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dc.contributor.authorMazzucchelli, J. T. L. [UNIFESP]
dc.contributor.authorBonfim, C.
dc.contributor.authorCastro, G. G.
dc.contributor.authorCondino-Neto, A. A.
dc.contributor.authorCosta, N. M. X.
dc.contributor.authorCunha, L.
dc.contributor.authorDantas, E. O.
dc.contributor.authorDantas, V. M.
dc.contributor.authorMoraes-Pinto, M. I. de [UNIFESP]
dc.contributor.authorFernandes, J. F.
dc.contributor.authorGoes, H. C.
dc.contributor.authorGoudouris, E.
dc.contributor.authorGrumach, A. S.
dc.contributor.authorGuirau, L. M. B.
dc.contributor.authorKuntze, G.
dc.contributor.authorMallozzi, M. C.
dc.contributor.authorMonteiro, F. P.
dc.contributor.authorMoraes, L. S. L.
dc.contributor.authorNudelman, V.
dc.contributor.authorPinto, J. A.
dc.contributor.authorRizzo, M. C. V.
dc.contributor.authorPorto-Neto, A. C.
dc.contributor.authorRoxo-Junior, P.
dc.contributor.authorRuiz, M.
dc.contributor.authorRullo, V. E. V.
dc.contributor.authorSeber, A. [UNIFESP]
dc.contributor.authorTakano, O. A.
dc.contributor.authorTavares, F. S.
dc.contributor.authorToledo, E.
dc.contributor.authorVilela, M. M. S.
dc.contributor.authorCosta-Carvalho, B. T. [UNIFESP]
dc.date.accessioned2018-06-15T18:02:42Z
dc.date.available2018-06-15T18:02:42Z
dc.date.issued2014-01-01
dc.identifierhttp://www.jiaci.org/summary/vol24-issue3-num1120
dc.identifier.citationJournal Of Investigational Allergology And Clinical Immunology. Barcelona: Esmon Publicidad S A, v. 24, n. 3, p. 184-191, 2014.
dc.identifier.issn1018-9068
dc.identifier.urihttp://repositorio.unifesp.br/11600/44461
dc.description.abstractBackground: Severe combined immunodeficiency (SCID) is one of the most severe forms of primary immunodeficiency. The objectives of this study were to analyze the diagnosis, treatment, and prognosis of SCID in Brazil and to document the impact of BCG vaccine.Methods: We actively searched for cases by contacting all Brazilian referral centers.Results: We contacted 23 centers and 70 patients from 65 families. Patients were born between 1996 and 2011, and 49 (70%) were male. More than half (39) of the diagnoses were made after 2006. Mean age at diagnosis declined from 9.7 to 6.1 months (P=.058) before and after 2000, respectively, and mean delay in diagnosis decreased from 7.9 to 4.2 months (P=.009). Most patients (60/70) were vaccinated with BCG before the diagnosis, 39 of 60 (65%) had complications related to BCG vaccine, and the complication was disseminated in 29 of 39 (74.3%). Less than half of the patients (30, 42.9%) underwent hematopoietic stem cell transplantation (HSCT). Half of the patients died (35, 50%), and 23 of these patients had not undergone HSCT. Disseminated BCG was the cause of death, either alone or in association with other causes, in 9 of 31 cases (29%, no data for 4 cases).Conclusions: In Brazil, diagnosis of SCID has improved over the last decade, both in terms of the number of cases and age at diagnosis, although a much higher number of cases had been expected. Mortality is higher than in developed countries. Complications of BCG vaccine are an important warning sign for the presence of SCID and account for significant morbidity during disease progression.en
dc.description.sponsorshipJeffrey Modell Foundation
dc.format.extent184-191
dc.language.isoeng
dc.publisherEsmon Publicidad S A
dc.relation.ispartofJournal Of Investigational Allergology And Clinical Immunology
dc.rightsAcesso aberto
dc.subjectPrimary immunodeficiencyen
dc.subjectSevere combined immunodeficiencyen
dc.subjectBCG vaccineen
dc.subjectBCG complicationsen
dc.subjectMycobacterium bovisen
dc.subjectStem cell transplantationen
dc.titleSevere Combined Immunodeficiency in Brazil: Management, Prognosis, and BCG-Associated Complicationsen
dc.typeArtigo
dc.contributor.institutionUniversidade Federal de São Paulo (UNIFESP)
dc.contributor.institutionUniv Fed Parana
dc.contributor.institutionUniversidade Federal da Bahia (UFBA)
dc.contributor.institutionUniversidade de São Paulo (USP)
dc.contributor.institutionPontifical Catholic Univ Goias
dc.contributor.institutionUniversidade Federal de Minas Gerais (UFMG)
dc.contributor.institutionDiadema Hosp
dc.contributor.institutionUniv Fed Rio Grande do Norte
dc.contributor.institutionAlbert Einstein Hosp
dc.contributor.institutionPrivate Off
dc.contributor.institutionUniversidade Federal do Rio de Janeiro (UFRJ)
dc.contributor.institutionFac Med ABC
dc.contributor.institutionDarcy Vargas Childrens Hosp
dc.contributor.institutionVidas Hosp
dc.contributor.institutionAsa Sul Reg Hosp
dc.contributor.institutionMenino Jesus Hosp
dc.contributor.institutionUniv Passo Fundo
dc.contributor.institutionBase Hosp
dc.contributor.institutionS Jose do Rio Preto Med Sch
dc.contributor.institutionUniversidade Estadual de Campinas (UNICAMP)
dc.description.affiliationUniv Fed Sao Paulo, Dept Pediat, Sao Paulo, Brazil
dc.description.affiliationUniv Fed Parana, Bone Marrow Transplant Unit, BR-80060000 Curitiba, Parana, Brazil
dc.description.affiliationUniv Fed Bahia, BR-41170290 Salvador, BA, Brazil
dc.description.affiliationUniv Sao Paulo, Inst Biomed Sci, Sao Paulo, Brazil
dc.description.affiliationPontifical Catholic Univ Goias, Goiania, Go, Brazil
dc.description.affiliationUniv Fed Minas Gerais, Belo Horizonte, MG, Brazil
dc.description.affiliationDiadema Hosp, Sao Paulo, Brazil
dc.description.affiliationUniv Fed Rio Grande do Norte, BR-59072970 Natal, RN, Brazil
dc.description.affiliationAlbert Einstein Hosp, Sao Paulo, Brazil
dc.description.affiliationPrivate Off, Macapa, Amapa, Brazil
dc.description.affiliationUniv Fed Rio de Janeiro, Rio De Janeiro, Brazil
dc.description.affiliationUniv Sao Paulo, Dept Dermatol, Sao Paulo, Brazil
dc.description.affiliationFac Med ABC, Santo Andre, Brazil
dc.description.affiliationDarcy Vargas Childrens Hosp, Sao Paulo, Brazil
dc.description.affiliationVidas Hosp, Sao Paulo, Brazil
dc.description.affiliationAsa Sul Reg Hosp, Brasilia, DF, Brazil
dc.description.affiliationMenino Jesus Hosp, Sao Paulo, Brazil
dc.description.affiliationUniv Passo Fundo, Casca, RS, Brazil
dc.description.affiliationUniv Sao Paulo, Fac Med Ribeirao Preto, Sao Paulo, Brazil
dc.description.affiliationCtr Univ Lusiada, Sch Med, Sao Paulo, Brazil
dc.description.affiliationUniv Fed Sao Paulo, GRAAC, Inst Pediat Oncol, Sao Paulo, Brazil
dc.description.affiliationBase Hosp, Brasilia, DF, Brazil
dc.description.affiliationS Jose do Rio Preto Med Sch, Sao Paulo, Brazil
dc.description.affiliationUniv Estadual Campinas, Fac Med Sci, Sao Paulo, Brazil
dc.description.affiliationUnifespUniv Fed Sao Paulo, Dept Pediat, Sao Paulo, Brazil
dc.description.affiliationUnifespUniv Fed Sao Paulo, GRAAC, Inst Pediat Oncol, Sao Paulo, Brazil
dc.description.sourceWeb of Science
dc.identifier.wosWOS:000339367800007


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