Olivier Danhaive, Csaba Galambos, Satyan Lakshminrusimha, Steven H. Abman
Saint-Luc University Hospital and University of California San Francisco. University of Colorado Anschutz School of Medicine. University of California, UC Davis Children’s Hospital.
Belgium and United States
Clinics in Perinatology
Clin Perinatol 2024; 51: 217-235
DOI: 10.1016/j.clp.2023.12.001
Abstract
Diverse genetic developmental lung diseases can present in the neonatal period with hypoxemic respiratory failure, often associated with with pulmonary hypertension. Intractable hypoxemia and lack of sustained response to medical management should increase the suspicion of a developmental lung disorder. Genetic diagnosis and lung biopsy are helpful in establishing the diagnosis. Early diagnosis can result in optimizing management and redirecting care if needed. This article reviews normal lung development, various developmental lung disorders that can result from genetic abnormalities at each stage of lung development, their clinical presentation, management, prognosis, and differential diagnoses.
Category
Class III. Pulmonary Hypertension Associated with Developmental Diseases of the Lung
Pulmonary Vascular Pathology
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No