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[疑似纤毛运动障碍的支气管黏膜超微结构研究]

[Ultrastructural study of bronchial mucosa in suspicion of ciliary dyskinesia].

作者信息

Tiszlavicz L, Rázga Z, Baktai G, Péterffy E, Márialigeti T, Gyurkovits K, Szabó A

机构信息

Szent-Györgyi Albert Orvos- és Gyógyszerésztudományi Centrum, Szegedi Tudományegyetem, Altalános Orvostudományi Kar.

出版信息

Orv Hetil. 2000 Jun 11;141(24):1349-53.

Abstract

In the ciliary dyskinesia (immotile cilia syndrome) shows the partial or total lacking of cilia's elements. The ciliary dyskinesia may be developed congenital or acquired. The authors report on the experience with 72 biopsies from bronchial mucosa of 68 children, submitted with the question of immotile cilia syndrome. On micrographs (M: 64,000x) of the specimens processed by routine electron microscopical method the number of outer and inner dynein arm, A and B peripheral tubules, central tubules and central sheet were determined to normal 9 + 2 structure. 50-100 ciliaris per case were examined. Total or partial lacking of dynein and non-dynein elements were expressed for the total number of ciliaris compartments. Considering any earlier quantitative examination with this expression there was ease to characterised the quantitative behaviour of the components of ciliaris. Seven Kartagener's syndrome cases was the positive control for determined the quantitative differences between the primer and secondary ciliary changes. In the primary ciliary defects where the situs inversus were presented the total lacking of outer or/and inner dynein arms, where the situs inversus were not presented only the total lacking of inner dynein arms could be found. In secondary ciliary defects the partial lacking of the dynein arms and tubular components were presented. The used method is suitable to characterise the primary or secondary ciliary defects of bronchial mucosa.

摘要

在睫状体运动障碍(不动纤毛综合征)中,显示出纤毛成分部分或完全缺失。睫状体运动障碍可能是先天性的或后天获得的。作者报告了对68名儿童支气管黏膜进行72次活检的经验,这些儿童被诊断为不动纤毛综合征。在通过常规电子显微镜方法处理的标本的显微照片(放大倍数:64,000倍)上,确定外动力蛋白臂和内动力蛋白臂、A和B外周微管、中央微管和中央鞘的数量为正常的9 + 2结构。每例检查50 - 100根纤毛。对于纤毛各部分的总数而言,动力蛋白和非动力蛋白成分存在全部或部分缺失。考虑到用这种表达方式进行的任何早期定量检查,很容易描述纤毛成分的定量行为。7例卡塔格内综合征病例作为阳性对照,以确定初级和次级纤毛变化之间的定量差异。在出现内脏反位的原发性纤毛缺陷中,可发现外动力蛋白臂或/和内动力蛋白臂完全缺失,而在未出现内脏反位的情况下,仅能发现内动力蛋白臂完全缺失。在继发性纤毛缺陷中,出现动力蛋白臂和微管成分部分缺失。所使用的方法适用于描述支气管黏膜的原发性或继发性纤毛缺陷。

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