Peripartum management of two parturients with ornithine transcarbamylase deficiency.

Journal Article (Journal Article)

Ornithine transcarbamylase deficiency is a rare X-linked disorder in which female carriers are usually heterozygous for the ornithine transcarbamylase deficiency gene. In pregnancy it has been associated with altered mental status, seizures, coma and death, especially in the postpartum period. We report the management of labor and delivery in two parturients with known ornithine transcarbamylase deficiency. Both patients were maintained on arginine, citrulline and sodium phenylacetate therapy with restricted protein intake during pregnancy. Neuraxial techniques were used for pain relief in labor and anesthesia for operative delivery. A dextrose infusion provided caloric intake during labor and perioperatively.

Full Text

Duke Authors

Cited Authors

  • Ituk, U; Constantinescu, OC; Allen, TK; Small, MJ; Habib, AS

Published Date

  • January 2012

Published In

Volume / Issue

  • 21 / 1

Start / End Page

  • 90 - 93

PubMed ID

  • 22138526

Electronic International Standard Serial Number (EISSN)

  • 1532-3374

Digital Object Identifier (DOI)

  • 10.1016/j.ijoa.2011.09.007


  • eng

Conference Location

  • Netherlands