Natural history of sickle cell anemia in Saudi Arabs. A study of 270 subjects.
We studied 270 Saudi Arabs with homozygous sickle cell anemia, using chart review, a register (since 1969), and home visiting in 42 cases. Average follow-up for the total group was 10 years. Seventy-four percent of those diagnosed by age 3 years presented on screening or with merely anemia; 26% presented with illness, abnormal physical findings, or pain. Compared with American or Jamaican blacks, serious complications occurred only 6% to 25% as frequently; leg ulcers did not occur at all; the mortality under age 15 years was 10% as great; mean levels of blood hemoglobin were higher (10 g/dl), reticulocyte count was lower (5% to 6%), and mean fetal hemoglobin (HbF), which was inversely correlated with reticulocytes, was higher (22% to 26.8%). The high HbF is believed to account for the very mild clinical manifestations.
D000293:Adolescent; D000328:Adult; D000368:Aged; D000755:Anemia, Sickle Cell / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology / Q000401:mortality; D044383:Black People; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006760:Hospitalization; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D012529:Saudi Arabia; D014481:United States; D001741:Black or African American