To improve the diagnostic quality of hepatolenticular degeneration by summarizing the clinicopathological features.

A retrospective analysis of 21 cases that were diagnosed as hepatolenticular degeneration with liver biopsy in our hospital from January 2013 to August 2018 was reviewed, and then their clinicopathologic features were analyzed. The pathomorphological differences between liver biopsy and liver biopsy after transplantation, and the relationship between histopathological patterns and biopsy types and clinical indicators were analyzed by Fisher's exact test.

Of the 21 patients with hepatolenticular degeneration, 10 patients had liver biopsy, and 11 patients underwent liver biopsy after liver transplantation. Among them, four cases were presented as simple fatty liver pattern (19.0%, 4/21), eight cases as steatohepatitis pattern (38.1%, 8/21), four cases as inflammatory necrosis without cirrhosis pattern (19.0%, 4/21), and five cases as inflammatory necrosis with cirrhosis pattern (23.9%, 5/21). Twelve cases had copper deposition in the liver (57.1%, 12/21), and the pattern of copper distribution in the liver was uneven.

A clinicalpathological features of hepatolenticular degeneration mainly manifests in four patterns, but lack characteristic changes. Hence, comprehensive judgment should rely on clinical history, laboratory examination, genetic test results and liver histopathological changes.

Hepatolenticular degeneration; Histopathologic patterns; Copper deposition