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[普通变异型免疫缺陷病(CVID):最常见的有症状的原发性免疫缺陷病的异质性临床表现]

[CVID (common variable immunodeficiency): heterogeneous clinical manifestation of the commonest symptomatic primary immunodeficiency disease].

作者信息

Pfluger H, Helbling A, Mordasini C, Pichler W J

机构信息

Abteilung für Allergologie, Klinik für Rheumatologie und Klinische Immunologie/Allergologie, Inselspital, Bern.

出版信息

Schweiz Med Wochenschr. 2000 Oct 28;130(43):1590-9.

Abstract

Common variable immunodeficiency is the most common symptomatic primary immunodeficiency disease. The patients typically present with a long history of respiratory tract infections, sometimes sarcoid-like lesions and in rare cases boils. Heterogeneity of initial clinical manifestations as well as insufficient knowledge of the syndrome often delay the diagnosis. However, early therapy is important to reduce infections and in particular the development of bronchiectasis. Documenting 19 cases, we discuss initial clinical manifestations, some clinical complications, diagnostic procedures and therapeutic management.

摘要

普通可变免疫缺陷是最常见的有症状的原发性免疫缺陷病。患者通常有长期的呼吸道感染病史,有时会出现类肉瘤样病变,罕见情况下会出现疖肿。初始临床表现的异质性以及对该综合征认识不足常常会延误诊断。然而,早期治疗对于减少感染尤其是预防支气管扩张的发生很重要。我们记录了19例病例,讨论了初始临床表现、一些临床并发症、诊断程序及治疗管理。

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