RICHARD P. PERRINE, M.D.; MARCUS E. PEMBREY, M.B., M.R.C.P. (U.K.); PETER JOHN, M.B.; SUSAN PERRINE, M.D.; FERIAL SHOUP, R.N.
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.
PERRINE RP, PEMBREY ME, JOHN P, et al. Natural History of Sickle Cell Anemia in Saudi Arabs: A Study of 270 Subjects. Ann Intern Med. 1978;88:1–6. doi: https://doi.org/10.7326/0003-4819-88-1-1
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Published: Ann Intern Med. 1978;88(1):1-6.
Hematology/Oncology, Red Cell Disorders.
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