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Paediatr Respir Rev · Jun 2016
ReviewPathomechanisms of Congenital Cystic Lung Diseases: Focus on Congenital Cystic Adenomatoid Malformation and Pleuropulmonary Blastoma.
- Olivier Boucherat, Lucie Jeannotte, Alice Hadchouel, Christophe Delacourt, and Alexandra Benachi.
- Institut Universitaire de Cardiologie et de Pneumologie de Québec, Université Laval, Québec, QC, Canada, G1 V 4G5.
- Paediatr Respir Rev. 2016 Jun 1; 19: 62-8.
AbstractIt 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. Copyright © 2016 Elsevier Ltd. All rights reserved.
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