Akademik Veri Yönetim Sistemi
Turkish Journal of Gastroenterology, cilt.10, sa.3, ss.227-231, 1999 (SCI-Expanded, Scopus, TRDizin)
Wilson's disease, as a rare cause of fulminant hepatic failure, has a 100% mortality rate without liver transplantation During last year we followed two cases of Wilson's disease which progressed into fulminant hepatic failure. Two female patients, nine and it years old were hospitalized due to acute hepatitis and Coombs negative hemolytic anemia of unknown etiology. Following clinical and laboratory examinations, they were diagnosed with Wilson's disease. The patients died after two months due to hepatic failure, in spite of adequate conservative treatment. In both cases, serum transaminase levels were not as high as expected at the beginning of the fulminant hepatitis during clinical follow up. In the first case, alkaline phosphatase (ALP) / total bilirubin ratio was over two and AST / ALT ratio was lower than four where as in the second case ALP / total bilirubin ratio was lower than two and AST / ALT ratio over four. An ALP / total bilirubin ratio lower than 2, AST / ALT ratio over 4 and transaminase levels lower than other fulminant hepatitis cases have recently been reported to be important criteria in the early diagnosis of fulminant hepatic failure. Although high total bilirubin levels and relatively low alkaline phosphatase and transaminase levels appear to be a warning criteria for fulminant Wison's disease in pediatric cases of fulminant hepatic failure of unknown etiology, we believe that they are insufficient criteria.