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Journal of Tropical Pediatrics Advance Access published online on July 22, 2008
Journal of Tropical Pediatrics, doi:10.1093/tropej/fmn043
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© The Author [2008]. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Assessment of Puberty in Relation to L-carnitine and Hormonal Replacement Therapy in β-thalassemic Patients

Amal El Beshlawya, Galila Mohtara, Esmat Abd El Ghafarb, Soha M. Abd El Dayema, Manal Hamdy El Sayeda, Azza Ahmed Alyb and Manal Faroka

aDepartment of Pediatrics, Cairo University, Ain Shams University and National Research Centre, Cairo, Egypt
bDepartment of Clinical pathology, National Research Centre, Cairo, Egypt

Correspondence: Soha Mahmoud Abd El Dayem, Professor of Pediatrics, Pediatrics Department, National Research Centre, Mathaf El Manial Street Flat 52, Cairo, Egypt. Tel.: 023648767, 0106716852. E-mail <S_eldayem{at}yahoo.com>


  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 β-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.

Key Words: puberty • L-carnitine • hormonal therapy • β-thalassemic


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