Clinical strategies for managing acute intermittent porphyria : biochemical insights in targeting pain pathways and implementing them into clinical practice

Abstract

This paper investigates clinical strategies for management and therapy of acute intermittent porphyria using a biochemical approach to understand and utilize the pathophysiology of the disease in special regard to its pain symptoms and pain pathways. Specifically, the thesis focuses on the toxicity of ALA (alpha aminolevulinic acid) as well as the biochemical implications of AIP regarding the heme biosynthesis disruption. Furthermore, literature regarding several treatment options with novel approaches were reviewed to connect and bridge the intricate pain pathophysiology of AIP with modern strategies of treatment. In exact, the genesis of several metabolic implications of AIP were explored. Studies regarding the interaction of the glucose metabolism and its connection to ALAS (alpha aminolevulnic acid synthase) as well as literature regarding experimental therapies with gene modification and vector associated enzyme delivery were revised to propose new ways of clinical AIP therapy with an emphasis on pain management