Acute Pancreatitis in a Patient with Maple Syrup Urine Disease: A Management Paradox.
The Journal of Pediatrics(2018)
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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