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, PERRINE S, SHOUP F. Natural History of Sickle Cell Anemia in Saudi Arabs: A Study of 270 Subjects. Ann Intern Med. ;88:1–6. doi: 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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