Document Detail


Liver transplantation: treatment of choice for hepatic and neurological manifestation of Wilson's disease.
MedLine Citation:
PMID:  9193846     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Liver transplantation (LTX) is an approved method to treat patients with end-stage liver cirrhosis and acute liver failure due to Wilson's disease. Initially, there was some consideration about the indication for LTX in the case of Wilson's disease with severe neurological impairment but normal liver function. From 1988 until 1995, 13 out of 700 LTX (1.9%) were performed for Wilson's disease. Indications for LTX were (I) intractable neurological impairment with normal liver function (n = 4; including one patient with Child A cirrhosis), (II) fulminant hepatic failure (n = 3), and (III) end-stage liver cirrhosis (n = 6) (Child B, n = 1; Child C, n = 5). There were 8 females and 5 males with a mean age of 27 yr (range 15-34 yr). All patients of group I required continuous nursing care before LTX, in spite of pretreatment with d-penicillamine and zinc. The most frequent symptoms in these patients were dysphagia (n = 4), dysarthria (n = 4), tremor (n = 4), sialorrhea (n = 3), ataxia (n = 3), dystonia (n = 3) and handwriting difficulties (n = 3). All patients of group II presented with hemolytic anemia. The survival rate was 100%, and all patients were doing well after a mean follow-up period of 32.8 months (range 8-68 months). The postoperative course was without severe infectious and other complications. All patients of group I revealed the first signs of improvement for all types of neurological symptoms 4-6 wk after LTX. One patient has been without any symptoms from 18 months until 5.5 yr after LTX. Two patients with short-term follow-up also had noticeable improvement of neurological impairment, but residual symptoms are still present. One patient showed only slight improvement. We conclude that Wilson's disease may be a good indication for LTX for both neurological manifestation with stable liver function and hepatic manifestation with cirrhosis or acute liver failure.
Authors:
G Schumacher; K P Platz; A R Mueller; R Neuhaus; T Steinmüller; W O Bechstein; M Becker; W Luck; M Schuelke; P Neuhaus
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Clinical transplantation     Volume:  11     ISSN:  0902-0063     ISO Abbreviation:  Clin Transplant     Publication Date:  1997 Jun 
Date Detail:
Created Date:  1997-08-04     Completed Date:  1997-08-04     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8710240     Medline TA:  Clin Transplant     Country:  DENMARK    
Other Details:
Languages:  eng     Pagination:  217-24     Citation Subset:  IM    
Affiliation:
Department of Surgery, Virchow Klinikum, Humboldt University Berlin, Germany.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Anemia, Hemolytic / physiopathology
Ataxia / physiopathology
Chelating Agents / therapeutic use
Deglutition Disorders / physiopathology
Dysarthria / physiopathology
Dystonia / physiopathology
Female
Follow-Up Studies
Handwriting
Hepatic Encephalopathy / surgery
Hepatolenticular Degeneration / drug therapy,  nursing,  physiopathology,  surgery*
Humans
Liver Cirrhosis / physiopathology,  surgery*
Liver Failure, Acute / physiopathology,  surgery*
Liver Transplantation*
Male
Movement Disorders / physiopathology,  surgery*
Penicillamine / therapeutic use
Sialorrhea / physiopathology
Survival Rate
Treatment Outcome
Tremor / physiopathology
Zinc / therapeutic use
Chemical
Reg. No./Substance:
0/Chelating Agents; 52-67-5/Penicillamine; 7440-66-6/Zinc

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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