@article {117544, title = {Assessment of puberty in relation to L-carnitine and hormonal replacement therapy in beta-thalassemic patients.}, journal = {Journal of tropical pediatrics}, volume = {54}, year = {2008}, month = {2008 Dec}, pages = {375-81}, 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.

PATIENTS: Thirty-two -thalassemic patients with arrested or failure of puberty were enrolled for 1 year in this study.

METHOD: 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.

CONCLUSION: 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.

}, keywords = {Adolescent, Adult, beta-Thalassemia, Carnitine, Estrogens, Female, Follow-Up Studies, Hormone Replacement Therapy, Humans, Male, Prevalence, Progesterone, Puberty, Delayed, Testosterone}, issn = {1465-3664}, doi = {10.1093/tropej/fmn043}, author = {Amal ElBeshlawy and Mohtar, Galila and Abd El Ghafar, Esmat and Abd El Dayem, Soha M and El Sayed, Manal Hamdy and Azza Ahmed Aly and Farok, Manal} }