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拉丁美洲的原发性免疫缺陷病:来自参与拉丁美洲原发性免疫缺陷病组织(LAGID)的八个国家的首次报告。拉丁美洲原发性免疫缺陷病组织

Primary immunodeficiency diseases in Latin America: first report from eight countries participating in the LAGID. Latin American Group for Primary Immunodeficiency Diseases.

作者信息

Zelazko M, Carneiro-Sampaio M, Cornejo de Luigi M, Garcia de Olarte D, Porras Madrigal O, Berrón Perez R, Cabello A, Rostan M V, Sorensen R U

机构信息

National Pediatric Hospital Juan P. Garrahan, Buenos Aires, Argentina.

出版信息

J Clin Immunol. 1998 Mar;18(2):161-6. doi: 10.1023/a:1023255102170.

Abstract

The Latin American Group for Primary Immunodeficiencies, formed in 1993, presently includes 12 countries. One goal was to study the frequency of primary immunodeficiencies in various regions of the American continent and to enhance knowledge about these diseases among primary-care physicians, as well as allergist-immunologists. Important for this purpose was the development of a registry of primary immunodeficiencies using a uniform questionnaire and computerized database. To date, eight countries have collected information on a total of 1428 patients. Predominantly antibody deficiencies were reported in 58% of patients, followed by cellular and antibody immunodeficiencies associated with other abnormalities in 18%, immunodeficiency syndromes associated with granulocyte dysfunction in 8%, phagocytic disorders in 9%, combined cellular and antibody immunodeficiencies in 5%, and complement deficiencies in 2% of patients. The information gathered from this initial analysis of data will serve to expand the patient database to more areas within participating countries and to new countries and to increase collaboration toward better diagnosis and treatment of these diseases.

摘要

拉丁美洲原发性免疫缺陷病研究小组成立于1993年,目前包括12个国家。其目标之一是研究美洲大陆不同地区原发性免疫缺陷病的发病率,并提高基层医疗医生以及过敏症专科免疫学家对这些疾病的认识。为此,利用统一问卷和计算机化数据库建立原发性免疫缺陷病登记册至关重要。迄今为止,八个国家已收集了总共1428名患者的信息。报告显示,58%的患者主要存在抗体缺陷,其次是18%的患者存在与其他异常相关的细胞和抗体免疫缺陷、8%的患者存在与粒细胞功能障碍相关的免疫缺陷综合征、9%的患者存在吞噬障碍、5%的患者存在细胞和抗体联合免疫缺陷,以及2%的患者存在补体缺陷。从这一初步数据分析中收集到的信息将有助于把患者数据库扩展到参与国的更多地区以及新的国家,并加强合作以更好地诊断和治疗这些疾病。

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