de Iongh R, Ing A, Rutland J
Respiratory Unit, Concord Hospital, Concord, New South Wales, Australia.
Thorax. 1992 Mar;47(3):184-7. doi: 10.1136/thx.47.3.184.
Mucociliary clearance is impaired in patients with Young's syndrome (obstructive azoospermia with recurrent sinobronchial disease), cystic fibrosis, and primary ciliary dyskinesia. No defect of cilia or mucus has been detected in Young's syndrome.
Ciliary function and ultrastructure, including ciliary orientation, were studied quantitatively in 20 patients with Young's syndrome and 20 normal subjects to determine the incidences of ciliary defects. Nasal ciliated epithelium was obtained from each subject and used for measurement of ciliary beat frequency and ultrastructural analyses. Ciliary orientation was determined by measuring ciliary deviation in electron micrographs; ciliary deviation is a measure of the relative orientation of cilia in relation to each other in which high values indicate ciliary disorientation.
Ciliary beat frequency and the incidence of microtubular defects and numbers of dynein arms did not differ between patients with Young's syndrome and control subjects. In patients with Young's syndrome basal ciliary deviation (16.0 degrees) was similar to that in control subjects (14.1 degrees), but at the ciliary tip ciliary deviation (21.9 degrees) was greater than in healthy subjects (14.5 degrees).
The relative disorientation of the distal ciliary axoneme in patients with Young's syndrome compared with normal subjects may be due to a structural defect but is more likely to be a consequence of abnormal mucus.
杨氏综合征（梗阻性无精子症伴复发性鼻窦支气管疾病）、囊性纤维化和原发性纤毛运动障碍患者的黏液纤毛清除功能受损。在杨氏综合征中未检测到纤毛或黏液缺陷。
对20例杨氏综合征患者和20名正常受试者的纤毛功能和超微结构，包括纤毛方向进行定量研究，以确定纤毛缺陷的发生率。从每个受试者获取鼻纤毛上皮，用于测量纤毛摆动频率和超微结构分析。通过测量电子显微镜照片中的纤毛偏差来确定纤毛方向；纤毛偏差是纤毛彼此相对方向的一种度量，高值表明纤毛方向紊乱。
杨氏综合征患者与对照受试者之间的纤毛摆动频率、微管缺陷发生率和动力蛋白臂数量没有差异。在杨氏综合征患者中，基底纤毛偏差（16.0度）与对照受试者（14.1度）相似，但在纤毛尖端，纤毛偏差（21.9度）大于健康受试者（14.5度）。
与正常受试者相比，杨氏综合征患者远端纤毛轴丝的相对方向紊乱可能是由于结构缺陷，但更可能是异常黏液的结果。
