Growth and sexual development before and after sex steroid therapy in patients with thalassemia major

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Abstract
Growth, sexual development and hypothalamic-pituitary-gonadal function were evaluated in 23 patients with thalassemia major (14 female and 9 male) aged 13-29 yr. Five women (group 1) with Hb levels of < 7 g/dl, which were maintained by transfusions during childhood, did not spontaneously enter puberty. They had evidence of severe hypothalamic-pituitary dysfunction. Maintaining Hb levels of about 8 g/dl resulted in spontaneous onset of puberty in 7 of 9 female patients (group 2), but had no such ameliorative effect on the 9 male patients. In the latter, peak luteinizing hormone (LH) responses to gonadotropin releasing hormone correlated with bone age. Treatment with testosterone produced inconsistent partial inhibition of LH and FSH responses to stimulation. After discontinuation of testosterone treatment, a rebound of basal testosterone, LH and FSH levels was observed, but this was not sustained. These findings are compatible either with dysfunction of hypothalamic maturation or with partial pituitary dysfunction. Four of the group 1 females and 6 of the males treated with appropriate sex hormones showed satisfactory pubertal progression. Acceleration in linear growth was observed in 4 of the male patients whose epiphyses were still open. Treatment was well tolerated in all patients.

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