ScientiaCME Acute hepatic porphyria: optimizing pharmacotherapeutic management strategies
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Activity Description / Statement of Need:
In this online, self-learning activity:
Acute hepatic porphyria (AHP) is an umbrella term for four types of acute porphyria, the most severe of which is acute intermittent porphyria (AIP). An estimated 80% of AHP cases are AIP, which is an inherited autosomal dominant condition that results from mutations of the third enzyme of heme synthesis, porphobilinogen deaminase. In the Western countries, it is estimated that approximately 1 in 2000 individuals are carriers of the relevant mutated genotype, although the majority have latent AIP and are clinically asymptomatic. Acute attacks occur in less than 10% of the at-risk population, reflecting the role of environmental factors, such as alcohol use, infections, and hormonal changes, among others. AHP symptoms are believed to be caused by ALAS1-mediated accumulation of ALA and PBG in the liver and bloodstream, leading to neurotoxicity.
The following HCPs: hematologists and gastroenterologists; physician assistants, nurse practitioners, and pharmacists who practice in any of the aforementioned areas of specialties; and any other healthcare professionals with an interest in or who clinically encounter patients with AHP.