How To Treat Porphyrias

Porphyrias are inherited or adquired diseases that result from an abnormal metabolism of heme biosynthesis pathway. The main causes are enzyme deficiencies that lead to heme pathways intermediates accumulation. Porphyrias are: Intermitent Acute Porphyria, Hereditary Coproporphyria, Variegate Porphyria and Porphyria Cutanea Tarda. It is important to recognize that the three most common types of porphyrias are Intermitent Acute Porphyria (IAP), Porphyria Cutanea Tarda (PCT) and Protoporphyria (PP). Heme biosynthesis occurs almost entirely in the liver and bone marrow and is composed by eight reactions, each one of these being catalyzed by an especific enzyme. There are certain forms of Porphyria that are believed to be inherited from one of the parents. In some cases if both the parents are infected then there is a likelihood that one gene from each parent may be inherited and the infection / disorder is passed on. There is a high chance that the infection / disorder can be passed on to the children.

Although acute intermittent porphyria (AIP) is the most common acute porphyria, it is still quite rare. It usually affects women between 20 and 40 years old and men between 30 and 50. Because patients with an acute attack of AIP can rapidly develop a life-threatening illness, physicians often recommend hospitalization.

Treatment For Porphyrias

Carbohydrate restriction should be avoided. Meals should provide adequate sources of complex carbohydrates to maintain blood glucose levels in reference ranges.

Women with acute porphyrias who experience cyclic attacks in the week prior to menstruation may benefit from suppression of ovulation by exogenous luteinizing hormone–releasing hormone agonist therapy

Mild attacks (those in which pain levels can be adequately addressed by standard doses of acetaminophen, aspirin, or codeine and in which vomiting does not develop) may remit over 1-2 days with conservative management. Any porphyrinogenic drug must be eliminated, and adequate fluid and carbohydrate intake must be ensured.



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