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Liver transplantation for acute intermittent porphyria is complicated by a high rate of hepatic artery thrombosis.

Dowman JK, Gunson BK, Mirza DF, Bramhall SR, Badminton MN, Newsome PN, UK Liver Selection and Allocation Working Par - Liver Transpl. (2012)

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.

View Article: PubMed Central - PubMed

Affiliation: Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom. j.k.dowman@bham.ac.uk

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Survival after LT for AIP. Two of the 10 patients who underwent LT for AIP died (1 patient at 98 days and 1 patient at 26 months). The median follow-up time was 23.4 months.
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fig01: Survival after LT for AIP. Two of the 10 patients who underwent LT for AIP died (1 patient at 98 days and 1 patient at 26 months). The median follow-up time was 23.4 months.

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).


Liver transplantation for acute intermittent porphyria is complicated by a high rate of hepatic artery thrombosis.

Dowman JK, Gunson BK, Mirza DF, Bramhall SR, Badminton MN, Newsome PN, UK Liver Selection and Allocation Working Par - Liver Transpl. (2012)

Survival after LT for AIP. Two of the 10 patients who underwent LT for AIP died (1 patient at 98 days and 1 patient at 26 months). The median follow-up time was 23.4 months.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3472026&req=5

fig01: Survival after LT for AIP. Two of the 10 patients who underwent LT for AIP died (1 patient at 98 days and 1 patient at 26 months). The median follow-up time was 23.4 months.
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).

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.

View Article: PubMed Central - PubMed

Affiliation: Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom. j.k.dowman@bham.ac.uk

Show MeSH
Related in: MedlinePlus