Zinc Acetate Dihydrate

Wilson's disease (also called hepatolenticular degeneration) is an autosomal recessive metabolic defect. The liver can't excrete copper properly into the bile, so too much copper builds up in the liver and then in other organs, including the brain, kidneys, eyes, bone and muscles. The liver cells store the extra copper for a while. When their capacity is exceeded, copper is released into the blood and taken up in sites outside the liver, such as the brain. This causes motor disorders (ataxia, tremors, speech difficulties) and psychiatric manifestations (irritability, depression, deterioration of work performance).

Redistribution of the excess copper within the hepatocytes leads to hepatocellular injury, inflammation, necrosis and eventually cirrhosis. Patients may present clinically with mainly hepatic, neurologic or psychiatric symptoms.

The disease has been treated by restricting copper in the diet and by using chelating agents, which bind free copper to reduce its toxicity and help its excretion. In symptomatic patients, the purpose of the first treatment with a chelating agent is to detoxify copper. Once the patient's symptoms have stabilized clinically, maintenance treatment begins. Clinical measures are then used to decide whether the patient remains stable.