Abstract: Bacillus calmette-guerin （BCG） disease is an abnormal reaction that may occur after inoculation with the BCG vaccine， especially in immunodeficient children. Inborn Errors of Immunity Alliance of National Children’s Medical and Clinical Research Center（IEI.CHN）has developed an expert consensus to standardize the diagnosis and treatment of BCG disease. The consensus states that BCG disease can be divided into local infection， regional infection， distant infection and disseminated infection. The diagnosis is based on BCG vaccination history， clinical presentation， and etiological or pathological evidence. Assessment of immune function is critical in children with distant and disseminated infections. Therapeutically， prophylactic anti-tuberculosis therapy is recommended for immunodeficient children susceptible to BCG. For IEI patients with BCG disease， long-term anti-tuberculosis therapy is required. Some types of IEI require radical treatment by hematopoietic stem cell transplantation. The consensus also highlights the importance of immunotherapy， especially for Mendelian genetic mycobacterium susceptibility diseases with reduced interferon-gamma production.

Key words: BCG, inborn errors of immunity, chronic granulomatous disease, severe combined immunodeficiency disease, Mendelian susceptibility to mycobacterial disease, diagnosis, treatment

摘要： 卡介苗病是接种卡介苗（bacillus calmette- guerin， BCG）后可能出现的异常反应，尤其在免疫缺陷儿童中较为常见。国家儿童医学暨临床研究中心出生免疫缺陷（inborn errors of immunity，IEI）联盟制定了专家共识，旨在规范卡介苗病的诊断和治疗。共识指出，卡介苗病可分为局部感染、区域感染、远距离感染和播散性感染。诊断依据包括BCG接种史、临床表现、病原学或病理证据。免疫功能评估对于远距离及播散性感染的患儿至关重要。治疗方面，对于易感BCG的免疫缺陷患儿推荐预防性抗结核治疗，而卡介苗病的IEI患儿需要长期抗结核治疗。部分IEI疾病类型需要造血干细胞移植根治。该共识还强调了免疫治疗的重要性，特别是对于干扰素-γ产生减少的孟德尔遗传分枝杆菌易感性疾病。

关键词: 卡介苗, 出生免疫缺陷, 慢性肉芽肿病, 重症联合免疫缺陷病, 孟德尔遗传分枝杆菌易感性疾病, 诊断, 治疗