• Raoul Santiago, Frédéric Gottrand, Dominique Debray, Laure Bridoux, Alain Lachaux, Alain Morali, Delphine Lapeyre, and Thierry Lamireau.
• *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.
• J. Pediatr. Gastroenterol. Nutr. 2015 Dec 1; 61 (6): 613-8.

Background And AimsZinc 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.MethodsA national survey was conducted in the 6 French centers using zinc acetate to treat WD. Clinical and biological parameters, dosage, and outcome were recorded.ResultsA 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.ConclusionsThe 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.

45 keywords...

hide…