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Plasma adrenocorticotropic hormone (ACTH), 17-hydroxycorticosteroids (17-OHCS), free fatty acids (FFA), and total cholesterol and its fractions (TG, cholesterol, HDL-cholesterol, LDL-cholesterol) were measured in 16 children with sickle cell disease in various stages of the disease. Ten children were asymptomatic, and six had a history of recurrent painful vaso-occlusive crisis. Plasma FFA and cholesterol levels were higher (P less than 0.001) in the sickle cell anemia (S/C) group than in the asymptomatic children, while plasma 17-OHCS, ACTH, and TG were lower (P less than 0.001). Plasma ACTH was higher (P less than 0.01) in the symptomatic group (S/C) than in the asymptomatic group. Plasma 17-OHCS showed a positive correlation (r = 0.82; P less than 0.001) with age in the sickle cell anemia group. Plasma 17-OHCS and FFA decreased after the administration of oral hydrocortisone. We conclude that the decrease of plasma 17-OHCS in the symptomatic children with sickle cell anemia may be due to the long-term use of hydrocortisone.

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