{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T10:49:53Z","timestamp":1792838993798,"version":"4.5.0"},"reference-count":14,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2007,5,1]],"date-time":"2007-05-01T00:00:00Z","timestamp":1177977600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2007,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Objectives:<\/jats:title><jats:p>To evaluate the efficacy of and adherence to trientine and\/or zinc therapy in children with Wilson disease (WD).<\/jats:p><\/jats:sec><jats:sec><jats:title>Materials and Methods:<\/jats:title><jats:p>We retrospectively reviewed the clinical records of all children with WD in the pediatric liver\/liver transplant program at our institution between 1998 and 2006.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results:<\/jats:title><jats:p>A total of 22 children with WD were evaluated and treated. Seven with fulminant disease required liver transplantation and 15 were treated with trientine and\/or zinc. Ten of those 15 had follow\u2010up for 12 to 60 months and 6 of the latter 10 were followed for 12 to 18 months. All 10 patients were started on a trientine treatment regimen. Mean alanine aminotransferase (ALT) levels decreased from 183 \u00b1 103 IU at presentation (n = 10) to 80 \u00b1 46 IU at 12 months (n = 10) and 66 \u00b1 40 IU at 18 months (n = 7). Mean 24\u2010hour urinary copper levels increased from 156 \u03bcg at presentation to 494 \u03bcg at 1 to 2 months, then decreased to 71 \u03bcg after 21 to 24 months of treatment. Three of 10 patients had normalized ALT levels and 1 patient with cirrhosis continued with normal ALT levels since presentation. Four of 10 patients were documented to be nonadherent, as manifested by increased ALT levels (99 \u00b1 31 IU); 1 patient had previously normalized ALT levels. In 3 of 10 patients, ALT level decreased but remained at an abnormal level (93 \u00b1 53 IU).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions:<\/jats:title><jats:p>Trientine and\/or zinc therapy is effective for children with WD. Nonadherence is a common cause of increased aminotransferase levels in patients with WD.<\/jats:p><\/jats:sec>","DOI":"10.1097\/mpg.0b013e3180467715","type":"journal-article","created":{"date-parts":[[2007,4,20]],"date-time":"2007-04-20T08:05:12Z","timestamp":1177056312000},"page":"596-602","source":"Crossref","is-referenced-by-count":55,"title":["Wilson Disease in Children: Serum Aminotransferases and Urinary Copper on Triethylene Tetramine Dihydrochloride (Trientine) Treatment"],"prefix":"10.1002","volume":"44","author":[{"given":"Ronen","family":"Arnon","sequence":"first","affiliation":[{"name":"Department of Pediatrics the Recanati\u2010Miller Transplant Institute Mount Sinai Medical Center Mount Sinai School of Medicine New York New York"},{"name":"the Recanati\u2010Miller Transplant Institute Mount Sinai Medical Center Mount Sinai School of Medicine New York New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith Flores","family":"Calderon","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology Hospital de Pediatria Centro Medico Nacional Siglo XXI Instituto Mexicano del Seguro Social Mexico City Mexico"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Schilsky","sequence":"additional","affiliation":[{"name":"Departments of Medicine Center for Liver Disease and Transplantation New York New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sukru","family":"Emre","sequence":"additional","affiliation":[{"name":"Department of Pediatrics the Recanati\u2010Miller Transplant Institute Mount Sinai Medical Center Mount Sinai School of Medicine New York New York"},{"name":"Department of Surgery the Recanati\u2010Miller Transplant Institute Mount Sinai Medical Center Mount Sinai School of Medicine New York New York"},{"name":"the Recanati\u2010Miller Transplant Institute Mount Sinai Medical Center Mount Sinai School of Medicine New York New York"},{"name":"Department of Surgery Weill Cornell Medical Center New York New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin L","family":"Shneider","sequence":"additional","affiliation":[{"name":"Department of Pediatrics the Recanati\u2010Miller Transplant Institute Mount Sinai Medical Center Mount Sinai School of Medicine New York New York"},{"name":"the Recanati\u2010Miller Transplant Institute Mount Sinai Medical Center Mount Sinai School of Medicine New York New 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