Wilson's disease

Wilson's disease

We present our new medical note of Wilson's disease, in which the selection of copper due to disturbances of copper accumulate in the liver, brain, kidney and other organs. The article describes the second half of Wilson's disease is a general dietary recommendations.



The detection of Wilson's disease of the liver zsugorodásról medical notes. The adult human body has about 150 mg of copper, which is the tenth part of the liver. The plasma proteins (70-90%, one of the makroglobulinok cöruloplazminhoz) binds. The body is not bioavailable copper is excreted in the bile, and feces excreted from the body.

Copper metabolism in normal laboratory values:

- Serum total copper edge: 70 to 150 micrograms / dl.
- Serum free copper edge: at 0-10 g / dl.
- Honorary serum ceruloplasmin: 20-60 mg / dl.
- Copper in urine: 3-50 micrograms / day.
- Copper in the liver: <250 micrograms / g dry weight.

Copper is an essential trace element for the body, multi-component enzyme and the enzyme plays an important role in maintaining operations. Both copper deficiency and the abnormal accumulation of copper causes serious metabolic disturbances. Wilson's disease is an inherited disease of the liver. The abnormal gene is located on chromosome 13. The selection of copper due to disturbances of copper accumulate in the liver, brain, kidney and other organs. Typical differences in plasma protein binding of copper (cöruloplazminnak) to decrease.

Wilson's disease in half of patients from the age of eight or ten symptoms suggestive of liver dysfunction, the patient has acute or chronic liver inflammation (hepatitis) and cirrhosis of the liver (cirrhosis) can be. For others, neurological or psychiatric disorders appear, and damages the central nervous system. Trouble swallowing, trembling hands, speech and movement disorders seen. The so-called Kayser-Fleischer symptoms, rings, gold or green-colored copper deposits associated with the edge of the cornea. They do not interfere with vision, but suggest that considerable amounts of copper from the liver liberated.


A healthy liver tissue copper simple histochemical methods can not be detected. Increased copper content in the case of the liver biopsy sample of copper fehérjeszemcsék observed. Thus, Wilson's disease is diagnosed on needle biopsy. The copper accumulates due to the treatment of symptoms of the disease improved.
If you think about it, the disease is not difficult to diagnose. Raises the suspicion of all patients under the age of forty, when the central nervous system symptoms of unclear origin, or if the existing hepatitis or cirrhosis of the findings are not due to virus infection, alcohol consumption or toxicity. In some laboratory findings (unexplained rise in transaminases levels, hemolytic anemia) can indicate Wilson's disease. It should be considered even if the patient's family history of this disease. The Wilson disease gene mutation detection is possible.


The diagnosis should be removed as soon as possible after the deposition of copper in the liver tissues, even if the patient is asymptomatic. This medication is achieved, which should be continued throughout life. D-penicillamine treatment is carried out, which is increasingly the treatment of zinc (zinc sulfate or zinc acetate) was up. This is more effective and less side effects. The drug prevents further copper deposition, and promotes the removal of accumulated copper.

The poverty of copper from 1.5 to 1.8 mg / day in duration, so every day to be reckoned with copper in the diet. Higher copper content of foods: cocoa, chocolate, hazelnut, coffee, walnut, veal and beef, beans, peas, green peas, lentils and soybeans. The dietary management of the liver caused by copper deposits in liver damage (inflammation, cirrhosis) is adapted.


References:

First Krutsay, M.: The histochemical rézkimutatásról. Labinfo, 2004 / 6th, 40-41.
Second Couple, A., Szalay, F., Lakatos, P. L. Nagy, Z., Mozso, Gy: Genetics in liver diseases. Medical Journal, 2002, 143/1., 3-12.
Third Tremmel, A, Telegdy, L.: Laboratory evaluation of results in clinical practice. Melania, Budapest, 1996.
4th Couple, A, Nógrád, K.: Small liver and epekönyv. B + V, Budapest, 2001.
5th Rodler, I (Eds.): New nutritional table. Medicine, Budapest, 2005. Dear Visitor! Please note that materials are informative and educational in nature, it can not provide answers to any questions relating to a specific disease or subject arise, and especially not substitute for doctors, pharmacists or other health professionals, personal encounters, conversations and careful investigation. - The Editors