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拉丁美洲免疫缺陷协会注册处。

The Latin American Society for Immunodeficiencies Registry.

机构信息

Department of Immunology, Institute of Biomedical Sciences, University of São Paulo, 1730 Lineu Prestes Avenue, São Paulo, 05508-000, Brazil.

Centro de Inmunología Clínica, Buenos Aires, Argentina.

出版信息

J Clin Immunol. 2024 Oct 22;45(1):28. doi: 10.1007/s10875-024-01822-6.

Abstract

Purpose - The Latin American Society of Immunodeficiencies (LASID) Registry was established in 2009 to collect data on Inborn Errors of Immunity (IEI) patients in the region. Although several reports have been published regarding LASID data, this is the first report of the entire dataset. Methods - The European Society of Immunodeficiencies (ESID) donated the online platform in 2008. Data was collected from participating centers from Apr 13, 2009, to Dec 31, 2022, and included demographic, clinical, and follow-up information. Results - A total of 9307 patients were included in the database. At the end of the study period, 8,805 patients were alive or lost to follow-up, and 502 were deceased. The most common type of IEI was predominantly antibody deficiency (PAD, 60.35%), and selective IgA deficiency was the most frequent diagnosis (1627 patients, 17.48%), followed by Common Variable Immune Deficiency (CVID, 1191 patients). Most patients (78.16%) were ≤ 18 years old at inclusion, and the median age at diagnosis was 4.77 years. The median time to diagnosis was 5.04 years. Antibiotics were prescribed in 32.3% of visits, followed by immunoglobulins (29.49% ). Hematopoietic stem cell transplantation was performed in 5.03% of patients. Omenn syndrome was the most common disease in deceased patients, with a mortality rate of 52.63%. Conclusion - This study contributes to our understanding of IEIs in Latin America and highlights the importance of early diagnosis, appropriate treatments, and improved data collection to optimize patient outcome.

摘要

目的 - 拉丁美洲免疫缺陷学会(LASID)注册中心成立于 2009 年,旨在收集该地区先天性免疫缺陷(IEI)患者的数据。尽管已经发表了几篇关于 LASID 数据的报告,但这是首次报告整个数据集。方法 - 欧洲免疫缺陷学会(ESID)于 2008 年捐赠了在线平台。数据于 2009 年 4 月 13 日至 2022 年 12 月 31 日从参与中心收集,包括人口统计学、临床和随访信息。结果 - 数据库共纳入 9307 例患者。研究期末,8805 例患者存活或失访,502 例患者死亡。最常见的 IEI 类型是主要抗体缺陷(PAD,60.35%),最常见的诊断是选择性 IgA 缺陷(1627 例,17.48%),其次是普通可变免疫缺陷(CVID,1191 例)。大多数患者(78.16%)入组时年龄≤18 岁,诊断时的中位年龄为 4.77 岁。中位诊断时间为 5.04 年。32.3%的就诊时处方抗生素,其次是免疫球蛋白(29.49%)。5.03%的患者接受了造血干细胞移植。欧门综合征是死亡患者中最常见的疾病,死亡率为 52.63%。结论 - 本研究有助于我们了解拉丁美洲的 IEI,并强调了早期诊断、适当治疗和改善数据收集的重要性,以优化患者预后。

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