Treatment of Wilson's disease with triethylene tetramine dihydrochloride. A case report.
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.
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*
D005029:Ethylenediamines; D003300:Copper; D010396:Penicillamine; D014266:Trientine