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

The Journal of Pediatrics（2018）

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摘要

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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关键词

fluid resuscitation,cerebral edema,renal-replacement therapy,anticoagulation,branched chain amino acids

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