Coeruloplasmin

Coeruloplasmin (synonyms: ceruloplasmin, caryuloplasmin, ferroxidase) is an acute-phase protein synthesized (produced) in the liver in hepatocytes (“liver cells”). It is the binding and transport protein for copper (copper storage) and contains 8 divalent copper ions (Cu++) per molecule. After incorporation of copper, secretion (excretion) from the liver occurs. Coeruloplasmin migrates to the tissues of copper consumption and is degraded there releasing the copper.

Another property is the catalytic function in the oxidation of iron (Fe²+ → Fe³+), catecholamines, polyamines and polyphenols.

The process

Material needed

• Serum

Preparation of the patient

• Not necessary

Standard values

Collective	Norm
Adult	20-60 mg/dl (200-600 mg/l)
Children	23-43 mg/dl (230-430 mg/l)
Newborn	6-20 mg/dl (60-200 mg/l)

Indications

• Suspected Wilson’s disease (copper storage disease).
• Suspected Menkes syndrome (synonym: Menkes disease; rare inborn error of metabolism due to copper metabolism disorder; no severe symptoms seen in the first eight to ten weeks of life; later, degenerative symptoms such as movement disorders and epileptic seizures)
• Clarification of liver dysfunction
• Hypochromic microcytic iron-refractory anemia (anemia that does not respond positively to iron treatment).
• Suspected nutritive (“dietary) copper deficiency.

Interpretation

Interpretation of increased values

• Acute or chronic inflammation (acute-phase protein).
• Liver disease:
  • Acute hepatitis (inflammation of the liver).
  • Cholestasis (bile stasis)
• Malignant (malignant) tumors
• Hodgkin’s disease (malignant neoplasm of the lymphatic system with possible involvement of other organs).
• Myocardial infarction (heart attack)
• Gravidity* (pregnancy)
• Oral contraceptives* (“pill”)

* False high values

Interpretation of lowered values

• Wilson’s disease:
  • Coeruloplasmin i. S. (in serum) ↓
  • Copper i. S. ↓
  • Copper excretion i. U. (in urine) ↑
• Menkes syndrome:
  • Coeruloplasmin i. S. (in serum) ↓
  • Copper i. S. ↓
• Protein loss syndromes (protein losses):
  • Intestinal diseases (e.g., exudative enteropathy/protein losses through the gastrointestinal tract).
  • Nephrotic syndrome – collective term for symptoms associated with various diseases of the glomerulus (renal corpuscles); symptoms include: Proteinuria (excretion of protein in the urine) with a protein loss of more than 1g/m²/body surface per day; Hypoproteinemia, peripheral edema (water retention) due to hypalbuminemia of < 2.5 g/dl in serum, hyperlipoproteinemia (lipid metabolism disorder) with LDL elevation.
• Malabsorption syndrome
• Severe hepatocellular insufficiency (failure of liver-specific metabolic functions).
• Malnutrition (nutritive (“nutritional) copper deficiency).

Other notes

• If Wilson disease is clinically suspected and the serum coeruloplasmin level is markedly decreased, a liver puncture (liver biopsy; evidentiary) should be performed to confirm the diagnosis.