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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

Pretransplant and posttransplant performance scores for 9 patients who underwent LT for AIP and had significant postoperative survival. The following scale is used to describe their quality of life: (1) the patient is able to carry out normal activities without restriction, (2) the patient is restricted only from physically strenuous activity, (3) the patient can move freely and is capable of self-care but is incapable of any form of work, (4) the patient is capable of only limited self-care and is mostly confined to a bed or a chair, and (5) the patient is completely reliant on nursing/medical care. All these patients were prevented from performing any type of work before LT; 6 patients were mostly confined to a bed or a chair or were completely reliant on medical/nursing care. LT led to significant improvements in the quality of life for most of the patients; their posttransplant limitations either depended on chronic neurological deficits sustained before transplantation or resulted from those deficits (eg, contractures).
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fig03: Pretransplant and posttransplant performance scores for 9 patients who underwent LT for AIP and had significant postoperative survival. The following scale is used to describe their quality of life: (1) the patient is able to carry out normal activities without restriction, (2) the patient is restricted only from physically strenuous activity, (3) the patient can move freely and is capable of self-care but is incapable of any form of work, (4) the patient is capable of only limited self-care and is mostly confined to a bed or a chair, and (5) the patient is completely reliant on nursing/medical care. All these patients were prevented from performing any type of work before LT; 6 patients were mostly confined to a bed or a chair or were completely reliant on medical/nursing care. LT led to significant improvements in the quality of life for most of the patients; their posttransplant limitations either depended on chronic neurological deficits sustained before transplantation or resulted from those deficits (eg, contractures).

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.


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)

Pretransplant and posttransplant performance scores for 9 patients who underwent LT for AIP and had significant postoperative survival. The following scale is used to describe their quality of life: (1) the patient is able to carry out normal activities without restriction, (2) the patient is restricted only from physically strenuous activity, (3) the patient can move freely and is capable of self-care but is incapable of any form of work, (4) the patient is capable of only limited self-care and is mostly confined to a bed or a chair, and (5) the patient is completely reliant on nursing/medical care. All these patients were prevented from performing any type of work before LT; 6 patients were mostly confined to a bed or a chair or were completely reliant on medical/nursing care. LT led to significant improvements in the quality of life for most of the patients; their posttransplant limitations either depended on chronic neurological deficits sustained before transplantation or resulted from those deficits (eg, contractures).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig03: Pretransplant and posttransplant performance scores for 9 patients who underwent LT for AIP and had significant postoperative survival. The following scale is used to describe their quality of life: (1) the patient is able to carry out normal activities without restriction, (2) the patient is restricted only from physically strenuous activity, (3) the patient can move freely and is capable of self-care but is incapable of any form of work, (4) the patient is capable of only limited self-care and is mostly confined to a bed or a chair, and (5) the patient is completely reliant on nursing/medical care. All these patients were prevented from performing any type of work before LT; 6 patients were mostly confined to a bed or a chair or were completely reliant on medical/nursing care. LT led to significant improvements in the quality of life for most of the patients; their posttransplant limitations either depended on chronic neurological deficits sustained before transplantation or resulted from those deficits (eg, contractures).
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.

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