Long-term outcomes and trends in liver transplantation for hereditary hemochromatosis in the United States
There 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...
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Published in | Liver transplantation Vol. 29; no. 1; pp. 15 - 25 |
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Main Authors | , , , , , , , , , , , |
Format | Journal Article |
Language | English |
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United States
01.01.2023
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Abstract | There 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. |
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AbstractList | There 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. There 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. |
Author | Goss, John A Kowdley, Kris V Jalal, Prasun Rana, Abbas Prakash, Sameer Shaikh, Anjiya Lymberopoulos, Peter Kanwal, Fasiha George Cholankeril Bhatnagar, Anshul Allam, Anthony K Goli, Karthik |
AuthorAffiliation | 1 Department of Medicine, State University of New York (SUNY) Downstate, Health Sciences University, Brooklyn, New York, USA 4 School of Medicine, Baylor College of Medicine, Houston, Texas, USA 7 Center for Innovations in Quality, Effectiveness and Safety, Michael E. DeBakey Veterans Affairs Medical Center, Houston, Texas, USA 2 Department of Internal Medicine, University of Iowa Hospitals & Clinics, Iowa City, Iowa, USA 3 Department of Medicine, University of Connecticut School of Medicine, Farmington, Connecticut, USA 5 Hepatology Program, Division of Abdominal Transplantation, Michael E. DeBakey Department of General Surgery, Baylor College of Medicine, Houston, Texas, USA 6 Section of Gastroenterology and Hepatology, Department of Medicine, Baylor College of Medicine, Houston, Texas, USA 9 Elson S. Floyd College of Medicine Washington State University, Seattle, Washington, USA 8 Liver Institute Northwest, Seattle, Washington, USA |
AuthorAffiliation_xml | – name: 8 Liver Institute Northwest, Seattle, Washington, USA – name: 9 Elson S. Floyd College of Medicine Washington State University, Seattle, Washington, USA – name: 5 Hepatology Program, Division of Abdominal Transplantation, Michael E. DeBakey Department of General Surgery, Baylor College of Medicine, Houston, Texas, USA – name: 2 Department of Internal Medicine, University of Iowa Hospitals & Clinics, Iowa City, Iowa, USA – name: 6 Section of Gastroenterology and Hepatology, Department of Medicine, Baylor College of Medicine, Houston, Texas, USA – name: 7 Center for Innovations in Quality, Effectiveness and Safety, Michael E. DeBakey Veterans Affairs Medical Center, Houston, Texas, USA – name: 3 Department of Medicine, University of Connecticut School of Medicine, Farmington, Connecticut, USA – name: 1 Department of Medicine, State University of New York (SUNY) Downstate, Health Sciences University, Brooklyn, New York, USA – name: 4 School of Medicine, Baylor College of Medicine, Houston, Texas, USA |
Author_xml | – sequence: 1 givenname: Peter orcidid: 0000-0002-4657-1349 surname: Lymberopoulos fullname: Lymberopoulos, Peter organization: Department of Medicine , State University of New York (SUNY) Downstate, Health Sciences University , Brooklyn , New York , USA – sequence: 2 givenname: Sameer orcidid: 0000-0002-4400-4666 surname: Prakash fullname: Prakash, Sameer organization: Department of Internal Medicine , University of Iowa Hospitals & Clinics , Iowa City , Iowa , USA – sequence: 3 givenname: Anjiya surname: Shaikh fullname: Shaikh, Anjiya organization: Department of Medicine , University of Connecticut School of Medicine , Farmington , Connecticut , USA – sequence: 4 givenname: Anshul surname: Bhatnagar fullname: Bhatnagar, Anshul organization: School of Medicine , Baylor College of Medicine , Houston , Texas , USA – sequence: 5 givenname: Anthony K surname: Allam fullname: Allam, Anthony K organization: School of Medicine , Baylor College of Medicine , Houston , Texas , USA – sequence: 6 givenname: Karthik surname: Goli fullname: Goli, Karthik organization: School of Medicine , Baylor College of Medicine , Houston , Texas , USA – sequence: 7 givenname: John A surname: Goss fullname: Goss, John A organization: Hepatology Program, Division of Abdominal Transplantation, Michael E. DeBakey Department of General Surgery , Baylor College of Medicine , Houston , Texas , USA – sequence: 8 givenname: Fasiha orcidid: 0000-0001-6715-3966 surname: Kanwal fullname: Kanwal, Fasiha organization: Center for Innovations in Quality, Effectiveness and Safety , Michael E. DeBakey Veterans Affairs Medical Center , Houston , Texas , USA – sequence: 9 givenname: Abbas surname: Rana fullname: Rana, Abbas organization: Hepatology Program, Division of Abdominal Transplantation, Michael E. DeBakey Department of General Surgery , Baylor College of Medicine , Houston , Texas , USA – sequence: 10 givenname: Kris V orcidid: 0000-0002-8553-3652 surname: Kowdley fullname: Kowdley, Kris V organization: Elson S. Floyd College of Medicine Washington State University , Seattle , Washington , USA – sequence: 11 givenname: Prasun surname: Jalal fullname: Jalal, Prasun organization: Section of Gastroenterology and Hepatology, Department of Medicine , Baylor College of Medicine , Houston , Texas , USA – sequence: 12 surname: George Cholankeril fullname: George Cholankeril organization: Section of Gastroenterology and Hepatology, Department of Medicine , Baylor College of Medicine , Houston , Texas , USA |
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SubjectTerms | Adult Hemochromatosis - etiology Hemochromatosis - surgery Humans Liver Diseases - etiology Liver Diseases - surgery Liver Transplantation - adverse effects Proportional Hazards Models Retrospective Studies United States - epidemiology |
Title | Long-term outcomes and trends in liver transplantation for hereditary hemochromatosis in the United States |
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