Familial Hepatic Copper Storage Disease: a Variant of Wilson's Disease.

Wilson's disease is one form of cirrhosis affecting young people that is often amenable to treatment. It is therefore important to pick out patients with this condition from the whole group of juvenile cirrhotics. We here report a patient who suffered from a familial cirrhosis. He showed some but not all of the biochemical features of Wilson's disease and had copper deposition in the liver much in excess of that usually found in this condition. The relation of this patient to sufferers from classical Wilson's disease will be discussed.