Document Detail

Congenital diaphragmatic hernia: a retinoid-signaling pathway disruption during lung development?
MedLine Citation:
PMID:  15981190     Owner:  NLM     Status:  MEDLINE    
Congenital diaphragmatic hernia (CDH) usually occurs sporadically. The prognosis remains poor, with a 50% perinatal mortality rate. Most deaths result from hypoxemia due to lung hypoplasia and abnormal development of pulmonary vasculature that results in persistent pulmonary hypertension. Our current understanding of the pathogenesis of CDH is based on an assumption linking herniation of abdominal viscera into the thorax with compression of the developing lung. Pulmonary hypoplasia, however, can also result from reduced distension of the developing lung secondary to impaired fetal breathing movements. Moreover, a nitrofen-induced CDH model shows that lung hypoplasia precedes the diaphragmatic defect, leading to a "dual-hit hypothesis." Recent data reveal the role of a retinoid-signaling pathway disruption in the pathogenesis of CDH. We describe the clinical and epidemiological aspects of human CDH, the metabolic and molecular aspects of the retinoid-signaling pathway, and the implications of retinoids in the development of the diaphragm and the lung. Finally, we highlight the existing links between CDH and disruption of the retinoid-signaling pathway, which may suggest an eventual use of retinoids in the treatment of CDH.
Denis Gallot; Geoffroy Marceau; Karen Coste; Hélène Hadden; Elisabeth Robert-Gnansia; Hélène Laurichesse; Pierre J Déchelotte; André Labbé; Bernard Dastugue; Didier Lémery; Vincent Sapin
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Birth defects research. Part A, Clinical and molecular teratology     Volume:  73     ISSN:  1542-0752     ISO Abbreviation:  Birth Defects Res. Part A Clin. Mol. Teratol.     Publication Date:  2005 Aug 
Date Detail:
Created Date:  2005-08-15     Completed Date:  2005-12-01     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  101155107     Medline TA:  Birth Defects Res A Clin Mol Teratol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  523-31     Citation Subset:  IM    
Copyright Information:
Copyright 2005 Wiley-Liss, Inc.
Maternal Fetal Medicine Unit, Maternité Hôtel-Dieu, Centre Hospitalier Universitaire, Clermont-Ferrand, France.
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MeSH Terms
Diaphragm / abnormalities,  embryology,  pathology
Fetal Diseases / drug therapy,  metabolism*,  pathology
Hernia, Diaphragmatic / congenital*,  drug therapy,  metabolism*,  mortality
Hypertension, Pulmonary / congenital,  metabolism,  mortality,  pathology
Lung / abnormalities,  embryology*,  metabolism
Respiratory System Abnormalities / metabolism*,  mortality,  pathology
Retinoids / metabolism*,  therapeutic use
Signal Transduction*
Reg. No./Substance:

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