Porphyrias: Surgical Therapy

In the setting of acute intermittent porphyria (AIP), liver transplantation (LTx; implantation of a foreign organ) is an option and may lead to cure. The procedure corrects the deficiency of the enzyme hepatic porphobilinogen deaminase (PBG-D), subsequently normalizing levels of porphobilinogen (PBG) and delta-aminolevulinic acid (ALA).

Liver transplantation may also be required for protoporphyria (secondary (acquired) porphyria) because of marked cholestasis (biliary congestion) and cirrhosis (liver shrinkage).