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. 2007 Dec;83(986):739–748. doi: 10.1136/gut.2006.097576

Table 2 Patient series with erythropoietic protoporphyria (EPP) worldwide with details of liver disease.

First author Year Country No of patients % with hepatobiliary disease Comment
Schmidt35 1974 Denmark 29 3% Biochemistry not performed in all cases; 1 patient with porphyrin deposits in liver biopsy
DeLeo36 1976 USA 32 4% No severe liver disease
Went,16 Baart de la Faille37 1984 Netherlands 200 6% 9 with gallstones, 3 young patients with cirrhosis
Murphy38 1985 UK 382 >1% 3 patients with acute hepatic failure
Gross,41 Lehmann,40 Frank39 1998 Germany 140 ∼25% Severe liver disease in 10% of patients
Minder,31 Rüfenacht30 2002 Switzerland and France 55 16% 9 patients with liver disease
Chen33 2002 USA 34 41% Highly selected series. Included 10 patients who underwent liver transplant
Wiman20 2003 Sweden 9 10% 1 patient with severe liver disease requiring liver transplantation