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Wilson’s disease (WD) is an inherited metabolic disorder which affects biliary copper excretion and synthesis as well as functional maturation of protein ceruloplasmin, leading to excessive copper accumulation in various organs, particularly the liver and brain.1 The mainstay of treatment includes chelating agents such as D-penicillamine (DP) and trientine, and zinc which reduces intestinal copper absorption. Although DP is the preferred first-line therapy, approximately 10–30% of patients experience significant adverse effects that may necessitate drug discontinuation.2 Trientine has a similar mechanism of action but has a lower incidence of side effects, making it a suitable alternative for patients who are intolerant to DP.3 In this study, we present a cohort of paediatric WD patients from a single tertiary care centre who required trientine for disease management. Their clinical characteristics, reasons for switching from penicillamine to trientine, and treatment outcomes are discussed.The medical records of 55 children (<16 years age) diagnosed with WD in the last 20 years at our center were reviewed. Seven patients (12.7%) received trientine at some point during their treatment course. A descriptive analysis of the clinical data of these patients was performed.Among the seven patients, three (42.9%) were detected from family screening, two (28.6%) of them had an incidental finding of liver enzymes elevation, one had co-existing autoimmune haemolytic anaemia and one presented with tremors who later developed systemic lupus erythematosus (SLE). The median age at WD diagnosis was 10 years (range 4–13 years). Six patients were initially started on DP. Indications for switching to trientine were penicillamine-induced hepatitis (n=2), proteinuria (n=1), Raynaud’s phenomenon (n=1), poor treatment response (n=2). The patient who presented with tremors was treated with zinc sulfate and later developed SLE was switched to trientine directly in view of worsening liver function. The median duration between WD diagnosis and trientine initiation was 2.9 years (range 1.1–8.3 years). At the most recent follow-up, four patients had transitioned to adult care while stable on trientine, and three remained under paediatric follow-up with mild transaminitis but good overall clinical stability. No patient discontinued trientine due to adverse effects.DP can cause early-onset (1-3 weeks) and late-onset adverse effects. Early hypersensitivity reactions are characterized by fever, cutaneous eruptions, cytopenias, and proteinuria. Much rarer late-onset (years or decades) reactions which are potentially fatal include drug-induced lupus, nephrotic syndrome leading to renal failure, severe thrombocytopenia, or aplastic anemia.4 All our patients experienced late-onset adverse effects, with Raynaud’s phenomenon being a particularly rare occurrence. In the event of a severe reaction, DP should be discontinued and patient switched to an alternative therapy. Although hypersensitivity reactions and pancytopenia can occur with trientine as well, however these were not seen in our cohort.To conclude, trientine is effective and well tolerated in children when used as second line for therapy for Wilson’s disease.References Leung M, Wu Lanzafame J, Medici V. Switching pharmacological treatment in wilson disease: case report and recommendations. J Invest Med High Impact Case Rep 2020 Jan;8:2324709619896876.Socha P, Jańczyk W, Zanetto A, Burra P, Czlonkowska A, Debray D, et al. EASL-ERN clinical practice guidelines on Wilson’s disease. J Hepatol 2025 Apr 1;82(4):690–728.Nagral A, Sarma MS, Matthai J, Kukkle PL, Devarbhavi H, Sinha S, et al. Wilson’s disease: clinical practice guidelines of the Indian national association for study of the liver, the Indian society of pediatric gastroenterology, hepatology and nutrition, and the movement disorders society of India. J Clin Exp Hepatol 2019 Jan;9(1):74–98.Aggarwal A, Bhatt M. Advances in treatment of Wilson disease. Tremor Hyperkinetic Movements 2018 Feb 28;8(0):525.