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 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 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.

Urgent liver transplantation for acute liver failure due to Wilson's disease

2019 ◽  
Vol 42 (6) ◽  
pp. 392-393
Author(s):  
Ignacio Estrada León ◽  
Ana Guiberteau Sánchez ◽  
Gema Vinagre Rodríguez ◽  
Gerardo Blanco Fernández ◽  
Demetrio Pérez Civantos ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
Wilson’S Disease ◽  
Wilson's Disease

scholarly journals Acute Liver Failure for Liver Transplantation Due to Hepatolenticular Degeneration:a Case Report and Literature Review

2020 ◽  
Author(s):  
Hai-Bo Yan ◽  
Jing-Wei Liu ◽  
Yu-mei Li
Keyword(s):  
Liver Transplantation ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
High Mortality ◽  
Wilson Disease ◽  
Wilson’S Disease ◽  
Varicose Veins ◽  
Flow Direction ◽  
Wilson's Disease ◽  
Rapid Progress

Abstract BackgroundWilson disease (WD) is a genetic disease of abnormal copper metabolism, pediatric acute liver failure (PALF) is a life-threatening illness. Wilson’s disease with acute liver failure has rapid progress, and high mortality, especially in children.Case presentationThe patient was a 14-year-old girl who developed vomiting, jaundice within 7 days, leading to the initial suspicion of acute liver failure. Results of further investigation by geen and histological examination were consistent with a diagnosis of Wilson disease. She was treated with plasma exchange and DPMAS. After six days of treatment, coagulation routine and bilirubin improved, and she was gradually conscious. On the 8th day after admission, the condition was deteriorate, she was confusion, and had dyspnea, bleeding. The coagulation routine continued to deteriorate, bilirubin did not decrease significantly. Doppler ultrasound examination showed that the portal vein blood flow direction was away from the liver. Abdominal CT showed gastric varicose veins. She underwent liver transplantation. Thirty-three days later, she recovered. Patients with PALF should be transferred to a center with a transplant unit early. Once conservative treatment fails, LT should be performed. ConclusionA review of the literature shows that Wilson disease can occur at any age, including childhood. Wilson’s disease with acute liver failure has rapid progress, and high mortality. Early diagnosis and treatment is the key to improving the prognosis.

scholarly journals Liver transplantation for late-onset presentations of acute liver failure in Wilson's disease: The UK experience over 2 decades

JHEP Reports ◽  
2020 ◽  
Vol 2 (3) ◽  
pp. 100096 ◽  
Author(s):  
Samuel Shribman ◽  
Gwilym Webb ◽  
Rhiannon Taylor ◽  
Thomas T. Warner ◽  
Adam Duckworth ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
Wilson’S Disease ◽  
Late Onset ◽  
Wilson's Disease ◽  
The Uk

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

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 Liver Transplantation as a Cure for Neurologically Advanced Wilson’s disease. Learning More from Experience

2020 ◽  
Vol 4 (4) ◽  
Author(s):  
Marina Moguilevitch ◽  
Tracey Straker ◽  
Keyword(s):  
Liver Transplantation ◽  
Liver Failure ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Clinical Findings ◽  
Chronic Liver ◽  
Older Children ◽  
Chronic Liver Failure ◽  
Long Term Follow Up ◽  
Short And Long Term

Clinical presentation and progression of Wilson’s disease can be diverse in different groups of patients. While young children most likely to present with acute or chronic liver failure, older children and adults may exhibit extrahepatic manifestation like neuropsychiatric, cardiac, renal, pancreatic and several others. Diagnosis of Wilson’s disease is based on the combination of specific clinical findings, laboratory and genetic testing. There are different modalities of treatment available for this condition. Liver transplantation has become a acceptable treatment option for acute and chronic liver failure related to Wilson’s disease. It allows for resolution of metabolic disorder and hepatic disease progression. There are no definitive criteria and established outcomes in transplantation of the patients with Wilson’s disease who have different degree of neuropsychiatric presentation. Therefore, it is very important to create specific criteria for selecting of these patients before transplantation and to have short-and long-term follow up system to improve their outcome and survival.

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