Treatment of the Acute Attack

Treatment of the Acute Attack

Treatment of acute porphyric attacks includes three different approaches: elimination of precipitating factors, specific therapy, and symptomatic treatment.
Elimination of Precipitating Factors
Acute porphyric attacks are often precipitated by factors such as certain drugs, excessive alcohol consumption, and fasting

Specific Therapy

Heme therapy and carbohydrate loading are specific therapies for acute porphyric attacks because these agents are able to reduce the overproduction of excess amounts of porphyrin precursors. Heme is much more effective in this regard than glucose.
Specific treatment is justified only when a patient has symptoms and signs compatible with an acute attack and increased excretion of porphobilinogen in the urine. The presence of symptoms without raised levels of porphobilinogen, or the finding of raised levels of porphobilinogen, 5-aminolevulinic acid, or porphyrins without symptoms, is not an indication for specific treatment.
Mild attacks are sometimes treated with glucose loading (by the oral or intravenous route, 400 to 500 grams daily) rather than heme. Severe attacks or symptoms that on the basis of clinical findings seem likely to progress should be treated with heme. When indicated, heme therapy should be started without delay because the effect of any treatment may be poor later in an attack after onset of neuropathy. Patients who have received heme may still require administration of some intravenous glucose and other nutrients for nutritional support.
The usual dose of heme is 3 mg per kg daily given intravenously for 4 consecutive days. The course may be shorter if the patient responds quickly or longer if the attack persists more than 4 days. There are two commercial heme preparations: hemin (Panhematin) and heme arginate (Normosang).
Heme arginate is the preferred preparation for intravenous use because it has been studied intensively and seems to have fewer side effects than hemin. Heme arginate has been registered in many European and other countries. In the United States, heme arginate has been under investigation but will be available soon. Hemin has been long available in the United States.

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