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Peripartum management of two parturients with ornithine transcarbamylase deficiency.
MedLine Citation:
PMID:  22138526     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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:
U Ituk; O C Constantinescu; T K Allen; M J Small; A S Habib
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-12-2
Journal Detail:
Title:  International journal of obstetric anesthesia     Volume:  -     ISSN:  1532-3374     ISO Abbreviation:  -     Publication Date:  2011 Dec 
Date Detail:
Created Date:  2011-12-5     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9200430     Medline TA:  Int J Obstet Anesth     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2011 Elsevier Ltd. All rights reserved.
Affiliation:
Department of Anesthesiology, Duke University Health System, Durham NC, USA.
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