Management of Acute Wilsonian Hepatitis with Severe Hemolysis: A Successful Combination of Chelation and MARS Dialysis
Wilson’s disease is a rare hereditary disorder of copper metabolism leading to progressive accumulation of copper in several organs including the brain and the liver. Acute liver failure is a relatively rare hepatic manifestation of WD which may require urgent liver transplantation if medical treatm...
Saved in:
| Main Authors: | , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2021-01-01
|
| Series: | Case Reports in Hepatology |
| Online Access: | http://dx.doi.org/10.1155/2021/5583654 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832547660640288768 |
|---|---|
| author | Jeremy Hassoun Nathalie Hammer Giulia Magini Belen Ponte Marie Ongaro Anne-Laure Rougemont Nicolas Goossens Jean-Louis Frossard Laurent Spahr |
| author_facet | Jeremy Hassoun Nathalie Hammer Giulia Magini Belen Ponte Marie Ongaro Anne-Laure Rougemont Nicolas Goossens Jean-Louis Frossard Laurent Spahr |
| author_sort | Jeremy Hassoun |
| collection | DOAJ |
| description | Wilson’s disease is a rare hereditary disorder of copper metabolism leading to progressive accumulation of copper in several organs including the brain and the liver. Acute liver failure is a relatively rare hepatic manifestation of WD which may require urgent liver transplantation if medical treatment fails. We report here the case of a young woman who presented with classic acute Wilsonian hepatitis complicated by liver and renal failure and a severe hemolysis related to massive nonceruloplasmin bound copper accumulation requiring repeated blood transfusions. The early initiation of a combined treatment including conventional chelation therapy and repeated MARS dialysis sessions allowed a rapid control of hemolysis, a progressive decrease of free copper overload, and clinical recompensation without liver transplantation. |
| format | Article |
| id | doaj-art-a98d15b078304734ab7b309a2813dad0 |
| institution | Kabale University |
| issn | 2090-6587 2090-6595 |
| language | English |
| publishDate | 2021-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hepatology |
| spelling | doaj-art-a98d15b078304734ab7b309a2813dad02025-02-03T06:43:57ZengWileyCase Reports in Hepatology2090-65872090-65952021-01-01202110.1155/2021/55836545583654Management of Acute Wilsonian Hepatitis with Severe Hemolysis: A Successful Combination of Chelation and MARS DialysisJeremy Hassoun0Nathalie Hammer1Giulia Magini2Belen Ponte3Marie Ongaro4Anne-Laure Rougemont5Nicolas Goossens6Jean-Louis Frossard7Laurent Spahr8Gastroenterology and Hepatology, University Hospitals of Geneva and Faculty of Medicine, Geneva, SwitzerlandNephrology, University Hospitals of Geneva and Faculty of Medicine, Geneva, SwitzerlandGastroenterology and Hepatology, University Hospitals of Geneva and Faculty of Medicine, Geneva, SwitzerlandNephrology, University Hospitals of Geneva and Faculty of Medicine, Geneva, SwitzerlandGastroenterology and Hepatology, University Hospitals of Geneva and Faculty of Medicine, Geneva, SwitzerlandClinical Pathology, University Hospitals of Geneva and Faculty of Medicine, Geneva, SwitzerlandGastroenterology and Hepatology, University Hospitals of Geneva and Faculty of Medicine, Geneva, SwitzerlandGastroenterology and Hepatology, University Hospitals of Geneva and Faculty of Medicine, Geneva, SwitzerlandGastroenterology and Hepatology, University Hospitals of Geneva and Faculty of Medicine, Geneva, SwitzerlandWilson’s disease is a rare hereditary disorder of copper metabolism leading to progressive accumulation of copper in several organs including the brain and the liver. Acute liver failure is a relatively rare hepatic manifestation of WD which may require urgent liver transplantation if medical treatment fails. We report here the case of a young woman who presented with classic acute Wilsonian hepatitis complicated by liver and renal failure and a severe hemolysis related to massive nonceruloplasmin bound copper accumulation requiring repeated blood transfusions. The early initiation of a combined treatment including conventional chelation therapy and repeated MARS dialysis sessions allowed a rapid control of hemolysis, a progressive decrease of free copper overload, and clinical recompensation without liver transplantation.http://dx.doi.org/10.1155/2021/5583654 |
| spellingShingle | Jeremy Hassoun Nathalie Hammer Giulia Magini Belen Ponte Marie Ongaro Anne-Laure Rougemont Nicolas Goossens Jean-Louis Frossard Laurent Spahr Management of Acute Wilsonian Hepatitis with Severe Hemolysis: A Successful Combination of Chelation and MARS Dialysis Case Reports in Hepatology |
| title | Management of Acute Wilsonian Hepatitis with Severe Hemolysis: A Successful Combination of Chelation and MARS Dialysis |
| title_full | Management of Acute Wilsonian Hepatitis with Severe Hemolysis: A Successful Combination of Chelation and MARS Dialysis |
| title_fullStr | Management of Acute Wilsonian Hepatitis with Severe Hemolysis: A Successful Combination of Chelation and MARS Dialysis |
| title_full_unstemmed | Management of Acute Wilsonian Hepatitis with Severe Hemolysis: A Successful Combination of Chelation and MARS Dialysis |
| title_short | Management of Acute Wilsonian Hepatitis with Severe Hemolysis: A Successful Combination of Chelation and MARS Dialysis |
| title_sort | management of acute wilsonian hepatitis with severe hemolysis a successful combination of chelation and mars dialysis |
| url | http://dx.doi.org/10.1155/2021/5583654 |
| work_keys_str_mv | AT jeremyhassoun managementofacutewilsonianhepatitiswithseverehemolysisasuccessfulcombinationofchelationandmarsdialysis AT nathaliehammer managementofacutewilsonianhepatitiswithseverehemolysisasuccessfulcombinationofchelationandmarsdialysis AT giuliamagini managementofacutewilsonianhepatitiswithseverehemolysisasuccessfulcombinationofchelationandmarsdialysis AT belenponte managementofacutewilsonianhepatitiswithseverehemolysisasuccessfulcombinationofchelationandmarsdialysis AT marieongaro managementofacutewilsonianhepatitiswithseverehemolysisasuccessfulcombinationofchelationandmarsdialysis AT annelaurerougemont managementofacutewilsonianhepatitiswithseverehemolysisasuccessfulcombinationofchelationandmarsdialysis AT nicolasgoossens managementofacutewilsonianhepatitiswithseverehemolysisasuccessfulcombinationofchelationandmarsdialysis AT jeanlouisfrossard managementofacutewilsonianhepatitiswithseverehemolysisasuccessfulcombinationofchelationandmarsdialysis AT laurentspahr managementofacutewilsonianhepatitiswithseverehemolysisasuccessfulcombinationofchelationandmarsdialysis |