Abstract
Clinical and laboratory examination of 41 multi-transfused β-thalassemic patients aged 16-24 years (18 boys and 23 girls)showed that 27 had entered puberty. The remaining 14 patients were at a prepubertal stage clinically with very low values of LH and FSH after LHRH test and with a ratio FSH/LH > 1. Daily administration of clomiphen citrate for 5 days showed no response of LH, FSH oestradiol and progesterone in females or LH, FSH and testosterone in males. Basal levels of testosterone and oestradiol were below normal values for puberty and there was no increase of testosterone release after HCG administration. The above results demonstrate that the hypogonadisme observed in one third of the patients aged 16-24 years is not perhaps exclusively secondary but that a primary damage of the target organ may also be present. Serum GH after insulin induced hypoglycemia,sleep or L-Dopa administration was ranging within normal levels. The study of T3, T4 and basal TSH in the 14 prepubertal patients showed normal levels in 5. A TRH test revealed normal TSH response only in 8 out of 14 prepubertal patients, the remaining 6 showed an increased response suggesting sub-clinical hypothyroidism. Our study shows that one third of the homozygous β-thalassemic patients aged more than 16 years were at a prepubertal stage and that at this age a more generalized endocrine dysfunction is present.
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Chatziljami, A., Mengreli, C., Anapliotou, M. et al. ENDOCRINE ASSESSMENT OF MULTI-TRANSFUSED PATIENTS WITH HOMOZYGOUS β-THALASSEMIA 16-24 YEARS OLD. Pediatr Res 20, 1204 (1986). https://doi.org/10.1203/00006450-198611000-00185
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DOI: https://doi.org/10.1203/00006450-198611000-00185