Alveolar capillary dysplasia [Supplementary Concept]
A disorder characterized histologically by failure of formation and ingrowth of alveolar CAPILLARIES that then do not make contact with alveolar EPITHELIUM, medial muscular thickening of small pulmonary ARTERIOLES with muscularization of the intraacinar arterioles, thickened alveolar walls, and anomalously situated PULMONARY VEINS running alongside pulmonary arterioles and sharing the same ADVENTITIAL TISSUE sheath. Mutations in the FOXF1 gene have been identified in sporadic cases. OMIM: 265380
Date introduced: August 25, 2010
MeSH Unique ID: C536590
Heading Mapped to:
Entry Terms:
- Congenital alveolar capillary dysplasia