title: Assessment of puberty in relation to L-carnitine and hormonal replacement therapy in beta-thalassemic patients.
abstract: OBJECTIVE
  To investigate puberty in a group of thalassemic patients with delayed or arrested pubertal development and to compare the effects of hormonal and L-carnitine therapy on puberty in those patients.
  
  
  METHODS
  Thirty-two -thalassemic patients with arrested or failure of puberty were enrolled for 1 year in this study.
  
  
  METHODS
  Clinical pubertal assessment and laboratory investigations were done for all patients at the beginning, 6 months later clinical pubertal assessment was done. Patients were divided into two groups (16 each): first group received L-carnitine therapy, while the second group received hormonal therapy. Pubertal and laboratory assessment were done 6 months after hormonal and L-carnitine therapy.
  
  
  RESULTS
  Failure of puberty was confirmed in 71.4% of boys and 33.3% of girls, while arrested puberty was observed in 28.6% of boys and 66.7% of girls. All girls had amenorrhea, primary amenorrhea in 88.9% and secondary amenorrhea in 11.1%. Menses occurred in 20% of female patients after L-carnitine therapy and in 37.5% of them after hormonal therapy. Improvement of pubertal staging was observed in 50% of males after L-carnitine therapy compared to 75% of them after hormonal therapy. While improvement of pubertal staging was seen in 90% of females after L-carnitine therapy compared to 100% of females after hormonal treatment. However, these results showed no significant difference between both groups.
  
  
  CONCLUSIONS
  Delayed puberty in beta-thalassemia major is either due to failure of gonads or failure of the whole hypothalamic pituitary gonadal axis. L-carnitine as well as hormonal replacement therapy had a positive effect on puberty in the thalassemic patients. Further studies are needed to clarify the role of L-carnitine on puberty in these patients.
authors.affiliation: Department of Pediatrics, Cairo University, Ain Shams University and National Research Centre, Cairo, Egypt.
authors.forename: Amal
authors.initials: A
authors.lastname: El Beshlawy
authors.forename: Galila
authors.initials: G
authors.lastname: Mohtar
authors.forename: Esmat
authors.initials: E
authors.lastname: Abd El Ghafar
authors.forename: Soha M
authors.initials: SM
authors.lastname: Abd El Dayem
authors.forename: Manal Hamdy
authors.initials: MH
authors.lastname: El Sayed
authors.forename: Azza Ahmed
authors.initials: AA
authors.lastname: Aly
authors.forename: Manal
authors.initials: M
authors.lastname: Farok
mesh_terms: D000293:Adolescent; D000328:Adult; D002331:Carnitine / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use*; D004967:Estrogens / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D020249:Hormone Replacement Therapy; D006801:Humans; D008297:Male; D015995:Prevalence; D011374:Progesterone / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use*; D011628:Puberty, Delayed / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D013739:Testosterone / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use*; D017086:beta-Thalassemia / Q000150:complications*
chemical_list: D004967:Estrogens; D013739:Testosterone; D011374:Progesterone; D002331:Carnitine