Konrádová V, Vavrová V, Hlousková Z, Copová M, Tománek A, Houstĕk J
Eur J Respir Dis. 1982 Nov;63(6):516-25.
The ultrastructure of the bronchial epithelium in three children with recurrent bronchopneumonia, four patients with cystic fibrosis and two with Kartagener's syndrome was studied. The children with recurrent bronchopneumonia and those with Kartagener's syndrome had mostly changes in the pseudostratified ciliated epithelium, and two of them had ultrastructural signs of developing squamous metaplasia. Developed stratified squamous epithelium was found in three of four patients with cystic fibrosis and in one patient with recurrent bronchopneumonia. The squamous epithelium showed striking pathological changes, but, only the oldest patient with cystic fibrosis showed signs of onset of keratinization. Both patients with Kartagener's syndrome had the defect of dynein arms typical of the immotile-cilia syndrome. In the youngest patient the partial absence of dynein arms was combined with a defect in the radial structures of the axonemes.
对三名复发性支气管肺炎患儿、四名囊性纤维化患者和两名卡塔格内综合征患者的支气管上皮超微结构进行了研究。复发性支气管肺炎患儿和卡塔格内综合征患者的假复层纤毛上皮大多有变化，其中两名患者有鳞状化生发展的超微结构迹象。在四名囊性纤维化患者中的三名以及一名复发性支气管肺炎患者中发现了发育成熟的复层鳞状上皮。鳞状上皮显示出明显的病理变化，但只有年龄最大的囊性纤维化患者有角化开始的迹象。两名卡塔格内综合征患者均有典型的不动纤毛综合征的动力蛋白臂缺陷。在最年轻的患者中，动力蛋白臂部分缺失与轴丝的径向结构缺陷同时存在。
