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首页> 外文期刊>Mediterranean Journal of Hematology and Infectious Diseases >SEVERE LIVER IRON CONCENTRATIONS (LIC) IN 24 PATIENTS WITH Β-THALASSEMIA MAJOR: CORRELATIONS WITH SERUM FERRITIN, LIVER ENZYMES AND ENDOCRINE COMPLICATIONS
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SEVERE LIVER IRON CONCENTRATIONS (LIC) IN 24 PATIENTS WITH Β-THALASSEMIA MAJOR: CORRELATIONS WITH SERUM FERRITIN, LIVER ENZYMES AND ENDOCRINE COMPLICATIONS

机译：重型β-地中海贫血患者中的严重肝铁浓度（LIC）：与血清铁蛋白，肝酶和内分泌并发症的关系

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. Introduction: Chronic blood transfusion is the mainstay of care for individuals with β-thalassemia major (BTM). However, it causes iron-overload that requires monitoring and management by long-term iron chelation therapy in order to prevent endocrinopathies and cardiomyopathies, that can be fatal. Hepatic R2 MRI method (FerriScan?) has been validated as the gold standard for evaluation and monitoring liver iron concentration (LIC) that reflects the total body iron-overload. Although adequate oral iron chelation therapy (OIC) is promising for the treatment of transfusional iron-overload, some patients are less compliant with it and others suffer from long-term effects of iron overload. Objective: The aim of our study was to evaluate the prevalence of endocrinopathies and liver dysfunction, in relation to LIC and serum ferritin level, in a selected group of adolescents and young adult BTM patients with severe hepatic iron overload (LIC from 15 to 43 mg Fe/g dry weight). Patients and Methods: Twenty-four selected BTM patients with severe LIC, due to transfusion-related iron-overload, followed at the Hematology Section, National Center for Cancer Care and Research, Hamad Medical Corporation of Doha (Qatar), from April 2015 to July 2017, were retrospectively evaluated. The prevalence of short stature, hypogonadism, hypothyroidism, hypoparathyroidism, impaired fasting glucose (IFG), diabetes, and adrenal insufficiency was defined and assessed according to the International Network of Clinicians for Endocrinopathies in Thalassemia (ICET) and American Diabetes Association criteria. Results: Patients have been transfused over the past 19.75 ± 8.05 years (ranging from 7 to 33 years). The most common transfusion frequency was every 3 weeks (70.8%). At the time of LIC measurements, the mean age of patients was 21.75 ± 8.05 years, mean LIC was 32.05 ± 10.53 mg Fe/g dry weight (range: 15 to 43 mg Fe/g dry weight). Their mean serum ferritin level was 4,488.6 ± 2,779 μg/L. The overall prevalence of growth failure was 26.1% (6/23), IFG was 16.7% (4/24), sub-clinical hypothyroidism was 14.3% (3/21), hypogonadism was 14.3% (2/14), diabetes mellitus was 12.5% (3/24), and biochemical adrenal insufficiency was 6.7% (1/15). The prevalence of hepatitis C positivity was 20.8% (5/24). No case of clinical hypothyroidism, adrenal insufficiency or hypoparathyroidism was detected in this cohort of patients. The prevalence of IFG impaired fasting glucose was significantly higher in BTM patients with very high LIC (30 mg Fe/g dry liver) versus those with lower LIC (p = 0.044). LIC was correlated significantly with serum ferritin levels (r = 0.512; p = 0.011), lactate dehydrogenase (r = 0.744; p = 0.022) and total bilirubin (r = 0.432; p = 0.035). Conclusions: A significant number of BTM patients, with high LIC and endocrine disorders, still exist despite the recent developments of new oral iron chelating agents. Therefore, physicians’ strategies shall optimize early identification of those patients in order to optimise their chelation therapy and to avoid iron-induced organ damage. We believe that further studies are needed to evaluate if serial measurements of quantitative LIC may predict the risk for endocrine complications. Until these data are available, we recommend a close monitoring of endocrine and other complications, according to the international guidelines.
机译：。简介：慢性输血是重度β地中海贫血（BTM）患者的主要治疗手段。但是，它会导致铁超负荷，需要通过长期的铁螯合疗法进行监测和管理，以防止可能致命的内分泌病和心肌病。肝R2 MRI方法（FerriScan？）已被确认为评估和监测反映全身铁超负荷的肝铁浓度（LIC）的金标准。尽管适当的口服铁螯合疗法（OIC）有望用于治疗输血铁超负荷，但有些患者对其依从性较差，而另一些患者则长期遭受铁超负荷的困扰。目的：我们的研究目的是评估一组重度肝铁超负荷（LIC从15至43 mg）的青少年和年轻成人BTM患者的内分泌病变和肝功能障碍的发生率与LIC和血清铁蛋白水平的相关性。铁/克干重）。患者和方法：2015年4月至2004年4月，在多哈哈马德医疗公司国家癌症护理和研究中心血液学科，选择了24名因输血相关的铁超载而患有严重LIC的BTM严重LIC患者。 2017年7月，进行了回顾性评估。矮身材，性腺功能低下，甲状腺功能低下，甲状旁腺功能低下，空腹血糖受损（IFG），糖尿病和肾上腺功能不全的患病率根据国际地中海贫血症内分泌病临床医生网络（ICET）和美国糖尿病协会的标准进行了评估。结果：在过去的19.75±8.05年（7至33年）内，患者已经输血。最常见的输血频率是每三周一次（70.8％）。在进行LIC测量时，患者的平均年龄为21.75±8.05岁，平均LIC为32.05±10.53 mg Fe / g干重（范围：15至43 mg Fe / g干重）。他们的平均血清铁蛋白水平为4,488.6±2,779μg/ L。生长衰竭的总体患病率为26.1％（6/23），IFG为16.7％（4/24），亚临床甲状腺功能减退症为14.3％（3/21），性腺功能减退症为14.3％（2/14），糖尿病为12.5％（3/24），生化肾上腺皮质功能不全为6.7％（1/15）。丙型肝炎阳性的患病率为20.8％（5/24）。在这组患者中未发现临床甲状腺功能减退，肾上腺功能不全或甲状旁腺功能低下的病例。 LIC很高（大于30 mg Fe / g干肝）的BTM患者的IFG受损空腹血糖的发生率明显高于LIC较低的BTM患者（p = 0.044）。 LIC与血清铁蛋白水平（r = 0.512; p = 0.011），乳酸脱氢酶（r = 0.744; p = 0.022）和总胆红素（r = 0.432; p = 0.035）显着相关。结论：尽管最近出现了新型口服铁螯合剂的开发，但仍有大量具有高LIC和内分泌失调的BTM患者。因此，医生的策略应优化对这些患者的早期识别，以优化他们的螯合疗法并避免铁引起的器官损害。我们认为，需要进一步的研究来评估定量LIC的连续测量是否可以预测内分泌并发症的风险。在获得这些数据之前，我们建议根据国际准则对内分泌和其他并发症进行密切监测。

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