Incidence, effects, and management of sickle cell disease in Brazil

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Abstract
Forty-five percent of the 121 million persons who live in Brazil have morphological indications of Black admixture, and 5-6% of them are carriers of the hemoglobin S gene. But even in persons identified as white, the hemoglobin AS genotype is present in about 1%. The total number of people with sickle cell disease is estimated as 45,000. The present analysis is based in 409 patients studied in Rio de Janeiro. The observed frequency of deformities of the vertebrae (18%), bone infarcts (12%), and gnathopathy (4%) was lower than those found in any other series. The proportion of adults with splenomegaly was also lower than that of other studies. Chest pains were more frequent in males, while bone or joint pains and splenic sequestration crises seemed to be more common in females. Effects of the disease in the viability and fertility of these patients were quantified. Fetal loss in 67 pregnancies was 48%. Hemoglobin F levels showed a clear age effect, and higher levels were associated with a more benign course of the disease. Information about the services for prevention and treatment of sickle cell disease that exist in Brazil and one place in Venezuela, is provided. Treatment is only symptomatic in 12 centers, while in São Paulo piracetam is being successfully used in the treatment of crises. A brief overview about the current Brazilian studies on this disease is also presented.

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