Long-term survival in patients with hereditary hemochromatosis
Long-term survival in patients with hereditary hemochromatosis
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DOI：
10.1053/gast.1996.v110.pm8613000
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发表时间：
1996-04-01
期刊：
影响因子：
29.4
通讯作者：
Strohmeyer, G
中科院分区：
文献类型：
--
作者：
Niederau, C;Fischer, R;Strohmeyer, G
背景与目的：遗传性血色素沉着症的病程可能取决于铁超负荷的程度和治疗干预的时间。该分析评估了早期诊断和除铁对遗传性血色病患者生存和并发症的影响。方法：对 251 名血色素沉着症患者进行了为期 14.1 +/- 6.8 年的随访，结果：与匹配的正常人群相比，整个患者组的生存率降低。非肝硬化和非糖尿病患者以及 1982 年至 1991 年间诊断的患者的生存率与预期相同。严重铁超负荷的患者的生存率降低。那些过载不太严重的人。 1947 年至 1969 年间，早期诊断的百分比比 1970 年至 1981 年间增加了三倍；过去十年仅进一步增加了 20%-25%。与预期相比，肝癌、心肌病、肝硬化和糖尿病导致的死亡有所增加。肝癌与肝硬化和可动铁量相关，但与乙型肝炎或丙型肝炎标志物无关。结论：血色素沉着症及其大多数并发症（包括肝癌）的预后取决于铁过量的量和持续时间。早期诊断和治疗在很大程度上可以预防铁过载的不良后果。
Background & Aims: The course of hereditary hemochromatosis may depend on the degree of iron overload and the time of therapeutic intervention. This analysis evaluates the impact of early diagnosis and iron removal on survival and complications in hereditary hemochromatosis. Methods: A cohort of 251 patients with hemochromatosis was followed up for 14.1 +/- 6.8 years, Results: Survival was reduced in the total group of patients when compared with a matched normal population. Survival in noncirrhotic and nondiabetic patients and in patients diagnosed between 1982 and 1991 was identical with rates expected. Survival was reduced in patients with severe iron overload us. those with less severe overload. The percentage of early diagnoses increased threefold between 1947 and 1969 to that between 1970 and 1981; there was only a further 20%-25% increase in the last decade. Deaths caused by liver cancer, cardiomyopathy, liver cirrhosis, and diabetes mellitus were increased as compared with expected rates. Liver cancers were associated with cirrhosis and amount of mobilizable iron but not with hepatitis B or C markers, Conclusions: Prognosis of hemochromatosis and most of its complications, including liver cancer, depend on the amount and duration of iron excess. Early diagnosis and therapy largely prevent the adverse consequences of iron overload.