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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
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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 103 16 137 92 0.7 20 4.1
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 (-) 110 12.0 148 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?