Hepatolenticular Degeneration
Wilson's disease
Introduction
Wilson's disease is an inherited disorder that causes too much copper to accumulate in your liver, brain and other vital organs. Wilson's disease shows up in a variety of different ways, but the disease can remain silent for years.
Copper plays a key role in the development of healthy nerves, bones, collagen and the skin pigment melanin. Normally, copper is absorbed from your food, and any excess is excreted through bile — a substance produced in your liver.
But in people with Wilson's disease, copper isn't eliminated properly and instead accumulates, possibly to a life-threatening level. Left untreated, Wilson's disease is fatal. When diagnosed early, Wilson's disease is treatable, and many people with the disorder live normal lives.