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101 Acute Hepatic Porphyria 2 of 3

IF THERE IS A CLINICAL SUSPICION OF PORPHYRIA

suspect
Please note: the rapid PBG test kit is no longer available

Clinical Features of AIP

Signs and symptoms of AIP usually occur intermittently and include abdominal pain, constipation, muscle weakness, pains in the arms and legs, insomnia, emotional difficulties, rapid pulse, and high blood pressure. Muscle weakness can be severe during a prolonged attack due to the effects of Porphyria on nerves that control muscle. In fact, all symptoms of AIP appear to be due to effects on peripheral nerves, the nerves in the abdomen or the central nervous system. Precisely how Porphyria produces pain and other symptoms related to the nervous system is not yet well understood. PBG is produced in excessive amounts by the liver in AIP, but it has not been proven that excess PBG can damage nerve tissue.
Because Porphyria is rare and its symptoms often suggest other diseases, the correct diagnosis is often delayed. Attacks due to harmful drugs commonly occur before AIP is recognized. Symptoms of Porphyria can develop in some people but not in others who inherit AIP. If they occur, symptoms are characteristically intermittent. Some reasons for this variability are as follows:
  1. Certain drugs are harmful to Porphyria patients. This is likely to occur if drugs are given for other illnesses or at a time when surgery is needed.
  2. Hormones are clearly important in activating Porphyria. Quite a number of facts suggest this relationship. Porphyria patients metabolize (or break down) hormones differently than do normal subjects, and these hormone break-down products can cause the liver to make more PBG and porphyrins. In children who inherit AIP (detected by the PBG-D test), the condition is latent before puberty. After puberty, AIP may become active in some of these children.
    Attacks are more common in women who inherit AIP than in men. Attacks in women often occur during the second half of each menstrual cycle, suggesting that female hormones are more significant than male hormones in bringing AIP from the latent to the active state. Attacks can be produced by oral birth control pills that contain synthetic types of female hormones.
    Some women with AIP, on the other hand, have fewer attacks of Porphyria after certain types of birth control pills are started. This is thought to be due to a reduction in the rate of formation of their own female sex hormones, as a result of their taking the synthetic hormones in birth control pills. Thus, such synthetic hormones may, at times, be helpful. Some women have benefited from using a low-dose estrogen patch.
  3. Nutrition is another important factor that can alter the course of an AIP patient. Fasting, a marked restriction of calories or a low carbohydrate diet can cause an attack. A high intake of carbohydrate is beneficial to shorten an attack when it occurs. Most AIP patients do well between attacks on a normal diet. A person with AIP who wishes to lose weight should do so gradually. For more detailed information, you may order the Diet and Nutrition brochure from the APF, or visit the Diet and Nutrition section of this website.
  4. Environmental factors such as chemicals of various types, may play a part in predisposing a patient to increased symptoms of AIP. Some studies suggest that the chemicals that are found in cigarettes, insecticides and weed killers have this potential.
  5. Alcoholic beverages should be avoided.
  6. Other factors may precipitate or exacerbate an AIP attack such as stress of illnesses unrelated to Porphyria, extreme emotional stress or physical fatigue.

Drugs and AIP

Certain drugs are extremely important in exacerbating AIP. Patients with AIP are often treated with harmful drugs such as tranquilizers and sedatives before the disease has been diagnosed. This may lead to a severe attack. Porphyria will improve greatly after these drugs are stopped. Severe and fatal attacks are almost always related to the use of harmful drugs. This is one very important reason for testing blood relatives of known Porphyria patients to determine if they have the genetic defect.

Harmful drugs

Recommendations about drugs in AIP are based on specific test results and experience in patients with Porphyria in whom attacks have been caused by drugs. Since most new, commonly used drugs have not been tested, they should be avoided if at all possible. If a question arises, a physician or medical center specializing in Porphyria should be contacted.
The most important harmful drugs are barbiturates and sulfonamides (sulfa drugs). Barbiturates are commonly used as tranquilizers, sleeping pills and general anesthetics. Sulfa drugs are antibiotics commonly used to treat kidney and bladder infections.
You will also find a searchable database of safe and unsafe drugs on this website.


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