An Adult Case with Hunters Syndrome Presenting Prominent Hepatic Failure: Light and Electron Microscopic Features of the Liver

Abstract

We describe a 40-year-old male patient with Hunters syndrome. His main manifestations were ascites and esophageal varices due to cirrhotic liver. We obtained hepatic biopsy samples and examined them. Ultrastructurally, the features of the hepatocytes and Kupffer cells were the same as those reported in young patients. The passage of 40 years led to gradual progression to fibrosis, and ultimately liver cirrhosis. Namely, with a longer survival time, the complications of liver cirrhosis become more remarkable. Hepatic fibrosis in Hunters syndrome is slowly progressive and patients who are expected to have a longer life span should be continuously monitored for hepatic complications.