Glucose supplementation in AIP management helps by doing what?

Glucose supplementation in AIP management helps by doing what?

• A. Inhibits ALA synthase
• B. Stimulates ALA synthase
• C. Has no effect on ALA synthase
• D. Increases iron absorption

Answer: (A)

Glucose supplementation helps by inhibiting ALA synthase, the first and rate-limiting step in hepatic heme synthesis. In acute intermittent porphyria, the problem is overactivity of this enzyme (ALAS1) leading to excess production of neurotoxic heme precursors like aminolevulinic acid (ALA) and porphobilinogen (PBG). Providing glucose increases insulin and energy supply, which downregulates ALAS1 expression, reducing the flux through the pathway and lowering ALA and PBG buildup. This helps alleviate symptoms. The other options don’t fit because glucose does not stimulate ALAS, has no effect, or affect iron absorption in this context.

Glucose supplementation helps by inhibiting ALA synthase, the first and rate-limiting step in hepatic heme synthesis. In acute intermittent porphyria, the problem is overactivity of this enzyme (ALAS1) leading to excess production of neurotoxic heme precursors like aminolevulinic acid (ALA) and porphobilinogen (PBG). Providing glucose increases insulin and energy supply, which downregulates ALAS1 expression, reducing the flux through the pathway and lowering ALA and PBG buildup. This helps alleviate symptoms. The other options don’t fit because glucose does not stimulate ALAS, has no effect, or affect iron absorption in this context.