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Histopathologic and Genetic Features of Alveolar Capillary Dysplasia with Atypical Late Presentation and Prolonged Survival. J Pediatr 2019 Jul;210:214-219.e2

Date

03/12/2019

Pubmed ID

30853201

Pubmed Central ID

PMC6592752

DOI

10.1016/j.jpeds.2019.01.047

Scopus ID

2-s2.0-85062429212 (requires institutional sign-in at Scopus site)   32 Citations

Abstract

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 RK

Author

Stephanie S. Handler MD Associate Professor in the Pediatrics department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Biopsy
Fatal Outcome
Female
Forkhead Transcription Factors
Humans
Hypertension, Pulmonary
Infant
Lung
Lung Transplantation
Male
Mutation
Persistent Fetal Circulation Syndrome
Pulmonary Alveoli
Pulmonary Edema