Propionic acidemia in a parturient presenting for induction of labor.

Abstract

Propionic acidemia is a congenital disorder of metabolism where the enzyme propionyl-CoA carboxylase is nonfunctional, resulting in an accumulation of propionic acid in the blood. It is important to avoid excess protein intake and a catabolic state to prevent acidemia. Additionally, it may be wise to avoid anesthetic drugs metabolized by propionyl-CoA carboxylase because use of these drugs may lead to acidosis. We present a case of a parturient with propionic acidemia who presented for induction of labor.