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- Efficacy and safety of pharmacological interventions for managing sickle cell disease in children and adolescents: protocol for a systematic review with network meta-analysisPublication . Tonin, Fernanda; Ginete, Catarina; Fernandez-Llimos, Fernando; Ferreira, Joana; Delgadinho, Mariana; Brito, MiguelIntroduction: Sickle cell disease (SCD), an inherited haemoglobinopathy, has an important impact on morbidity and mortality, especially in paediatrics. Previous systematic reviews are limited to adult patients or focused only on a few therapies. We aim to synthesise the evidence on the efficacy and safety of pharmacological interventions for managing SCD in children and adolescents. Methods and analysis: This systematic review protocol is available at Open Science Framework (doi:10.17605/OSF.IO/CWAE9). We will follow international recommendations on the conduction and report of systematic reviews and meta-analyses. Searches will be conducted in PubMed, Scopus, and Web of Science (no language nor time restrictions) (first pilot searches performed in May 2022). We will include randomised controlled trials comparing the effects of disease-modifying agents in patients with SCD under 18 years old. Outcomes of interest will include: vaso-occlusive crisis, haemoglobin levels, chest syndrome, stroke, overall survival, and adverse events. We will provide a narrative synthesis of the findings, and whenever possible, results will be pooled by means of pairwise or Bayesian network meta-analyses with the surface under the cumulative ranking curve analyses. Different statistical methods and models will be tested. Dichotomous outcomes will be reported as OR, risk ratio, or HR, while continuous data will be reported as standard mean differences, both with 95% CI/credibility interval. The methodological quality of the trials will be evaluated using the Risk of Bias 2.0 tool, and the certainty of the evidence will be assessed with the Grading of Recommendations Assessment, Development, and Evaluation approach. Ethics and dissemination: This study refers to a systematic review, so no ethics approval is necessary. We intend to publish our findings in an international, peer-reviewed journal. Data will also be presented to peers at scientific events. Additionally, the results obtained in this study may contribute towards the update of therapeutic guidelines and the development of health policies for SCD.
- Pregnancy surveillance in sickle cell disease patients: a cohort study in an African country – AngolaPublication . Brito, Miguel; Ginete, Catarina; Ferreira, Joana; Mendes, Manuela; Vasconcelos, Jocelyne; Delgadinho, Mariana; Sebastião, Cruz; Quinto, Francisco; Simão, Fernanda; Mateus, Anabela; Fernandes, FilipeSickle Cell Disease (SCD) is a severe monogenic disease caused by the inherence of a recessive mutation in the β-globin gene, with an especially high prevalence in sub-Saharan Africa. In Angola, the prevalence of the disease is almost 2%, and the carriers reach 21% of the population. Although its presentation tends to be very heterogeneous, chronic hemolytic anemia, frequent painful crises, and extensive organ damage are common features of these patients. Pregnancy in SCD patients is associated with an increase in severe outcomes, namely, a high risk of eclampsia and pre-eclampsia, stroke, and even death. Therefore, it is crucial to maintain continuous medical surveillance during pregnancy, especially in women with previous strokes. Moreover, health services in low- and middle-income countries are generally not prepared to follow these patients. The present cohort study, conducted at the Lucrecia Paim Maternity Hospital (Luanda, Angola), aims to determine pregnancy complications in SCD women, especially those responsible for maternal death, and, by supporting the obstetric consultations in this hospital, contribute to the reduction of mortality and morbidity rates. Pregnancy monitoring includes analysis of clinical history and incidents (number of hospitalizations, blood transfusions, strokes, and other clinical complications), hematological and biochemical analysis, transcranial Doppler to assess cerebral hemodynamics, and genetic analysis (confirmation of the diagnosis, genotyping of four SNPs in the β-cluster to assess the haplotype, and evaluation of the presence of the 3.7kb deletion of the α-globin gene).
- O projeto artístico-pedagógico como meio de pensar e construir a aprendizagemPublication . Ferreira, Joana; Matos, JoanaO projeto artístico-pedagógico, tem vindo a ser desenvolvido na Unidade Curricular de Artes Plásticas II da Licenciatura em Educação Básica, na Escola Superior de Educação de Lisboa como um meio de pensar e construir a aprendizagem. Enquadrado numa metodologia de trabalho assente em processos criativos no campo das artes plásticas, os projetos desenvolvidos têm vindo a promover a utilização de recursos de natureza diversa, processos de trabalho de pesquisa e integração de conhecimentos de natureza variada, traduzindo-se em abordagens que se valorizam sempre como resultado de caminhos pessoais e de descoberta. O desenvolvimento de processos criativos no campo das artes plásticas permite ainda perceber como estruturar as aprendizagens tendo em vista as práticas profissionais em contexto educativo formal e não formal. Pretende-se mostrar alguns dos processos de trabalho desenvolvidos, nomeadamente os processos criativos, entendidos enquanto método de investigação e pesquisa em educação artística, através de utilização de técnicas e ferramentas que tornam o pensamento mais visual e percetível, tais como o brainstorming ou o mindmap. Pretende-se ainda evidenciar que o processo de trabalho de natureza artística, como exercício de investigação multidisciplinar, promove um maior e mais ancorado envolvimento com o projeto em si mesmo, desenvolvendo simultaneamente o sentido crítico e criativo sobre os assuntos. Em termos de resultados, analisam-se alguns projetos exemplificativos que mobilizam conhecimentos de natureza técnica, estética e cultural tendo em conta as questões pedagógicas, como revisão e reflexão sobre as metodologias de trabalho desenvolvidas, cujos meios passam ainda pela utilização de recursos digitais.
- Efficacy and safety of pharmacological interventions for managing sickle cell disease complications in children and adolescents: systematic review with network meta‐analysisPublication . Tonin, Fernanda; Ginete, Catarina; Ferreira, Joana; Delgadinho, Mariana; Santos, Brígida; Fernandez‐Llimos, Fernando; Brito, MiguelThis study aimed to synthesize the evidence on the effects of disease-modifying agents for managing sickle cell disease (SCD) in children and adolescents by means of a systematic review with network meta-analyses, the surface under the cumulative ranking curve (SUCRA) and stochastic multicriteria acceptability analyses (SMAA) (CRD42022328471). Eighteen randomized controlled trials (hydroxyurea [n = 7], l-arginine [n = 3], antiplatelets [n = 2], immunotherapy/monoclonal antibodies [n = 2], sulfates [n = 2], docosahexaenoic acid [n = 1], niprisan [n = 1]) were analyzed. SUCRA and SMAA demonstrated that hydroxyurea at higher doses (30 mg/kg/day) or at fixed doses (20 mg/kg/day) and immunotherapy/monoclonal antibodies are more effective for preventing vaso-occlusive crisis (i.e., lower probabilities of incidence of this event; 14, 25, and 30%, respectively), acute chest syndrome (probabilities ranging from 8 to 30%), and needing of transfusions (11-31%), while l-arginine (100-200 mg/kg) and placebo were more prone to these events. Therapies were overall considered safe; however, antiplatelets and sulfates may lead to more severe adverse events. Although the evidence was graded as insufficient and weak, hydroxyurea remains the standard of care for this population, especially if a maximum tolerated dose schedule is considered.