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dc.contributor.authorLymberopoulos, Peter
dc.contributor.authorPrakash, Sameer
dc.contributor.authorShaikh, Anjiya
dc.contributor.authorBhatnagar, Anshul
dc.contributor.authorAllam, Anthony K
dc.contributor.authorGoli, Karthik
dc.contributor.authorGoss, John A
dc.contributor.authorKanwal, Fasiha
dc.contributor.authorRana, Abbas
dc.contributor.authorKowdley, Kris V
dc.contributor.authorJalal, Prasun
dc.contributor.authorGeorge Cholankeril
dc.date.accessioned2023-01-20T16:42:29Z
dc.date.available2023-01-20T16:42:29Z
dc.date.issued2022-07-21
dc.identifier.citationLymberopoulos P, Prakash S, Shaikh A, Bhatnagar A, Allam AK, Goli K, Goss JA, Kanwal F, Rana A, Kowdley KV, Jalal P, George Cholankeril. Long-term outcomes and trends in liver transplantation for hereditary hemochromatosis in the United States. Liver Transpl. 2023 Jan 1;29(1):15-25. doi: 10.1002/lt.26539. Epub 2022 Jul 21. PMID: 35770428; PMCID: PMC9800641.en_US
dc.identifier.eissn1527-6473
dc.identifier.doi10.1002/lt.26539
dc.identifier.pmid35770428
dc.identifier.urihttp://hdl.handle.net/20.500.12648/8110
dc.description.abstractThere have been conflicting data regarding liver transplantation (LT) outcomes for hereditary hemochromatosis (HH), with no recent data on LT outcomes in patients with HH in the past decade. Using the United Network for Organ Sharing registry, we evaluated waitlist and post-LT survival in all adult patients listed for HH without concomitant liver disease from 2003 to 2019. Post-LT survival for HH was compared with a propensity-matched (recipient and donor factors) cohort of recipients with chronic liver disease (CLD). From 2003 to 2019, 862 patients with HH were listed for LT, of which 55.6% ( n = 479) patients underwent LT. The 1- and 5-year post-LT survival rates in patients with HH were 88.7% (95% confidence interval [CI], 85.4%-91.4%) and 77.5% (95% CI, 72.8%-81.4%), respectively, and were comparable with those in the propensity-matched CLD cohort ( p value = 0.96). Post-LT survival for HH was lower than for Wilson's disease, another hereditary metabolic liver disease with similar LT volume ( n = 365). Predictors for long-term (5-year) post-LT mortality included presence of portal vein thrombosis (hazard ratio [HR], 1.96; 95% CI, 1.07-3.58), obesity measurements greater than Class II (HR, 1.98; 95% CI, 1.16-3.39), and Karnofsky performance status (HR, 0.98; 95% CI, 0.97-0.99) at the time of LT. The leading cause of post-LT death ( n = 145) was malignancy (25.5%), whereas cardiac disease was the cause in less than 10% of recipients. In conclusion, short- and long-term survival rates for HH are excellent and comparable with those of other LT recipients. Improving extrahepatic metabolic factors and functional status in patients with HH prior to LT may improve outcomes.
dc.language.isoenen_US
dc.relation.urlhttps://journals.lww.com/lt/Abstract/2023/01010/Long_term_outcomes_and_trends_in_liver.4.aspxen_US
dc.rightsCopyright © 2022 American Association for the Study of Liver Diseases.
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 International*
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/*
dc.titleLong-term outcomes and trends in liver transplantation for hereditary hemochromatosis in the United States.en_US
dc.typeArticle/Reviewen_US
dc.source.journaltitleLiver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Societyen_US
dc.source.volume29
dc.source.issue1
dc.source.beginpage15
dc.source.endpage25
dc.source.countryUnited States
dc.source.countryUnited States
dc.source.countryUnited States
dc.description.versionAMen_US
html.description.abstractThere have been conflicting data regarding liver transplantation (LT) outcomes for hereditary hemochromatosis (HH), with no recent data on LT outcomes in patients with HH in the past decade. Using the United Network for Organ Sharing registry, we evaluated waitlist and post-LT survival in all adult patients listed for HH without concomitant liver disease from 2003 to 2019. Post-LT survival for HH was compared with a propensity-matched (recipient and donor factors) cohort of recipients with chronic liver disease (CLD). From 2003 to 2019, 862 patients with HH were listed for LT, of which 55.6% ( n = 479) patients underwent LT. The 1- and 5-year post-LT survival rates in patients with HH were 88.7% (95% confidence interval [CI], 85.4%-91.4%) and 77.5% (95% CI, 72.8%-81.4%), respectively, and were comparable with those in the propensity-matched CLD cohort ( p value = 0.96). Post-LT survival for HH was lower than for Wilson's disease, another hereditary metabolic liver disease with similar LT volume ( n = 365). Predictors for long-term (5-year) post-LT mortality included presence of portal vein thrombosis (hazard ratio [HR], 1.96; 95% CI, 1.07-3.58), obesity measurements greater than Class II (HR, 1.98; 95% CI, 1.16-3.39), and Karnofsky performance status (HR, 0.98; 95% CI, 0.97-0.99) at the time of LT. The leading cause of post-LT death ( n = 145) was malignancy (25.5%), whereas cardiac disease was the cause in less than 10% of recipients. In conclusion, short- and long-term survival rates for HH are excellent and comparable with those of other LT recipients. Improving extrahepatic metabolic factors and functional status in patients with HH prior to LT may improve outcomes.
dc.description.institutionN/Aen_US
dc.description.degreelevelN/Aen_US
dc.identifier.journalLiver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society


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Copyright © 2022 American Association for the Study of Liver Diseases.
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