Alveolar capillary dysplasia: lung biopsy diagnosis, nitric oxide responsiveness, and bronchial generation count.
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.
Sirkin
W
W
Department of Pathology, Hospital for Sick Children, Toronto, Ontario, Canada.
O'Hare
B P
BP
Cox
P N
PN
Perrin
D
D
Cutz
E
E
Silver
M M
MM
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*
D009569:Nitric Oxide