what enzyme is deficient in acute intermittent porpharia?
acute intermittent porphyria is a autosomal dominant disorder caused by a defect in the activity of porphobilinogen deaminase enzyme. Many case exist in latent form, but in manifest case it is more frequently seen in women. The estimated prevalence of the disorder is 5-10 case per 100,000 population. The latent form of the disease may exist indefinitely. Certain drugs, infections, and excessive dieting (starvation) can precipitate attacks. The most common drugs are sulfonamides and barbiturates (often seen when give Phenobarbital for pain relief with dental surgery).
