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: The median follow-up time was 23.4 months (range = 3.2-109 months), and there were 2 recorded deaths at 98 days and 26 months. The patient who died at 98 days had been ventilator-dependent for several months before transplantation. This patient required the evacuation of a hematoma 2 weeks after transplantation, and renal failure, pressure sores, and recurrent chest and line sepsis developed; the patient never regained independence from intensive organ support and died from multiorgan failure. The death at 26 months was also a result of multiorgan failure, but no further details could be obtained. One patient underwent regrafting after 13 days for hepatic artery thrombosis (HAT) but remained alive and well 53 months later. The remaining 8 patients were alive for 3.2 to 109 months after transplantation (Fig. 1). The rates of survival for these patients are comparable to the 3-month and 5-year survival rates for elective adult LT at the reporting centers over the same time period (93% and 77%, respectively).
Affiliation: Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom. firstname.lastname@example.org