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膳食性铁过载和特发性血色素沉着症中铁储存的模式。

Patterns of iron storage in dietary iron overload and idiopathic hemochromatosis.

作者信息

Brink B, Disler P, Lynch S, Jacobs P, Charlton R, Bothwell T

出版信息

J Lab Clin Med. 1976 Nov;88(5):725-31.

PMID:978039
Abstract

Bone marrow iron stores rise in proportion to the total body iron store in dietary iron overload. The situation in the genetic disorder of idiopathic hemochromatosis is not as clear. A method for measuring the storage iron concentration chemically on samples of bone marrow obtained by trephine needle biopsy was therefore developed. Its value as a measure of tissue iron stores was established in a preliminary investigation in which specimens of liver, spleen, and bone marrow were obtained at necropsies on 66 South African Negroes among whom dietary iron overload is common. A wide range of nonheme iron concentrations was found, but in each individual there was a highly significant correlation between the concentrations in the three tissues. Nonheme iron concentrations were then determined on trephine bone marrow biopsy specimens from eight Caucasian patients with untreated idiopathic hemochromatosis, and on percutaneous liver biopsy specimens from four of them. The concentrations in the livers were in the anticipated range of 5,000 mug per gram wet weight (2 per cent dry weight). In contrast the geometric mean value for bone marrow iron concentration was 186 mug per gram wet weight, a figure that fell below the fiftieth percentile for marrow iron concentrations in the South African Negroes, whereas the geometric mean liver iron concentration was above the ninetieth percentile. These findings indicate that subjects with idiopathic hemochromatosis whose liver iron stores are grossly increased do not show a comparable rise in bone marrow iron stores.

摘要

在膳食性铁过载情况下,骨髓铁储存量与全身铁储存量成比例增加。而在特发性血色素沉着症这种遗传性疾病中,情况则不太明确。因此,开发了一种通过环钻针吸活检获取的骨髓样本化学测量储存铁浓度的方法。在一项初步研究中确定了其作为组织铁储存量指标的价值,该研究对66名南非黑人进行尸检,获取肝脏、脾脏和骨髓样本,这些人膳食性铁过载情况较为常见。发现非血红素铁浓度范围很广,但在每个个体中,三种组织中的浓度之间存在高度显著的相关性。然后对8名未经治疗的特发性血色素沉着症白种人患者的环钻骨髓活检样本以及其中4人的经皮肝活检样本进行了非血红素铁浓度测定。肝脏中的浓度在预期范围内,即每克湿重5000微克(干重的2%)。相比之下,骨髓铁浓度的几何平均值为每克湿重186微克,这个数字低于南非黑人骨髓铁浓度的第50百分位数,而肝脏铁浓度的几何平均值则高于第90百分位数。这些发现表明,肝脏铁储存量大幅增加的特发性血色素沉着症患者,其骨髓铁储存量并未出现类似的增加。

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Patterns of iron storage in dietary iron overload and idiopathic hemochromatosis.膳食性铁过载和特发性血色素沉着症中铁储存的模式。
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