Objective: To investigate the prevalence of hypogonadism in patients with β-Thalassemia Major (β-TM) and explore the risk factors.
Method: 42 β-TM patients (≥= 10 years old) were investigated by questionnaire, physical examination and laboratory examination to evaluate their stages of puberty development and sex hormone level.And then,effects of age, the beginning age of chelation, iron overload, genotype and other factors on pubertal development in β-TM patients were analyzed.
Result: Hypogonadism was one of the most common endocrine complications in β-TM patients and the incidence rate was 57.14%, and there was no difference between male and female. Age, the beginning age of chelation, serum ferritin, hepatic T2*,cardiac T2*,genotype, liver function, vitamin D level and diabetes mellitus were important factors influencingpuberty developmentof β-TM patients. The multivariate logistic regression analysis showed that cardiac T2* and age were independent risk factors for hypogonadism in β-TM patients.
Conclusion: Hypogonadotrophic hypogonadism is the most common endocrinopathy inβ-TMpatients.Although it is rarely life threatening, it canseverely impair the quality of life.Iron overload is a significant risk factor.Hence, proper and effective iron chelation therapyis essential.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology