Treatment of Wilson's Disease with Triethylene Tetramine Dihydrochloride

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Abstract
Wilson's desease is an autosomal recessive disorder characterized by progressive cirrhosis or neurological signs. Early detection and prompt treatment can reverse the relentless course of the disease. Treatment with D-penicillamine substantially improves the outlook for such patients unless undesirable side effects preclude its use. We report the use of triethylene tetramine dihydrochloride (Trien), a new non-sulfhydryl-chelating agent, in a girl who was unable to tolerate D-penicillamine in spite of steroid coverage. The drug has been well tolerated without side effcets for approximately 2 1/2 years. Our patient's favorable clinical response would suggest that Trien is a safe alternative agent for the treatment of Wilson's disease "hcn D-penicillamine is not tolerated.

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