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  • Title: Pregnancy in a woman with maple syrup urine disease.
    Author: Grünewald S, Hinrichs F, Wendel U.
    Journal: J Inherit Metab Dis; 1998 Apr; 21(2):89-94. PubMed ID: 9584259.
    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.
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