1 Department of Pediatrics, Division of Endocrinology, Hamad General Hospital, Doha, Qatar.
2 ICET-A Network (International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescence Medicine) and Pediatric and Adolescent Outpatient Clinic, Quisisana Hospital, Ferrara, Italy.
3 Hematology Department, Hamad General Hospital, Doha, Qatar.
World Journal of Advanced Research and Reviews, 2023, 18(02), 1063-1073
Article DOI: 10.30574/wjarr.2023.18.2.0955
DOI url: https://doi.org/10.30574/wjarr.2023.18.2.0955
Received on 11 April 2023; revised on 20 May 2023; accepted on 22 May 2023
Introduction: A recent review from 14 Mediterranean and Middle East countries (n =4477, mean age = 16.5 years) showed that the pooled prevalence of delayed puberty / hypogonadism in patients with BTM was 45.6%. Studying the consequences of hypogonadism and the potential benefits versus unwanted side effects of sex-steroid hormone replacement therapy (HRT) in children and adolescents with Beta Thalassemia Major (BTM) represent an important issue for these patients.
Objectives: We reviewed the literature (Pubmed, Google scholar, Scopus, Research gate) (1995: 2022) on the consequences of hypogonadism and benefits versus side effects of using sex Steroid therapy (HRT) in hypogonadal adolescents with BTM. Thirty-five papers were included and analyzed.
Results: HRT had a variable but significant beneficial effect on improving bone mineral density (BMD), pubertal growth spurt, development of secondary sexual characteristics and subsequently improved quality of life (QOL) in children and adolescents with BTM. Increased side effects of HRT in these patients may occur due to their state of hypercoagulability especially in splenectomized patients and those with cardiomyopathy, chronic hepatitis, and dysglycemia. Gynecomastia and local pain in the site of injection confirmed in a considerable number of males on testosterone therapy. Local reaction to sex steroid patches appeared in around 30% of patients. Based on this review recommendations for using HRT in these patients were updated.
Conclusion: Proper and wise use of sex steroid therapy in patients with BTM can significantly improve many consequences of hypogonadism.
Hypogonadism; Beta Thalassemia; Sex Steroid Therapy; Benefits; Side Effects.
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Ashraf Soliman, Vincenzo De Sanctis, Mohamed Yassin, Ahmed Khalil, Fawzia Alyafei, Shayma Ahmed, Nada Alaaraj and Noor Hamed. The potential benefits and risks of sex-steroids therapy in thalassemic patients with hypogonadism,. World Journal of Advanced Research and Reviews, 2023, 18(02), 1063-1073. Article DOI: https://doi.org/10.30574/wjarr.2023.18.2.0955
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