Management of a Woman With Maple Syrup Urine Disease During Pregnancy, Delivery, and Lactation.
JPEN J Parenter Enteral Nutr
; 39(7): 875-9, 2015 Sep.
Article
em En
| MEDLINE
| ID: mdl-24618664
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.
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Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Complicações na Gravidez
/
Lactação
/
Doença da Urina de Xarope de Bordo
Idioma:
En
Ano de publicação:
2015
Tipo de documento:
Article