Fulminant hepatic failure resulting from coexistent Wilson's disease and hepatitis E.
Fulminant hepatic failure resulting from hepatitis E and coexistent Wilson's disease was diagnosed in a six year old girl six weeks after returning from a holiday in India. Wilson's disease was diagnosed on the basis of histological evidence of hepatocellular copper deposition, confirmed by biochemical estimation of liver copper concentration. Although severely damaged, the liver was non-cirrhotic. Hepatitis E virus (HEV) was diagnosed by nested polymerase chain reaction, the specificity of which was confirmed by direct sequencing of amplified DNA. Replication of HEV within the liver at the time of diagnosis was confirmed by selective amplification of the antigenomic strand of the virus obtained from total liver RNA. The patient had an orthotopic liver transplantation without recurrence of hepatitis and remains well at 19 months. Viral excretion, recorded by serial amplification of HEV RNA extracted from stool samples, persisted for 30 days after liver grafting. Severe vitiligo, present preoperatively, dramatically improved after liver grafting and institution of immunosuppressive treatment. This case suggests that viral infection may play a part in the acute decompensation seen in some cases of Wilson's disease.
Institute of Liver Studies, King's College Hospital, London.
R
R
Sallie
J
J
Chiyende
K C
KC
Tan
D
D
Bradley
B
B
Portmann
R
R
Williams
A P
AP
Mowat
G
G
Mieli-Vergani
D001483:Base Sequence; D002648:Child; D004279:DNA, Viral / Q000032:analysis; D005243:Feces / Q000382:microbiology; D005260:Female; D006501:Hepatic Encephalopathy / Q000209:etiology* / Q000601:surgery; D016751:Hepatitis E / Q000150:complications*; D016752:Hepatitis E virus / Q000302:isolation & purification; D006527:Hepatolenticular Degeneration / Q000150:complications*; D006801:Humans; D008099:Liver / Q000473:pathology; D016031:Liver Transplantation; D008969:Molecular Sequence Data; D016133:Polymerase Chain Reaction; D012367:RNA, Viral / Q000032:analysis
D004279:DNA, Viral; D012367:RNA, Viral