Wilson’s disease (WD) is an autosomal recessive disease caused by a defect in the ATP7B gene, which encodes a metal P-type ATPase responsible for trans-membrane copper transport within hepatocytes.¹ Standard treatment of Wilson’s disease includes copper chelators such as penicillamine and Zinc. Penicillamine is used first line, however 30% of patients discontinue therapy due to adverse effects.² Trientine is indicated in WD patients who are intolerant to penicillamine.

We undertook a retrospective review of our centre’s WD patients aiming to describe use of trientine. 25 WD patients were identified. 16, 2 and 1 patients were on pencillamine , zinc and zinc + penicillamine treatment respectively. 3/25 were on trientine. 3/25 who presented with acute liver failure underwent liver transplantation.

Trientine group All three children presented with deranged liver function tests (LFTs). All were started on Penicillamine initially. Reasons for switching were drug-induced hepatitis, inadequate response to penicillamine and proteinuria. No patients reported side effects from trientine.

Characteristics of patients treated with trientine are shown in table 1 (see table 1).

Patient 1 was also noted to be non-compliant with gluten free diet for coeliac disease which may have contributed to their raised ALT.

In conclusion, trientine is effective as Penicillamine in treating Wilson’s disease with good tolerability.

References

1. Schilsky M, To UK; Wilson’s Disease BMJ Best Practice Oct 2022. Available at https://bestpractice.bmj.com/topics/en-gb/427

2. Woimant F, Debray D, Morvan E, Obadia MA, Poujois A. Efficacy and safety of two salts of trientine in the treatment of wilson’s disease. Journal of Clinical Medicine 2022;11(14):3975.