Thursday, May 3, 2007

Porphyria - Nicole Bounty


Porphyria is a disease that occurs when the enzymes of the porphyrin’s (molecules in the macrocycle) in a person’s body become infected. Basically what happens is the infected enzymes in the porphyrin pathway start to decrease in production of heme. The main problem is that porphyrin is being over produced, while the production of heme is decreasing. People can also get Porphyria from a previous liver disease, which could lead into other diseases such as jaundice (discoloration of the skin).

There are two different types of the disease; one is Acute Porphyria and the other Cutaneous Porphyria. Acute Porphyria mostly affects the nervous system, creating problems such as seizures, abdominal pain, mental disturbances, vomiting, hallucinations, depression, fast heart rate and anxiety. The disease can sometimes be caused by genetics but not always. The attacks from the disease can be caused by drugs, alcohol, some antibiotics, certain foods and also fasting. These attacks can only be triggered once the person actually has the disease.

Cutaneous Porphyria mainly affects the skin of the person causing blisters, swelling, and necrosis of the skin (death of skin cells). Also, the accumulated porphyrin changes the color of the body’s fluids to a purple color, including fingernails and teeth.

The most popular treatment for the disease is to take a certain drug, for example Hematin to try and reduce the attacks. The drug is only affective if it is given to the patient early in on in the attack of porphyria, otherwise it will not help. Also, the drug will not cure the disease, but will only decrease the painful attacks as much as possible.

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