In this study, 80 male and female sickle cell patients, aged 4-50 years, with mild (severity index, SI < 6) and severe (SI > or = 6) forms of the disease were investigated). The levels of luteinizing hormone (LH), follicle stimulating hormone (FSH), testosterone, cortisol, growth hormone (GH), free thyroxine (T4), and free triiodothyronine (T3) were determined. The results were evaluated and the mean +/- 2 SD values were compared with those obtained in age- and sex-matched normal controls. The findings indicated gonadal hypofunction in the sickle cell patients, but with varied deviations from the mean results. Patients with the severe form of the sickle cell disease showed more frequent abnormalities of LH, FSH, cortisol and testosterone in comparison with the patients with a mild disease. The LH, FSH, cortisol and testosterone levels were lower, while T3 and T4 did not show significant differences between patients and the controls. The results suggest that the sickle cell gene abnormality has an adverse effect on endocrine functions. Follow-up and appropriate management of endocrine dysfunctions are advocated in such patients.
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