Effects of plasma transfusion on hepcidin production in human congenital hypotransferrinemia
Effects of plasma transfusion on hepcidin production in human congenital hypotransferrinemia
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DOI：
10.3324/haematol.11377
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发表时间：
2007-10-01
期刊：
影响因子：
10.1
通讯作者：
Piperno, Alberto
中科院分区：
文献类型：
--
作者：
Trombini, Paola;Coliva, Tiziana;Piperno, Alberto
铁调素是全身铁稳态的关键调节剂。我们描述了先天性低转铁蛋白血症患者输注血浆诱导的铁调素产生的调节，该模型为研究铁调素和红细胞生成调节铁调素的机制提供了一个独特的模型。当血红蛋白和血清转铁蛋白较低时，尿铁调素从基线时的零增加到第 60 天的最高 98 ng/mg 肌酐，然后下降。尿铁调素和血清转铁蛋白浓度的时程表明，铁调素的产生受到骨髓铁需求和转铁蛋白铁供应的结合的调节。
Hepcidin is the key regulator of systemic iron homeostasis. We describe the modulation of hepcidin production induced by plasma transfusions in a patient with congenital hypotransferrinemia that offers a unique model in which to study the mechanism of hepcidin regulation by iron and erythropoiesis. Urinary hepcidin increased from zero at baseline, when hemoglobin and serum transferrin was low, to a maximum of 98 ng/mg creatinine on day 60, and subsequently decreased. Time-course of urinary hepcidin and serum transferrin concentration suggests that hepcidin production is regulated by the combination of marrow iron requirements and iron supply by transferrin.