HEPATIC PATHOLOGY IN RELATIVES OF PATIENTS WITH HAEMOCHROMATOSIS
HEPATIC PATHOLOGY IN RELATIVES OF PATIENTS WITH HAEMOCHROMATOSIS
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DOI：
10.1002/path.1700840107
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发表时间：
1962-01-01
期刊：
影响因子：
--
通讯作者：
MUIR, AR
中科院分区：
文献类型：
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作者：
SCHEUER, PJ;WILLIAMS, R;MUIR, AR
本文对16例血色病患者的46例近亲属的肝活检进行了光镜和电镜观察。通过后者的铁蛋白含量的肝脏进行了评估。46例活检中有34例（74%）显示铁过量; 24例存在脂肪改变，7例存在再生、纤维化或肝硬化。20例肝细胞脂褐素过多。电子显微镜显示脂褐素和铁蛋白之间的密切联系，并建议脂褐素形成含铁血黄素颗粒的基础。一般认为，亲属中的肝铁质沉着症不一定是血色沉着症的前兆。200例肝活检及29例意外死亡或自杀死亡者尸检中肝铁质沉着症的检出率分别为30.5%和28%。
liver biopsies of 46 close relatives of 16 patients with hemochromatosis were examined by light and electron microscopy. By means of the latter the ferritin content of the livers was assessed. Thirty-four (74%) of the 46 biopsies showed excess iron; fatty change was present in 24, and regeneration, fibrosis or cirrhosis in 7. Liver cell lipofuscin was excessive in 20. Electron microscopy showed a close association between lipofuscin and ferritin, and suggested that lipofuscin formed the basis of hemosiderin granules. It is thought that hepatic siderosis in relatives is not necessarily a precursor of hemochromatosis. The incidence of hepatic siderosis in 200 routine liver biopsies and in the post-mortem livers of 29 cases of accidental or suicidal death was 30.5% and 28%.