Dextrocardia with situs inversus associated with non-compaction cardiomyopathy

OBJECTIVE: To conduct a literature review on the diagnosis and management of primary hyperparathyroidism including the classical hipercalcemic form as well as the normocalcemic variant. MATERIALS AND METHODS: This scientific statement was generated by a request from the Brazilian Medical Association (AMB) to the Brazilian Society for Endocrinology as part of its Clinical Practice Guidelines program. Articles were identified by searching in PubMed and Cochrane databases as well as abstracts presented at the Endocrine Society, Brazilian Society for Endocrinology Annual Meetings and the American Society for Bone and Mineral Research Annual Meeting during the last 5 years. Grading quality of evidence and strength of recommendation were adapted from the first report of the Oxford Centre for Evidence-based Medicine. All grades of recommendation, including "D", are based on scientific evidence. The differences between A, B, C and D, are due exclusively to the methods employed in generating evidence. CONCLUSION: We present a scientific statement on primary hyperparathyroidism providing the level of evidence and the degree of recommendation regarding causes, clinical presentation as well as surgical and medical treatment. OBJETIVO: Conduzir uma atualizacao das ultimas evidencias cientificas a respeito da apresentacao, do diagnostico e do manejo clinico e cirurgico do hiperparatireoidismo primario classico e normocalcemico. MATERIAIS E METODOS: Este documento foi concebido pelo Departamento de Metabolismo Osseo da Sociedade Brasileira de Endocrinologia e Metabologia (SBEM) a partir daquele oriundo do Programa de Diretrizes da Associacao Medica Brasileira (AMB) da SBEM. Realizamos uma revisao dos artigos mais relevantes obtidos nos bancos de dados PubMed e Cochrane, alem de abstracts apresentados nos encontros anuais da Endocrine Society, da Sociedade Brasileira de Endocrinologia e da American Society for Bone and Mineral Research dos ultimos cinco anos, e classificamos as evidencias em niveis de recomendacoes de acordo com a forca cientifica por tipo de estudo, adaptando o primeiro relato do "Oxford Centre for Evidence-based Medicine". Todos os graus de recomendacao, incluindo-se o "D", foram basea-dos em evidencia cientifica, sendo as diferencas entre o A, B, C e D devidas exclusivamente ao desenho empregado na geracao da evidencia. CONCLUSAO: Apresentamos uma atualizacao cientifica a respeito do hiperparatireoidismo primario, classificando e graduando em niveis de recomendacoes as principais evidencias cientificas sobre as suas causas, as variadas formas de apresentacao, seu diagnostico e tratamento.