Wilson's disease is a
hereditary disease which causes the
body to retain
copper.
The liver of a person who has Wilson's disease does not release copper into bile as it should. Bile is a liquid produced by the liver that helps with digestion. As the intestines absorb copper from food, the copper builds up in the liver and injures liver tissue. Eventually, the damage causes the liver to release the copper directly into the bloodstream, which carries the copper throughout the body. The copper buildup leads to damage in the kidneys, brain, and eyes. If not treated, Wilson's disease can cause severe brain damage, liver failure, and death.
Symptoms usually appear between the ages of 6 and 20 years, but sometimes not until the age of 30, and in rare instances up to age 50. The most characteristic sign is the Kayser-Fleischer ring--a rusty brown ring around the cornea of the eye that can be seen only through an
eye exam[?]. Other signs depend on whether the damage occurs in the liver,
blood,
central nervous system,
urinary system, or
musculoskeletal system[?]. Many signs would be detected only by a doctor, like swelling of the liver and
spleen; fluid buildup in the lining of the
abdomen;
anemia; low platelet and
white blood cell count in the blood; high levels of
amino acids,
protein,
uric acid, and
carbohydrates in
urine; and softening of the
bones. Some symptoms are more obvious, like
jaundice, which appears as yellowing of the eyes and skin;
vomiting blood; speech and language problems; tremors in the arms and hands; and rigid
muscles.
Wilson's disease is
diagnosed through tests that measure the amount of copper in the blood, urine, and liver. An eye exam would detect the Kayser-Fleischer ring.
The disease is treated with lifelong use of D-
penicillamine[?] or
trientine hydrochloride[?],
drugs that help remove copper from
tissue. Patients will also need to take
vitamin B6 and follow a low-copper
diet, which means avoiding
mushrooms,
nuts,
chocolate, dried
fruit, liver, and
shellfish. Taking extra
zinc may be helpful in blocking the intestines' absorption of copper.
Wilson's disease requires lifelong treatment. If the disorder is detected early and treated correctly, a person with Wilson's disease can enjoy completely normal health.
Original text is from a
public domain source found at:
http://www.niddk.nih.gov/health/digest/summary/wilson/wilson.htm
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