A woman with maple syrup urine disease (MSUD) can have a successful pregnancy if she is carefully managed before, during and after the pregnancy.
The woman should be carefully followed by someone experienced with MSUD during pregnancy for:
(1) blood levels of amino acids
(2) adequacy of nutritional intake
(3) acute illness
The mother may experience acute metabolic decompensation:
(1) with vomiting
(2) during infection
(3) during the post-partum state
Hazards to the fetus:
(1) teratogenic effects if leucine and branched chain amino acids are elevated
(2) deficiency of essential amino acids as fetus matures
(3) birth with phenylketonuria
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Specialty: Obstetrics & Gynecology, Endocrinology
