Liver transplantation for acute intermittent porphyria is complicated by a high rate of hepatic artery thrombosis.
Bottom Line: The effects of previous neuronal damage such as joint contractures were not improved by transplantation.Thus, impaired quality of life in the surviving patients was usually a result of preoperative complications.Refractory AIP is an excellent indication for LT, and long-term outcomes for carefully selected patients are good.
Affiliation: Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom. email@example.comShow MeSH
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Mentions: LT resulted in no further porphyria attacks for the recipients, who experienced a complete biochemical resolution of their urinary PBG excretion (Fig. 2A). Urinary PBG and ALA levels can be expected to return to normal within 24 and 72 hours of transplantation, respectively (Fig. 2B), and to remain normal thereafter. Monitoring PBG levels after transplantation is, therefore, unnecessary. One patient who underwent transplantation in 2002 at the center in Birmingham returned to full-time employment and gave birth to a healthy child.
Affiliation: Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom. firstname.lastname@example.org