Fabian Doktor, Lina Antounians, Martin Lacher, Augusto Zani
The Hospital for Sick Children and University of Toronto. University of Leipzig.
Canada and Germany
Seminars in Pediatric Surgery
Semin Pediatr Surg 2022; 31:
DOI: 10.1016/j.sempedsurg.2022.151228
Abstract
Congenital lung malformations comprise a diverse group of anomalies including congenital pulmonary airway malformation (CPAM, previously known as congenital cystic adenomatoid malformation or CCAM), bronchopulmonary sequestration (BPS), congenital lobar emphysema (CLE), bronchogenic cysts, and hybrid lesions. Little is known about the signaling pathways that underlie the pathophysiology of these lesions and the processes that may promote their malignant transformation. In the last decade, the use of transgenic/knockout animal models and the implementation of next generation sequencing on surgical lung specimens have increased our knowledge on the pathophysiology of these lesions. Herein, we provide an overview of normal lung development in humans and rodents, and we discuss the current state of knowledge on the pathophysiology and molecular pathways that are altered in each congenital lung malformation.
Category
Review Articles Concerning Pulmonary Vascular Disease
Genetic Factors Associated with Pulmonary Vascular Disease
Class III. Pulmonary Hypertension Associated with Lung Hypoplasia
Abnormal Systemic to Pulmonary Arterial Collaterals or Connections
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No