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[关注卡塔格内综合征。关于一例病例]

[Focus on Kartagener's syndrome. Apropos of a case].

作者信息

Sauvaget J, Gamet L, Capron F, Hecquet I, Csech J

出版信息

Rev Pneumol Clin. 1985;41(3):187-91.

PMID:3876586
Abstract

Kartagener syndrome, identified 40 years ago, is an obstructive bronchopulmonary condition of early onset which rapidly becomes chronic and linked to immobility of the bronchial cilia. This clinical entity is based upon a kinetic problem secondary to ultrastructural abnormalities of the cilia. Current techniques for the study of ciliary movement and electron microscopy in particular are sufficiently accurate to be able to link certain ciliary abnormalities to particular clinical manifestations. Although it is a frequent neonatal condition, Kartagener syndrome may be compatible with prolonged survival. The authors report a case in an adult, with a favorable course, and give an updated review of the condition.

摘要

卡塔格内综合征于40年前被发现,是一种起病早的阻塞性支气管肺部疾病,很快会发展为慢性疾病,并与支气管纤毛运动障碍有关。这一临床病症是由纤毛超微结构异常继发的动力学问题所致。目前研究纤毛运动的技术,尤其是电子显微镜技术,已经足够精确,能够将某些纤毛异常与特定临床表现联系起来。尽管卡塔格内综合征在新生儿中很常见,但患者也可能长期存活。作者报告了一例成年患者的病例,病情发展良好,并对该病症进行了最新综述。

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