Alveolar Capillary Dysplasia
Alveolar Capillary Dysplasia
复制标题
DOI：
10.1164/rccm.201010-1697ci
复制
发表时间：
2011-07-15
影响因子：
24.7
通讯作者：
Steinhorn, Robin H.
中科院分区：
文献类型：
--
作者：
Bishop, Naomi B.;Stankiewicz, Pawel;Steinhorn, Robin H.
Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare, fatal developmental lung disorder of neonates and infants. This review aims to address recent findings in the etiology and genetics of ACD/MPV and to raise awareness of this poorly known disease, which may also present as milder, unclassified forms. Successively discussed are what is known about the epidemiology, pathogenesis, pathophysiology, diagnostic indicators and approaches, genetic testing, treatment, and cases of delayed onset. The review concludes with suggestions for future directions to answer the many unknowns about this disorder.
