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

Nina B Gold, Jennifer A Blumenthal, Ann E Wessel, Deborah R Stein, Adam Scott, Victor L Fox, Amy Turner, Amy Kritzer, Farrah Rajabi, Katherine Peeler, Wen-Hann Tan

Journal of Pediatrics 2018 July

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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