{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T01:54:12Z","timestamp":1790992452295,"version":"4.1.0"},"reference-count":32,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2020,8,28]],"date-time":"2020-08-28T00:00:00Z","timestamp":1598572800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Hepato Biliary Pancreat"],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>As there is paucity of exclusive literature on pediatric hepatic Wilson's disease (WD), this study was undertaken to evaluate the efficacy of chelation on hepatocellular function and portal hypertension in WD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Wilson's disease patients with \u22659\u00a0months of follow\u2010up were evaluated for response to chelation therapy in the following categories: (a) complete remission, (b) partial remission (c) progression of disease; (d) drug toxicity. Pediatric end\u2010stage liver disease (PELD), Nazar and New Wilson Index scores were compared. Hemodynamically stable patients underwent esophagogastroduodenoscopy (baseline and surveillance) and received prophylaxis (primary or secondary). Endoscopic outcome was assessed at follow\u2010up.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of the 111 WD children (aged 9 [3\u201315] years; PELD score 16 [\u221211 to 60]), 65 with follow\u2010up of 3.6 (0.8\u201312) years on chelation (83% D\u2010penicillamine monotherapy, 17% D\u2010penicillamine and zinc) were analyzed. 81% had severe disease at presentation. Favorable outcome (complete and or partial remission), progression of disease and drug toxicity were seen in 71%, 29% and 10.8%, respectively. Two\u2010thirds had esophageal varices which did not show progression. Large esophageal varices (16%) were effectively downgraded in 3 (2\u20136) therapeutic endoscopic sessions. Nazar score and PELD score at baseline were independent predictors of outcome with favorable correlation with each other (<jats:italic>r<\/jats:italic>\u00a0=\u00a0.864, <jats:italic>P<\/jats:italic>\u00a0&lt;\u00a0.001). PELD cutoff 9.45 (AUC: 71%, sensitivity: 87%, specificity: 50%; <jats:italic>P<\/jats:italic>\u00a0=\u00a0.009) and Nazar score cut off 3.5 (AUC: 68%, sensitivity: 83%, specificity: 50%; <jats:italic>P<\/jats:italic>\u00a0=\u00a0.02) were associated with poor prognosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Despite severe liver disease, the majority of hepatic WD can be managed on D\u2010penicillamine monotherapy. PELD score and Nazar score effectively determine the outcome.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jhbp.812","type":"journal-article","created":{"date-parts":[[2020,8,3]],"date-time":"2020-08-03T21:13:56Z","timestamp":1596489236000},"page":"336-345","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Effect of chelation therapy in pediatric Wilson\u2019s disease: Liver and endoscopic outcome"],"prefix":"10.1002","volume":"28","author":[{"given":"Mridul Chandra","family":"Das","sequence":"first","affiliation":[{"name":"Department of Pediatric Gastroenterology Sanjay Gandhi Post Graduate Institute of Medical Sciences Lucknow India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2015-4069","authenticated-orcid":false,"given":"Moinak","family":"Sen Sarma","sequence":"additional","affiliation":[{"name":"Department of Pediatric Gastroenterology Sanjay Gandhi Post Graduate 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