Abstract | BACKGROUND AND AIMS:
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 (nā=ā4) or Trientine (nā=ā1) 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.
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Authors | Raoul Santiago, Frédéric Gottrand, Dominique Debray, Laure Bridoux, Alain Lachaux, Alain Morali, Delphine Lapeyre, Thierry Lamireau |
Journal | Journal of pediatric gastroenterology and nutrition
(J Pediatr Gastroenterol Nutr)
Vol. 61
Issue 6
Pg. 613-8
(Dec 2015)
ISSN: 1536-4801 [Electronic] United States |
PMID | 26230903
(Publication Type: Journal Article)
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Chemical References |
- Chelating Agents
- Trace Elements
- Copper
- Transaminases
- Zinc Acetate
- Penicillamine
- Zinc
- Trientine
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Topics |
- Abdominal Pain
(etiology)
- Adolescent
- Chelating Agents
(therapeutic use)
- Child
- Child, Preschool
- Copper
(metabolism)
- Female
- France
- Health Care Surveys
- Health Facilities
- Hepatolenticular Degeneration
(blood, complications, drug therapy, pathology)
- Humans
- Infant
- Liver
(drug effects, metabolism, pathology)
- Male
- Pediatrics
- Penicillamine
(therapeutic use)
- Retrospective Studies
- Stomach
(drug effects)
- Trace Elements
(adverse effects, metabolism, therapeutic use)
- Transaminases
(blood)
- Treatment Outcome
- Trientine
- Zinc
(adverse effects, therapeutic use)
- Zinc Acetate
(adverse effects, therapeutic use)
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