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阿拉伯人的原发性抗体缺陷:沙特阿拉伯东部的首例报告。

Primary antibody deficiency in Arabs: first report from eastern Saudi Arabia.

作者信息

al-Attas R A, Rahi A H

机构信息

Department of Immunology, Regional Laboratory & Blood Bank, Ministry of Health, Dammam, Saudi Arabia.

出版信息

J Clin Immunol. 1998 Sep;18(5):368-71. doi: 10.1023/a:1023247117133.

Abstract

Epidemiological studies have shown wide geographical and racial variations in the prevalence and pattern of immunodeficiency diseases. As there is no national registry, very little is known of the prevalence and nature of humoral immunodeficiency in the Arabian peninsula. We report here for the first time the analysis of serum immunoglobulin (Ig) levels in 2000 consecutive patients (age, 1-80 years). They were seen over a period of 6 years and were referred to us from six district hospitals for suspected immunodeficiency, autoimmunity, allergy, or immunoglobulin dyscrasia. Forty-six were found to be immunodeficient, in whom at least one of the Ig class was low; 15 had secondary immunodeficiency. The remaining 31 cases, representing 1.5% of the population studied (giving a prevalence of 1550/100,000 hospital registered patients), were categorized into four primary humoral immunodeficiency groups: these included, in order of frequency, (i) selective IgA deficiency (45%; 700/100,000) (ii) common variable immunodeficiency (CVID) (29%; 450/100,000), (iii) agammaglobulinemia (16%; 250/100,000), and (iv) selective IgG deficiency (10%; 150/100,000). Compared with similar hospital-based surveys in the west the prevalence of humoral immunodeficiency seems to be higher in Arabs; this in part may be related to race and higher rate of consanguinity. Most patients with IgA deficiency had either infection, atopy or autoimmunity. Compared with some other races, agammaglobulinemia (X- and non-X-linked) seems to be more prevalent.

摘要

流行病学研究表明,免疫缺陷疾病的患病率和模式在地域和种族上存在广泛差异。由于没有国家登记系统,对于阿拉伯半岛体液免疫缺陷的患病率和性质了解甚少。我们在此首次报告对2000例连续患者(年龄1至80岁)血清免疫球蛋白(Ig)水平的分析。这些患者在6年期间就诊,由六家地区医院因疑似免疫缺陷、自身免疫、过敏或免疫球蛋白异常血症转诊而来。发现46例免疫缺陷患者,其中至少一种Ig类别水平低下;15例为继发性免疫缺陷。其余31例患者,占所研究人群的1.5%(医院登记患者患病率为1550/100,000),被分为四个原发性体液免疫缺陷组:按频率依次为,(i)选择性IgA缺乏(45%;700/100,000),(ii)常见可变免疫缺陷(CVID)(29%;450/100,000),(iii)无丙种球蛋白血症(16%;250/100,000),以及(iv)选择性IgG缺乏(10%;150/100,000)。与西方类似的基于医院的调查相比,阿拉伯人体液免疫缺陷的患病率似乎更高;这部分可能与种族和较高的近亲结婚率有关。大多数IgA缺乏患者患有感染、特应性或自身免疫。与其他一些种族相比,无丙种球蛋白血症(X连锁和非X连锁)似乎更为普遍。

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