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JPEN J Parenter Enteral Nutr. 2015 Sep;39(7):875-9. doi: 10.1177/0148607114526451. Epub 2014 Mar 11.

Management of a Woman With Maple Syrup Urine Disease During Pregnancy, Delivery, and Lactation.

Author information

1
Center for Nutrition, Boston Children's Hospital, Boston, Massachusetts ann.wessel@childrens.harvard.edu.
2
Department of Nutrition, Brigham and Women's Hospital, Boston, Massachusetts.
3
Center for Nutrition, Boston Children's Hospital, Boston, Massachusetts.
4
Division of Genetics and Genomics, Boston Children's Hospital, Boston, Massachusetts Department of Pediatrics, Harvard Medical School, Boston, Massachusetts.
5
Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts.
6
Division of Maternal Fetal Medicine and Reproductive Genetics, Brigham and Women's Hospital, Boston, Massachusetts.
7
Division of Maternal Fetal Medicine and Reproductive Genetics, Brigham and Women's Hospital, Boston, Massachusetts Department of Obstetrics, Gynecology and Reproductive Biology, Harvard Medical School, Boston, Massachusetts.

Abstract

Maple syrup urine disease (MSUD) is an inherited disorder of metabolism of the branched-chain amino acids leucine, isoleucine, and valine. Complications of acute elevation in plasma leucine include ketoacidosis and risk of cerebral edema, which can be fatal. Individuals with MSUD are at risk of metabolic crisis throughout life, especially at times of physiological stress. We present a case of successful management of a woman with MSUD through pregnancy, delivery, postpartum, and lactation, including nutrition therapy using modified parenteral nutrition.

KEYWORDS:

MSUD; lactation; maple syrup urine disease; parenteral nutrition; pregnancy

PMID:
24618664
DOI:
10.1177/0148607114526451
[Indexed for MEDLINE]
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