Acute Pancreatitis in a Patient with Maple Syrup Urine Disease: A Management Paradox.

Gold, Nina B; Blumenthal, Jennifer A; Wessel, Ann E; Stein, Deborah R; Scott, Adam; Fox, Victor L; Turner, Amy; Kritzer, Amy et al. · J Pediatr · 2018

case_report · Level V

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Abstract

Maple syrup urine disease (MSUD) is an inborn error of metabolism that causes elevated leucine in the setting of acute illnesses. We describe an 8-year-old boy with MSUD who developed acute pancreatitis and subsequent leucinosis. This case highlights the complexities of fluid management in patients with MSUD.

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