What is being tested?This test measures the amount of copper in the blood, urine, or liver (hepatic). Copper is an essential mineral that the body incorporates into
enzymes. These enzymes play a role in the regulation of iron
metabolism, formation of connective tissue, energy production at the cellular level, the creation of melanin, and the function of the nervous system and brain.
Copper is found in many foods including nuts, chocolate, mushrooms, shellfish, whole grains, dried fruits, and liver. Drinking water may acquire copper as it flows through copper pipes, and food may acquire it when people cook or serve food in copper dishes. Normally, the body absorbs copper from the intestines, makes it nontoxic by binding it to a protein, and transports it to the liver. The liver stores some of the copper and binds most of the rest to a protein called apoceruloplasmin that, when copper is attached, becomes the enzyme ceruloplasmin. About 95% of the copper in the blood is bound to ceruloplasmin. The liver excretes excess copper into the bile and it is removed from the body in the stool. Some copper is also excreted in the urine.
Both excesses and deficiencies of copper are rare. Wilson’s disease, an inherited disorder, can lead to excess storage of copper in the liver, brain, and other organs. This can cause tissue damage and signs and symptoms such as:
anemia
nausea, abdominal pain
jaundice
fatigue
behavioral changes
tremors
difficulty walking and/or swallowing
dystonia
If the kidneys are involved, then urine production may be decreased or absent. Some of these symptoms may also be seen with copper poisoning that is due to acute or chronic environmental exposure to copper or due to conditions such as liver disease or obstructions that prevent or inhibit copper metabolism and excretion.
Copper deficiencies may occasionally occur in patients who have conditions associated with severe malabsorption, such as cystic fibrosis and celiac disease, and in infants exclusively fed cow-milk formulas. Symptoms may include neutropenia, osteoporosis, and microcytic anemia. A rare X-linked genetic condition called Menkes kinky hair syndrome leads to copper deficiencies in the brain and liver of affected infants. The disease, which affects primarily males, is associated with seizures, delayed development, abnormal artery development in the brain, and unusual gray brittle kinky hair.
