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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\u2010induced CDH model shows that lung hypoplasia precedes the diaphragmatic defect, leading to a \u201cdual\u2010hit hypothesis.\u201d Recent data reveal the role of a retinoid\u2010signaling 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\u2010signaling 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\u2010signaling pathway, which may suggest an eventual use of retinoids in the treatment of CDH. Birth Defects Research (Part A), 2005. \u00a9 2005 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/bdra.20151","type":"journal-article","created":{"date-parts":[[2005,6,24]],"date-time":"2005-06-24T16:19:33Z","timestamp":1119629973000},"page":"523-531","source":"Crossref","is-referenced-by-count":54,"title":["Congenital diaphragmatic hernia: A retinoid\u2010signaling pathway disruption during lung development?"],"prefix":"10.1002","volume":"73","author":[{"given":"Denis","family":"Gallot","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Geoffroy","family":"Marceau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen","family":"Coste","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H\u00e9l\u00e8ne","family":"Hadden","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisabeth","family":"Robert\u2010Gnansia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H\u00e9l\u00e8ne","family":"Laurichesse","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre J","family":"D\u00e9chelotte","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andr\u00e9","family":"Labb\u00e9","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernard","family":"Dastugue","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Didier","family":"L\u00e9mery","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Sapin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2005,6,24]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-9861(19970616)382:4<459::AID-CNE3>3.0.CO;2-1"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1152\/jappl.1997.83.2.338"},{"key":"e_1_2_9_4_1","first-page":"888","article-title":"Incidence of congenital diaphragmatic hernia in the young of rats bred on a diet deficient in vitamin","volume":"62","author":"Anderson DH","year":"1941","journal-title":"Am J Dis Child"},{"key":"e_1_2_9_5_1","first-page":"163","article-title":"Effect of diet during pregnancy upon the incidence of congenital hereditary diaphragmatic hernia in the rat","volume":"25","author":"Anderson DH","year":"1949","journal-title":"Am J Pathol"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00257.2002"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00403.2003"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.gene.2003.12.005"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0925-4773(97)00167-6"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.117.5_suppl_1.235S"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0098-2997(03)00039-6"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.20464"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200204-304CC"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1096\/fasebj.10.9.8801176"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0925-4773(03)00048-0"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1159\/000068932"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.1099"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1096\/fasebj.10.9.8801181"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3468(03)00494-9"},{"key":"e_1_2_9_20_1","first-page":"12","article-title":"Hypoplastic lungs in fetal lambs with surgically produced congenital diaphragmatic hernia","volume":"62","author":"De Lorimier A","year":"1967","journal-title":"Surgery"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00140.2003"},{"key":"e_1_2_9_22_1","doi-asserted-by":"crossref","first-page":"1133","DOI":"10.1242\/dev.110.4.1133","article-title":"Retinoic acid receptors and cellular retnoid binding proteins. 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