Document Detail


Etiology of congenital diaphragmatic hernia: the retinoid hypothesis.
MedLine Citation:
PMID:  12621107     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Congenital diaphragmatic hernia (CDH) is a major life-threatening cause of respiratory failure in the newborn. Although significant efforts have been undertaken to unravel the pathophysiology of CDH, our current understanding of the etiology remains spare. Here we outline recent evidence suggesting that abnormalities linked with the retinoid signaling pathway early in gestation may contribute to the etiology of CDH. These studies include 1) the effect of altering the retinoid system in vitamin A deficient and transgenic animals; 2) disruption of the retinoid system in teratogen-induced CDH in rodents, 3) the effect of co-administration of retinoids in nitrofen-induced CDH on lung and diaphragm development, and 4) clinical evidence suggesting decreased markers of vitamin A status in human CDH. Given the substantial mortality and morbidity associated with this serious developmental anomaly, advancements in this area will be critical. We feel that there is now sufficient circumstantial and direct experimental evidence to warrant further testing of the retinoid-CDH etiology hypothesis, including examination of retinoid-regulated target genes that could be candidates for involvement in CDH.
Authors:
John J Greer; Randal P Babiuk; Bernard Thebaud
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review     Date:  2003-03-05
Journal Detail:
Title:  Pediatric research     Volume:  53     ISSN:  0031-3998     ISO Abbreviation:  Pediatr. Res.     Publication Date:  2003 May 
Date Detail:
Created Date:  2003-04-18     Completed Date:  2003-12-04     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0100714     Medline TA:  Pediatr Res     Country:  United States    
Other Details:
Languages:  eng     Pagination:  726-30     Citation Subset:  IM    
Affiliation:
Department of Physiology, Perinatal Research Centre, Vascular Biology Research Group, University of Alberta, Edmonton, Canada. john.greer@ualberta.ca
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MeSH Terms
Descriptor/Qualifier:
Animals
Hernia, Diaphragmatic / congenital,  etiology*,  physiopathology*
Humans
Infant, Newborn
Signal Transduction / physiology*
Tretinoin / physiology*
Chemical
Reg. No./Substance:
302-79-4/Tretinoin

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


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