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

To read more or access our algorithms and calculators, please log in or register.