Bertrand B, Collet S, Eloy P, Rombaux P
ORL and HNS Department, Cliniques Universitaires de Mont-Godinne, Yvoir, Belgium.
Acta Otorhinolaryngol Belg. 2000;54(3):309-16.
The system of mucociliary clearance has the important task to remove from the airways inhaled substances and locally formed secretions. Inborn disorders of the mucociliary transport are due to ciliary dysfunction (Primary Ciliary Dyskinesia) (PCD) or of increased viscosity of the bronchial secretions (Cystic Fibrosis). To differentiate PCD from the ultrastructural abnormalities found during or after injuries such as respiratory infections, the name of Secondary--or acquired--Ciliary Dyskinesia (SCD) was created. In controls, less than 4% of the cilia may show ultrastructural abnormalities. The most frequent of these are the compound cilia and the peripheral microtubular abnormalities. Compound cilia often appear after infection and therefore are thought to arise secondarily. Secondary ultrastructural abnormalities of cilia include also blebs of the axoneme membrane, ciliary disorientation, and absence of axoneme membrane. No increase in ultrastructural ciliary abnormalities has been found in a variety of respiratory disorders: smoking, asthma and allergic rhinitis, chronic rhinitis and sinusitis, chronic bronchitis, cystic fibrosis, and lung carcinoma. But severe modifications of the respiratory epithelium can be seen. Important for the secondary ciliary disorders is their local and reversible character. To distinct from ultrastructural images between primary and secondary ciliary dyskinesia is often uneasy because some of the findings in secondary ciliary dyskinesia obviously mimic those dedicated to primary ciliary dyskinesia.
黏液纤毛清除系统肩负着从气道清除吸入物质和局部形成的分泌物这一重要任务。黏液纤毛运输的先天性疾病是由于纤毛功能障碍(原发性纤毛运动障碍)(PCD)或支气管分泌物黏稠度增加(囊性纤维化)所致。为了将PCD与在诸如呼吸道感染等损伤期间或之后发现的超微结构异常区分开来,人们创造了继发性或获得性纤毛运动障碍(SCD)这一名称。在对照组中,不到4%的纤毛可能会出现超微结构异常。其中最常见的是复合纤毛和外周微管异常。复合纤毛常在感染后出现,因此被认为是继发产生的。纤毛的继发性超微结构异常还包括轴丝膜的泡状结构、纤毛定向紊乱以及轴丝膜缺失。在多种呼吸系统疾病中均未发现纤毛超微结构异常增加,这些疾病包括吸烟、哮喘和过敏性鼻炎、慢性鼻炎和鼻窦炎、慢性支气管炎、囊性纤维化以及肺癌。但可见呼吸道上皮有严重改变。继发性纤毛疾病的重要之处在于其局部性和可逆性。区分原发性和继发性纤毛运动障碍的超微结构图像往往并不容易,因为继发性纤毛运动障碍中的一些发现明显类似于原发性纤毛运动障碍的表现。