马凡综合征患者中支气管扩张的患病率。

The prevalence of bronchiectasis in patients with Marfan syndrome.

作者信息

Hwang H S, Yi C A, Yoo H, Yang J H, Kim D-K, Koh W-J

机构信息

Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Division of Pulmonary and Critical Care Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

出版信息

Int J Tuberc Lung Dis. 2014 Aug;18(8):995-7. doi: 10.5588/ijtld.13.0763.

DOI:10.5588/ijtld.13.0763

PMID:25199019

Abstract

An association has been suggested between Marfan syndrome (MFS) and the nodular bronchiectatic form of lung disease caused by non-tuberculous mycobacteria (NTM). We evaluated the prevalence of bronchiectasis in 79 adult patients with MFS using computed tomography (CT) imaging. Airway dilation indicative of bronchiectasis (22/79, 28%) and bronchioloectasis (10/79, 13%) were relatively common, although the extent of dilation was not severe and was frequently confined to a single lobe. However, bronchiolitis was evident in only three patients (4%), and no patient was diagnosed with NTM lung disease.

摘要

有人提出马凡综合征（MFS）与非结核分枝杆菌（NTM）引起的结节性支气管扩张型肺病之间存在关联。我们使用计算机断层扫描（CT）成像评估了79例成年MFS患者的支气管扩张患病率。提示支气管扩张（22/79，28%）和细支气管扩张（10/79，13%）的气道扩张相对常见，尽管扩张程度不严重且常局限于单个肺叶。然而，仅3例患者（4%）有细支气管炎，且无患者被诊断为NTM肺病。