BCG Moreau Polish Substrain Infections in Patients With Inborn Errors of Immunity: 40 Years of Experience in the Department of Immunology, Children's Memorial Health Institute, Warsaw

被引：2

作者：

Bernatowska, Ewa ^{[1
]}

Pac, Malgorzata ^{[1
]}

Heropolitanska-Pliszka, Edyta ^{[1
]}

Pietrucha, Barbara ^{[1
]}

Dabrowska-Leonik, Nel ^{[1
]}

Skomska-Pawliszak, Malgorzata ^{[1
]}

Bernat-Sitarz, Katarzyna ^{[1
]}

Krzysztopa-Grzybowska, Katarzyna ^{[2
]}

Wolska-Kusnierz, Beata ^{[1
]}

Bohynikova, Nadia ^{[1
]}

Augustynowicz, Ewa ^{[3
]}

Augustynowicz-Kopec, Ewa ^{[4
]}

Korzeniewska-Kosela, Maria ^{[5
]}

Wieteska-Klimczak, Anna ^{[6
]}

Ksiazyk, Janusz ^{[6
]}

Jackowska, Teresa ^{[7
,8
]}

Van den Burg, Mirjam ^{[9
]}

Casanova, Jean-Laurent ^{[10
,11
,12
,13
,14
]}

Picard, Capucine ^{[15
,16
]}

Mikoluc, Bozena ^{[17
]}

机构：

[1] Childrens Mem Hlth Inst, Dept Immunol, Warsaw, Poland

[2] Natl Inst Publ Hlth, Natl Inst Hyg, Dept Sera & Vaccines Evaluat, Warsaw, Poland

[3] Natl Inst Publ Hlth, Natl Inst Hyg, Dept Epidemiol, Warsaw, Poland

[4] Natl TB & Lung Dis Res Inst, Dept Microbiol, Natl TB Reference Lab, Warsaw, Poland

[5] Natl TB & Lung Dis Res Inst, Dept TB Epidemiol & Surveillance, Warsaw, Poland

[6] Childrens Mem Hlth Inst, Dept Paediat, Nutr & Metab Dis, Warsaw, Poland

[7] Med Ctr Postgrad Educ, Dept Paediat, Warsaw, Poland

[8] Bielanski Hosp, Dept Paediat, Warsaw, Poland

[9] Leiden Univ Med Ctr, Dept Pediat, Lab Pediat Immunol, Leiden, Netherlands

[10] Howard Hughes Med Inst, New York, NY USA

[11] Rockefeller Univ Hosp, St Giles Lab Human Genet Infect Dis, New York, NY USA

[12] Paris Descartes Univ, Necker Hosp Sick Children, Paris, France

[13] Necker Hosp Sick Children, Imagine Inst, Lab Human Genet Infect Dis, Paris, France

[14] Univ Paris 05, Necker Hosp, Sch Med, Paris, France

[15] Univ paris, Imagine Inst, Paris, France

[16] Malad Hosp, Assistance Publ Hop Paris, Study Ctr Primary Immunodeficiency, Necker Enfants, Paris, France

[17] Med Univ Bialystok, Dept Paediat Rheumatol Immunol & Metab Bone Dis, Bialystok, Poland

来源：

FRONTIERS IN PEDIATRICS | 2022年 / 10卷

关键词：

Inborn Errors of Immunity; BCG infection; susceptibility to BCG infection; BCG Moreau vaccine; disseminated BCG infection; NK cells; SEVERE COMBINED IMMUNODEFICIENCY; CALMETTE-GUERIN INFECTION; MYCOBACTERIUM-BOVIS; COMPLICATIONS; TUBERCULOSIS; DOMINANT; VACCINE; PROTECTION; FEATURES; DISEASE;

D O I：

10.3389/fped.2022.839111

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

ObjectiveWe aimed to assess BCG (Bacillus Calmette-Guerin) complications in patients with Inborn Errors of Immunity (IEI), according to the inherited disorders and associated immunological defects, as well as the different BCG substrains. MaterialWe studied adverse reactions to the locally-produced BCG Moreau vaccine, analyzed in patients with IEI diagnosed between 1980 and 2020 in the Department of Immunology, Children's Memorial Health Institute (CMHI), Warsaw. These results were compared with previously published studies. ResultsSignificantly fewer disseminated BCG infections (BCGosis) were found in 11 of 72 (15%) SCID (Severe Combined Immunodeficiency) NK (Natural Killer)-phenotype patients, when compared with the 119 out of 349 (34%) (p = 0.0012) patients with SCID with BCG in other countries. Significantly fewer deaths caused by BCGosis were observed (p = 0.0402). A significantly higher number of hematopoietic stem cell transplantations (HSCTs) were performed in the CMHI study (p = 0.00001). BCGosis was found in six patients with Mendelian susceptibility to mycobacterial diseases (MSMD). Other patients with IEI prone to BCG complications, such as CGD (Chronic Granulomatous Disease), showed no case of BCGosis. ConclusionThe BCG Moreau substrain vaccine, produced in Poland since 1955, showed genetic differences with its parental Brazilian substrain together with a superior clinical safety profile in comparison with the other BCG substrains, with no BCGosis in patients with IEI other than SCID and MSMD. Our data also confirmed significantly fewer cases of BCGosis and deaths caused by BCG infection in patients with SCID with this vaccine substrain. Finally, they confirmed the protecting role of NK cells, probably via their production of IFN-gamma.

引用

页数：9

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