Mediterranean Journal of Hematology and Infectious Diseases

نویسندگان

  • Antonio Amato
  • C. Giordano
چکیده

Prevention of Hemoglobinopathies has developed around the world based upon the experience done in pioneering endemic countries and is now facing a new phase in nonendemic areas with a recent immigration history. We describe two situations, taking Latium (central Italy) and The Netherlands as two models for endemic and non-endemic countries both confronted with a large multi-ethnic immigrant society. We present prevention results and discuss aspects such as local knowledge and organization. We illustrate the importance of issues like information, carrier diagnostics, screening, counseling and prenatal diagnosis in particular situation of contrasting interest an different ethical opinions. We conclude by underlining the importance of implementing primary prevention at the European level, based upon better information, diagnostics and counseling. Hemoglobinopathies (HbP’s) are the most common recessive autosomal disorders in man because carriers are protected against child mortality due to malaria. This advantage has strongly selected the traits in all tropic and subtropic areas of the old world were most carriers live today. However, due to ancient and recent migrations HbP’s are now present virtually everywhere including those non-endemic industrialized areas where people from endemic countries migrate in search of work. Severe Hemoglobinopathies (Thalassemia and Sickle Cell Disease) are conditions affecting our oxygen transport protein hemoglobin A (HbA). This tetramer consists of 4 globin chains (α2/β2) coded by genes located on chromosome 16 (the alpha genes) and 11 (the beta genes). While β-Thalassemia is caused by defects impairing the expression of the β globin genes, Sickle Cell Disease (SCD) is induced by a single β-gene mutation (HbS), either in homozygous form (HbS/S) or in a number of common compound heterozygous conditions such as HbS/β-thal, HbS/C, HbS/D, HbS/E and others. The WHO has been recommending primary prevention for many years because these severe diseases, although treatable, cannot be “cured” unless a bone marrow transplant is successfully performed. Therefore primary prevention has been offered for the last 30 years to couples at risk in the Mediterranean area where the incidence of β-Thalassemia major in pioneering

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تاریخ انتشار 2010