Department of Pediatrics, Faculty of Medicine, Cairo University, Ali Ibrahim Basha St., Cairo, Egypt.
Department of Clinical Pathology, Cairo University, Cairo, Egypt.
Indian J Pediatr. 2017 Oct;84(10):745-750. doi: 10.1007/s12098-017-2375-4. Epub 2017 Jun 10.
To investigate potential usefulness of serum hepcidin in the diagnosis of iron overload in children with β-thalassemia.
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.
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.
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.
探讨血清铁调素在β-地中海贫血患儿铁过载诊断中的潜在应用价值。
本研究纳入了 30 例重型地中海贫血（TM）患儿、30 例中间型地中海贫血（TI）患儿和 60 例健康儿童作为对照组。采用人铁调素 ELISA 试剂盒检测血清铁调素。
与对照组相比，β-地中海贫血患儿的血清铁调素水平更高（p<0.001）。TM 组的铁调素和铁蛋白水平均高于 TI 组（p=0.034；<0.001）。在对照组中，铁调素与年龄无相关性（r=0.225，p=0.084）。在β-地中海贫血患儿中，铁调素与年龄呈正相关（r=0.4；p=0.001）、疾病持续时间（r=0.5；p<0.001）、输血频率（r=0.35；p=0.007）、总输血量（r=0.4；p=0.003）和铁蛋白（r=0.3；p=0.027）呈正相关。总血红蛋白和血清铁蛋白与铁调素显著相关，血红蛋白每升高 1g/dl，铁调素升高 0.514ng/ml（p=0.023），血清铁蛋白每升高 1ng/ml，铁调素升高 0.002ng/ml（p=0.002）。铁过载[血清铁蛋白（SF）≥1500ng/ml]与 TM 独立相关（p=0.001），与血清铁调素升高相关（p=0.02）。与铁调素与铁蛋白比值相比，血清铁调素对严重铁过载的总体预测能力具有统计学意义。
β-地中海贫血患儿的血清铁调素升高；但在 TM 患儿严重铁过载时更为明显。因此，铁调素可作为 TM 患者严重铁过载的潜在标志物。建议进一步研究比较血清铁调素和铁蛋白在稳定期和感染或炎症期间预测严重铁过载的能力。
