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Domino liver transplantation for familial amyloid polyneuropathy
Author(s) -
Hemming, Alan W.,
Cattral Mark S.,
Chari Ravi S.,
Greig Paul D.,
Lilly Leslie B.,
Ashby Peter,
Levy Gary A.
Publication year - 1998
Publication title -
liver transplantation and surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.814
H-Index - 150
eISSN - 1527-6473
pISSN - 1074-3022
DOI - 10.1002/lt.500040303
Subject(s) - amyloid polyneuropathy , liver transplantation , transthyretin , polyneuropathy , amyloid (mycology) , medicine , transplantation , amyloidosis , pathology , domino , biology , disease , age of onset , biochemistry , catalysis
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.