title: Misalignment of pulmonary veins with alveolar capillary dysplasia: affected siblings and variable phenotypic expression.
abstract: Misalignment of pulmonary veins with alveolar capillary dysplasia is recognized as a rare cause of persistent pulmonary hypertension of the neonate. Until now, misalignment of pulmonary veins was thought to be a random occurrence, but its appearance in siblings at our institution suggests that there may be a familial predisposition. There have been reports of variable expression and variable severity in this disease; our report describes this variability in family members.
authors.affiliation: Division of Neonatology, University of Pennsylvania School of Medicine, Philadelphia.
authors.forename: S
authors.initials: S
authors.lastname: Boggs
authors.forename: M C
authors.initials: MC
authors.lastname: Harris
authors.forename: D J
authors.initials: DJ
authors.lastname: Hoffman
authors.forename: R
authors.initials: R
authors.lastname: Goel
authors.forename: D
authors.initials: D
authors.lastname: McDonald-McGinn
authors.forename: C
authors.initials: C
authors.lastname: Langston
authors.forename: E
authors.initials: E
authors.lastname: Zackai
authors.forename: E
authors.initials: E
authors.lastname: Ruchelli
mesh_terms: D000015:Abnormalities, Multiple / Q000235:genetics* / Q000473:pathology; D002196:Capillaries / Q000002:abnormalities; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000098:blood supply / Q000473:pathology; D008297:Male; D010547:Persistent Fetal Circulation Syndrome / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D010641:Phenotype; D011650:Pulmonary Alveoli / Q000098:blood supply* / Q000473:pathology; D011667:Pulmonary Veins / Q000002:abnormalities*