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Diagnostic Dilemma and Treatment Outcome in Acute Liver Failure Due to Wilson’s Disease

BACKGROUND: Wilson’s disease (WD) manifesting as acute liver failure (ALF) is a life-threatening condition, and spontaneous recovery is rare. Diagnostic scores like the alkaline phosphatase elevation/total bilirubin elevation ratio and aspartate aminotransferase/alanine aminotransferase ratio can di...

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Autores principales: Stankiewicz, Rafał, Patkowski, Waldemar, Zieniewicz, Krzysztof
Formato: Online Artículo Texto
Lenguaje:English
Publicado: International Scientific Literature, Inc. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8140524/
https://www.ncbi.nlm.nih.gov/pubmed/34001844
http://dx.doi.org/10.12659/AOT.930146
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author Stankiewicz, Rafał
Patkowski, Waldemar
Zieniewicz, Krzysztof
author_facet Stankiewicz, Rafał
Patkowski, Waldemar
Zieniewicz, Krzysztof
author_sort Stankiewicz, Rafał
collection PubMed
description BACKGROUND: Wilson’s disease (WD) manifesting as acute liver failure (ALF) is a life-threatening condition, and spontaneous recovery is rare. Diagnostic scores like the alkaline phosphatase elevation/total bilirubin elevation ratio and aspartate aminotransferase/alanine aminotransferase ratio can distinguish WD from other ALF etiologies. Liver transplantation plays a major role in treating these patients, and the revised Wilson Index is useful in patient selection for this procedure. The aim of this study was to evaluate diagnostic scores, treatments, and outcomes of a large cohort of patients with WD-ALF. MATERIAL/METHODS: Twenty adult patients of a historical cohort admitted from January 2001 to December 2017 were prospectively observed. Demographic, clinical, laboratory, and radiology data, and treatment, time on the waiting list for liver transplantation, and outcomes were recorded. RESULTS: No diagnostic laboratory scores were 100% positive in patients with WD-ALF. Cut-off values for the alkaline phosphatase/total bilirubin ratio and aspartate aminotransferase/alanine aminotransferase ratio were met by 65.0% and 80.0% of patients, respectively. All patients met at least 1 criterion for high risk of death (Nazer or revised Wilson Index) and qualified for liver transplantation. In 9 patients, albumin dialysis was used before surgery. Survival after liver transplantation was 85.0% and 74.4% after 1 month and 1 year, respectively. CONCLUSIONS: Further research on a novel diagnostic score in WD-ALF is warranted. Adult patients suspected to have WD as the cause of ALF should be treated in the referral liver transplantation unit. Liver transplantation makes long-term survival possible for patients with this critical illness.
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spelling pubmed-81405242021-05-25 Diagnostic Dilemma and Treatment Outcome in Acute Liver Failure Due to Wilson’s Disease Stankiewicz, Rafał Patkowski, Waldemar Zieniewicz, Krzysztof Ann Transplant Original Paper BACKGROUND: Wilson’s disease (WD) manifesting as acute liver failure (ALF) is a life-threatening condition, and spontaneous recovery is rare. Diagnostic scores like the alkaline phosphatase elevation/total bilirubin elevation ratio and aspartate aminotransferase/alanine aminotransferase ratio can distinguish WD from other ALF etiologies. Liver transplantation plays a major role in treating these patients, and the revised Wilson Index is useful in patient selection for this procedure. The aim of this study was to evaluate diagnostic scores, treatments, and outcomes of a large cohort of patients with WD-ALF. MATERIAL/METHODS: Twenty adult patients of a historical cohort admitted from January 2001 to December 2017 were prospectively observed. Demographic, clinical, laboratory, and radiology data, and treatment, time on the waiting list for liver transplantation, and outcomes were recorded. RESULTS: No diagnostic laboratory scores were 100% positive in patients with WD-ALF. Cut-off values for the alkaline phosphatase/total bilirubin ratio and aspartate aminotransferase/alanine aminotransferase ratio were met by 65.0% and 80.0% of patients, respectively. All patients met at least 1 criterion for high risk of death (Nazer or revised Wilson Index) and qualified for liver transplantation. In 9 patients, albumin dialysis was used before surgery. Survival after liver transplantation was 85.0% and 74.4% after 1 month and 1 year, respectively. CONCLUSIONS: Further research on a novel diagnostic score in WD-ALF is warranted. Adult patients suspected to have WD as the cause of ALF should be treated in the referral liver transplantation unit. Liver transplantation makes long-term survival possible for patients with this critical illness. International Scientific Literature, Inc. 2021-05-18 /pmc/articles/PMC8140524/ /pubmed/34001844 http://dx.doi.org/10.12659/AOT.930146 Text en © Ann Transplant, 2021 https://creativecommons.org/licenses/by-nc-nd/4.0/This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/) )
spellingShingle Original Paper
Stankiewicz, Rafał
Patkowski, Waldemar
Zieniewicz, Krzysztof
Diagnostic Dilemma and Treatment Outcome in Acute Liver Failure Due to Wilson’s Disease
title Diagnostic Dilemma and Treatment Outcome in Acute Liver Failure Due to Wilson’s Disease
title_full Diagnostic Dilemma and Treatment Outcome in Acute Liver Failure Due to Wilson’s Disease
title_fullStr Diagnostic Dilemma and Treatment Outcome in Acute Liver Failure Due to Wilson’s Disease
title_full_unstemmed Diagnostic Dilemma and Treatment Outcome in Acute Liver Failure Due to Wilson’s Disease
title_short Diagnostic Dilemma and Treatment Outcome in Acute Liver Failure Due to Wilson’s Disease
title_sort diagnostic dilemma and treatment outcome in acute liver failure due to wilson’s disease
topic Original Paper
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8140524/
https://www.ncbi.nlm.nih.gov/pubmed/34001844
http://dx.doi.org/10.12659/AOT.930146
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