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患儿高钙保护蛋白血症和高锌血症时的组织锌水平:病例报告及文献复习。

Tissue zinc levels in a child with hypercalprotectinaemia and hyperzincaemia: A case report and a review of the literature.

机构信息

Department of Pediatrics, Örebro University Hospital, Orebro, Sweden.

出版信息

Scand J Clin Lab Invest. 2012 Feb;72(1):34-8. doi: 10.3109/00365513.2011.623177. Epub 2011 Oct 21.

Abstract

BACKGROUND

A girl suffering from a rare syndrome of unknown aetiology, termed hypercalprotectinaemia, was evaluated for tissue zinc status, because calprotectin is a protein which chelates Zn at multiple binding-sites, which might have affected the distribution of Zn in her body.

METHODS

Measurement of serum, urine, hair and nail zinc (Zn) concentration, complemented with measurement of total Zn in ultrafiltrates of plasma.

RESULTS

Her serum Zn concentration was 105-133 μmol/L. Zn levels in her hair (102 μg/g), nail (90 μg/g) and urine (3-12 μmol/L; 20-80 μg/dL) were all at the lower end of the reference intervals described in the sparse literature. Zn concentrations in ultrafiltrates of plasma were below the detection limit (<100 nmol/L). Thus, the elevated serum Zn did not translate into a similarly increased level of Zn in any of the tissues tested, nor in free Zn concentrations. Instead it appeared to be a result of Zn being chelated to binder proteins, most probably calprotectin.

CONCLUSION

Her grossly elevated serum calprotectin concentration is probably able to raise circulating total Zn concentrations without raising ionized concentrations, but this Zn remains confined to the circulating blood as well as to excreted body fluids, particularly faeces.

摘要

背景

一名患有病因不明的罕见综合征的女孩,被称为高钙蛋白血症,她的组织锌状态受到评估,因为钙保护蛋白是一种在多个结合部位螯合 Zn 的蛋白质,这可能影响了她体内 Zn 的分布。

方法

测量血清、尿液、头发和指甲中的锌(Zn)浓度,并测量血浆超滤液中的总 Zn。

结果

她的血清 Zn 浓度为 105-133 μmol/L。她的头发(102 μg/g)、指甲(90 μg/g)和尿液(3-12 μmol/L;20-80 μg/dL)中的 Zn 水平均处于稀疏文献中描述的参考区间的低端。血浆中超滤液中的 Zn 浓度低于检测限(<100 nmol/L)。因此,升高的血清 Zn 并未转化为任何测试组织中 Zn 水平的相应增加,也未转化为游离 Zn 浓度的增加。相反,它似乎是 Zn 与结合蛋白(最可能是钙保护蛋白)螯合的结果。

结论

她的血清钙保护蛋白浓度明显升高可能能够提高循环总 Zn 浓度而不提高离子化浓度,但这种 Zn 仍然局限于循环血液和排泄的体液,特别是粪便。

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