Natural history of sickle cell anemia in Saudi Arabs. A study of 270 subjects.

Autor: Perrine, Richard P., Pembrey, Marcus E., John, Peter, Perrine, Susan, Shoup, Ferial, Perrine, R P, Pembrey, M E, John, P, Perrine, S, Shoup, F
Předmět:
Zdroj: Annals of Internal Medicine; Jan78, Vol. 88 Issue 1, p1-6, 6p, 3 Charts, 1 Graph
Abstrakt: 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. [ABSTRACT FROM AUTHOR]
Databáze: Complementary Index