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Pediatric Renal Rounds
By Brent Lee Lechner MAJ, MC, USA
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Agenda One Case Presentation: Oh, Dear Abby ! One Trivia Question
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History 4-year-old white female Dx: Left otitis media
Started on Amoxicillin Developed Rash on Back: Day 5 Antibiotic change to Zithromax Day 2 on Zithromax – nausea/vomiting (bilious) Noted to have hematuria on dipstick Sent to the Emergency Department
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Emergency Department Vitals: normal including BP and HR
Diffuse, tender abdomen 10 % Volume Depletion Lab: Normal LFTs, Amylase and Lipase UA: SG: LE(2+) Glucose(-) Protein (2+) Nitrite (-) WBC /hpf Blood (Large) Ketones(-) RBC > 50/ hpf CT Abdomen with contrast 137 103 16 92 4.1 20 0.7
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Admission: Outside Hospital
Negative CT scan of Abdomen Normal Vitals Normal Exam except volume depletion Admitted to Pediatrics for serial abdominal exam and volume repletion Urine output tapered off even with Rehydration
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Admission History Negative History for the following:
Fever URI symptoms or UTI symptoms Joint Pain, Swollen Joints Sore throat Impetigo Rashes (Drug Rash disappeared) Transferred to our hospital after overnight hydration and minimal UOP
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Labs on Transfer Urine Culture: negative
Serologies: UA: Micro of urine: C3: SG RBC/hpf ASO: Negative Blood (Large) (40 % Dysmorphic) ANA: Negative Protein (2+) Granular Cast/hpf ESR: LE(2+) RBC casts Nitrite(-) Ketones (-) Glucose (-) 12.0 148 110 63 15.8 440 82 35.8 5.0 16.5 3.2
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Hospital Course Urine Output: minimal (<0.2 cc/kg/d)
Peritoneal Dialysis initiated in am T-catheter placement by Transplant Surgery Renal US: [two days after admission] Hyperechogenic kidneys 9 cm Bilaterally (Large for Age) No Hydronephrosis Patent Renal Vessels with good blood flow
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Hospital Course Peritoneal Dialysis Complication:
Nausea/Vomiting persisted Work-up included upper GI series & abdominal US Pancreatitis: amylase and lipase Very Large pancreas on ultrasound TPN initiated on Day 4 Repeat Serologies: C3, C4, and ANA (-), C3NeF, Factor H
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Next Move Day 18: Renal Biopsy!
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Treatment Approach Methylprednisolone 500 mg IV for 3 Days
35 mg/kg/d for 3 days Converted to Methylprednisolone (2mg/kg/d)
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Abby Goes Home After 28 days of PD and minimal urine output, Abby began to produce urine and her creatinine dropped to 0.8 mg/dl at the time of discharge !
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MPGN Type II Children > 5 years of age (5-15), Male=Female
Caucasians and reports of familial clusters Less than 4% of Children with Nephrotic Syndrome 95% low plasma C3 Less than 20 % of all MPGN in children 80% of MPGN Type II patients are positive for C3NeF Genetic Mutations Factor H Partial Lipodystrophy (C3NeF) 50% Anemic (hemolysis driven – autoantibody)
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Complement refresher C3NeF
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MPGN Type II Initial Studies
50 % 10-year renal survival (Cameron et al) 1983 West CD Alternate day 60 mg/m2 Prednisone Retrospective study 71 patients: Renal Survival at 10 year mark 82, 56% after 20 years
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Do steroids aid in the regression of MPGN ?
“Regression of Membranoproliferative GN Type II (DDD): Observations in Six Children” McEnery and Adams AJKD 1988
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Do steroids aid in the regression of MPGN ?
“Treatment of MPGN with qod prednisone – a report of The International Study of Kidney Disease in Children” Tarish (1992) Ped Nephrology Controlled, double-blinded Study Years: Ages: 5-17 80 Children End Point: Increase in creatinine 0.4 mg.dl
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Do steroids aid in the regression of MPGN ?
“Treatment of MPGN with qod prednisone – a report of The International Study of Kidney Disease in Children” Tarish (1992) Ped Nephrology
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Trivia Question The current active U.S. Navy fleet of aircraft carriers includes the following list of vessels named after Presidents. USS Abraham Lincoln USS Theodore Roosevelt USS Ronald Reagan USS Dwight D. Eisenhower USS George Herbert Walker Bush
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What is the only current active aircraft carrier named after democratic President?
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