Overview:
Acute hepatic porphyria (AHP) is characterized by acute attacks in which excessive heme production is induced following an exposure to a trigger. The majority of these attacks initially manifest with non-specific symptoms such as abdominal pain, mental symptoms (eg, severe fatigue, inability to concentrate) with or without autonomic dysfunction. The clinical expression is greatly variable and can mimic many other diseases, leading to misdiagnosis in many cases. Undiagnosed patients can be subjected to unnecessary medical and surgical treatments.
If left untreated, attacks can result in seizures, paralysis, and, in rare situations, death. Serious complications of acute attacks are often a consequence of administration of porphyrinogenic drugs that might aggravate the attack symptoms. Early and definitive diagnosis is therefore mandatory to improve outcomes and assure that potentially harmful drugs are avoided. The phases of AHP treatment and management include treatment of the acute attack, prevention of attacks, and monitoring for long term complications.
This program aims to provide clinicians with up-to-date information regarding best practices in the diagnosis and management of AHP. Through education, clinicians across a variety of disciplines will be better placed to provide optimal care for their patients with AHP and thereby improve outcomes and quality of life.
Learning Objectives:
Upon completion of this activity, participants will be able to:
- Implement current assessment strategies to accurately diagnose patients with AHP based on clinical presentation
- Evaluate clinical trial data for current AHP therapies
- Design multidisciplinary management plans for patients with AHP
