The effect of sex steroids on pubertal disorders of beta thalassemia major

Purpose: Pubertal disorders are well knownComplications of beta thalassemia Major(TM.( . The aim of study was to assess theeffect of sex steroid administration onthalassemic patient attending Boo Ali SinaHospital, Sari, Iran.Materials and Methods: all of the TMpatients at Growth Clinic from 2005 to 2011were underwent treatment withethinylestradiol 2.5 to 25  g/ day plusmedroxy progesterone acetate 5mg/day for 15days of each month for females, ortestosterone enenthate 50 –l00  g / month asIM injection for males. Weight, height,pubertal stage of the subjects was recordedbefore and after the intervention. Thyroidfunction, serum ferritin, Ca , P, ALP , LFT,OGTT tests were performed. Compliance totreatment measured by looking at missedvisits.Results: 173 TM Patients (54% Female) werefollowed, age at study in girls and boys were14 3.5 and 14.3  2.5 years respectively.Follow up period for girls and boys were 31.2 and 3.31.5 years respectively. Increasingof BW SDS and BH SDS in both sexes werestatistically significant (P<0.01). SMRchanges by McNemar test was significantonly in girl (p<0.01). Compliance to treatmentwas better in girls.Discussion: Sex steroids are cheap andavailable treatment for growth and pubertaldisorders in TM patients. Compliance totreatment is essential for successful treatment.

Keywords:

Beta thalassmia major , puberty , Sex steroids , Treatment