scholarly journals Fulminant Wilson’s Disease Managed with Plasmapheresis as a Bridge to Liver Transplant

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Talal Hilal ◽  
R. Scott Morehead
Keyword(s):  
Liver Failure ◽  
Liver Transplant ◽  
Wilson’S Disease ◽  
Acne Vulgaris ◽  
Wilson's Disease ◽  
Serum Copper ◽  
Adjunctive Treatment ◽  
Fulminant Liver Failure ◽  
Parenchymal Liver ◽  
Pathologic Processes

New-onset jaundice can be a manifestation of multiple pathologic processes including hemolysis, parenchymal liver disease, and cholestasis; the differential diagnosis is broad and requires a systematic approach. We report a case of a patient who presented with jaundice after starting minocycline for the treatment of acne vulgaris and rapidly developed fulminant liver failure found to be due to Wilson’s disease. She also manifested severe Coomb’s negative hemolytic anemia and renal failure secondary to hepatorenal syndrome. As a bridge to liver transplant, she was successfully treated with plasmapheresis to decrease serum copper in addition to hemodialysis for acidosis and hyperkalemia. She was able to receive a liver and made a full recovery. The case highlights the use of plasmapheresis as an adjunctive treatment modality in cases of fulminant liver failure due to Wilson’s disease.

scholarly journals Failure of simple biochemical indexes to reliably differentiate fulminant wilson's disease from other causes of fulminant liver failure

Hepatology ◽  
1992 ◽  
Vol 16 (5) ◽  
pp. 1206-1211 ◽  
Author(s):  
Richard Sallie ◽  
Leah Katsiyiannakis ◽  
Dianne Baldwin ◽  
Sue Davies ◽  
John O'Grady ◽  
...  
Keyword(s):  
Liver Failure ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Fulminant Liver Failure

Predictive Factors for Survival in Children Receiving Liver Transplants for Wilson’s Disease: A Cohort Study Using European Liver Transplant Registry Data

2018 ◽  
Vol 24 (9) ◽  
pp. 1186-1198 ◽  
Author(s):  
Eva-Doreen Pfister ◽  
André Karch ◽  
René Adam ◽  
Wojciech G. Polak ◽  
Vincent Karam ◽  
...  
Keyword(s):  
Cohort Study ◽  
Liver Transplant ◽  
Predictive Factors ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Registry Data ◽  
Liver Transplants ◽  
Transplant Registry

scholarly journals Acute Liver Failure Due to Wilson's Disease

2020 ◽  
Author(s):  
M. Gillespie ◽  
R. Tadros ◽  
T. Haddad ◽  
J. Lyou ◽  
J. Hanje ◽  
...  
Keyword(s):  
Liver Failure ◽  
Acute Liver Failure ◽  
Wilson’S Disease ◽  
Wilson's Disease

Expression of the Caeruloplasmin Gene in the Adult and Neonatal Rat Liver

1989 ◽  
Vol 77 (3) ◽  
pp. 259-263 ◽  
Author(s):  
L. Barrow ◽  
M. S. Tanner ◽  
D. R. Critchley
Keyword(s):  
Steady State ◽  
Rat Liver ◽  
Messenger Rna ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Serum Copper ◽  
Neonatal Rat ◽  
Neonatal Rats ◽  
Significant Difference ◽  
Steady State Levels

1. It has been suggested that low levels of serum caeruloplasmin in Wilson's disease result from the failure to switch from a fetal to an adult mode of caeruloplasmin gene expression. To investigate postnatal expression of the caeruloplasmin gene, steady-state levels of caeruloplasmin messenger RNA in adult and neonatal rat liver were measured. 2. Copper parameters observed in neonatal rats were similar to those seen in Wilson's disease: hepatic copper concentration was significantly elevated (neonatal 164 ± 35 μg/g, adults 50 ± 8 μg/g, P < .001) and serum copper and caeruloplasmin levels were low (neonatal 0.5 ± 0.1 μg/ml, adults 1.3 ± 0.2 μg/ml, P < .001; neonatal 0.20 ± 0.04 arbitrary units, adults 0.69 ± 0.16 arbitrary units, P < .001), respectively. 3. Caeruloplasmin messenger RNA levels were analysed by Northern and dot blotting using a 12P-labelled caeruloplasmin complementary DNA probe. A caeruloplasmin messenger RNA of approximately 4.4 kilobases was detected in both adult and neonatal rat liver, with no significant difference observed in steady-state levels. 4. A step subsequent to caeruloplasmin gene transcription must therefore be impaired in neonatal rats.

d-Penicillamine in Wilson's disease presenting as acute liver failure with hemolysis

1982 ◽  
Vol 27 (12) ◽  
pp. 1126-1129 ◽  
Author(s):  
W. Vielhauer ◽  
V. Eckardt ◽  
K. H. Holterm�ller ◽  
J. B. L�th ◽  
B. Schulte ◽  
...  
Keyword(s):  
Liver Failure ◽  
Acute Liver Failure ◽  
Wilson’S Disease ◽  
Wilson's Disease

scholarly journals Májátültetés Wilson-kóros betegekben, 1996–2017

2019 ◽  
Vol 160 (51) ◽  
pp. 2021-2025
Author(s):  
Dániel Németh ◽  
Anikó Folhoffer ◽  
Szilvia Bianka László ◽  
László Kóbori ◽  
Dénes Görög ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
Wilson’S Disease ◽  
Copper Metabolism ◽  
Wilson's Disease ◽  
Chronic Liver ◽  
Diagnostic Process ◽  
Decompensated Liver Cirrhosis ◽  
The Mean

Abstract: Introduction: Wilson’s disease is a lethal-without-treatment inherited disorder of copper metabolism. Despite the increased focus on the diagnosis and treatment, liver transplantation is needed in a number of cases even nowadays. Aim: To collect and analyze the data of the Hungarian Wilson’s disease patients who underwent liver transplantation. Method: Data of 24 Wilson’s disease patients who underwent liver transplantation at the Semmelweis University have been analyzed retrospectively. The diagnosis of Wilson’s disease was based on the international score system. The diagnosis of acute liver failure corresponded to the King’s College criteria. All liver transplantations had been performed at the Department of Transplantation and Surgery of Semmelweis University, in 1996 for the first time. Results: The mean age was 26 years, F/M = 13/11. Twelve patients needed urgent liver transplantation for acute liver failure, and 12 underwent transplantation for decompensated liver cirrhosis. One patient had been retransplanted because of chronic rejection. Three patients with acute on chronic liver failure were transplanted via the Eurotransplant program. The mean time on the waiting list was 3 vs 320 days in acute liver failure and chronic liver disease groups, respectively. The overall 5-year survival was 66%, but it was 80% after 2002 indicating both the learning curve effect and the improvement of vigilance in Hungary. Despite difficulties of the diagnostic process, Wilson’s disease was identified in 21/24 patients prior to the transplantation. Conclusion: Liver transplantation is needed in a number of cases of Wilson’s disease. The ideal indication and timing of transplantation may improve the survival of the patients. Orv Hetil. 2019; 160(51): 2021–2025.

WILSON'S DISEASE AND LIVER FAILURE IN CHILDHOOD

2009 ◽  
Vol 59 ◽  
pp. 236-239
Author(s):  
E. Sagen ◽  
O. Lange ◽  
G. Westgaard ◽  
J. Bland ◽  
I. Romslo
Keyword(s):  
Liver Failure ◽  
Wilson’S Disease ◽  
Wilson's Disease
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