Abstract
Daniel Schafer and Michael Sorrell1Schafer DF Sorrell MF Hepatocellular carcinoma.Lancet. 1999; 353: 1253-1257Summary Full Text Full Text PDF PubMed Scopus (515) Google Scholar fail to mention Budd-Chiari syndrome and acute hepatic porphyrias among the conditions associated with HCC. The evidence of HCC in patients with Budd-Chiari syndrome is high. In a study of Japanese patients with chronic Budd-Chiari syndrome, Okuda and colleagues2Okuda H Yamagata H Obata H et al.Epidemiological and clinical studies of Budd-Chiari syndrome in Japan.in: Scheuer P Chapman R Brighton: International Association for the Study of the Liver, Biennial Scientific Meeting, June 3–6, 1992. IASL, Brighton1992: OP-80Google Scholar found 6% incidence of HCC. Association between primary HCC and acute hepatic porphyrias have been reported in two retrospective and one case-control study.3Andant D Puy H Faivre J Deybach JC Acute hepatic porphyrias and primary liver cancer.N Engl J Med. 1998; 338: 1853-1854Crossref PubMed Scopus (26) Google Scholar Andant and colleagues' prospective cohort study3Andant D Puy H Faivre J Deybach JC Acute hepatic porphyrias and primary liver cancer.N Engl J Med. 1998; 338: 1853-1854Crossref PubMed Scopus (26) Google Scholar assessed the crude annual incidence rate of primary liver cancer in 650 French carriers of the gene for acute hepatic porphyria who were followed up for 7 years. Primary liver cancer was found in seven patients, all of whom had HCC. Five patients had acute intermittent porphyria, one had variegate porphyria, and one had hereditary coproporphyria. The overall standardised rate ratio was 29. The risk of developing HCC in carriers of the porphyria gene was particularly increased in women.
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