P12 Reviewing the largest experience of liver transplantation for Wilson’s disease in the UK: single centre review of patient and graft outcomes and survival

<h3>Introduction</h3> Wilson’s disease (WD) is a genetic metabolic liver condition caused by mutation of the ATP7B transporter leading to accumulation of copper particularly in brain and hepatic tissue which can result in liver failure. Transplantation is often necessary in those who present in acute liver failure or decompensation of chronic disease. King’s College Hospital, London (KCH) has the largest cohort of WD patients in the UK. We aim to review the outcomes from liver transplantation performed at KCH for WD patients. <h3>Method</h3> We reviewed clinical, genetic and biochemical data for all patients transplanted for WD at KCH from 1990 to 2023. Data was collected from current electronic records. Statistical analysis was performed using Microsoft excel and R studio <h3>Results</h3> There were 66 patients transplanted for WD since 1990 (38F:28M). Age range was from 7–56 years(yr). Listing for transplant was 42 (63%) as super urgent (SU), 16 (25%) as chronic and 12% with unavailable data. There were 62 DBD grafts, 3 DCD (listed for chronic) and 1 living un-related donor. 5% had graft loss within 90 days and overall 17% required multiple transplants (2Tx: 10 patients, 3Tx:1 patient). Overall graft survival was 94% at 1 year, 86% at 5 years and 82% at 10 years. Kaplan Meier survival curve with chi2 analysis was performed with overall survival (OS) and graft survival (GS) with age group, age, gender, UKELD, blood group, urgency and 30 day infection post transplant as variables. In OS, only age reached statistical significance (p=0.03) with gender and infection almost reaching significance (p=0.06). Multiple linear regression analysis was performed for OS and death, GS and graft loss with gender, UKELD, blood group, urgency, age and infection as factors. In GS, infection reached statistical significance (p=0.03) and UKELD was reaching significance (p=0.058). <h3>Conclusion</h3> This is the largest single centre experience of liver transplantation for WD in the UK. Overall adult survival after transplant was comparable to the national level at 1 yr both overall and for SU, but was significantly higher locally at 5 yr for both categories. Overall paediatric survival was lower than the national level at 1yr but significantly higher for SU, while at 5yr was lower for both categories. This may be explained by the low number of patients and the timing of data used to calculate the national level (CI at 5yr is 74–97%). Overall liver transplant remains an excellent long term curative treatment for WD.

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Published
2024-10-01
DOI
https://doi.org/10.1136/gutjnl-2024-basl.23
Primary Topic
Trace Elements in Health
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article
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article

P12 Reviewing the largest experience of liver transplantation for Wilson’s disease in the UK: single centre review of patient and graft outcomes and survival

Krishna Menon, Deepak Joshi, Rosa de Miguel Buckley, Michael A. Heneghan et al.
Trace Elements in Health
article

P12 Reviewing the largest experience of liver transplantation for Wilson’s disease in the UK: single centre review of patient and graft outcomes and survival

Krishna Menon, Deepak Joshi, Rosa de Miguel Buckley, Michael A. Heneghan, Abid Suddle, Anil Kumar Dhawan, Aftab Ala, Marianne Samyn, Varuna Aluvihare, Alberto Sánchez‐Fueyo, Claire Kelly, James Liu Yin, Niloufar Safinia, Kosh Agarwal, Adrian Bomford, Nigel Heaton
article en

Abstract

<h3>Introduction</h3> Wilson’s disease (WD) is a genetic metabolic liver condition caused by mutation of the ATP7B transporter leading to accumulation of copper particularly in brain and hepatic tissue which can result in liver failure. Transplantation is often necessary in those who present in acute liver failure or decompensation of chronic disease. King’s College Hospital, London (KCH) has the largest cohort of WD patients in the UK. We aim to review the outcomes from liver transplantation performed at KCH for WD patients. <h3>Method</h3> We reviewed clinical, genetic and biochemical data for all patients transplanted for WD at KCH from 1990 to 2023. Data was collected from current electronic records. Statistical analysis was performed using Microsoft excel and R studio <h3>Results</h3> There were 66 patients transplanted for WD since 1990 (38F:28M). Age range was from 7–56 years(yr). Listing for transplant was 42 (63%) as super urgent (SU), 16 (25%) as chronic and 12% with unavailable data. There were 62 DBD grafts, 3 DCD (listed for chronic) and 1 living un-related donor. 5% had graft loss within 90 days and overall 17% required multiple transplants (2Tx: 10 patients, 3Tx:1 patient). Overall graft survival was 94% at 1 year, 86% at 5 years and 82% at 10 years. Kaplan Meier survival curve with chi2 analysis was performed with overall survival (OS) and graft survival (GS) with age group, age, gender, UKELD, blood group, urgency and 30 day infection post transplant as variables. In OS, only age reached statistical significance (p=0.03) with gender and infection almost reaching significance (p=0.06). Multiple linear regression analysis was performed for OS and death, GS and graft loss with gender, UKELD, blood group, urgency, age and infection as factors. In GS, infection reached statistical significance (p=0.03) and UKELD was reaching significance (p=0.058). <h3>Conclusion</h3> This is the largest single centre experience of liver transplantation for WD in the UK. Overall adult survival after transplant was comparable to the national level at 1 yr both overall and for SU, but was significantly higher locally at 5 yr for both categories. Overall paediatric survival was lower than the national level at 1yr but significantly higher for SU, while at 5yr was lower for both categories. This may be explained by the low number of patients and the timing of data used to calculate the national level (CI at 5yr is 74–97%). Overall liver transplant remains an excellent long term curative treatment for WD.

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Trace Elements in Health
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