Kauppinen R, Mustajoki P
Third Department of Medicine, University of Helsinki, Finland.
Br J Cancer. 1988 Jan;57(1):117-20. doi: 10.1038/bjc.1988.23.
In this study we examined the case histories of 163 living and 82 deceased adult Finnish patients with acute hepatic porphyria. There were 184 patients with acute intermittent porphyria and 61 patients with variegate porphyria. Among the 124 of the 163 living patients, who were traced 1984-1985, no hepatocellular carcinoma was found. Among the 82 deceased patients the cause of death was porphyria in 29 (36%), cardiovascular disease in 23 (29%) and hepatocellular carcinoma in 7 (9%). Of the 7 patients with hepatocellular carcinoma, 6 had acute intermittent porphyria and one had variegate porphyria. In acute hepatic porphyria, as compared with the total population, the calculated risk of hepatocellular carcinoma is increased 61-fold.
在本研究中,我们检查了163例存活的和82例已故的成年芬兰急性肝卟啉病患者的病历。其中有184例急性间歇性卟啉病患者和61例杂合性卟啉病患者。在1984 - 1985年追踪的163例存活患者中的124例中,未发现肝细胞癌。在82例已故患者中,29例(36%)死于卟啉病,23例(29%)死于心血管疾病,7例(9%)死于肝细胞癌。在7例肝细胞癌患者中,6例患有急性间歇性卟啉病,1例患有杂合性卟啉病。与总人口相比,急性肝卟啉病患者患肝细胞癌的计算风险增加了61倍。