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Model for End‐Stage Liver Disease/Pediatric End‐Stage Liver Disease exception policy and outcomes in pediatric patients with hepatopulmonary syndrome requiring liver transplantation

Hepatopulmonary syndrome (HPS) is associated with increased waitlist mortality in liver transplantation (LT) candidates. Children with HPS are granted Model for End‐Stage Liver Disease (MELD)/Pediatric End‐Stage Liver Disease (PELD) exception points for waitlist prioritization in the United States b...

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Autores principales: Raza, Muhammad H., Kwon, Yong, Kobierski, Pierre, Misra, Asish C., Lim, Angelina, Goldbeck, Cameron, Etesami, Kambiz, Kohli, Rohit, Emamaullee, Juliet
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9868062/
https://www.ncbi.nlm.nih.gov/pubmed/35876731
http://dx.doi.org/10.1002/lt.26548
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author Raza, Muhammad H.
Kwon, Yong
Kobierski, Pierre
Misra, Asish C.
Lim, Angelina
Goldbeck, Cameron
Etesami, Kambiz
Kohli, Rohit
Emamaullee, Juliet
author_facet Raza, Muhammad H.
Kwon, Yong
Kobierski, Pierre
Misra, Asish C.
Lim, Angelina
Goldbeck, Cameron
Etesami, Kambiz
Kohli, Rohit
Emamaullee, Juliet
author_sort Raza, Muhammad H.
collection PubMed
description Hepatopulmonary syndrome (HPS) is associated with increased waitlist mortality in liver transplantation (LT) candidates. Children with HPS are granted Model for End‐Stage Liver Disease (MELD)/Pediatric End‐Stage Liver Disease (PELD) exception points for waitlist prioritization in the United States based on criterion developed for adults. In this study, the impact of this MELD/PELD exception policy on post‐LT survival in children was examined. A retrospective cohort of patients aged younger than 18 years with a MELD/PELD exception request who underwent LT between 2007 and 2018 were identified in the Scientific Registry of Transplant Recipients. Patients were stratified by waitlist partial pressure of arterial oxygen (PaO(2)) to assess risk factors for waitlist mortality and post‐LT survival. Among 3082 pediatric LT recipients included in the study, 124 patients (4%) received MELD/PELD exception points for HPS. Patients with HPS were a median age of 9 years (interquartile range: 6, 12 years), 54.8% were girls, and 54% were White. Most patients (87.9%) were listed with laboratory MELD/PELD scores <15. Waitlist mortality for patients with HPS exception points was rare and not different from patients without HPS. When stratified by pre‐LT PaO(2), hypoxemia severity was not associated with differences in 1‐, 3‐, or 5‐year survival rates after LT (p = 0.13). However, patients with HPS showed a slightly lower survival rate at 5 years compared with patients without HPS (88.7% vs. 93.4%; p = 0.04). MELD/PELD exceptions for children with HPS mitigated waitlist mortality, and recipients with HPS experienced excellent 5‐year survival after LT, although slightly lower than in patients without HPS. Unlike adults with HPS, the severity of pre‐LT hypoxemia in children does not impact post‐LT survival. These data suggest that adult criteria for granting MELD/PELD exception points may not appropriately capture HPS severity in pediatric patients. Further prospective multicenter studies to examine the risk factors predicting negative survival outcomes in children with HPS are warranted.
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spelling pubmed-98680622023-01-27 Model for End‐Stage Liver Disease/Pediatric End‐Stage Liver Disease exception policy and outcomes in pediatric patients with hepatopulmonary syndrome requiring liver transplantation Raza, Muhammad H. Kwon, Yong Kobierski, Pierre Misra, Asish C. Lim, Angelina Goldbeck, Cameron Etesami, Kambiz Kohli, Rohit Emamaullee, Juliet Liver Transpl Original Articles: Liver Failure and Portal Hypertension Hepatopulmonary syndrome (HPS) is associated with increased waitlist mortality in liver transplantation (LT) candidates. Children with HPS are granted Model for End‐Stage Liver Disease (MELD)/Pediatric End‐Stage Liver Disease (PELD) exception points for waitlist prioritization in the United States based on criterion developed for adults. In this study, the impact of this MELD/PELD exception policy on post‐LT survival in children was examined. A retrospective cohort of patients aged younger than 18 years with a MELD/PELD exception request who underwent LT between 2007 and 2018 were identified in the Scientific Registry of Transplant Recipients. Patients were stratified by waitlist partial pressure of arterial oxygen (PaO(2)) to assess risk factors for waitlist mortality and post‐LT survival. Among 3082 pediatric LT recipients included in the study, 124 patients (4%) received MELD/PELD exception points for HPS. Patients with HPS were a median age of 9 years (interquartile range: 6, 12 years), 54.8% were girls, and 54% were White. Most patients (87.9%) were listed with laboratory MELD/PELD scores <15. Waitlist mortality for patients with HPS exception points was rare and not different from patients without HPS. When stratified by pre‐LT PaO(2), hypoxemia severity was not associated with differences in 1‐, 3‐, or 5‐year survival rates after LT (p = 0.13). However, patients with HPS showed a slightly lower survival rate at 5 years compared with patients without HPS (88.7% vs. 93.4%; p = 0.04). MELD/PELD exceptions for children with HPS mitigated waitlist mortality, and recipients with HPS experienced excellent 5‐year survival after LT, although slightly lower than in patients without HPS. Unlike adults with HPS, the severity of pre‐LT hypoxemia in children does not impact post‐LT survival. These data suggest that adult criteria for granting MELD/PELD exception points may not appropriately capture HPS severity in pediatric patients. Further prospective multicenter studies to examine the risk factors predicting negative survival outcomes in children with HPS are warranted. Lippincott Williams & Wilkins 2023-02 2023-01-17 /pmc/articles/PMC9868062/ /pubmed/35876731 http://dx.doi.org/10.1002/lt.26548 Text en Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by-nc/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (https://creativecommons.org/licenses/by-nc/4.0/) (CCBY-NC), where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/)
spellingShingle Original Articles: Liver Failure and Portal Hypertension
Raza, Muhammad H.
Kwon, Yong
Kobierski, Pierre
Misra, Asish C.
Lim, Angelina
Goldbeck, Cameron
Etesami, Kambiz
Kohli, Rohit
Emamaullee, Juliet
Model for End‐Stage Liver Disease/Pediatric End‐Stage Liver Disease exception policy and outcomes in pediatric patients with hepatopulmonary syndrome requiring liver transplantation
title Model for End‐Stage Liver Disease/Pediatric End‐Stage Liver Disease exception policy and outcomes in pediatric patients with hepatopulmonary syndrome requiring liver transplantation
title_full Model for End‐Stage Liver Disease/Pediatric End‐Stage Liver Disease exception policy and outcomes in pediatric patients with hepatopulmonary syndrome requiring liver transplantation
title_fullStr Model for End‐Stage Liver Disease/Pediatric End‐Stage Liver Disease exception policy and outcomes in pediatric patients with hepatopulmonary syndrome requiring liver transplantation
title_full_unstemmed Model for End‐Stage Liver Disease/Pediatric End‐Stage Liver Disease exception policy and outcomes in pediatric patients with hepatopulmonary syndrome requiring liver transplantation
title_short Model for End‐Stage Liver Disease/Pediatric End‐Stage Liver Disease exception policy and outcomes in pediatric patients with hepatopulmonary syndrome requiring liver transplantation
title_sort model for end‐stage liver disease/pediatric end‐stage liver disease exception policy and outcomes in pediatric patients with hepatopulmonary syndrome requiring liver transplantation
topic Original Articles: Liver Failure and Portal Hypertension
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9868062/
https://www.ncbi.nlm.nih.gov/pubmed/35876731
http://dx.doi.org/10.1002/lt.26548
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