Infants with Atypical Presentations of Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins Who Underwent Bilateral Lung Transplantation

• Published in: The Journal of pediatrics Vol. 194; pp. 158 - 164.e1
• Main Authors: Towe, Christopher T., White, Frances V., Grady, R. Mark, Sweet, Stuart C., Eghtesady, Pirooz, Wegner, Daniel J., Sen, Partha, Szafranski, Przemyslaw, Stankiewicz, Pawel, Hamvas, Aaron, Cole, F. Sessions, Wambach, Jennifer A.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.03.2018
• Subjects: diffuse developmental lung disorder; Female; Forkhead Transcription Factors - genetics; Humans; Infant; Infant, Newborn; Lung - pathology; Lung Transplantation - methods; Male; Mutation; Original; Persistent Fetal Circulation Syndrome - complications; Persistent Fetal Circulation Syndrome - diagnosis; Persistent Fetal Circulation Syndrome - surgery; Pulmonary Alveoli - abnormalities; Pulmonary Alveoli - surgery; Pulmonary Veins - abnormalities; Survival Rate; DOL; ILD; CT; FVC; RV; ACDMPV; CNV; FEV1; TLC; diffuse developmental lung disorder; BVB

To describe disease course, histopathology, and outcomes for infants with atypical presentations of alveolar capillary dysplasia with misalignment of the pulmonary veins (ACDMPV) who underwent bilateral lung transplantation. We reviewed clinical history, diagnostic studies, explant histology, geneti...