| Congenital diaphragmatic hernia: a retinoid-signaling pathway disruption during lung development? | |
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MedLine Citation:
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PMID: 15981190 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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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:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Review |
Journal Detail:
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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:
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Created Date: 2005-08-15 Completed Date: 2005-12-01 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 101155107 Medline TA: Birth Defects Res A Clin Mol Teratol Country: United States |
Other Details:
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Languages: eng Pagination: 523-31 Citation Subset: IM |
Copyright Information:
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Copyright 2005 Wiley-Liss, Inc. |
Affiliation:
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Maternal Fetal Medicine Unit, Maternité Hôtel-Dieu, Centre Hospitalier Universitaire, Clermont-Ferrand, France. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Animals Diaphragm / abnormalities, embryology, pathology Female Fetal Diseases / drug therapy, metabolism*, pathology Hernia, Diaphragmatic / congenital*, drug therapy, metabolism*, mortality Humans Hypertension, Pulmonary / congenital, metabolism, mortality, pathology Lung / abnormalities, embryology*, metabolism Pregnancy Respiratory System Abnormalities / metabolism*, mortality, pathology Retinoids / metabolism*, therapeutic use Signal Transduction* |
| Chemical | |
Reg. No./Substance:
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0/Retinoids |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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