INDUCTION OF PUBERTY IN HYPOGONADIC FEMALES WITH THALASSEMIA MAJOR - PRELIMINARY DATA

Sexual maturation and growth during induction of puberty with oral eth inylestradiol at low doses were evaluated in seven thalassemic girls, aged 13.6-16.5 years with hypogonadotropic hypogonadism (group 1). The y were compared with nine thalassemic girls, aged 9-11.4 years with sp ontaneous puberty (group 2). Estrogen therapy was started at chronolog ical age (CA) of 14.3 +/- 1.12 (M +/- SD) years corresponding to bone age (BA) of 11.4 +/-1.07 years. Five patients had vaginal bleeding aft er about 1 year of therapy, attaining a Tanner pubertal stage of B3-B4 . Two patients lacking vaginal bleeding after 15 months of continuativ e therapy, received estrogen with the addition of medroxyprogesterone acetate. Vaginal bleeding occurred at CA of 15.7 +/- 1.0 year (BA 13.1 +/-1.01 years). In group 2, at onset of puberty CA was 10.3 +/- 1.05 years (BA 10.1 +/- 1.05 years) and at menarche CA was 12.6 +/- 0.46 ye ars (BA 12.4 +/- 0.32 years). After 3 months of therapy, all patients of group 1 showed a height velocity peak of 7 +/- 1.9 cm smaller than in group 2 (cm 9.1 +/- 0.98). Height gain until vaginal bleeding, in g roup 1 (4.8 +/- 1.57 cm) was smaller than in group 2 (11.9 +/- 3.66 cm ). There was no significant difference in either mean height at first vaginal bleeding or in predictive final height between the two groups. No side effects were observed during therapy. It is interesting to no te that patients of group 1 showed a serious degree of osteoporosis; i n fact, the mean value of bone mineral density was smaller than in Ita lian normal girls (0.49 +/- 0.08 vs. 0.61 +/- 0.06 (g/cm2), p < 0.05). In conclusion, the results of this preliminary study indicate that th e time of the start of treatment has no significant effect on height; nevertheless, therapy should be started at normal mean age both to avo id psychosocial problems and to reduce the risk of osteoporosis.