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J Inherit Metab Dis. 1998 Apr;21(2):89-94.

Pregnancy in a woman with maple syrup urine disease.

Author information

1
Department of Pediatrics, Heinrich-Heine-University Düsseldorf, Germany.

Abstract

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.

PMID:
9584259
DOI:
10.1023/a:1005396823030
[Indexed for MEDLINE]

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