
Background
Pathophysiology
- Porphyrias encompass a group of 8 genetic disorders and may be1-6
- Inherited in an autosomal dominant (e.g., acute hepatic porphyria [AHP]) or recessive X-linked pattern (e.g., congenital erythropoietic porphyria [CEP]); or6
- Sporadic (e.g., porphyria cutanea tarda [PCT])6
Why sign up with Medthority?
Develop your knowledge with our disease and condition focused Learning Zones
Access content from credible sources, including expert-led commentary, videos, podcasts, and webinars as well as clinical trials, treatment information and guidelines
Personalised dashboard providing updates and recommendations for content within your areas of interest
Precipitating Factors
- Certain precipitating factors can cause symptomatic disease and strongly influence the occurrence and severity of signs and symptoms of porphyria (see table).1,2
- The most frequent recognised cause of acute attacks is medications.15
- An observational study in 90 patients diagnosed with acute intermittent porphyria (AIP) reported that medications and weight-loss diets triggered acute attacks in 37% and 22% of cases, respectively.16
- Other precipitants are metabolic, emotional, or physical stresses, including those brought on by intercurrent infection or surgery.3,7,9
Why sign up with Medthority?
Develop your knowledge with our disease and condition focused Learning Zones
Access content from credible sources, including expert-led commentary, videos, podcasts, and webinars as well as clinical trials, treatment information and guidelines
Personalised dashboard providing updates and recommendations for content within your areas of interest
of interest
are looking at
saved
next event
This program was sponsored and funded by Alnylam Pharmaceuticals, Inc, based on an interview with Herbert L. Bonkovsky, MD, FACP, FACG, FAGA, FAASLD. Dr Bonkovsky received a fee for participation in this program. AS1-CEMEA-00184 . Date of preparation: February 2021.
For Healthcare Professionals Only.