Eculizumab interruption in atypical hemolytic uremic syndrome due to shortage: analysis of a Brazilian cohort

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MetadadosDescriçãoIdioma
Autor(es): dc.contributorUniversidade Estadual Paulista (Unesp)-
Autor(es): dc.contributorHospital BP-a Beneficência Portuguesa de São Paulo-
Autor(es): dc.contributorFresenius Medical Care-Unidade Perdizes-
Autor(es): dc.contributorHospital de Urgências Governador Otávio Lage de Siqueira (HUGOL)-
Autor(es): dc.contributorUniversidade Federal do Rio Grande do Norte-
Autor(es): dc.contributorInstituto Hospital de Base-
Autor(es): dc.contributorHospital Federal de Bonsucesso-Serviço de Nefrologia e Transplante-
Autor(es): dc.contributorUniversidade de São Paulo (USP)-
Autor(es): dc.contributorUniversidade Federal do Espírito Santo (UFES)-
Autor(es): dc.contributorUniversidade Federal de São Paulo (UNIFESP)-
Autor(es): dc.contributorUniversidade Estadual de Campinas (UNICAMP)-
Autor(es): dc.creatorNeto, Miguel Ernandes [UNESP]-
Autor(es): dc.creatorde Moraes Soler, Lucas [UNESP]-
Autor(es): dc.creatorVasconcelos, Halita Vieira Gallindo-
Autor(es): dc.creatorNga, Hong Si [UNESP]-
Autor(es): dc.creatorBravin, Ariane Moyses [UNESP]-
Autor(es): dc.creatorBorges, Julio Cesar Andriotti-
Autor(es): dc.creatorGonçalves, Rodrigo Costa-
Autor(es): dc.creatorVon Kriiger, Rodrigo Brum-
Autor(es): dc.creatorQuinino, Raquel Martins-
Autor(es): dc.creatorde Mello Santana, Viviane Brandão Bandeira-
Autor(es): dc.creatorde Holanda, Maria Izabel-
Autor(es): dc.creatorVaisbich, Maria Helena-
Autor(es): dc.creatorNaseri, Alice Pignaton-
Autor(es): dc.creatorKirsztajn, Gianna Mastroianni-
Autor(es): dc.creatorPalma, Lilian Monteiro Pereira-
Autor(es): dc.creatorAndrade, Luís Gustavo Modelli [UNESP]-
Data de aceite: dc.date.accessioned2022-02-22T00:44:54Z-
Data de disponibilização: dc.date.available2022-02-22T00:44:54Z-
Data de envio: dc.date.issued2021-06-25-
Data de envio: dc.date.issued2021-06-25-
Data de envio: dc.date.issued2020-12-31-
Fonte completa do material: dc.identifierhttp://dx.doi.org/10.1007/s40620-020-00920-z-
Fonte completa do material: dc.identifierhttp://hdl.handle.net/11449/205648-
Fonte: dc.identifier.urihttp://educapes.capes.gov.br/handle/11449/205648-
Descrição: dc.descriptionBackground: The risk of eculizumab therapy discontinuation in patients with atypical hemolytic uremic syndrome (aHUS) is unclear. The main objective of this study was to analyze the risk of aHUS relapse after eculizumab interruption due to drug shortage in Brazil. Methods: We screened all the registered dialysis centers in Brazil (n = 800), willing to participate in the aHUS Brazilian shortage cohort, through electronic mail and formal invitation by the Brazilian Society of Nephrology. We included patients with aHUS whose eculizumab therapy underwent unplanned discontinuation for at least 30 days between January 1st, 2016 and December 31st, 2019 during the maintenance phase of treatment. Relapse was defined by the development of thrombocytopenia, hemolytic anemia, acute kidney injury or thrombotic microangiopathy (TMA) in a kidney biopsy. Results: We analyzed 25 episodes of exposure to risk of relapse, from 24 patients. Median age was 33 (6–53) years, 18 (72%) were female, 9 (36%) had a functioning renal graft, 5 (20%) were undergoing dialysis. CFH variant was found in 8 (32%) episodes. There were 11 relapses. The risk of relapse was 34%, 44.5% and 58% at 114, 150 and 397 days, respectively. No baseline variable was related to relapse in Cox multivariate analysis, including CFH variant. Conclusions: In this study, the cumulative incidence of aHUS relapse at 397 days was 58% after eculizumab interruption. The presence of complement variant does not seem to be associated with a higher relapse rate. The eculizumab interruption was deemed not safe, considering that the rate of relapse was high.-
Descrição: dc.descriptionDepartment of Internal Medicine São Paulo State University (UNESP)-
Descrição: dc.descriptionHospital BP-a Beneficência Portuguesa de São Paulo-
Descrição: dc.descriptionDepartamento de Nefrologia da Santa Casa de São Carlos-
Descrição: dc.descriptionFresenius Medical Care-Unidade Perdizes-
Descrição: dc.descriptionHospital de Urgências Governador Otávio Lage de Siqueira (HUGOL)-
Descrição: dc.descriptionHospital Universitário Onofre Lopes Universidade Federal do Rio Grande do Norte-
Descrição: dc.descriptionInstituto Hospital de Base-
Descrição: dc.descriptionHospital Federal de Bonsucesso-Serviço de Nefrologia e Transplante-
Descrição: dc.descriptionInstituto da Criança-University of São Paulo-
Descrição: dc.descriptionSetor de Nefrologia da Unidade de Gestão de Transplantes da Universidade Federal do Espírito Santo-
Descrição: dc.descriptionDepartamento de Medicina (Nefrologia) da Universidade Federal de São Paulo (UNIFESP)-
Descrição: dc.descriptionNefrologia Pediátrica-Departamento de Pediatria da Universidade Estadual de Campinas (UNICAMP)-
Descrição: dc.descriptionDepartment of Internal Medicine São Paulo State University (UNESP)-
Idioma: dc.languageen-
Relação: dc.relationJournal of Nephrology-
???dc.source???: dc.sourceScopus-
Palavras-chave: dc.subjectAtypical hemolytic uremic syndrome-
Palavras-chave: dc.subjectComplement inactivating agents-
Palavras-chave: dc.subjectEculizumab-
Título: dc.titleEculizumab interruption in atypical hemolytic uremic syndrome due to shortage: analysis of a Brazilian cohort-
Tipo de arquivo: dc.typelivro digital-
Aparece nas coleções:Repositório Institucional - Unesp

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