Practical Recommendations in the Treatment of Acute and Chronic Life-Threatening Infectious Diseases in Patients with Acute Hepatic Porphyria.

BACKGROUND: Acute hepatic porphyrias (AHPs) represent inherited metabolic disorders of the heme biosynthesis pathway, leading to neurological and systemic impairment. Despite the presence of well-recognized chronic symptoms and signs, acute neurological, both neuromuscular and central neurological complications pose a significant challenge in clinical practice, with a potential risk of greater severity and mortality during acute decompensation episodes of AHPs. Care related to the prescription of medications, considering the risk of porphyrinogenicity, is a major and recurring concern in the acute and chronic management of AHP patients. Infectious clinical complications are significant issues in both outpatient and hospital settings for patients with AHPs. It is crucial to identify therapeutic regimens with the best safety and efficacy profiles for treating such infectious complications in AHP patients. The scarcity of structured knowledge available in guidelines and recommendations often leads to the use of therapeutic options with higher potential risks in treating patients with AHPs.

OBJECTIVES: This review article aims to provide practical recommendations for managing the most significant infectious complications in clinical practice, with a focus on their impact on the clinical care of patients with AHPs.