| Description |
Porphyrias are a group of inherited or acquired enzymatic defects of heme biosynthesis. Porphyria is a rare yet potentially life threatening disorder which may be induced by multiple factors including particular medications. There is a need for rapid and accurate diagnosis of acute porphyria because treatment is available if given early in the attack. The purpose of this evidence-based project was to give an overview of hepatic porphyria as it relates to anesthesia. A case summary is provided. A basic description of the physiology of porphyria is included. Three types of hepatic porphyria may be induced by drugs. Tables are incorporated which differentiate between benign drugs in the porphyric patient as well as drugs shown to potentially precipitate a porphyric crisis. Signs and symptoms of porphyria along with diagnostic tools are discussed. Anesthetic management including careful pre-operative history, suggested laboratory tests, medications involved, IV fluids, avoidance of regional anesthesia, monitoring, and management of acute porphyric crisis is reviewed. |
