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Question

Please may I have some information about acute intermittent porphyria?

Answer

Acute intermittent porphyria (AIP) is an inherited disease, caused by a defect in one of the enzymes (PGB deaminase) controlling the manufacture of heme, which is a component of red blood cells. This results in a disease which affects the system which manufactures blood.

AIP is the most common of the genetic porphyrias. The highest incidence occurs in Lapland, Scandinavia, and in the United Kingdom.

Symptoms first arise after puberty, and the disease is slightly more common in women than in men.

The main symptom is that of abdominal pain, which is usually the first symptom of an acute attack. It can be localised to one area of the abdomen, or generalised, and can be confused with abdominal pain which has a surgical cause.

There may also be nausea, vomiting, constipation, and abdominal distention. The person may also have a fast heart beat and high blood pressure. Up to 40% of patients have high blood pressure between attacks.

Problems with the nervous system are common in AIP. Muscle weakness often begins in the muscles of the lower legs, and can involve the arms and hands and feet.

Acute attacks can be accompanied by seizures, particularly in patients who have been vomiting.

The course of an attack is very variable, both in individuals and between patients, with attacks lasting from a few days to several months.

Exacerbations may occur just before a period in women, and in those who are dieting or reducing their food intake for other reasons. Stress is also considered to exacerbate the condition, as are alcohol, other illnesses and surgery.

Diagnosis is usually made by demonstrating decreased activity of PGB deaminase in the red blood cells.

Treatment between attacks consists of ensuring adequate food intake, avoiding drugs which precipitate porphyria and prompt treatment of any other illnesses.

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