Severe bronchiectasis in patients with "cystlike" structures within the ciliary shafts

被引:21
作者
Tsang, KWT
Tipoe, G
Sun, J
Ho, JCM
Lam, B
Zheng, L
Ooi, GC
Ip, M
Lam, WK
机构
[1] Univ Hong Kong, Queen Mary Hosp, Univ Dept Med, Hong Kong, Hong Kong, Peoples R China
[2] Univ Hong Kong, Queen Mary Hosp, Univ Dept Anat, Hong Kong, Hong Kong, Peoples R China
[3] Univ Hong Kong, Queen Mary Hosp, Univ Dept Diagnost Radiol, Hong Kong, Hong Kong, Peoples R China
关键词
D O I
10.1164/ajrccm.161.4.9904088
中图分类号
R4 [临床医学];
学科分类号
1002 ; 100602 ;
摘要
Cilia are minute hairlike structures which sweep the respiratory tract clean by continuous beating. Ciliary dysfunction can occur which leads to development of bronchiectasis. We describe the clinical and investigation profiles of four patients who had early-onset severe and otherwise idiopathic bronchiectasis associated with a previously undescribed ciliary defect in which numerous cystlike structures are present within the ciliary shafts. The patients had otherwise normal ciliary microtubular ultrastructure and central microtubular orientation. Our newly described ciliary abnormality appears to be a primary defect and more systematic studies should be performed to evaluate this further.
引用
收藏
页码:1300 / 1305
页数:6
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