title: Liver transplantation: treatment of choice for hepatic and neurological manifestation of Wilson's disease.
abstract: 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.affiliation: Department of Surgery, Virchow Klinikum, Humboldt University Berlin, Germany.
authors.forename: G
authors.initials: G
authors.lastname: Schumacher
authors.forename: K P
authors.initials: KP
authors.lastname: Platz
authors.forename: A R
authors.initials: AR
authors.lastname: Mueller
authors.forename: R
authors.initials: R
authors.lastname: Neuhaus
authors.forename: T
authors.initials: T
authors.lastname: Steinmüller
authors.forename: W O
authors.initials: WO
authors.lastname: Bechstein
authors.forename: M
authors.initials: M
authors.lastname: Becker
authors.forename: W
authors.initials: W
authors.lastname: Luck
authors.forename: M
authors.initials: M
authors.lastname: Schuelke
authors.forename: P
authors.initials: P
authors.lastname: Neuhaus
mesh_terms: D000293:Adolescent; D000328:Adult; D000743:Anemia, Hemolytic / Q000503:physiopathology; D001259:Ataxia / Q000503:physiopathology; D002614:Chelating Agents / Q000627:therapeutic use; D003680:Deglutition Disorders / Q000503:physiopathology; D004401:Dysarthria / Q000503:physiopathology; D004421:Dystonia / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006236:Handwriting; D006501:Hepatic Encephalopathy / Q000601:surgery; D006527:Hepatolenticular Degeneration / Q000188:drug therapy / Q000451:nursing / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D008103:Liver Cirrhosis / Q000503:physiopathology / Q000601:surgery*; D017114:Liver Failure, Acute / Q000503:physiopathology / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D009069:Movement Disorders / Q000503:physiopathology / Q000601:surgery*; D010396:Penicillamine / Q000627:therapeutic use; D012798:Sialorrhea / Q000503:physiopathology; D015996:Survival Rate; D016896:Treatment Outcome; D014202:Tremor / Q000503:physiopathology; D015032:Zinc / Q000627:therapeutic use
chemical_list: D002614:Chelating Agents; D010396:Penicillamine; D015032:Zinc