[Study of hemoglobinopathies found in Belgium].
Our purpose is to illustrate our contributions to the study of the haemoglobinopathies observed in Belgium. We described a new deletion leading to major thalassaemia and have established that the erythropoietin production is adequate in this disease. We were particularly interested in some manifestations of iron overload (polyendocrinopathy, roles of the decreased phagocytosis and of the desferrioxamine therapy in the predisposition to infections, protective role of desferrioxamine in the protection against the progression of HIV-1 infection). The prevention of thalassaemia major has deserved our particular attention. As concerns sickle cell anemia, we have underlined some particular aspects of the clinical expression of the disease. The use of new therapeutic approaches (bone marrow transplantation, hydroxyurea) has also been outlined.
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D000755:Anemia, Sickle Cell / Q000503:physiopathology* / Q000628:therapy; D000986:Antisickling Agents / Q000627:therapeutic use; D001530:Belgium; D016026:Bone Marrow Transplantation; D002648:Child; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D017086:beta-Thalassemia / Q000150:complications / Q000235:genetics / Q000503:physiopathology*
D000986:Antisickling Agents; D006918:Hydroxyurea