title: Pathomechanisms of Congenital Cystic Lung Diseases: Focus on Congenital Cystic Adenomatoid Malformation and Pleuropulmonary Blastoma.
abstract: It is well established that a number of birth defects are associated with improper formation of the respiratory tract. Important progress has been made in the identification of components of the regulatory networks controlling lung morphogenesis. They comprise a variety of soluble factors, receptors, transcription factors, and miRNAs. However, the underlying molecular mechanisms remain unsolved and fundamental questions, such as those related to lung branching are still unanswered. Congenital cystic lung diseases consist of a heterogeneous group of rare lung diseases mainly detected prenatally and characterized by airway dilatation. Despite their apparent phenotypic heterogeneity, these malformations are proposed to be related to a common malformation sequence occurring during lung branching morphogenesis.
authors.affiliation: Institut Universitaire de Cardiologie et de Pneumologie de Québec, Université Laval, Québec, QC, Canada, G1 V 4G5.
authors.forename: Olivier
authors.initials: O
authors.lastname: Boucherat
authors.affiliation: Centre de recherche sur le cancer de l'Université Laval, CRCHUQ, L'Hôtel-Dieu de Québec, QC, Canada; Département de Biologie Moléculaire, Biochimie Médicale et Pathologie, Université Laval, Québec, Canada.
authors.forename: Lucie
authors.initials: L
authors.lastname: Jeannotte
authors.affiliation: AP-HP, Hôpital Necker-Enfants Malades, Service de Pneumologie Pédiatrique, Centre de Référence pour les Maladies Respiratoires Rares de l'Enfant, Paris, France; INSERM, U955, IMRB, Equipe 04, Créteil, France; Université Paris-Descartes, Paris, France.
authors.forename: Alice
authors.initials: A
authors.lastname: Hadchouel
authors.affiliation: AP-HP, Hôpital Necker-Enfants Malades, Service de Pneumologie Pédiatrique, Centre de Référence pour les Maladies Respiratoires Rares de l'Enfant, Paris, France; INSERM, U955, IMRB, Equipe 04, Créteil, France; Université Paris-Descartes, Paris, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Delacourt
authors.affiliation: AP-HP, Hôpital Antoine-Béclère, Université Paris-Sud, Service de Gynécologie Obstétrique et Médecine de la Reproduction, 92141 Clamart, France; INSERM, UMR 986, Université Paris-Sud, Bicêtre, France.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Benachi
mesh_terms: D000818:Animals; D015615:Cystic Adenomatoid Malformation of Lung, Congenital / Q000235:genetics* / Q000473:pathology*; D004195:Disease Models, Animal; D006801:Humans; D018202:Pulmonary Blastoma / Q000235:genetics* / Q000473:pathology*
keywords: Airway dilatation; Congenital cystic adenomatoid malformation; Lung branching morphogenesis; Pleuropulmonary blastoma.