developing their paediatric emergency medicine knowledge and skills. The division continues to run Paediatric Life Support Courses (PALS and APLS). We also have a well-developed weekly half-day of educational activities for trainees and all Health care providers in the Emergency Department. In the fall the division will present the fourth annual Paediatric Emergency Medicine Update which in the past three years has been an overwhelming success. All of these will allow us to provide more efficient and effective care to our patients. In addition we look forward to the redevelopment of the physical plant. This redevelopment will ensure that the ED at SickKids remains a leader in paediatric emergency care. Research in the division remains clinically focused, covering a wide range of topics from respiratory disease acute care management, orthopaedic injuries, pain and sedation, to drug errors. Research done within the division continues to be presented nationally and internationally with publications in peerreviewed journals. Overall a substantial increase in clinical research activities has resulted which has set us on par with the other North American leaders in Paediatric Emergency Medicine. Dr. Kathy Boutis and Joanna Crudden, Research Coordinator The Emergency Department (ED) at The Hospital for Sick Children provides acute care to children who are ill or who have been injured. We are both the primary care centre for children in Toronto, plus the tertiary referral centre for the Greater Toronto Area (GTA), in addition to being the designated Paediatric Trauma Centre for the GTA. The division currently has 19 full-time staff, the majority of whom are recertified in paediatrics and paediatric emergency medicine, plus a cadre of part-time paediatricians. Over the last year we have introduced a number of innovative clinical processes such as two-tier triage, an urgent care clinic and soon will be introducing a remodelling of our team system. Dr. Steven Moss Dr. Patricia Neelands Dr. Carole O’Beirne Dr. Linda Pedder Dr. Jennifer Riley Dr. Lillian Rojnica Dr. Domenick Shelton Dr. Heshy Sturm Dr. Rahim Valani Dr. Jeff Weisbrot Dr. Sheldon Wise Dr. Hirotaka Yamashiro Cross-Appointees Dr. Eyal Cohen Dr. Astrid Guttmann Dr. Jonathan Tolkin Dr. Hilary Whyte Dr. Jamie Hutchison Clinical Fellows Dr. Samim Al-Qadhi Dr. Sam Deiratany Dr. Yaron Finkelstein Dr. Silvio Gonzalez Dr. Maria Oliva Dr. Joanne Paul Dr. Rodica Retezar Dr. Chris Ricks Research Fellows Dr. Abdullah Al-Dhafian Dr. Faraj Alharbi Dr. Nesrin Alharthy Dr. Alawi Baaboud Dr. Seen Chung Dr. Vered Gazit Dr. April Kam Dr. Melissa Parker Dr. Ayelet Rimon-Babad Administrative Staff Jo Azarraga Joanna Barnes Dana Doll Viki Dukic Ritsa Iriotakis Sandra Miller Patricia Nedanovsk Majda Savaglio Andrew Serrano Nicole Winters Benseler S: Clinical Recognition Award, Division of Paediatric Emergency Medicine, The Hospital for Sick Children, 2007. Boutis K: Runner up for Best Researcher in The Hospital for Sick Children Emergency Department, 2007. Freedman S: Clinical Recognition Award-MD, Runner Up. Division of Paediatric Emergency Medicine, 2006. Freedman S: Research Recognition Award-MD, Division of Paediatric Emergency Medicine, The Hospital for Sick Children, 2006. Jarvis A: Dr. JC Fallis Clinical Teaching Award, Division of Emergency Medicine, The Hospital for Sick Children, 2007. Schneeweiss S: Nominee for Colin Woolf Award for Excellence in Course Coordination, 2006. Schuh S: Research Recognition Award-MD, Division of Paediatric Emergency Medicine, The Hospital for Sick Children, 2007. Gaboury I, Merali Z, Mackenzie AE, Hutchison JS: Hypoxia alters the expression of inhibitor of apoptosis Proteins after brain trauma in the mouse. Journal of Neurotrauma 2007: 24: pp 338-353. Schuh S: Physician Research Award for Career Excellence Nominee, University of Toronto, Faculty of Medicine, 2007. Goldman RD: Alternating ibuprofen and acetaminophen may be more effective in the treatment of fever in children. Journal of Pediatrics [Invited Commentary] 2006: 149: pp 140-141. Scolnik D: Runner up for Clinical Recognition Award in Recognition of Excellence in Outstanding Effort and Contribution to the Emergency Department, 2007. Goldman RD, Amin P, Macpherson A: Language and length of stay in the pediatric emergency department. Pediatric Emergency Care 2006: 22: pp 640-643. Scolnik D: Runner up for Clinical Teaching Award – Staff Physician, Division of Paediatric Emergency Medicine, 2007. Goldman RD, Ong M, Macpherson A: Unscheduled return visits to the pediatric emergency department – One year experience. Pediatric Emergency Care 2006: 22: pp 545-549. PUBLICATIONS Lalani A: Continuing Education Award for Excellence in Course Coordination, Paediatric Emergency Medicine Conference, Department of Paediatrics, The Hospital for Sick Children, 2006. Al Ansari K, Howard A, Boutis K: Minimally angulated wrist fractures: Is immobilization without casting enough? Canadian Journal of Emergency Medicine 2007: 9: pp 9-15. Gupta V, Goldman RD: Pediatric pearls: Emergency contraceptive options available for adolescents. Canadian Family Physician 2006: 52: pp 1219-1220. Mehta S: PAIRO Award for Clinical Excellence in Teaching, Runner-Up, 2007. Boutis K, Schuh S, Babyn P, Alman B, Willan A, Narayanan U: Randomized controlled trial of casting vs. air-stirrup ankle brace in children with acute low risk ankle fractures. Pediatrics 2007: 119: pp 1256-1263. Joffe A, Lalani A: Injury admissions to pediatric intensive care are predictable and preventable: A call to action. Journal of Intensive Care Medicine 2006: 21: pp 227-234. Breakey VR, Pirie J, Goldman RD: Pediatric and emergency medicine residents’ attitudes and practices for analgesia and sedation during lumbar puncture in pediatric patients. Pediatrics 2007: 119: pp 631-636. Kelly WN, Arellano FM, Barnes J, Bergman U, Edwards RI, Fernandez AM, Freedman SB, Goldsmith DI, Huang KA, Jones JK, McLeay R, Moore N, Stather RH, Trenque T, Troutman WG, Puijenbroek E, Williams F, Wise RP: Guidelines for submitting adverse event reports for publication. Drug Safety 2007: 30: pp 367-373. Mehta S: James C. Fallis Teaching Award for Paediatric Emergency Medicine Staff Physician Presentation, Runner-Up, 2007. Minnes B: Clinical Teaching Award for Excellence in Clinical Teaching – Staff Physician, Division of Paediatric Emergency Medicine, The Hospital for Sick Children, 2007. Mikrogianakis A: Anna Jarvis Award for Postgraduate Teaching Excellence in Emergency Medicine, University of Toronto, 2007. Mounstephen W: Winning Poster Award Noise Levels in the ED. 1st Annual Patient Safety and Quality Academic Day, The Hospital for Sick Children, 2007. Pirie J: James C. Fallis Teaching Award, RunnerUp, Division of Paediatric Emergency Medicine, The Hospital for Sick Children, 2007. Ratnapalan S: David Fear Fellowship Award for Continuing Medical Education, University of Toronto, 2006. Schneeweiss S: Department of Paediatrics Continuing Education Award, The Hospital for Sick Children, 2007. 88 DEPARTMENT OF PAEDIATRICS Cheng A, Ratnapalan S: Improving the palatability of activated charcoal in paediatric patients. Pediatric Emergency Care 2007: 23: pp 384-386. Cohen-Kerem R, Ratnapalan S, Xu Z, Chandra R, Ito S: The attitude of physicians toward cold remedies for upper respiratory infection in infants and children: a questionnaire survey. Clinical Pediatrics 2006: 45: pp 828-834. Constantine E, Steele DW, Eberson C, Boutis K, Amanullah S, Linakis JG: The management of forearm fracture reduction in children: Variations in practice among pediatric emergency departments in North America. Pediatric Emergency Care 2007: 23: pp 209-211. Doria AS, Moineddin R, Kellenberger CJ, Epelman M, Beyene J, Schuh S, Babyn PS, Dick PT: US or CT for diagnosis of appendicitis in children and adults? A meta-analysis. Radiology 2006: 241: pp 83-94. ANNUAL REPORT 2006-07 Kelly WN, Arellano FM, Barnes J, Bergman U, Edwards RI, Fernandez AM, Freedman SB, Goldsmith DI, Huang KA, Jones JK, McLeay R, Moore N, Stather RH, Trenque T, Troutman WG, Puijenbroek E, Williams F, Wise RP: Guidelines for submitting adverse event reports for publication. Pharmacoepidemiology and Drug Safety 2007: 16: pp 581-587. Khoosal D, Goldman RD: Pediatric pearls: Vitamin E for treating children’s scars. Does it help reduce scarring? Canadian Family Physician 2006: 52: pp 855-856. Klein-Kremer A, Goldman RD: Analgesia for children with acute abdominal pain in the emergency department. Israeli Journal of Emergency Medicine 2006: 6: pp 53-56. Dr. Kevin Chan HONOURS AND AWARDS Klein-Kremer A, Goldman RD: Opioid administration for acute abdominal pain in the pediatric emergency department. Journal of Opioid Management 2007: 3: pp 11-14. Mosko JD, Goldman RD: PRETx update: White blood cell count in the diagnosis of pediatric appendicitis. International Pediatrics 2007: 22: pp 38-40. Kozer E, Scolnik D, Jarvis A, Koren G: The effect of detection approaches on the reported incidence of tenfold errors – An overview. Drug Safety 2006: 29: pp 169-174. Nasr A, Mikrogianakis A, McDowall D, Wales P: External validation and modification of a pediatric trauma triage tool. Journal of Trauma 2007: 62: pp 606-609. Kozer E, Scolnik D, MacPherson A, Rauchwerger D, Koren G: The effect of a short tutorial on the incidence of prescribing errors in pediatric emergency care. Canadian Journal of Clinical Pharmacology 2006: 13: pp e285-e291. Ratnapalan S, Schneeweiss S: Guidelines to practice: The process of planning and implementing a paediatric sedation program. Pediatric Emergency Care 2007: 23: pp 262-266. Lalani A: Pediatric Emergency Medicine in Uganda. Royal College Outlook 2006: 3: pp 56-57. Maimon M, Ratnapalan S, Do A, Kirsh J, Wilson GJ, Benson L: Cardiac perforation 6 weeks after percutaneous atrial septal defect repair using an Amplatzer septal occluder. Pediatrics 2006 5: pp 1572-1575. Mater A, Dirks P, Goldman RD: PRETx update – Ventriculoperitoneal shunt dysfunction. International Pediatrics 2006: 21: pp 184-185. Mehta SV: Review of recent AAP bronchiolitis guidelines. ACEP Section of Paediatric Emergency Medicine Newsletter 2007: 18. Mehta SV: Paediatric minor head injuries. ACEP Section of Paediatric Emergency Medicine Newsletter 2007: 18. Mikrogianakis A, Shaye RE, Griffin P, Lockwood J, Gendron NH, Gaboury I, Merali Z, MacKenzie AE, Hutchison JS: Hypoxia alters the expression of inhibitor of apoptosis proteins after brain trauma in the mouse. Journal of Neurotrauma 2007: 24: pp 338-353. Schneeweiss S, Ratnapalan S: Impact of a multifaceted paediatric sedation course: self-directed learning versus a formal continuing medical education course to improve knowledge of sedation guidelines. Canadian Journal of Emergency Medicine 2007: 9: pp 93-100. BOOKS AND BOOK CHAPTERS Benseler S: Rheumatologic emergencies. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Benseler S: Kawasaki Disease In: In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Do A: Pelvic Inflammatory disease. In: Hospital for Sick Children Handbook of Paediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Do A: Gynecologic emergencies. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Schuh S, Dick P, Stephens D, Hartley M, Khaikin S, Coates A: High-dose inhaled fluticasone does not replace oral prednisolone in children with mild to moderate acute asthma. Pediatrics 2006: 118: pp 644-650. Freedman SB: Fluid and electrolyte disorders. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Schuh S, Lalani A, Allen U, Manson D, Babyn P, Stephens D, MacPhee S, Mokanski M, Khaikin S, Dick P: Utility of chest radiographs in infants with acute bronchiolitis. Journal of Pediatrics 2007: 150: pp 429-33. Goldman RD, Mace, SE: Local anesthetics: Injectable agents. In: Critical Decisions in Emergency Medicine. American College of Emergency Physician, Dallas, Texas, 2006. Schwartz JR, Goldman RD: PRETx update – Topiramate for prophylactic treatment of pediatric migraine. International Pediatrics 2006: 21: pp 244-246. Scolnik D, Coates AL, Stephens D, Da Silva Z, Lavine E, Schuh S: Controlled delivery of high vs low humidity vs mist therapy for croup in emergency departments: A randomized controlled trial. Journal of the American Medical Association 2006: 295: pp 1274-1280. Harrington L, Schneeweiss S: Gastroenteritis and oral rehydration. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Harrington L: Constipation. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Dr. Savithiri Ratnapalan Scolnik D: Humidity therapy for croup. Italian Journal of Pediatrics 2006: 32: pp 330-332. DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 91 Harrington L: Abdominal emergencies. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Jarvis A, Kirby M: Sickle Cell Disease. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Harrington L: Gastrointestinal bleeding. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Jarvis A: Altered level of consciousness. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Harrington L: Urologic emergencies. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Harrington L: Limp. In: In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Jain S: Toxicology Part 1: Approach to ingestions. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Jarvis A: Intraosseous access. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Jarvis A: Child maltreatment. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Keogh KA, Mason A: Osteomyelitis and septic arthritis. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Jain S: Toxicology Part 2: Specific toxins. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Komar L: Cardiac emergencies. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Jarvis DA: Pediatric triage. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Lalani A: Fever and tropical infections. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Jarvis DA: Shock. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Lalani A: Foreign bodies. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Jarvis A: Head injury. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Lalani A: Diabetic emergencies. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Lalani A: Otitis media. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Lalani A. Valani R: Hematologic emergencies. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Lalani A, Schneeweiss S: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. 92 DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 Lalani A, Schneeweiss S, Shamsah A: Burns. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Mehta SV: Hyperthermia/hypothermia. In: Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones & Bartlett, Sudbury, MA, 2007 Mehta SV: Psychiatric emergencies. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Mehta SV: Syncope. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Mikrogianakis A: Pediatric trauma. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Mikrogianakis A: Ventriculoperitoneal shunts. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Mikrogianakis A, Ricks C: Common emergency procedures. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Minnes B: Paediatric appendicitis. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Mounstephen W: Adrenal crisis. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Mounstephen W: Dental emergencies. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Pirie J: Bite wounds. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Schneeweiss S. Orthopedic injuries. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Pirie J: Oropharyngeal infections. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Schneeweiss S. Pain management. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Pirie J: Fever. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Schneeweiss S. Meningitis and encephalitis. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Ratnapalan S: Hypertensive emergencies in children. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Ratnapalan S, Schneeweiss S: Procedural sedation. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Riley J: Street drugs. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Rogovik AL, Goldman RD: Hypnosis as treatment for pain. In: Pain Management & Sedation (Mace SE, Ducharme J, Murphy MF, eds). McGraw-Hill Publishing, Medical Publishing Division, New York, 2006. Schneeweiss S. Cardiopulmonary resuscitation. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Schneeweiss S. Newborn emergencies. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Schneeweiss S. Sinusitis. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Schneeweiss S. Pediatric sedation of children in the emergency setting. In: Encyclopedia of Pain (Schmidt RF and Willis WD eds). Springer-Verlag, Berlin, Heidelberg, 2007. Schuh S: Asthma. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Schuh S: Croup. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Schuh S: Bronchiolitis. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Scolnik D: Urinary tract infections. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Thull-Freedman JD: Renal emergencies. In: Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, MA, 2007. Warren D, Jarvis DA, Bullard M, Leblanc L, Cotton D, Policicchio C: Canadian Triage and Acuity Scale (CTAS) Training Manual for Combined Adult & Paediatric Triage Educational Program, 2006. FUNDING A randomized controlled trial of casting versus wrist splint in children with minimally angulated distal radius fractures in children. Boutis K, Howard A, Babyn P, Willan A. SickKids Foundation ($129,775 2006-2008) Emergency Department Rapid Intravenous Rehydration (RIVR) for pediatric gastroenteritis: A randomized controlled trial. Freedman S, Parkin P, Willan A, Schuh S. Physicians’ Services Incorporated Foundation ($165,000 2006-2008) Evaluating addiction medicine curriculum in Saudi Arabia. AL-Shamarry SA, Ratnapalan S. Ministry of Health, Saudi Arabia ($10,000 2006-2007) Impact of oximetry on hospitalization in acute bronchiolitis. Schuh S, Freedman S, Coates A, Parkin P, Allen U, Willan A, Ungar W, Da Silva Z. Physicians’ Services Incorporated Foundation ($163,000 2007-2009) Isotonic versus hypotonic IV maintenance fluids in children: A randomized controlled trial. Freidman JN, Freedman SB, Beck C, Geary D. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($9,956 2007-2008) Medication error reduction initiative in the emergency department. Ratnapalan S, Jarvis A. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($5,837 2006-2007) Predictors for diagnostically accurate ultrasound in children with suspected appendicitis. Doria A, Schuh S, Cheng A, Langer J, Tomlinson G. SickKids Foundation ($128,000 2007-2009) Programme for Global Paediatric Research Workshop/Symposium, Toronto, Ontario. Chan K, Zipursky A, Manley M. Canadian International Development Agency ($30,800 2007) The impact of an Emergency Department and community based gastroenteritis education protocol on knowledge and emergency department utilization. Freedman SB. Pediatric Consultants Educational Research Grant ($4,527 2005-2007) The MAST project: Magnesium for sickle cell trial. Goldman RD, Kirby-Allen M, Mounstephen W, Friedman J. Physicians’ Services Incorporated Foundation ($157,000 2006-2008) Workshop on NGO/academia collaboration in global child health. Chan K. Trudeau Foundation ($6,300, 2007) Paediatric Medicine DIVISIONAL OVERVIEW It was with tremendous sadness that we mourned the passing of Dr. Norman Saunders in March 2007 after a courageous and dignified fight against illness. Dr. Saunders set a very high standard for paediatricians at SickKids and even in the last years of his illness continued to make a tremendous contribution to the hospital to which he was so loyal and dedicated over the course of his thirty year career. Thanks to his efforts together with a group of his friends and former patients, a Complex Care Initiative was launched through the SickKids Foundation and has raised over 1.3 million dollars to go toward academic study and research into improving the care delivered to the complex paediatric patient. He also co-edited two books for parents, on behalf of the Division of Paediatric Medicine, which were published in the final year of his outstanding career. We also said farewell to Dr. Marcellina Mian after 25 years at SickKids and wish her well in her new leadership role in paediatric medical education in Qatar. Marcie has been a pioneer in the field of child abuse, is internationally respected as an expert in the area, and led the International Child Abuse Society as president a few years ago. She has led the hospital’s undergraduate education program admirably. She will be missed and leaves big shoes to fill! Congratulations to Dr. Stacey Bernstein who was appointed as the Director of Undergraduate Medical Education for the hospital and has taken over from Dr. Mian in this capacity. Division Head Dr. Jeremy Friedman Full-Time Dr. Hosanna Au Dr. Carolyn Beck Dr. Stacey Bernstein Dr. Catherine Birken Dr. Trey Coffey Dr. Emma Cory Dr. Robert Hilliard Dr. Sanjay Mahant Dr. Marcellina Mian Dr. Patricia Parkin Dr. Elena Pope Dr. Michelle Shouldice Dr. Susan Tallett Dr. Michael Weinstein Major Part-Time Dr. Eyal Cohen Dr. Mark Feldman Dr. Saul Greenberg On the clinical side, the Paediatric Medicine Inpatient Units remained extremely busy with responsibility for over 20 percent of the hospital inpatient population. There were a number of new quality initiatives piloted on the Paediatric Medicine wards including the MCAP utilization tool and medication reconciliation process, and a number of innovative restructuring projects aimed at improving efficiencies. The first Paediatric Medicine 7th floor inpatient Retreat was held for all members of the team and it was highly successful. Dr. Sanjay Mahant and Sherri Adams (nurse practitioner) both won awards for their clinical contribution in palliative care. The Division of Paediatric Medicine continues to contribute much to the department’s educational enterprise at both the undergraduate and postgraduate levels through the full-time faculty as well as the tremendous efforts of the community paediatricians. These contributions are not only impressive quantitatively in terms of hours spent in medical education, but also qualitatively in terms of leadership, teaching awards and evaluations. Congratulations to Dr. Susan Tallett who has been appointed as the first chief of the hospital’s new Institute of Learning. Dr. Bernstein has taken over as Director, Undergraduate Medical Education. It is notable that of the 22 core rotations through which the paediatric residents rotate in their three core years of training, the first and second highest overall ratings were for the rotations on the Paediatric Medicine inpatient ward service for first and third year residents. The Harry Bain Award for excellence in clinical teaching was awarded to Dr. Michael Weinstein (Director, Inpatient Paediatric Medicine) and the Subspecialty Teaching Award was awarded to Dr. Elena Pope (Director, Section of Paediatric Dermatology). The Paediatric Medicine fellowship program continues to grow and currently there are 10 Canadian and international fellows participating in the various streams of fellowship including Academic General Paediatrics, Dermatology, and Hospitalist fellowships. A number are pursuing Masters degrees in medical education, clinical epidemiology and bioethics. Dr. Astrid Guttmann Dr. Moshe Ipp Dr. Sheila Jacobson Dr. Charlotte Moore Dr. Michael Peer Dr. Norman Saunders Dr. Miriam Weinstein Part-Time Dr. Tony Barozzino Dr. Salvatore Cammisuli (Dermatology) Dr. Paul Dick Dr. Nhung Ho (Dermatology) Dr. Alex Levin (SCAN) Dr. Michael Sgro Dr. Christine Webster (Dermatology) Associates Dr. Michelle Farine Dr. Beth D. Gamulka Dr. Hartley A. Garfield Dr. Charlotte Moore with Dr. Sarah Wan (locum) and Dr. Navneet Sharma (fellow) The 2006-07 academic year was another excellent year for the Division of Paediatric Medicine. In January, we welcomed two new faculty members. Dr. Hosanna Au, a graduate of the University of Toronto and the paediatric residency program at SickKids joined as a member of the Section of SCAN, after an 18 month locum. In addition to her SCAN responsibilities, part of her time will be spent attending on the inpatient paediatric medicine wards in addition to her activities in medical education. We also welcomed Dr. Charlotte Moore who obtained her MD at Johns Hopkins and did her paediatric residency at Harvard. She has a strong interest in health policy and has joined our division in a major part-time role with the rest of her time being spent with the Ontario Ministry of Health. She will attend on the inpatient wards during her clinical time. We look forward to productive and exciting careers for both of them. Dr. Morton Goldbach Dr. Stuart Gordner Dr. James D. Hilton Dr. Tommy Ho Dr. Anthony Y.T. Hui Dr. Beverley Kupfert Dr. Cinda B.S. Lee Dr. S. Joseph Porepa Dr. Percy Rajchgot Dr. Clive J. Schwartz Dr. David H. Shawn Dr. Gary Sibbald Dr. Joseph Telch Dr. Ethel Ying Adjunct Dr. Ronik Kanani Cross-appointees Dr. Glen B. Berall Dr. Alan Berzen Dr. Bonnie J. Cameron Dr. Douglas Campbell Dr. Carlos E. Contreras Dr. Gordon J. Donsky Dr. Katy Driver Dr. Leona Fishman Dr. John S. Freedman Dr. Lee Ann Gallant Dr. Rose Geist Dr. Sabine R. George Dr. William B. Hanley Dr. Nicole G. Inch Dr. Jaya Islur Dr. D. Anna Jarvis Dr. Ian C. Kitai Dr. Leo Levin Dr. Patricia M. Linders Dr. John S. Lovering Dr. Gilbert Miller Dr. Golda Milo-Manson Dr. John D.M. Murray Dr. Jay D. Rosenfield Dr. Suzanne Schuh Dr. Jeffrey J. Weisbrot Dr. Sheldon A. Wise Dr. Martin G. Wolfish Honorary Dr. Lionel B.H. Boxall Dr. Michael Braudo Dr. Andrew Chlebus Dr. William Davidson Dr. Till A. Davy Dr. James Dorey Dr. Andrew D. Duic Dr. Anthony S.R. Duke Dr. Thomas J. Egan Dr. Robert Farber Dr. Hyder H. Fazal Dr. William Feldman Dr. Adelaide L. Fleming Dr. Raphael Folman Dr. Marvin H. Gans Dr. Katerina Haka-Ikse Dr. Dirk W. Huyer Dr. Martin H. Kosoy Dr. Howard M. Langer Dr. Michael Lester Dr. David C. Maresky Dr. Janette A. Milne Dr. Paul Munk Dr. Jack Newman Dr. Raymond C.W. Ng Dr. Larry B. Pancer Dr. Evangelia Papadouris Dr. Helene A. Richards Dr. Chandrakant P. Shah Dr. Donald Stewart Dr. Peter G. Strachan Dr. Robert E. Sutton On the research side, it was a highly successful year for the division with 25 abstracts being presented at the annual international Pediatric Academic Society meeting in May as well as the national Canadian Paediatric Society meeting in June. In addition, Dr. Jonathan Maguire who will be an academic general paediatric fellow in 2007 was selected to represent the residency program at the annual paediatric residents competition in Winnipeg (supervisor Dr. Patricia Parkin) and Dr. Elizabeth Berger won a research award in the core paediatric resident category of the department’s Annual Research Day (supervisor Dr. Jeremy Friedman). Research Rounds occur regularly every Thursday afternoon at lunchtime under the auspices of the Paediatric Outcomes Research Team (PORT) and continue to gain in popularity with a wide range of research being presented and reviewed. A Divisional Journal Club co-ordinated by the fellows and staff was also reinstituted this year and has been well attended and enjoyed by all. We also welcomed Magda Mekky as the PORT Clinical Research Project Co-ordinator (Epidemiology) in January 2007. Details of the many peer reviewed publications and research grants from this academic year are listed in the next few pages. On the advocacy side, the paediatric residents under the supervision of Drs. Catherine Birken and Hosanna Au have been very successful in working together with school children to produce the Kids on Kids Health magazine which has been presented in a number of forums including the national CPS meeting. A new book co-edited by Drs. Jeremy Friedman and Norman Saunders, with contributions from six of the Paediatric Medicine faculty, entitled Canada’s Baby Care Book, was published by Robert Rose in May is already a best seller in Canada. There are plans to release it internationally in the upcoming year. ‘Caring for Kids. The Complete Canadian Guide to Child Health’ published by Key Porter by the same editors, with contributions from 13 Paediatric Medicine faculty has become a bestseller in Canada since its publication in March 2006, and is now selling internationally as well. HONOURS AND AWARDS Campbell D: Postgraduate Community Paediatric Teaching Award (Postgraduate Teaching Award), Department of Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada, 2007. Saunders N: Marvin Gerstein Award (Postgraduate Teaching Award), Department of Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada, 2007. Guttmann A: 5 year Canadian Child Health Clinician Scientist Program Career Enhancement Award. December, 2006-2011. Simone A: Undergraduate Community Paediatric Teaching Award (Undergraduate Teaching Award), Department of Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada, 2007. Garfield H: Lionel Weinstein Clerkship Teaching Award (Undergraduate Teaching Award), Department of Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada, 2007. Mahant S: Palliative and Bereavement Care Service Award, The Hospital for Sick Children, Toronto, Canada, 2006. Dr. Jeremy Friedman on rounds Mahant S: Royal College of Physicians and Surgeons of Canada: Clinical Traineeship Grant, 2007. Pope E: Department of Paediatrics’ Postgraduate Subspecialty Teaching Award, Department of Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada, 2007. Tallett S: The Charles Mickles Fellowship Award. 2007. Weinstein Michael: Harry Bain Award (Postgraduate Teaching Award), Department of Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada, 2007. PUBLICATIONS Agha M, Glazier R, Guttmann A: Relationship between social inequalities and ambulatory caresensitive hospitalizations persists for up to 9 years among children born in a major Canadian urban center. Ambulatory Pediatrics 2007: 7: pp 258-262. Ardelean D, Pope E: Incontinenti pigmenti in boys: a series and review of the literature. Pediatric Dermatology 2006: 23: pp 523-527. Fellows Dr. Zia Bismilla Dr. Mariana Boragina Dr. Malini Dave Dr. Perla Lansang Dr. Irene Lara Corrales Dr. Michael McNeely Dr. Masooma Mohamed Dr. Adam Rapoport Dr. Barry Seltz Dr. Navneet Sharma Research Associates Jennifer Bennie Amina Khambalia Dr. Colin Macarthur Noorulain Malik Magda Mekky Dr. Jyothi Sehgal Kathryn (Kate) Smith Vineet Syan Au H: Neonatal seizures. Paediatrics and Child Health 2007: 12: pp 37-40. Au H: Recurrent meningitis in a child due to occult spinal lesion. Canadian Medical Association Journal 2006: 175: pp 737-738. Beck CE, Khambalia A, Parkin PC, Raina P, Macarthur C: Does day of discharge influence hospital readmission rates for children? Paediatrics & Child Health 2006: 11: pp 409-412. Birken CS, Parkin PC, To T, Macarthur C: Trends in unintentional injury mortality rates in Canadian children living in census metropolitan areas: influence of socioeconomic status. Canadian Medical Association Journal 2006: 175: pp 867-868. Boragina M, Cohen E: The setting-sun phenomenon. Canadian Medical Association Journal 2006: 175: p 878. Chang B, Mirabella G, Yagev R, Banh M, Mezer E, Parkin PC, Westall CA, Buncic JR: Screening and diagnosis of optic pathway gliomas in children with neurofibromatosis type 1 using sweep visual evoked potentials. Investigative Ophthalmology and Visual Science 2007: 48: pp 2895-902. Administrative Staff Sarah Ahmad Tiziana Altobelli Josie Marcantonio Marg Mather Daffodil Morrison Kathryn Robertson Pauline Smith Jan White Donna Whitely Connolly B, Mahant S: The pediatric hospitalist and interventional radiologist: A model for clinical care in pediatric interventional radiology. Journal of Vascular and Interventional Radiology 2006: 17: pp 1733-1738. Feldman M: Guiding Parents in their Search on the Internet for High Quality Health Information. Paediatrics and Child Health 2007: 12: pp 239-240. Friedman JN: The case for...writing case reports. Paediatrics and Child Health 2006: 11: pp 343-344. Guttmann A, Razzaq A, Lindsay P, Zagorski B, Anderson GM: Development of measures of the quality of emergency department care for children using a structured panel process. Pediatrics 2006: 118: pp 114-123. Hilliard RI, Harrison C, Madden S: Ethical conflicts and moral distress experienced by paediatric residents during their training. Paediatrics and Child Health 2007: 12: pp 29-35. Ho NTC, Lansang P, Pope E: Topical imiquimod in the treatment of infantile hemangiomas: A retrospective study. Journal of the American Academy of Dermatology 2007: 56: pp 63-68. Ipp M, Cohen E, Goldbach M, Macarthur CM: Pain response to M-M-R vaccinations in 4-6 year old children. Canadian Journal of Clinical Pharmacology 2006: 13: pp e296-e299. Jao-Tan C, Pope E: Cutaneous poisoning syndromes in children: a review. Current Opinion in Pediatrics 2006: 18: pp 410-416. Kwinter J, Pelletier J, Khambalia A, Pope E: High-potency steroid use in children with vitiligo: A retrospective study. Journal of the American Academy of Dermatology 2007: 56: pp 947-950. Kwinter J, Weinstein M: Plica neuropathica: Novel presentation of a rare disease. Clinical and Experimental Dermatology 2006: 31: pp 790-792. Lam J, Pope E: A rash-ional approach to recalcitrant diaper dermatitis. Paediatrics and Child Health 2007: 12: pp 129-131. Lieberman L, Hilliard RI: How well do pediatric residency programs prepare residents for clinical practice and their future careers? Medical Education 2006: 40: pp 539-546. MacDonald NE, Ford-Jones L, Friedman JN, Hall, J: Preparing a manuscript for publication: A userfriendly guide. Paediatrics and Child Health 2006: 11: pp 339-344. MacGregor D, Tallett S, MacMillan S, Gerber R, O’Brodovich H: Clinical and education workload measurements using personal digital assistant based software. Pediatrics 2006: 118: pp e985-e991. 98 DEPARTMENT OF PAEDIATRICS MacLean JE, Mahant S, Bitnum A, Read S, Capra M, Solomon M: Family matters: A twelve-year-old male with respiratory symptoms, cachexia and clubbing. Journal of Paediatrics and Child Health 2006: 42: pp 739-749. Macpherson AK, Macarthur C, To TM, Chipman ML, Wright JG, Parkin PC: Economic disparity in bicycle helmet use by children six years after the introduction of legislation. Injury Prevention 2006: 12: pp 231-235. Mahant S, Feigenbaum A: An infant with developmental delay. Canadian Medical Association Journal 2006: 175: pp 1369-1370. O’Brodovich H, Beyene J, Tallett S, MacGregor D, Rosenblum N: Performance of a career development and compensation program at an academic health science centre. Pediatrics 2007: 119: pp e791-e797. Pope E, Krafchik BR, Macarthur C, Stempak D, Stephens D, Weinstein M, Ho N, Baruchel S: Oral versus high dose pulse corticosteroids in problematic hemangiomas of infancy: A randomized controlled trial. Pediatrics 2007: 119: pp 1239-1247. Pope E: TIMs for AD: What are the risks? The Canadian Journal of CME 2007: 19: pp 60-62. Schull MJ, Stukel TA, Vermeulen MJ, Guttmann A, Zwarenstein M: Surge capacity associated with restrictions on nonurgent hospital utilization and expected admissions during an influenza pandemic: Lessons from the Toronto Severe Acute Respiratory Syndrome outbreak. Academic Emergency Medicine 2006: 3: pp 1228-1231. Schull MJ, Stukel TA, Vermeulen MJ, Zwarenstein M, Alter DA, Manuel DG, Guttmann A, Laupacis A, Schwartz B: Effect of widespread restrictions on the use of hospital services during an outbreak of severe acute respiratory syndrome. Canadian Medical Association Journal 2007: 176: pp 1827-1832. Soon G, Saunders N, Ipp M, Sherman PM, Macarthur C: Community-based case-control study of childhood chronic abdominal pain: Role of selected laboratory investigations. Journal of Pediatric Gastroenterology and Nutrition 2007: 4: pp 524-526. Sutcliffe TL, Khambalia A, Westergard S, Jacobson S, Peer M, Parkin PC: Iron depletion is associated with daytime bottle feeding in the second and third years of life. Archives of Pediatrics and Adolescent Medicine 2006: 160: pp 1121-1125. Sy K, Mahant S, Taback N, Vajsar J, Chait PG, Friedman JN: Enterostomy tube placement in children with spinal muscular atrophy Type 1. Journal of Pediatrics 2006: 149: pp 837-839. Weinstein M: Pediatric patient care: Part 1: Pediatric lumps and bumps. Skin and Aging 2006: 14: pp 36-40. ANNUAL REPORT 2006-07 BOOKS AND BOOK CHAPTERS Al-Anazi A, Pope E: Dermatology. In: The Hospital for Sick Children Handbook of Pediatric Emergency Medicine (Lalani A, Schneeweiss S, eds). Jones and Bartlett Publishers, Sudbury, Massachusetts, 2007. Au H: Paediatric section. In: The Toronto Notes 2007 (Heng M, Greenwald JA, eds). Toronto, 2007. Baharestani MM, Pope E: Chronic wounds in neonates and children. In: Chronic Wound Care: A Clinical Source Book for Healthcare Professionals, 4th Edition (Krasner DL, Rodeheaver GT, Sibbald RG, eds). HMP Communications, Malvern, PA, USA, 2007. Beck CE: Your baby’s first few days: Your baby’s months 2 to 6, introducing solid food. In: Canada’s Baby Care Book: A Complete Guide from Birth to 12 Months Old (Friedman JN, Saunders N, eds). Robert Rose Inc., Toronto, 2007. Birken CS: Your baby’s first few days: Your baby’s first month, helping your baby sleep. In: Canada’s Baby Care Book: A Complete Guide from Birth to 12 Months Old (Friedman JN, Saunders N, eds). Robert Rose Publishing, Inc., Toronto, 2007. Feldman M: Bed-Wetting [Primary Nocturnal Enuresis]. In: Sleep Disorders In Childhood (Weiss S, ed). Robert Rose Publishing, Inc., Toronto, 2006. Friedman JN: Paediatric section. In: The Toronto Notes 2007, 23rd Edition (Heng M, Greenwald JA, eds). Toronto, 2007. Friedman JN, Saunders N: Canada’s Baby Care Book. Robert Rose Publishing, Toronto, Ontario, 2007. Guttmann A, Lam K, Schultz S, Jaakkimainen LB: Primary care for children. In: Primary Care in Ontario ICES Atlas, Toronto, 2006. Jaakkimainen L, Klein-Geltink JE, Guttmann A, Barnsley J, Zagorski B, Kopp A, Saskin R, Leong A, Wang L: Indicators of primary care based on administrative data. In: Primary Care in Ontario ICES Atlas, Toronto, 2006. Maaten S, Guttmann A, Kopp A, Handa M, Jaakkimainen L: Care of women during pregnancy and childbirth. In: Primary Care in Ontario ICES Atlas, Toronto, 2006. Pope E, Krafchik B: Dermatologic disorders. In: Residents Handbook of Neonatology (Kirpalani H, Moore AM, Perlman M, eds). B.C. Decker Inc., Hamilton, Ontario. 2007. Schultz S, Tepper J, Guttmann A, Jaakkimainen L: Characteristics of primary care practice. In: Primary Care in Ontario ICES Atlas, Toronto, 2006. Dr. Elena Pope and clinic team Cohen E, Uleryk E, Jasuja M, Parkin PC: An absence of pediatric randomized controlled trials in general medical journals, 1985-2004. Journal of Clinical Epidemiology 2007: 60: pp 118-123. FUNDING A complex care clinic in a paediatric tertiary academic health sciences centre. Cohen E, Mahant S, Friedman JN, Adams S, Rosenbaum P. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($10,000 2006-2007) A prospective study on the age of onset, incidence, geographic distribution and risk factors associated with atopic dermatitis. Pope, E, Krafchik BR, Pelletier J. Canadian Dermatology Foundation ($35,000 1996-2007) A randomized, prospective, double-blind placebocontrolled clinical study assessing the efficacy of topical Tacrolimus 0.1% ointment and topical Clobetasol propionate 0.05% ointment in the treatment of vitiligo. Ho N, Krafchik BR, Pope E, Greenberg S, Webster C, Weinstein M. Fujisawa Canada Inc. ($62,425 2004-2007) Access to care and health outcomes in Canadian children. Guttmann A. Canadian Institutes of Health Research New Investigator Salary Award ($250,000 2006-2011) An office based intervention to improve media use in preschool children: A randomized controlled trial. Birken CS, Beck CE, McCrindle B, Parkin PC. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($9,975 2006-2007) Assessing Ontario’s Universal Influenza Immunization Program. Manuel D, Guttmann A, Kwong J, Pourbohloul B, Moran MK. Canadian Institutes of Health Research ($1,000,000 2007-2010) Complementary medicine in Canadian paediatric patients with atopic dermatitis: A cross sectional survey. Weinstein M, Pope E, Redlick F. Canadian Dermatology Foundation ($10,000 2006-2008) Does the administration of intravenous ketorolac alter the kinetics of intravenous morphine in children with vaso-occlusive sickle cell crisis? Scolnik D, Valani R, Friedman JN. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($10,000 2004-2006) Does the order in which vaccines are administered affect pain response? A randomized, double-blind, clinical trial of Pentacel vs. Prevnar. Ipp M, Taddio A, Lear N, Goldbach M, Parkin PC. Sanofi Pasteur ($17,250 2006-2007) Dr. Hosanna Au Emergency department rapid intravenous rehydration (RIVR) for paediatric gastroenteritis: A randomized controlled trial. Freedman SB, Parkin P, Willan AR, Schuh S. The Physicians’ Services Incorporated Foundation ($156,000 2005-2007) Evaluation of the efficacy and safety of Imiquimod 5% topical cream in plaque morphea: A prospective, multiple baseline, open label pilot study. Pope E, Laxer R, Doria A, Babyn P. 3M Inc./Graceway Pharmaceuticals Canada ($29,500 2005-2008) Genetic analysis of PHACE(S) Association. An International registry based out of the University of California, San Francisco. Frieden IJ, Pope E, Siegel D. University of Southern California at San Francisco ($20,000 2006-2008) Guidelines for the ethical use of children in medical education. Hilliard RI. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($1,200 2006-2007) Image guided gastrojejunostomy tube vs. laparascopic nissen fundoplication neurologic impaired children with gastroesophageal reflux disease: A study examining health related quality of life. Langer J, Mahant S, Connolly BC, Friedman JN, Wales P, Unger W, Pastor A. Ontario Federation for Cerebral Palsy ($29,098 2007-2009) Improving practice outcomes via electronic health records. Stewart M, Manuel D, Guttmann A. Ontario Ministry of Health and Long-Term Care ($499,700 2007-2008) International registry for paediatric cutaneous Tcell lymphoma: a longitudinal, prospective, observation study of cutaneous T-cell lymphoma in the paediatric population. Pope E, Weinstein M, Weitzman S, Taylor G. Canadian Dermatology Foundation ($10,000 2004-2008) International registry of paediatric cutaneous T-cell lymphoma: a longitudinal, prospective, observation study of cutaneous T-cell lymphoma in the paediatric population. Pope E. University of Toronto Dean’s Fund New Staff Grants ($10,000 2004-2009) Isotonic versus hypotonic IV maintenance fluid in children: A randomized controlled trial. Friedman JN, Freedman S, Beck C, Geary D. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($9,956 2007-2008) Knowledge and attitudes of paediatric residents towards disclosure of adverse events. Matlow A, Coffey T, Tallett S. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($5,000 2007-2008) Magnesium for Sickle Cell Trial (MAST). Goldman R., Mounstephen W, Kirby M, Friedman JN. The Physicians’ Services Incorporated Foundation ($163,846 2005-2007) Modifiable risk factors for acute chest syndrome in children with sickle cell disease admitted to hospital with a painful crisis. Birken CS, Khambalia A, Dekker A, Pastor A, Lee M, Padavattan K, Parkin PC. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($3,750 2005-2007) Nutritional education and the prevention of iron depletion in children 9 months to 2 years: A randomized controlled trial. Parkin PC, Maguire J, Khambalia A, Jacobson S, Peer M. Danone Institute ($61,148 2006-2008) Open label extension study to determine safety of etanercept in paediatric subjects with plaque psoriasis. Pope E, Weinstein M. Amgen Inc. ($43,000 2006-2008) Palindromy at the NF1 locus as a risk factor for NF1-associated malignancy. Lewis S, Parkin P. SickKids Research Institute New Ideas Grant Competition ($45,023 2006-2009) Paediatric outcomes research. Parkin PC, Macarthur C. SickKids Foundation ($1,000,000 2007-2012) Paediatric surgeries in Ontario. Guttmann A, Stukel T. Ontario Ministry of Health and LongTerm Care. ($160,000 2007) PEWS translation project: From retrospective algorithm to real-time bedside tool. Parshuram C, Hutchison J, Parkin PC, Beyene J, Dryden-Palmer K. HSFO Grant-in-Aid ($74,652 2006-2007) Placebo-controlled multicentre study with etanercept to determine safety and efficacy in paediatric subjects with plaque psoriasis. Pope E, Weinstein M. Amgen Inc. ($42,000 2005-2006) Practices and beliefs of nurses and paediatricians in level II nurseries toward newborn breastfeeding supplementation methods. Feldman M, Tamim H. York University Faculty of Arts Internal Research Grant ($5,000 2006-2007) Presentation, management and outcomes evaluation of children with vascular tumours. Pope E, Mahant S, Weinstein M, Blanchette V. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($9,900 2006-2007) Primary care practice characteristics and regional physician supply: effects on access to primary and specialty care for children in Ontario: A first look. Guttmann A (PI), Stukel T, To T, Manuel D, Glazier R, Dick P. The Physicians’ Services Incorporated Foundation ($123,000 2006-2008) The efficacy of Trimethoprim in wound healing of patients with epidermolysis bullosa: A randomized, double blinded, placebo controlled, cross-over pilot study. Pope E, Lara-Corrales I, Weinstein M, Parkin P, Sibbald G. dEBra Canada ($10,000 2006-2008) TKT024EXT: An open-label extension of study TKT024 evaluating the long-term safety and clinical outcomes in MPS II patients receiving Iduronate-2-Sulfatase enzyme replacement therapy. Clarke JTR, Friedman J. Transkaryotic Therapies Inc. ($111,497 2005-2007) Treatment choices for children with typical acute immune thrombocytopenic purpura: development of a decision aid. Beck C, Birken CS, Blanchette V, Boydell K, Llewellyn-Thomas H, Parkin PC. Dean’s Fund New Staff Competition, Spring 2006, Faculty of Medicine, University of Toronto ($10,000 2006-2011) What makes an excellent clinician in an academic health science centre? Mahant S, Wadhwa A. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($8,300 2006) DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 101 Respiratory Medicine DIVISIONAL OVERVIEW The Respiratory Medicine Program provides comprehensive care for the full range of paediatric pulmonary diseases such as asthma, cystic fibrosis, interstitial lung diseases, primary ciliary dyskinesia and chronic respiratory insufficiency in infancy due to prematurity and/or congenital malformations. We also provide consultation support for the Hospital in all areas of paediatric respiratory disease. We maintain an ongoing follow-up program for children technologically dependent with chronic respiratory insufficiency. The Sleep Laboratory (the only dedicated paediatric sleep laboratory in Ontario) provides diagnostic services and follow-up in care for children with sleep apnea, sleep related respiratory insufficiency. It is affiliated with Division of Neurology in caring for children with complex neuro-behavioural sleep disorders. The Division also provides a flexible bronchoscopy program in the diagnosis of anatomical abnormalities, infection in the immunocompromised host and children with interstitial lung disease. Over the last years we have broadened our diagnostic spectrum of tests to assess diseases with airway inflammation including test of nitric oxide in exhaled air. The addition of an infant pulmonary function laboratory has broadened the potentials for diagnostic tools to objectively assess lung disease in infants and young children and is now open for the assessment of both patients in the hospital and for outpatients. It is currently the only laboratory in the country offering this service. We are currently in the process to close the gap by developing a pulmonary function test for preschool children as well. Dr. Indra Narang in the sleep lab As part of the Toronto Lung Transplantation Program the Division provides pre- and post transplantation care for children with endstage lung disease. The lung transplant program continues to be the largest paediatric lung transplant program in the country. The training program has independent Royal College of Physicians and Surgeons of Canada accreditation and is the largest training program for paediatric respiratory medicine in the country. It provides the full range of training that is required for Board Certification in Pediatric Pulmonology in the United States. As well as offering training to Royal College of Physicians and Surgeons of Canada eligible candidates, it also trains physicians from abroad. Dr. Indra Narang, a Paediatric Pulmonologist has joined our team from Great Ormond Street in London after an interim training period at the Children’s Hospital in Philadelphia and has taken over the care of patients with sleep related respiratory disorders, which also forms the focus of her future research. Dr. MacLusky, who has been involved in many clinical activities over the last two decades, has left our division to become the Head of Respiratory Division Head Dr. Felix Ratjen Full-Time Dr. Allan L. Coates Dr. Sharon Dell Dr. Hartmut Grasemann Dr. Indra Narang Dr. Hugh O’Brodovich Dr. Melinda Solomon Dr. Padmaja Subbarao Dr. Neil B. Sweezey Part-Time Dr. Peter Bikangaga Dr. James MacFarlane Dr. Theo Moraes Dr. Charlotte D’E. Miller Adjunct Scientist Dr. Hiran Selvadurai Cross-Appointees Dr. T. Douglas Bradley Dr. Theodore Marras Honorary Dr. Gregory Downey Dr. Dennis Gurwitz Medicine at the Children’s Hospital in Ottawa. Dr. Hugh O’Brodovich, who has served as the Chair of Paediatrics for 10 years and who was on sabbatical this year, has accepted a position as Chair of Pediatrics at the Children’s Hospital in Stanford. Members of the Division are actively involved in an international program to develop a new classification for interstitial lung diseases (Dr. Dell), a NIH sponsored study on primary ciliary dyskinesia (Dr. Dell). Dr. Sharon Dell, in collaboration with researchers from the University of California in Berkeley, is leading a study entitled T-CHEQ or Toronto Child Health Evaluation Questionnaire which is examining the association between asthma and traffic-related air pollution including parents of over 6,000 Grade 1 and 2 schools in Toronto. Dr. Solomon has received an educational development grant from the Centre of Faculty Development to study the cognitivebehavioural aspects of teaching communication. The division is also involved in the planning of an AllerGen sponsored birth cohort study to elucidate the factors important for the development of asthma in early childhood (Dr. Subbarao). In addition, the CF team is involved in a number of clinical studies including a multi-centre study on azithromycin in Pseudomonas negative CF patients that will be the first collaborative study funded by the US CF foundation conducted in Canada and the US (Dr. Ratjen), studies to elucidate the role of arginase and NO deficiency in CF lung disease sponsored by CIHR and the Canadian CF foundation (Dr. Grasemann) and a study on the role of aspergillus infection in CF (Dr. Ratjen in collaboration with Dr. Shawn Aaron in Ottawa) that received initial funding from both the Canadian CF foundation and Physician Service Incorporated and which is planned to involve multiple sites in Canada and Australia. The division is also involved in developing new outcomes measures to assess improvement in CF lung disease within the BREATHE program, a study to develop CFTR corrector therapy the Canadian CF Foundation (Dr. Ratjen). The division also continues to be involved in the NIH Childhood Asthma Management Program Continuation Study (Dr. Subbarao, Dr. Grasemann). Other studies are ongoing and supported by research grants as well as generous support by individuals such as the Irwin family. The major research foci include clinical and epidemiological studies in patients with asthma and cystic fibrosis, translational studies on airway inflammation in cystic fibrosis, basic science studies on molecular biology of electrolyte transport in the lung epithelium as well as on novel genes in lung development, studies in aerosol therapy and exercise physiology. For a more complete description of our research interests, please visit our website. Dr. Michael Hutcheon Dr. Jacqueline Nemni Dr. Elizabeth Tullis Fellows Dr. Dimas Mateos-Corral Dr. Harutai Kamalaporn Dr. Mustafa Osman Subspecialty Residents Dr. Suhail Al-Saleh Dr. Reshma Amin Dr. Rakesh Bhattacharjee Dr. Tauyee Hsieh Dr. Dhenuka Radhakrishnan Dr. Anwar Sallam Administrative Staff Tara Beattie Jennifer Chay Rea Magi Victoria Snell Büscher R, Grasemann H: Disease modifying genes in cystic fibrosis: Therapeutic option or oneway road? Naunyn Schmiedebergs Archives of Pharmacology 2006: 374: pp 65-77. Chan EY, Avcin T, Dell SD, Manson D, Cutz E, Schneider R, Ratjen F: Massive hemoptysis in an 11-year-old girl with isolated pulmonary arteries. Pediatric Pulmonology 2007: 42: pp 177-180. Elias N, Rafii B, Otulakowski G, Cutz E and O’Brodovich H: The role of α, ß, and γ-ENaC subunits in distal lung epithelial fluid absorption induced by pulmonary edema fluid. American Journal of Physiology – Lung Cellular and Molecular Physiology May 18, 2007 [Epub ahead of print]. Gandhi SG, Rafii R, Harris M, Mahuran D, Chen X-J, Bao H-F, Eaton D, Otulakowski G and O’Brodovich H: Effect of cardiogenic edema fluid on ion and fluid transport in the adult lung. American Journal of Physiology – Lung Cellular and Molecular Physiology June 8, 2007 [Epub ahead of print]. Grasemann H, Schwiertz R, Grasemann C, Vester U, Racké K, Ratjen F: Decreased systemic bioavailability of L-arginine in cystic fibrosis patients. Respiratory Research 2006: 7: p 87. Grasemann H, Stehling F, Brunar H, Widmann R, Laliberte T, Molina L, Döring G, Ratjen F: Safety and efficacy of inhaled Moli1901 in cystic fibrosis. Chest 2007: 131: pp 1461-1466. Grasemann H, Kurtz F, Ratjen F: Inhaled L-arginine improves exhaled nitric oxide and pulmonary function in patients with cystic fibrosis. American Journal of Respiratory and Critical Care Medicine 2006: 174: pp 208-212. Henke M, Ratjen F: Mucolytics in cystic fibrosis. Pediatric Respiratory Reviews 2007: 8: pp 24-29. Katz S, Adatia I, Louca E, Leung K, Humpl T, Reyes J, Coates AL: Nebulized therapies for childhood pulmonary hypertension: An in vitro model. Pediatric Pulmonology 2006: 41: pp 666-673. Kennedy MP, Omran H, Leigh MW, Dell SD, Morgan L, Zariwala MA, Molina PL, Minnix SL, Severin T, Ahrens P, Lange L, Noone PG, Knowles MR: Congenital heart disease and other heterotaxic defects in a large cohort of patients with primary ciliary dyskinesia. Circulation 2007: 115: pp 2814-2821. Lam W, Tjon J, Seto W, Dekker A, Wong C, Atenafu E, Bitnun A, Waters V, Yau Y, Solomon M, Ratjen F: Pharmacokinetic modelling of a once-daily dosing regimen for intravenous tobramycin in paediatric cystic fibrosis patients. Journal of Antimicrobial Chemotherapy 2007: 59: pp 1135-1140. 104 DEPARTMENT OF PAEDIATRICS Leung K, Louca E, Munson K, Dutzar B, Anklesaria P, Coates AL: Calculating expected lung deposition of aerosolized administration of AAV vector in human clinical studies. Journal of Genetics and Medicine 2007: 9: pp 10-21. Louca E, Leung K, Coates AL, Mitchell JP, Nagel MW: Comparison of three valved holding chambers for the delivery of fluticasone propionate-HFA to an infant face model. Journal of Aerosol Medicine 2006: 19: pp 160-167. MacGregor, D, Tallett S, MacMillan S, Gerber R, O’Brodovich H: Academic physician clinical and education workload measurements using personal digital assistant based software (T-Res®). Pediatrics 2006: 118: pp e985-e991. O’Brodovich H, Beyene J, Tallett S, MacGregor D, Rosenblum N. Performance of a career development and compensation program at an academic health science center. Pediatrics 2007: 119: pp e791-e797. O’Brodovich H: Fluid clearance from the lungs of newborns, infants and children. Paediatric Respiratory Review 2006: 7: pp S62-S64. Otulakowski G, Duan W, O’Brodovich H: Oxygen and dexamethasone modulate translation of aENaC in fetal lung epithelia via eIF4F. American Journal of Respiratory and Cell Molecular Bioliology June 7, 2007 [Epub ahead of print]. Otulakowski G, Rafii B, Harris M, O’Brodovich H: Oxygen and glucocorticoids modulate translation of the epithelial sodium channel a – subunit mRNA in fetal distal lung epithelium. American Journal of Respiratory and Cell Molecular Biology 2006: 34: pp 204-212. Otulakowski, G, O’Brodovich H: Invited Commentary: Thyroid hormone and Na+,K+ATPase: More than simple transcription. American Journal of Physiology – Lung Cellular and Molecular Physiology 2007: 292: pp L4-L5. Radhakrishnan D, Corey M, Dell SD: Are patients followed in a pediatric cystic fibrosis clinic able to expectorate sputum? Archives of Pediatric Adolescent Medicine 2007: 161: pp 603-606. Ratjen F, Beier H, Grasemann H: Pharmacokinetics of inhaled colistin in patients with cystic fibrosis: authors’ response. Journal of Antimicrobial Chemotherapy 2006: 58: p 223. Ratjen F, Walter H, Haug M, Meisner C, Grasemann H, Döring G: Diagnostic value of serum antibodies in early Pseudomonas aeruginosa infection in cystic fibrosis patients. Pediatric Pulmonology 2007: 42: pp 249-255. Ratjen F: Inhaled hypertonic saline produces small increases in lung function in patients with cystic fibrosis. Journal of Pediatrics 2006: 149: p 142. ANNUAL REPORT 2006-07 Ratjen F: Treatment of early P. aeruginosa infection in patients with cystic fibrosis. Current Opinion in Pulmonology Medicine 2006: 12: pp 428-432. Ratjen F: Diagnosing and managing infection in CF. Paediatric Respiratory Review 2006: 7: pp S151-S153. Ratjen F: What’s new in CF airway inflammation: An update. Paediatric Respiratory Review 2006: 7: pp S70-S72, Schnabel D, Grasemann C, Staab D, Wolfmann H, Ratjen F: A multicenter, randomized, double-blind, placebo-controlled trial to evaluate the metabolic and respiratory effects of growth hormone in children with Cystic Fibrosis. Pediatrics 2007: 119: pp e1230-1238. Schuh S, Dick P, Stephens D, Hartley M, Khaikin S, Rodrigues L, Coates AL: High-dose inhaled Fluticasone does not replace oral Prednisolone in children with mild to moderate acute asthma. Pediatrics 2006: 118: pp 644-650. Scolnik D, Coates AL, Stephens D, Da Silva Z, Lavine E, Schuh S: A randomized trial of controlled delivery of high versus low humidity versus mist therapy in croup. Journal of the American Medical Association 2006: 295: pp 1274-1280. Starosta V, Ratjen F, Rietschel E, Paul K, Griese M: Anti-inflammatory cytokines in cystic fibrosis lung disease. European Respiratory Journal 2006: 28: pp 581-587. Stehling F, Roll C, Ratjen F, Grasemann H: Nasal nitric oxide to diagnose primary ciliary dyskinesia in newborns. Archives of Disease in Childhood – Fetal and Neonatal Edition 2006: 91: p F233. Stocks J, Coates AL, Bush: Lung function in infants and young children with chronic lung disease of infancy: The next steps? Pediatric Pulmonology 2007: 42: pp 3-9. Subbarao P, Balkovec S, Solomon M, Ratjen F: Safety and tolerability of hypertonic saline inhalation in infants with Cystic Fibrosis. Pediatric Pulmonology 2007: 42: pp 471-476. Subbarao P, Duong M, Adelroth E, Otis J, Obminiski G, Inman M, Pedersen S, O’Byrne PM: Effect of ciclesonide dose and duration of therapy on exercise-induced bronchoconstriction in patients with asthma. Journal of Allergy and Clinical Immunology 2006: 117: pp 1008-1013. Subbarao P, Ratjen F: Beta2 agonists for asthma: The pediatric perspective. Clinical Review in Allergy and Immunology 2006: 31: pp 209-218. Subbarao P, Balkovec S, Solomon M, Ratjen F: Pilot study of safety and tolerability of inhaled hypertonic saline in infants with cystic fibrosis. Pediatric Pulmonology 2007: 42: pp 471-476. Dr. Neil Sweezey and lab group PUBLICATIONS Sweezey N, Smith D, Corey M, Ellis L, Carpenter S, Tullis E, Durie P, O’Brodovich H: Amilorideinsensitive nasal potential difference varies with the menstrual cycle in cystic fibrosis. Pediatric Pulmonology 2007: 42: pp 519-524. Taylor L, Corey M, Matlow A, Sweezey NB, Ratjen, F: Comparison of Throat swabs and nasopharyngeal suction specimens in nonsputum-producing patients with cystic fibrosis. Pediatric Pulmonology 2006; 41: pp 839-843. Tschiedel E, Grasemann H, Ratjen F: Mycobacterium chelonae in a CF patient with anaplastic large cell lymphoma. Journal of Cystic Fibrosis 2006: 5: pp 133-136. Waters V, Ratjen F: Multi-drug resistant organisms in cystic fibrosis: Management and infection control issues. Expert Review of Antiinfective Therapy 2006: 4: pp 807-819. BOOKS AND BOOK CHAPTERS Gjevre J, Coates AL, Jeanneret A, Kovesi T, Lands L, Saiman L, Solomon M, Tullis E, Wilcox P: Canadian Consensus Statement On Aerosolized Antibiotic Use in Cystic Fibrosis. Toronto, The Canadian Cystic Fibrosis Foundation, 2006. Grasemann H: Disease modifier genes in CF. In: European Respiratory Monograph (Webb AK, Ratjen FA, eds). London, European Respiratory Society Journals Ltd., 2006. Grasemann H, Ratjen F. Fehlbildungen des unteren respirationstrakts. In: Duale Reihe Pädiatrie (Friedrich Carol Sitzmann, ed). Stuttgart, Thieme, 2007. Grasemann H, Ratjen F. Spezielle krankheitsbilder des unteren respirationstrakts. In: Duale Reihe Pädiatrie (Friedrich Carol Sitzmann, ed). 2007. Lemarié E, Diot P, Coates AL, Lerondel S, Le Pape A, Grimbert D, Gagnadoux F, Urban T : Autres indications et nouvelles approches thérapeutiques. In: L’aérosolthérapie Par Nébulisation. Paris, Editions Margaux Orange, 2006. Ratjen F, Grasemann H. Leitsymptom husten. In: Duale Reihe Pädiatrie (Friedrich Carol Sitzmann, ed). 2007. Ratjen F, Grasemann H. Leitsymptom akute atemnot. In: Duale Reihe Pädiatrie (Friedrich Carol Sitzmann, ed). 2007. 106 DEPARTMENT OF PAEDIATRICS Ratjen F. Spezielle krankhetsbilder des oberen Respirationstrakts. In: Duale Reihe Pädiatrie (Sitzmann FC, ed). 2007. Lung development. O’Brodovich H. Canadian Institutes of Health Research ($140,265 2004-2009) Webb AK, Ratjen, FA (eds): Cystic fibrosis. In: European Respiratory Monograph, London, European Respiratory Society Journals Ltd., 2006. Multi-center, multi-national, randomized, placebocontrolled trial of azithromycin in participants with Cystic Fibrosis, 6-18 years old, culture negative for Pseudomonas aeruginosa, Ratjen F. Cystic Fibrosis Foundation Therapeutics Inc. (salary support US$36,546 2006-2007) FUNDING A multistage population-based study to determine the association between traffic-related air pollution and childhood asthma. S Dell, D Stieb, N Gilbert, M Jerrett, D Miller, T To. Health Canada ($562,273 2005-2007) Are exhaled nitric oxide levels different in paediatric sickle cell disease patients with asthma? A cross-sectional study. Grasemann H, Kirby-Allen M, Subbarao P, Liberman L. The Hospital for Sick Children Paediatric Consultants Partnership’s Grant ($10,000 2006-2007) CAMP-CS II: The Hospital for Sick Children Clinical Centre for Study of a Childhood Asthma Management Program – Continuation Study Part II. Grasemann H, Solomon M, Subbarao P. Johns Hopkins University Centre for Clinical Trials: National Institutes of Health, National Heart, Lung and Blood Institute ($631,133 2003-2007) Characterization of factors and mechanisms affecting physical activity, muscular function and lung health in Cystic Fibrosis. Coates AL, Ratjen R. Canadian Cystic Fibrosis Foundation ($180,000 2006-2008) Diagnostic utility of induced sputum using hypertonic saline to evaluate airway inflammation and inflammation in CF. Ratjen F, Al-Saleh S, Dell SD, Grasemann H. The Lynne & Arnold Irwin Foundation ($30,000 2006-2007) Equipment grant. Grasemann G. The Lynne & Arnold Irwin Foundation ($10,000 2006) Functional analyses of two novel, developmentally-regulated glucocorticoid responsive genes in fetal lung. Kaplan F, Sweezey N. Canadian Institutes of Health Research ($131,119/year 2004-2009) Glucocorticoid receptor and glucocorticoidresponsive genes in developing lung. Sweezey N, Kaplan F. Canadian Institutes of Health Research ($563,500 2004-2009) How should nasal nitric oxide be measured to screen for PCD in children? Dell S, Grasemann H, Ratjen F. The Hopsital for Sick Children Paediatric Consultants Partnership’s Grant ($10,000 2006-2007) ANNUAL REPORT 2006-07 Mucociliary clearance consortium, rare diseases center. Dell S (SickKids site), Knowles M (Primary site: Chapel Hill). National Institute of Health ($40,000 2005-2007) Ontario asthma database – Asthma plan of action strategy. To T, Cicutto L, Dell S, Dick PT. The Ontario Ministry of Health and Long-Term Care ($500,000 2003-2007) Project #2.9 – Planning a Canadian longitudinal birth cohort study of asthma and allergy in childhood. Sears M, Subbarao P, Cyr M, Becker A, O’Byrne P. AllerGen NCE Inc. ($240,000 2004-2007) Project #FP-2005-10-development of objective measurement of airway inflammation and lung function in infants. Subbarao P, Sears M, Post M, Palaniyar M. AllerGen NCE Inc. ($342,300 2004-2007) Randomized, open labeled, cross over deposition study of Tobramycin 100 Pari nebulized with eFlow® versus TOBI® nebulized with Pari LC PLUS® in subjects with CF. Coates AL, Ratjen F, Charron M. Pari Gmb ($130,000 2006) Role of arginine metabolism in allergic asthma. Scott J, Grasemann H. Ontario Thoracic Society. ($43,650 2006-2007) Training program in clinical nutrition. Pencharz P, Anderson H, Coates A, Darling P, O’Connor D, Archer M, Wolever T. Canadian Institutes of Health Research ($1,590,000 2002-2008) Translational regulation of perinatal lung Na+ transport. Otulakowski G, O’Brodovich H. Canadian Institutes of Health Research ($743,580 2004-2009) T5602 New approach to augment clearance of pulmonary edema. O’Brodovich H. Heart and Stroke Foundation of Ontario ($270,832 2005-2008) Rheumatology DIVISIONAL OVERVIEW The Division of Rheumatology provides comprehensive care for children with rheumatic diseases. The ambulatory clinics have approximately 5,000 patient visits per year at The Hospital for Sick Children and Bloorview Kids Rehab sites and the outreach clinic in Sudbury. In addition to the general rheumatology clinics, there are unique multidisciplinary subspecialty clinics for children with dermatomyositis, morphea, systemic JIA and systemic lupus erythematosus. A new clinic for children with central nervous system vasculitis has been initiated. The division makes extensive use of the medical day care unit, where children receive joint injections under anaesthesia, therapeutic infusions or undergo investigations which would otherwise require inpatient admission. An increasing number of children have benefited from treatment with biologic agents on the medical day care unit. Research foci include the development of trial design for the study of rare diseases, outcome, prognosis and treatment studies in juvenile arthritis, SLE, neonatal lupus, scleroderma, dermatomyositis and vasculitis. Basic research focuses on the immunopathogenesis of Kawasaki disease using an animal model. Dr. Susanne Benseler was appointed as a full-time faculty member in the Division of Rheumatology. Dr. Benseler is a clinician-investigator with a major research interest in central nervous system vasculitis. The Paediatric Rheumatology Training Program had an extremely positive review by the Royal College of Physicians and Surgeons Accreditation team and was recommended full approval for the next six years. The Division of Rheumatology has the largest paediatric rheumatology fellowship training program in North America and is fully accredited by the Royal College of Physicians and Surgeons of Canada. This academic year, our fellows originated from Czechoslovakia, Israel, Turkey, India and Canada. Paediatric Rheumatology graduates have done extremely well in obtaining academic placements across Canada and around the world and trainees have been productive in the research arena. Division Head Dr. Rayfel Schneider Full-Time Dr. Susanne Benseler Dr. Brian Feldman Dr. Ronald Laxer Dr. Shirley Tse Dr. Rae Yeung Major Part-Time Dr. Earl Silverman Dr. Lynn Spiegel Part-Time Dr. Bonnie Cameron Cross Appointees Dr. Vivian Bykerk Dr. Hans-Michael Dosch Dr. Paul Fortin Clinical Fellows Dr. Yonatan Butbul Dr. Serdar Cantez Dr. Linda Hiraki Dr. Roman Jurencak Dr. Sathish Kumar Dr. Heinrike Schmeling Dr. Elizabeth Stringer Administrative Staff Sandra Bergeron Shona Magee Roseanne Richard HONOURS AND AWARDS Feldman B: Canada Research Chair in Childhood Arthritis, Tier II, 2007. Feldman B: Department of Paediatrics Research Achievement Award, June 2007 Feldman B: Winner of the Arthritis Foundation (Alabama Chapter) Outstanding Speaker Award for Rheumatology on the Beach, 2007. Silverman E: Advisor, Arthritis Panel, U.S. Food and Drug Administration. Tse S: Promoted to Program Director in the Division of Rheumatology. Yeung R: The Arthritis Society Investigator Award, 2007. Stringer E: Tied for third place at the May 2007 Department of Paediatrics Annual Research Day for her Oral Presentation: Predicting the disease course of juvenile dermatomyositis – significance of early and clinical laboratory features. Stringer E: Winner of the Canadian Rheumatology Association Paediatric Research Prize. Stringer E: Runner up for the Department of Paediatrics 2007 CanMeds Award. Tyrrell P: IMS, PhD candidate at the University of Toronto under the supervision of Dr. Earl Silverman, was one of six finalists in the University of Toronto, Institute of Medical Science Annual Laidlaw Manuscript Competition. The title of his presentation was Atherosclerosis & lipid abnormalities in paediatric systemic lupus erythematosus. PUBLICATIONS Avcin T, Schneider R, Tse S, Silverman ED: Macrophage Activation Syndrome as the presenting manifestation of rheumatic diseases in childhood. Journal of Pediatrics 2006: 148: pp 683-686. Avcin, T, Schneider R, Silverman ED, Singh-Grewal D: Severe digital ischemia in a child with Wegener’s granulomatosis. Scandinavian Journal of Rheumatology 2007: 36: pp 78-80. Aviv RI, Benseler SM, DeVeber G, Silverman ED, Tyrrell PN, Tsang LM, Armstrong D: Angiography of primary CNS vasculitis of childhood (cPACNS): Conventional angiography (CA) versus magnetic resonance angiography (MRA) at presentation. American Journal of Neuroradiology 2007: 28: pp 9-15. Dr. Rayfel Schneider Aviv RI, Benseler SM, Silverman ED, Tyrrell PN, Deveber G, Tsang LM, Armstrong D: MR Imaging and angiography of primary CNS vasculitis of childhood. American Journal of Neuroradiology 2006: 27: pp 192-199. Benseler SM, Silverman E, Aviv RI, Schneider R, Armstrong D, Tyrrell PN, de Veber G: Primary Central Nervous System Vasculitis in Children. Arthritis and Rheumatism 2006: 54: pp 1291-1297. Brown VE, Pilkington CA, Feldman BM, Davidson JE: An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM). Journal of Rheumatology (Oxford) 2006: 45: pp 990-993. Cabral DA, Laxer RM, Schrader J, Turvey S, Stevens A, Yeung RS, Foster HE, Emery H, Prendiville J, Tucker LB, Foeldvari I, Sherry DD, Klinkhoff AB, Wallace CA, Houghton K: Old challenges and new directions in pediatric rheumatology. Journal of Rheumatology 2006: 33: pp 173-184. Chagnon P, Schneider R, Hebert J, Fortin PR, Provost S, Belisle C, Gingras M, Bolduc V, Perreault C, Silverman E, Busque L: Identification and characterization of an Xp22.33;Yp11.2 translocation causing a triplication of several genes of the pseudoautosomal region 1 in an XX male patient with severe Systemic Lupus Erythematosus. Arthritis and Rheumatism 2006: 54: pp 1270-1278. Chan EY, Avcin T, Dell S, Manson D, Cutz E, Schneider R, Ratjen F: Massive haemoptysis in an 11-year-old girl with isolated pulmonary arteritis. Pediatric Pulmonology 2007: 42: pp 177-180. Doria A, Babyn PS, Feldman B: A Critical appraisal of radiographic scoring systems for assessment of Juvenile Idiopathic Arthritis. Pediatric Radiology 2006: 36: pp 759-772. Feldman BM, Pai M, Rivard GE, Israels S, Poon MC, Demers C, Robinson S, Luke KH, Wu JK, Gill K, Lillicrap D, Babyn PM: Tailored prophylaxis in severe haemophilia A: Interim results from the first 5 years of Canadian Haemophilia. Journal of Thrombosis and Haemostasis 2006: 4: pp 1228-1236. Gong GWK, McCrindle BW, Ching JC, Yeung RSM: Arthritis presenting during the acute phase of Kawasaki disease. Journal of Pediatrics 2006: 148: pp 800-805. Hashkes PJ, Laxer RM: Updates on the medical treatment of juvenile idiopathic arthritis. Current Rheumatology Report 2006: 8: pp 450-458. Hilliard P, Blanchette V, Feldman B, McLimont M, Abad A: Haemophilia Joint Health Score Reliability Study. Haemophilia Journal 2006: 12: pp 518-525. Hui-Yuen JS, Duong TT, Yeung RSM: TNF-alpha is necessary for induction of coronary artery inflammation and aneurysm formation in an animal model of Kawasaki disease. The Journal of Immunology 2006: 176: pp 6294-6301. Lang BA, Yeung RSM, Oen KG, Malleson PN, Huber AM, Riley M, Ebbeson R, Ramsey SE, Laxer RM, Silverman ED, McCrindle BW, Ratnapalan S, Feldman BM: Corticosteroid treatment of refractory Kawasaki disease. Journal of Rheumatology 2006: 33: pp 803-809. Laxer RM, Zulian F: Localized scleroderma. Current Opinion in Rheumatology 2006: 18: pp 606-613. Levine D, Akikusa J, Manson D, Silverman E, Schneider R: Chest CT findings in pediatric Wegener’s Granulomatosis. Pediatric Rheumatology 2007: 37: pp 57-62. Lovell DJ, Giannini EH, Reiff A, Jones OY, Schneider R, Olson JC, Stein LD, Gedalia A, Ilowite NT, Wallace CA, Lange M, Finck BK, Burge DJ; for the Pediatric Rheumatology Collaborative Study Group: Long-term efficacy and safety of etanercept in children with polyarticular-course juvenile rheumatoid arthritisArthritis and Rheumatism 2006: 54: pp 1987-1994. Matlow A, Stevens P, Harrison C, Laxer RM: Disclosure of medical errors. Pediatric Clinics of North America 2006: 53: pp 1091-1104. Nathan PC, Tomlinson G, Dupuis LL., Greenberg ML., Ota S, Bartels U, Feldman BM: A pilot study of ondansetron plus metopimazine vs. ondansetron monotherapy in children receiving highly emetogenic chemotherapy: A Bayesian randomized serial N-of-1 trials design. Supportive Care in Cancer 2006: 14: pp 268-276. Newburger JW, Sleeper LA, McCrindle BW, Minich LL, Gersony W, Vetter VL, Atz AM, Li JS, Takahashi M, Baker AL, Colan SD, Mitchell PD, Klein GL, Sundel RP; Pediatric Heart Network Investigators. Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease. New England Journal of Medicine 2007: 356: pp 663-675. Ota S, McLimont M, Carcao M, Blanchette V, Graham N, Paradis E, Feldman BM: Definitions for haemophilia prophylaxis and its outcomes: The Canadian Consensus Study. Haemophilia 2007: 13: pp 12-20. Pope E, Janson A, Khambalia A, Feldman BM: Childhood acquired lipodystrophy: A retrospective study. Journal of the American Academy of Dermatology 2006: 55: pp 947-950. Sarkissian T, Beyene J, Feldman B, Adeli K, Silverman E: The complex nature of the interaction between disease activity and therapy on the lipid profile in patients with pediatric Systemic Lupus Erythematosus. Arthritis and Rheumatism 2006: 54: pp 1283-1290. Johnson SR, Laxer RM: Classification in systemic sclerosis. Editorial. Journal of Rheumatology 2006: 33: pp 840-841. DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 109 Saurenmann RK, Levin AV, Feldman BM, Rose JB, Laxer RM, Schneider R, Silverman ED: Prevalence, risk factors, and outcome of uveitis in Juvenile Idiopathic Arthritis. Arthritis and Rheumatism 2007: 56: pp 647-657. Saurenmann RK, Levin AV, Rose JB, Parker S, Rabinovitch T, Tyrrell PN, Feldman BM, Laxer RM, Schneider R, Silverman, ED: Tumour necrosis factor alpha inhibitors in the treatment of childhood uveitis. Rheumatology (Oxford) 2006: 45: pp 982-989. Saurenmann T, Schneider R, Silverman ED, Laxer RM, Levin AV: Risk of new-onset uveitis in patients with Juvenile Idiopathic Arthritis treated with anti-TNF agents. Journal of Pediatrics 2006: 149: pp 833-836. Sharieff W, Zlotkin S, Tondeur M, Feldman B and Tomlinson G: Physiologic mechanisms can predict hematologic responses to iron supplements in growing children: A computer simulation model. The American Journal of Clinical Nutrition 2006: 83: pp 681-687. Silverman ED, Strand V: Leflunomide in Juvenile Idiopathic Arthritis. Future Medicine 2006: 1: pp 673-682. Silverman ED: Pediatric Systemic Lupus Erythematosus. Future Medicine 2007: 2: pp 23-50. Singh-Grewal D, Schneider R, Bayer N, Feldman BM: Predictors of disease course and remission in systemic-onset juvenile Idiopathic arthritis: Significance of early clinical and laboratory features. Arthritis and Rheumatism 2006: 54: pp 1595-1601. Singh-Grewal D, Stephens S, Cameron B, Laxer RM, Le Blanc C, Schneider R, SchneidermanWalker J, Selvadurai H, Silverman E, Spiegel L, Tse S, Wright V, Feldman BM: Physical activity and exercise in kids: The effects of vigorous exercise training on motor function and functional fitness in children with arthritis. Arthritis and Rheumatism 2006: 54: p S686. Singh-Grewal D, Wright V, Bar-Or O, Feldman BM: Pilot study of fitness training and exercise testing in polyarticular childhood arthritis. Arthritis and Rheumatism 2006: 55: pp 364-372. 110 DEPARTMENT OF PAEDIATRICS Spence D, Hornberger LK, Hamilton RM, Silverman ED: Increased risk of complete congenital heart block in infants born to women with hypothyroidism and anti-Ro and/or anti-La antibodies. Journal of Rheumatology 2006: 33: pp 167-170. Stevens P, Matlow A, Laxer RM: Blueprint for patient safety. Pediatric Clinics of North America 2006: 53: pp 1253-1267. Stinson J, Petroz G, Tait G, Feldman B, Steiner D, McGrath PJ, Stevens BJ: e-Ouch: Usability testing of an electronic chronic pain diary for adolescents with arthritis. Clinical Journal of Pain 2006: 22: pp 295-305. Sung L, Feldman BM: N-of-1 trials: innovative methods to evaluate complementary and alternative medicines in pediatric cancer. Journal of Hematology Oncology 2006: 28: pp 263-266. Sung L, Tomlinson GA, Greenberg ML, Koren G, Judd P, Ota S, Feldman BM: Validation of the oral mucositis assessment scale in pediatric cancer. Pediatric Blood Cancer 2007: 49: pp 149-153. Tse SML, Laxer RM, Babyn P, and Doria A: Radiologic improvement of enthesitis related arthritis following anti-tumor necrosis factor alpha blockade with Etanercept. Journal of Rheumatology 2006: 33: pp 1186-1188. Tse SML, Laxer RM: Approach to acute limb pain in childhood. Pediatrics in Review 2006: 27: pp 170-179. Wylie J, Campbell C, Pope J, Akikusa J, Laxer RM, Nicolle D: Convergence paralysis as a manifestation of polyarteritis nodosa. Canadian Journal of Neurological Science 2006: 33: pp 423-425. Yeung RSM: Clinical Phenotype and Coronary Outcome in Kawasaki Disease. The Lancet 2007: 369: pp 85-88. Yeung RSM: Lessons learned from an animal model of Kawasaki disease. Clinical and Experimental Rheumatology 2007: 25: pp S69-S71. Zulian F, Athreya BH, Laxer R, Nelson AM, Feitosa de Oliveira SK, Punaro MG, Cuttica R, Higgins GC, Van Suijlekom-Smit LW, Moore TL, Lindsley C, Garcia-Consuegra J, Esteves Hilario MO, Lepore L, Silva CA, Machado C, Garay SM, Uziel Y, Martini G, Foeldvari I, Peserico A, Woo P, Harper J: Juvenile localized scleroderma: clinical and epidemiological features in 750 children: An international study. Rheumatology (Oxford) 2006: 45: pp 614-620. ANNUAL REPORT 2006-07 Zulian F, Woo P, Athreya BH, Laxer RM, Medsger TA Jr, Lehman TJ, Cerinic MM, Martini G, Ravelli A, Russo R, Cuttica R, de Oliveira SK, Denton CP, Cozzi F, Foeldvari I, Ruperto N: The Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for juvenile systemic sclerosis. Arthritis Care & Research 2007: 57: pp 203-212. BOOKS AND BOOK CHAPTERS Feldman BM: Clinical trials in JIA. In: Arthritis in Children and Adolescents. Juvenile Idiopathic Arthritis (Szer I, Kimura Y, Malleson P, Southwood T, eds). Oxford University Press, London, 2006. Matlow A, Laxer RM, Guest Editors: Pediatric Clinics of North America. Elsevier, Inc., Philadelphia, 2006. Tse SML: You, your child and arthritis (Tse SML, ed). The Arthritis Society, Toronto, 2006. Yeung RSM, Ohashi P, Saunders MF, Mak TW: T Cell development in primary immunodeficiency diseases, a molecular and genetic approach. In: Primary Immunodeficiency Disease: A Molecular and Genetic Approach-Second Edition (Ochs HD, Smith CIE, Puck J, eds). Oxford University Press, New York, 2006. FUNDING 1000 Canadian Faces of Lupus. Peschken C, Pope J, Cooper GS, Silverman ED. The Arthritis Society ($381,000 2004-2007) A conceptual framework for outcome measurement in children: Consequences for health economic evaluation and decision-making. Ungar W, Marshall DA, Willan AR, Wright JG, Feldman BM. Canadian Institutes of Health Research ($224,726 2003-2006) Biologically-based outcome predictors in juvenile idiopathic arthritis. Rosenberg A, Baxter-Jones AD, Gordon J, Muhajarine N, Oen K, Pahwa P, Scherer S, Turvey S, von Baever C, Whiting SJ, Yeung RSM, Bernatsky S, Duffy C, Silverman ED. Canadian Institutes of Health Research/Canadian Arthritis Network ($1,500,000 2006-2011) Childhood Arthritis and Rheumatology Research Alliance. Sandborg C, Brewer E, Feldman B, Hirsch R, Ilowite N, Jung L, Lovel D, Malleson P, Mellins E, O’Neil K, Schanberg L, Wallace C. Arthritis Foundation ($525,000 2004-2007) Canada Research Chair Tier II. Feldman BM. Canadian Institutes of Health Research ($500,000 2002-2007) Dr. Susanne Benseler and the Vasculitis team Sarkissian T, Beyenne J, Feldman B, McCrindle B, Silverman ED: Longitudinal examination of lipid profiles in pediatric Systemic Lupus Erythematosus. Arthritis and Rheumatism 2007: 56: pp 631-638. Determinants of risk and outcome in inflammatory arthritis. Siminovitch K, Bernatsky S, Bombardier C, Briollais L, MacPherson A, Oen K, Yeung RSM. Canadian Institutes of Health Research ($2,000,000 2007-2012) Development of a feasible tool to measure oral mucositis in children with cancer. Sung L, Judd P, Tomlinson D, Doyle J, Hendershot E, Maloney AM, Feldman B. Paediatric Oncology Group of Ontario ($13,334 2007-2008) Feasibility and impact of Qigong as compared to aerobic exercise in the treatment of childhood chronic musculoskeletal pain: A pilot randomized controlled trial. Tse SML, Feldman BM (Co-PIs), Fairbairn J, O’Brien C, Bar-Or O, SchneidermanWalker J, Singh-Grewal D, Stephens S, Stinson J, Tyrell P, Whitney K, Wright V, Cameron B, Lefebvre A, Laxer R, Schneider R, Silverman E, Spiegel L (Co-Investigators), Wu H, Bustamante K (Collaborators). SickKids Foundation Complementary and Alternative Health Care and Paediatrics Research Grant ($128,223 2005-2007) Kawasaki disease: On our way to unraveling the pathogenesis. Yeung RSM. Canadian Institutes of Health Research ($318,873 2005-2008) Moderate term musculoskeletal outcomes with escalating dose: The Canadian Haemophilia prophylaxis follow-up study (CHPS-2). Feldman BM, Blanchette V, Babyn P, Hedden D. Bayer Inc. Research Fund ($585,077 2003-2006) New onset juvenile idiopathic arthritis: A Canadian multi-centre collaborative outcomes study. Duffy C, Oen K, Tucker L, Yeung RSM, Feldman B, Feldman D, Young N. Canadian Institutes of Health Research ($998,000 2004-2009) 112 DEPARTMENT OF PAEDIATRICS Pain in Child Health (PICH: An innovative, transdisciplinary, cross-Canada, research training consortium. McGrath P, Craig K, von Baeyer C, Stevens B, Johnston C, Finley A (Co-PIs), Schellinck H, Unruh A, Kenny N, Camfield C, Harmon K, Frager G, Rathwell T, Howlett A, Otley T, Connolly J, Feldman BM, Langer J, Taddio A, Baruchel S, Brown S, Gibbins S, Salter M, Hetherington R, Grunau R, Oberlander T, Chambers C, Dube L, Ohisson A, Murray M, Barr R, Koren G (Co-Investigators). Canadian Institutes of Health Research Training Grant ($300,000 2002-2008) Research to improve outcomes for inflammatory arthritis. Inman R, Gladman D, Maksymowych W, Rahman P (Co-PIs), Davis A, Tsui F, Greenwood C, Badley E, Scherer S, Reeve J, Cook R (Project CoInvestigators), Boonen A, Choquette D, Colbert R, Frank J, Ghahary A, Gignac M, Husted J, Keen K, Lambert R, Laxer R, Marra C, Mosher D, Mulholland K, Nair B, Poole R, Richards C, Salanen D, Shojania K, Thomson G, Tse S (Collaborators). The Arthritis Society, National Research Initiative (NRI) ($2,500,000 2005-2010) Rituximab Therapy in Refractory Adult and Juvenile IIM. Oddis CV, Reed AM, Pestronk A, Rider L, Miller F, Eisenberg R, Feldman BM, Ytterberg S, Levine T. National Institutes of Health ($7,732,309 2004-2009) Superantigens in autoimmunity. Yeung RSM. The Arthritis Society ($294,474 2003-2006) Superantigens in autoimmunity. Yeung RSM. Canadian Institutes of Health Research ($172,553 2006-2008) The effects of vigorous exercise training on motor function and functional fitness in Juvenile Arthritis. Feldman B, Bar-Or O, Cameron BJ, Laxer RM, Leblanc CMA, Schneider R, Schneiderman-Walker JE, Selvadurai H, Silverman ED, Singh-Grewal D, Spiegel L, Wright V. Canadian Institutes of Health Research ($74,926 2005-2006) ANNUAL REPORT 2006-07 The role of chronic inflammation and Systemic Lupus Erythematosus-related factors in the development of premature atherosclerosis in paediatric SLE. Silverman ED, Bargman J, Bradley T, Beyene J. The Heart and Stroke Foundation of Canada ($275,000 2006-2009) The role of traditional and non-traditional risks in the development of atherosclerosis in paediatric SLE. Silverman ED, McCrindle B, Adeli K, Bargman J. The Heart and Stroke Foundation of Canada ($271,854 2003-2006) Towards a better understanding of Kawasaki disease. Yeung RSM. The Heart and Stroke Foundation of Canada ($160,565 2006-2008) Towards guidelines for use of corticosteroids in children with Lupus. Brunner H, Silverman ED. National Institutes of Health ($150,000 2003-2006) Towards understanding the mechanism of congenital heart block. Hamilton RH, Gross G, Silverman ED. Canadian Institutes of Health Research ($197,000 2006-2009) Validation of a new paediatric scoring system from the IPSG: Validity reliability of the combined HJHS. Feldman B, McHugh FS, Bergstrom B-M, Zourikian N, Hilliard P, Van der Net J, Engelbert RHH, Petrini P, Manco-Johnson M, Van den Berg HM, Rivard GE, Blanchette VS. Bayer HealthCare LLC ($246,039 2005-2006) Validation of accelerometry as a measure of physical activity and inactivity in children with chronic disease. Feldman B, Stephens S, Takken T, Wright V, Tramblay M, Beyenne J, Biggar W, Esliger D, Longmuir P, McCrindle B, Schneiderman-Walker J, Van Der Net J. Canadian Institutes of Health Research – Institute of Musculoskeletal Health and Arthritis (IMHA) ($64,985 2007-2008) Bloorview Kids Rehab DIVISIONAL OVERVIEW BLOORVIEW INTERPROFESSIONAL GRAND ROUNDS In November 2006, the inpatient Bloorview site relocated to our new facility to complete the final phase of our move. Inpatients, outpatients, clients and staff are all enjoying our state-of-the-art facility, which sets standards for universal accessibility. As a provincial facility, we see almost 7,000 children and youth on an outpatient basis and we have over 650 patient admissions each year. • Introduced in January 2007 and occurring monthly to bring leaders in their respective fields to Bloorview Bloorview Kids Rehab offers both inpatient and outpatient developmental and rehabilitation services to the children and families of Greater Toronto and provides tertiary services in paediatric rehabilitation to the Province of Ontario. • Evidence-based practice, key patient safety and interprofessional initiatives are a few examples MEDICAL SERVICES STAFFING Bloorview Kids Rehab • Medical staff plus active consulting staff 9.5 FTEs • Subspecialists 17 Department of Pharmacy • Pharmacists 2.0 FTEs Through the support of the Bloorview Kids Foundation, which funds three positions, and Surrey Place Centre, which funds one position, we continue to provide the largest Royal College accredited training program for developmental paediatrics in the country. • Pharmacy Assistant 1.0 FTE The Bloorview Research Institute continues to grow under the leadership of Dr. Colin Macarthur, Vice-President, Research. • Chief of Dentistry and Manager All of our medical staff members have appointments in the Department of Paediatrics at the University of Toronto. MEDICAL EDUCATION 2006 Our medical staff members lecture at the undergraduate level and throughout the year to paediatric, neurology and physiatry residents. Dr. Milo-Manson coordinates the developmental seminar for second year medical students at the University of Toronto. Paediatric R4 30 days Neonatology 150 days Neurology 180 days Psychiatry 20 days Dr. Anne Kawamura is our new paediatric resident rotation coordinator. Department of Dental Services • Dr. Rob Carmichael appointed new Chief of Dental Services, June 1, 2007 • Part-time staff Paediatric RII PM&R Medical students 8 440 days 120 days 200 days HIGHLIGHTS Developmental paediatric subspecialty residents 1490 days • Dr. Darcy Fehlings appointed Division Head of Developmental Paediatrics, University of Toronto Total 2630 days • Second Roszell Developmental Paediatrics Research Day, February 23, 2007 • Successful Royal College accreditation April 2007 Chief of Medical Staff Dr. Golda Milo-Manson Faculty Dr. Doug Biggar Dr. Elizabeth Jimenez Division Head, Dr. Tara Kennedy Developmental Paediatrics, Dr. Tamarah University of Toronto Kagan-Kushnir Dr. Darcy Fehlings Dr. Anne Kawamura Dr. Tara Kennedy Dr. Jeff Kobayashi Dr. Gilbert Miller Dr. Peter Rumney Dr. Jonathan Tolkin Developmental Subspecialty Residents Dr. Angela Orsino Dr. Jonathan Ponesse Dr. Kassia Johnson Dr. Lyn Sonnenberg PUBLICATONS Biggar WD, Harris VA., Eliasoph L, Alman B: Longterm benefits of deflazacort treatment for boys with Duchenne Muscular Dystrophy in their second decade. Neuromuscular Disorders 2006: 16 pp 249-255. Thomas-Stonell N, Johnson P, Rumney P, Wright V, Oddson B: An evaluation of the responsiveness of a comprehensive set of outcome measures for children and adolescents with traumatic brain injury. Pediatric Rehabilitation 2006: 9: pp 14-23. Biggar W: Duchenne Muscular Dystrophy. Paediatrics Vajsar J, Zhang W, Dobyns WB, Biggar WD, Holden KR, Hawkins C, Ray P, Olney AH, Burson CM, in Review 2006: 27: pp 83-87. Srivastava AK, Schachter H: Carriers and patients with Georgiades S, Szatmari P, Zwaigenbaum L, Duku E, muscle-eye-brain disease can be rapidly diagnosed Bryson S, Roberts W, Goldberg J, Mahoney W: by enzymatic analysis of fibroblasts and lymphoblasts. The structure of the autism symptom phenotype: Neuromuscular Disorders 2006: 16: pp 132-136. A proposed multidimensional model. Journal BOOKS AND BOOK CHAPTERS of the American Academy of Child and Adolescent Lingard LA, Kennedy TJT: Qualitative research in Psychiatry 2007: 42: pp 188-196. medical education. In: Understanding Medical Ickowicz A, Feng Y, Wigg K, Quist J, Pathare T, Education (Swanwick T, ed). Edinburgh, Association Roberts W, Malone M, Schachar R, Tannock R, for the Study of Medical Education, 2007. Kennedy JL, Barr CL: Serotonin receptor HTR1B – Gene polymorphisms in attention deficit hyperactivity Better Sleep for your Baby and Child: A Parent’s Step-by-step Guide to Healthy Sleep Habits disorder Neuropsychiatric Genetics 2007: 144B: (Feldman M, Macfarlane J, Weiss S, MacLusky IB, pp 121-125. Milo-Manson G, eds). Robert Rose Publishers Loh A, Soman T, Brian J, Bryson SE, Roberts W, Toronto, 2006. Szatmari P, Smith I, Zwaigenbaum L: Stereotyped FUNDING motor behaviours associated with autism in A fine balance: Patient safety and trainee education high-risk infants: A pilot videotape analysis of a on clinical teaching teams. Kennedy T, Regehr G, sibling sample. Journal of Autism and Baker GR, Lingard LA. Canadian Institutes of Health Developmental Disabilities 2007: 37: pp 25-36. Research ($230,316 2004-2007) Kawamura A, Campbell K, Lam-Damji S, Fehlings: Autism spectrum disorders: Pathways to better A randomized controlled trial comparing botulinum outcomes. Szatmari P, Bryson SE, Fombonne E, toxin A dosage in the upper extremity of children Zwaigenbaum L, Smith I, Waddell C, Vaillancourt T, with spasticity. Developmental Medicine & Child Mirenda P, Roberts W, Volden J. Canadian Institutes Neurology 2007: 49: pp 331-337. of Health Research ($2,125,415 2004-2009) Kennedy T, Lingard L: Making sense of Grounded Theory. Medical Education 2006: 40: pp 101-108. Loh A, Soman T, Brian J, Bryson SE, Roberts W, Szatmari P, Smith I, Zwaigenbaum L: Stereotyped motor behaviours associated with autism in high-risk infants: A pilot videotape analysis of a sibling sample. Journal of Autism and Developmental Disabilities 2007: 37: pp 25-36. Narayanan U, Fehlings D, Weir S, Kiran S, Campbell K: Initial development and validation of the caregiver priorities and child health index of life with disabilities (CPCHILD). Developmental Medicine and Child Neurology 2006: 48: pp 804-812. Oddson B, Rumney P, Johnson P, Thomas-Stonell N: Clinical use of the Mayo-Portland Adaptability Inventory in rehabilitation after acquired brain injury. Developmental Medicine and Child Neurology 2006: 48: pp 918-922. Sanger T, Chen D, Delgado M, Gaebler-Spira D, Hallett M, Mink J, and the Taskforce on Childhood Motor Disorders, D. Fehlings (member): Definition and classification of negative motor signs in childhood, Paediatrics 2006: 118: pp 2159-2167. Scherer SW, Szatmari P, Roberts W (The Autism Genome Project Consortium): Mapping autism risk loci using genetic linkage and chromosomal rearrangements. Nature Genetics 2007: 39: pp 319-328. 114 DEPARTMENT OF PAEDIATRICS Books for siblings of children with autism: Children’s assessments. Johnson K, McKeever P, Kawamura A. Bloorview Research Institute Seed Grant ($5,500 2007-2008) Evaluation with a message: Interprofessional assessment of professionalism. Kennedy TJT, MiloManson G, Lingard LA. Medical Education Grant, Royal College of Physicians and Surgeons of Canada ($11,124.95 2006) Evaluation of the reliability, validity and responsiveness of the kinematic dystonia measure in children with upper extremity dystonia. Kawamura A, Fehlings D. Bloorview Research Institute Seed Grant ($16,652 2007-2008) Genetic epidemiology of autism: Family and molecular studies. Szatmari P, Bryson S, Goldberg J, Patterson A, Mahoney W, Merette C, Roberts W, Zwaigenbaum L, Scherer S. Canadian Institutes of Health Research ($725,000 2003-2008) Evaluating outcomes associated with multidisciplinary care for children with cerebral palsy using clinical practice improvement methodology. Damiano D, Chambers H, Revivo G, Brunstrom J, Horn S, Gassaway J, Krach L, Gormley M, Novacheck T, Matthews D, Chang F, Stevenson R, Abel M, McLaughlin J, Bjornson K, Rathjen K, Delgado M, Dabrowski E, Gaebler-Spira D, Dias L, Narayanan U, Fehlings D, Graham K, Koman A. American Academy for Cerebral Palsy and Developmental Medicine ($25,000 2006) ANNUAL REPORT 2006-07 Functional outcomes following orthopaedic surgery based on gait laboratory versus observational gait analysis in ambulatory children with cerebral palsy: A pilot randomized controlled trial. Narayanan U, Fehlings D. Bloorview Research Institute ($25,015 2006-2007) Functional outcomes following orthopaedic surgery based on gait laboratory versus observational gait analysis in ambulatory children with cerebral palsy: A randomized controlled trial. Narayanan U, Fehlings D. Pediatric Orthopaedic Society of North America (29,820 2006) Identifying early markers of autism: A prospective study of infant siblings. Zwaigenbaum L, Roberts SW, Smith I, Szatmari P, Bryson S. Canadian Institutes of Health Research ($331,650 2003-2006) Longitudinal study of the relationship between impairment, activity limitation, participation and quality of life in persons with Duchenne muscular dystrophy – five-year study. Biggar D. CINRG, Washington DC (reimbursed $200/ patient 2006-2011) Self-perception, behavioural attributions and perceptions of social relationships of adolescents with ADHD and LD. Roberts W, Weiner J, Malone M. Social Services & Humanities Research Council of Canada ($143,877 2005-2008) Training program in autism research. Fombonne E, Paterson A, Scherer S, Roberts W, Smith I, Moore C. Canadian Institutes of Health Research/National Alliance for Autism Research/Fonds de la Recherche en Sante du Quebec ($1,440,000 2003-2009) The development of a virtual reality therapy homebased system to improve hand usage and function in children with hemiplegia. Chau T, Fehlings D, Lam-Damji S, Li W. Bloorview Research Institute Seed Grant ($24,972 2007-2008) The experience of hope in the psychosocial adaptation of parents of a child with DMD. Tomiak E, Biggar D. CHEO Research Institute ($10,000 2007-2008) The development and evaluation of the HAT-D: A discriminative tool differentiating types of hypertonia in children. Jethwa A, Fehlings D. Bloorview Research Institute ($9,600 2006-2007) Validation of accelerometry as a measure of physical activity and inactivity in children with chronic disease. Feldman B, Biggar D. Canadian Institutes of Health Research/Institute of Musculoskeletal Health and Arthritis ($64,985 2007-2009) Western Canada standards of care Initiative for Duchenne muscular dystrophy. Mah J, Moore M, Biggar D. CINRG International Grant Award, University of Calgary ($20,000 2007-2008) Paediatric Academic Multi-Organ Transplant Program (PAMOT) DIVISIONAL OVERVIEW As a nationally and internationally recognized transplant program, PAMOT is focused on the delivery of cutting-edge and state-of-theart family-centered care for patients. Excellence in clinical care is enhanced by an active multi-specialty educational program, a fellowship training program for emerging transplant physicians, and advancing knowledge in the field of transplantation by promoting and encouraging fundamental, translational and patientbased research activities. PAMOT is one of the largest paediatric solid organ transplant programs in North America, with well over 1000 children in receipt of a solid organ transplant at this hospital. This past year has continued to be a busy clinical year for all the organ transplant groups. On May 18, 2007, we celebrated the opening of the 6A Liam’s Light Step Down Unit; a project launched by the Liam’s Light Charity in conjunction with SickKids Foundation. We also celebrated the 20th year of liver transplantation at the hospital, with a well attended multidisciplinary event at the Toronto Metro Zoo in September 2006. The training of a cadre of fellows who have been successful in obtaining academic appointments globally is another achievement of the PAMOT program. Our international trainees assuming faculty positions in their country of origin also serve as international ambassadors for The Hospital for Sick Children, the University of Toronto and Canada. Trainees continue to reflect the international nature of the fellowship program and the multi-disciplinary nature of transplantation, as illustrated by the following selection of recent trainees: Yaron Avitzur (Israel, Gastroenterology); Anne Durkan (England, Nephrology); Sara Fernandez (Costa Rica, Nephrology); Daljit Hothi (England, Nephrology); Victoria Samonte (Philippines, Critical Care); and Jason Yap (New Zealand, Gastroenterology). Interim Director Dr. David Grant Transplant Physicians and Surgeons Dr. Upton Allen Dr. Anne Dipchand Dr. Walid Farhat Dr. Annie Fecteau Dr. Diane Hebert Dr. Nicola Jones Dr. Shaf Keshavjee Dr. Tony Khoury Dr. Simon Ling Dr. Vicky Ng Current basic and clinical research activities are focused on optimizing outcomes in children after solid organ transplantation. Examples of research projects with extramural funding include: vaccination strategies; prevention of Epstein-Barr virus and Respiratory Syncytial virus complications post-transplantation (Upton Allen); long term quality of life following heart transplantation (Anne Dipchand); xenotransplantation (David Grant); adolescent issues of transition to adult care (Miriam Kaufman); techniques to enhance lung recovery for transplantation (Shaf Keshavjee); quality of life study in liver transplantation (Vicky Ng); and leukocyte trafficking in allograft rejection (Lisa Robinson). We continue active participation in international paediatric transplantation clinical research groups including the North American Pediatric Renal Transplant Cooperative Study Group, the Pediatric Heart Transplant Study Group, NIH funded fulminate liver failure study group, and the SPLIT of Pediatric Liver Transplantation Registry. Members of PAMOT continued to serve as advocates for paediatric solid organ transplantation at the provincial, national and international levels including for example: participation in the International Pediatric Lung Transplant committee (Mindy Solomon); and membership on the Trillium Gift of Life Board (David Grant, Diane Hebert). Dr. Lisa Robinson Dr. Mindy Solomon Dr. Paul Wales Paediatric Housestaff PGY4S Chief Residents Dr. Sanjeev Luthra Dr. Jonathon Maguire Associate Chief Residents Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Heidi Al-Wassia Youness Al-Darazi Nirit Bernhard Dan Flanders Sloane Freeman Elizabeth Gold Fotini Kavadas Shaun Morris Nileshwa Senthe Sarah Wan PGY3S Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Ghassan Abu Kuwaik Naifain Al-Kalbani Nashat Al-Sukaiti Daniela Ardelean Celia Atkinson Elly Berger Karoon Danayan Krishanthi Gunawardena Clare Hutchinson Marissa Joseph Mary Kiaira Vy Kim Catherine Lam Mathieu Lemaire Carey McLaughlin Michelle Porepa Tania Samanta Rayzie Shulman Joanna Swinburne Eitan Weinberg PGY2S Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dalia Al-Abdulrazzaq Fatoumah Al-Abdulrazzaq Amal Al-Maani Sowmya Balasubramanian Ereny Bassilious Michelle Batthish Glenda Bendiak Nicholas Blanchette Michelle Bridge Sumit Gupta Joelene Huber Sangeet Kathuria Tarek Khalefih Melanie Makhija Shruti Mehrotra Briseida Mema Ewurabena Simpson Andrea Wan Kevin Weingarten PGY1S Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Fahed Aljaser Ahmed Alterkait Kesete Bezu Joyce Ching Avram Denburg Alisha Kassam Rae Koffman Dianne Lim Nadia Luca Arif Manji Gagandeep Saund Deena Savlov Ksenia Slywynska Gordon Soon Christopher Sulowski Mark Tessaro Nisha Thampi Alène Toulany Kirsten Wentlandt (PGY1 neuro) PRACTICE READY ASSESSMENT (PRA) Drs. Clare Hutchinson and Sanjeev Luthra Dr. Wang Cheong Mak Paediatric Grand Rounds The 2006-07 academic year saw another successful and exciting Department of Paediatrics Grand Rounds series. The purpose of Paediatric Grand Rounds in the Department of Paediatrics at the University of Toronto is to bring the world’s most eminent clinicians, scientists, and medical educators to SickKids. Grand Rounds provides an opportunity for SickKids’ faculty, house-staff, and students to experience cutting-edge research relevant to the health care of children. All presentations highlight the link between the bench and bedside, demonstrating how new scientific advances translate into stateof-the-art health care for children. The Grand Rounds program is organized by a committee that consists of Drs. Meredith Irwin, Lisa Robinson, Jamie Hutchison, Saul Greenberg, Johanna Rommens, Rayfel Schneider, Ron Laxer, Neil Sweezey, Jill Hamilton, and Anne Opavsky, as well as our paediatric Chief Residents, Drs. Jonathon Maguire and Sanjeev Luthra. One of the highlights of the 2006-07 series was the 2006 Robert H.A. Haslam Lecture, which was delivered by Dr. Denis Daneman, incoming Chair of the Department of Paediatrics here at SickKids. Dr. Daneman gave an interesting lecture on the everyday challenges of caring for children with diabetes. Dr. Georges Rivard, Director of the Haemostasis Program at the Centre Hospitalier Universitaire Sainte-Justine in Montreal gave a fascinating presentation on the genetics, diagnosis, and emerging treatment options for haemophilia. Dr. Nicholas Evans from the University of Sydney was invited to give the 2006 Barry Smith Lecture. He delivered a state-of-the-art presentation on the role of assessment and support of the transitional circulation in preventing brain injury to preterm infants. Dr. Jeffrey Towbin, Director of the Heart Failure and Cardiomyopathy Service at Texas Children’s Hospital and Baylor College of Medicine, presented important findings on the pathogenesis and outcomes of cardiomyopathies and myocarditis in children. Dr. Robert Brent, Distinguished Professor of Pediatrics, Radiology, and Pathology, and Head of the Clinical and Environmental Teratology Research Lab at Thomas Jefferson University presented his ground-breaking research on children’s outcomes following exposure to radiation during pregnancy. Other highlights included lectures from Drs. Ulrika Kreicbergs (Children’s Hospital of Boston), Robert Truog (Children’s Hospital of Boston), Brent Hagel (University of Calgary), Eric Shoubridge (McGill University), Jessica Kahn (Cincinnati Children’s Hospital), Martin Tobin (Loyola University Hospital), Robert Black (Johns Hopkins University), Deborah Cook (McMaster University), and Paul Sharek (Stanford University). Once again this year, our own faculty delivered outstanding presentations, including Drs. Sheila Weitzman (Haematology/ Oncology), Brian Feldman (Rheumatology), Stanley Zlotkin (Gastroenterology, Hepatology, and Nutrition), and Upton Allen (Infectious Diseases). Dr. Jill Hamilton from the Division of Endocrinology and Dr. Gabrielle Boulianne from the Research Institute program in Developmental and Stem Cell Biology gave a memorable joint clinical science/basic science presentation. Their work clearly illustrated how research studies using fruit flies and clinical studies involving children can further our understanding of obesity, a very common health problem among children. In addition to these lectures, our Associate Chief Residents presented interesting cases from the paediatric service. Dr. Lars Feuk from the Genetics and Genome Biology program presented his research as the recipient of the 2007 Andrew Sass-Kortsak Award for Excellence in Paediatric Research during a Fellowship. These represent only a fraction of the outstanding rounds which made up the 2006-07 series. Grand Rounds will continue to be held on Wednesdays from 9:00 a.m. to 10:00 a.m. in the Main Auditorium at SickKids. The Department of Paediatrics Grand Rounds Committee would like to acknowledge the help and support of the following people, to whom we are very grateful: The Paediatric Executive Committee; Sylvia Muir, Division of Haematology/Oncology; Valerie Daniels, Department of Paediatrics; and Frank Ferrari and Sam Mendolia from the Audio-Visual Department at SickKids. PAEDIATRIC GRAND ROUNDS 2006 Speaker Presentation 2006 Speaker Presentation July 5-August 30 Summer Grand Rounds Chief Residents October 25 Stanley Zlotkin OC, MD, PhD, FRCPC Division Head and Medical Director of the Nutrition Support Program, Division of Gastroenterology, Hepatology and Nutrition, SickKids Micronutrient Sprinkles to control childhood anemia: Addressing an unresolved global problem through innovation, focused research and partnerships November 1 Sarah Cheang MD Sanjeev Luthra MD Elizabeth Gold MD Residents, Department of Paediatrics, SickKids Associate Rounds – Case presentations November 8 Robert D. Truog MD Professor of Medical Ethics and Anaesthesia, Harvard Medical School, Children’s Hospital of Boston, Massachusetts Donation after cardiac death: The next great advance in organ transplantation? Bioethics Week Lecture September 6 September 13 No Grand Rounds Sheila Weitzman MB, BCh, FCP(SA), FRCPC HLH – Diagnostic and therapeutic dilemmas Senior Staff Oncologist and Director of Clinical Services, Division of Haematology/ Oncology, SickKids September 20 September 27 Jeffrey Towbin MD Director, Heart Failure and Cardiomyopathy Service Baylor College of Medicine, Texas Children’s Hospital Houston, Texas Cardiomyopathies and myocarditis in children Denis Daneman The everyday challenges of diabetes 2006 Robert Haslam Lecture MB, BCh, FRCPC Chair, Department of Paediatrics, SickKids October 4 October 11 October 18 Brian Feldman MD, FRCPC Staff Rheumatologist, Division of Rheumatology, SickKids The quality of life for children with chronic illness Ulrika Kreicbergs RN, PhD Postdoctoral Research Fellow Dana-Farber Cancer Institute, Children’s Hospital Boston, Boston, Massachusetts Can we learn from those who already know: A nationwide study on parents who lost their child to cancer Palliative Care Grand Rounds Georges Rivard MD Director, Haemostasis Program and the Haemostasis Clinical Laboratory, Centre Hospitalier Universitaire Sainte-Justine, Montreal, Quebec Haemophilia: From royal genes to diagnosis and treatment 2006 Bernard Laski Lecture November 15 Brent Hagel PhD Department of Paediatrics and Community Health Sciences, Calgary, Alberta November 22 Upton Allen MBBS, MSc, FAAP, FRCP The Epstein-Barr virus and Head, Division of Infectious weakened immune Diseases, SickKids systems: Hopes, expectations and challenges November 29 Eric Shoubridge PhD Principal Investigator, Molecular Neurogenetics, Molecular Neurogenetics Institute, McGill University, Montreal, Quebec The assembly of the respiratory chain and the mystery of tissue specificity in mitochondrial disease 2006 Jacob’s Ladder Lecture December 6 The role of assessment and support of the transitional circulation in prevention of brain injury to preterm babies 2006 Barry Smith Lecture Nicholas Evans DM, MRCPCH Clinical Associate Professor in Neonatal Medicine, Royal Prince Alfred Hospital, University of Sydney, Sydney, Australia December 13 Department of Paediatric Residents 120 DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 The reasons for and against helmet use in recreational activities: What to tell your patients and their parents Annual Holiday Presentation PAEDIATRIC GRAND ROUNDS 2007 Speaker Presentation January 3 No Grand Rounds January 10 Associate Rounds #2 January 17 Jessica Kahn MD, MPH Associate Professor of Pediatrics, Cincinnati Children’s Hospital, Cincinnati, Ohio HPV: New vaccine, and issues in adolescents January 24 Martin Tobin MD Professor of Medicine, Pulmonary, and Critical Care Medicine, Anesthesiology, Loyola University Hospital, Maywood, Illinois Evidence based medicine issues January 31 Berge Minassian MD Canada Research Chair in Paediatric Neurogenetics and Staff Neurologist, SickKids The paediatric genetics epilepsies February 7 Lorelei Lingard MD Professional Educator, Medical Education – Undergraduate, Assistant Professor, Department of Paediatrics, University of Toronto Medical education February 14 February 21 February 28 March 7 No Grand Rounds due to renovations No Grand Rounds due to renovations No Grand Rounds due to renovations No Grand Rounds due to renovations March 14 March Break March 21 No Grand Rounds due to renovations March 28 Robert Black MD, MPH Professor and Chair, Department of International Health, Johns Hopkins University, Baltimore, Maryland April 4 No Grand Rounds due to renovations Child survival in developing countries: Problems but promise UHN Auditorium 2007 Speaker Presentation April 11 Deborah Cook MD, FRCPC, MSc(Epid) Professor, Department of Medicine, Clinical Epidemiology and Biostatistics, Academic Chair, Critical Care Medicine, McMaster University, Ontario Changing behaviour to implement evidence UHN Auditorium April 18 No Grand Rounds due to renovations April 25 Scott Halperin MD Professor of Paediatrics, Microbiology and Immunology and Head, Paediatric Infectious Diseases, Dalhousie University, Halifax May 2 SOCIETY OF PAEDIATRIC RESEARCH SEMINAR May 9 Dr. Robert Brent AB, MD, PhD Head, Clinical and Environmental Teratology Research Lab, A.I. DuPont Hospital for Children, Distinguished Professor of Pediatrics, Radiology and Pathology, Thomas Jefferson University Saving lives and changing family histories: The consequences of utilizing appropriate and inappropriate information when counseling families about developmental risks Motherisk Lecture May 16 Gabrielle Boulienne PhD (Senior scientist) Jill Hamilton MD (Associate scientist and staff endocrinologist) Exploring mechanisms for weight gain: Lessons from children with brain tumours and fat flies May 23 RESEARCH DAY May 30 Sass-Kortsak Lecture June 6 Dr. Paul Sharek MD Stanford University June 13 AWARDS DAY June 20 Dr. John Pettifor MBBCH Dept. of Paediatrics, Chris-Hani Baragwanath Hospital and the University of Witwatersrand, South Africa June 27 No Grand Rounds DEPARTMENT OF PAEDIATRICS Vaccines: What’s hot and what is on the horizon Paediatric Update Keynote Lecture Pediatric patient safety – Defining and detecting adverse events in children Rickets in children – How important is dietary calcium intake? ANNUAL REPORT 2006-07 121 Promotions/Recognition PROMOTED TO PROFESSOR OF PAEDIATRICS Dr. Anne Matlow Dr. Jay Rosenfield PROMOTED TO ASSOCIATE PROFESSOR OF PAEDIATRICS Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Dr. Elizabeth Asztalos Anne Dipchand Annette Feigenbaum Gil Gross Joel Kirsh Vicky Ng Suzan Schneeweiss Vibhuti Shah Gary Smith THOSE WHO REACHED NORMAL RETIREMENT AGE The following reached normal retirement age in June of 2007 Dr. Douglas Biggar Dr. J. Michael Dorey Dr. Raphael Folman Dr. Howard Langer Dr. Irena Nulman Dr. Joyce Nesker Simmons Dr. Arne Ohlsson Dr. Eve Roberts 25 YEARS OF SERVICE AWARDS The following were honoured for their 25 years of service to The Hospital for Sick Children in June of 2007 Dr. Amos Cohen Dr. Denis Daneman Dr. Lee Ford-Jones Dr. Miriam Rossi Dr. Sheila Weitzman Faculty Awards AWARDS Teaching/Education Awards R.H.A. Haslam Preclerkship Teaching Award to the faculty member who has demonstrated excellence in preclerkshop teaching Dr. Lillian Rojnica The Lionel Weinstein Clerkship Teaching Award to the faculty member who has demonstrated excellence in clerkship teaching Dr. Angelo Simone The Undergraduate Community Teaching Award in recognition of excellence in the teaching of medical students within community-based hospitals and offices Dr. Hartley Garfield Harry Bain Award to a full-time member of the Department of Paediatrics who has demonstrated excellence in teaching as nominated by core paediatric residents Dr. Michael Weinstein Marvin Gerstein Award to a part-time member of the Department of Paediatrics who has demonstrated excellence in teaching as nominated by core paediatric residents Dr. Norman Saunders Postgraduate Community Teaching Award in recognition of excellence in the teaching of paediatric residents within community-based hospitals and offices Dr. Douglas Campbell PERLS Award (Paediatric Educational Resident Lecture Series Award) to the faculty member who has demonstrated commitment to excellence in teaching at the Paediatric academic sessions Dr. Aru Bitnun Subspecialty Teaching Award to the faculty member who has demonstrated commitment to excellence in teaching the subspecialty residents and clinical fellows Dr. Elena Pope Continuing Medical Education Award to the faculty member in recognition of excellence in course coordination in an event sponsored by the Department of Paediatrics/University of Toronto and/or long-term contribution to Continuing Education in the Department of Paediatrics Dr. Suzan Schneeweiss Clinical Awards Richard Rowe Award for Clinical Excellence in Paediatric Medical Care to the physician in the Department of Paediatrics who has demonstrated excellence in the delivery of clinical care in the broadest sense over a significant period of time Dr. Lee Benson Junior Faculty Award for Clinical Excellence in Paediatric Medical Care to a physican within the first 5-10 years of appointment to an Academic Health Sciences Centre, who has demonstrated excellence in the delivery of clinical care Dr. Jill Hamilton Research Awards Physician Researcher Award for Scientific Accomplishment to recognize the accomplishments of a physician who has generated a body of research that is exerting a major impact in a field of study. The awardee will be more than six and less than 15 years beyond the date of appointment as a faculty member at an Academic Health Sciences Centre. This award was first awarded in 2005. Dr. Brian Feldman Physician Research Award for Career Excellence to recognize sustained excellence by a physician who has generated a body of work with relevance beyond a specific field of study. The awardee will be 15 or more years beyond the initial date of appointment as a faculty member at an Academic Health Sciences Centre. This award was first awarded in 2005. Dr. Paul Pencharz Paediatric Administrative Award In recognition of an administrative support staff member in the Department of Paediatrics who has demonstrated outstanding performance and excellence in all aspects of their role. This individual possesses outstanding communication and interpersonal skills and is motivated to perform above and beyond their daily responsibilities. This award was first awarded in 2007. Ms. Shona Magee Resident and Fellow Awards and Achievements TEACHING/EDUCATION AWARDS Christopher Ondaatje Award to a senior resident for excellence in clinical teaching as nominated by core paediatric residents. Dr. Joanna Swinburne Dr. Jonathon Maguire CanMEDS Award – Core Residents Dr. Joelene Huber Dr. Catherine Lam Dr. Michelle Porepa Elizabeth Stringer, Rheumatology Predicting the disease course of juvenile dermatomyositis – Significance of early and clinical laboratory features. E Stringer, D Singh-Grewal, R Laxer, B Feldman Mark Walsh, Cardiology Left ventricular morphology and function predict outcome after Norwood palliation. MA Walsh, BW McCrindle, A Dipchand, D Manlhiot, E Hickey, CA Caldarone, GS Van Arsdell, SM Schwartz Poster Presentations CanMEDS Award – Subspecialty Fellow Dr. Dan Turner Shaker Abdullah, Haematology/Oncology Expression of MYCN in paediatric synovial sarcoma. S Abdullah, M Zielenska, C Sherman, S Chan, PS Thorner, GR Somers CLINICAL AWARDS The Saunders Prize to a senior resident who has demonstrated excellence in clinical paediatrics. Dr. Mathieu Lemaire Samina Afzal, Haematology/Oncology Role of lymphocyte recovery after allogeneic haematopoietic stem cell transplantation in predicting relapse in paediatric acute leukaemia. S Afzal, MK Ishaqi, J Doyle, A Gassas RESEARCH AWARDS Fadia Al-Buhairan, Adolescent Medicine Paediatric Residents’ experiences in Adolescent Medicine. F Al-Buhairan, K Leslie, E Goldberg Junior Trainees Dr. Catherine Lam Dr. Elizabeth Berger Senior Trainees – Clinical Science Dr. Adam Kirton Dr. Arvind Sehgal (Tie 2) Dr. Mark Walsh (Tie 2) Dr. Dragos Predescu (Tie 3) Dr. Elizabeth Stringer (Tie 3) Senior Tranees – Basic Science Dr. Aleixo Muise (Tie 1) Dr. Adam Durbin (Tie 1) Todd R. Alexander, Nephrology The cytosoloic C-terminus of NHE3 does not medicate its apical localization or baseline activity – Implications for blood pressure and pH homeostasis. RT Alexander, I Peltekova, A Durkan, S Grinstein Samim Al-Qadhi, Emergency Medicine A randomized controlled comparison of the effectiveness of different teaching strategies on proficiency in intraosseous placement skills. S Al-Qadhi, H Carnahan, A Dubrowski, S Mehta Abdulkader Al-Rezqi, Infectious Diseases Invasive mold infections in paediatric cancer and transplant patients: An eight year review. A Al-Rezqi, M Hawkes, J Doyle, S Richardson, U Allen ANNUAL RESIDENT AND FELLOW RESEARCH DAY May 23, 2007 Oral Presentations Chhaya S. Ambekar, Haematology/Oncology Loss of Sbds results in dysregulation of Fas at the plasma membrane level. CS Ambekar, H Wang, K-i Watanabe, A Schimmer, Y Dror Elizabeth Berger, Core Paediatrics Sickle cell disease in children: Osteomyelitis or vaso-occlusive crises? E Berger, N Saunders, A Khambalia, JN Friedman Juan Appendino, Neurology EEG in newborns with sinus venous thrombosis. J Appendine, M Cortez, E Sell, T Soma Paul Gill, Nephrology Suppressor of Fused is required for normal development of the murine cerebellum. PS Gill, R Mo, C-C Hui, ND Rosenblum Michelle Barton, Infectious Diseases Immunogenicity of pneumococcal conjugate vaccine in paediatric solid organ recipients. M Barton, S Wasfy, A Dipchand, D Hébert. V Ng, M Solomon, U Allen April Kam, Emergency Medicine Paediatric cellulitis: Success of Emergency Department short course intravenous antibiotics. A Kam, J Leal, SB Freedman Michelle Batthish, Rheumatology Residents’ perceptions of an interactive web-based rheumatology teaching module. M Batthish, E Bassilious, G Bendiak, S Gupta, B Feldman, R Schneider, S Tse Loretta Lau, Haematology/Oncology HDM2 antagonist Nutlin-3 inhibits p73-HDM2 binding and activates p73. L Lau, J Nugent, MS Irwin Wanatpreeya Phongsamart, Infectious Diseases Tuberculosis in children and adolescents in Ontario: A population-based study. W Phongsamart, I Kitai, M Gardam, J Wang, K Khan, the Ontario TB Outcome Study Network Arvind Sehgal, Neonatology Impaired coronary artery perfusion is associated with early myocardial diastolic dysfunction in premature infants after ligation of the ductus arteriosus. A Sehgal, PJ McNamara Tina Biss, Haematology/Oncology Clinical characteristics of pulmonary embolism in childhood – Experience from a single centre. TT Biss, LR Brandao, AK Chan, S Williams Darren J. Bridgewater, Nephrology Beta-catenin controls murine kidney development and espression of kidney patterning genes. DJ Bridgewater, ND Rosenblum Aviel Y. Butbul, Rheumatology Juvenile psoriatic arthritis (JPSA) is it a separate disease? AY Butbul, S Dhillon, PN Tyrell, ED Silverman Jason E. Cain, Nephrology Lineage specific functions of GLI activators during murine kidney development. JE Cain, E Nieuwenhuis, C-C Hui, ND Rosenblum Harold Chui, Adolescent Medicine Impact of weight and menstrual function recovery on brain structure and function in adolescent-onset anorexia nervosa. HT Chui, B Christensen, RB Zipursky, DK Katzman Min Rui Crow, Nephrology Neuronal guidance cues Slit and Robo – new inhibitors of leukocyte trafficking. MR Crow, M Yan, G-Y Liu, S Tole, LA Robinson Roberto Jose Diaz, Haematology/Oncology Assessment of chemotherapeutic response in paediatric patients with optic nerve gliomas associated with proptosis. RJ Diaz, S Laughlin, R Buncic, G Nicolin, E Bouffet, U Bartels Valeria E. Di Giovanni, Nephrology The BMP receptor, ALK3, controls murine nephrogenesis. VE Di Giovanni, ND Rosenblum Gaute Dohlen, Cardiology Improving outcomes in high risk tetralogy of Fallot patients with right ventricular outflow tract stenting. G Dohlen, LN Benson, RR Chaturvedi, A Ozawa, D Fruitman-Power, K-J Lee Adam Durbin, Haematology/Oncology A JNK-dependent activation switch determines the oncogenic or tumour suppressive activity of integrin-linked kinase in rhabdomyosarcoma. AD Durbin, GR Somers, M Forrester, GE Hannigan, D Malkin Walid I. El-Nagger, Neonatal-Perinatal Medicine Role of amplitude integrated electroencephalography (aEEG) in Neonates with cardiovascular compromise. WI El-Naggar, M Keyzers, PJ McNamara Amr El Shahed, Neonatal-Perinatal Medicine Surfactant for meconium aspiration syndrome in full term infants. AI El Shahed, P Dargaville, A Ohlsson, RF Soll Emer Finan, Neonatal-Perinatal Medicine Clinical predicators of post-ligation cardiac syndrome (PLCS) in neonates. E Finan, SP Shivananda, L Teixeira, W Mak, PJ McNamara Ayataka Fujimoto, Neurology Magnetoencephalographic interictal epileptiform discharges under total intra venous anesthesia in children with lesion or non-lesion epilepsy. A Fujimoto, A Ochi, K Imai, R Sharma, A Hunjan, B Chu, S Holowka, SM Kemp, SH Chuang, A Matsumura, C Snead III, H Otsubo Alya Galtseva, Nephrology GLI3 is expressed in the primary cilium in the murine embryonic kidney. A Galtseva, C-C Hui, ND Rosenblum Facundo Garcia-Bournissen, Clinical Pharmacology External contamination of hair with MDMA (Ecstasy) determined by hair analysis in two young girls from a meth lab. F Garcia-Bournissen, F Asrar, Z Verjee, T Karaskov, G Koren Joshua Greenberg, Haematology/Oncology Rhabdomyosarcoma cells have an active estrogen receptor-β-mediated pathway that exhibits sensitivity to estrogen, and undergo apoptosis in response to 4’OH-Tamoxifen. JA Greenberg, S Somme, R Giercksky, D Malkin Lars Grosse-Wortmann, Cardiology Magnetic resonance imaging in the preoperative assessment of marginally hypoplastic left ventricles. L Grosse-Wortmann, TJ Yu, O Al-Radi, S-J Yoo, G van Arsdell, A Redington Michael Hawkes, Infectious Diseases Development of Canadian guidelines for the prevention and management of community-associated methicillin-resistant Staphylococcus aureus in Canada: an example of knowledge translation in a CanMEDS framework. M Hawkes, M Barton, EL Ford-Jones Joelene Huber, Core Paediatrics Kids on Kids’ Health: Creating a child health magazine with students from an urban, multicultural, low income elementary school. JF Huber, M Porepa, C Lam, H Au, CS Birken Ryan Hung, Neurology Vitamin D levels, vitamin D intake and sunlight exposure in paediatric multiple sclerosis. R Hung, R Vieth, R Goldman, E Sochett, B Banwell Miho Inoue, Clinical Pharmacology Safety of Ketorolac for analgesia in children after cardiac surgery. M Inoue, CA Caldarone, PN Cox, S Ito, H Frndova, A Taddio, AM Guerguerian Mohammed Kashif Ishaqi, Haematology/Oncology Outcome of allogeneic haematopoietic stem cell transplantation for children with acute myelogenous leukaemia in second complete remission, single center experience. MK Ishaqi, S Afzal, A Dupuis, J Doyle, A Gassas Shailly Jain, Medical Genetics Increased elastolysis in Cutis Marmorata Telangiectasis Congenita. S Jain, A Hinek, M Beghetti, H Tresurer, G Taylor, M Silver, D Nykanen, D Chitayat Anita Jethwa, Developmental Paediatrics The development and evaluation of the HAT-D: A discriminative tool differentiating types of hypertonia in children. A Jethwa, D Fehlings, C Macarthur Ben-Hur Johnson, Neonatal-Perinatal Medicine Role of IL-1 in the neonatal rat model of chronic lung injury induced by exposure to 60% oxygen. B-H Johnson, M Yi, S Shek, R Belcastro, A Masood, RP Jankov, AK Tanswell Kassia Johnson, Developmental Paediatrics Books for siblings of children with Autism: Children’s assessments. K Johnson, P McKeever, A Kawamura Roman Jurencak, Rheumatology Antibody clustering in paediatric systemic lupus erythematosus (SLE). R Jurencak, M Fritzler, P Tyrell, L Hiraki, E Silverman Fotini Kavadas, Immunology/Allergy Antibiotic skin testing for children labeled with Type 1 hypersensitivity: A useful clinical tool. FD Kavadas, KR Seaban, Y Nofech-Mozes, M Husain, E White, AR Atkinson Adam Kirton, Neurology Novel corticospinal tract pre-wallerian degeneration in paediatric stroke: Acute MRI prediction of outcome and TMS correlates of reorganization. A Kirton, T Domi, R Chen, M Shroff, C Gunray, E Kouzmitcheva, G deVeber Dror Koltin, Endocrinology Idiopathic infantile hypercalcemia (IIH) revisited: Positive results in a cohort of 20 infants screened for mutations of the calcium sensing receptor (CASR) gene. D Koltin, M Rachmiel, EB Sochett, K Parker, BYL Wong, DEC Cole Catherine Lam, Core Paediatrics Efficacy of intravenous immunoglobulin therapy in juvenile dermatomyositis. C Lam, C Manlhiot, E Pullenayegum, PN Tyrell, BM Feldman Andrew C. Lau, Rheumatology Induction of MMP-9 by inflammatory cells leads to coronary artery aneurysm formation in a murine model of Kawasaki Disease. AC Lau, TT Duong, S Ito, RS Yeung DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 127 Elana Lavine, Immunology/Allergy Clinical course and immunological features of siblings with IRAK-4 deficiency. E Lavine, C Roifman Melanie Makhija, Infectious Diseases Paediatric neurocysticercosis – A twenty year review. M Makhija, J Brophy, D Tran, L Ford-Jones, J Keystone Azhar Masood, Neonatal-Perinatal Medicine Therapeutic hypercapnia protects against chronic neonatal lung injury, and prevents vascular remodeling, in the neonatal rat. A Masood, RP Jankov, M Yi, R Balcastro, S Shek, PJ McNamara, AK Tanswell Chantal Morel, Medical Genetics Cord blood stem cell transplantation in Niemann-Pick type 1A. CF Morel, A Gassas, J Doyle, JTR Clarke Aleixo Muise, Gastroenterology/Nutrition Protein tyrosine phosphate sigma is a susceptibility gene for ulcerative colitis. A Muise, A Griffiths, D Rotin Angela Orsino, Developmental Paediatrics A qualitative observational study of resident oral paediatric developmental case presentations. A Orsino, A Kawamura, L Lingard Joanne F. Paul, Emergency Medicine Perceptions of Paediatric Emergency Medicine fellows and program directors about their fellowship resuscitation teaching curricula. JF Paul, S Mehta Jonathan S. Ponesse, Developmental Paediatrics Patterns of nervous system and somatic malformation in Comorbid Mobius Syndrome and Autism Spectrum Disorder. JS Ponesse, H Wood, I Drmic, W Roberts Michelle Porepa, Core Paediatrics The diagnostic utility of MRI in differentiating between acute osteomyelitis and acute osteoinfarction in children with sickle cell disease. M Porepa, CS Birken, PS Babyn, A Doria, PC Parkin Dragos Predescu, Cardiology Resting cardiac index, ejection fraction and exercise capacity in a paediatric heart failure clinic population. D Predescu, G Smith, C Slorach, AN Redington, PF Kantor Rodica Retazar, Emergency Medicine Spinal injuries in paediatric trauma patients. R Retazar, V Wu, P Wales, A Mikrogianakis Dan W. Rhodes, Clinical Pharmacology Calls to a support center for pregnant and planning women regarding exposures: the Motherisk Program. DW Rhodes, G Koren Tal Schechter, Haematology/Oncology Dosing adequacy and safety of unfractionated heparin in infants with thrombotic complications. T Schechter, Y Finkelstein, S Williams, AK Chan, M Ali, LR Brandao Shazhan, Amed, Endocrinology Risk factors for Type 2 Diabetes (T2D) in youth with medication induced Diabetes (MID). A Shazan, H Dean, J Hamilton, NT1DM Study Team Sandesh Shivananda, Neonatal-Perinatal Medicine Is 02 administration safe during transport of neonates with CHD. S Shivananda, K Muthulaly, H Whyte, P McNamara Adam Shlien, Haematology/Oncology Genomic DNA copy number variations in Li-Fraumeni Syndrome: Distinguishing heritable and nonheritable tumours. A Shlien, M Pienkowska, B Baskin, S Nanda, H Druker, U Tabori, P Ray, D Malkin 128 DEPARTMENT OF PAEDIATRICS ANNUAL REPORT 2006-07 Lyn Sonnenberg, Developmental Paediatrics Addressing the rehabilitation needs of children with Spina Bifida in Kenya using participatory rural appraisal and community based rehabilitation: Bridging the gap. LK Sonnenberg, WD Biggar, T Kennedy, J Wee, R Bransford Arthur Staroselsky, Clinical Pharmacology Prenatal alcohol and parental psychopathology predict childhood behaviours: Preliminary results. A Staroselsky, E Fantus, G Koren, P Sandor, I Nulman Emily W.Y. Tam, Neurology Cerebellar diffusivity on neuroimaging correlates with intraventricular hemorrhage in the preterm neonate. EWY Tam, SP Miller, D Xu, JI Berman, DB Vigneron, AJ Barkovich, DM Ferriero Marilyn Tan, Neurology Occipital bone compression of the superior sagittal sinus as a contributory factor in neonatal cerebral sinovenous thrombosis. M Tan, M Shroff, G deVeber, M Moharir, A Pontigon, A Kirton Toshihiro Tanaka, Clinical Pharmacology The incidence of poor neonatal adaptation syndrome after intrauterine exposure to venlafaxine. T Tanaka, J Choi, Y Nomura, A Eirarson, G Koren, S Ito Tony Truong, Haematology/Oncology Features at presentation predict children with acute lymphoblastic leukaemia at low risk for tumour lysis syndrome. T Truong, J Beyene, J Hitzler, O Abla, AM Maloney, S Weitzman, L Sung Aoife Waters, Nephrology Ectopic notch activation in podocytes leads to structural alterations of the glomerular slit diaphragm. AM Waters, M Wu, T Onay, TD Piscione Constance Williams, Neonatal-Perinatal Medicine Do Neonatal Intensive Care Units follow parents’ preferences in withdrawal of life- sustaining treatment? C Williams, H Kirpalani Etan Wine, Gastroenterology/Nutrition A model of pathogen-induced intestinal barrier injury in chronic inflammatory bowel diseases: Campylobacter jejuni-mediated disruption of polarized epithelial cell monolayers. E Wine, PM Sherman Derek Wong, Cardiology Intra-operative trans-esophageal coronary Doppler flow patterns in patients with D-Transposition of the great arteries undergoing arterial switch operation. D Wong, L Hess, F Golding, C Calderone, B McCrindle, C Malhout, L Nield Man Yi, Neonatal-Perinatal Medicine Inhibition of apoptosis in 60% oxygen-induced neonatal rag lung injury. M Yi, A Ziino, B-H Johnson, A Masood, R Jankov, K Tanswell Meijian Zhao, Neonatal-Perinatal Medicine Cisapride exerts a prokinetic effect in infants with abdominal wall defects and severe dysmotility. M Zhao, NT de Silva, AM Moore, JH Kim Adrian J.A. Ziino, Neonatal-Perinatal Medicine Role of cyclooxygenase-2 in 60% oxygen-medicated lung injury and alveolar formation in the neonatal rat. AJA Ziino, M Yi, R Belcastro, S Shek, CR Pace-Asciak, RP Jankov, AK Tanswell Mohammed Zolaly, Haematology/Oncology Non-Hodgkin’s lymphoma in children under 5 years of age. M Zolaly, S Weitzman, A Naqvi, O Abla With thanks to Dodge Baena, Lesia Olexandra and Rob Teteruk for their vision and creativity 555 University Avenue Toronto, ON, Canada M5G 1X8 www.sickkids.ca/paediatrics www.paeds.utoronto.ca