Acute intermittent porphyria Symptoms

Acute intermittent porphyria (AIP) is a rare metabolic disorder that affects heme production as a result of porphobilinogen deaminase deficiency. The heme is a substance that biosynthesized in both the bone marrow and the liver. It is precursive to hemoglobin, which is necessary to bind oxygen in the bloodstream. The mean age at diagnosis is 33 years old and more likely to present in women versus men.

The clinical presentation of AIP is non-specific and highly variable. The acute attack happens when porphyrin’s level becomes elevated. In very severe cases hospital visits with emergency procedures are needed to prevent death.

Attacks of acute porphyria may unfold or progress with the following symptoms:

1. Anxiety and insomnia
2. Severe abdominal pain
3. Pain in arms, legs, or back
4. Reddish colored urine
5. Constipation 
6. Hypertension
7. Confusion and hallucinations 
8. Breathing difficulties 
9. Vomiting 

Hemin (Panhematin) is approved by FDA for Amelioration of recurrent attacks of acute intermittent porphyria (AIP) temporarily related to the menstrual cycle in susceptible women and similar symptoms which occur in other patients with AIP, porphyria variegata, and hereditary coproporphyria.