Lung development and implications for hypoplasia found in congenital diaphragmatic hernia.
Congenital diaphragmatic hernia (CDH) is associated with various degrees of pulmonary hypoplasia and severe persistent pulmonary hypertension in the newborn. These conditions have significant implications for the outcome for the patient. Defects in early lung development are likely to be central to the generation of hypoplasia. A number of mouse models with defects in pathways that are central to lung development were found to have CDH. Understanding all aspects of early lung development will provide fresh insight into the pathogenesis of CDH and its associated conditions.
Kinane
T Bernard
TB
Pediatric Pulmonary, Massachusetts General Hospital for Children, Boston, MA 02114, USA. tkinane@partners.org
D000818:Animals; D004195:Disease Models, Animal; D051526:Fibroblast Growth Factor 10 / Q000235:genetics; D006548:Hernia, Diaphragmatic / Q000150:complications / Q000196:embryology; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000196:embryology; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities / Q000196:embryology*; D051379:Mice; D018345:Mice, Knockout; D009024:Morphogenesis; D015619:Respiratory System Abnormalities / Q000196:embryology* / Q000235:genetics
C496464:Fgf10 protein, mouse; D051526:Fibroblast Growth Factor 10