van der Baan S, Veerman A J, Wulffraat N, Bezemer P D, Feenstra L
Acta Otolaryngol. 1986 Sep-Oct;102(3-4):274-81. doi: 10.3109/00016488609108677.
In primary ciliary dyskinesia (PCD) the cilia of the respiratory tract are immotile or they show an incoordinate, abnormal beating pattern. Consequently, mucociliary clearance is lacking. Most patients with PCD have ultrastructural abnormalities in their cilia. In some patients, however, the ciliary ultrastructure is completely normal. In this study a semi-quantitative scoring method is described for studying ciliary motility in biopsies of nasal mucosa. For every cell the frequency, coordination and amplitude of the ciliary beat are scored. Thereafter, a total score of ciliary activity per cell can be calculated. This method is simple and has proved to be reproducible. By using this scoring method the presence of a PCD can be established with certainty, whether the ciliary ultrastructure is abnormal or not. In patients with a normal ciliary ultrastructure the cilia have a rather high beating frequency (vibration). By scoring the ciliary motility in mucosal biopsies, the time-consuming electronmicroscopic investigation of the cilia is no longer necessary in most patients.
在原发性纤毛运动障碍（PCD）中，呼吸道的纤毛无法运动，或者表现出不协调、异常的摆动模式。因此，黏液纤毛清除功能缺失。大多数PCD患者的纤毛存在超微结构异常。然而，在一些患者中，纤毛的超微结构完全正常。在本研究中，描述了一种半定量评分方法，用于研究鼻黏膜活检组织中的纤毛运动。对每个细胞的纤毛摆动频率、协调性和幅度进行评分。此后，可以计算每个细胞的纤毛活动总分。该方法简单且已被证明具有可重复性。通过使用这种评分方法，无论纤毛超微结构是否异常，都可以确定是否存在PCD。在纤毛超微结构正常的患者中，纤毛具有相当高的摆动频率（振动）。通过对黏膜活检组织中的纤毛运动进行评分，在大多数患者中不再需要耗时的纤毛电子显微镜检查。
