Department of Pharmacy, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore.
Rheumatology and Immunology Service, Department of Pediatric Medicine, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore; Duke-National University of Singapore Medical School, 8 College Road, Singapore 169857, Singapore.
Int J Infect Dis. 2020 Aug;97:117-125. doi: 10.1016/j.ijid.2020.05.117. Epub 2020 Jun 2.
Disseminated Bacillus Calmette-Guérin (BCG) disease (BCGosis) is a classical feature of children with primary immunodeficiency disorders (PIDs).
A 15-year retrospective review was conducted in KK Women's and Children's Hospital in Singapore, from January 2003 to October 2017.
Ten patients were identified, the majority male (60.0%). The median age at presentation of symptoms of BCG infections was 3.8 (0.8 - 7.4) months. All the patients had likely underlying PIDS - four with Severe Combined Immunodeficiency (SCID), three with Mendelian Susceptibility to Mycobacterial Diseases (MSMD), one with Anhidrotic Ectodermal Dysplasia with Primary Immunodeficiency (EDA-ID), one with combined immunodeficiency (CID), and one with STAT-1 gain-of-function mutation. Definitive BCGosis was confirmed in all patients by the identification of Mycobacterium bovis subsp BCG from microbiological cultures. The susceptibility profiles of Mycobacterium bovis subsp BCG are as follows: Rifampicin (88.9%), Isoniazid (44.47%), Ethambutol (100.0%), Streptomycin (100.0%), Kanamycin (100.0%), Ethionamide (25.0%), and Ofloxacin (100.0%). Four patients (40.0%) received a three-drug regimen. Five patients (50.0%) underwent hematopoietic stem cell transplant (HSCT), of which three (60%) have recovered. Overall mortality was 50.0%.
Disseminated BCG disease (BCGosis) should prompt immunology evaluation to determine the diagnosis of the immune defect. A three-drug regimen is adequate for treatment if the patient undergoes early HSCT.
卡介苗（BCG）播散病（BCGosis）是原发性免疫缺陷病（PID）患儿的典型特征。
对新加坡 KK 妇女儿童医院 2003 年 1 月至 2017 年 10 月进行的 15 年回顾性研究。
共发现 10 例患者，大多数为男性（60.0%）。出现 BCG 感染症状的中位年龄为 3.8（0.8-7.4）个月。所有患者均存在潜在 PID-4 例严重联合免疫缺陷（SCID），3 例孟德尔易感性分枝杆菌病（MSMD），1 例无汗性外胚层发育不良伴原发性免疫缺陷（EDA-ID），1 例联合免疫缺陷（CID）和 1 例 STAT-1 功能获得性突变。所有患者均通过微生物培养鉴定出牛分枝杆菌亚种 BCG 来确诊为明确的 BCGosis。牛分枝杆菌亚种 BCG 的药敏谱如下：利福平（88.9%）、异烟肼（44.47%）、乙胺丁醇（100.0%）、链霉素（100.0%）、卡那霉素（100.0%）、乙硫异烟胺（25.0%）和氧氟沙星（100.0%）。4 例（40.0%）患者接受了三联药物治疗。5 例（50.0%）患者接受了造血干细胞移植（HSCT），其中 3 例（60%）已康复。总体死亡率为 50.0%。
播散性卡介苗病（BCGosis）应提示进行免疫学评估以确定免疫缺陷的诊断。如果患者早期进行 HSCT，则三联药物治疗即可。
