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卡塔格内综合征。关于纤毛结构、功能及上呼吸道症状的初步报告。

Kartagener's syndrome. Preliminary report on cilia structure, function, and upper airway symptoms.

作者信息

Pedersen M, Morkassel E, Nielsen M H, Mygind N

出版信息

Chest. 1981 Dec;80(6 Suppl):858-60. doi: 10.1378/chest.80.6.858.

Abstract

Our study was designed to examine the motility and ultrastructure of cilia from the nose of patients with Kartagener's syndrome. Microphoto-oscillographic recording from single cells showed that the patients had in fact motile cilia, although the number was reduced. Asynchrony within the single cell was a more consistent feature. The first results of blind, quantitative microscopy showed the ultrastructural defects, described earlier, but the overlapping with a normal control group was considerable. Only one of nine patients had no dynein arms and completely immotile cilia; an observation which renders the term "the immotile-cilia syndrome" a misnomer. The ear-nose-throat symptoms were characterized by daily nose-blowings since birth, recurrent sinusitis, and chronic secretory otitis media. On the other hand, the frequency of acute purulent otitis media and of common colds appeared to be normal.

摘要

我们的研究旨在检查卡塔格内综合征患者鼻腔纤毛的运动性和超微结构。单细胞的显微摄影振荡记录显示,患者实际上有可运动的纤毛,尽管数量减少。单细胞内的不同步是一个更一致的特征。盲法定量显微镜检查的初步结果显示了先前描述的超微结构缺陷,但与正常对照组的重叠相当大。9名患者中只有1人没有动力蛋白臂且纤毛完全不动;这一观察结果表明“不动纤毛综合征”这个术语并不恰当。耳鼻喉症状的特点是自出生以来每天擤鼻涕、复发性鼻窦炎和慢性分泌性中耳炎。另一方面,急性化脓性中耳炎和普通感冒的发生率似乎正常。

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