• Int J Obstet Anesth · Jan 2012

    Case Reports

    Peripartum management of two parturients with ornithine transcarbamylase deficiency.

    • U Ituk, O C Constantinescu, T K Allen, M J Small, and A S Habib.
    • Department of Anesthesiology, Duke University Health System, Durham NC 27710, USA. unyime@gmail.com
    • Int J Obstet Anesth. 2012 Jan 1;21(1):90-3.

    AbstractOrnithine 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.Copyright © 2011 Elsevier Ltd. All rights reserved.

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