Sex Steroid Priming for Induction of Puberty in Thalassemia Patients with Pulsatile Reversible Hypogonadotrophic Hypogonadism

Abstract

Growth and pubertal disturbances are the most common causes of morbidity, affecting 60–80% β-thalassemia major (β-TM) patients worldwide, due mainly to hypogonadotrophic hypogonadism (HH). We undertook a 6-year prospective study of 55 Indian β-TM children with stunted growth and absent or arrested puberty, aged 15–18 years with pulsatile HH, to evaluate the role of low dose sex steroid priming (6–12 months) for physiological induction of puberty. Eighty percent responded favorably with increase in height, growth spurt and completed pubertal maturation [Tanner stage 4–5 (T4-T5)] and 20% moved from T2 to T3. There was biochemical improvement in maturation of hypothalamic-pituitary (H-P) axis. Those younger than 15 years with minimal iron load had the best outcome. Our data suggest that sex steroid priming is a feasible method of induction of physiological puberty in β-TM patients with sexual infantilism and reversible apulsatile HH, especially in younger patients with minimal iron loads.

• Puberty
• Growth
• Reversible pulsatile hypogonadotrophic hypogonadism (HH)
• Sex steroid priming
• Thalassemia
• Growth hormone