Document Detail

Pregnancy in a woman with maple syrup urine disease.
MedLine Citation:
PMID:  9584259     Owner:  NLM     Status:  MEDLINE    
We present the favourable outcome of a pregnancy in a woman with maple syrup urine disease. Keeping the maternal plasma levels of the branched-chain amino acids between 100 and 300 mumol/L is compatible with delivery of a normal infant. Leucine tolerance increased progressively from the 22nd week of gestation from 350 to 2100 mg/day. The risk of metabolic decompensation in the postpartum period can be minimized by careful monitoring of the mother after delivery.
S Grünewald; F Hinrichs; U Wendel
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of inherited metabolic disease     Volume:  21     ISSN:  0141-8955     ISO Abbreviation:  J. Inherit. Metab. Dis.     Publication Date:  1998 Apr 
Date Detail:
Created Date:  1998-06-11     Completed Date:  1998-06-11     Revised Date:  2007-03-21    
Medline Journal Info:
Nlm Unique ID:  7910918     Medline TA:  J Inherit Metab Dis     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  89-94     Citation Subset:  IM    
Department of Pediatrics, Heinrich-Heine-University Düsseldorf, Germany.
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MeSH Terms
Infant, Newborn
Leucine / administration & dosage,  blood
Maple Syrup Urine Disease / metabolism*
Pregnancy Complications / metabolism*
Reg. No./Substance:

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