Abstract
Children affected by thalassemia major frequently present growth disturbances. To investigate the role of delayed sexual development in the growth impairment associated with thalassemia. we studied 29 patients (age range 10.7-19.5 years) with short stature (< 3rd centile), reduced growth velocity (< 10th centile) and delayed bone age. All patients were under regular transfusion and chelation therapy according to standard protocols. Growth hormone (GH) secretion was evaluated by peak GH response to arginine-insulin. Results are shown in the Table. Patients were divided in subgroups based on bone age, pubertal status and whether they had been submitted to sex steroid priming. Patients in group 4) where tested both before and after priming. Patients in group 5) were already on sex steroid treatment. Responders are patients who exhibited a peak GH level > 10 ng/ml at least to 1 stimulus.We have treated twelve patients belonging to groups 2) and 5) for 6-21 months (mean 11±4) with sex steroid in the attempt to increase growth velocity and induce puberty. Treatment consisted of monthly injection of 50-100 mg of testosterone enanthate for boys and transdermic estrogens (0.025-0.05 mg/die) for girls. We observed a significant increase of growth velocity during treatment (2.6±0.8 cm /yr vs. 6.1±1.3 cm/yr; P <0.01): bone age advanced from −2.6 to −1.7 SDS. These data suggest that GH deficiency is not frequent among short thalassemic patients and when present is mainly due to delayed puberty: in addition sex steroids therapy is effective in inducing a significant growth acceleration in these patients.
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