title: Peripartum management of two parturients with ornithine transcarbamylase deficiency.
abstract: 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.
authors.affiliation: Department of Anesthesiology, Duke University Health System, Durham NC 27710, USA. unyime@gmail.com
authors.forename: U
authors.initials: U
authors.lastname: Ituk
authors.forename: O C
authors.initials: OC
authors.lastname: Constantinescu
authors.forename: T K
authors.initials: TK
authors.lastname: Allen
authors.forename: M J
authors.initials: MJ
authors.lastname: Small
authors.forename: A S
authors.initials: AS
authors.lastname: Habib
mesh_terms: D000328:Adult; D000773:Anesthesia, Obstetrical; D005260:Female; D006801:Humans; D020163:Ornithine Carbamoyltransferase Deficiency Disease / Q000628:therapy*; D058725:Peripartum Period; D011247:Pregnancy; D011248:Pregnancy Complications / Q000628:therapy*; D014508:Urea / Q000378:metabolism
chemical_list: D014508:Urea