title: Zinc Therapy for Wilson Disease in Children in French Pediatric Centers.
abstract: OBJECTIVE
  Zinc therapy is considered a good option in Wilson disease (WD), as a first-line treatment in presymptomatic children and a maintenance therapy after the initial chelator therapy. The aim of the study was to determine the practical use of zinc treatment in French pediatric centers.
  
  
  METHODS
  A national survey was conducted in the 6 French centers using zinc acetate to treat WD. Clinical and biological parameters, dosage, and outcome were recorded.
  
  
  RESULTS
  A total of 26 children were reported to be treated with zinc acetate, alone or in association with chelators. Of the 9 children (35%) who received zinc alone as a first-line therapy, 2 were switched to D-penicillamine because of inefficacy and 7 remained on zinc alone, but serum transaminase levels normalized in only 4 of them. Five children (19%) were initially treated with zinc in association with D-penicillamine (n = 4) or Trientine (n = 1) with good efficacy. Among the 12 children (46%) who received zinc as a maintenance therapy after D-penicillamine, no relapse of hepatic cytolysis occurred during a median follow-up of 5.2 years, but 2 of them were switched to Trientine because of zinc-related adverse effects. Epigastric pain was observed in 4 children, and a gastric perforation occurred in 1 child.
  
  
  CONCLUSIONS
  The present study demonstrates poor efficacy of zinc as first-line therapy to control liver disease in half presymptomatic children and a high incidence of related gastrointestinal adverse effects in children with WD.
authors.affiliation: *Department of Pediatric Gastroenterology and Hepatology, University Hospital, Bordeaux †Department of Pediatric Gastroenterology and Hepatology, University Hospital, Lille ‡Department of Pediatric Hepatology, Necker University Hospital, Paris §Department of Pediatric Gastroenterology and Hepatology, University Hospital, Rennes ||Department of Pediatric Gastroenterology and Hepatology, University Hospital, Lyon ¶Department of Pediatric Gastroenterology and Hepatology, University Hospital, Nancy, France.
authors.forename: Raoul
authors.initials: R
authors.lastname: Santiago
authors.forename: Frédéric
authors.initials: F
authors.lastname: Gottrand
authors.forename: Dominique
authors.initials: D
authors.lastname: Debray
authors.forename: Laure
authors.initials: L
authors.lastname: Bridoux
authors.forename: Alain
authors.initials: A
authors.lastname: Lachaux
authors.forename: Alain
authors.initials: A
authors.lastname: Morali
authors.forename: Delphine
authors.initials: D
authors.lastname: Lapeyre
authors.forename: Thierry
authors.initials: T
authors.lastname: Lamireau
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D000293:Adolescent; D002614:Chelating Agents / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003300:Copper / Q000378:metabolism; D005260:Female; D005602:France; D019538:Health Care Surveys; D006268:Health Facilities; D006527:Hepatolenticular Degeneration / Q000097:blood / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007223:Infant; D008099:Liver / Q000187:drug effects* / Q000378:metabolism / Q000473:pathology; D008297:Male; D010372:Pediatrics; D010396:Penicillamine / Q000627:therapeutic use*; D012189:Retrospective Studies; D013270:Stomach / Q000187:drug effects; D014131:Trace Elements / Q000009:adverse effects / Q000378:metabolism / Q000627:therapeutic use*; D000637:Transaminases / Q000097:blood; D016896:Treatment Outcome; D014266:Trientine; D015032:Zinc / Q000009:adverse effects / Q000627:therapeutic use*; D019345:Zinc Acetate / Q000009:adverse effects / Q000627:therapeutic use
chemical_list: D002614:Chelating Agents; D014131:Trace Elements; D003300:Copper; D000637:Transaminases; D019345:Zinc Acetate; D010396:Penicillamine; D015032:Zinc; D014266:Trientine