Details

Autor(en) / Beteiligte

• Grÿnewald, S.
• Hinrichs, F.
• Wendel, U.

Titel

Pregnancy in a woman with maple syrup urine disease

Ist Teil von

• Journal of inherited metabolic disease, 1998-04, Vol.21 (2), p.89-94

Ort / Verlag

Dordrecht: Kluwer Academic Publishers

Erscheinungsjahr

1998

Link zum Volltext

Quelle

SpringerLink (Online service)

Beschreibungen/Notizen

• 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 µmol/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.