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Post by Master - Doctor Do Thi Hoang Ha - Doctor of Biochemistry, Vinmec Hai Phong International General Hospital
Test to determine the level of ceruloplasmin (a copper-binding protein in the body) is usually indicated for people with symptoms of copper deficiency, Wilson's disease, Menkes syndrome and early screening of Wilson's disease in asymptomatic people. .
1. What is Ceruloplasmin?
Ceruloplasmin is a glycoprotein produced in the liver that is primarily responsible for transporting copper in the blood. Copper is an important trace metal in the body, they bind to enzymes and these enzymes regulate iron metabolism, formation of connective tissues, energy production at the cellular level, melanin production. pigment), forms connective tissue and supports central nervous system activities. Copper is found in many foods such as nuts, chocolate, mushrooms, shellfish, whole grains, dried fruit, and liver. Normally, the body absorbs copper from food in the intestines, copper is attached to proteins to become non-toxic and transported to the liver.About 95% of the copper in the blood is attached to ceruloplasmin. The remainder is attached to other proteins such as albumin, with only a small amount of copper in the blood in the free form. After being secreted from the liver, ceruloplasmin travels to the tissue that requires copper. Here, the copper atom is released during the metabolism of the ceruloplasmin molecule. In addition to its function of transporting copper atoms, ceruloplasmin is involved in maintaining the normal function of many enzymes, antioxidant, neutralizing free radicals, and synthesizing neurotransmitters (polyamines, catecholamines, and polyphenols). The liver excretes excess copper by the biliary tract, to the intestines to be excreted in the faeces and a part is excreted in the urine.
Ceruloplasmin testing is done to determine ceruloplasmin levels in the body, especially in the diagnosis of Wilson's disease, a rare genetic disorder that causes liver disease and neuropsychiatric symptoms. Wilson's disease occurs due to an excessive accumulation of copper in the liver, brain, and other organ tissues in the body.
2. When is the patient ordered to test for ceruloplasmin?
2.1. Diagnosis of Wilson's disease Ceruloplasmin testing is usually indicated for diagnosis when the patient has the following symptoms, suspected Wilson's disease:
Fatigue Yellowing of the skin or eyes Skin rash Nausea, abdominal pain Joint pain Drooling Ease bruising Loss of appetite Anemia Changes in behavior Restlessness Difficulty controlling movement or difficulty walking or swallowing Dystonic dystonia Ceruloplasmin testing is also indicated for early Wilson diagnosis, in cases that run in families have a history of Wilson's disease even before symptoms of the disease. Symptoms of Wilson's disease usually appear between the ages of 5 and 35, but can appear earlier or later.
In Wilson's disease, a decrease in ceruloplasmin levels occurs during recessive liver degeneration. From a chemical-pathological perspective, accompanied by a decrease in ceruloplasmin synthesis, the disease occurs when there is no binding of Cu2+ to the molecule due to a defect in metallothionine. As a result, there is pathological copper accumulation in the liver (with progressive cirrhosis), brain (with neurological symptoms), cornea (Kayser-Fleischer rings), and kidneys (hematuria, proteinuria, amino aciduria). In homozygous recessive carriers, ceruloplasmin levels are very low. Heterozygous carriers show either no or only a very slight decrease in the amount of ceruloplasmin in the blood.
To accurately diagnose Wilson's disease, in addition to the ceruloplasmin test, the doctor may order other blood and urine copper tests. Where a patient has been diagnosed with Wilson's disease, a ceruloplasmin test will be used to assess the effectiveness of the treatment being used.
2.2.Diagnosis of copper deficiency If a patient is suspected of having a copper deficiency, the doctor will also order a ceruloplasmin test. Symptoms of copper deficiency in the body include:
Pale skin Abnormally low white blood cell count Osteoporosis Fatigue Tingling in hands and feet. Anemia 2.3. Diagnosing Menkes Syndrome Children may also be ordered to have a ceruloplasmin test if they have symptoms of Menkes syndrome, including:
Fragile, thinning or tangled hair Difficulty feeding No or slow growth Lack of muscle tone Convulsions . Menkes syndrome is an inherited disorder of copper absorption caused by mutations in the recessive ATP7A gene located on the X chromosome with decreased levels of ceruloplasmin, meaning that only girls and women can manifest disease. Boys and men carry only 1 gene X, so they are only carriers of the disease gene. Protein loss syndrome and hepatocellular failure are among the most important causes of acquired ceruloplasmin deficiency. Since ceruloplasmin is an acute phase sensitive reactant, proliferation occurs during acute or chronic inflammation. These mutations lead to an uneven distribution of copper in the body, allowing copper to accumulate in intestinal tissues and kidneys, leading to copper deficiency in other organs such as the brain. The incidence of this syndrome is about 1 in 100,000 newborns, symptoms of this syndrome usually develop in infancy and most children will die within the first few years of life. When detected and treated early, children can live longer.
3. How is the Ceruloplasmin test performed?
To perform a ceruloplasmin test, a vein in the arm needs to be taken and sent to a laboratory. After the results are available, the doctor will explain the meaning of the test as well as evaluate the ceruloplasmin concentration for each specific patient.
4. Are there any risks to testing for ceruloplasmin?
The risks associated with performing a ceruloplasmin test are relatively small, mainly related to the injection site, and include:
Difficulty drawing blood, requiring multiple needle punctures Heavy bleeding at the site of blood collection Fainting due to blood loss or panic - this is very rare Subcutaneous coagulation Skin infection at the site of blood collection.
5. What to prepare for the Ceruloplasmin test?
Before taking the ceruloplasmin test, the patient does not need to do any special preparation. However, ask your doctor if there is anything special or unclear before taking the test.
6. What does the Ceruloplasmin test mean?
6.1. What is the normal range of ceruloplasmin? The normal range for ceruloplasmin for people 20 years of age and older is:
Men: 22 - 40 mg/dL Women not taking birth control pills: 25 - 60 mg/dL Women taking birth control pills or estrogen: 27 - 66 mg/dL Pregnant women: 30 - 120 mg/dL Children 7 months to 3 years old: 31 - 90 mg/dL. Note: The reference range in ceruloplasmin test results may vary slightly between laboratories depending on the chemical brand and equipment used by that laboratory.
6.2. Low levels of ceruloplasmin in the blood Low levels of ceruloplasmin can be an indicator of Wilson's disease and be a sign of other conditions such as:
Liver disease Liver failure Cirrhosis Liver malabsorption syndrome Malnutrition Menkes disease Nephrotic syndrome if accompanied by other manifestations such as protein in the urine, low protein in the blood, high cholesterol levels, high triglyceride levels. Systemic fibrosis syndrome Celiac disease - a digestive disease that damages the small intestine and interferes with the absorption of nutrients from food Babies only eat cow's milk. 6.3. High levels of ceruloplasmin in the blood The level of ceruloplasmin in the blood is higher than normal in the following cases:
Pregnancy Taking estrogen pills Taking a combined oral contraceptive, which is a type of birth control pill that contains estrogen and progesterone Having an infection or disease cancer. Copper toxicity can occur when a person is exposed to and absorbs large amounts of copper for a short time (acute exposure) or different amounts of copper over a long time (chronic exposure). Organic copper in food is an essential micronutrient for health, but inorganic copper in copper utensils is a heavy metal that is neurotoxic, physically and mentally. Rheumatoid arthritis (RA) Severe infection Primary cholangitis Systemic lupus erythematosus Lymphoma, such as Hodgkin's disease Leukemia Carcinoma . If the test shows any other abnormalities, your doctor will explain the results to you, and if the condition is suspected to be related to a copper metabolism disorder, your doctor will order additional tests.
Hai Phong International General Hospital owns modern and synchronous automatic combined testing equipment of famous and reputable firms in the field of testing in the world such as Beckman Coulter (USA) ), Roche (Switzerland):
Modern system for transporting specimens and returning results by compressed air of Beckman - Coulter (USA) to avoid contamination, sample confusion, and at the same time avoid the impact of the environment on patient samples, giving accurate test results with fast response time. VITEK2 COMPACT Biormerieux automatic identification and antibiotic system for microbiological tests is the system of equipment being used at leading hospitals in Hanoi and Hai Phong. Pathology: The first BenchMark XT automatic in situ hybridization and immunohistochemistry staining machine in Hai Phong identifies the origin and classification of cancers. With the system of modern testing equipment mentioned above, Vinmec Hai Phong Hospital ensures the accuracy of test results such as:
Biochemistry, hematology, coagulation, immunological tests to serve the examination. general health, detecting thyroid disease and cancer markers (liver, colon, pancreas, prostate, ovary, breast...) Histopathological examination: Basic test to detect cancer (through aspiration, cell biopsy) Prenatal screening tests, newborns detect congenital malformations, congenital hypothyroidism, G6PD enzyme deficiency causing reduced erythrocyte lifespan, detect abnormalities usually adrenal gland... Microbiological tests in blood and urine detect the cause of infection. Diagnostic tests for hepatitis A, B, and C virus infections; HPV, HIV, Chlamydia, rubella... Customers who receive medical examination and treatment at Hai Phong International General Hospital will be provided with highly accurate test results, helping to diagnose and treat the disease. effective.
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References: Healthline.com, Medlineplus.gov
