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原发性纤毛运动障碍、支气管扩张症患者及正常受试者呼吸道纤毛的方向

Orientation of respiratory tract cilia in patients with primary ciliary dyskinesia, bronchiectasis, and in normal subjects.

作者信息

De Iongh R, Rutland J

机构信息

Respiratory Unit, Concord Hospital, Sydney, New South Wales, Australia.

出版信息

J Clin Pathol. 1989 Jun;42(6):613-9. doi: 10.1136/jcp.42.6.613.

DOI:10.1136/jcp.42.6.613
PMID:2738166
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1141989/
Abstract

A reproducible technique, utilising a graphics tablet and a personal computer for measuring ciliary orientation from electron micrographs of ciliated epithelium, was assessed. Ciliary deviation was measured in 47 normal subjects (mean ciliary deviation +/- 1 SD was 14.6 (3.3 degrees)), in eight patients with bronchiectasis and normal ciliary function (15.1 (6.5 degrees], and in seven patients with primary ciliary dyskinesia (38.7 (7.8 degrees); significantly greater than the first two groups). Measurements of ciliary deviation at the tip, base, and basal feet showed very little variation along the ciliary shaft in all three groups, suggesting that valid measurements of ciliary deviation can be made at any level of the cilium. Mean ciliary deviation in normal subjects was always less than 30 degrees; all patients with a mean ciliary deviation of greater than 30 degrees had recurrent respiratory tract disease. Four of seven patients with primary ciliary dyskinesia had ciliary disorientation; in one this was the only defect. Measurements of inter- and intraobserver variability using this method showed a maximum difference between observations of 4.1 degrees. It is suggested that ciliary orientation should be measured in patients suspected of having defective ciliary function or structure, or both.

摘要

评估了一种利用数位绘图板和个人计算机从纤毛上皮电子显微照片测量纤毛方向的可重复技术。对47名正常受试者(平均纤毛偏差±1标准差为14.6(3.3度))、8名支气管扩张且纤毛功能正常的患者(15.1(6.5度))以及7名原发性纤毛运动障碍患者(38.7(7.8度);显著大于前两组)进行了纤毛偏差测量。在所有三组中,对纤毛尖端、基部和基脚处的纤毛偏差测量显示,沿纤毛轴的变化非常小,这表明可以在纤毛的任何水平进行有效的纤毛偏差测量。正常受试者的平均纤毛偏差始终小于30度;所有平均纤毛偏差大于30度的患者都有反复呼吸道疾病。7名原发性纤毛运动障碍患者中有4名存在纤毛定向障碍;其中1名患者这是唯一的缺陷。使用该方法对观察者间和观察者内变异性的测量显示,观察结果之间的最大差异为4.1度。建议对怀疑有纤毛功能或结构缺陷或两者皆有的患者测量纤毛方向。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6d5b/1141989/04015ae619f7/jclinpath00350-0055-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6d5b/1141989/d91e1342eddc/jclinpath00350-0054-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6d5b/1141989/04015ae619f7/jclinpath00350-0055-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6d5b/1141989/d91e1342eddc/jclinpath00350-0054-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6d5b/1141989/04015ae619f7/jclinpath00350-0055-a.jpg

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