Meeting Report: The Equality Project on Endocrine Complications in Thalassemia: Selected Highlights from the First Turkish Congress, Antalya, 10th-11th December 2018.

Major difficulties reported by endocrinologists /pediatricians/ hematologists in the care of thalassemic patients with endocrine complications were: lack of facilities, correct interpretation of tests, unfamiliarity with medical treatment and the cost of diagnostics and therapeutics. Therefore, there is a felt need to educate and train more endocrinologists/pediatricians/hematologists in this field in order to optimise growth and prevent endocrine complications. To achieve this goal, in 2015, a project called Equality was submitted by three countries (Turkey, Spain and Italy) and approved by the European Union (EU) with the aim to train doctors and nurses, taking care of youth and young adults TM patients, in the prevention, diagnosis, and management of endocrine disorders. The selected highlights of the First Turkish Congress held in Antalya (10th-11th December 2018) are reported. Overall the conference provided a wide coverage of conventional treatment of thalassemias and endocrine complications in patients with ß-thalassemia major. Regular surveillance, early diagnosis, treatment and follow-up in a multi-disciplinary specialized setting are recommended.

Assessment of the relationship between fragmented QRS and cardiac iron overload in patients with beta-thalassemia major.

OBJECTIVE: Beta-thalassemia major (TM) is a genetic hemoglobin disorder causing chronic hemolytic anemia. Since cardiac insufficiency and arrhythmias are the primary causes of mortality in such patients, monitoring of cardiac iron load is important in management of the disorder. The purpose of this study was to investigate the importance of fragmented QRS (fQRS) and its relation to the cardiac T2* value for the evaluation of cardiac iron load in TM patients. METHODS: This retrospective study included 103 TM patients. The patients' T2* values, measured by cardiac MRI and 12-lead surface ECGs, were interpreted. The cardiac T2* values under 20 were considered as cardiac iron overload. The relationship between the cardiac T2* value and fQRS in ECG was investigated. RESULTS: The median age of the patients was 22.6 ± 6.6 years. All patients were on regular blood transfusions and iron chelators. The patients had no risk factors for coronary artery disease. In 50 (48%) patients fQRS was detected, and in 37 (74%) of these the T2* values were low. 86% of patients with cardiac involvement (37) had fQRS, but 22% of patients with non-involvement (13) had fQRS (p < 0.001). CONCLUSION: Since cardiac involvement is the primary cause of mortality in TM patients, the early diagnosis of cardiac dysfunction is of vital importance. The search for fQRS in the ECGs of these patients, particularly when cardiac T2* values cannot be determined and followed, is a non-expensive and easy-to-attain method for therapy management.