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

BACKGROUND: 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.

Uma perspectiva mundial do carcinoma de células escamosas de pele / A world perspective of the squamous cell carcinoma of the skin

O Carcinoma de Células Escamosas (SCC) é a segunda neoplasia maligna cutânea mais freqüente, causada primordialmente pela exposição crônica e excessiva à radiação ultravioleta. O objetivo deste trabalho é discutir o perfil epidemiológico global do SCC através de uma revisão sistemática. Foi realizada uma busca na base de dados PUBMED com a utilização dos termos: squamous cells skin cancer, epidemiology squamous cells skin cancer, penile squamous cell carcinoma, squamous cell carcinoma of the penis, vulva SCC epidemiology e depletion of ozone layer. Outra busca foi realizada na base de dados SCIENCE DIRECT, com os termos: epidemiology of non-melanoma skin cancer, epidemiology of squamous cells carcinoma, squamous cells carcinoma, skin cancer, e, por fim, o termo UV radiation. No total, 19 artigos foram selecionados, sendo considerados aqueles publicados a partir de 2000. A revisão permitiu verificar que estudos em países de todos os continentes mostram um número crescente de casos, o que configura um grave problema de saúde pública mundial.