title: Sickle cell anemia in Rio de Janeiro, Brazil: demographic, clinical and laboratory data.
abstract: A sample of 409 individuals with sickle cell anemia in the city of Rio de Janeiro was studied. Information about the age of onset, first manifestations, most common clinical features, physical and sexual development, types of crises, laboratory determinations, and age at death was provided. The most salient findings were the low frequencies of bone lesions (12-18%), gnathopathy (4%), persistence of splenomegaly in adults (4%), and abnormal cardiograms (16%). Bone infarctions and osteomyelitis were correlated significantly, the frequency of the latter increasing with age. A significant sex difference was observed in the types of crises. Chest pains were more frequent in males, while bone or joint pains, as well as splenic sequestration, were more common in females.
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000175:diagnosis / Q000401:mortality; D001938:Brazil; D002648:Child; D002675:Child, Preschool; D003710:Demography; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged