Gonzalez B, Moreno S, Burdach R, Valenzuela M T, Henriquez A, Ramos M I, Sorensen R U
Departmento de Pediatria, Hospital Luis Calvo Mackena, Santiago, Chile.
Pediatr Infect Dis J. 1989 Apr;8(4):201-6.
Nine children with immunodeficiency syndromes who developed persistent or disseminated Bacillus Calmette-Guérin (BCG) infections after BCG vaccination at birth were observed in Santiago, Chile, over a period of 10 years. This represents a risk for persistent or disseminated BCG infections of 3.4/1,000,000 vaccinated newborns. This may closely reflect the incidence of severe combined immunodeficiency syndromes, cellular immunodeficiency syndromes and chronic granulomatous disease in the study area. The clinical presentation and course of the infection varied considerably depending on the underlying immunodeficiency syndrome. Two patients with severe combined immunodeficiency presented with cutaneous nodules in the absence of any local reaction at the site of BCG vaccination. Both patients died of disseminated BCG infection within the first year of life. Four patients with cellular immunodeficiency syndromes presented with regional lymphadenitis resistant to treatment after the fifth month of life. Three of these patients had specific unresponsiveness to tuberculin and survived from 5 to 6 years of age. Two boys with X-linked chronic granulomatous disease presented with regional lymphadenitis in the first 3 months of life. A girl with autosomal recessive chronic granulomatous disease presented at 18 months of age with regional lymphadenitis. All three patients with chronic granulomatous disease had positive tuberculin reactions and died from infections other than BCG.
在智利圣地亚哥，10年间观察到9名患有免疫缺陷综合征的儿童，他们在出生时接种卡介苗后发生了持续性或播散性卡介苗感染。这代表每100万接种卡介苗的新生儿中发生持续性或播散性卡介苗感染的风险为3.4例。这可能密切反映了研究区域内严重联合免疫缺陷综合征、细胞免疫缺陷综合征和慢性肉芽肿病的发病率。感染的临床表现和病程因潜在的免疫缺陷综合征而异。两名严重联合免疫缺陷患者在卡介苗接种部位无任何局部反应的情况下出现皮肤结节。两名患者均在出生后第一年内死于播散性卡介苗感染。四名细胞免疫缺陷综合征患者在出生后第五个月后出现对治疗有抵抗的局部淋巴结炎。其中三名患者对结核菌素无特异性反应，存活至5至6岁。两名患有X连锁慢性肉芽肿病的男孩在出生后的前3个月出现局部淋巴结炎。一名患有常染色体隐性慢性肉芽肿病的女孩在18个月大时出现局部淋巴结炎。所有三名慢性肉芽肿病患者结核菌素反应均为阳性，并死于卡介苗以外的感染。
