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[原发性纤毛运动障碍:功能与形态学研究]

[Primary ciliary dyskinesia: functional and morphological study].

作者信息

Armengot M, Carda C, Basterra J

机构信息

Servicio de ORL, Hospital General Universitario, Facultad de Medicina, Valencia.

出版信息

Acta Otorrinolaringol Esp. 1993 Jul-Aug;44(4):297-301.

PMID:8217273
Abstract

We present 7 patients with a typical symptomatology of immotile cilia syndrome, three of them with complete situs inversus. Nasal mucociliary transport was studied by sero-albumin marked with technetium 99m. In all cases there was an absence of transport. The ultrastructure of the nasal cilia was studied. The findings were: In four cases alterations in the dynein arms, in three cases alterations in the central and peripheral microtubules associated por not to the defects in the dynein arms, and in one cases absence of cilia were observed. The ciliary complexes were common to all cases.

摘要

我们报告了7例具有典型不动纤毛综合征症状的患者,其中3例完全性内脏转位。通过用99m锝标记的血清白蛋白研究鼻黏膜纤毛运输。在所有病例中均未发现运输现象。研究了鼻纤毛的超微结构。结果发现:4例患者的动力蛋白臂有改变,3例患者的中央和外周微管有改变,这些改变与动力蛋白臂的缺陷有关或无关,1例患者观察到无纤毛。所有病例的纤毛复合体均相同。

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