血清血红蛋白作为重型地中海贫血症铁超载标志物的作用

Chaithra P., Pragalatha Kumar A., Aruna G., Hemalatha L.
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摘要

背景:重型β地中海贫血(BTM)是最常见的溶血性贫血。定期输血是一种基本的治疗方式,反复输血会导致铁超载及其并发症。研究发现,肝素是铁平衡的关键调节因子,在铁超载的重型地中海贫血患儿中,肝素明显升高。本研究的主要目的是了解血清肝素作为 BTM 儿童铁超载潜在标志物的作用,并对 BTM 儿童血清肝素和血清铁蛋白水平之间的关系进行相关分析。研究方法这是一项基于医院的前瞻性观察研究,于 2019 年 1 月至 2019 年 12 月在英迪拉-甘地儿童健康研究所进行,为期 12 个月。研究纳入了100名年龄介于2个月至18岁之间、经输血确诊为BTM的儿童,以及50名年龄和性别匹配的健康对照者。研究结果在研究组中,70%的儿童输血超过5次。总输血次数较多(超过 5 次)的儿童血清中位血红素水平(2.354 纳克/毫升)明显较高。此外,肝磷脂水平与总输血次数呈良好的正相关(r=0.608,p5 输血血清肝磷脂水平明显升高,血清肝磷脂与血清铁蛋白水平呈正相关)。因此,血钙素可被视为 BTM 患者铁负荷过重的潜在标志物。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Role of serum hepcidin as a marker of iron overload in beta thalassemia major
Background: Beta thalassemia major (BTM) is the most common hemolytic anemia. Regular blood transfusion is a basic treatment modality, recurrent blood transfusion which leads to iron overload and its complications. Hepcidin hormone is found to be a key regulator of iron homeostasis and is significantly increased in children of BTM with iron overload. The main objective of the study is to find out the role of serum hepcidin as a potential marker of iron overload in children with BTM, and to correlate the relationship between serum hepcidin and serum ferritin level in BTM children. Methods: This was a hospital based prospective observational study conducted at Indira Gandhi institute of child health for 12 months from January 2019 to December 2019. Included 100 children between age group of 2 months to 18 years diagnosed with BTM on blood transfusion and 50 age and sex matched healthy controls. Results: In the study group 70% children had >5 transfusions. The median serum hepcidin level (2.354 ng/ml) was significantly higher among those with higher number of total transfusions (>5 transfusions). In addition, hepcidin level showed good positive correlation with total number of transfusions (r=0.608, p<0.001). Also, serum hepcidin showed positive correlation with serum ferritin levels with 87% sensitivity and 88% specificity which was statistically significant (r=0.749, p<0.001). Conclusions: In the present study, BTM children who received >5 transfusions serum hepcidin level was significantly elevated and serum hepcidin showed positive correlation with serum ferritin levels. Thus, hepcidin can be considered as a potential marker of iron overload in patients with BTM.
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