{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,25]],"date-time":"2026-04-25T09:58:12Z","timestamp":1777111092699,"version":"3.51.4"},"reference-count":34,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2024,1,23]],"date-time":"2024-01-23T00:00:00Z","timestamp":1705968000000},"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":[[2024,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>Long\u2010term D\u2010penicillamine (D\u2010pen) therapy in Wilson disease (WD) has numerous adverse effects which advocates its withdrawal, but with an inherent risk of relapse. This prospective observational study was conducted with the objective of evaluating incidence of relapse following withdrawal of D\u2010pen from combination (D\u2010pen\u2009+\u2009zinc) therapy in maintenance phase of previously symptomatic hepatic WD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Hepatic WD patients &lt;18 years of age and on combination therapy for &gt;2 years with 6 months of biochemical remission were included. Biochemical remission was defined as achievement of (i) aspartate aminotransferase (AST) and alanine aminotransferase (ALT)\u00a0\u22641.5 times upper limit of normal (ULN), (ii) serum albumin &gt;3.5\u2009g\/dL, international normalized ratio (INR)\u2009&lt;1.5 and (iii) 24\u2010h urinary copper excretion (UCE) &lt;500\u2009mcg\/day, nonceruloplasmin\u2010bound\u2010copper (NCC)\u2009&lt;15\u2009mcg\/dL. After D\u2010pen withdrawal, monthly liver function test (LFT) and INR\u00a0and 3 monthly UCE and NCC\u00a0were done till 1 year or relapse (elevation of AST\/ALT\/both &gt;2 times ULN or total bilirubin &gt;2\u2009mg\/dL), whichever occurred earlier.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Forty\u2010five patients enrolled with median combination therapy duration of 36 months. Sixty percent of them had their index presentation as decompensated cirrhosis. Fourteen patients (31.8%) relapsed (cumulative incidence: 4 at 3 months, 11 at 6 months, and 14 at 12 months after D\u2010pen discontinuation). All relapsers had index presentation as decompensated cirrhosis. On Cox\u2010regression, ALT at D\u2010pen withdrawal was an independent predictor of relapse (hazard ratio [HR]: 1.077, 95% confidence interval [CI]: 1.014\u20131.145, <jats:italic>p<\/jats:italic>\u2009=\u20090.017) with area under the receiver operating characteristic (AUROC) of 0.860. ALT \u226540\u2009U\/L predicted risk of relapse with 85.7% sensitivity, 70.9% specificity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Incidence of relapse after withdrawal of D\u2010pen from combination therapy is 31.8% in hepatic WD. ALT \u226540\u2009U\/L, at the time of D\u2010pen stoppage, predicts future relapse.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jpn3.12128","type":"journal-article","created":{"date-parts":[[2024,1,23]],"date-time":"2024-01-23T09:38:18Z","timestamp":1706002698000},"page":"1017-1026","source":"Crossref","is-referenced-by-count":1,"title":["Relapse following withdrawal of D\u2010penicillamine from combination (D\u2010penicillamine\u2009+\u2009zinc) therapy in hepatic Wilson disease"],"prefix":"10.1002","volume":"78","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7280-5743","authenticated-orcid":false,"given":"Kalpana","family":"Panda","sequence":"first","affiliation":[{"name":"Department of Pediatric Hepatology Institute of Liver and Biliary Sciences New Delhi India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6275-7736","authenticated-orcid":false,"given":"Bikrant 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