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血色素沉着症中的放血疗法:它会影响微量元素稳态吗?

Bloodletting therapy in hemochromatosis: Does it affect trace element homeostasis?

作者信息

Bolann Bjørn J, Distante Sonia, Mørkrid Lars, Ulvik Rune J

机构信息

Department of Clinical Science, University of Bergen, Bergen, Norway; Laboratory of Clinical Biochemistry, Haukeland University Hospital, Bergen, Norway.

Department of Medical Biochemistry, Oslo University Hospital, Oslo, Norway.

出版信息

J Trace Elem Med Biol. 2015;31:225-9. doi: 10.1016/j.jtemb.2014.07.021. Epub 2014 Aug 7.

Abstract

Hemochromatosis is the most common hereditary disorder in the Nordic population, if left untreated it can result in severe parenchymal iron accumulation. Bloodletting is mainstay treatment. Iron and trace elements partially share cellular uptake and transport mechanisms, and the aim of the present study was to see if bloodletting for hemochromatosis affects trace elements homeostasis. We recruited patients referred for diagnosis and treatment of hemochromatosis, four women and 22 men 23-68 years of age. Thirteen were C282Y homozygote, one was C282Y heterozygote, three were H63D homozygote, seven were compound heterozygote and two had none of the mutations above. Iron and liver function tests were performed; serum levels of trace elements were measured using inductively coupled plasma mass spectrometry. Results before the start of treatment and after normalization of iron parameters were compared. On completion of the bloodlettings the following average serum concentrations increased: Co from 5.6 to 11.5 nmol/L, serum Cu 16.2-17.6 μmol/L, Ni increased from 50.0 to 52.6 nmol/L and Sb from 13.2 to 16.3 nmol/L. Average serum Mn concentration declined from 30.2 to 28.3 nmol/L. All changes were statistically significant (by paired t-test). B, Ba, Cs, Mo, Se, Sr and Zn were not significantly changed. We conclude that bloodlettings in hemochromatosis lead to changes in trace element metabolism, including increased absorption of potentially toxic elements.

摘要

血色素沉着症是北欧人群中最常见的遗传性疾病,若不治疗可导致实质器官铁蓄积。放血疗法是主要治疗手段。铁与微量元素部分共享细胞摄取和转运机制,本研究旨在观察血色素沉着症放血疗法是否会影响微量元素稳态。我们招募了因血色素沉着症前来诊断和治疗的患者,包括4名女性和22名年龄在23至68岁之间的男性。其中13人为C282Y纯合子,1人为C282Y杂合子,3人为H63D纯合子,7人为复合杂合子,2人无上述突变。进行了铁和肝功能检查;使用电感耦合等离子体质谱法测量血清微量元素水平。比较了治疗开始前和铁参数正常化后的结果。放血结束后,以下平均血清浓度升高:钴从5.6纳摩尔/升升至11.5纳摩尔/升,血清铜从16.2微摩尔/升升至17.6微摩尔/升,镍从50.0纳摩尔/升升至52.6纳摩尔/升,锑从13.2纳摩尔/升升至16.3纳摩尔/升。血清锰平均浓度从30.2纳摩尔/升降至28.3纳摩尔/升。所有变化均具有统计学意义(配对t检验)。硼、钡、铯、钼、硒、锶和锌无显著变化。我们得出结论,血色素沉着症放血疗法会导致微量元素代谢改变,包括潜在有毒元素吸收增加。

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