Authors: Gómez Raccio, Andrea C.; Orellana, Julio Cesar; Liberatore, Diana; Bezrodnik, Liliana; Marciano, Beatriz E.; Huang, Chiung Yu; Joshi, Gyan; Rezaei, Nima; Costa Carvalho, Beatriz; Cunha, Luciana; Pinto, Jorge A.; Espinosa Padilla, Sara E.; Hernandez Nieto, Leticia; Elfeky, Reem A.; Ariga, Tadashi; Toshio, Heike; Dogu, Figen; Cipe, Funda; Formankova, Renata; Nuñez Nuñez, M. Enriqueta; Gonçalo Marques, Jose; Pereira, María I.; Listello, Viviana; Slatter, Mary A.; Nademi, Zohreh; Kowalczyk, Danuta; Fleisher, Thomas A.; Davies, Graham; Neven, Bénédicte; Rosenzweig, Sergio D.
Publication Date: 2014.
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.
Author affiliation: Gómez Raccio, Andrea C.. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños ; Argentina
Author affiliation: Orellana, Julio Cesar. Provincia de Córdoba. Hospital de Niños de la Santísima Trinidad. División de Alergia e Inmunología Clínica; Argentina
Author affiliation: Liberatore, Diana. Hospital Italiano; Argentina
Author affiliation: Bezrodnik, Liliana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños ; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Author affiliation: Marciano, Beatriz E.. National Institutes of Health; Estados Unidos
Author affiliation: Huang, Chiung Yu. National Institutes of Health; Estados Unidos
Author affiliation: Joshi, Gyan. National Institutes of Health; Estados Unidos
Author affiliation: Rezaei, Nima. Teheran University of Medical Sciences. Children's Medical Center Hospital. Pediatric Center of Excellence; Irán
Author affiliation: Costa Carvalho, Beatriz. Federal University of São Paulo; Brasil
Author affiliation: Cunha, Luciana. Federal University of Minas Gerais; Brasil
Author affiliation: Pinto, Jorge A.. Federal University of Minas Gerais; Brasil
Author affiliation: Espinosa Padilla, Sara E.. Secretaría de Salud. Instituto Nacional de Pediatría; México
Author affiliation: Hernandez Nieto, Leticia. Secretaría de Salud. Instituto Nacional de Pediatría; México
Author affiliation: Elfeky, Reem A.. Ain Shams University; Egipto
Author affiliation: Ariga, Tadashi. Hokkaido University Graduate School of Medicine; Japón
Author affiliation: Toshio, Heike. Kyoto University Hospital; Japón
Author affiliation: Dogu, Figen. Ankara University Medical School; Turquía
Author affiliation: Cipe, Funda. Ankara University Medical School; Turquía
Author affiliation: Formankova, Renata. Charles University; República Checa. University Hospital Motol; República Checa
Author affiliation: Nuñez Nuñez, M. Enriqueta. Western National Medical Center; México
Author affiliation: Gonçalo Marques, Jose. Santa María Hospital. Lisbon Academic Center; Portugal
Author affiliation: Pereira, María I.. Provincia de Córdoba. Hospital de Niños de la Santísima Trinidad. División de Alergia e Inmunología Clínica; Argentina
Author affiliation: Listello, Viviana. Provincia de Córdoba. Hospital de Niños de la Santísima Trinidad. División de Alergia e Inmunología Clínica; Argentina
Author affiliation: Slatter, Mary A.. Great North Children's Hospital; Reino Unido
Author affiliation: Nademi, Zohreh. Great North Children's Hospital; Reino Unido
Author affiliation: Kowalczyk, Danuta. Children's University Hospital. Department of Clinical Immunology and Transplantology; Polonia
Author affiliation: Fleisher, Thomas A.. National Institutes of Health; Estados Unidos
Author affiliation: Davies, Graham. Great Ormond Street Hospital for Children; Reino Unido
Author affiliation: Neven, Bénédicte. Necker Hospital. Immunology-Hematology and Rheumatology Service; Francia
Author affiliation: Rosenzweig, Sergio D.. National Institute of Health. National Institute of Allergy and Infectious. Laboratory of Host Defenses. Primary Immunodeficiency Clinic and Infectious Diseases Susceptibility Unit; Estados Unidos
Keywords: Hematopoietic stem cell transplant; IL-2 receptor g; Recombination-activating gene; Severe combined immunodeficiency; Primary immunodeficiency; Vaccine; BCG; Immune reconstitution syndrome; Mycobacteria; Newborn screening; Medicina Critica y de Emergencia; Medicina Clínica; CIENCIAS MÉDICAS Y DE LA SALUD.
Repository: CONICET Digital (CONICET). Consejo Nacional de Investigaciones Científicas y Técnicas
Publication Date: 2007.
Symptomatic hypogammaglobulinaemia in children younger than 2 years of age was studied to rule out a primary immunodeficiency. Thirty-four patients were referred to the Immunology Service to study the hypogammaglobulinaemia- associated clinical picture. Food allergy was documented in 10 patients by personal and familial history, presence of specific immunoglobulin E (IgE) and elevated total serum IgE levels. Coeliac disease and human immunodeficiency virus infection were also ruled out. Protein loss through stools was assessed by clearance of α1-antitrypsin (AAT). Serum immunoglobulin levels were determined by nephelometry and functional antibodies were studied by enzyme-linked immunosorbent assay. The cellular immune response was assessed by in vitro lymphocyte proliferation in response to mitogens and cell subsets were analysed by flow cytometry. In five patients of the 10 patients we suspected a protein loss through the mucosa. Four of these five patients showed an increased AAT and the other showed an extensive cutaneous lesion. Immunological studies revealed normal antibody function, in vitro lymphoproliferative responses and cell numbers in four of the 5 patients. One patient showed abnormally low numbers of CD4+ T cells as well as a defective proliferative response to mitogens. After diagnosis of cow milk allergy, milk was replaced with infant milk formula containing hydrolysed proteins. Recovery of immunoglobulin values and clinical resolution were achieved. Hypogammaglobulinaemia during early childhood in some children may be secondary to cow milk allergy, and immunoglobulins and cells may leak through the inflamed mucosa. Resolution of symptoms as well as normalization of immunoglobulin values may be easily achieved by avoidance of the offending allergen.
Laboratorio de Investigaciones del Sistema Inmune
Keywords: Ciencias Exactas.
Repository: SEDICI (UNLP). Universidad Nacional de La Plata