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Porphyria, Acute Intermittent



Porphyria, Acute Intermittent Definition


Acute intermittent porphyria (AIP) is a metabolic disorder characterized by decreased level of the enzyme porphobilinogen deaminase. It is a rare disease that affects the production of heme, a prosthetic group of hemoglobin responsible for oxygen binding.


Porphyria, Acute Intermittent Symptoms and Signs


Patients with AIP experience ?attacks? characterized by severe nausea, chronic pain and seizures. AIP attacks are often induced by one or a combination of the ?four Ms,? namely, menstruation, medication, malnutrition and maladies. AIP patients who experience frequent attacks often develop neurophatic pain in their extremities and in their gut. Most AIP also suffer from depression as caused by offending symptoms. Persons with AIP often have reddish urine during attacks. They may also turn purple when exposed to ultraviolet light for an extended period of time. Other symptoms include constipation, abdominal pain and muscle weakness.


Porphyria, Acute Intermittent Treatment


AIP sufferers are advised to have a high-carbohydrate diet because glucose can aid in recovery during attacks. If a particular substance is discovered to be causing attacks then that substance must be avoided. In instances where pain is severe, opiates are recommended to make condition tolerable. Nausea can be treated with drugs as well as hot water baths. The patient though must be be cautious of being burned or falling in the shower or bath.


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