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原发性和继发性铁储存疾病患者十二指肠铁摄取的体外研究。

In vitro studies of duodenal iron uptake in patients with primary and secondary iron storage disease.

作者信息

Cox T M, Peters T J

出版信息

Q J Med. 1980;49(195):249-57.

PMID:7465761
Abstract

Initial rates of iron uptake into fresh specimens of human duodenal mucosa incubated with radio-iron in vitro were examined over a range of physiological iron concentrations. In normal control subjects and patients with iron overload, mucosal iron uptake showed saturation kinetics, thus providing evidence for a carrier-mediated transport process. In five patients with idiopathic haemochromatosis uptake at the lower concentrations of iron was increased up to 80 per cent when compared with 12 control subjects. Uptake at the highest medium iron concentration was not increased. These changes were related to a significant reduction in the apparent affinity constant (Kt) for iron transport and were independent of iron stores. In contrast, the uptake rates in six patients with secondary haemochromatosis were not different from controls except in a subgroup with severe iron-loading anaemias where uptake was modestly enhanced at the higher concentrations only. The studies point to a disorder of mucosal iron transport in idiopathic haemochromatosis which appears to be a primary abnormality rather than a consequence of the iron overload.

摘要

在一系列生理铁浓度范围内,检测了用放射性铁体外孵育的人十二指肠黏膜新鲜标本中铁摄取的初始速率。在正常对照受试者和铁过载患者中,黏膜铁摄取呈现饱和动力学,从而为载体介导的转运过程提供了证据。与12名对照受试者相比,5名特发性血色素沉着症患者在较低铁浓度下的摄取增加了80%。在最高的中等铁浓度下摄取并未增加。这些变化与铁转运的表观亲和常数(Kt)显著降低有关,且与铁储存无关。相比之下,6名继发性血色素沉着症患者的摄取率与对照组无差异,只是在一个严重铁负荷性贫血的亚组中,仅在较高浓度下摄取略有增加。这些研究表明,特发性血色素沉着症存在黏膜铁转运紊乱,这似乎是一种原发性异常,而非铁过载的结果。

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