The Need for Consensus About Liver Transplantation For Patients With Neuropsychiatric Wilson’s Disease

2021 ◽  
pp. 152692482110028
Author(s):  
Alberto Ferrarese ◽  
Patrizia Burra
Keyword(s):  
Liver Transplantation ◽  
Patient Outcomes ◽  
Wilson’S Disease ◽  
Therapeutic Option ◽  
Copper Metabolism ◽  
Wilson's Disease ◽  
Published Data ◽  
Neurological Improvement ◽  
Effective Therapeutic Option

Liver transplantation is considered an effective therapeutic option for Wilson’s disease (WD) patients with hepatic phenotype, since it removes the inherited defects of copper metabolism, and is associated with excellent graft and patient outcomes. The role of liver transplantation in WD patients with mixed hepatic and neuropsychiatric phenotype has remained controversial over time, mainly because of high post-operative complications, reduced survival and a variable, unpredictable rate of neurological improvement. This article critically discusses the recently published data in this field, focussing in more detail on isolated neuropsychiatric phenotype as a potential indication for liver transplantation in WD patients.

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.

No neurological improvement after liver transplantation for Wilson's disease

2009 ◽  
Vol 92 (5) ◽  
pp. 405-408 ◽  
Author(s):  
M. Guarino ◽  
A. Stracciari ◽  
R. D'Alessandro ◽  
P. Pazzaglia
Keyword(s):  
Liver Transplantation ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Neurological Improvement

scholarly journals Resolved Psychosis after Liver Transplantation in a Patient with Wilson’s Disease

2011 ◽  
Vol 7 (1) ◽  
pp. 182-184 ◽  
Author(s):  
Orazio Sorbello ◽  
Daniela Riccio ◽  
Margherita Sini ◽  
Mauro Carta ◽  
Luigi Demelia
Keyword(s):  
Liver Transplantation ◽  
Liver Function ◽  
Wilson’S Disease ◽  
Psychiatric Symptoms ◽  
Copper Metabolism ◽  
Wilson's Disease ◽  
Hepatic Disease ◽  
Behavioral Symptoms ◽  
Psychiatric Disease ◽  
History Of

A psychiatric involvement is frequently present in Wilson’s disease. Psychiatric symptoms are sometimes the first and only manifestation of Wilson’s disease. More often a psychiatric involvement is present beside a neurologic or hepatic disease. We describe the case of a 18 years-old male patient who shows a clinic and laboratoristic pattern of cirrhosis and an history of subchronic hallucinatory psychosis, behavioral symptoms and mood disturbances with depressed mood. He hadn’t familiar history of liver or psychiatric disease. Laboratory and imaging tests confirmed the diagnosis of Wilson’s disease with psichiatric involvement. After liver transplantation copper metabolism and liver function normalised and we noticed no recurrency of the psichiatric illness. Very few cases of psychiatric improvement after orthotopic liver transplantation (OLT) has been described until now.

Neurological improvement of Wilson's disease after liver transplantation

1997 ◽  
Vol 29 (1-2) ◽  
pp. 497-498 ◽  
Author(s):  
C.L. Chen ◽  
Y.S. Chen ◽  
C.C. Lui ◽  
S.P. Hsu
Keyword(s):  
Liver Transplantation ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Neurological Improvement

Wilson's disease

2011 ◽  
Author(s):  
R. Mark Beattie ◽  
Anil Dhawan ◽  
John W.L. Puntis
Keyword(s):  
Liver Transplantation ◽  
Young Adults ◽  
Liver Disease ◽  
Clinical Features ◽  
Neurological Disease ◽  
Wilson’S Disease ◽  
Copper Metabolism ◽  
Wilson's Disease ◽  
Age Group ◽  
Group 3

Clinical features 412Diagnosis 413Management 414Indications for liver transplantation 415• Wilson's disease (WD) is an autosomal disorder of copper metabolism. The gene ATP7B codes for a copper carrier which both exports copper from hepatocyte to bile, and enables caeruloplasmin synthesis.• WD occurs worldwide with reported incidences of 5–30 per million population. WD may present with almost any variety of liver disease in the age group 3–12 years, with psychiatric and/or neurological disease in adolescence or young adults, with combined hepatic and neurological problems, or less commonly with haemolysis or arthritis....

scholarly journals Copper and Trace Elements in Gallbladder form Patients with Wilson’s Disease Imaged and Determined by Synchrotron X-ray Fluorescence

2021 ◽  
Vol 7 (12) ◽  
pp. 261
Author(s):  
Wolf Osterode ◽  
Gerald Falkenberg ◽  
Fritz Wrba
Keyword(s):  
Linear Relationship ◽  
Genetic Disease ◽  
Wilson’S Disease ◽  
Copper Metabolism ◽  
Wilson's Disease ◽  
Fundamental Parameter ◽  
X Ray ◽  
Significant Linear Relationship ◽  
Tissue Alterations

Investigations about suspected tissue alterations and the role of gallbladder in Wilson’s disease (WD)—an inherited genetic disease with impaired copper metabolism—are rare. Therefore, tissue from patients with genetically characterised WD was investigated by microscopic synchrotron X-ray fluorescence (µSRXRF). For two-dimensional imaging and quantification of elements, X-ray spectra were peak-fitted, and the net peak intensities were normalised to the intensity of the incoming monochromatic beam intensity. Concentrations were calculated by fundamental parameter-based program quant and external standardisation. Copper (Cu), zinc (Zn) and iron (Fe) along with sulphur (S) and phosphorus (P) mappings could be demonstrated in a near histological resolution. All these elements were increased compared to gallbladder tissue from controls. Cu and Zn and Fe in WD-GB were mostly found to be enhanced in the epithelium. We documented a significant linear relationship with Cu, Zn and sulphur. Concentrations of Cu/Zn were roughly 1:1 while S/Cu was about 100:1, depending on the selected areas for investigation. The significant linear relationship with Cu, Zn and sulphur let us assume that metallothioneins, which are sulphur-rich proteins, are increased too. Our data let us suggest that the WD gallbladder is the first in the gastrointestinal tract to reabsorb metals to prevent oxidative damage caused by metal toxicity.

scholarly journals Anti-neoplastic Potential of Flavonoids and Polysaccharide Phytochemicals in Glioblastoma

Molecules ◽  
2020 ◽  
Vol 25 (21) ◽  
pp. 4895
Author(s):  
Ayesha Atiq ◽  
Ishwar Parhar
Keyword(s):  
Cancer Progression ◽  
Therapeutic Option ◽  
Normal Brain ◽  
Current Standard ◽  
Pharmacological Agents ◽  
Initiation Phase ◽  
Therapeutic Outcomes ◽  
Effective Therapeutic Option ◽  
Patient Prognosis

Clinically, gliomas are classified into four grades, with grade IV glioblastoma multiforme being the most malignant and deadly, which accounts for 50% of all gliomas. Characteristically, glioblastoma involves the aggressive proliferation of cells and invasion of normal brain tissue, outcomes as poor patient prognosis. With the current standard therapy of glioblastoma; surgical resection and radiotherapy followed by adjuvant chemotherapy with temozolomide, it remains fatal, because of the development of drug resistance, tumor recurrence, and metastasis. Therefore, the need for the effective therapeutic option for glioblastoma remains elusive. Previous studies have demonstrated the chemopreventive role of naturally occurring pharmacological agents through preventing or reversing the initiation phase of carcinogenesis or arresting the cancer progression phase. In this review, we discuss the role of natural phytochemicals in the amelioration of glioblastoma, with the aim to improve therapeutic outcomes, and minimize the adverse side effects to improve patient’s prognosis and enhancing their quality of life.

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