Patient #1

Transcription

Patient #1
Patient #1
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A 7y/o boy presents to your office with his mother with a
CC of abdominal pain. He is an active child who was in
his USOH until this morning when he began to c/o nonspecific abdominal pain. Mom also reports that his urine
was darker than usual this morning. She thought it was
secondary to dehydration, but became more concerned
when the abd pain began.
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The child is noted to be active and in no distress; mom
explains that the abdominal pain has since resolved and
he is now asking for food
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On PE, pt is afebrile. Vital signs are normal except for a
BP of 127/78. No other abnormalities are found.
Abdominal exam is benign w/ no tenderness
Patient #2
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A 7 y/o girl presents to your office for a routine check
up. She appears well and mom does not report any
problems
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You have never seen this patient in the past and decide
to get a screening urinalysis b/c she has never had one
sent in the past
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The results to her urine dipstick demonstrate 2+ blood;
otherwise nl
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A UA w/ micro is sent during the same visit and
demonstrates 15-20RBCs/hpf w/ no other abnormalities
Evaluation of Hematuria
Morning Report
January 13th 2005
Definitions
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Macroscopic (gross) hematuria- visible to the naked eye
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Microscopic hematuria- confirmed by microscopic
examination of the spun urine sediment
– >5 RBCs/hpf on a spun urine
– Trace blood or higher by urine dipstick
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Absence of RBCs in the urine with a positive dipstick
suggests hemoglobinuria or myoglobinuria
– Visualizing a spun urine will help distinguish b/w true hematuria
and other causes of red urine
Glomerular vs. Non-glomerular
Glomerular
Extra-Glomerular
Color
Red, smoky brown or
“coca cola”
Red or Pink
Clots
Absent
May be present
(or crystals)
Proteinuria
May be >500mg/day
<500mg/day
RBC morphology
Dysmorphic
Normal
RBC casts
May be present
(highly specific)
Absent
History
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Abdominal or flank pain
Dysuria, frequency,
urgency
Trauma
Strenuous exercise
Menstruation
Recent URI/ sore throat
Skin rashes/ skin infx
Diarrhea (especially
bloody)
Joint pains/swellings
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Medications/toxins
h/o sickle cell disease or
trait
*Family history:
hematuria, hearing loss,
HTN, stones, renal
disease, dialysis or
transplant, coagulopathy,
sickle cell trait
Physical Exam
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Blood pressure
Fever
Abdomen for masses, tenderness
(flank, suprapubic)
Rashes, evidence or trauma, bruising
Edema (especially periorbital)
Joint erythema, swelling, warmth
Paleness, jaundice
Careful inspection of external genitalia
If BP is elevated, further evaluation is immediately warranted
General Rules
At least 2 of 3 urinalyses should show
microhematuria over 2-3 weeks before further
evaluation is done
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Initial evaluation for any type of hematuria always
includes:
– microscopic evaluation of a spun urine
– Urine culture
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Indications for prompt evaluation include hematuria
with:
– hypertension, edema, oliguria, significant proteinuria
or RBC casts
Macroscopic Hematuria
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Requires prompt evaluation to exclude potentially lifethreatening causes!
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Most common cause presenting to an ER is UTI
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Most common glomerular causes are
post-strep GN and IgA nephropathy
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Other causes: blunt trauma, renal calculi, hypercalciuria,
sickle cell trait, local irritation or trauma to perineal area,
exercise, coagulopathy, tumors
Macroscopic Hematuria: Evaluation
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OBTAIN A THOROUGH HISTORY AND PHYSICAL!
GN suspected (edema, HTN, protein, RBC casts)
– BMP, CBC, C3, C4, albumin, ASO titer, anti-DNAseB, ANA
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History of Trauma
– CT scan abdomen/pelvis
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UTI suspected
– Urine culture, +/- renal ultrasound
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Stones suspected or Family h/o stones
– Imaging (KUB, ultrasound, CT) Urine Ca:Cr (or 24 hr urine)
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No obvious cause?Æ consider renal U/S, urine culture,
test parents, sibs for hematuria, Hgb electrophoresis,
Urine Ca:Cr ratio
Microscopic Hematuria:
„ Common
causes include urinary tract
infection, benign familial hematuria (or thin
basement membrane nephropathy), hypercalciuria, IgA
nephrophathy, Alport’s syndrome
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children with isolated asymptomatic
microscopic hematuria do not have a
treatable or serious cause and do not
require extensive evaluation
Note that most of these conditions can also present w/ gross hematuria
Asymptomatic Microscopic Hematuria:
Evaluation
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Repeat UA weekly X 2 (w/out exercise)
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Persistent hematuria:
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If work-up is negative (and no other concerning signs/sx),
follow w/ yearly UA (UA w/ micro, dipsick for protein, BP)
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BUN/Cr, urine cx (low yield)
Test parents and sibs for hematuria
Urine Ca:Cr ratio or 24hr urine for calcium excretion
Consider hearing test, renal ultrasound, Hgb
electrophoresis, coagulation studies (LOW YIELD)
Recent studies have questioned the need for a diagnostic evaluation for
asymptomatic microscopic hematuria
Microscopic Hematuria w/ abnormal findings
(history, physical or UA)
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protein, edema or HTN:
– Work-up according to associated findings
ƒ h/o trauma- CT if > 50 RBCs/hpf
ƒ S/Sx of UTI- urine culture
ƒ S/Sx of stones- imaging studies, Urine Ca:Cr
ƒ Abdominal mass- renal U/S or CT
ƒ +family history- hearing test, Urine Ca:Cr, imaging
– If diagnosis is still unclear- refer to
nephrologist
Microscopic Hematuria w/ abnormal findings
(history, physical or UA)
„ Presence
of protein, HTN or edema
– Hematuria + proteinuria strongly suggests GN
– If patient is acutely ill
ƒ BMP, CBC, C3, C4, albumin; consider ASO, DNAseB
titers, ANA
– If patient is not acutely ill (protein w/out HTN, edema)
ƒ Recheck UA in one week
– Persistent hematuria/proteinuria
ƒ Send labs listed above and consider referral
Glomerular Causes of Hematuria
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Acute Post-infectious glomerulonephritis
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IgA Nephropathy
Henoch-Schonlein purpura
System lupus erythematosus
Rapidly progressive glomerulonephritis
Alport hereditary nephritis
Benign familial hematuria (Thin basement
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membrane nephropathy)
Note that most glomerular causes of hematuria will also
have proteinuria
Acute Post-infectious Glomerulonephritis
poststreptococcal glomerulonephritis
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Most common cause is nephritogenic strains of
GrA strep (can also follow other bacterial and viral illnesses)
Acute onset of tea-colored urine (or microscopic)
edema, HTN, oliguria, malaise, abd pain OR
asymptomatic
previous illness or skin infection 1-3 weeks prior
to onset
UA w/ protein, RBC casts; +/- elevated BUN/Cr
Elevated ASO or DNaseB titer confirms infx
Most useful lab: Decreased C3
Pathophysiology of GN
Process
Manifestation
Glomerular
Inflammation
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⇓ Glomerular
Blood Flow
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⇑ Na+ & H2O
Retention
Hematuria &
Proteinuria
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⇓ GFR &
Azotemia
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Oliguria, Edema
& Hypertension
Slide courtesy of Dr. Clardy
Acute Post-infectious Glomerulonephritis
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Supportive/Symptomatic treatment
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Most important considerations:
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Gross hematuria and other Sx resolve w/in a few weeks
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C3 should return to normal in 6-8 wks
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Prognosis is excellent
– Na restriction, diuretics, ACE inhibitors
– monitor BP and manage hypertension
– Must distinguish PSAGN from other forms of GN
– Microscopic hematuria may persist for 1-2 yrs
– If C3 remains elevatedÆ further evaluation is indicated
Glomerulonephritis: differential
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Hypocomplementemic (low C3)
– Post Streptococcal GN
– Membranoproliferative GN
– Systemic Lupus Erythematosus
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Normocomplementemic (normal C3)
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IgA Nephropathy
Henoch-Schönlein Purpura
Idiopathic Vasculitis
Rapidly Progressive GN
Slide courtesy of Dr. Clardy
Indications for Biopsy
all patients unlikely to have poststreptococcal GN as determined by…
„ Biopsy
– Normal initial C3
– Failure of low C3 to normalize after 8 weeks
– Positive ANA
– Progressive azotemia
Slide courtesy of Dr. Clardy
IgA Nephropathy
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Most common cause of glomerulonephritis
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Presents w/ gross hematuria during a URI or GI illness
OR asymptomatic microscopic hematuria/proteinuria
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Episodes of gross hematuria resolve in a few days
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Renal biopsy is required for diagnosis
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~25% develop slowly progressive renal dysfunction
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No specific treatment
– Mesangial IgA deposits are seen on immunoflorescence
– Renal bx for definitive dx in patients w/ asymptomatic hematuria
is controversial
Rapidly Progressive Glomerulonephritis
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One of the few emergencies in pediatric
nephrology
Clinical presentation similar to APIGN (but nl C3)
Many causes of GN can present w/ rapid
progression or it can be idiopathic (juvenile acute
nonproliferative glomerulonephritis)
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Labs w/ signs of renal insufficiency; renal bx w/
glomerular crescents
Untreated RPGN can progress to ESRD in a few
weeks
Prompt Dx and Tx with pulse steroids may
prevent progression to ESRD
Benign Familial Hematuria
(Thin glomerular basement membrane nephropathy)
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common cause of persistent glomerular bleeding in
children
Inheritance is autosomal dominant or recessive
Family history is helpful in that there are no relatives
with significant renal disease
Hematuria is usually microscopic (gross hematuria may occur w/
URIs); Proteinuria is rare
Diagnosis of exclusion
– Send UA on family members to detect hematuria
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Monitor w/ yearly UA for development of proteinuria and
BP measurement
– Any abnormal findings would suggest a dx other than BFH
Interstitial and Vascular Causes of
Hematuria
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Infectious
(pyelonephritis)
Tumors (esp. Wilm’s Tumor)
Nephrocalcinosis
Interstitial nephritis
(NSAIDs)
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Cystic renal disease
(simple,PCKD, multicystic dysplasia)
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Renal transplant
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Sickle cell
disease/trait
Renal vein thrombosis
Renal artery
thrombosis
AVM
Urinary Tract causes of Hematuria
„ Infection
(UTI) (bacterial or viral-esp. adenovirus)
„ Hypercalciuria
„ Nephrolithiasis
„ Anatomic abnormality (UPJ obstruction, VUR)
– Common presentation of UPJ obstruction is gross
hematuria after minor trauma in an adolescent
Hypercalciuria (w/out stones)
„ Increased
urinary excretion of calcium
despite normal serum calcium
„ Pathogenesis of hematuria is not well
understood
„ Family history of renal stones is common
„ Asymptomatic microscopic hematuria is
most common
– Gross hematuria, flank pain, dysuria can occur
Hypercalciuria (w/out stones)
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Diagnosis: Spot Urine Ca:Cr >.2 in children
>6yrs is abnormal
– >.42 in 19mo-6yrs is abnormal
– 24hr urine for calcium: >4mg/kg/day is abnormal
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Treat w/ increased fluid intake
– Thiazide diuretics and Na restriction can be used if
+stones or family h/o stones
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~15% of patients develop stones in the next 4-6
years
Approach for Referral
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Nephrologist:
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Urologist:
– APSGN if patient has HTN, azotemia, or hyperkalemia
– Other forms of GN (especially if proteinuria, HTN, or
persistent low C3 is present)
– Family h/o renal failure
– Systemic disease
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Abnormal GU anatomy
Trauma
Stones (Nephrologist for metabolic w/u)
Tumor
Non-glomerular gross hematuria
Screening Urinalysis
„ AAP
recommends that screening
urinalyses be done routinely on 2 groups:
– 5-year old children
– Sexually active male and female adolescents
(11-20 yrs)
-Committee on Practice and Ambulatory
Medicine. Recommendations for Preventive
Pediatric Health Care. Pediatrics 2000;105