Chronically transfused pediatric sickle cell patients are protected from cardiac iron overload

Iron overload is a major toxicity of chronic transfusions. Myocardial iron overload is associated with cardiac dysfunction. Cardiac and liver magnetic resonance imaging (MRI) was performed on 14 chronically transfused sickle cell disease (SCD) and non-sickle cell disease (non-SCD) patients seen at Vanderbilt Children's Hospital from 1 January 2000 to 10 March 2010. Retrospective review was conducted to assess cardiac T2*, liver T2*, ventricular dimensions and function, echocardiogram, length of transfusion, hemoglobin, and ferritin measurements. Ten patients had SCD and 4 had non-SCD, including α-thalassemia, β-thalassemia, and Diamond-Blackfan anemia. Cardiac T2* was normal in all SCD patients (mean 39 ± 12 ms), but abnormal in 3 of 4 non-SCD patients (mean 11.8 ± 2.4 ms). Liver T2* was similar between SCD (mean 6.2 ± 1.6 ms) and non-SCD patients (mean 5.9 ± 1.9 ms), and did not correlate with serum ferritin. Comparing SCD and non-SCD patients with similar transfusion duration, SCD patients had normal cardiac T2* and non-SCD patients had abnormal cardiac T2*. No patients had cardiomyopathy, but ventricular dilatation was common among SCD patients. Chronically transfused pediatric SCD patients are relatively spared of myocardial iron overload, which is unlikely to be due to lower total body iron burden in SCD patients than non-SCD patients.     

重型β地中海贫血铁过载患者胰腺磁共振T2*检测及影响因素分析

目的探讨重型β地中海贫血(β-TM)患者胰腺铁过载状况及其与临床各监测指标之间的关系。方法 51例β-TM患者,进行胰腺MRI T2*检测。按是否有铁过载分为两组,组间比较性别、年龄、空腹血糖、血红蛋白(Hb);与血清铁蛋白(SF)、心脏T2*、肝脏T2*及输血时间进行相关性分析;比较铁螯合剂去铁胺联合去铁酮及单用地拉罗司(DFO+DFP组与DFX组)对胰腺T2*和心脏T2*的影响。结果 51例患者中有42例胰腺铁过载,占83%。胰腺铁过载与胰腺无铁过载组间比较,性别、年龄、空腹血糖、Hb差异无显著性(P>0.05);肝脏T2*、SF、输血时间与胰腺T2*之间呈低度相关(r值分别为-0.36、0.35及0.48);心脏T2*与胰腺T2*之间呈中度相关(r=0.50);心脏铁过载患者中91%有胰腺铁过载,89%胰腺无铁过载患者中心脏也无铁过载发生;DFX治疗组胰腺T2*显著高于DFO+DFP组(U=-2.23,P=0.03),但心脏T2*两组间差异无显著性(U=-0.67,P=0.51)。结论胰腺铁过载发生普遍;年龄、空腹血糖、是否足量输血对胰腺铁过载影响不明显;SF、肝脏T2*不能准确反映胰腺铁过载的情况,输血时间对胰腺铁过载影响不明显;胰腺与心脏有相同的铁动力学,可以通过胰腺铁过载来预测心脏铁过载;DFX可有效降低胰腺铁过载,但对心脏铁过载疗效有待进一步观察。     

重型β地中海贫血患者肝脏胰腺和心脏铁负荷磁共振成像T2*的多中心研究

目的 探讨使用磁共振成像T2*（MRI T2*）方法检测重型β地中海贫血（重型β地贫）患者肝脏、胰腺和心脏铁过载状况,并评估其有效性。方法 总结2010年5月至2010年12月中国广东及广西省共7所医院的153例重型β地贫患者的临床资料,对其肝脏、胰腺、心脏MRI T2*与年龄、血清铁蛋白作相关性分析,并按肝脏不同铁沉积等级比较患者心脏和（或）胰腺铁沉积的情况。结果 心脏铁沉积重度37例, 轻度21例, 正常95例。肝脏、胰腺和心脏MRI T2*值与血清铁蛋白水平有相关性,但与年龄无相关性,血清铁蛋白水平也与年龄无关。肝脏与胰腺T2*值（r = 0.529, P = 0.000）、肝脏与心脏的T2*值（r = 0.369,P = 0.000）以及胰腺与心脏T2*值（r = 0.715,P = 0.000）有相关性。随着肝脏铁沉积等级的增加,患者同时合并心和胰腺的比例也随之增大（χ2=20.78,P = 0.000）。结论 肝脏、胰腺、心脏三器官间的MRI T2*值有相关性。随着肝脏铁沉积等级增加,患者合并心脏、胰腺铁沉积的比例逐渐增高。     

T2* MRI in Regularly Transfused Children with Thalassemia Intermedia: Serum Ferritin Does Not Reflect Liver Iron Stores

Nontransfused patients with thalassemia intermedia (TI) accumulate iron due to increased gastrointestinal absorption of iron. Recent studies using T2* MRI revealed that serum ferritin does not reflect the severity of iron overload in nontransfused TI patients. We evaluated the iron overload status in TI children on monthly transfusion. Based on serum ferritin levels, 11 such patients (mean age 13.18 ± 4.09 years), were classified into two groups, group 1 (six patients) and group 2 (five patients) with serum ferritin levels below and above 1000 ng/mL, respectively. T2* MRI assessments were done for evaluation of hepatic and cardiac iron status. Group 1 and group 2 had mean serum ferritin levels of 817.300 ± 244.690 ng/mL and 1983.80 ± 662.862 ng/mL, respectively (P = .003). T2* MRI showed comparable moderate to severe hepatic iron overload status in both. None of the patients had myocardial iron deposition. We conclude that serum ferritin does not reflect the hepatic iron overload status in our patients with TI on regular transfusion.     

MRI T2*技术对中、重型β地中海贫血患者心脏及肝脏铁沉积状态的评估

目的：了解中、重型β地中海贫血患者体内铁沉积状况。方法：对39例中、重型β地中海贫血患者的输血、排铁的情况进行统计,检测患者体内铁蛋白水平,并运用MRI T2*技术检测心脏及肝脏铁沉积状况。结果：患者血清铁蛋白水平最低为1500 ng/mL,最高达 11491 ng/mL。肝脏铁重度沉积者15例（38%）,中度沉积者15例（38%）,轻度沉积者7例（18%）,正常者 2例（5%）。 心脏铁重度沉积者7例（18%）,轻度沉积者5例（13%）,正常者27例（69%）。1例出现心律紊乱症状,4例年龄超过20岁者均呈现性腺功能发育不全。大多患者因家庭经济原因未能进行规律输血及排铁治疗,且开始排铁时间较晚。患者血清铁蛋白水平与开始排铁的时间、剂量密切相关。结论：未进行早期规律的输血和排铁治疗的地中海贫血患者,体内铁的沉积发生年龄早,易早期出现重要器官的功能损害而引发相关并发症,应引起临床医师和患者家属的高度重视并制定相应的诊疗措施提高患者的生活质量。     

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??Abstracts?? Objective To study the T2* magnetic resonance imaging for evaluation of iron deposition of liver?? pancreas and heart in β- thalassemia major ??β-TM?? patients and the relationship of the serum ferritin with iron overload. Methods Measurement of hepatic?? pancreatic and cardiac MRI T2* was performed in 153 patients with β-TM from May 1?? 2010 to December 31?? 2010??The demographics and serum ferritin ??SF?? were collected. The rate of the iron overload in liver?? pancreas and heart was analyzed. The rates of combined pancreatic and cardiac iron overload in different hepatic iron overload groups were compared. Results According to MRI T2*?? there was serious hepatic iron overload in 85 cases?? moderate in 39?? mild in 21?? no iron overload in 8. Similarly?? there was pancreatic iron overload in 134?? no iron overload in 19. In terms of cardiac iron overload?? there was serious cardiac iron overload in 37 cases?? mild in 21?? no iron overload in 95. No correlation was found between hepatic?? pancreatic and cardiac MRI T2* and age or SF?? but hepatic MRI T2* correlated with pancreatic and cardiac MRI T2*??P = 0.000?? r = 0.529?? r = 0.369???? and pancreatic MRI T2* correlated with cardiac MRI T2* well ??P = 0.000?? r = 0.715??. As grade of hepatic iron overload increases?? the patients demonstrated higher rate of combination of pancreatic and cardiac iron overload simultaneously ??χ2 = 20.78??P = 0.000??.     

T2* MRI in regularly transfused children with thalassemia intermedia: serum ferritin does not reflect liver iron stores

Nontransfused patients with thalassemia intermedia (TI) accumulate iron due to increased gastrointestinal absorption of iron. Recent studies using T2* MRI revealed that serum ferritin does not reflect the severity of iron overload in nontransfused TI patients. We evaluated the iron overload status in TI children on monthly transfusion. Based on serum ferritin levels, 11 such patients (mean age 13.18 ± 4.09?years), were classified into two groups, group 1 (six patients) and group 2 (five patients) with serum ferritin levels below and above 1000?ng/mL, respectively. T2* MRI assessments were done for evaluation of hepatic and cardiac iron status. Group 1 and group 2 had mean serum ferritin levels of 817.300 ± 244.690?ng/mL and 1983.80 ± 662.862?ng/mL, respectively (P = .003). T2* MRI showed comparable moderate to severe hepatic iron overload status in both. None of the patients had myocardial iron deposition. We conclude that serum ferritin does not reflect the hepatic iron overload status in our patients with TI on regular transfusion.     

地拉罗司治疗重型β-地中海贫血铁过载患儿临床疗效及安全性研究

目的：探讨铁螯合剂地拉罗司（deferasirox,DFX）治疗重型β-地中海贫血（β-thalassemia major,β-TM）铁过载患儿的疗效及安全性。方法随机选择24例规律输血的β-TM铁过载患儿,参加DFX不同服药剂量的临床研究,调查血清铁蛋白（SF）的变化及不良反应。并将持续服用DFX 5年患儿与同期使用去铁胺联合去铁酮治疗患儿（对照组）的心脏MRI T2*、肝脏MRI T2*值进行比较。结果DFX每日20～30 mg/kg的起始剂量对于铁过载患儿无明显效果,加量至每日30～40 mg/kg 后SF水平下降显著(U=58,P＜0.01);不良反应以血清肝脏转氨酶升高最为常见,其次为血清肌酐非进行性升高。持续DFX 治疗5年组SF水平明显低于对照组（1748±481 ng/mL vs 3462±1744 ng/mL,P<0.05）;肝脏MRI T2* 值明显高于对照组（8.5±2.9 ms vs 2.7±1.9 ms,P<0.01）。两组心脏MRI T2*均值比较差异无统计学意义。结论DFX能显著降低β-TM 患儿SF水平,并显示出剂量依赖性变化;其对心脏铁负荷的减少未显示出明显优势,而对肝脏铁负荷的减低疗效显著。DFX治疗的不良反应以肝酶升高、血清肌酐非进行性升高为主。     

不同铁螯合剂治疗重型β地中海贫血铁过载的临床研究

目的 比较去铁胺(DFO)、去铁酮(DFP)、地拉罗司(DFX)三种不同铁螯合剂治疗重型β地中海贫血(β-TM)铁过载患者5年的临床疗效.方法 随机选择规律输血的β3-TM铁过载患者31人,按DFO、DFP和DFX分为三个治疗组,分别对血清铁蛋白(SF)、心脏MRIT2*、肝脏MRIT2*值变化进行分析.结果 服药5年后,三组SF均明显降低、心脏MRIT2*、肝脏MRIT2*明显升高(P＜0.05);组间比较,三组SF水平5年连续监测差异无显著性(P＞0.05);心脏MRIT2*组间比较差异无显著性(P＞0.05),但与基线相比,第2年DFP组心脏MRIT2*值上升幅度明显高于DFX组、DFO组(P＜0.05),后3年差异无显著性;5年连续监测肝脏MRIT2* DFO组高于DFP组、DFX组(P＜0.05),与基线相比,DFO组第2年肝脏MRIT2*值上升幅度明显高于DFP组、DFX组(P＜0.05),后3年差异无显著性.结论 三种铁螯合剂均能有效降低重型β-TM患者SF浓度、去除心脏和肝脏铁沉积;短期内DFP降低心脏铁负荷优于DFO及DFX,DFO降低肝脏铁负荷优于DFP及DFX.     

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目的探讨重型β珠蛋白生成障碍性贫血(beta-thalassaemia,简称β-TM)患儿长期输血、去铁治疗与铁过载的关系。方法深圳市第二人民医院2001年成立"地贫之友"与"地贫服务队",对β-TM患儿进行规范性的长期输血和去铁治疗。每3个月监测血清铁蛋白浓度(SF)、肝肾功能、心肌酶谱、心功能、心脏和肝脾B超、血糖和尿糖。2001年2月至2010年6月对其中51例患儿进行核磁共振检测心脏T2*、左心室射血分数(LVEF)、肝脏T2*、胰腺T2*和垂体T2*。根据治疗方法分为足疗程去铁胺+去铁酮(DFO+DFP)联合去铁治疗组(足疗程联合组)10例、不足疗程DFO+DFP联合去铁治疗组(不足疗程联合组)31例、单用足疗程地拉罗司(DFX)去铁治疗组(单用DFX组)10例。根据SF质量浓度分为SF≤2000μg/L组(A组)12例、SF~3000μg/L组(B组)17例、SF>3000μg/L组(C组)22例。结果各组LVEF、心脏T2*、垂体T2*值差异无统计学意义(P>0.05);足疗程联合组肝脏T2*高于不足疗程联合组(P<0.05),单用DFX组肝脏T2*、胰腺T2*、垂体T2*均高于足疗程联合组和不足疗程联合组(P<0.05)。足疗程联合组SF低于不足疗程联合组,单用DFX组SF低于足疗程联合组和不足疗程联合组,差异均具有统计学意义(P<0.05)。C组肝脏T2*和胰腺T2*明显低于A组和B组,差异具有统计学意义(P<0.05)。心肌铁过载11例(21.6%),肝脏铁过载43例(84.3%)。SF与心脏T2*无相关性(r=0.254,P>0.05),与肝脏T2*呈中度负相关(r=0.558,P<0.01)。结论足疗程DFO+DFP联合去铁治疗和单用足疗程DFX去铁治疗均能有效降低血清铁蛋白浓度,动员肝脏组织铁,效果优于不足疗程DFO+DFP联合去铁治疗。不同去铁方式均能减轻心脏铁过载。     

Can myocardial remodeling be a useful surrogate predictor of myocardial iron load? A 3D echocardiographic multicentric study

The relationship between myocardial iron load and eccentric myocardial remodeling remains an under‐investigated area; it was thought that remodeling is rather linked to fibrosis. This study aims to determine whether or not measures of remodeling can be used as predictors of myocardial iron. For this purpose, 60 patients with thalassemia were studied with 3D echocardiography and myocardial relaxometry (T2*) by Cardiac MRI. 3D derived sphericity index was significantly higher in patients with myocardial iron load. It was correlated with T2* with a 100% sensitivity and specificity (cut‐off value of 0.34) to discriminate between patients with and without myocardial iron overload.     

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??To study the status of myocardial T2* and liver T2* in β- thalassemia major??β-TM?? patients with iron overload and its relationship with clinical test data. Methods??In June 2010??on a voluntary basis??out of the 80 β-TM patients over 7 years under regular blood transfusion therapy??51 were chosen to receive myocardial MRI T2* ??myocardial T2*?? and liver MRI T2* ??liver T2*?? tests. The results were compared with age??SF??LVEF??transfusion time??chelation time and Hb. Results??Eleven out of 51 cases ??21.6%?? were myocardial iron overload??including 3 mild cases??3 moderate cases and 5 severe cases. Forty-three out of 51 cases ??84.3%?? were liver iron overload??including 14 mild cases??17 moderate cases and 12 severe cases. There was no correlation between myocardial T2* and SF??LVEF or liver T2*. SF was positively correlated with liver T2*??r = 0.558??P < 0.01??. The transfusion time of myocardial T2* > 20 ms group was less than that of myocardial T2* < 20 ms group ??P < 0.05??. There was no statistical significance between the liver iron overload incidence ratios of the two groups ??P > 0.05?? . Two out of 11 myocardial iron overload cases had lower LVEF??18.2%??. Conclusion??The group of TM patients demonstrates lower myocardial iron overload incidence and higher liver iron overload incidence. As SF increases??liver iron overload becomes more severe??myocardial iron overload can not be predicted or determined by examining SF level. There is no correlation between myocardial iron overload and liver iron overload. LVEF can not be a reliable factor to predict myocardial iron overload.     

Effect of Deferasirox Chelation on Liver Iron and Total Body Iron Concentration

Objective

To determine efficacy of Deferasirox (DFX) on total body iron and liver iron concentration (LIC) as estimated by serum ferritin (SF) and liver MRI T2*.

Methods

Thirty patients had baseline MRI T2* of the liver performed to determine LIC before starting DFX therapy and classified as normal >6.3 milliseconds (ms), mild 6.3?2.7 ms, moderate 2.7?1.4 ms and severe iron overload <1.4 ms. DFX was given 25–35 mg/kg/d. The serum ferritin (SF) level was estimated every 3 monthly. Liver iron is expressed as liver R2*?=?1000/T2*. The primary end point of the study was to determine change in SF and liver MRI R2* values after 18 mo of therapy.

Results

All 30 patients had some degree of liver iron overload; 11 (36.6 %) had severe, 15 (50 %) had moderate while 4 (13.3 %) had mild overload. The pre-DFX therapy median SF of all was 3604.5 ng/mL (IQR 2357.0–5056.0) and median liver R2* was 574.71 Hz (IQR 411.3–770.8). After 18 mo, SF dropped significantly to a median of 2036.5 ng/mL (IQR 1700.0–3162.0) (p?=?0.0011), while median liver R2* decreased from 574.71 to 568.18Hz (IQR 393.4–803.2) which was not significant (p?=?0.986).

Conclusions

DFX monotherapy at the doses used decreases total body iron, but does not significantly decrease liver iron. It is well tolerated by Indian thalassemia patients, with observed side effects including rash, diarrhea, and transient albuminuria. MRI T2* (and derived R2*) can serve as useful method in non invasive monitoring of LIC in thalassemia patient management.     

Serum ferritin underestimates liver iron concentration in transfusion independent thalassemia patients as compared to regularly transfused thalassemia and sickle cell patients

Serum ferritin (SF) and liver iron concentration (LIC), as measured by SQUID biosusceptometry, were assessed in a convenience sample of transfusion independent thalassemia patients (nTx-Thal, n=26), regularly transfused thalassemia (Tx-Thal, n=89), or sickle cell patients (SCD, n=45) to investigate the severity of iron overload and the relationship between SF and LIC in nTx-Thal compared to SCD and Tx-Thal. SF correlated with LIC (RS=0.53, P<0.001), but was found to be a poor predictor for LIC. SF was significantly lower (P<0.001) in nTx-Thal patients than in other groups, despite similar LIC values. The SF-to-LIC ratio was significantly lower in nTx-Thal compared to Tx-Thal and SCD patients (median of 0.32, 0.87, and 1.2, respectively: P<0.001). Due to underestimation of LIC by ferritin levels, chelation treatment may be delayed or misdirected in patients with thalassemia intermedia.     

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??Repeated blood transfusion therapy is the major cause of iron overload in thalassaemia major and increased GI absorption is more important in non-transfusion dependent thalassaemia??NTDT??. Iron overload is inevitable because the human body lacks a mechanism to excrete excess iron. Iron accumulation is toxic to many tissues??causing heart failure??cirrhosis??diabetes??growth retardation and multiple endocrine abnormalities. MRI T2* and MRI R2 technique is now validated as a true measure of cardiac iron and liver iron concentration respectively. Serum ferritin underestimates liver iron concentration in NTDT Patients. Assessment of iron overload status in NTDT patients should be done through liver iron concentration measurement.     

Comparative Assessment of Deferiprone and Deferasirox in Thalassemia Major Patients in the First Two Decades-Single Centre Experience

Iron overload is mainly responsible for the morbidity and mortality in patients with beta thalassemia major (TM). Our aim was to compare treatment outcomes with oral iron chelators, deferiprone (DFP), and deferasirox (DFX) in the first two decades on therapy. Seventy patients with TM (mean age ± SD, 7.9 ± 4.2; range 1.5–17 years) attending the pediatric day care unit for regular transfusional support were enrolled in this cross-sectional cohort study. The patients were treated either with DFP at the dose of 75–100 mg/kg/d in three divided doses after food or DFX at the dose of 25–40 mg/kg/d as single dose before food. Mean serum ferritin (±SD) was lower in patients below 10 years (n = 44) at 1283 (±600) ng/mL when compared with patients ≥10 years (n = 19) at 1546 (±589) ng/mL. There was no significant difference in mean serum ferritin (±SD) level in patients receiving DFP (1360 ± 589) versus DFX (1260 ± 641) in this cohort, P > 0.05. 67% of the patients had Vitamin D deficiency (<50 umol/L). Our results show comparable efficacy of DFP and DFX with regards to iron chelation as estimated by serial serum ferritin levels; however, MRI T2* values were higher in the DFP-treated patients compared to DFX treatment.     

Efficacy of hepatic T2* MRI values and serum ferritin concentration in predicting thalassemia major classification for hematopoietic stem cell transplantation

Liver biopsy has been performed for many decades for classifying the patients with TM. Meanwhile, using non‐invasive methods such as T2* MRI technique has been recently much more considered to determine the hepatic iron overload. Ninety‐three pediatric HSCT candidates with TM who underwent liver biopsy were included in this study. Hepatic T2* MRI values and serum ferritin concentrations were assessed to investigate and determine the useful method in detection of patients with TM class III whom received different conditioning regimens, in comparison with class I and II. Twenty (21.5%) patients were categorized as class III. Hepatic T2* MRI could detect TM class III patients with 60% sensitivity and 87.67% specificity (LR+: 4.867, accuracy: 81.72%), while predictive feature of ferritin values for distinguishing patients with TM class III was not statistically significant (p‐value >0.01). Combination of T2*MRI with age (T2*‐age) could detect TM class III with 85% sensitivity and 72.6% specificity (LR+: 3.1, accuracy: 75.27%).T2*‐age may be considered as an alternative and non‐invasive method to liver biopsy for differentiation and classification of patients with TM before transplantation.     

Serum Angiogenin Level in Sickle Cell Disease and Beta Thalassemia Patients

Introduction: Angiogenesis has been investigated in different kinds of anemia. However, its role as a marker of angiogenesis has not been investigated in thalassemia or sickle cell disease (SCD). Objectives: We aimed to investigate serum angiogenin level in children and adolescents with beta thalassemia or SCD and its relation to possible risk factors of angiogenesis. Materials and Methods: This study included; 32 β-thalassemia major (β-TM) patients aged 14.2 ± 3.8 years, 20 β-thalassemia intermedia (β-TI) patients aged 14.3 ± 4.8 years, 20 SCD patients aged 14.1 ± 2.4 years; 8 with (HbSS) and 12 with sickle thalassemia (HbS/β-thalassemia) and 35 age and sex-matched controls. Data collected regarding; age, sex, disease duration, blood transfusion frequency, transfusion index, chelation type and duration, CBC, Hb electrophoresis, serum ferritin and serum angiogenin level (by ELISA). Results: Angiogenin level was significantly higher in patients with SCD [250 (100–300) pg/mL] compared to β-TM [180 (140–230) pg/mL] and controls [89 (80–103) pg/mL] (P < .001) especially those with HbSS (P = .06). There was a significant negative correlation between serum angiogenin and age of patients, age of onset and duration of chelation in β-TM (P < .01, P < .001, P = .003) and β-TI (P = .009, P = .03, P < .001) and with serum ferritin in β-TI group (r = ?0.573, P = .008). In SCD, angiogenin level was negatively correlated with both frequency of blood transfusion (r = ?0.731, P < .001) and duration of hydroxyurea therapy (P = .017). Conclusions: High angiogenin level detected among patients with SCD may be negatively influenced by regular blood transfusion and hydroxyurea therapy, while; early onset of chelation therapy may decrease angiogenin level in β-TM.     

Serum Hepcidin as a Diagnostic Marker of Severe Iron Overload in Beta-thalassemia Major

Objectives

To investigate potential usefulness of serum hepcidin in the diagnosis of iron overload in children with β-thalassemia.

Methods

A study was conducted on 30 thalassemia major (TM), 30 thalassemia intermedia (TI) and 60 healthy children as controls. Serum hepcidin was measured by Human Hepcidin, ELISA Kit.

Results

β-thalassemia patients had a higher serum hepcidin compared to the controls (p < 0.001). TM group had higher hepcidin and ferritin compared to the TI group (p = 0.034; < 0.001, respectively). Among controls, hepcidin did not correlate with age (r = 0.225, p = 0.084). Among β-thalassemia patients, it correlated positively with age (r = 0.4; p = 0.001), disease duration (r = 0.5; p < 0.001), transfusion frequency (r = 0.35; p = 0.007), total number of transfusions (r = 0.4; p = 0.003), and ferritin (r = 0.3; p = 0.027). Total hemoglobin and serum ferritin were significantly related to hepcidin, which tended to increase by 0.514 ng/ml with each 1 g/dl rise in hemoglobin (p = 0.023) and by 0.002 ng/ml with each 1 ng/ml rise in serum ferritin (p = 0.002). Iron overload [serum ferritin (SF) ≥ 1500 ng/ml] was independently associated with TM (p = 0.001) and elevated serum hepcidin (p = 0.02). The overall predictability of serum hepcidin in severe iron overload was statistically significant when compared to hepcidin to serum ferritin ratio.

Conclusions

Serum hepcidin is elevated in children with β-thalassemia; but this elevation is more evident in TM patients with severe iron overload. Thus, hepcidin can be a potential marker of severe iron overload in patients with TM. Further studies are recommended to compare serum hepcidin and serum ferritin in the prediction of severe iron overload in steady state and during infection or inflammation.