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Vacinas vivas em crianças com síndrome de DiGeorge/Deleção 22q11.2

dc.contributor.authorMiranda, Mariana
dc.contributor.authorMartins, Andreia Teixeira
dc.contributor.authorCarvalho, Sara
dc.contributor.authorSerra-Caetano, Ana
dc.contributor.authorEsteves, Isabel
dc.contributor.authorMarques, José Gonçalo
dc.date.accessioned2020-10-26T13:46:24Z
dc.date.available2020-10-26T13:46:24Z
dc.date.issued2019-07
dc.descriptionCopyright © Ordem dos Médicos 2019pt_PT
dc.description.abstractChildren with DiGeorge syndrome/chromosome 22q11.2 deletion syndrome might have a variable degree of immunodeficiency, which may limit the use of live vaccines. The aim of this study was to review the adverse effects of live vaccines and possible relation with immune status in patients with DiGeorge Syndrome/partial 22q11.2 deletion syndrome.Material e Métodos: Foi realizado um estudo retrospetivo por revisão dos processos clínicos das crianças com deleção do cromossoma 22q11.2 e fenótipo de síndrome de DiGeorge, seguidos num centro de referência de imunodeficiências primárias. Foi realizada colheita de dados, incluindo: características demográficas; história médica; historial de vacinação com vacinas vivas; contagem de linfócitos T-CD4+ e respostas proliferativas linfocitárias a antigénios e mitogénios; reações adversas; falências vacinais. Resultados: Foram incluídas 23 crianças com síndrome de DiGeorge/deleção 22q11.2, 65,2% do sexo masculino e idade média de diagnóstico de 11,3 meses. Destas, 18 crianças (78%) receberam a vacina bacillus Calmette-Guérin: todas com evidência de atividade tímica; três apresentaram linfopénia T-CD4+ moderada e respostas proliferativas linfocitárias anormais; uma com respostas proliferativas linfocitárias anormais para mitogénios, quatro para derivado de proteína purificada e uma para toxóide tetânico. A vacina tríplice contra o sarampo, parotidite e rubéola foi administrada a 15 crianças, três com imunossupressão moderada e respostas proliferativas linfocitárias anormais. A vacina viva atenuada contra poliomielite foi administrada a quatro crianças sem imunossupressão e a vacina contra o rotavírus a três crianças, uma com imunossupressão moderada. Não foram reportadas reações adversas. Discussão: Estes dados estão de acordo com as conclusões de outros estudos internacionais. Conclusão: Na nossa amostra, as vacinas vivas atenuadas foram bem toleradas, incluindo em crianças com linfopénia T-CD4+ moderada e com respostas proliferativas linfocitárias a antigénios/mitogénios anormais.pt_PT
dc.description.abstractIntroduction: Children with DiGeorge syndrome/chromosome 22q11.2 deletion syndrome might have a variable degree of immunodeficiency, which may limit the use of live vaccines. The aim of this study was to review the adverse effects of live vaccines and possible relation with immune status in patients with DiGeorge Syndrome/partial 22q11.2 deletion syndrome. Material and Methods: Retrospective study with analysis of the clinical records of children with chromosome 22q11.2 deletion syndrome and DiGeorge syndrome phenotype, followed in a Primary Immunodeficiency center. Data were collected on: demographic characteristics; medical and vaccination history with live vaccines; T-CD4+ lymphocyte counts and lymphocyte proliferative responses to antigens and mitogens; adverse reactions; vaccine failure. Results: Twenty three children with DiGeorge syndrome/22q11.2 deletion syndrome were included, 65.2% male, with average age at diagnosis of 11.3 months. Eighteen children (78%) received bacillus Calmette-Guérin vaccine: all with evidence of thymic activity; three presented moderate T-CD4+ lymphopenia and abnormal lymphocyte proliferative responses; one had abnormal lymphocyte proliferative responses for mitogens, four for purified protein derivative and one for tetanus toxoid. Measles, mumps and rubella vaccine was administered to 15 children, three of them with moderate immunosuppression and abnormal lymphocyte proliferative responses. Live attenuated polio vaccine was administered to 4 children without immunosuppression and the rotavirus vaccine to three children, one with moderate immunosuppression. No significant adverse reactions were reported. Discussion: These data are in line with the findings of other international studies. Conclusion: In our sample, live vaccines were well-tolerated, even in children with moderate T-CD4+ lymphopenia and abnormal lymphocyte proliferative responses to antigens/mitogens.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationActa Med Port 2019 Jul–Aug;32(7–8):514–519pt_PT
dc.identifier.doi10.20344/amp.9802pt_PT
dc.identifier.eissn1646-0758
dc.identifier.issn0870-399X
dc.identifier.urihttp://hdl.handle.net/10451/44661
dc.language.isoporpt_PT
dc.peerreviewedyespt_PT
dc.publisherActa Médica Portuguesapt_PT
dc.relation.publisherversionhttps://www.actamedicaportuguesa.compt_PT
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/pt_PT
dc.subjectChildpt_PT
dc.subjectDiGeorge syndromept_PT
dc.subjectChromosome deletionpt_PT
dc.subjectViral Vaccines/adverse effectspt_PT
dc.subjectVaccines, attenuated/adverse effectspt_PT
dc.subjectChromosome disorderspt_PT
dc.subjectChromosomes, human, Ppir 22pt_PT
dc.titleVacinas vivas em crianças com síndrome de DiGeorge/Deleção 22q11.2pt_PT
dc.title.alternativeLive vaccine in children with DiGeorge/22q11.2 Deletion syndromept_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.endPage519pt_PT
oaire.citation.issue7-8pt_PT
oaire.citation.startPage514pt_PT
oaire.citation.titleActa Médica Portuguesapt_PT
oaire.citation.volume32pt_PT
person.familyNameMiranda
person.familyNameCaetano
person.givenNameMariana
person.givenNameAna
person.identifier.ciencia-idCE1E-9913-5BFB
person.identifier.orcid0000-0003-3831-8973
person.identifier.orcid0000-0002-1626-4364
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublication9aea6e6b-12f9-40f2-bd30-cbff15175177
relation.isAuthorOfPublication6e3ee8ec-15fa-4624-9d9d-14bf01a95ca9
relation.isAuthorOfPublication.latestForDiscovery9aea6e6b-12f9-40f2-bd30-cbff15175177

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