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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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Related in: MedlinePlus

AIP activity after LT. (A) Urine PBG/creatinine ratios for 9 of the 10 AIP patients before and after LT. All 9 patients experienced a complete biochemical resolution of their disease (reference level < 1.5 μmol/mmol). The 10th patient had an elevated pretransplant ratio of 74.3 μmol/mmol, but no posttransplant level was available. However, this patient was asymptomatic after transplantation, and the posttransplant level was, therefore, expected to be normal. (B) Short-term urinary PBG/creatinine and ALA/creatinine ratios for a single AIP patient before and after LT. The urinary PBG level returned to normal within 24 hours and remained normal thereafter. The urinary ALA excretion level returned to normal within approximately 72 hours.
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fig02: AIP activity after LT. (A) Urine PBG/creatinine ratios for 9 of the 10 AIP patients before and after LT. All 9 patients experienced a complete biochemical resolution of their disease (reference level < 1.5 μmol/mmol). The 10th patient had an elevated pretransplant ratio of 74.3 μmol/mmol, but no posttransplant level was available. However, this patient was asymptomatic after transplantation, and the posttransplant level was, therefore, expected to be normal. (B) Short-term urinary PBG/creatinine and ALA/creatinine ratios for a single AIP patient before and after LT. The urinary PBG level returned to normal within 24 hours and remained normal thereafter. The urinary ALA excretion level returned to normal within approximately 72 hours.

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.


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)

AIP activity after LT. (A) Urine PBG/creatinine ratios for 9 of the 10 AIP patients before and after LT. All 9 patients experienced a complete biochemical resolution of their disease (reference level < 1.5 μmol/mmol). The 10th patient had an elevated pretransplant ratio of 74.3 μmol/mmol, but no posttransplant level was available. However, this patient was asymptomatic after transplantation, and the posttransplant level was, therefore, expected to be normal. (B) Short-term urinary PBG/creatinine and ALA/creatinine ratios for a single AIP patient before and after LT. The urinary PBG level returned to normal within 24 hours and remained normal thereafter. The urinary ALA excretion level returned to normal within approximately 72 hours.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig02: AIP activity after LT. (A) Urine PBG/creatinine ratios for 9 of the 10 AIP patients before and after LT. All 9 patients experienced a complete biochemical resolution of their disease (reference level < 1.5 μmol/mmol). The 10th patient had an elevated pretransplant ratio of 74.3 μmol/mmol, but no posttransplant level was available. However, this patient was asymptomatic after transplantation, and the posttransplant level was, therefore, expected to be normal. (B) Short-term urinary PBG/creatinine and ALA/creatinine ratios for a single AIP patient before and after LT. The urinary PBG level returned to normal within 24 hours and remained normal thereafter. The urinary ALA excretion level returned to normal within approximately 72 hours.
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.

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