Beatriz E Marciano1, Chiung-Yu Huang2, Gyan Joshi2, Nima Rezaei3, Beatriz Costa Carvalho4, Zoe Allwood5, Aydan Ikinciogullari6, Shereen M Reda7, Andrew Gennery8, Vojtech Thon9, Francisco Espinosa-Rosales10, Waleed Al-Herz11, Oscar Porras12, Anna Shcherbina13, Anna Szaflarska14, Şebnem Kiliç15, Jose L Franco16, Andrea C Gómez Raccio17, Persio Roxo18, Isabel Esteves19, Nermeen Galal20, Anete Sevciovic Grumach21, Salem Al-Tamemi22, Alisan Yildiran23, Julio C Orellana24, Masafumi Yamada25, Tomohiro Morio26, Diana Liberatore27, Yoshitoshi Ohtsuka28, Yu-Lung Lau29, Ryuta Nishikomori30, Carlos Torres-Lozano31, Juliana T L Mazzucchelli4, Maria M S Vilela32, Fabiola S Tavares4, Luciana Cunha33, Jorge A Pinto33, Sara E Espinosa-Padilla10, Leticia Hernandez-Nieto10, Reem A Elfeky7, Tadashi Ariga25, Heike Toshio30, Figen Dogu6, Funda Cipe6, Renata Formankova34, M Enriqueta Nuñez-Nuñez31, Liliana Bezrodnik17, Jose Gonçalo Marques19, María I Pereira24, Viviana Listello24, Mary A Slatter8, Zohreh Nademi8, Danuta Kowalczyk14, Thomas A Fleisher35, Graham Davies5, Bénédicte Neven36, Sergio D Rosenzweig37. 1. Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md. 2. Biostatistics Research Branch, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md. 3. Pediatric Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran. 4. Department of Pediatrics, Federal University of São Paulo, São Paulo, Brazil. 5. Immunology Department, Great Ormond Street Hospital for Children, London, United Kingdom. 6. Department of Pediatric Immunology and Allergy, Ankara University Medical School, Ankara, Turkey. 7. Department of Pediatric Allergy and Immunology, Children's Hospital, Ain Shams University, Cairo, Egypt. 8. Paediatric Immunology Department, Great North Children's Hospital, Newcastle upon Tyne, United Kingdom. 9. Department of Clinical Immunology and Allergy, Medical Faculty of Masaryk University, St Anne's University Hospital, Brno, Czech Republic. 10. National Institute of Pediatrics, Mexico City Federal District, Mexico City, Mexico. 11. Department of Pediatrics, Faculty of Medicine, Kuwait University, Al-Sabah Hospital, Kuwait City, Kuwait. 12. Department of Immunology and Rheumatology, National Children's Hospital "Dr. Carlos Sáenz Herrera", San Jose, Costa Rica. 13. Department of Clinical Immunology, Center for Pediatric Hematology, Oncology and Immunology, Moscow, Russia. 14. Department of Clinical Immunology and Transplantology, Children's University Hospital, Krakow, Poland. 15. Pediatric Immunology Division, Uludag University Medical Faculty, Bursa, Turkey. 16. Primary Immunodeficiency Group, University of Antioquia, Medellin, Colombia. 17. Immunology Unit, Children's Hospital Ricardo Gutierrez, Buenos Aires, Argentina. 18. Faculty of Medicine of Ribeirão Preto, University of São Paulo, São Paulo, Brazil. 19. Primary Immunodeficiency Center, Lisbon Academic Center, Santa María Hospital, Lisbon, Portugal. 20. Cairo University, Cairo, Egypt. 21. Faculty of Medicine of ABC, Santo André, São Paulo, Brazil. 22. Department of Child Health, Sultan Qaboos University Hospital, Muscat, Oman. 23. Department of Pediatric Immunology-Allergy, Ondokuz Mayis University, Atakum-Samsun, Turkey. 24. Division of Allergy and Clinical Immunology, Santísima Trinidad Children's Hospital, Córdoba, Argentina. 25. Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan. 26. Tokyo Medical and Dental University, Tokyo, Japan. 27. Pediatric Immunology, Italian Hospital, Buenos Aires, Argentina. 28. Hyogo College of Medicine, Hyogo, Japan. 29. Department of Pediatrics and Adolescent Medicine, LKS Faculty of Medicine, University of Hong Kong, Hong Kong, China. 30. Department of Pediatrics, Kyoto University Hospital, Kyoto, Japan. 31. Department of Clinical Immunology and Allergology, Western National Medical Center, Guadalajara, Mexico. 32. Department of Pediatrics, Center for Investigation in Pediatrics-CIPED, University of Campinas Medical School-Unicamp, Campinas, SP, Brazil. 33. Federal University of Minas Gerais, Minas Gerais, Brazil. 34. Charles University, and University Hospital Motol, Prague, Czech Republic. 35. Immunology Service, DLM, CC, National Institutes of Health, Bethesda, Md. 36. Immunology-Hematology and Rheumatology Service, Necker Hospital, Paris, France. 37. Primary Immunodeficiency Clinic and Infectious Diseases Susceptibility Unit, LHD, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md. Electronic address: srosenzweig@cc.nih.gov.
Abstract
BACKGROUND: Severe combined immunodeficiency (SCID) is a syndrome characterized by profound T-cell deficiency. BCG vaccine is contraindicated in patients with SCID. Because most countries encourage BCG vaccination at birth, a high percentage of patients with SCID are vaccinated before their immune defect is detected. OBJECTIVES: We sought to describe the complications and risks associated with BCG vaccination in patients with SCID. METHODS: An extensive standardized questionnaire evaluating complications, therapeutics, and outcomes regarding BCG vaccination in patients given a diagnosis of SCID was widely distributed. Summary statistics and association analysis was performed. RESULTS: Data on 349 BCG-vaccinated patients with SCID from 28 centers in 17 countries were analyzed. Fifty-one percent of the patients had BCG-associated complications, 34% disseminated and 17% localized (a 33,000- and 400-fold increase, respectively, over the general population). Patients receiving early vaccination (≤1 month) showed an increased prevalence of complications (P = .006) and death caused by BCG-associated complications (P < .0001). The odds of experiencing complications among patients with T-cell numbers of 250/μL or less at diagnosis was 2.1 times higher (95% CI, 1.4-3.4 times higher; P = .001) than among those with T-cell numbers of greater than 250/μL. BCG-associated complications were reported in 2 of 78 patients who received antimycobacterial therapy while asymptomatic, and no deaths caused by BCG-associated complications occurred in this group. In contrast, 46 BCG-associated deaths were reported among 160 patients treated with antimycobacterial therapy for a symptomatic BCG infection (P < .0001). CONCLUSIONS: BCG vaccine has a very high rate of complications in patients with SCID, which increase morbidity and mortality rates. Until safer and more efficient antituberculosis vaccines become available, delay in BCG vaccination should be considered to protect highly vulnerable populations from preventable complications. Published by Mosby, Inc.
BACKGROUND: Severe combined immunodeficiency (SCID) is a syndrome characterized by profound T-cell deficiency. BCG vaccine is contraindicated in patients with SCID. Because most countries encourage BCG vaccination at birth, a high percentage of patients with SCID are vaccinated before their immune defect is detected. OBJECTIVES: We sought to describe the complications and risks associated with BCG vaccination in patients with SCID. METHODS: An extensive standardized questionnaire evaluating complications, therapeutics, and outcomes regarding BCG vaccination in patients given a diagnosis of SCID was widely distributed. Summary statistics and association analysis was performed. RESULTS: Data on 349 BCG-vaccinated patients with SCID from 28 centers in 17 countries were analyzed. Fifty-one percent of the patients had BCG-associated complications, 34% disseminated and 17% localized (a 33,000- and 400-fold increase, respectively, over the general population). Patients receiving early vaccination (≤1 month) showed an increased prevalence of complications (P = .006) and death caused by BCG-associated complications (P < .0001). The odds of experiencing complications among patients with T-cell numbers of 250/μL or less at diagnosis was 2.1 times higher (95% CI, 1.4-3.4 times higher; P = .001) than among those with T-cell numbers of greater than 250/μL. BCG-associated complications were reported in 2 of 78 patients who received antimycobacterial therapy while asymptomatic, and no deaths caused by BCG-associated complications occurred in this group. In contrast, 46 BCG-associated deaths were reported among 160 patients treated with antimycobacterial therapy for a symptomatic BCG infection (P < .0001). CONCLUSIONS: BCG vaccine has a very high rate of complications in patients with SCID, which increase morbidity and mortality rates. Until safer and more efficient antituberculosis vaccines become available, delay in BCG vaccination should be considered to protect highly vulnerable populations from preventable complications. Published by Mosby, Inc.
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