Evaluation of domino liver transplantations in Germany

Abstract

SummaryA retrospective multicenter study has been conducted to evaluate domino liver transplantations (DLTs) in Germany. The study provides insight into survival and features having an impact on the assessment of neuropathy after DLT. In addition, a neurologic follow-up program with a scheme to estimate the likelihood of de novo amyloidosis is presented. A series of 61 DLTs at seven transplant centers in Germany was enrolled. The mean age of domino recipients at the time of transplantation was 58 years, 46 of them being men, and 15 being women. The median follow-up was 46 months. The overall 1-, 3-, and 5-year survival of domino recipients was 81.6%, 70.8% and 68.8%, respectively. Causes of death were primarily not related to familial amyloidosis. The main indication of DLT was hepatocellular carcinoma. Two of the reported domino recipients developed symptoms and signs of de novo amyloidosis within 10 years after transplantation. A total of 30 domino graft recipients (49.18%) presented with diabetes post transplantation. In conclusion, an advanced follow-up program is crucial to evaluate the risk of transmitting familial amyloidosis by DLT and to establish more strict selection criteria for domino recipients.

Evaluation of Operative Risk in De Novo Familial Amyloid Polyneuropathy Retransplantation

“…In 1995, at the University Hospitals of Coimbra, Furtado proceeded to use FAP livers to perform LTx, known as domino or sequential liver transplantation (FAP livers are anatomically and functionally normal except for production of mutant TTR Met 30), thereby increasing the pool of donors and reducing the time spent on waiting lists [6,15,16]. It was expected that the time it took to develop FAP in nongenetically predetermined people would be equal to or slower than the time it took to develop the genetic disease [6,15,16].…”

“…It was expected that the time it took to develop FAP in nongenetically predetermined people would be equal to or slower than the time it took to develop the genetic disease [6,15,16]. About 7 to 9 years after LTx, some domino FAP liver recipients developed symptoms, including progressive peripheral sensory and motor neuropathy [10,14,17].…”

“…The disease progresses with ongoing deposition of amyloid protein, resulting in sensory and motor changes of the extremities, alternating diarrhea and constipation, malabsorption, hypoglycemic crises, orthostatic hypotension, changes in cardiac conductivity, neurogenic bladder, erectile dysfunction, restrictive cardiomyopathy, renal failure, and vitreous opacity [1,10,13]. The initial symptoms usually appear between the ages of 20 and 35, with slow and progressive evolution until death [1,6,10,13,14]. To date, the only treatment is liver transplantation (LTx), which was first performed in 1990 [7,10].…”

Die Domino-Transplantation von Organen

“…In der Literatur sind vereinzelte Fälle der Übertragung der Krankheit des Domino-Spenders auf den Empfän-ger des Organs bereits binnen zehn Jahren nachgewiesen 40 ; in Deutschland sind zwei solcher Fälle bekannt 41 …”

Transthyretin-Derived (ATTR) Amyloidotic Cardiomyopathy After Receiving a Domino Liver Allograft

“…The explanted liver is often used for patients with liver insufficiency (domino transplantation). 3 Amyloidosis after receiving a liver allograft donated by a patient with ATTR amyloidosis is an established, albeit relatively uncommon phenomenon. 3 However, even if it does occur, it usually takes many years for overt amyloidosis to develop in the recipient, reflecting the slow development of amyloidosis in donor patients with a TTR mutation, which legitimizes the use of these livers for transplantation.…”