| Management of ornithine transcarbamylase deficiency in pregnancy. | |
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MedLine Citation:
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PMID: 20458665 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Ornithine transcarbamylase (OTC) deficiency is the most common enzymatic deficiency in the urea cycle. In catabolic states, such as the intrapartum and immediate postpartum periods, hyperammonemic comas with permanent neurological damage and death can develop. We report six cases of OTC deficiency during pregnancy managed at our institution and review the literature on OTC deficiency during pregnancy. Using the patient database from our Metabolic Clinic, pregnant OTC deficiency carriers were identified. The antenatal, intrapartum, and postpartum periods were analyzed. Corresponding literature was reviewed and an extensive multidisciplinary management plan developed. All six pregnant women had favorable outcomes. No hyperammonemic episodes occurred, and intensive care unit admissions and hemodialysis were not required. Although risk to women with OTC deficiency during the intra- and postpartum period exists, multidisciplinary management and a coherent plan usually result in successful labor, delivery, and postpartum. A comprehensive plan for patients who develop hyperammonemia is recommended. |
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Authors:
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Hector Mendez-Figueroa; Kerri Lamance; V Reid Sutton; Kjersti Aagaard-Tillery; Ignatia Van den Veyver |
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Publication Detail:
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Type: Case Reports; Journal Article; Review Date: 2010-05-10 |
Journal Detail:
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Title: American journal of perinatology Volume: 27 ISSN: 1098-8785 ISO Abbreviation: Am J Perinatol Publication Date: 2010 Nov |
Date Detail:
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Created Date: 2010-10-14 Completed Date: 2010-11-29 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8405212 Medline TA: Am J Perinatol Country: United States |
Other Details:
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Languages: eng Pagination: 775-84 Citation Subset: IM |
Copyright Information:
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© Thieme Medical Publishers. |
Affiliation:
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Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Baylor College of Medicine, Houston, TX 77030, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Disease Management Female Genetic Counseling Heterozygote Humans Hyperammonemia / therapy Infant, Newborn Ornithine Carbamoyltransferase Deficiency Disease / genetics, therapy* Pregnancy Pregnancy Complications / genetics, therapy* Young Adult |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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