ESPE Abstracts

Thalassemia is a common genetic blood disease that is characterized by a decrease or absence of normal globin chain synthesis. This results in an imbalance of alpha and beta globin chain synthesis, leading to ineffective erythropoiesis and hemolysis, which causes anemia. The severe form of thalassemia is called thalassemia major and is usually associated with lifelong transfusion-dependent anemia. The incidence rate of thalassemia is higher in the Middle East, and it is a major public health issue in the UAE, where 8.7% of the population having beta thalassemia major. Despite recent improvements in chelation therapy to reduce iron overload, endocrine disorders remain a serious problem that threatens the quality of life for thalassemia patients.

Objectives: To determine the prevalence of endocrine disorders in thalassemia patients under the age of 18 who receive regular blood transfusions at the Dubai Thalassemia Center from January 2017 to January 2022.

Methods: We conducted a retrospective, cross-sectional study using electronic medical records to identify 62 thalassemia patients under the age of 18 who were referred to the thalassemia endocrine clinic at the Dubai Thalassemia Center between January 2017 and January 2022. Of these patients, 46 had active endocrine issues.

Results: Out of the 62 thalassemia patients who were referred to the endocrine clinic for a routine check- up, 46 had active endocrine complaints. Among the overall studied population, gender-wise, more than half of the population were male (56%), and UAE nationals were more than non-UAE nationals (74%). Among those that developed endocrine disorders, the most frequent complaint was short stature (25%) followed by failure to thrive (16.1%). The frequency of endocrine disorders was more common in UAE nationals than non-UAE nationals (73%). The mean age of developing short stature was 13.31 (± 3.281), and failure to thrive was 11.9 (±1.912). Age is shown to be a clinically significant factor with a P-value of 0.054, but it is not statistically significant.

Conclusion & recommendations: The results of our study showed that children with BTM are not immune to endocrine disorders. To improve their quality of life, it is important to detect these disorders early and manage them through a protocol-based, multidisciplinary approach. This can be achieved through regular surveillance, early detection and treatment, as well as collaborative follow-up with a team of specialists. By implementing these measures, we can reduce the frequency and severity of endocrine complications in children with BTM.