Orthotopic liver transplantation is an established treatment for end-stage liver diseases and for some severe metabolic disorders and hepatic cancers, but an organ shortage is the limiting factor in meeting the need for the procedure. Explanted livers from patients with metabolic liver diseases who are undergoing liver transplantation, so-called domino livers, can be one of the solutions to diminish the organ shortage. Familial amyloidotic polyneuropathy (FAP) is an autosomal dominant disease associated with a mutation of the TTR gene. The liver produces variant transthyretin (TTR) amyloid fibrils, which accumulate in body connective tissues and various organs such as the heart, kidney, and small intestine. The result is dysfunction of these organs, leading to severe disability. The patients die 9–15 years after the onset of symptoms, due to malnutrition or heart complications. Until recently, the only potentially curative treatment has been liver transplantation. Because the FAP liver is entirely normal, apart from producing the mutated variant TTR, these explanted livers are well suited to be used for transplantation in selected patients. Obviously, there is a risk of transmitting the FAP disease by the explanted liver, but given the slow progression of FAP and the desperate situation of the potential domino liver recipient, we believe that the benefits outweigh the risks . The first domino liver transplantation (DLT) using the explanted liver from an FAP patient was performed in Portugal in 1995. With time, the procedure has evolved strongly, and the technique is today used worldwide with good outcome [2, 3].
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