FULMINANT HEPATIC-FAILURE RESULTING FROM COEXISTENT WILSONS-DISEASE AND HEPATITIS-E

Fulminant hepatic failure resulting from hepatitis E and coexistent Wi lson's disease was diagnosed in a six year old girl six weeks after re turning from a holiday in India. Wilson's disease was diagnosed on the basis of histological evidence of hepatocellular copper deposition, c onfirmed by biochemical estimation of liver copper concentration. Alth ough severely damaged, the liver was non-cirrhotic. Hepatitis E virus (HEV) was diagnosed by nested polymerase chain reaction, the specifici ty of which was confirmed by direct sequencing of amplified DNA. Repli cation of HEV within the liver at the time of diagnosis was confirmed by selective amplification of the antigenomic strand of the virus obta ined from total liver RNA. The patient had an orthotopic liver transpl antation without recurrence of hepatitis and remains well at 19 months . Viral excretion, recorded by serial amplifiation of HEV RNA extracte d from stool samples, persisted for 30 days after liver grafting. Seve re vitiligo, present preoperatively, dramatically improved after liver grafting and institution of immunosuppressive treatment. This case su ggests that viral infection may play a part in the acute decompensatio n seen in some cases of Wilson's disease.