Acute Intermittent Porphyria Treatment & AIP Drugs Information

Acute intermittent is one type of the porphyrias, a group of diseases connecting defects in heme metabolism and that result in excessive secretion of porphyrins. It is characterized by a deficiency of the enzyme porphobilinogen deaminase. This enzyme speeds the conversion of porphobilinogen to hydroxymethylbilane. In this disease the porphyrin precursors, porphobilinogen and amino-levulinic acid (ALA), accumulate. The major problem appears to be neurologic injury that leads to peripheral and autonomic neuropathies and psychiatric manifestations.

Most of patients become symptomatic at age from 18-40 years. Attacks occurring prior to teenage years or after age of 40 years is unusual unless a major provocation, such as new use of phenobarbital or estrogens, has occurred. The sequence of events in attacks usually is (1) abdominal pain, (2) psychiatric symptoms, such as hysteria, and (3) peripheral neuropathies, mainly motor neuropathies.

The symptoms of AIP are never observed in pre-pubescent children and are seen more frequently in women than in men.

Acute Intermittent Porphyria Causes and Treatment

In normal circumstances, heme combination comes in the mitochondridon, proceeds into the cytoplasm,and finishes back in the mitochondrion. Without porphobilinogen deaminase, an essential cytoplasmic enzyme, heme synthesis cannot finish, and the metabolite porphobilinogen accumulates in the cytoplasm.

The action goal for acute attacks of porphyria is to reduce heme synthesis and reduce the production of porphyrin precursors.

People experiencing severe attacks, especially those with strict neurologic symptoms, should be treated with hematin in a dose of 4 mg/kg/d for 4 days.

High doses of glucose (400 g/d) can inhibit heme synthesis and are useful for treatment of mild attacks.

Pain control is best achieved with narcotics. Laxatives and stool softeners should be administered with the narcotics to avert exacerbating existing constipation.Treat seizures with Neurontin.

Most classic antiseizure medicines can lead to acute porphyria attacks.

Patient should receive a high-carbohydrate diet during the attack. If the patient is not capable to eat, intravenous glucose should be administered.Eating a balanced diet is more important than eating one rich in glucose between attacks.

Acute Intermittent Porphyria in pregnancy

Pregnant women with severe abdominal pain are very similar to that for non pregnant women with acute abdomen, but the physiologic changes related with pregnancy must be considered when findings from the history and physical test. The case of a patient with acute yellow atrophy of the liver subsequently had two uncomplicated pregnancies bringing the total number to 8 cases.

Patients with acute pregnancy related hypertension were randomized to treatment with 200 mg oral labetalol or 10 mg intramuscular hydralazine. Labetalol was found to be at least as effective, more predictable, and more acceptable and produced no change in heart rate compared to hydralazine.

1. By Karen at 14th November, 2010

   We have found in our experience flair ups can be controlled by avoiding triggers.
   My husbands triggers are ANYTHING containing SOY (it is in almost Everything ! oil & shortening which is in everything), peppers (vegetable, not black pepper), anything in the broccoli / cauliflower / cabbage family, processed foods, tobacco & alcohol.