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Propionic acidemia in a parturient presenting for induction of labor

Alison Schmeck1, Yaakov Beilin

  • 1From the Departments of *Anesthesiology and †Obstetrics, Gynecology and Reproductive Sciences, Icahn School of Medicine at Mount Sinai, New York, New York.

A & a Case Reports
|January 23, 2015
PubMed

Insights

Propionic acidemia is a rare metabolic disorder. This case highlights the importance of avoiding certain anesthetic drugs during labor induction in affected patients to prevent acidosis.

Area of Science:

  • Biochemistry
  • Genetics
  • Obstetrics

Background:

  • Propionic acidemia is an inherited metabolic disorder caused by a nonfunctional propionyl-CoA carboxylase enzyme.
  • This enzyme deficiency leads to the accumulation of propionic acid in the blood, a condition known as acidemia.
  • Management involves dietary protein restriction and avoiding catabolic states to prevent metabolic crises.

Purpose of the Study:

  • To present a case of a parturient with propionic acidemia undergoing labor induction.
  • To discuss the potential risks associated with anesthetic drug metabolism in patients with propionic acidemia.
  • To emphasize the importance of careful anesthetic selection in managing pregnant patients with this metabolic disorder.

Main Methods:

  • Case report presentation.
  • Review of relevant literature on propionic acidemia and anesthetic considerations.
  • Clinical management of a parturient with propionic acidemia during labor induction.

Main Results:

  • The case involved a pregnant patient with a known diagnosis of propionic acidemia.
  • The potential for anesthetic drugs metabolized by propionyl-CoA carboxylase to induce acidosis was considered.
  • Successful labor induction was managed with careful consideration of the patient's metabolic condition.

Conclusions:

  • Patients with propionic acidemia require specialized anesthetic management during labor.
  • Avoiding anesthetic agents metabolized by propionyl-CoA carboxylase is crucial to prevent acidosis.
  • This case underscores the need for multidisciplinary collaboration in managing pregnant patients with rare metabolic disorders.

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