Histopathologic and Genetic Features of Alveolar Capillary Dysplasia with Atypical Late Presentation and Prolonged Survival. J Pediatr 2019 Jul;210:214-219.e2
Date
03/12/2019Pubmed ID
30853201Pubmed Central ID
PMC6592752DOI
10.1016/j.jpeds.2019.01.047Scopus ID
2-s2.0-85062429212 (requires institutional sign-in at Scopus site) 32 CitationsAbstract
Alveolar capillary dysplasia typically presents with neonatal pulmonary hypertension and early mortality. However, there is growing evidence for a subset of disease with atypical late onset and/or prolonged survival. Here, we present the variable clinical, genetic, and pathology findings of 4 such patients.
Author List
Edwards JJ, Murali C, Pogoriler J, Frank DB, Handler SS, Deardorff MA, Hopper RKAuthor
Stephanie S. Handler MD Associate Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
BiopsyFatal Outcome
Female
Forkhead Transcription Factors
Humans
Hypertension, Pulmonary
Infant
Lung
Lung Transplantation
Male
Mutation
Persistent Fetal Circulation Syndrome
Pulmonary Alveoli
Pulmonary Edema