Abstract: Objective The aim of this study was to analyze partial epidemiological characteristics of patients with inherited metabolic liver disease (IMLD) of Beijing You'an Hospital from 1996 to 2020.Methods All patients with IMLD admitted in Beijing You'an Hospital from January 1996 to December 2020 were included, and the prevalence of disease catalogues, gender and ages of patients were analyzed.Results There were 552 cases of IMLD (some admitted more than one time) presented in our hospital during this period, accounting for 3.6 ‰ of inpatients with liver disease; out of the 393 patients with IMLD, the percentage of male patients was higher than that of female, with an average age of 29 years; among all patients with IMLD, 223 (56.7%) had Wilson's disease and 136 (34.6%) had congenital hyperbilirubinemia, both accounting for 91.3%; compared with in the previous 10 years, the number of rare diseases increased in recent 10 years and the age of onset increased slightly from 24.9 years to 31.4 years ( P < 0.01), with middle-aged and elderly patients also increasing.Conclusion Adult inherited metabolic liver disease is not rare, and the disease spectrum is gradually rich, to which should be paid more attention by hepatologists.

Key words: Inherited metabolic liver disease, Epidemiology, Disease spectrum