Pedersen M, Mygind N
Br J Dis Chest. 1980 Jul;74(3):239-44. doi: 10.1016/0007-0971(80)90049-2.
Using a microphoto-oscillographic technique the authors studied ciliary beating of nasal epithelial cells from 11 patients with the 'immotile cilia syndrome' (Kartagener's syndrome) and from normal controls. Ciliary motility was found in all patients, except one. Although the patients had a significantly reduced number of cells with motile cilia, one-third of all ciliated cells showed motility. The beating frequency was normal, but the motility pattern was highly abnormal, with a predominance of asynchronous motility in the patients. These results suggest that the lack of dynein arms in cilia in these patients only is partial and that direct observation of ciliated cells, obtained by gentle scraping on the nasal mucosa, may be a suitable screening test for early diagnosis of this syndrome.
作者采用显微摄影振荡技术,研究了11例“不动纤毛综合征”(卡塔格内综合征)患者及正常对照者鼻上皮细胞的纤毛摆动情况。除1例患者外,所有患者均发现有纤毛运动。尽管患者中具有运动纤毛的细胞数量显著减少,但所有纤毛细胞中有三分之一显示出运动能力。纤毛摆动频率正常,但运动模式高度异常,患者中异步运动占主导。这些结果表明,这些患者纤毛中动力蛋白臂的缺失仅是部分性的,并且通过轻轻刮取鼻黏膜获得的纤毛细胞直接观察,可能是该综合征早期诊断的合适筛查试验。