Idiopathic hemochromatosis, an interim report.
Idiopathic hemochromatosis, an interim report.
复制标题
特发性血色素沉着病，临时报告。
作者：
M. Milder;J. Cook;S. Stray;C. Finch
总结了过去 20 年 34 例特发性血色病患者的治疗经验并回顾了文献。现在的方法对于铁超负荷的诊断非常有效，并且几乎所有诊断都是在生前进行的。通过放血去除铁，疾病的性质发生了变化。早期死亡仅限于患有严重且快速进展的心脏病的患者以及患有肿瘤的患者。主要死亡率已转移到更晚的时期，肝癌的发病率正在增加。目前，人们对家庭研究特别感兴趣，因为在家庭中经常发现过量的铁储存。中度铁超负荷的意义尚不清楚，但未来应注意早在临床表现出现之前识别铁超负荷。
Experience over the last 20 years with 34 patients with idiopathic hemochromatosis is summarized and the literature is reviewed. Methods are now available which are highly effective in the diagnosis of iron overload and virtually all diagnoses are made antemortem. The nature of the disease has changed through the removal of iron by phlebotomy. Early deaths are limited to patients with severe and rapidly progressive heart disease and to those presenting with neoplasm. The major mortality has shifted to a much later period and the incidence of hepatoma is increasing. There is particular interest at the present time in family studies since excessive iron stores are frequently found within the family. The significance of intermediate degrees of iron overload is unclear, but future attention should be given to the recognition of iron overload long before clinical manifestations appear.