拉丁美洲免疫缺陷协会登记处。

IF 7.2 2区 医学 Q1 IMMUNOLOGY
Gisela Seminario, Maria Edith Gonzalez-Serrano, Carolina Sanchez Aranda, Anete Sevciovic Grumach, Gesmar Rodrigues Silva Segundo, Lorena Regairaz, Aristoteles Alvares Cardona, Juan Carlos Aldave Becerra, Cecilia Poli, Alejandra King, Fatima Rodrigues Fernandes, Lily Leiva, Jose Luis Franco, Francisco Javier Espinosa-Rosales, Ricardo Sorensen, Beatriz Tavares Costa Carvalho, Liliana Bezrodnik, Antonio Condino-Neto
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引用次数: 0

摘要

目的 - 拉丁美洲免疫缺陷协会(LASID)登记处成立于 2009 年,旨在收集该地区先天性免疫缺陷(IEI)患者的数据。尽管有关 LASID 数据的报告已发表过数篇,但这是有关整个数据集的第一份报告。方法 - 欧洲免疫缺陷协会(ESID)于 2008 年捐赠了在线平台。从 2009 年 4 月 13 日至 2022 年 12 月 31 日,从参与中心收集数据,包括人口统计学、临床和随访信息。结果 - 共有 9307 名患者被纳入数据库。研究结束时,有8805名患者存活或失去随访,502名患者死亡。最常见的 IEI 类型主要是抗体缺乏症(PAD,60.35%),选择性 IgA 缺乏症是最常见的诊断(1627 名患者,17.48%),其次是常见变异性免疫缺陷症(CVID,1191 名患者)。大多数患者(78.16%)入组时年龄小于 18 岁,诊断时的中位年龄为 4.77 岁。确诊时间中位数为 5.04 年。32.3%的就诊者使用了抗生素,其次是免疫球蛋白(29.49%)。5.03%的患者进行了造血干细胞移植。奥曼综合征是死亡患者中最常见的疾病,死亡率为 52.63%。结论 - 该研究有助于我们了解拉丁美洲的 IEI,并强调了早期诊断、适当治疗和改进数据收集以优化患者预后的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
The Latin American Society for Immunodeficiencies Registry.

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.

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来源期刊
CiteScore
12.20
自引率
9.90%
发文量
218
审稿时长
2 months
期刊介绍: The Journal of Clinical Immunology publishes impactful papers in the realm of human immunology, delving into the diagnosis, pathogenesis, prognosis, or treatment of human diseases. The journal places particular emphasis on primary immunodeficiencies and related diseases, encompassing inborn errors of immunity in a broad sense, their underlying genotypes, and diverse phenotypes. These phenotypes include infection, malignancy, allergy, auto-inflammation, and autoimmunity. We welcome a broad spectrum of studies in this domain, spanning genetic discovery, clinical description, immunologic assessment, diagnostic approaches, prognosis evaluation, and treatment interventions. Case reports are considered if they are genuinely original and accompanied by a concise review of the relevant medical literature, illustrating how the novel case study advances the field. The instructions to authors provide detailed guidance on the four categories of papers accepted by the journal.
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