Pituitary-Gonadal Response to Short-term Pulsatile Luteinizing Hormone-Releasing Hormone Administration in Young Thalassemic Patients
Delayed puberty is one of the most common endocrine problems in severe thalassemia . Because of its clinical and psychological manifestations it remains a serious problem, especially for male patients. Impaired secretion of both luteinizing hormone (LH) and follicle-stimulation hormone after LH-releasing hormone (LHRH) and, to a lesser extent, a decrease in sex-steroid secretion have been ascribed to pituitary and/or testicular damage by chronic iron overload . However, because pubertal changes are linked to an increase in hypothalamic LHRH activity , a defect in the central operator of pubertal activation in thalassemia can also be hypothesized. On the other hand, hypothalamic dysfunction has been suggested in the early stage of genetic hemochromatosis . An impairment in LHRH discharge has been recently described in uremic boys, in whom delayed puberty is a common finding , and short-term pulsatile LHRH administration seems to restore normal pituitary-gonadal secretion in uremic boys with delayed puberty . We studied the effect of short-term pulsatile LHRH administration in a group of thalassemic patients with delayed or disordered puberty in order to evaluate the degree of hypothalamic involvement.
KeywordsLuteinizing Hormone Testicular Volume Luteinizing Hormone Secretion Hypothalamic Dysfunction Hypothalamic Involvement
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