Glomerulonephritis

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Orange Urine on Halloween
Eva Delgado, MD
Morning Report
Overview
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Case Presentation
Indications for Referral/Admission
Work-up based on Differential
Discussion of Pathophysiology
Treatment and Prognosis
Take Home Points
Case Presentation
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12 y/o F with OCD develops “orange” urine
with sediment.
PMD advises watchful waiting.
Case Presentation
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Orange urine persists x 4 days, so mom makes
appointment with PMD.
PMD notes elevated BP, weight gain, and then
orders one key test………..
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URINALYSIS: + hematuria, + proteinuria
A Word on Urinalysis
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UA with >/= 5 RBC/hpf on 3 samples over
several weeks = HEMATURIA
Only UA can distinguish between confounders:
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Myoglobin, hemoglobin, toxins, foods/coloring
Massengill, Peds In Review, 2008
A Word on Disposition
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Hematuria AND proteinuria
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Proteinuria may be due to notable hematuria, BUT..
Combination of both increases risk of renal disease
Hypertension
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Can be a symptom of fluid overload warranting
diuresis and further observation
PMD sends patient to the ED…..
Boineau and Lewy, Peds in Review, 1989.
Simckes and Spitzer, Ped in Review, 1995.
Physical Exam in ED
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Wt 60kg (↑ over last 6 mos)
VS: T 36.4, P 68, BP 146/80, R 20, 100% O2
Gen: no distress
HEENT: no peri-orbital edema, MMM
Chest: CTA b/l
CV: RRR, no murmur
Abd: soft, NTND
Ext: slight erythema in skin folds, no edema
Differential and Work-Up
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Urinalysis
Chemistry panel
CBC
C3, C4
ASO +/- Throat swab
Imaging
Do you need a biopsy?
Thinking of Glomerulonephritis
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Clues to look for in H&P:
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Edema, discolored urine (in 30-50%), HTN due to
↓GFR and hypervolemia, oliguria
Clues to look for in UA:
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+ hematuria, + proteinuria, + casts (60-85%)
RBC dysmorphology implies glomerular damage
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Sensitivity 95%, specificity 90%
McCory, Peds in Review, 1983.
Boineau and Lewy, Peds in Review 1989.
Glomerulopathy vs. Glomerulonephritis
Nephrotic Syndrome
Glomerulonephritis
Edema
R = Red urine (hematuria)
Proteinuria
O = Oliguria
Hypoproteinemia
P = Proteinuria
Elevated LDL
E = Elevated BP, BUN
Hypercoagulability
(↑fibrinogen, factor V)
Low C3 = Post-Strep,
Membranoproliferative, SLE
Silverstein, Laughing your way to passing the pediatric boards, 2008.
Laboratory Results
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UA: 3+ blood, 2+ protein, 1+ LE, 0 nitrites,
11-20 WBC, numerous RBC, 1-3 granular casts
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Chemistry: BUN/Cr = 17/0.8
CBC: Hbg 11.3, HCT 32.5, MCV 79
C3: 26 (86-184)
C4: 21.5 (20-59)
ASO: 2130 (<400 unit/ml); and + Rapid strep
Poststreptococcal GN
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Most common type of Acute GN
Usually occurs in 5-15 y/o
50% of cases are asymptomatic
Diagnosed by evidence of Group A Strep
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ASO titers detectable 2-4 weeks s/p pharyngitis
Anti-DNase-B titers helpful in post-pyoderma type
The Role of Grp A Strep
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Nephritogenic strains of streptococci
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Wall protein M12 in pharyngitis, M49 in pyoderma
These strains pose ~ 15% risk of  PSGN approx 2
weeks after initial infection
Antibiotic treatment to prevent GN?
Simckes and Spitzer, Peds in Review, 1995.
The Role of Grp A Strep
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PANDAS =
Post-infectious Autoimmune Neuropsychiatric Disorders
Associated with Streptococcal Infection
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Syndenham’s Chorea and Rheumatic Fever
OCD/Tic disorders shown to emerge or worsen with
temporal relation to Grp A Strep infection
PANDAS patients may have higher susceptibility to
Grp A Strep infection, + family h/o Rheumatic Fever
Kurlan et al. 121 (6): 1188. (2008) Pediatrics
Considering the DDX of PSGN
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Low C3
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80-90% of PSGN cases have low C3 x 2 mos
Also seen in GN due to SLE
Consider MPGN if low C3 > 2 mos
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MPGN can also present after infection
MPGN may also  low C4
Diagnose by biopsy
TRAM-TRACKING 
Considering DDX of PSGN
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Normal C3:
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IgA Nephropathy
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Alport’s
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Suspect if recurrent hematuria with URIs/Infections
+ Family History
HUS or HSP can present with gross hematuria
Post-viral GN
Boineau and Lewy Peds in Review 1989.
Treatment of AGN in General
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Admit if HTN, edema, or signs of renal failure
Monitor/correct electrolyte anomalies
Treat HTN to avoid sequelae
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Diuresis  loop diuretics like lasix
Fluid and salt restriction
Anti-hypertensives like the Ca-channel blockers
Treatment Specific to PSGN
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Antibiotics to target Grp A Strep
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Cultures often positive even if no symptoms,
suggesting active infection
Treatment may  milder course of PSGN
Epidemics of Grp A Strep may warrant ppx to
prevent PSGN, especially in underdeveloped
societies
Unclear/controversial role for impact on OCD
Rodriguez-Iturbe and Musser, J Am Soc Nephrology, 2008
Prognosis and Sequelae
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Good prognosis in children
CLOSE follow-up!
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HTN resolves in ~ 1-2 weeks
C3 levels return to normal in ~ 6 weeks
Gross hematuria resolves in ~ 6 weeks
Microscopic hematuria resolves in ~ 1 year
Proteinuria resolves in ~ 6 months
Progression to renal dysfunction RARE
McCrory, Peds in Review 1983.
Take Home Points
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Urinalysis is KEY test to w/u discolored urine
UA with casts/dysmorphic RBCs = GN
HTN, edema, or renal dysfunction  admit
Poststrep GN = most common, due to
characteristics of Strep and/or patient
Treat Strep infection and co-morbidities
Guarantee follow-up
Works Cited
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Boineau and Lewy, “Evaluation of Hematuria in Children and
Adolescents,” Pediatrics in Review, 1989.
Kurlan et al., “Streptococcal Infections and Exacerbations of
Childhood Tics and OCD Symptoms: A Prospective Blinded
Cohort Study,” Pediatrics 2008.
Massengill, “Hematuria,” Pediatrics in Review, 2008.
Rodriguez-Iturbe and Musser, “The Current State of
Poststreptococcal Glomerulonephritis,” Journal of American
Society of Nephrology, 2008.
Simckes and Spitzer, “Poststreptococcal Acute
Glomerulonephritis,” Pediatrics in Review, 1995.
McCrory, “Glomerulonephritis,” Pediatrics in Review, 1983.
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