Hereditary amyloidosis with progressive peripheral neuropathy associated with apolipoprotein Al Gly26Arg: Outcome of hepatorenal transplantation

Abstract

Liver transplantation (LT) has been reported in only 1 patient with hereditary variant apolipoprotein AI (apoAI) amyloidosis and was associated with a 50% decrease in production of variant apoAI. The potential for this to benefit clinical manifestations of apoAI amyloidosis such as peripheral neuropathy has not been determined. A 59-yr-old Irish-born male with hereditary systemic amyloidosis associated with apoAI Gly26Arg, which was suspected to be the cause of a progressive peripheral neuropathy, along with end-stage renal failure and hepatic dysfunction, underwent hepatorenal transplantation. Evaluation of his clinical outcome included serial neurological examinations and nerve conduction ..