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Publication Record


Re-evaluation of the indications for liver transplantation in Wilson's disease based on the outcomes of patients referred to a transplant center.
Ohya Y, Okajima H, Honda M, Hayashida S, Suda H, Matsumoto S, Lee KJ, Yamamoto H, Takeichi T, Mitsubuchi H, Asonuma K, Endo F, Inomata Y
(2013) Pediatr Transplant 17: 369-73
MeSH Terms: Adolescent, Adult, Chelating Agents, Child, Female, Follow-Up Studies, Hepatic Encephalopathy, Hepatolenticular Degeneration, Humans, Liver Failure, Acute, Liver Function Tests, Liver Transplantation, Male, Referral and Consultation, Treatment Outcome, Young Adult
Show Abstract · Added February 11, 2015
The aim of this study was to re-evaluate the indications and timing of LT for WD. From 2000 to 2009, eight patients with WD who had been referred to our institution for LT were enrolled in this study. The mean patient age was 15.9 yr (range, 7-37 yr). Four patients could not receive LT, because there were no available donors. All four patients were treated with chelating agent medication. Three of them (two of two patients with fulminant WD and one of two with cirrhotic WD) who did not undergo LT are still alive and doing well with stable liver functional tests. Only one of the patients with cirrhotic WD who did not undergo LT died of hepatic failure. Even among the four patients who underwent LT, one with fulminant WD recovered from hepatic encephalopathy with apheresis therapy and chelating agent. He later required LT because of severe neutropenia from d-penicillamine. The other three patients who underwent LT recovered and have been doing well. Some of the patients with WD can recover and avoid LT with medical treatment. Even when WD has progressed liver cirrhosis and/or fulminant hepatic failure at the time of diagnosis, medical treatment should be tried before considering LT.
© 2013 John Wiley & Sons A/S.
0 Communities
1 Members
0 Resources
16 MeSH Terms
Extraocular muscle dystonia due to acquired (non-Wilsonian) hepatocerebral degeneration.
Ferrara J, Gupta D, Foster E, Garman K, Stacy M
(2008) Mov Disord 23: 875-8
MeSH Terms: Diagnosis, Differential, Dyskinesias, Dystonic Disorders, Female, Globus Pallidus, Hepatolenticular Degeneration, Humans, Liver Cirrhosis, Magnetic Resonance Imaging, Middle Aged, Oculomotor Muscles, Substantia Nigra, alpha 1-Antitrypsin Deficiency
Show Abstract · Added February 28, 2014
We present a video report of a patient with advanced non-Wilsonian cirrhotic liver disease who developed extraocular muscle dystonia (oculogyric crisis) and severe orofaciolingual dyskinesias. Acquired hepatocerebral degeneration causes choreic movements, especially of cranial muscles, but dystonic ocular spasm is an infrequent manifestation of this disorder. This case illustrates that AHD should be considered in the differential diagnosis of extraocular muscle dystonia.
(c) 2008 Movement Disorder Society.
0 Communities
1 Members
0 Resources
13 MeSH Terms