Eur J Endocrinol. 2013 Oct 21;169(6):785-93. doi: 10.1530/EJE-13-0627. Print 2013 Dec.
Endocrine complications characterised patients with β thalassaemia (βT). In particular, thyroid dysfunction occurs frequently in βT major, but its long-term natural history is poorly understood.
A total of 72 βT patients were followed for 8 years. The incidence of thyreopathies, defined as the primary study endpoint, was assessed. The aim of this study was to analyse the prognostic role of ferritin for thyreopathies in patients with major and intermedia βT. The power of different iron chelators to treat iron overload and to prevent or reverse thyreopathies was also assessed.
Patients were treated with chelators with different chelation strategies during the study. Receiver operating characteristics analysis was employed to calculate the area under the curve for serum ferritin to find the best cutoff values capable of identifying thyroid dysfunction in thalassaemic patients. Kaplan-Meier curves were generated to assess incidence of thyreopathy. Adjusted risk estimates for thyreopathy were calculated using univariate followed by multivariate Cox proportional hazard regression analysis.
PATIENTS WITH THYROID DYSFUNCTION WERE CHARACTERISED BY HIGHER FERRITIN WHEN COMPARED WITH PATIENTS WITHOUT THYREOPATHIES (1500 (8722336) VS 513 (370698) G/L; P0.0001). PATIENTS WITH FERRITIN VALUES ABOVE 1800G/L EXPERIENCED A SIGNIFICANTLY FASTER EVOLUTION TO ENDPOINT (LOG-RANK ((2)): 7.7; P=0.005). Ferritin predicted high risk of thyroid dysfunction independently of confounding factors (hazard ratio: 1.20; P<0.0001). The intensification of chelation therapy led to an amelioration of thyroid function.
Ferritin represents a prognostic marker for βT patients and a predictive factor for progression to thyroid dysfunction. Intensive chelation therapy allows the prevention and reversibility of thyroid complications.
β地中海贫血(βT)患者的内分泌并发症具有特征性。特别是甲状腺功能障碍在βT 重型中经常发生,但对其长期自然史了解甚少。
对 72 名βT 患者进行了 8 年的随访。评估了甲状腺疾病的发生率,这是主要的研究终点。本研究旨在分析铁蛋白在βT 重型和中间型患者甲状腺疾病中的预后作用。还评估了不同铁螯合剂治疗铁过载和预防或逆转甲状腺疾病的能力。
在研究期间,患者接受了不同螯合策略的螯合剂治疗。采用受试者工作特征分析计算血清铁蛋白的曲线下面积,以找到能够识别地中海贫血患者甲状腺功能障碍的最佳截断值。生成 Kaplan-Meier 曲线以评估甲状腺疾病的发生率。使用单变量和多变量 Cox 比例风险回归分析计算甲状腺疾病的调整风险估计。
与无甲状腺疾病的患者相比,甲状腺功能障碍患者的铁蛋白更高(1500(8722336)比 513(370698)μg/L;P<0.0001)。铁蛋白值高于 1800μg/L 的患者达到终点的速度明显更快(对数秩检验(2):7.7;P=0.005)。铁蛋白独立于混杂因素预测甲状腺功能障碍的高风险(风险比:1.20;P<0.0001)。螯合治疗的强化导致甲状腺功能得到改善。
铁蛋白是βT 患者的预后标志物和进展为甲状腺功能障碍的预测因素。强化螯合治疗可预防和逆转甲状腺并发症。