Emanuele Felli1, Pietro Addeo2, François Faitot2, Gennaro Nappo2, Constantin Oncioiu2, Philippe Bachellier2. 1. Hepato-Pancreato-Biliary Surgery and Liver Transplantation, Pôle des Pathologies Digestives, Hépatiques et de la Transplantation, Hôpital de Hautepierre-Hôpitaux Universitaires de Strasbourg, 67000 Strasbourg, France. Electronic address: emanuele.felli@chru-strasbourg.fr. 2. Hepato-Pancreato-Biliary Surgery and Liver Transplantation, Pôle des Pathologies Digestives, Hépatiques et de la Transplantation, Hôpital de Hautepierre-Hôpitaux Universitaires de Strasbourg, 67000 Strasbourg, France.
Abstract
AIM: To evaluate the indications, timing and results of liver transplantation in patients affected by hereditary hemorrhagic telangiectasia (HHT), by undertaking a systematic review of the current literature. METHODS: Electronic bibliographical databases were searched on MEDLINE and Pubmed according to the PRISMA criteria. A total of 58 articles were initially found, 11 have been excluded because of single center series later included in the European Liver transplant Registry (ELTR), already reported in this study. Thirty-eight articles have been excluded because they did not report specifically new cases of liver transplantation for hereditary hemorrhagic telangiectasia. Finally 9 articles were included in the analysis. RESULTS: A total of 56 patients who underwent liver transplantation for HHT are present in the English literature. One additional patient is presented in this article, for a total of 57 patients worldwide. To date, the most consistent published series is the one of the ELTR, including patients from 15 liver transplantation centers in the period 1985-2003 with a mean follow-up of 69 months. Ten-year patient and graft survival is 82.5% CONCLUSION: Liver transplantation should be considered as a radical but definitive treatment option in patients affected by HHT with liver or cardiac involvement not responsive to medical treatment.
AIM: To evaluate the indications, timing and results of liver transplantation in patients affected by hereditary hemorrhagic telangiectasia (HHT), by undertaking a systematic review of the current literature. METHODS: Electronic bibliographical databases were searched on MEDLINE and Pubmed according to the PRISMA criteria. A total of 58 articles were initially found, 11 have been excluded because of single center series later included in the European Liver transplant Registry (ELTR), already reported in this study. Thirty-eight articles have been excluded because they did not report specifically new cases of liver transplantation for hereditary hemorrhagic telangiectasia. Finally 9 articles were included in the analysis. RESULTS: A total of 56 patients who underwent liver transplantation for HHT are present in the English literature. One additional patient is presented in this article, for a total of 57 patients worldwide. To date, the most consistent published series is the one of the ELTR, including patients from 15 liver transplantation centers in the period 1985-2003 with a mean follow-up of 69 months. Ten-year patient and graft survival is 82.5% CONCLUSION: Liver transplantation should be considered as a radical but definitive treatment option in patients affected by HHT with liver or cardiac involvement not responsive to medical treatment.
Authors: J M Mora-Luján; A Iriarte; E Alba; M A Sánchez-Corral; P Cerdà; F Cruellas; Q Ordi; X Corbella; J Ribas; J Castellote; A Riera-Mestre Journal: Orphanet J Rare Dis Date: 2020-03-02 Impact factor: 4.123
Authors: Antoni Riera-Mestre; Pau Cerdà; Yoelimar Carolina Guzmán; Adriana Iriarte; Alba Torroella; José María Mora-Luján; Jose Castellote; Amelia Hessheimer; Constantino Fondevila; Laura Lladó Journal: J Clin Med Date: 2022-09-24 Impact factor: 4.964
Authors: Lilian B Olsen; Anette D Kjeldsen; Mikael K Poulsen; Jens Kjeldsen; Annette D Fialla Journal: Orphanet J Rare Dis Date: 2020-11-26 Impact factor: 4.123