Carson J L, Collier A M, Fernald G W, Hu S C
Department of Pediatrics and Cell Biology and Anatomy, University of North Carolina at Chapel Hill.
Ultrastruct Pathol. 1994 May-Jun;18(3):327-32. doi: 10.3109/01913129409023201.
A critical relationship exists between ordered ciliary ultrastructure and optimal mucociliary clearance in the respiratory airways. Structurally defective cilia derived from heritable syndromes or from epithelial cell injury may promote or exacerbate chronic disease processes. A lesion of airway epithelial cilia characterized by microtubular discontinuities and previously associated with primary ciliary dyskinesia (PCD) has been documented in other forms of chronic airways diseases, including cystic fibrosis (CF). Nasal cilia obtained by curettage of the inferior nasal turbinate from 89 patients without CF but exhibiting symptoms favoring PCD were evaluated by transmission electron microscopy. Of the 89 patients in the study group, 19 (21.4%) were diagnosed with PCD. Among the PCD patients, 16 (84.2%) exhibited microtubular discontinuities. Nine patients from this group without ultrastructural evidence of PCD also exhibited these defects, however. Furthermore, seven of eight nasal biopsy specimens from patients with CF in a separate disease control group exhibited microtubular discontinuities. Microtubular discontinuities were quantitatively negligible among control groups of healthy human subjects and individuals experimentally and naturally subjected to acute airway injury. These data provide evidence that ciliary microtubular discontinuities represent acquired ciliary defects reflective of chronic airway disease injury and are not components of a primary structural abnormality in PCD.
呼吸道中有序的纤毛超微结构与最佳黏液纤毛清除功能之间存在着关键关系。源自遗传性综合征或上皮细胞损伤的结构缺陷性纤毛可能会促进或加剧慢性疾病进程。一种以微管连续性中断为特征且先前与原发性纤毛运动障碍（PCD）相关的气道上皮纤毛病变，已在包括囊性纤维化（CF）在内的其他形式的慢性气道疾病中得到记录。通过刮取下鼻甲从89例无CF但有提示PCD症状的患者获取鼻纤毛，并通过透射电子显微镜进行评估。在研究组的89例患者中，19例（21.4%）被诊断为PCD。在PCD患者中，16例（84.2%）表现出微管连续性中断。然而，该组中9例无PCD超微结构证据的患者也表现出这些缺陷。此外，在一个单独的疾病对照组中，8例CF患者的鼻活检标本中有7例表现出微管连续性中断。在健康人类受试者以及实验性和自然性遭受急性气道损伤的个体的对照组中，微管连续性中断在数量上可忽略不计。这些数据提供了证据，表明纤毛微管连续性中断代表反映慢性气道疾病损伤的后天性纤毛缺陷，而非PCD原发性结构异常的组成部分。
