Porphyrins
are chemical compounds that are stepping stones along the pathway which
leads to the formation of heme in man, and to chlorophyll in plants.
They are responsible for the fact that "blood is red, and grass
is green." Heme is essential if the body is to work properly.
In
porphyria, the cells do no convert porphyrins to heme in a normal manner.
Because of this, porphyrins build up in the body and are excreted in
the urine and stool in excessive amounts. When present in very high
levels, they cause the urine to have a spectacular port wine color.
The
symptoms of porphyria fall into two major groups. Some patients have
attacks in which the nerves of the body do not function properly. Abdominal
pain and weakness result. Other patients have problems with the parts
of the skin that are exposed to the sun and can cause pain and swelling
of the skin or the formation of blisters. Treatment is available for
both types of symptoms.
The
disease can appear in childhood, but the onset most frequently occurs
between the ages of 20 and 40. The disease affects men less often than
women, in whom attacks are related to the menstrual cycle. Long latent
periods may separate these attacks, which can be precipitated by drugs,
infections, alcohol consumption and dieting.
Proper
diagnosis and treatment of porphyria depends on chemical studies of
the blood, urine and stool. Since certain types of porphyria are genetic
diseases, studies should also be done on children and blood relatives
of affected individuals.
The
American Liver Foundation is the only national voluntary health organization
dedicated to preventing, treating, and curing hepatitis and all other
liver and gallbladder diseases through research and education.
American Liver Foundation
1425 Pompton Avenue, Cedar Grove, NJ 07009
1-800-223-0179
Copyright
© 1995
The American Liver Foundation
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