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: There was no evidence of neurological deterioration in the perioperative period, which had been a theoretical concern. Although the performance scores improved after transplantation (see Fig. 3), there was no evidence of any marked improvements in longstanding neurological deficits or in complications due to those neurological deficits. These residual complications were most likely due to the effects of neuronal deficits (eg, contractures) rather than actual ongoing neuronal deficits, but nerve conduction studies would have been required for confirmation. The side effects of earlier interventions, such as tracheal damage from ventilation, may also remain.
Affiliation: Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom. firstname.lastname@example.org