Domino liver transplantation for familial amyloid polyneuropathy

Familial amyloid polyneuropathy is an autosomal dominant disorder in which the liver produces a variant prealbumin that is deposited along nerves, leading to a progressive and fatal polyneuropathy that begins in the third decade of life. Liver transplantation has been the only successful treatment t...

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Veröffentlicht in:Liver Transplantation and Surgery 1998-05, Vol.4 (3), p.236-238
Hauptverfasser: Hemming, Alan W., Cattral, Mark S., Chari, Ravi S., Greig, Paul D., Lilly, Leslie B., Ashby, Peter, Levy, Gary A.
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Sprache:eng
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Zusammenfassung:Familial amyloid polyneuropathy is an autosomal dominant disorder in which the liver produces a variant prealbumin that is deposited along nerves, leading to a progressive and fatal polyneuropathy that begins in the third decade of life. Liver transplantation has been the only successful treatment to date. Apart from the production of the variant protein, there are no other abnormalities in these amyloid livers. We describe two cases in which, at the time of transplantation, the amyloid livers were subsequently used for transplantation in another patient, and we discuss the implications.
ISSN:1074-3022
1527-6465
1527-6473
DOI:10.1002/lt.500040303