Csaba Galambos, J. Wells Logan, Pawel Stankiewicz, Przemyslaw Szafranski, Carol Zalles, Jose Gonzales, Sfurti Nath, Shalinkumar Patel, Steven H. Abman
University of Colorado Anschutz School of Medicine. Wolfson Children’s Hospital and the University of Florida College of Medicine. Baylor College of Medicine.
United States
Pediatric Pulmonology
Pediatr Pulmonol 2023;
DOI: 10.1002/ppul.26571
Abstract
We report the case of a preterm infant who died at 10 months of age with severe bronchopulmonary dysplasia (sBPD) with refractory pulmonary hypertension and respiratory failure who had striking histologic features compatible with the diagnosis of alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) but without genetic confirmation of the diagnosis. We further demonstrate dramatic reductions in lung FOXF1 and TMEM100 content in sBPD, suggesting common mechanistic links between ACDMPV and sBPD with impaired FOXF1 signaling.
Category
Class III. Pulmonary Hypertension Associated with Lung Disease
Class III. Pulmonary Hypertension Associated with Developmental Diseases of the Lung
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