| Summary: | Wilson disease is one of the uncommon, hereditary, slowly progressing, and autosomal recessive diseases that cause motion disorders. The pathogenesis of Wilson disease is attributed to the accumulation of copper in different parts of body mainly the brain, eyes, liver, etc. due to the mutation taking place in the ATP7B gene. This disease thus leads to dysfunction of liver, neurons, brain, eyes and infertility. Thus, there is a need of proper treatment for this disease. There are numerous treatments available, including penicillamine, zinc, ammonium tetrathiomolybdate, trientine, and combination drugs; however, these treatments have a variety of side effects and poor pharmacokinetics, leading to the approval of trientine salts, of which trientine tetrahydrochloride was found to be an efficient, safe, and drug with few side effects. In this review, various aspects related to the disease and trientine salts have been summarized including the pathogenesis of Wilson disease, treatments of Wilson disease, mechanism of trientine as well as comparative pharmacokinetics with the safety and efficacy of both the salts of trientine. We have also tried to explain the preference of usage of trientine tetrahydrochloride over trientine dihydrochloride with some drugs in clinical trials. Here various factors that lead to treatment failure and consideration of alternate therapies have been discussed. Clinical trials and investigations are still going on over gene therapy and stem cells usage in the treatment of Wilson disease which can be used as an effective treatment in future.
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