Encyclopedia of Health and Medicine

V–W
vasoactive intestinal peptide (VIP)
HORMONES.
See DIGESTIVE
• NAUSEA and VOMITING
• abdominal discomfort or PAIN
Wilson’s disease A hereditary disorder in which
the body does not properly metabolize copper,
which allows deposits of copper to accumulate in
various organs. Without treatment Wilson’s disease
is fatal; with treatment it is easily manageable.
Copper is an important mineral for the body’s
production of various enzymes, including those
that facilitate HEMOGLOBIN synthesis. However, the
body needs only a very small quantity of copper.
In health the LIVER discharges excess copper,
which enters the body through dietary sources,
into the BILE. The bile carries the copper into the
gastrointestinal tract for removal from the body in
the feces. The KIDNEYS also extract some copper
from the BLOOD circulation, passing it from the
body in the URINE. In Wilson’s disease a genetic
MUTATION prevents the liver from discharging
excess copper. Instead, it stores copper within its
own tissues as well as sends it back out into the
blood circulation. The blood deposits the copper in
tissues throughout the body. As the copper accumulates,
it causes scarring and other damage that
interferes with the normal functions of the tissues.
Symptoms and Diagnostic Path
The symptoms of Wilson’s disease vary according
to the body system most severely affected, which
is most often the liver or the BRAIN and SPINAL CORD.
Liver involvement produces symptoms such as
• JAUNDICE (yellowish color to the SKIN)
• HEPATOMEGALY (enlarged liver) and SPLENOMEGALY
(enlarged SPLEEN)
• ASCITES (fluid accumulation in the abdomen)
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Involvement of the brain, spinal cord, and
other structures of the NERVOUS SYSTEM produces
symptoms that may include
• difficulty moving the arms and legs
• tremors
• confusion
• difficulty speaking and swallowing
• cognitive and memory dysfunction
A conclusive clinical sign for Wilson’s disease is
the appearance of a copper-colored ring around
the iris of the EYE, called a Kayser-Fleischer ring.
Other diagnostic indications include low levels of
copper in the blood and high levels of copper in
the urine. Biopsy of the liver or the kidneys shows
the copper deposits in the tissues.
Treatment Options and Outlook
Treatment targets blocking the body’s absorption
of copper as well as removal of excessive copper
already in the body (chelation). Commonly used
chelation agents include penicillamine and trientine.
These drugs bind with the copper, allowing
the kidneys to excrete the bound molecules from
the body in the urine. Zinc acetate can help
reduce gastrointestinal absorption of copper from
the diet. Doctors recommend eating foods that are
low in copper. People who have Wilson’s disease
should drink bottled water because copper is common
in household plumbing and should avoid
cooking with copper pans or implements. The
endocrinologist may also recommend a zinc supplement,
as zinc interferes with copper absorption.