What is another name for hepatolenticular degeneration?

Hepatolenticular degeneration is another name for Wilson disease, a genetic disorder that leads to an accumulation of copper in the body, particularly affecting the liver and the brain. This disease is caused by a defect in the ATP7B gene, which is responsible for the transport of copper.

In Wilson disease, excess copper accumulates in various tissues, leading to hepatic dysfunction, neurological symptoms, and psychiatric manifestations. Early identification and management are crucial, as untreated Wilson disease can result in severe liver damage and neurologic consequences.

The other options listed refer to different conditions. Hemochromatosis involves iron overload rather than copper, while acute liver failure and cirrhosis are complications resulting from various liver diseases but do not specifically involve the genetic dysregulation of copper metabolism seen in Wilson disease. Therefore, associating hepatolenticular degeneration with Wilson disease accurately reflects the underlying pathology and its clinical significance.