Pediatric Renal Rounds By Brent Lee Lechner MAJ, MC, USA

Agenda One Case Presentation: Oh, Dear Abby ! One Trivia Question

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

Emergency Department Vitals: normal including BP and HR Diffuse, tender abdomen 10 % Volume Depletion Lab: Normal LFTs, Amylase and Lipase UA: SG: 1.030 LE(2+) Glucose(-) Protein (2+) Nitrite (-) WBC 10-20 /hpf Blood (Large) Ketones(-) RBC > 50/ hpf CT Abdomen with contrast 137 103 16 92 4.1 20 0.7

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

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

Labs on Transfer Urine Culture: negative Serologies: UA: Micro of urine: C3: 112 SG 1.030 50+ RBC/hpf ASO: Negative Blood (Large) (40 % Dysmorphic) ANA: Negative Protein (2+) 10 Granular Cast/hpf ESR: 59 LE(2+) 0 RBC casts Nitrite(-) Ketones (-) Glucose (-) 12.0 148 110 63 15.8 440 82 35.8 5.0 16.5 3.2

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

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

Next Move Day 18: Renal Biopsy!

Treatment Approach Methylprednisolone 500 mg IV for 3 Days 35 mg/kg/d for 3 days Converted to Methylprednisolone (2mg/kg/d)

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 !

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)

Complement refresher C3NeF

MPGN Type II Initial Studies 50 % 10-year renal survival (Cameron et al) 1983 West CD 1980 Alternate day 60 mg/m2 Prednisone Retrospective study 71 patients: Renal Survival at 10 year mark 82, 56% after 20 years

Do steroids aid in the regression of MPGN ? “Regression of Membranoproliferative GN Type II (DDD): Observations in Six Children” McEnery and Adams AJKD 1988

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: 1970-1980 Ages: 5-17 80 Children End Point: Increase in creatinine 0.4 mg.dl

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

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

What is the only current active aircraft carrier named after democratic President?