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通过电子显微镜诊断出一例卡塔格内综合征患者存在原发性纤毛运动障碍。

Primary ciliary dyskinesia diagnosed by electron microscopy in one case of Kartagener syndrome.

作者信息

Rugină Aniela Luminiţa, Dimitriu Alexandru Grigore, Nistor Nicolai, Mihăilă Doina

机构信息

"Grigore T. Popa" University of Medicine and Pharmacy, Iassy, Romania;

出版信息

Rom J Morphol Embryol. 2014;55(2 Suppl):697-701.

Abstract

Primary ciliary dyskinesia (PCD) is associated with abnormalities in the structure of a function of motile cilia, causing impairment of muco-ciliary clearence, with bacterial overinfection of the upper and lower respiratory tract (chronic oto-sino-pulmonary disease), heterotaxia (situs abnormalities), with/without congenital heart disease, abnormal sperm motility with male infertility, higher frequency of ectopic pregnancy and female subfertility. The presence of recurrent respiratory tract infections in the pediatric age requires differentiation between primary immunodeficiency, diseases with abnormal mucus (e.g., cystic fibrosis) and abnormal ciliary diseases. This case was hospitalized for recurrent respiratory tract infections and total situs inversus at the age of five years, which has enabled the diagnosis of Kartagener syndrome. The PCD confirmation was performed by electron microscopy examination of nasal mucosa cells through which were confirmed dynein arms abnormalities. The diagnosis and early treatment of childhood PCD allows a positive development and a good prognosis, thus improving the quality of life.

摘要

原发性纤毛运动障碍(PCD)与运动性纤毛的结构和功能异常有关,导致黏液纤毛清除功能受损,进而引起上、下呼吸道细菌反复感染(慢性耳-鼻-肺疾病)、内脏异位(脏器位置异常),可伴有或不伴有先天性心脏病、精子运动异常导致男性不育、异位妊娠发生率增加以及女性生育力低下。小儿反复呼吸道感染时,需要鉴别原发性免疫缺陷、黏液异常疾病(如囊性纤维化)和纤毛异常疾病。该病例5岁时因反复呼吸道感染和完全性内脏反位住院,从而得以诊断为卡塔格内综合征。通过对鼻黏膜细胞进行电子显微镜检查证实了PCD,发现其中存在动力蛋白臂异常。儿童PCD的诊断和早期治疗有利于病情积极发展和预后良好,从而提高生活质量。

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