Carson J L, Collier A M, Gambling T M, Knowles M R, Boucher R C
Department of Pediatrics, University of North Carolina, Chapel Hill 27514.
Hum Pathol. 1990 Jun;21(6):640-7. doi: 10.1016/s0046-8177(96)90011-8.
Alterations in transepithelial ion fluxes are a primary pathophysiologic feature in cystic fibrosis (CF). Chronic respiratory infections and host responses are secondary aggravating pulmonary complications of this disease. In the present study, the application of the freeze-fracture technique to samples of nasal and tracheal epithelium from patients with CF has provided a perspective of large areas of cell membrane for the evaluation of possible structural correlates to the pathophysiology of this disease. A variety of aberrant configurations in stranding pattern and disorganization of the epithelial tight junctional complexes in CF airway epithelium are described. Additionally, examination of ciliary membranes revealed the presence of compound cilia and dysmorphology of ciliary necklace configuration. These features are thought to represent acquired structural lesions possibly derived from chronic infection and/or host responses which may further exacerbate abnormal ion transport properties and decrements of ciliary function that appear to be associated with the airway epithelium of individuals with CF.
跨上皮离子通量的改变是囊性纤维化（CF）的主要病理生理特征。慢性呼吸道感染和宿主反应是该疾病继发性加重的肺部并发症。在本研究中，将冷冻断裂技术应用于CF患者的鼻和气管上皮样本，为评估与该疾病病理生理学可能的结构相关性提供了大面积细胞膜的视角。描述了CF气道上皮中上皮紧密连接复合体的绞合模式和紊乱的各种异常构型。此外，对纤毛膜的检查发现存在复合纤毛和纤毛项链构型的畸形。这些特征被认为代表了可能源自慢性感染和/或宿主反应的获得性结构损伤，这可能会进一步加剧异常的离子转运特性和纤毛功能减退，而这些似乎与CF患者的气道上皮有关。
