title: 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.
authors.affiliation: Institute for Women's Health, University College London Hospitals, London, UK. r.chatterjee@ucl.ac.uk
authors.forename: Ratna
authors.initials: R
authors.lastname: Chatterjee
authors.forename: Tarak Nath
authors.initials: TN
authors.lastname: Mukhopadhyay
authors.forename: Sharmila
authors.initials: S
authors.lastname: Chandra
authors.forename: Rekha
authors.initials: R
authors.lastname: Bajoria
mesh_terms: D000293:Adolescent; D004997:Ethinyl Estradiol / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007006:Hypogonadism / Q000150:complications* / Q000188:drug therapy*; D011628:Puberty, Delayed / Q000188:drug therapy* / Q000209:etiology*; D043343:Testosterone Propionate / Q000008:administration & dosage / Q000627:therapeutic use*; D016896:Treatment Outcome; D017086:beta-Thalassemia / Q000150:complications*
chemical_list: D004997:Ethinyl Estradiol; D043343:Testosterone Propionate