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 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

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.

scholarly journals Acute liver failure with hemolytic anemia in children with Wilson’s disease: Genotype-phenotype correlations?

2021 ◽  
Vol 13 (10) ◽  
pp. 1428-1438
Author(s):  
Tudor Lucian Pop ◽  
Alina Grama ◽  
Ana Cristina Stefanescu ◽  
Claudia Willheim ◽  
Peter Ferenci
Keyword(s):  
Liver Failure ◽  
Acute Liver Failure ◽  
Hemolytic Anemia ◽  
Wilson’S Disease ◽  
Wilson's Disease

Plasmapheresis Combined with Continuous Plasma Filtration Adsorption Rescues Severe Acute Liver Failure in Wilson’s Disease before Liver Transplantation

2018 ◽  
Vol 47 (1-3) ◽  
pp. 120-125 ◽  
Author(s):  
Yu Zhang ◽  
Liang Li ◽  
Xiaole Zhang ◽  
Wen Xu ◽  
Qun Guo ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
Critical Period ◽  
Wilson’S Disease ◽  
Clinical Symptoms ◽  
Wilson's Disease ◽  
Blood Purification ◽  
Plasma Filtration ◽  
Almost All

Wilsonian crisis (hemolytic crisis and acute liver failure [ALF] in Wilson’s disease) is fatal and almost all patients ultimately need a timely liver transplantation to save their lives. How to safely pass the critical period to liver transplantation is still a big challenge to doctors especially to pediatricians facing more difficult situations in blood purification. Here, we report about a 7-year-old child (weight 21 kg) presenting with severe hemolysis and impending ALF that made a rapid recovery with prompt initiation of plasmapheresis combined with continuous plasma filtration adsorption (CPFA) and chelation therapy. Rapidly efficient removal of copper, bilirubin, and albumin-binding toxins by hybrid blood purification alleviated hemolysis and liver injury and successfully bridged the patient over the critical period to late liver transplantation. Moreover, a review of the literature was performed examining the use of plasmapheresis, molecular adsorbent recirculation system, single-pass albumin dialysis, and continuous veno-venous hemodiafiltration in Wilson disease. The experience of our case points to that plasmapheresis combined with CPFA treatment can improve clinical symptoms and bridge children over through Wilsonian crisis to late live transplantation, and CPFA treatment is feasible and safe in children weighing more than 20 kg.

scholarly journals Insights into the management of Wilson’s disease

2017 ◽  
Vol 10 (11) ◽  
pp. 889-905 ◽  
Author(s):  
Mohmadshakil Kathawala ◽  
Gideon M. Hirschfield
Keyword(s):  
Liver Disease ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
Clinical Features ◽  
Wilson’S Disease ◽  
Early Recognition ◽  
Clinical Signs ◽  
Wilson's Disease ◽  
Biochemical Tests ◽  
Causative Gene

Wilson’s disease is a rare, inherited autosomal recessive disease of copper metabolism, in which the causative gene, ATP7B, results in absent or reduced function of the ATP7B transporter important for biliary excretion of copper and incorporation of copper into caeruloplasmin. Affected patients accumulate excessive copper within the liver, brain and other tissues. A disease mainly of children, adolescents and young adults; clinical features vary from the asymptomatic state to chronic liver disease, acute liver failure, and neuropsychiatric manifestations. Diagnosis requires a high index of suspicion and is based on a combination of clinical signs, biochemical tests, hepatic copper content assay and mutation analysis of the ATP7B gene; to date, there are more than 500 mutations of ATP7B in patients with Wilson’s disease. Early recognition and treatment can result in an excellent prognosis whereas untreated disease is almost always fatal. Drug therapies include chelating agents, such as penicillamine or trientine, and zinc salts. Liver transplantation is curative correcting the underlying pathophysiology and is traditionally indicated in acute liver failure or end-stage liver disease refractory to medical therapy. This review provides an overview of various aspects of Wilson’s disease including molecular basis of the disease, clinical features, diagnostic and management strategies with their current limitations.

scholarly journals Right lobe liver transplantation in patient with fulminant form of the Wilson’s disease from AB0-incompatible relative donor

2017 ◽  
Vol 19 (2) ◽  
pp. 41-46
Author(s):  
A. R. Monakhov ◽  
O. M. Tsiroulnikova ◽  
T. A. Dzhanbekov ◽  
D. Dzhiner ◽  
I. E. Pashkova ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Donor Liver ◽  
Course Of Disease ◽  
Donor Liver Transplantation ◽  
Right Lobe ◽  
P Type

Wilson’s disease is a rare congenital disease caused by deficiency of the copper-transporting P-type ATPase-B enzyme. The course of disease varies widely from the latent form to the acute liver failure which is observed in 5% of Wilson’s disease cases. This clinical case represents experience of liver transplantation as the only curative treatment for patients with fulminant form of Wilson’s disease demonstrating excellent postoperative results. Living donor liver transplantation allows performing the operation in the shortest possible time which is necessary in acute liver failure. The plasmapheresis with plasma exchange AB (IV) allows preparing the patient for transplantation with incompatible blood group.

scholarly journals Wilsons Disease Presenting as Acute Fulminant Hepatic Failure

2021 ◽  
Vol 10 (16) ◽  
pp. 1185-1186
Author(s):  
Kolluru Karthik Raja
Keyword(s):  
Liver Failure ◽  
Acute Liver Failure ◽  
Fulminant Hepatic Failure ◽  
Hepatic Failure ◽  
Wilson’S Disease ◽  
Genetic Disorder ◽  
Young Male ◽  
Copper Metabolism ◽  
Wilson's Disease ◽  
Heavy Metal Accumulation

Wilson’s disease (WD) is a genetic disorder characterised by mutations in copper metabolism. Adenosine triphosphate (ATPase) ATP7B gene is responsible for disturbance in copper metabolism that leads to accumulation of copper mainly in liver and also in extra hepatic organs (like brain, cornea, heart and kidney).1 Heavy metal accumulation in brain (mainly in basal ganglia) leads to neuropsychiatric manifestations.2 Kayser Fleischer (KF) ring is golden brown ring distributed along the periphery of cornea. It is due to abnormal deposition of copper in the Descemet’s membrane of cornea. Kayser Fleischer (KF) ring is a pathognomonic sign of Wilson’s disease. Fulminant hepatic failure can be the first presentation of WD. Patients presenting with fulminant Wilson’s disease (FWD) clinically present as acute liver failure with encephalopathy and coagulopathy. The mortality of patients is high and orthotropic liver transplantation is the only option, which has been shown effective in patients with Fulminant Wilson’s disease.3 We are report a case of a young male who presented as acute liver failure first time in life and later was diagnosed as FWD.

Acute liver failure in Hungarian Wilson's disease patients

2009 ◽  
Vol 47 (05) ◽  
Author(s):  
E Horváth ◽  
A Folhoffer ◽  
T Csák ◽  
A Horváth ◽  
J Osztovits ◽  
...  
Keyword(s):  
Liver Failure ◽  
Acute Liver Failure ◽  
Wilson’S Disease ◽  
Wilson's Disease
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