Wilson's Disease and Nephrotic Syndrome

Wilson’s disease is a rare inherited disorder that causes copper to accumulate in your liver, brain and other vital organs. Most people with Wilson’s disease are diagnosed between the ages of 5 and 35, but it can affect younger and older people, as well.

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 excess is excreted through a substance produced in your liver (bile).

But in people with Wilson’s disease, copper isn’t eliminated properly and instead accumulates, possibly to a life-threatening level. When diagnosed early, Wilson’s disease is treatable, and many people with the disorder live normal lives.

However the most common and effective treatment for Wilson’s Disease, D-penicillamine, has frequently lead to the development of Early stage Nephrotic Syndrome. While in most cases the condition resolves, it sometimes does not.

An alternative treatment using trientene is a possibility to avoid this complication. prior to the development of D-penicillamine as a copper chelating treatment, dimercaprol was used. D-penicillamine however is so much more effective, the use of dimercaprol has been used.

A broad discussion with your medical team as to the risks and benefits of available therapies for Wilsons disease and the avoidance of Nephrotic Syndrome is ALWAYS in order.