Casanova J L, Blanche S, Emile J F, Jouanguy E, Lamhamedi S, Altare F, Stéphan J L, Bernaudin F, Bordigoni P, Turck D, Lachaux A, Albertini M, Bourrillon A, Dommergues J P, Pocidalo M A, Le Deist F, Gaillard J L, Griscelli C, Fischer A
Unité d'Immunologie et Hématologie Pédiatrique, CHU Necker Enfants Malades, Paris, France.
Pediatrics. 1996 Oct;98(4 Pt 1):774-8.
Disseminated bacillus Calmette-Guérin (BCG) infection after inoculation of live vaccine is considered to result from impaired immunity of the child. However, in half of the cases, regarded as idiopathic, no well-defined immunodeficiency condition can account for the infection. The objective of the present study is to report the prevalence, clinical features, associated infections, and outcomes of children with idiopathic disseminated BCG infection.
National retrospective survey during the period from 1974 through 1994 in France.
All neonatology and pediatrics units in primary care and referral centers throughout France.
Data were collected from 595 (82%) of 721 units, 377 (93%) of 407 centers, and 320 (93%) of 345 cities. Selection criteria included BCG infection, dissemination to at least two areas beyond the inoculation site, and no well-defined immunodeficiency condition. Sixteen children (8 girls and 8 boys), born to families unrelated to each other but often consanguinous (5 of 16) or abroad (5 of 16).
The minimal prevalence rate was estimated at 0.59 cases per 1 million vaccinated children born in France. Clinical features included fever and cachexia, disseminated BCG infection to lymph nodes (15 of 16), skin (13 of 16), soft tissues (11 of 16), lungs (11 of 16), spleen (11 of 16), liver (11 of 16), and bones (9 of 16). Eight children had associated or subsequent severe opportunistic infection (50%), with either nontyphi Salmonella enterica serotypes (7 of 16) or Mycobacterium abscessus (1 of 16). The outcome was poor: 8 children (50%) died; the cause of death was BCG infection for most children (7 of 8); 8 survived until the last follow-up (50%).
Idiopathic disseminated BCG infection is a rare but severe complication of BCG vaccination. The infection probably results from an as yet unknown genetically determined immunodeficiency condition that affects the killing of intracellular bacteria such as BCG and Salmonella.
接种活疫苗后发生播散性卡介苗（BCG）感染被认为是由于儿童免疫功能受损所致。然而，在一半被视为特发性的病例中，没有明确的免疫缺陷状况可以解释这种感染。本研究的目的是报告特发性播散性BCG感染儿童的患病率、临床特征、相关感染及转归。
1974年至1994年期间在法国进行的全国性回顾性调查。
法国各地初级保健和转诊中心的所有新生儿科和儿科病房。
数据收集自721个单位中的595个（82%）、407个中心中的377个（93%）以及345个城市中的320个（93%）。入选标准包括BCG感染、播散至接种部位以外至少两个区域且无明确的免疫缺陷状况。16名儿童（8名女孩和8名男孩），出生于互不相关但常有近亲关系（16例中有5例）或来自国外（16例中有5例）的家庭。
估计法国每100万接种疫苗的儿童中最低患病率为0.59例。临床特征包括发热和恶病质，播散性BCG感染累及淋巴结（16例中有15例）、皮肤（16例中有13例）、软组织（16例中有11例）、肺（16例中有11例）、脾（16例中有11例）、肝（16例中有11例）和骨（16例中有9例）。8名儿童伴有或随后发生严重机会性感染（50%），感染菌为非伤寒沙门氏菌血清型（16例中有7例）或脓肿分枝杆菌（16例中有1例）。转归较差：8名儿童（50%）死亡；大多数儿童（8例中有7例）的死因是BCG感染；8名儿童存活至最后一次随访（50%）。
特发性播散性BCG感染是BCG疫苗接种罕见但严重的并发症。这种感染可能是由一种尚未明确的基因决定的免疫缺陷状况引起的，该状况影响对细胞内细菌如BCG和沙门氏菌的杀灭。
