Any of various organic compounds containing four pyrrole rings, occurring universally in protoplasm, and functioning as a metal-binding cofactor in hemoglobin, chlorophyll, and certain enzymes.
2006-06-13 12:16:23
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answer #1
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answered by What?! 4
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Porphyria
Pronounced: por-fir-E-ah
by Debra Wood, RN
Definition
The term porphyria refers to a group of at least eight disorders that differ clinically from each other, but all share the same problem of accumulation of "porphyrins" or "porphyrin precursors" in the body. Porphyrin is a building block for heme. And heme is the iron-containing part of the hemoglobin in red blood cells that carries oxygen to all of the organs in our body.
The build up of the prophyrins in the body cause symptoms in two major organ systems: the nervous system and the skin.
Effects on the nervous system occur in the acute porphyrias. The diagnosis is often delayed because the symptoms are nonspecific. Some patients have attacks in which the nerves of the body do not function properly. Abdominal pain and weakness can be a symptom.
Skin manifestations can include burning, blistering and scarring of sun-exposed areas.
The terms "porphyrin" and "porphyria" are derived from the Greek word "porphyrus" meaning purple. Urine from some porphyria patients may be reddish in color due to the presence of excess porphyrins and related substances, and the urine may darken after standing in the light. Excess prophyrins are also excreted in the stool.
Some types of porphyria have their onset in early childhood, some at puberty, and others during adulthood. 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 periods may separate attacks, which can be precipitated by drugs, infections, alcohol consumption and dieting.
Causes
Most types of porphyrias are inherited. One subtype, porphyria cutanea tarda, is acquired. The porphyrias may be inherited as either autosomal dominant (caused by inheriting one abnormal copy of the gene), or autosomal recessive (requires inheritance of two abnormal genes) traits depending on which type of porphyria is involved.
Risk Factors
A risk factor is something that increases your chances of getting a disease or condition. The most common risk factor for porphyria is having a family member with this disease. Also, whites are at greater risk than blacks or Asians.
If you have porphyria, certain factors can increase your risk of a porphyria attack. These include: drugs, chemicals, alcohol, dieting, and sunlight.
2006-06-13 12:19:25
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answer #2
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answered by Mollie 2
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