科研之友
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摘要
objective: to summarize recently published data on the pathophysiology, diagnosis and treatment of sickle cell diseases and 汕-thalassemias, the most relevant hereditary hemoglobinopathies in the global population. sources: searches were run on the medline and scielo databases, limited to the period from 2003 to may 2008, using the terms hereditary hemoglobinopathies, sickle cell diseases and 汕-thalassemia. two books and two chapters were also included. summary of the findings: more than 2,000 articles were identified; those providing the most important information and broadest views were selected. conclusions: morbidity and mortality rates from sickle cell diseases and 汕-thalassemia are still very high and represent an important challenge. increased understanding of pathophysiological aspects has lead to significant improvements in treatment and prevention of these diseases.
