Liver Transplantation in Wilson's Disease with Neurological Impairment: Evaluation in 4 Patients

2016 ◽  
Vol 77 (1-2) ◽  
pp. 5-15 ◽  
Author(s):  
Chloé Laurencin ◽  
Anne Sophie Brunet ◽  
Jérôme Dumortier ◽  
Laurence Lion-Francois ◽  
Stéphane Thobois ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Wilson’S Disease ◽  
Neurological Impairment ◽  
Wilson's Disease

Moyamoya disease as a possible cause of neurological impairment following liver transplantation for Wilson's disease

2011 ◽  
Vol 41 (3) ◽  
pp. 282-286 ◽  
Author(s):  
Federico Desideri ◽  
Massimo Marignani ◽  
Olivier Vanovermeire ◽  
Isabelle Colle ◽  
Gianfranco Delle Fave ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Moyamoya Disease ◽  
Wilson’S Disease ◽  
Neurological Impairment ◽  
Wilson's Disease ◽  
Possible Cause

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.

Liver transplantation for fulminant Wilson's disease, the significance of prothrombin time on admission

2000 ◽  
Vol 118 (4) ◽  
pp. A1495
Author(s):  
Pavel Trunecka ◽  
Miroslav Ryska ◽  
Jan Sperl ◽  
Lubomir Kozak ◽  
Milan Jirsa ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Prothrombin Time ◽  
Wilson’S Disease ◽  
Wilson's Disease

Successful treatment of fulminant Wilson's disease without liver transplantation

2014 ◽  
Vol 56 (3) ◽  
pp. 429-432 ◽  
Author(s):  
Mitsuo Motobayashi ◽  
Tetsuhiro Fukuyama ◽  
Yoshiko Nakayama ◽  
Kenji Sano ◽  
Shunsuke Noda ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Successful Treatment ◽  
Wilson’S Disease ◽  
Wilson's Disease

Cerebral manifestation of Wilson's disease successfully treated with liver transplantation

Neurology ◽  
1998 ◽  
Vol 51 (3) ◽  
pp. 863-865 ◽  
Author(s):  
R. T. Bax ◽  
A. Hassler ◽  
W. Luck ◽  
H. Hefter ◽  
I. Krageloh-Mann ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Wilson’S Disease ◽  
Wilson's Disease

Differential expression of proteins associated with liver transplantation in wilson’s disease patients

1998 ◽  
Vol 30 (7) ◽  
pp. 3263-3264
Author(s):  
K.C Chiang ◽  
T.L Pan ◽  
S Goto ◽  
Y.C Lin ◽  
C.Y Lai ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Differential Expression ◽  
Wilson’S Disease ◽  
Wilson's Disease

Long-term results of liver transplantation for Wilson's disease

2008 ◽  
Vol 32 (4) ◽  
pp. 378-381 ◽  
Author(s):  
V. Pabón ◽  
J. Dumortier ◽  
R. Gincul ◽  
J. Baulieux ◽  
C. Ducerf ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Long Term Results

Stomatocytosis Heralding a Case of Acute Wilsonian Crisis: Case Report and Review of the Literature.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 5090-5090
Author(s):  
Amit R. Mehta ◽  
Gratian Salaru ◽  
Jonathan Harrison
Keyword(s):  
Liver Transplantation ◽  
Case Report ◽  
Hemolytic Anemia ◽  
Wilson’S Disease ◽  
Total Bilirubin ◽  
Conflicts Of Interest ◽  
Wilson's Disease ◽  
Direct Bilirubin ◽  
Clinical Scenarios ◽  
New Onset

Abstract Abstract 5090 Introduction The evaluation of a patient with new onset hemolytic anemia is among the more common clinical scenarios prompting formal input by a hematologist. Although there are both common and uncommon etiologies, the presentation of Wilson's Disease, itself an uncommon disorder, as a fulminant hemolytic anemia, is very uncommon, and for this reason may not be readily considered within the differential diagnosis of new onset hemolytic anemia. Wilson's Disease occurs worldwide with an average prevalence of only 30 affected individuals per million population. In order to illustrate hemolytic anemia as an initial presentation of Wilson's Disease, a case is reported. Case Report A 28-year-old female patient presented with a recent diarrheal infection and new onset jaundice. Examination revealed an afebrile patient with gross jaundice, but no other abnormal findings. Initial significant laboratory findings included a hemoglobin of 7.1 gm/dL, white blood cell count of 30,900 per microliter, platelet count of 181,000 per microliter, creatinine of 2 mg/dL, and total bilirubin of approximately 10 mg/dL. Within two days, laboratory studies worsened to a serum creatinine of 3.5 mg/dL, total bilirubin 19.5 mg/dL, direct bilirubin 13.1 mg/dL, AST 130 IU/L, ALT 16 IU/L, and alkaline phosphatase < 5 IU/L. The serum LDH was markedly elevated at 1258 IU/L. Review of the peripheral blood film showed 87% segmented neutrophils with no schistocytosis. However, a striking stomatocytosis was present, comprising about 20% of the red blood cells (see Figure 1). In the next two days, the clinical condition dramatically worsened. The hemoglobin dropped to 4.4 gm/dL, and the prothrombin time increased from within normal limits to 52.7 seconds. Disseminated intravascular coagulation was not present. Haptoglobin and LDH remained consistent with a brisk intravascular hemolysis, and Coombs testing was negative. In addition, the total bilirubin rose to 52.5 mg/dL, with a direct bilirubin of 38.7 mg/dL. The patient's sensorium was becoming gradually impaired, consistent with a hepatic encephalopathy. Based on this constellation of findings, the possibility of a fulminant manifestation of Wilson's Disease, featuring severe hemolysis, was identified (see Table 1). This led to definitive testing for Wilson's Disease, which included a liver biopsy with qualitative and quantitative measurements for copper (copper dry weight = 966.7 micrograms/gm). With the diagnosis established, and using support from published literature, daily plasmapheresis was utilized to stabilize the patient in anticipation of orthotopic liver transplantation (Jhang JS et al. Journal of Clinical Apheresis 22(1):10-14, 2007). Subsequently, the patient was successfully transplanted, and continues to do well three months afterwards. Conclusion The present case highlights the value of understanding the possible etiologies of stomatocytosis, as observed in the peripheral smear, in the setting of a hemolytic anemia (see Table 2). In addition, as illustrated by this case, plasmapheresis can be an effective bridge to stabilize patients for liver transplantation in patients with Wilson's Disease who present with fulminant hemolytic anemia. Disclosures No relevant conflicts of interest to declare.

LIVING-DONOR ORTHOTOPIC LIVER TRANSPLANTATION FOR CHILDREN WITH WILSON’S DISEASE

2004 ◽  
Vol 78 ◽  
pp. 446-447
Author(s):  
F Özcay ◽  
H Karakayali ◽  
G Moray ◽  
R Emiroglu ◽  
A Dalgic ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Orthotopic Liver Transplantation ◽  
Living Donor ◽  
Wilson’S Disease ◽  
Wilson's Disease
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