| Autor: |
Marta Magaz, Heloïse Giudicelli-Lett, Oana Nicoară-Farcău, Neil Rajoriya, Ashish Goel, Karlien Raymenants, Sophie Hillaire, Gonzalo Crespo, Luis Téllez, Laure Elkrief, Constantino Fondevila, Lara Orts, Filipe Nery, Akash Shukla, Hélène Larrue, Yiliam Fundora, Helena Degroote, Victoria Aguilera, Elba LLop, Laura Turco, Federica Indulti, Stefania Gioia, Giulia Tosetti, Niccolò Bitto, Chiara Becchetti, Edilmar Alvarado, Cristina Roig, Raquel Diaz, Michael Praktiknjo, Anna-Lena Konicek, Guillem Soy, Pol Olivas, José Ignacio Fortea, Helena Masnou, Ángela Puente, Alba Ardèvol, Carmen Álvarez-Navascués, Marta Romero, Bernhard Scheiner, Georg Semmler, Mattias Mandorfer, Filipe Damião, Anna Baiges, Fanny Turon, Macarena Simón-Talero, Carlos González-Alayón, Alba Díaz, Ángeles García-Criado, Andrea de Gottardi, Enric Reverter, Annabel Blasi, Joan Genescà, Olivier Roux, Claire Francoz, Carlos Noronha Ferreira, Thomas Reiberger, Manuel Rodríguez, Rosa María Morillas, Javier Crespo, Jonel Trebicka, Rafael Bañares, Càndid Villanueva, Annalisa Berzigotti, Massimo Primignani, Vincenzo La Mura, Oliviero Riggio, Filippo Schepis, Bogdan Procopet, Xavier Verhelst, José Luis Calleja, Christophe Bureau, Agustín Albillos, Frederik Nevens, Virginia Hernández-Gea, Dhiraj Tripathi, Pierre-Emmanuel Rautou, François Durand, Juan Carlos García-Pagán |
| Rok vydání: |
2023 |
| Předmět: |
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| Zdroj: |
Transplantation. 107:1330-1340 |
| ISSN: |
0041-1337 |
| Popis: |
BACKGROUND AND AIMS Porto-sinusoidal vascular liver disorder (PSVD) is a rare disease that occasionally requires liver transplantation (LT), despite usually presenting preserved liver function. There remains a paucity of data pertaining to LT in PSVD. The aim was to identify features associated with post-LT outcomes in PSVD. METHODS Retrospective multicentre study of 79 patients who received LT for PSVD. RESULTS Median post-LT follow-up was 37 (range 1-261) mo. Refractory ascites 24 (30%), hepatic encephalopathy 16 (20%), and hepatopulmonary syndrome 13 (16.3%) were the most frequent indications for LT. Hepatocellular carcinoma was the indication in only 2 patients. Twenty-four patients died, 7 due to liver and 17 to non-liver related causes. Post-LT survival was 82.2%, 80.7%, and 68.6% at 1, 2, and 5 y, respectively. Post-LT survival was significantly better in patients without (n = 58) than in those with a persistent severe PSVD-associated condition (n = 21). Pre-LT hyperbilirubinemia levels and creatinine >100 µmol/L were also independently associated with poor survival. Six patients (7.6%) required a second LT. Recurrence of PSVD was confirmed by liver biopsy in only 1 patient and in 3 further patients it was likely. CONCLUSIONS LT in PSVD is associated with an acceptable outcome in the absence of associated severe conditions. However, persistence of a severe associated condition, pre-LT high bilirubin levels, or creatinine >100 µmol/L impact outcome, and these are features that should be considered when evaluating PSVD patients for LT. PSVD recurrence is possible after LT and needs to be explored, at least, in cases of posttransplant portal hypertension. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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