Assessment of sexual maturation in patients with β-thalassemia major receiving iron chelation therapy in Assuit University Hospital

Authors

Abstract

Background
As the survival of persons with thalassemia (TM) major has improved, extending into their third and fourth decades of life, growth, sexual development, and fertility have become crucial issues to address.
Aim and objectives
The aim of this work was to assess sexual maturation among patients with transfusion-dependent β-TM major on iron chelation therapy.
Patients and methods
This study was conducted at Assiut University Children's Hospital on previously diagnosed patients with transfusion-dependent TM undergoing chelation therapy.
Results
The study included 84 patients aged from 13 years in girls and 14 years in boys up to 18 years in both sexes. There were 38 (45.2%) females and 46 (54.8%) males. Overall, 59.6% of the cases had at least a Tanner stage 2 on the maturity rating scale appropriate to the age of onset of puberty (normal puberty), where 34 patients (40.5%) had no signs of puberty at the appropriate age of onset of puberty (delayed puberty).
Conclusion
Patients with transfusion-dependent TM major have a significant prevalence of delayed puberty, which is linked to a higher level of serum ferritin and poor adherence to chelation therapy. We advocate the use of modern treatment procedures, as well as transfusion optimization and chelation therapy, to reduce or eliminate such occurrences.

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