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Pronunciations celiac disease ceruloplasmin chorea cirrhosis electrolytes epinephrine fibrosis hemolysis hepatitis penicillamine

Most of the copper in the body is located in the liver, bones, and muscle, but traces of copper occur in all tissues of the body. The liver excretes excess copper into the bile for elimination from the body. Copper is a component of many enzymes. Some of these enzymes are necessary for energy production or for the formation of the hormone epinephrine Some Trade Names
ADRENALIN
, red blood cells, bone, or connective tissue (which binds other tissues and organs together). Other enzymes act as antioxidants. They help protect cells against damage by free radicals, which are reactive by-products of normal cell activity.

Wilson's Disease: When Copper Accumulates In Wilson's disease, a rare hereditary disorder, the liver does not excrete excess copper into the bile as it normally does. As a result, copper accumulates in and damages the liver. The damaged liver releases copper directly into the bloodstream, and copper is carried to other organs, such as the brain and eyes, where it also accumulates. Symptoms usually begin after age 5. In almost half of affected people, the first symptoms result from brain damage. They include tremors, difficulty speaking and swallowing, incoordination, involuntary jerky movements (chorea), personality changes, and even psychosis (such as schizophrenia or manic-depressive illness). In most of the other people, the first symptoms result from liver damage, which causes hepatitis and eventually cirrhosis. In the cornea of the eyes, the accumulated copper produces gold Some Trade Names MYOCHRYSINE or greenish gold Some Trade Names MYOCHRYSINE rings. Doctors suspect Wilson's disease on the basis of symptoms, such as unexplained hepatitis, tremors, and personality changes. The diagnosis is confirmed by blood tests and a liver biopsy. For children who have a family history of the disease, tests are performed at about age 2 years. Treatment consists of drugs that bind with copper, such as penicillamine Some Trade Names CUPRIMINE , taken by mouth. Taking zinc supplements may help decrease absorption of copper. Without lifelong treatment, Wilson's disease is fatal. People who do not take the drugs as directed, especially younger people, may develop liver failure. Liver transplantation can cure the disease.

Copper Deficiency

Copper deficiency is rare among healthy people. It occurs most commonly among infants who are premature, who are recovering from severe undernutrition, or who have persistent diarrhea. A severe disorder that impairs absorption of nutrients (such as celiac disease, Crohn's disease, cystic fibrosis, or tropical sprue) may cause this deficiency. A high intake of zinc or iron can decrease the absorption of copper.

Symptoms of copper deficiency include fatigue, bleeding under the skin, damage to blood vessels, and an enlarged heart. Anemia is common, and the number of white blood cells is decreased.

The diagnosis of copper deficiency is based on symptoms and on blood tests that detect low levels of copper and ceruloplasmin (a protein that contains copper). Copper deficiency is treated with a copper supplement.

Copper Excess

Excess consumption of copper is rare. Any copper not bound to a protein is toxic. Acidic food or beverages in prolonged contact with copper vessels, tubing, or valves can be contaminated with small amounts of unbound copper. Consuming even relatively small amounts of unbound copper may cause nausea, vomiting, and diarrhea. Large amounts can damage the kidneys, inhibit urine production, and cause anemia due to the rupture of red blood cells (hemolysis) and even death.

The diagnosis is made by measuring copper and ceruloplasmin levels in the blood or urine. Treatment involves use of drugs that bind with copper.

Last full review/revision February 2003