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墨西哥瓜纳华托州参考和高专科医院的原发性免疫缺陷病。

Primary immunodeficiency diseases at reference and high-specialty hospitals in the state of Guanajuato, Mexico.

机构信息

Departamento de Inmunología, Hospital Regional de Alta Especialidad del Bajío, Boulevard Milenio 130 San Carlos La Roncha, 37660 León, GTO, Mexico.

出版信息

Biomed Res Int. 2013;2013:187254. doi: 10.1155/2013/187254. Epub 2013 Sep 1.

Abstract

BACKGROUND

In general, primary immunodeficiency diseases (PIDs) are underdiagnosed in most countries. The objective of this study was to describe the frequency and clinical spectrum of PID in the most important tertiary hospitals in our region.

METHODS

An observational, cross-sectional, with retrospective chart, review study was conducted. A total of 26 patients were included and grouped according to the updated classification of PIDs.

RESULTS

PIDs spectra were as follows: predominantly antibody deficiency diseases were the most common category (65.38%), followed by other well-defined immunodeficiency syndromes (11.55%), congenital defects of phagocyte number and/or function (7.69%), complement deficiencies (3.85%), combined T- and B-cell immunodeficiencies (3.85%), and defects in innate immunity (3.85%). The mean time elapsed from the onset of symptoms to the reference and diagnosis by a tertiary hospital was of 4.65 ± 6.95 years.

CONCLUSIONS

Predominant antibody deficiency disease was the most common group of PIDs, agreeing with international reports. Awareness of underdiagnosis by physicians is crucial for a prompt diagnosis and treatment, which in turn should improve the quality of life among patients with PIDs.

摘要

背景

在大多数国家,原发性免疫缺陷病(PID)的诊断率都较低。本研究的目的是描述我们所在地区最重要的三级医院中 PID 的频率和临床谱。

方法

进行了一项观察性、横断面、回顾性图表审查研究。共纳入 26 例患者,并根据 PID 的最新分类进行分组。

结果

PID 谱如下:以抗体缺陷疾病为主(65.38%),其次是其他明确的免疫缺陷综合征(11.55%)、吞噬细胞数量和/或功能的先天性缺陷(7.69%)、补体缺陷(3.85%)、T 和 B 细胞联合免疫缺陷(3.85%)和固有免疫缺陷(3.85%)。从症状发作到三级医院就诊和确诊的平均时间间隔为 4.65±6.95 年。

结论

以抗体缺陷疾病为主的 PID 是最常见的一组,与国际报道一致。医生对漏诊的认识对于及时诊断和治疗至关重要,这反过来又应提高 PID 患者的生活质量。

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Primary immunodeficiency diseases in Australia and New Zealand.澳大利亚和新西兰的原发性免疫缺陷疾病
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