期刊
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
卷 184, 期 2, 页码 172-179出版社
AMER THORACIC SOC
DOI: 10.1164/rccm.201010-1697CI
关键词
interstitial lung disease; persistent pulmonary hypertension of the newborn; alveolar air-blood barrier; FOXF1 transcription factor gene; comparative microarray analysis
资金
- U.S. Centers for Disease Control and Prevention
- 3 Angels Memorial Fund for ACD Research [3 U50DD000478-01W1]
- National Institutes of Health [HL101975-01, HL 54705-14, HL102235-01]
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.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据