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[原发性纤毛运动障碍;临床方面的问卷调查研究]

[Primary ciliary dyskinesia; a questionnaire study of the clinical aspects].

作者信息

de Boode W P, Collins J M, Veerman A J, van der Baan S

出版信息

Ned Tijdschr Geneeskd. 1989 Nov 25;133(47):2338-41.

PMID:2586663
Abstract

With the aid of a questionnaire form we have gathered information about the clinical picture of patients suffering from primary ciliary dyskinesia. The study group numbered 34 persons, whose diagnosis was confirmed by electron microscopy. Chronic cough and common cold symptoms are present from shortly after birth. Twenty-three respondents reported respiratory tract problems in the neonatal period. The dysfunctional cilia result in chronic respiratory tract infections (chronic bronchitis; bronchiectasis; pneumonia; chronic sinusitis, rhinitis or otitis media). These lead to the following complaints: frequent blowing of the nose (in 32 pat.; 94%), chronic productive cough (in 28 pat.; 82%), chronic common cold (in 26 pat.; 77%), hearing problems (in 24 pat.; 71%), shortness of breath (in 23 pat.; 68%), frequent headache (in 13 pat.; 38%) and sore throat (in 9 pat.; 27%). In order to prevent the invalidating consequences of this disorder appropriate steps should be taken as soon as possible. These should include physiotherapy and adequate antibiotic therapy.

摘要

借助一份调查问卷,我们收集了有关原发性纤毛运动障碍患者临床表现的信息。研究组有34人,其诊断经电子显微镜证实。慢性咳嗽和普通感冒症状自出生后不久就存在。23名受访者报告在新生儿期有呼吸道问题。功能异常的纤毛会导致慢性呼吸道感染(慢性支气管炎、支气管扩张、肺炎、慢性鼻窦炎、鼻炎或中耳炎)。这些会引发以下症状:频繁擤鼻(32例;94%)、慢性咳痰咳嗽(28例;82%)、慢性普通感冒(26例;77%)、听力问题(24例;71%)、呼吸急促(23例;68%)、频繁头痛(13例;38%)和喉咙痛(9例;27%)。为了预防这种疾病带来的致残后果,应尽快采取适当措施。这些措施应包括物理治疗和适当的抗生素治疗。

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