title: Treatment of Wilson's disease with triethylene tetramine dihydrochloride. A case report.
abstract: Wilson's disease 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 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 effects 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 when D-penicillamine is not tolerated.
mesh_terms: D000293:Adolescent; D003300:Copper / Q000378:metabolism; D005029:Ethylenediamines / Q000627:therapeutic use*; D005260:Female; D006527:Hepatolenticular Degeneration / Q000188:drug therapy*; D006801:Humans; D010396:Penicillamine / Q000009:adverse effects / Q000627:therapeutic use; D014266:Trientine / Q000494:pharmacology / Q000627:therapeutic use*
chemical_list: D005029:Ethylenediamines; D003300:Copper; D010396:Penicillamine; D014266:Trientine