In:
Journal of Gastroenterology and Hepatology, Wiley, Vol. 30, No. 3 ( 2015-03), p. 535-539
Kurzfassung:
Data on risk of hepatocellular carcinoma ( HCC ) in patients with W ilson disease are scarce. We determine HCC risk in a well‐defined cohort of W ilson patients. Methods All patients with a confirmed diagnosis of W ilson disease ( L eipzig score ≥ 4) in three D utch university referral hospitals were included in this retrospective cohort study. End of follow‐up was defined as date of diagnosis of HCC , liver transplantation, death, or last available hospital visit. Also, a meta‐analysis was performed to determine incidence and mortality rate of HCC in W ilson disease based on all published cohorts. Results In total, 130 patients with W ilson disease were followed during a median follow‐up of 15 years (range 0.1–51.2). At baseline, cirrhosis was present in 74 patients (57% of total: 64% compensated, and 36% decompensated). At end of follow‐up, liver disease severity was improved, stable or deteriorated in 20%, 46%, and 24% of all cases (10% unknown), respectively. Two patients developed HCC (one despite excellent decoppering after 50 years follow‐up, the other with newly diagnosed Wilson disease). Estimated annual HCC risk for all patients was 0.09% (95% confidence interval [ CI ]: 0.01–0.28). Subgroup analysis in cirrhotic patients revealed an annual HCC risk of 0.14% (95% CI : 0.02–0.46). The meta‐analysis showed an annual HCC risk of 0.04% (95% CI : 0.01–0.10) and HCC mortality rate of 2.6/10 000 person‐years (95% CI : 0.7–7.0). Conclusions Even in case of cirrhosis, HCC risk is low in Wilson disease. Our data do not support regular HCC surveillance in W ilson disease.
Materialart:
Online-Ressource
ISSN:
0815-9319
,
1440-1746
DOI:
10.1111/jgh.2015.30.issue-3
Sprache:
Englisch
Verlag:
Wiley
Publikationsdatum:
2015
ZDB Id:
2006782-3
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