Abstract: Objective To analyze the clinical features of patients with hepatolenticular degeneration.Methods The clinical manifestations，laboratory and other special examination data in 12 patients with hepatolenticular degeneration were retrospectively analyzed． Results The age of the patients in this series was between 11 to 40 year old. Seven patients recovered，two were stable and one died in 1 to 5 year follow-up. Conclusions The early therapy is very important to its prognosis． We suggest that screening examination including Kayser—Fleischer rings and copper metabolism should be done once patients is suspected.

Key words: Hepatolenticular degeneration, Ceruloplasmin, Cirrhosis