Incidence of Relapse Following Withdrawal Of D-Penicillamine from Combination (D-Penicillamine + Zinc) Therapy in previously symptomatic Hepatic Wilson Disease

Background and Aim: Long-term D-Penicillamine (D-Pen) therapy in Wilson disease (WD) has numerous adverse effects which advocates its withdrawal, but with an inherent risk of relapse. This prospective study was conducted aim to evaluate incidence relapse following withdrawal D-Pen from Combination (D-Pen + Zinc) maintenance phase previously symptomatic hepatic WD. Methods: Hepatic WD patients <18 years age on combination for > 2 6 months biochemical remission were included. Biochemical defined as fulfilment 3 criteria (i) AST & ALT 1.5 times upper limit normal (ULN) (iii) serum albumin 3.5 gm/dl INR <1.5, (ii) 24 hours urinary copper excretion (UCE) 500 mcg/day non-ceruloplasmin bound (NCC) 15 mcg/dl. After monthly LFT &INR UCE&NCC done till 1 year or (elevation ULN total bilirubin 2mg/dl), whichever occurred earlier. Results: Total 45 enrolled median enrolment 13.5 a duration 36 months. Index presentations decompensated chronic liver (CLD): 22/45 (48.8%), acute failure: 5/45 (11.1%), compensated CLD: 18/45 (40%). Out 45, 14 (31%) relapsed (one:1 month, three:3 months, seven:6 two:9 one:12 after withdrawal). All relapsers had index presentation CLD. On multivariate cox-regression, at is predictor (HR: 1.097, 95%CI: 1.020-1.180, p =0.013) AUROC 0.786. cut-off >40 IU/L predicted 88.8% sensitivity, 70.6% specificity. Conclusion: The thirty-one percent IU/L, the time future