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79 例慢性肉芽肿病患者队列中 BCG 与结核病的描述。

Description of BCG and Tuberculosis Disease in a Cohort of 79 Patients with Chronic Granulomatous Disease.

机构信息

Laboratory of Immunodeficiency, National Institute of Pediatrics, Mexico City, Mexico.

Department of Clinical Immunology, National Institute of Pediatrics, Mexico City, Mexico.

出版信息

J Clin Immunol. 2024 Aug 5;44(8):171. doi: 10.1007/s10875-024-01778-7.

Abstract

PURPOSE

Chronic granulomatous disease (CGD) is an inherited immunodeficiency caused by pathogenic variants of genes encoding the enzyme complex NADPH oxidase. In countries where tuberculosis (TB) is endemic and the Bacillus Calmette-Guérin (BCG) vaccine is routinely administered, mycobacteria are major disease-causing pathogens in CGD. However, information on the clinical evolution and treatment of mycobacterial diseases in patients with CGD is limited. The present study describes the adverse reactions to BCG and TB in Mexican patients with CGD.

METHODS

Patients with CGD who were evaluated at the Immunodeficiency Laboratory of the National Institute of Pediatrics between 2013 and 2024 were included. Medical records were reviewed to determine the clinical course and treatment of adverse reactions to BCG and TB disease.

RESULTS

A total of 79 patients with CGD were included in this study. Adverse reactions to BCG were reported in 55 (72%) of 76 patients who received the vaccine. Tuberculosis was diagnosed in 19 (24%) patients. Relapse was documented in three (10%) of 31 patients with BGC-osis and six (32%) of 19 patients with TB, despite antituberculosis treatment. There was no difference in the frequency of BCG and TB disease between patients with pathogenic variants of the X-linked CYBB gene versus recessive variants.

CONCLUSIONS

This report highlights the importance of considering TB in endemic areas and BCG complications in children with CGD to enable appropriate diagnostic and therapeutic approaches to improve prognosis and reduce the risk of relapse.

摘要

目的

慢性肉芽肿病(CGD)是一种遗传性免疫缺陷病,由编码 NADPH 氧化酶复合物的酶基因的致病性变异引起。在结核病(TB)流行且卡介苗(BCG)疫苗常规接种的国家,分枝杆菌是 CGD 中的主要致病病原体。然而,关于 CGD 患者分枝杆菌病的临床演变和治疗的信息有限。本研究描述了墨西哥 CGD 患者对 BCG 和 TB 的不良反应。

方法

纳入 2013 年至 2024 年期间在国家儿科研究所免疫缺陷实验室评估的 CGD 患者。回顾病历以确定对 BCG 和 TB 疾病不良反应的临床过程和治疗。

结果

本研究共纳入 79 例 CGD 患者。76 例接受疫苗的患者中有 55 例(72%)报告了 BCG 不良反应。19 例(24%)诊断为结核病。尽管进行了抗结核治疗,但在 BGC-osis 的 31 例患者中有 3 例(10%)和 TB 的 19 例患者中有 6 例(32%)记录了复发。X 连锁 CYBB 基因突变与隐性变异患者中 BCG 和 TB 疾病的发生率无差异。

结论

本报告强调了在流行地区考虑 TB 和 CGD 儿童中 BCG 并发症的重要性,以采取适当的诊断和治疗方法改善预后并降低复发风险。

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