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ORIGINAL ARTICLE
Year : 2013  |  Volume : 38  |  Issue : 4  |  Page : 149-154

Deferiprone and desferrioxamine combined chelation could improve puberty of adolescent males with β-thalassemia major with preserved pituitary and testicular function


1 Pediatrics Department, Thalassemia Unit, Ain Shams University, Cairo, Egypt
2 Research Department, National Center for Examinations and Educational Evaluation, Ain Shams University, Egypt
3 Endocrinology Unit, Ain Shams University, Cairo, Egypt

Correspondence Address:
Eman Ragab
MD, Pediatrics Department, Ain Shams University, Ramzy Aziz Streetout of Toman Bay, Zeitoun, Cairo 12112
Egypt
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Source of Support: None, Conflict of Interest: None


DOI: 10.7123/01.EJH.0000434285.33634.83

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Background

Transfusion-chelation increased the longevity of patients with β-thalassemia major; thus, puberty and fertility disorders became more apparent.

Aim

We aimed to evaluate the pituitary–testicular axis and the progress of puberty after using deferiprone (DFP) and desferrioxamine (DFO) combined chelation in patients with preserved pituitary and testicular functions and to assess the quality of semen analysis in relation to chelation type.

Patients and methods

We carried out a 3-year prospective study of pubertal status including 42 polytransfused males (≥14 years) with thalassemia. Patients with delayed puberty were tested for pituitary–testicular axis function. Those with good pituitary and testicular response were shifted to combination chelation. Compliance on chelation and biannual serum ferritin were assessed. Spermiograms were performed at least twice for pubertal males.

Results

The median age of the participants was 20 years at enrollment, and 13 patients (30.9%) had normal puberty; they were on DFP 7/28, DFO 2/10, or combination 4/4 for at least 2 years before enrollment. Of the 29 nonpubertal males, 13 (44.8%) had good pituitary–testicular responses; within 3 years, 8/13 progressed to puberty using the combination chelation. Of the 21 pubertal males, 19 consented to spermiograms, with a low sperm count in 4/19 (21.1%) and poor motility in 6/19 (31.6%) patients, respectively.

Conclusion

Initial evaluation of adolescents and young adult thalassemic males showed that almost two-third were nonpubertal. Combination chelation (DFP and DFO) for those with good pituitary–testicular function led to progression of pubertal development after 3 years in half of them; meanwhile, semen quality was still impaired in one-third of them.



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