β（ベータ）サラセミアメジャー患者における内分泌系の機能異常：病院ベースの観察研究からの洞察

Background Beta-thalassemia major (BTM) predisposes children to several endocrine complications, primarily driven by iron overload and repeated transfusions. This study aimed to determine the prevalence, patterns, and risk factors of endocrine dysfunction among multi-transfused pediatric patients with BTM. Methods A hospital-based cross-sectional study was conducted at Rajendra Institute of Medical Sciences (RIMS), Ranchi, including 100 pediatric patients aged two to 14 years diagnosed with BTM. Clinical profiles, transfusion history, anthropometric parameters, and laboratory investigations, including HbA1c, thyroid profile, serum ferritin, ALP, calcium, phosphate, parathyroid hormone, and cortisol, were analyzed. Statistical tests were performed using IBM SPSS Statistics version 26, with p ≤ 0.05 considered significant. Results Endocrine disorders were highly prevalent: diabetes mellitus (14.6%), growth hormone deficiency (11.2%), hypothyroidism (9.8%), and hypoparathyroidism (6.3%). Mean HbA1c was 6.8 ± 1.2%, with significantly higher levels in patients with diabetes (p = 0.002), hypothyroidism (p = 0.035), and growth hormone deficiency (p = 0.018). Logistic regression showed that iron overload (p = 0.022), frequent transfusions (p = 0.059), and older age (>10 years) (p = 0.041) were major predictors of endocrine dysfunction, while chelation therapy did not show a strong protective effect (p = 0.072). Conclusion Multi-transfused BTM patients are at substantial risk of developing endocrine abnormalities due to cumulative iron overload and chronic transfusion exposure. Regular endocrine screening, optimized iron chelation, and individualized transfusion protocols are essential to prevent long-term metabolic and hormonal complications. Strengthening multidisciplinary care is crucial for improving growth, development, and overall outcomes in this vulnerable population.