[Anesthesia in patients with acute porphyria]

Area of Science:

• Biochemistry
• Genetics
• Metabolic Disorders

Background:

• Porphyrias are a group of rare, inherited metabolic disorders impacting heme biosynthesis.
• Specific enzyme deficiencies lead to the accumulation and excretion of heme precursors.
• Diagnosis relies on biochemical detection of porphyrins and precursors in bodily fluids.

Purpose of the Study:

• To review the classification and clinical presentation of porphyrias.
• To highlight the relevance of acute hepatic porphyrias in anesthesiology.
• To emphasize safe anesthetic management and treatment strategies for porphyria.

Main Methods:

• Review of existing literature on porphyrias.
• Analysis of diagnostic criteria and biochemical markers.
• Discussion of clinical manifestations and management protocols.

Main Results:

• Porphyrias are classified into acute and non-acute forms with diverse neurovisceral and cutaneous symptoms.
• Acute hepatic porphyrias pose risks in the perioperative setting due to common precipitating factors.
• Adherence to recommendations ensures safe anesthetic management.

Conclusions:

• Early identification and biochemical confirmation are key for porphyria diagnosis.
• Awareness of porphyria is critical for anesthesiologists managing perioperative risks.
• Immediate heme arginate administration is vital for treating acute porphyric attacks.