慢性肉芽肿病临床管理与预防接种专家共识OA
Expert consensus on clinical management and vaccination for chronic granulomatous disease
慢性肉芽肿病(CGD)是一种由吞噬细胞功能缺陷所致的出生免疫缺陷.该病由于烟酰胺腺嘌呤二核苷酸磷酸(NADPH)氧化酶缺陷,导致吞噬细胞无法正常生成超氧阴离子(O2-),从而引起反复严重的细菌及真菌感染,并伴有肉芽肿形成.目前尚缺少针对CGD系统管理及预防接种的规范化共识或指南.中华医学会儿科学分会免疫学组及国家儿童医学暨临床研究中心出生免疫缺陷联盟(IEI.CHN)组织专家基于现有证据并结合临床实践经验形成本共识.共识针对CGD相关的10个常见临床问题形成18条推荐意见,旨在为CGD的诊断、治疗和长期随访管理提供参考.
Chronic granulomatous disease(CGD)is an inborn error of immunity caused by phagocyte dysfunction.Due to NADPH oxidase deficiency,phagocytes are unable to generate superoxide anions(O2)normally,leading to recurrent and severe bacterial and fungal infections accompanied by granuloma formation.Currently,there is a lack of standardized consensus or guidelines for the systematic management and vaccination for CGD.Group of Immunology of the Society of Pediatrics of Chinese Medical Association and Inborn Errors of Immunity Alliance of National Children's Medical and Clinical Research Center(IEI.CHN)have organized experts to develop this consensus based on existing evidence and clinical practice experience.The consensus addresses 10 common clinical issues related to CGD and formulates 18 recommendations,aiming to provide a reference for the diagnosis,treatment,and long-term follow-up management of CGD.
中华医学会儿科学分会免疫学组;国家儿童医学暨临床研究中心IEI联盟;孙金峤;王晓川
医药卫生
慢性肉芽肿病出生免疫缺陷感染造血干细胞移植预防接种
chronic granulomatous diseaseinborn errors of immunityinfectionHSCTvaccination
《中国实用儿科杂志》 2026 (7)
529-537,9
国家重点研发计划(2022YFC2704905)上海市市级科技重大专项"重大突发传染病防控关键核心技术研究"(ZD2021CY001)
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