Alveolar Capillary Dysplasia

被引：166

作者：

Bishop, Naomi B. ^{[1
]}

Stankiewicz, Pawel ^{[2
]}

Steinhorn, Robin H. ^{[3
,4
]}

机构：

[1] Weill Cornell Med Coll, New York, NY 10065 USA

[2] Baylor Coll Med, Dept Mol & Human Genet, Houston, TX 77030 USA

[3] Childrens Mem Hosp, Div Neonatol, Chicago, IL 60614 USA

[4] Northwestern Univ, Chicago, IL 60611 USA

来源：

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE | 2011年 / 184卷 / 02期

基金：

美国国家卫生研究院;

关键词：

interstitial lung disease; persistent pulmonary hypertension of the newborn; alveolar air-blood barrier; FOXF1 transcription factor gene; comparative microarray analysis; PERSISTENT PULMONARY-HYPERTENSION; EXTRACORPOREAL MEMBRANE-OXYGENATION; INHALED NITRIC-OXIDE; RESPIRATORY-DISTRESS; LUNG VESSELS; LIFE-SUPPORT; 2ND COURSE; INTRAVENOUS PROSTACYCLIN; SEPTAL-DEFECT; MISALIGNMENT;

D O I：

10.1164/rccm.201010-1697CI

中图分类号：

R4 [临床医学];

学科分类号：

1002 ; 100602 ;

摘要：

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.

引用

页码：172 / 179

页数：8

共 66 条

[1] LATE PRESENTATION OF MISALIGNMENT OF LUNG VESSELS WITH ALVEOLAR CAPILLARY DYSPLASIA [J].