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Journal of Tropical Pediatrics 1994 40(4):219-224; doi:10.1093/tropej/40.4.219
© 1994 by Oxford University Press
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research-article

Iron-endocrine Pattern in Patients with ß-Thalassaemia

M. A F. El-Hazmi*,, A. S. Warsy** and I. Al-Fawaz***

*Department of Medical Biochemistry, College of Medicine & King Khalid University Hospital, King Sand University Riyadh, Saudi Arabia
**Department of Biochemistry, College of Science, King Saud University Riyadh, Saudi Arabia
***Department of Paediatrics, College of Medicine, King Saud University Riyadh, Saudi Arabia

Correspondence: Professor M. A. F. El-Hazmi, Medical Biochemistry Department (30), College of Medicine & King Khalid University Hospital, P.O. Box 2925, Riyadh 11461, Saudi Arabia

Patients with ß-thalassaemia (major, minor, and in association with sickle cell gene) frequently suffer from growth retardation and delayed sexual development. This is believed to be due, at least in part, to a direct effect of iron overload on the endocrine mechanisms of puberty. The role of iron overload remains speculative in relation to the disease severity and ethnic variations.

This study determined the level of testosterone, cortisol, luteinizing hormone (LH), follicle stimulating hormone (FSH), free thyroxine (T4), tri-iodothyronine (T3), growth hormone (GH), iron, ferritin, and haematological parameters in 44 ß-thalassaemia patients (21 =ß-thal. major, 23 ß-thal minor), 25 Hb S/ß°-thalassaemia patients, and 50 normal controls with age range 2–15 years. The iron endocrine status and haematological findings were evaluated, and the results were correlated and compared with age-and sex-matched controls.

In comparison with controls the ß-thalassaemia-major and the Hb S/ß°-thalassaemia patients had a significantly higher level of plasma ferritin (P<0.01) while the mean level of total haemoglobin was significantly lower. The level of LH, FSH, testosterone, and plasma cortisol were lower in both ß-thalassaemia-major and Hb S/ß°-thalassaemia patients with a negative correlation with plasma ferritin level. Free T3 and T4 were raised, but the difference was not statistically significant. The data demonstrate the occurrence of impaired endocrine function in the ß-thalassaemia and Hb S/ß°-thalassaemia patients.


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