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特发性血色素沉着症患者的肝脏和血清铁蛋白浓度

Hepatic and serum ferritin concentrations in patients with idiopathic hemochromatosis.

作者信息

Beaumont C, Simon M, Smith P M, Worwood M

出版信息

Gastroenterology. 1980 Nov;79(5 Pt 1):877-83.

PMID:7419012
Abstract

Hepatic iron and ferritin concentrations have been measured in needle biopsy specimens from patients with idiopathic hemochromatosis and from patients with other liver diseases. There was an excellent correlation between liver iron and ferritin concentrations in patients with miscellaneous liver diseases, but this was not found for the patients with hemochromatosis either before or after treatment. The proportion of liver iron bound to ferritin was lower in treated and untreated hemochromatosis than in the other patients. This probably reflects the formation of increasing amounts of hemosiderin with increasing iron deposition. Three patients in the early stage of idiopathic hemochromatosis were studied. These patients had high concentrations of hepatic iron and ferritin, but had serum ferritin concentrations within, or just above, the normal stage. These results suggest that, in the early stage of hemochromatosis, there is no failure of ferritin synthesis in the liver and that normal levels of serum ferritin are present either because the total body iron content is within the normal range or because the liver parenchymal cells are not, in the absence of liver damage, an important source of plasma ferritin.

摘要

已对特发性血色素沉着症患者和其他肝脏疾病患者的肝穿刺活检标本中的肝铁和铁蛋白浓度进行了测量。在患有各种肝脏疾病的患者中,肝铁和铁蛋白浓度之间存在良好的相关性,但在血色素沉着症患者中,无论治疗前还是治疗后均未发现这种相关性。与其他患者相比,经治疗和未经治疗的血色素沉着症患者中与铁蛋白结合的肝铁比例较低。这可能反映了随着铁沉积增加,含铁血黄素的形成量也在增加。对3例特发性血色素沉着症早期患者进行了研究。这些患者肝铁和铁蛋白浓度较高,但血清铁蛋白浓度在正常范围内或仅略高于正常范围。这些结果表明,在血色素沉着症早期,肝脏中铁蛋白的合成并未失败,血清铁蛋白水平正常要么是因为全身铁含量在正常范围内,要么是因为在没有肝损伤的情况下,肝实质细胞不是血浆铁蛋白的重要来源。

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