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Abstract Objective: The study aims to assess the endocrine and metabolic complications in a cohort of regularly transfused and chelated beta thalassemia major patients. Methods: This is a retrospective cross-sectional study in adults aged >13years, with beta thalassemia major who were treated at a tertiary center between January 2013 to December 2022 with regular blood transfusion and chelation therapy. Iron overload was monitored by serial serum ferritin (SF) along with cardiac and liver T2* studies by MRI. Patients also underwent a comprehensive endocrine and metabolic bone disease evaluation, including screening for delayed puberty, hypogonadism, altered glucose metabolism with diabetes mellitus, hypothyroidism, hypoparathyroidism and adrenal insufficiency. Bone mineral density was evaluated by dual energy X-ray absorptiometry (DEXA) scanning along with serial serum calcium, alkaline phosphatase and 25(OH) Vitamin D studies. Results: Out of 91 patients (41 males) who are transfusion-dependent, the median (IQR) age was 33 (9) years. Hypogonadism was the commonest endocrine deficiency seen in 47 (51.6%), followed by abnormal glucose metabolism in 43 (47.3%), low bone mineral density in 34 (37.4%), delayed puberty in 31 (34.1%), hypothyroidism in 17 (18.7%), and hypoparathyroidism in 9 (9.9%) patients. Diabetes mellitus was seen in 17 (18.7%) patients with IDDM in 10(10.9%). Although, 25 (27.5%) patients had no endocrinopathy, 66 (72.5%) had at least one endocrinopathy. Conclusion: Tissue iron overload is an inevitable eventuality due to the current standard of care of beta thalassemia major patients. Hence, early screening and robust monitoring are essential to prevent complications of iron overload leading to endocrinopathies.
Rahbi et al. (Mon,) studied this question.