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原发性纤毛运动障碍者,多缺乏外及/或内动力蛋白臂(dynein arm)。用电镜观察纤毛超微结构,要计数每根纤毛的外和内侧动力蛋白臂才能确诊,既费时,又昂贵。另外有些原发性纤毛运动障碍者纤毛超微结构可以正常。为此,作者改用光镜半定最分析方法,检测87例病人,包括(1)原发性纤毛运动障碍23人,其中Kartagener综合征11例(10例并有动力蛋白臀缺失,1例纤毛超微结构正常),12例呼吸道感染并动力蛋白臂缺失,但无内脏转位;(2)原因不明慢性呼吸道感染64人,既无动力蛋白臀缺损,也无内脏转位。这些病人的呼吸道感染不是变态反应、囊性纤维病及免疫缺陷所引起。这种半定量分析方法系选择患者呼吸道病变稳定期进行,检查前两天停止鼻腔滴药,在不用局部麻醉的情况下用杯状钳
Patients with primary ciliary dyskinesia lack extra and / or dynein arms. Using electron microscopy to visualize the ultrastructure of cilia, it is both time-consuming and expensive to count the outer and inner motor protein arms of each cilia. In addition, some primary ciliary dyskinesis cilia ultrastructure can be normal. To this end, the authors use light microscopy semi-quantitative analysis of the most 87 patients, including (1) 23 cases of primary ciliary dyskinesia, of which 11 cases of Kartagener syndrome (10 cases with kinetic protein buttock loss, 1 case Ciliary ultrastructure normal), 12 cases of respiratory tract infection and motor protein arm missing, but no visceral transposition; (2) unexplained chronic respiratory tract infection in 64 people, both without kinetic protein hip defect, no visceral transposition. Respiratory infections in these patients are not caused by allergies, cystic fibrosis and immunodeficiency. This method of semi-quantitative analysis of patients with respiratory disease was selected stable phase, nasal drops stopped two days before the test, with no local anesthesia with cup-shaped pliers