title: Alveolar capillary dysplasia: lung biopsy diagnosis, nitric oxide responsiveness, and bronchial generation count.
abstract: Alveolar capillary dysplasia, a rare cause of neonatal pulmonary hypertension characterized by a developmental abnormality in the pulmonary vasculature, was diagnosed by lung biopsy in a male newborn maintained on nitric oxide therapy for 18 days. Autopsy confirmed the pulmonary vascular defect and demonstrated deficient airspace formation. In addition, a bronchial generation count was low, suggesting that the abnormal lung vascular development in this condition represents a special form of pulmonary hypoplasia that starts in early fetal life.
authors.affiliation: Department of Pathology, Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: W
authors.initials: W
authors.lastname: Sirkin
authors.forename: B P
authors.initials: BP
authors.lastname: O'Hare
authors.forename: P N
authors.initials: PN
authors.lastname: Cox
authors.forename: D
authors.initials: D
authors.lastname: Perrin
authors.forename: E
authors.initials: E
authors.lastname: Cutz
authors.forename: M M
authors.initials: MM
authors.lastname: Silver
mesh_terms: D001706:Biopsy; D001980:Bronchi / Q000473:pathology; D002102:Cadaver; D002196:Capillaries / Q000002:abnormalities; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities / Q000473:pathology*; D008297:Male; D009569:Nitric Oxide / Q000627:therapeutic use*; D011650:Pulmonary Alveoli / Q000098:blood supply*
chemical_list: D009569:Nitric Oxide