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3篇 您的检索式:作者名="Maxime Foguenne"
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1Symptomatic Val122del mutated hereditary transthyretin amyloidosis: Need for early diagnosis and prioritization for heart and liver transplantation显示文摘Background: Hereditary transthyretin(ATTRv) amyloidosis is an autosomal dominant disease linked to transthyretin gene mutations which cause instability of the transthyretin tetramer. After dissociation and misfolding they reassemble as insoluble fibrils(i.e. amyloid). Apart from the common Val30 Met mutation there is a very heterogeneous group of non-Val30 Met mutations. In some cases, the clinical picture is dominated by a rapidly evolving restrictive and hypertrophic cardiomyopathy. Methods: A case series of four liver recipients with the highly clinically relevant, rare and particularly aggressive Val122 del mutation is presented. Medical and surgical therapeutic options, waiting list policy for ATTRv-amyloidosis, including the need for heart transplantation, and status of heart-liver transplantation are discussed. Results: Three patients needed a staged(1 patient) or simultaneous(2 patients) heart-liver transplant due to rapidly progressing cardiac failure and/or neurologic disability. Domino liver transplantation was impossible in two due to fibrotic hepatic transformation caused by cardiomyopathy. After a follow-up ranging from 3.5 to 9.5 years, cardiac(allograft) function was maintained in all patients, but neuropathy progressed in three patients, one of whom died after 80 months. Conclusions: This is the first report in(liver) transplant literature about the rare Val122 del ATTRv mutation. Due to its aggressiveness, symptomatic patients should be prioritized on the liver and, in cases with cardiomyopathy, heart waiting lists in order to avoid the irreversible neurological and cardiac damage that leads to a rapid lethal outcome.Adriano-Valerio Schettini Laura Llado JulieK Heimbach JoseGonzalez Costello Marie Tranäng Olivier Van Caenegem Richard C Daly Peter Van den Bergh Carlos Casasnovas Joan Fabregat John J Poterucha Maxime Foguenne Bo Göran Ericzon Jan Lerut 2021Hepatobiliary & Pancreatic Diseases International2021,20,4:1
2Total hepatectomy and modified temporary porto-caval shunt as a useful bridge to urgent retransplantation显示文摘To the Editor:The original technique of liver transplantation(LT)described by Starzl in 1963 included the removal of the recipient inferior vena cava(IVC)with use of extracorporeal veno-venous bypass(VVB).Since then,the majority of transplant centres evolved towards an IVC sparing hepatectomy with or without temporary porto-caval shunting(PCSh)[1].In 1988,the Hannover group reported about a two-stage total hepatectomy technique with temporary PCSh and subsequent liver re-transplantation(re-LT)in order to overcome the consequences of the“toxic liver allograft syndrome”[2].Samuele Iesari Maxime Foguenne Jan Lerut 2018Hepatobiliary & Pancreatic Diseases International2018,17,4:0
3Hepatocellular carcinoma recurrence after acute liver allograft rejection treatment: A multicenter European experience显示文摘Background:During the last decades,several risk factors for the recurrence of hepatocellular carcinoma(HCC)after liver transplantation(LT)have been investigated.However,the impact of two important drivers of oncogenesis,namely the immunosuppression and the treatment of acute cellular rejection(ACR)have been marginally addressed.This study aimed at investigating the impact of ACR treatment on the incidence of tumor recurrence in a large European HCC-LT population.Methods:Seven hundred and eighty-one adult patients transplanted between February 1,1985 and June 30,2016 were retrospectively analyzed.After propensity score match,116 patients treated for ACR using steroid boluses were compared with 115 patients who did not present any ACR or a histologic but clinical irrelevant ACR.Results:Steroid boluses treated patients had a 18-fold higher overall incidence of HCC recurrence than those non-treated patients(16.4%vs.0.9%;P<0.0001).At multivariate Cox regression analysis,steroid boluses used to treat ACR were an independent risk factor for HCC recurrence(HR=14.2;95%CI:1.8–110.4;P=0.010).Conclusions:The decision to treat ACR as well as to reinforce immunosuppression load should be cautiously taken in view of the presented results.Prospective studies are needed to further elucidate the clinical impact of immunosuppression on HCC recurrence after transplantation.Quirino Lai Samuele Iesari Armin Finkenstedt Maria Hoppe-Lotichius Maxime Foguenne Konrad Lehner Gerd Otto Jan Lerut 2019Hepatobiliary & Pancreatic Diseases International2019,18,6:0
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