Document Detail


The genetics of congenital diaphragmatic hernia.
MedLine Citation:
PMID:  16050525     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Congenital diaphragmatic hernia (CDH) is a common birth defect with a high mortality and morbidity. A clear understanding of the pathogenesis of CDH is critical for determining prognosis and planning treatment, but to date, information on the genetic etiology of both nonsyndromic and syndromic CDH is limited. This paper summarizes the current knowledge concerning the genes, syndromes, and chromosome aberrations associated with CDH in humans and in animal model systems. Mutations in several different genes have been described in syndromic CDH, but there is only one mutation that has been reported in non-syndromic CDH to date. However, animal models suggest that genes involved in cell migration, myogenesis, and connective tissue formation are critical to normal diaphragm formation, and these data provide a starting point for the search for other genes involved in the pathogenesis of CDH.
Authors:
Anne M Slavotinek
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Seminars in perinatology     Volume:  29     ISSN:  0146-0005     ISO Abbreviation:  Semin. Perinatol.     Publication Date:  2005 Apr 
Date Detail:
Created Date:  2005-07-29     Completed Date:  2005-10-20     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  7801132     Medline TA:  Semin Perinatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  77-85     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, University of California, San Francisco, CA 94143-0748, USA. slavotia@peds.ucsf.edu
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / genetics*
Animals
Disease Models, Animal
Female
Genetic Predisposition to Disease*
Hernia, Diaphragmatic / congenital,  genetics*,  physiopathology
Humans
Mutation
Pregnancy
Syndrome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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