Pregnancy in a woman with maple syrup urine disease.
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.
Grünewald
S
S
Department of Pediatrics, Heinrich-Heine-University Düsseldorf, Germany.
Hinrichs
F
F
Wendel
U
U
D000328:Adult; D000818:Animals; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007930:Leucine / Q000008:administration & dosage / Q000097:blood; D008375:Maple Syrup Urine Disease / Q000378:metabolism*; D011247:Pregnancy; D011248:Pregnancy Complications / Q000378:metabolism*
D007930:Leucine