title: Histologic evolution and long-term outcome of Wilson's disease:  results of a single-center experience.
abstract: OBJECTIVE
  Wilson's disease (WD) is a rare inborn disease related to copper storage, leading to liver cirrhosis and neuropsychological deterioration. The aim of this study was to determine the clinical presentation and long-term outcome, and to examine the progression of hepatic histopathology in serial liver biopsies from WD patients.
  
  
  METHODS
  We carried out a retrospective analysis of 60 patients with WD treated with zinc and/or penicillamine. Demographic, clinical, and laboratory data were gathered and 40 patients underwent an initial biopsy and at least one repeat biopsy. Patients were divided into two groups: progressors (patients who presented worsening of at least one unit of fibrosis) and nonprogressors (patients who presented stable or improved fibrosis scores).
  
  
  RESULTS
  A total of 33/40 (83%) patients (nonprogressors) showed stable hepatic histology or improvement. Seven of 40 (17%) patients (progressors) showed worsening of fibrosis. There was no significant correlation between the histological findings and clinical parameters or initial presentation.
  
  
  CONCLUSIONS
  In our study cohort, liver disease was stable or improving in most of the patients, and development of progressive hepatic symptoms while under treatment was a rare event. The development of new symptoms while under treatment or progression of pre-existing symptoms was more often recorded for neurological than for hepatic symptoms.
authors.affiliation: Department of Gastroenterology, AOU of Cagliari, Cagliari, Italy.
authors.forename: Margherita
authors.initials: M
authors.lastname: Sini
authors.forename: Orazio
authors.initials: O
authors.lastname: Sorbello
authors.forename: Francesco
authors.initials: F
authors.lastname: Sanna
authors.forename: Federica
authors.initials: F
authors.lastname: Battolu
authors.forename: Alberto
authors.initials: A
authors.lastname: Civolani
authors.forename: Daniela
authors.initials: D
authors.lastname: Fanni
authors.forename: Gavino
authors.initials: G
authors.lastname: Faa
authors.forename: Luigi
authors.initials: L
authors.lastname: Demelia
mesh_terms: D000293:Adolescent; D000328:Adult; D001706:Biopsy; D002614:Chelating Agents / Q000627:therapeutic use; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007558:Italy; D008099:Liver / Q000187:drug effects / Q000473:pathology*; D008103:Liver Cirrhosis / Q000188:drug therapy / Q000209:etiology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010396:Penicillamine / Q000627:therapeutic use; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D014266:Trientine / Q000627:therapeutic use; D055815:Young Adult; D019287:Zinc Sulfate / Q000627:therapeutic use
chemical_list: D002614:Chelating Agents; D019287:Zinc Sulfate; D010396:Penicillamine; D014266:Trientine