title: Gastrointestinal side effects in children with Wilson's disease treated with zinc sulphate.
abstract: OBJECTIVE
  To investigate the side effects of a zinc sulphate therapy in a cohort of Polish pediatric patients with Wilson's disease.
  
  
  METHODS
  We retrospectively analyzed a cohort of 53 pediatric patients with Wilson's disease treated at the Children's Memorial Health Institute in Warsaw, Poland between the years 1996 and 2011 with zinc sulphate. Patients were diagnosed with Wilson's disease according to the scoring system of Ferenci, with 49 cases confirmed by mutation analysis. Data about the dosage scheme of zinc sulphate, side effects and efficacy and toxicity of the treatment were collected and recorded in the patient's medical chart at each visit to the hospital.
  
  
  RESULTS
  Mean age of diagnosis for the entire cohort was 10 years (range, 2.5-17 years). Duration of treatment with zinc sulfate was 83.3 wk (range, 8-344 wk). Side effects, all of gastrointestinal origin, were observed in 21 patients (40%--9 males and 12 females), irrespective of the duration of therapy. Thirteen out of 21 patients were over the age of 10 years. The most common ATP7B mutation was p.H1069Q. Esophagogastroduodenoscopy, performed in 7 patients (33.3%) suffering from persistent and severe abdominal pain, revealed gastrointestinal ulcerations or erosions with negative Helicobacter pylori tests in all subjects investigated. The above mentioned 7 patients were treated with proton pump inhibitors. Three of those experienced resolution of symptoms, whereas proton-pump inhibitors failed to alleviate symptoms of the remaining four children and conversion of therapy to D-penicillamine was needed.
  
  
  CONCLUSIONS
  Zinc sulphate appears to cause significant gastrointestinal side effects, which children on therapy for Wilson's disease should be closely monitored for.
authors.affiliation: Department of Gastroenterology, Hepatology and Malnutrition, the Children's Memorial Health Institute, 04-730 Warsaw, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Wiernicka
authors.forename: Wojciech
authors.initials: W
authors.lastname: Jańczyk
authors.forename: Maciej
authors.initials: M
authors.lastname: Dądalski
authors.forename: Yesim
authors.initials: Y
authors.lastname: Avsar
authors.forename: Hartmut
authors.initials: H
authors.lastname: Schmidt
authors.forename: Piotr
authors.initials: P
authors.lastname: Socha
mesh_terms: D000251:Adenosine Triphosphatases / Q000235:genetics; D000293:Adolescent; D027682:Cation Transport Proteins / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D000073840:Copper-Transporting ATPases; D016145:Endoscopy, Digestive System; D005260:Female; D005767:Gastrointestinal Diseases / Q000139:chemically induced*; D041981:Gastrointestinal Tract / Q000187:drug effects / Q000473:pathology*; D016481:Helicobacter Infections; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D008297:Male; D009154:Mutation; D010396:Penicillamine / Q000627:therapeutic use; D011044:Poland; D012189:Retrospective Studies; D014456:Ulcer / Q000139:chemically induced*; D019287:Zinc Sulfate / Q000009:adverse effects*
chemical_list: D027682:Cation Transport Proteins; D019287:Zinc Sulfate; D000251:Adenosine Triphosphatases; C000612464:ATP7B protein, human; D000073840:Copper-Transporting ATPases; D010396:Penicillamine
keywords: Abdominal pain; Gastrointestinal ulcer; Therapy; Wilson’s disease; Zinc