Chauhan Richa, Sharma Sunita, Chandra Jagdish
Department of Pathology, Lady Hardinge Medical College and Kalawati Saran Children's Hospital,New Delhi, India.
Indian J Pathol Microbiol. 2014 Jan-Mar;57(1):39-42. doi: 10.4103/0377-4929.130891.
Hepcidin, a key regulator of iron homeostasis, is increased by iron overload and inflammation while suppressed by hypoxia. In spite of iron overload in β-Thalassemia Major (β-TM), a paradoxical decrease in hepcidin is observed.
To assess the opposing effects of enhanced erythropoiesis due to anemia and iron overloading on hepcidin in β-TM patients.
This prospective observational study was done at our tertiary care hospital.
Eighty-three pediatric polytransfused (> 20 transfusions) patients of β-TM were compared with 70 children who served as controls. Serum assays for ferritin, transferrin receptors (sTfR) and hepcidin were performed.
Independent Student t test was used to compare variables between both the groups. A Pearson correlation coefficient was used to find any correlation between ferritin, sTfR and hepcidin.
The mean value of hepcidin in β-TM children was 13.88±10.68 ng/ml (range, 0.9-60 ng/ml) and showed significant negative correlation with sTfR (r = -0.296, P < 0.0066). However, there was no correlation of hepcidin with ferritin. Ferritin and sTfR were significantly elevated in β-TM children compared to controls (P < 0.001). The mean serum hepcidin/ferritin index in the study group (0.00552) was significantly lower (P value < 0.001) than the controls (0.378) thus indicating inappropriate levels of hepcidin to iron overload.
In polytransfused β-TM children increased iron demand dominates over iron overload in regulating hepcidin. In spite of excessive iron load, the inappropriate hepcidin levels may further contribute to iron overload enhancing iron toxicity.
铁调素是铁稳态的关键调节因子，铁过载和炎症会使其增加，而缺氧则会抑制它。尽管重型β地中海贫血（β-TM）存在铁过载，但铁调素却出现了矛盾性降低。
评估β-TM患者中因贫血导致的红细胞生成增加和铁过载对铁调素的相反作用。
这项前瞻性观察研究在我们的三级护理医院进行。
将83例接受多次输血（>20次输血）的β-TM儿科患者与70例作为对照的儿童进行比较。进行了铁蛋白、转铁蛋白受体（sTfR）和铁调素的血清检测。
采用独立样本t检验比较两组之间的变量。使用Pearson相关系数来发现铁蛋白、sTfR和铁调素之间的任何相关性。
β-TM儿童中铁调素的平均值为13.88±10.68 ng/ml（范围为0.9 - 60 ng/ml），与sTfR呈显著负相关（r = -0.296，P < 0.）。然而，铁调素与铁蛋白无相关性。与对照组相比，β-TM儿童的铁蛋白和sTfR显著升高（P < 0.001）。研究组的平均血清铁调素/铁蛋白指数（0.00552）显著低于对照组（0.378）（P值< 0.001），这表明铁调素水平与铁过载不匹配。
在多次输血的β-TM儿童中，增加的铁需求在调节铁调素方面比铁过载占主导地位。尽管铁负荷过高，但不适当的铁调素水平可能会进一步导致铁过载，增强铁毒性。
