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[原发性纤毛运动障碍。纤毛超微结构的电子显微镜研究]

[Primary ciliary dyskinesia. Electron microscopy study of ciliary ultrastructure].

作者信息

Royo J A, Barrueco M, Ludeña D, Gómez F, González J M, Capurro M, Riesco J A

机构信息

Servicio de Neumología, Hospital Clínico, Salamanca.

出版信息

Med Clin (Barc). 1994 May 21;102(19):744-6.

PMID:8041205
Abstract

A 75 year old patient with respiratory infections since childhood, bronchiectasis and situs inversus is presented. Fiberoptic bronchoscopy was performed obtaining samples of bronchial mucosa which allowed the observation of the existence of ultrastructural lesions such as ciliary fusion, changes in the form of the cilia, microtubular changes and changes of the dynein arms by electronic microscopic study. Diagnosis of Kartagener's syndrome was established. In the case presented moderate clinical involvement was present which allowed the survival of the patient despite his advanced age.

摘要

报告了一名75岁自童年起患有呼吸道感染、支气管扩张和内脏转位的患者。进行了纤维支气管镜检查,获取支气管黏膜样本,通过电子显微镜研究观察到存在超微结构病变,如纤毛融合、纤毛形态改变、微管变化和动力蛋白臂改变。确诊为卡塔格内综合征。在该病例中,患者有中度临床症状,尽管年事已高,但仍存活。

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