ESPE2023 Poster Category 2 Multisystem Endocrine Disorders (11 abstracts)
Consequences of Hypogonadism and Potential Benefits of Sex Steroid therapy (HRT) in Children and Adolescents with Beta Thalassemia Major (BTM).
Ashraf Soliman 1 , Mohamed Yassin 2 , Fawzia Alyafei 3 , Nada Alaaraj 3 & Nada Soliman 4
1Hamad General Hospital, Doha, Réunion. 2Hamad Medical Centre, Doha, Qatar. 3Hamad General Hospital, Doha, Qatar. 4North Dakota State University, Fargo, USA
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%.
Objectives: We reviewed the literature (Pubmed, Google scholar, Scopus, Research gate) (1995: 2022) on the consequences of hypogonadism and benefits of Sex Steroid therapy (HRT) in children and adolescents with BTM. 26 papers were included and analyzed.
Results: (table)
| Consequences of Hypogonadism in BTM | Potential Benefits of Sex Steroid therapy |
| Osteopenia and osteoporosis. The prevalence of osteoporosis and low bone mineral density (BMD) in BTM patients varies. The highest rate is 40-72% depending on age, studied parameter. Hypogonadal (untreated) BTM patients had the lowest BMD and the highest BMD were observed in patients on continuous HRT. | HRT can maintain BMD in BTM patients, with significant improvement of BMD Z score in thalassemic females who received HRT for around 5 years. The BMD values increased during the first 2-3 years of HRT treatment by (7.7% at lumbar spine and of 8.9% at femoral neck). |
| Short stature (disproportionate) 74 studies showed a pooled prevalence of short stature = 48.9%. Etiology: Disturbed GH-IGF1 axis, loss of pubertal growth spurt, and nutritional. | Low dose sex steroid therapy priming (6-12 months) induced pubertal maturation and increased growth spurt and final adult height in 80% of BTM adolescents. |
| Delayed/absent puberty, primary amenorrhea (PA) (47%) and secondary (SA) (23%) were reported in BTM. | 50% of BTM females with PA respond to 2-3 years of sex steroid treatment with a mature uterus and endometrial thickness. 45.2% had partial response and 4.8% failed to respond. |
| Decreased muscle mass (MM) and strength Decreased libido Decreased Quality of life (QOL) BTM adolescents had poor perception of their general health and scored significantly lower in all the subscales compared with the controls. The high prevalence of short stature and pubertal delay was associated with lowest scores for physical and psychological domains | HRT can increase MM and strength Increase libido and erectile function Improve QOL: A metanalysis confirmed the broad and sustained benefits of TRT across major QOL dimensions, including sexual, somatic, and psychological health, which were sustained over 36 months in the HRT treatment cohort |
Conclusion: Proper and wise use of sex steroid therapy in patients with BTM significantly improved many consequences of hypogonadism.
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