Maintenance Zinc Therapy After Initial Penicillamine Chelation to Treat Symptomatic Hepatic Wilson's Disease in Resource Constrained Setting

Abstract

Background and Aim: Experience with zinc in treating symptomatic hepatic Wilson's disease (WD) is limited. The study was done to evaluate the efficacy of penicillamine followed by zinc in treating symptomatic hepatic Wilson's disease. Methods: We retrospectively analysed case records of 31 symptomatic hepatic WD patients in whom disease severity scores (Child's, MELD, Nazer's and New Wilson Index score) and 24-h Urinary copper were compared at 3 time points—baseline at presentation, at transition from penicillamine to zinc and at end of follow-up. Results: Of the 31 patients with symptomatic hepatic WD studied, 10 had associated neuro-psychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (in 28 patients) or due to adverse effects of penicillamine (in 3). Child's grade was A in 6 patients, B in 5 and C in 17 at presentation (baseline). Duration of initial penicillamine chelation therapy was 134 (2–320) weeks, median (range) and of subsequent zinc therapy was 363 (35–728) weeks. There was significant improvement in liver function tests and disease severity scores (Nazer's, New Wilson Index, Child's and MELD score) at transition from penicillamine to zinc compared to baseline. This improvement was maintained till end of study period. 17 patients with Child C cirrhosis at presentation were treated with penicillamine for 111 (2–320) weeks followed by zinc for 344 (41–652) weeks, of whom 15 had significant improvement in liver function and disease severity scores until end of follow-up. 3 of 31 study patients died at 284, 112 and 437 weeks. No patient underwent liver transplantation. Conclusions: Penicillamine followed by zinc maybe safe and effective treatment in resource constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. Our data also suggests that some patients with decompensated cirrhosis due to Wilson's disease may be managed with medical treatment, avoiding liver transplantation. The authors have none to declare.