Which factor worsens acute intermittent porphyria symptoms?

Which factor worsens acute intermittent porphyria symptoms?

• A. Sleep deprivation
• B. Stimulation of ALA synthase
• C. Vitamin C
• D. Prolonged hydration

Answer: (B)

In acute intermittent porphyria, the problem lies downstream in the heme synthesis pathway because a key enzyme (porphobilinogen deaminase) is deficient. The amount of heme precursor entering the pathway is set by ALA synthase, the first and rate-limiting step. When ALA synthase is stimulated, more ALA and downstream porphyrin precursors are produced. Since the downstream step is defective, these precursors accumulate and trigger the neurovisceral symptoms of AIP. So factors that induce ALA synthase directly fuel the crisis. Sleep deprivation can act as a general stressor but does not specifically drive the excess production of porphyrin precursors the way inducing ALA synthase does. Vitamin C is not a known trigger, and prolonged hydration helps prevent dehydration-related triggers rather than worsen them.

In acute intermittent porphyria, the problem lies downstream in the heme synthesis pathway because a key enzyme (porphobilinogen deaminase) is deficient. The amount of heme precursor entering the pathway is set by ALA synthase, the first and rate-limiting step. When ALA synthase is stimulated, more ALA and downstream porphyrin precursors are produced. Since the downstream step is defective, these precursors accumulate and trigger the neurovisceral symptoms of AIP. So factors that induce ALA synthase directly fuel the crisis. Sleep deprivation can act as a general stressor but does not specifically drive the excess production of porphyrin precursors the way inducing ALA synthase does. Vitamin C is not a known trigger, and prolonged hydration helps prevent dehydration-related triggers rather than worsen them.