Orange Urine on Halloween

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Orange Urine on Halloween

• Eva Delgado, MD
• Morning Report

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Overview

• Case Presentation
• Indications for Referral/Admission
• Work-up based on Differential
• Discussion of Pathophysiology
• Treatment and Prognosis
• Take Home Points

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Case Presentation

• 12 y/o F with OCD develops orange urine with
  sediment.
• PMD advises watchful waiting.

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Case Presentation

• Orange urine persists x 4 days, so mom makes
  appointment with PMD.
• PMD notes elevated BP, weight gain, and then
  orders one key test..
• URINALYSIS hematuria, proteinuria

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A Word on Urinalysis

• UA with gt/ 5 RBC/hpf on 3 samples over several
  weeks HEMATURIA
• Only UA can distinguish between confounders
• Myoglobin, hemoglobin, toxins, foods/coloring

Massengill, Peds In Review, 2008
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A Word on Disposition

• Hematuria AND proteinuria
• Proteinuria may be due to notable hematuria,
  BUT..
• Combination of both increases risk of renal
  disease
• Hypertension
• 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.
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Physical Exam in ED

• 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

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Differential and Work-Up

• Urinalysis
• Chemistry panel
• CBC
• C3, C4
• ASO /- Throat swab
• Imaging
• Do you need a biopsy?

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Thinking of Glomerulonephritis

• Clues to look for in HP
• Edema, discolored urine (in 30-50), HTN due to
  ?GFR and hypervolemia, oliguria
• Clues to look for in UA
• hematuria, proteinuria, casts (60-85)
• RBC dysmorphology implies glomerular damage
• Sensitivity 95, specificity 90

McCory, Peds in Review, 1983. Boineau and Lewy,
Peds in Review 1989.
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Glomerulopathy vs. Glomerulonephritis
Silverstein, Laughing your way to passing the
pediatric boards, 2008.
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Laboratory Results

• UA 3 blood, 2 protein, 1 LE, 0 nitrites,
• 11-20 WBC, numerous RBC, 1-3 granular casts
• 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 (lt400 unit/ml) and Rapid strep

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Poststreptococcal GN

• 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
• ASO titers detectable 2-4 weeks s/p pharyngitis
• Anti-DNase-B titers helpful in post-pyoderma type

Simckes and Spitzer, Peds in Review 1995.
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The Role of Grp A Strep

• Nephritogenic strains of streptococci
• 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. Rodriguez-Iturbe and Musser, J Am Soc
Nephrology, 2008
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The Role of Grp A Strep

• PANDAS
• Post-infectious Autoimmune Neuropsychiatric
  Disorders Associated with Streptococcal Infection
• Syndenhams 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
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Considering the DDX of PSGN

• Low C3
• 80-90 of PSGN cases have low C3 x 2 mos
• Also seen in GN due to SLE
• Consider MPGN if low C3 gt 2 mos
• MPGN can also present after infection
• MPGN may also ? low C4
• Diagnose by biopsy

TRAM-TRACKING ?
McCrory, Peds In Review, 1983.
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Considering DDX of PSGN

• Normal C3
• IgA Nephropathy
• Suspect if recurrent hematuria with
  URIs/Infections
• Alports
• Family History
• HUS or HSP can present with gross hematuria
• Post-viral GN

Boineau and Lewy Peds in Review 1989.
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Treatment of AGN in General

• Admit if HTN, edema, or signs of renal failure
• Monitor/correct electrolyte anomalies
• Treat HTN to avoid sequelae
• Diuresis ? loop diuretics like lasix
• Fluid and salt restriction
• Anti-hypertensives like the Ca-channel blockers

Simckes and Spitzer Peds in Review, 1995.
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Treatment Specific to PSGN

• Antibiotics to target Grp A Strep
• 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
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Prognosis and Sequelae

• Good prognosis in children
• CLOSE follow-up!
• 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.
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Take Home Points

• 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

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Works Cited

• 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.