Free Copper Wilson's disease

This blood test measures the level of free copper in blood.In addition to ceruloplasmin, copper in plasma is also determined, free copper can then be calculated.
This test consists of:

• ceruloplasmin
• copper
• albumin
• crp

This test is requested when someone has symptoms that seem to fit Wilson's disease, Menkes syndrome, copper stacking, copper poisoning or copper deficiency. These are often symptoms such as anemia, nausea, abdominal pain, fatigue, jaundice, and difficulty walking and swallowing. Unexplained high ALAT values. If there are problems with copper metabolism in the family, this can also be a reason to request copper.

To exclude the acute phase reaction, CRP is also determined to see if there is acute inflammation, which may influence the results.95% is in plasma bound to ceruloplasmin, 5% to albumin and is characterized as free copper. Copper causes iron to be fixed in hemoglobin, the red dye in our blood, thus playing a role in oxygen transport in the body. Copper is also involved in the pigmentation of skin and hair and in connective tissue and bone formation. Copper is also important for good immunity and contributes to our body's energy supply. According to Dr. Grabowski, many cases of alleged iron deficiency anemia are actually due to copper deficiency. Like iron, copper is involved in the formation of hemoglobin. Be aware of low copper levels in anemia which additional iron will not solve. Copper deficiency presents exactly like iron deficiency and you never know the difference unless you test for it.

What is copper in?


Copper is found mainly in organ meats, marine fish, seafood, nuts and grain products. Furthermore, fruits and vegetables and cocoa products are sources of copper. The recommended daily amount for adults (22-50 years) is set by the Health Council at 1.5 - 3.5 mg. Pregnant women need extra copper because about 16 mg of copper is sequestered in the placenta and fetus during pregnancy. Breastfeeding women also need more copper to compensate for the amount of copper that leaves the body with the breast milk.


Copper absorption is inhibited by zinc and by a vitamin C intake of at least 1500 milligrams per day. This is equivalent to about 25 oranges.
According to the Health Council, the maximum safe dose for adults is 5 milligrams of copper/day. This corresponds to 550 grams of cooked brown rice. The safe dose is an average value, with a wide margin. This means that exceeding the maximum safe dose once or for a short period of time is not an immediate danger.

What are the consequences of too much copper?








An excess of copper is usually caused by contamination of food and/or drinks. Symptoms that occur are an excess of saliva, pain in the abdomen, nausea, vomiting and diarrhea.


Symptoms commonly seen with copper deficiency are anemia, impairment of the immune system and bone abnormalities, such as osteoporosis.
This test is requested by doctors when a person has symptoms that seem to fit Wilson's disease, Menkes syndrome, copper stacking, copper poisoning or copper deficiency. These are often symptoms such as anemia, nausea, abdominal pain, fatigue, jaundice, and problems with walking and swallowing. Also when there are problems with copper metabolism in the family, this can be a reason to request copper.

Copper is a mineral that is essential to the body. It is built into enzymes by the body. These enzymes play an important role in all kinds of processes in the body, for example in the production of energy and the functioning of the brain. Copper is absorbed through food (e.g. nuts, chocolate, mushrooms, shellfish and grains). Water may also contain copper if the water pipes are made of copper or is cooked in copper pans. The copper is absorbed by the intestine and transported to the liver. In the liver, the copper is stored or bound to the protein ceruloplasmin, to which it is transported in the blood. Copper is excreted through the stool and a small amount leaves the body through the urine.

Wilson's disease is a rare, inherited metabolic disease characterized by accumulation of copper in the liver, among other places.
Too little of the protein ceruloplasmin is made in the liver. Ceruloplasmin is a binding protein for copper. Due to a lack of the protein, copper accumulates in the liver, brain, kidneys and the cornea of the eye. The amount of copper in the blood is reduced. The excretion of copper in the bile is reduced, the excretion of copper with the urine is increased.

Our body needs copper to function properly. In our daily diet there are small amounts of copper. These are absorbed into our body through the intestinal wall. The excess copper is removed from our body by the liver, with the bile fluid. In healthy people there is a balance between the intake of copper and the excretion of copper. In Wilson's disease, this balance is disturbed. In people with Wilson's disease, the liver does not process the small amounts of copper as it should. The excess copper is not eliminated but accumulates in the liver cells. In a later stage, accumulation also takes place in the brain which can cause considerable damage.

Cause Wilson's disease

Norm
ally
, the excess copper we take in is excreted through the liver. People with Wilson's disease lack an enzyme that makes it impossible to process copper properly. Wilson's disease is hereditary and affects both men and women. The inheritance is recessive, meaning that both the father and the mother must be 'carriers' of the gene that causes the disease. As a 'carrier' you do not have the disease because in addition to the abnormal gene you also have a healthy gene. If a child inherits the abnormal gene from his mother and father, he or she will have Wilson's disease.

What to do when you have Wilson's disease?
























Early treatment with drugs is usually possible. These drugs bind the excess copper and then remove it with the urine. A medication with zinc can also be given. Zinc inhibits the absorption of copper from food. The medication must be used for life.

In addition, a low copper diet can support this drug treatment. A dietitian can help you establish this diet When the disease is diagnosed at a late stage, the liver may be damaged beyond repair.

Wilson's disease is an inherited disease. If you have been diagnosed with the disease, it is important that family members are also examined. The doctor treating you, or a genetics expert, can explain this to you. When Wilson's disease is diagnosed in people (children) who have no symptoms yet, symptoms can possibly be prevented altogether by starting with medication.

Menkes syndrome (kinky hair syndrome) is a hereditary disease that occurs mainly in boys. In this disease, the intestine absorbs copper from the food, but does not then release it to the rest of the body. As a result, there is a surplus of copper in the intestine and a deficiency in the rest of the body.

Adding ALAT (see slide) to this test for Wilson's disease provides important insight into liver health. Since Wilson's disease often damages the liver due to copper accumulation, ALAT can help determine the extent of liver damage, aid diagnosis and monitor treatment progress. Therefore, it makes sense to add ALAT to this test to get a more complete picture of liver health in patients with suspected copper accumulation.