Wilson's Disease: Liver Cirrhosis and Virus-like Particles

Abstract

Hepatolenticular degeneration (Wilson's disease) manifests itself in many ways the most obvious of which is a disorder of copper metabolism. Normally copper beyond a certain threshhold is transferred to ceruplasmin which may circulate in the plasma. In patients with Wilson's disease this transfer apparently does not take place. There follows an accumulation of the metal in body tissues most notably the lenticular nucleus and the liver.Needle biopsies taken of the liver of an 11 year old male with Wilson's disease was prepared for light and electron microscopy by conventional methods. Thin sections were examined with Elmiskop 1A.Histologically the liver alteration was that of cirrhosis in which the cells showed fatty and glycogenic degeneration, cytoplasmic coagulation, fibrosis, and pigment deposition. Electron microscopy confirmed and extended light microscopy.