Format

Send to

Choose Destination
J Pediatr. 2018 Jul;198:313-316. doi: 10.1016/j.jpeds.2018.02.063. Epub 2018 Apr 19.

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

Author information

1
Division of Genetics and Genomics, Boston Children's Hospital, Boston, MA; Harvard Medical School Genetics Training Program, Harvard Medical School, Boston, MA.
2
Division of Critical Care Medicine, Boston Children's Hospital, Boston, MA; Division of Infectious Diseases, Boston Children's Hospital, Boston, MA.
3
Division of Genetics and Genomics, Boston Children's Hospital, Boston, MA.
4
Division of Nephrology, Boston Children's Hospital, Boston, MA.
5
Division of Gastroenterology, Hepatology and Nutrition, Boston Children's Hospital, Boston, MA.
6
Division of Medicine Critical Care, Boston Children's Hospital, Boston, MA.
7
Division of Genetics and Genomics, Boston Children's Hospital, Boston, MA. Electronic address: wen-hann.tan@childrens.harvard.edu.

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.

KEYWORDS:

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

PMID:
29681447
DOI:
10.1016/j.jpeds.2018.02.063
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center