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lüll The genetics of blood disorders: hereditary hemoglobinopathies Sonati Mde F; Costa FFJ Pediatr (Rio J) 2008[Aug]; 84 (4 Suppl): S40-51OBJECTIVE: To summarize recently published data on the pathophysiology, diagnosis and treatment of sickle cell diseases and beta-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 beta-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 beta-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.|Anemia, Sickle Cell/*genetics/physiopathology/therapy[MESH]|Animals[MESH]|Disease Models, Animal[MESH]|Humans[MESH]|beta-Thalassemia/*genetics/physiopathology/therapy[MESH] |