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Objective: To systematically establish whether there is an association between polymorphisms and avascular necrosis in patients with sickle cell disease. Data source: The review, conducted according to PRISMA guidelines and registered with PROSPERO, was based on research of studies in PubMed, SciELO, LILACS, BVS databases and in the gray literature (Google Scholar and Open Gray) published until June 2020. The STROBE initiative was used to analyze the articles’ quality. Data synthesis: Ten articles were selected from the databases and two were included through manual search, totaling 12 studies. All samples gathered 2,362 patients. According to STROBE, seven studies fully and/or partially covered more than 70% of the essential items and two studies reached less than 60%, with an overall variation of 86.4–54.5%. The results indicate that polymorphisms in the genes of the bone morphogenetic protein 6 (BMP6), Klotho (KL) and Annexin A2 (ANXA2) may be associated with osteonecrosis in the context of sickle cell disease. Six articles addressed the polymorphism in the MTHFR enzyme gene, but only one found a positive association. Polymorphisms associated with the DARC receptor, the ITGA4 gene, CD36 and thrombophilia protein genes were not associated in any of the studies. Conclusions: The results indicate that the polymorphisms in BMP6, Klotho and ANXA2 genes may be associated with avascular necrosis in patients with sickle cell disease. However, in order to confirm these genetic changes as risk factors, further studies with greater statistical power and methodological rigor are needed.

AIM: Medicine has been based on empirical knowledge since Galen time. The term evidence-based medicine is more recent, appearing for the first time in 1992. To analyze the articles on Shoulder and Elbow Surgery published by Brazilian authors between 2008 and 2017. METHODS: Two researchers independently performed a search of journal articles on Shoulder and Elbow Surgery published by Brazilian authors in two orthopedic journals: Brazilian Journal of Orthopedics (RBO) and Brazilian Orthopedic Act (Acta) between 2008 and 2017. A third researcher searched PUBMED for Brazilian Shoulder and Elbow Surgery specialists to identify relevant articles in other national and international journals. After selection, the articles were classified by the level of evidence based on the classification proposed by the Oxford Center for Evidence-based Medicine. RESULTS: Between 2008 and 2017, 333 articles on Shoulder and Elbow surgeries were published by Brazilian authors in national and international journals. Of these, 93 were published in international journals, 24 in national journals (excluding Acta and RBO), 172 in the Brazilian Journal of Orthopedics, and 44 in the Acta Ortopédica Magazine. Authors from the state of São Paulo accounted for about 68% of the articles. There was a predominance of articles with a level of evidence of 4 and 5, and of the works with level 1, 66.7% were published in international journals. CONCLUSION: There was a predominance of articles with a level of evidence of 4 and 5, and of the works with level 1, 66.7% were published in international journals.

Resumo Objetivo: Estabelecer, de modo sistemático, se existe associação entre polimorfismos e a necrose avascular em pacientes com doença falciforme. Fontes de dados: A revisão, conduzida segundo as diretrizes Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) e registrada no International Prospective Register of Systematic Reviews (PROSPERO), foi baseada na busca de estudos nas bases de dados PubMed, Scientific Electronic Library Online (SciELO), Literatura Latino-Americana e do Caribe em Ciências da Saúde (LILACS), Biblioteca Virtual em Saúde (BVS) e na literatura cinza (Google Scholar e Open Gray) até junho de 2020. A análise da qualidade dos artigos foi baseada nos critérios do Strengthening the Reporting of Observational Studies in Epidemiology (STROBE). Síntese dos dados: Dez artigos foram selecionados nas bases de dados e dois incluídos por meio da busca manual, totalizando 12 estudos elencados. As amostras resultaram em 2.362 pacientes incluídos. Com base na iniciativa STROBE, sete estudos atenderam total e/ou parcialmente mais de 70% dos itens essenciais e dois atingiram menos que 60% deles, com variação geral de 86,4–54,5%. Os resultados mostram que os polimorfismos nos genes da proteína morfogenética óssea 6 (BMP6), da Klotho (KL) e da Anexina A2 (ANXA2) podem ter associação com osteonecrose no contexto da doença falciforme. Seis artigos estudaram o polimorfismo no gene da enzima MTHFR, mas apenas um obteve associação positiva. Os polimorfismos associados ao receptor DARC, ao gene ITGA4, ao CD36 e aos genes de proteínas trombofílicas não demonstraram associação em nenhum dos estudos. Conclusões: Os polimorfismos nos genes BMP6, KL e ANXA2 estão possivelmente associados com a necrose avascular em indivíduos com doença falciforme. Entretanto, para a confirmação dessas alterações genéticas como fatores de risco, é necessário que mais estudos com maior poder estatístico e com maior rigor metodológico sejam realizados.