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<p>Transfusion-dependent beta-thalassemia (TDT) is frequently associated with endocrine dysfunction secondary to hemosiderosis involving multiple organs. This study aimed to evaluate long-term endocrine dysfunction, neuroradiological manifestations, and biochemical markers of iron overload in adult TDT subjects, alongside analyzing associations between pituitary siderosis and endocrine deficits. A cross-sectional study was conducted on 63 adult TDT subjects treated at a tertiary care center in North India. Patient history, clinical examination, biochemical evaluation, and neuroimaging findings were recorded. Hormonal assessments included insulin-like growth factor-1 (IGF-1), growth hormone (GH), thyroid function, cortisol, adrenocorticotropic hormone, sex steroids, and gonadotropins. Dynamic hormone testing was conducted to assess GH status (<i>n</i> = 44) and adrenal reserve (<i>n</i> = 45). Iron deposition in the brain, liver, and heart was assessed radiologically using MRI. Sixty-three subjects (43 males), median age 27 years (IQR: 22–30), were analyzed. Most (59/63) had thalassemia major (TM); the remainder had thalassemia intermedia (TI). Endocrine deficits included low IGF-1 (75.8%), low DHEAS (73%), hypocortisolism (64.5%), hypogonadism (58%), growth hormone deficiency on provocation test (56%), and central hypothyroidism (24%). MRI revealed pituitary siderosis in 76.6%, hepatic siderosis in 53.4%, and cardiac siderosis in 38.2%. Calvarial thickening and diploic space widening in 81% each were common findings. No significant association was observed between pituitary siderosis, pituitary height, and endocrine deficits. Most TDT subjects experience hypothalamo-pituitary dysfunction despite chelation therapy, emphasizing the need for close monitoring. While MRI is valuable for assessing systemic hemosiderosis, its utility in predicting endocrine dysfunction is limited.</p>

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Hypothalamo-Pituitary Dysfunction and Association with Pituitary Siderosis in Adult Subjects with Transfusion-Dependent Beta-Thalassemia: all Is not Dark

  • 期刊论文

  • 发表日期：

    2025年10月13日

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  • Subin Siddiqui,
  • Jayaditya Ghosh,
  • Chirag K. Ahuja,
  • Alka Khadwal,
  • Liza Das,
  • Paramjeet Singh,
  • Ravi Shah,
  • Durairaj Arjunan,
  • Debajyoti Chatterjee,
  • Pankaj Malhotra,
  • Sanja Medenica,
  • Pinaki Dutta

摘要

Transfusion-dependent beta-thalassemia (TDT) is frequently associated with endocrine dysfunction secondary to hemosiderosis involving multiple organs. This study aimed to evaluate long-term endocrine dysfunction, neuroradiological manifestations, and biochemical markers of iron overload in adult TDT subjects, alongside analyzing associations between pituitary siderosis and endocrine deficits. A cross-sectional study was conducted on 63 adult TDT subjects treated at a tertiary care center in North India. Patient history, clinical examination, biochemical evaluation, and neuroimaging findings were recorded. Hormonal assessments included insulin-like growth factor-1 (IGF-1), growth hormone (GH), thyroid function, cortisol, adrenocorticotropic hormone, sex steroids, and gonadotropins. Dynamic hormone testing was conducted to assess GH status (n = 44) and adrenal reserve (n = 45). Iron deposition in the brain, liver, and heart was assessed radiologically using MRI. Sixty-three subjects (43 males), median age 27 years (IQR: 22–30), were analyzed. Most (59/63) had thalassemia major (TM); the remainder had thalassemia intermedia (TI). Endocrine deficits included low IGF-1 (75.8%), low DHEAS (73%), hypocortisolism (64.5%), hypogonadism (58%), growth hormone deficiency on provocation test (56%), and central hypothyroidism (24%). MRI revealed pituitary siderosis in 76.6%, hepatic siderosis in 53.4%, and cardiac siderosis in 38.2%. Calvarial thickening and diploic space widening in 81% each were common findings. No significant association was observed between pituitary siderosis, pituitary height, and endocrine deficits. Most TDT subjects experience hypothalamo-pituitary dysfunction despite chelation therapy, emphasizing the need for close monitoring. While MRI is valuable for assessing systemic hemosiderosis, its utility in predicting endocrine dysfunction is limited.