Detailing the epidemiological and clinical characteristics of chronic lymphocytic leukaemia in Portugal—Results from a population-based cancer registry cohort study

Publisher Copyright: © 2021 Cardoso Borges et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.Background Chronic lymphocytic leukaemia (CLL) is the most common leukaemia among adults in western countries. Considering the increasing incidence and prevalence of this condition, it is highly relevant to better characterise these patients in Portugal, where data is still scarce. Methods To determine incidence, clinical presentation, survival and second malignancies, a population-based historical cohort study was conducted. Cases of interest were identified through the South Region Cancer Registry database and additional data sources. Patients aged ≥18 years, with a confirmed diagnosis of CLL or small lymphocytic lymphoma between January 1st, 2013 and December 31st, 2014 were included. Patients were followed-up until death or cut-off date (December 31st, 2019). Results A total of 496 patients were included and median follow-up time was 5.46 years. Crude incidence rates were 5.03 and 5.22 per 100,000 inhabitants for 2013 and 2014, respectively, and age-adjusted incidence rates were 3.18:100,000 European population for 2013 and 3.35:100,000 European population for 2014. Median age at diagnosis was 71 years and the male/female ratio was 1.40. The majority of patients had leukemic presentation of the disease (86.09%), was diagnosed in Binet stage A (75.58%) and did not present B symptoms (84.01%), anaemia (haemoglobin ≤10g/dL; 90.63%) nor thrombocytopenia (platelet count ≤100 000/μL; 91.73%). Five-year overall survival (OS) rate was 70.53% (95%CI 66.31–74.34) and age, lactate dehydrogenase, Binet stage and a ≥5 Charlson comorbidity index score were independently associated with OS. Standardised-incidence ratios for any second malignancy and cutaneous squamous cell carcinoma were 1.59 (95%CI 1.19–2.08) and 10.15 (95%CI 6.28–15.51), respectively. Conclusion Incidence, clinical presentation and survival of CLL Portuguese patients are similar to those reported for other western countries. The increased risk of second malignancies raises concerns and needs adequate clinical watchfulness.publishersversionpublishe

Intensive monitoring studies for assessing medicines : a systematic review

Copyright © 2019 Torre, Cary, Borges, Ferreira, Alarcão, Leufkens, Costa and Martins. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.Introduction: Intensive monitoring (IM) is one of the methods of post-marketing active surveillance based upon event monitoring, which has received interest in the current medicines regulatory landscape. For a specific period of time, IM involves primary data collection and is actively focused on gathering longitudinal information, mainly safety, since the first day of drug use. Objectives: To describe IM systems and studies' data published over 11-years period (2006-2016). Specifically, we reviewed study population/event surveillance, methodological approaches, limitations, and its applications in the real-world evidence generation data. Methods: We completed a systematic search of MEDLINE and EMBASE to identify studies published from 2006 to 2016, that used IM methodology. We extracted data using a standardized form and results were analyzed descriptively. The methodological quality of selected studies was assessed using the modified Downs and Black checklist. Results: From 1,400 screened citations, we identified 86 papers, corresponding to 69 different studies. Seventy percent of reviewed studies corresponded to established IM systems, of which, more than half were prescription event monitoring (PEM) and modified-PEM. Among non-established IM systems, vaccines were the most common studied drugs (n = 14). The median cohort size ranged from 488 (hospitals) to 10,479 (PEM) patients. Patients and caregivers were the event data source in 39.1% of studies. The mean overall quality score was similar between established and non-established IM. Conclusions: Over the study period, IM studies were implemented in 26 countries with different maturity levels of post-marketing surveillance systems. We identified two major limitations: only 20% of studies were conducted at hospital-level, which is a matter of concern, insofar as healthcare systems are facing a lack of access to new medicines at ambulatory care level. Additionally, IM access to data of drug exposure cohorts, either at identification or at follow-up stages, could somehow constitute a barrier, given the complexity of managerial, linkable, and privacy data issues.The publication fee was supported by the Center for Health Evaluation and Research (CEFAR), National Association of Pharmacies, Lisbon, Portugal.info:eu-repo/semantics/publishedVersio

Results from a population-based cohort study

Funding Information: We have read the journal's policy and the authors of this manuscript have the following competing interests: ARF reports travel grants from Roche and advisory board fees from Daiichi Sankyo, Gilead, Merck Sharp & Dohme, Novartis and Roche, outside the submitted work. DMB reports travel grants from LEO Farmacêuticos, Merck Sharp & Dohme, Ipsen, Janssen, Roche, and Novartis, advisory board fees from Janssen, Pfizer, Merck Sharp & Dohme, Angelini, AstraZeneca, and Novartis, and institutional grants from F. Hoffmann-La Roche, outside the submitted work. The other authors have declared that no competing interests exist. Funding Information: The authors acknowledge the RON network that cooperated in providing up-to-date information on cases diagnosed and treated with the drug of interest (participating institutions: Centro Hospitalar Universit?rio de S?o Jo?o, Centro Hospitalar Universit?rio Lisboa Norte, Centro Hospitalar Universit?rio do Algarve, Hospital de Braga, Centro Hospitalar e Universit?rio de Coimbra, Centro Hospitalar de Tr?s-os-Montes e Alto Douro, Hospital Central do Funchal, Centro Hospitalar de Vila Nova de Gaia/Espinho, Centro Hospitalar Lisboa Ocidental, Hospital Garcia de Orta, Centro Hospitalar Universit?rio Lisboa Central, Hospital Distrital de Santar?m, Centro Hospitalar de Entre o Douro e Vouga, Hospital da Senhora da Oliveira Guimar?es, Centro Hospitalar de Set?bal, Centro Hospitalar e Universit?rio do Porto, Centro Hospitalar Tondela Viseu, Hospital do Esp?rito Santo de ?vora, Centro Hospitalar Barreiro Montijo, Hospital Beatriz ?ngelo, Hospital do Santo Esp?rito da Ilha Terceira, Hospital do Divino Esp?rito Santo de Ponta Delgada, Hospital Pedro Hispano ? ULS Matosinhos, Hospital do Litoral Alentejano ? Santiago do Cac?m ? ULS Litoral Alentejano, Centro Hospitalar do Oeste, Centro Hospitalar M?dio Tejo, Hospital Jos? Joaquim Fernandes ? Beja ? ULS Baixo Alentejo, Centro Hospitalar Universit?rio da Cova da Beira, Centro Cl?nico Champalimaud, Hospitais CUF, Hospitais da Luz, Hospitais dos Lus?adas, Hospital Particular do Algarve). Publisher Copyright: © 2022 The AuthorsBackground: Real-world (RW) data may provide valuable information on the effectiveness and safety of medicines, which is particularly relevant for clinicians, patients and third-party payers. Evidence on the effectiveness of palbociclib plus fulvestrant is scarce, which highlights the need of additional studies. The aim of this study was to evaluate the effectiveness of palbociclib plus fulvestrant in advanced breast cancer (ABC). Materials and methods: We conducted a population-based retrospective cohort study and cases of interest were identified through the Portuguese National Cancer Registry database and additional data sources. Patients aged≥18 years, diagnosed with ABC and exposed to palbociclib plus fulvestrant between May 31, 2017 and March 31, 2019 were included. Patients were followed-up until death or cut-off date (February 28, 2021). Primary outcome was rw-progression-free survival (rwPFS). Secondary outcomes were rw-overall survival (rwOS), rw-time to palbociclib failure (rwTPF) and rw-time to next treatment (rwTTNT). Results: A total of 210 patients were included. Median age was 58 years (range 29–83) and 99.05% were female. Median follow-up time was 23.22 months and, at cut-off date, treatment had been discontinued in 189 patients, mainly due to disease progression (n = 152). Median rwPFS was 7.43 months (95% confidence interval [CI] 6.28–9.05) and 2-year rwPFS was 16.65% (95%CI 11.97–22.00). Median rwOS was 24.70 months (95%CI 21.58–29.27), median rwTPF was 7.5 months (95%CI 6.51–9.08) and median rwTTNT was 11.74 months (95%CI 10.33–14.08). Conclusion: Palbociclib plus fulvestrant seems an effective treatment for ABC in real-world context. Compared to registrations studies, rwPFS and rwOS were shorter in real-life setting.publishersversionpublishe

Manifestações psiquiátricas na reumatologia: uma revisão sistemática / Psychiatric manifestations in rheumatology: a systematic review

Doenças reumatológicas, são doenças crônicas que geralmente apresentam múltiplas causas. São representadas principalmente pela artrite reumatoide e lúpus eritematoso sistêmico. Sabe-se, no entanto, que existe uma ligação entre os processos autoimunes subjacentes às doenças reumáticas e aos transtornos mentais. O objetivo desta revisão foi avaliar as manifestações psiquiátricas em pacientes com condições reumatológicas. Uma busca sistemática na literatura foi realizada, nos portais BIREME e PubMed de estudos publicados nos últimos 10 anos. A busca foi realizada utilizando descritores em português e seus correspondentes em inglês: “artrite reumatoide”, “lúpus eritematoso sistêmico”, “esclerose sistêmica” e “síndrome de Sjögren” e “manifestações psiquiátricas”. Os artigos que foram incluídos após leitura na íntegra, tiveram seus dados coletados em instrumento padronizado e elaborado antes do início da busca. foram identificados e adicionados através da estratégia de busca 14 artigos. Ansiedade, depressão, incapacidade cognitiva e insônia estão entre as manifestações psiquiátricas mais prevalentes. A frequente presença de manifestações psiquiátricas na reumatologia acende um alerta entre os profissionais para priorizar a qualidade de vida de seus pacientes, reduzindo suas limitações

Educação Bilíngue nas Políticas Educacionais e Linguísticas para Surdos: discursos e estratégias de governamento

Resumo: Neste artigo, apresento algumas problematizações sobre os discursos e as estratégias de governamento utilizados pelas atuais políticas educacionais e linguísticas para surdos. Argumento que tanto a educação bilíngue oferecida na escola comum quanto a educação bilíngue que acontece nas escolas de surdos são estratégias para que as identidades e a diferença surda sejam governadas. São analisados textos das políticas, relatórios e documentos produzidos por especialistas ou líderes surdos sobre a educação bilíngue, a fim de demonstrar como a população escolar surda vem sendo subjetivada, normalizada e conduzida para uma participação ativa e produtiva no mundo contemporâneo. Destaco a existência de distintos discursos sobre essa educação, alguns investimentos realizados por parte do governo federal e propostas de organização e distribuição dos sujeitos surdos em tempos e espaços escolares na atualidade

Pervasive gaps in Amazonian ecological research

Biodiversity loss is one of the main challenges of our time,1,2 and attempts to address it require a clear un derstanding of how ecological communities respond to environmental change across time and space.3,4 While the increasing availability of global databases on ecological communities has advanced our knowledge of biodiversity sensitivity to environmental changes,5–7 vast areas of the tropics remain understudied.8–11 In the American tropics, Amazonia stands out as the world’s most diverse rainforest and the primary source of Neotropical biodiversity,12 but it remains among the least known forests in America and is often underrepre sented in biodiversity databases.13–15 To worsen this situation, human-induced modifications16,17 may elim inate pieces of the Amazon’s biodiversity puzzle before we can use them to understand how ecological com munities are responding. To increase generalization and applicability of biodiversity knowledge,18,19 it is thus crucial to reduce biases in ecological research, particularly in regions projected to face the most pronounced environmental changes. We integrate ecological community metadata of 7,694 sampling sites for multiple or ganism groups in a machine learning model framework to map the research probability across the Brazilian Amazonia, while identifying the region’s vulnerability to environmental change. 15%–18% of the most ne glected areas in ecological research are expected to experience severe climate or land use changes by 2050. This means that unless we take immediate action, we will not be able to establish their current status, much less monitor how it is changing and what is being lostinfo:eu-repo/semantics/publishedVersio

Práticas Educomunicativas

Esta publicação pretende divulgar as práticas educomunicativas realizadas em diferentes regiões do país e que estão sendo implantadas por nossos associados. O e-book Práticas Educomunicativas, que visa oferecer um material de uso prático que possa servir de apoio pedagógico em diferentes contextos, escolar ou de ações junto a instituições, apresenta 20 artigos de profissionais e pesquisadores que implementam ações que inter-relacionam comunicação e educação no contexto da educação apontando as experiências e processos de educomunicação e valorizando desta forma, o trabalho realizado por cada educomunicador oferecendo, ao leitor, um material de uso prático que possa servir de apoio pedagógico em diferentes contextos