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在16152例尸检中发现的患有严重多器官铁过载的白人和黑人之间的比较。

A comparison between whites and blacks with severe multi-organ iron overload identified in 16,152 autopsies.

作者信息

Barton James C, Acton Ronald T, Anderson Laura E, Alexander C Bruce

机构信息

Southern Iron Disorders Center, Birmingham, Alabama, USA.

出版信息

Clin Gastroenterol Hepatol. 2009 Jul;7(7):781-785.e2. doi: 10.1016/j.cgh.2009.03.016. Epub 2009 Mar 21.

Abstract

BACKGROUND & AIMS: Little is known about differences in the prevalence of severe iron overload at death in whites and blacks. We evaluated data and samples from 16,152 autopsies (8484 whites, 7668 blacks) performed at a single university hospital.

METHODS

Cases of severe multi-organ iron overload were identified by review of autopsy protocols and Perls-stained tissue specimens, analysis of hepatocyte and Kupffer cell iron levels, and measurement of liver tissue iron concentrations.

RESULTS

We analyzed autopsy data from 10,345 adults (age > or =21 years), 1337 children (1-20 years), and 4470 infants (<1 year). Iron overload without reports of excessive exogenous iron was observed in 18 adults; the prevalence in whites and blacks was 0.0019 and 0.0015, respectively (P = .6494). Twenty-nine adults and 2 children had iron overload with reports of excessive exogenous iron. In adults, the prevalences of iron overload with reports of excessive exogenous iron in whites and blacks were 0.0040 and 0.0013, respectively (P = .0107). Among adults, the prevalence of cirrhosis was 6-fold greater in those with iron overload. In adults with severe iron overload, 67% without reports of excessive exogenous iron and 14% with reports of excessive exogenous iron died of hepatic failure or cardiomyopathy caused by siderosis. The overall prevalence of deaths caused by severe iron overload in whites and blacks was 0.0021 and 0.0009, respectively (P = .0842).

CONCLUSIONS

The prevalence of severe iron overload without reports of excessive exogenous iron did not differ significantly between whites and blacks. The prevalence of iron overload with reports of excessive exogenous iron was greater in whites. Hepatic failure and cardiomyopathy were common causes of death in severe iron overload cases.

摘要

背景与目的

关于白人和黑人死亡时严重铁过载患病率的差异,人们了解甚少。我们评估了在一所大学医院进行的16152例尸检(8484例白人,7668例黑人)的数据和样本。

方法

通过审查尸检方案和普鲁士蓝染色的组织标本、分析肝细胞和库普弗细胞铁水平以及测量肝组织铁浓度,确定严重多器官铁过载病例。

结果

我们分析了10345例成年人(年龄≥21岁)、1337例儿童(1 - 20岁)和4470例婴儿(<1岁)的尸检数据。在18例成年人中观察到无过量外源性铁摄入报告的铁过载;白人和黑人中的患病率分别为0.0019和0.0015(P = 0.6494)。29例成年人和2例儿童有过量外源性铁摄入报告的铁过载。在成年人中,有过量外源性铁摄入报告的铁过载在白人和黑人中的患病率分别为0.0040和0.0013(P = 0.0107)。在成年人中,铁过载患者的肝硬化患病率高6倍。在严重铁过载的成年人中,67%无过量外源性铁摄入报告的患者和14%有过量外源性铁摄入报告的患者死于铁沉着症引起的肝衰竭或心肌病。白人和黑人中由严重铁过载导致死亡的总体患病率分别为0.0021和0.0009(P = 0.0842)。

结论

无过量外源性铁摄入报告的严重铁过载患病率在白人和黑人之间无显著差异。有过量外源性铁摄入报告的铁过载患病率在白人中更高。肝衰竭和心肌病是严重铁过载病例常见的死亡原因。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f6d4/3931257/71f601a3c37a/nihms104339f1.jpg

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