Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Penicillamine is the drug of choice for the treatment of Wilson's disease, whatever the stage of the illness. Toxic manifestations may preclude the use of this life-saving drug in some patients and discontinuation of penicillamine therapy usually leads to death. We report our experience with Trientine in seven patients, aged 13 to 33 years, with Wilson's disease who developed toxic manifestations with penicillamine that required discontinuation of therapy. These include two with nephrosis, one with neutropenia, two with thrombocytopenia, and one each with a SLE-like and a Henoch-Schonlein-like syndrome. The patients were treated for periods from 6 weeks to 16 years with a dose of 0.5 to 2 g/day. Trientine proved to be an effective alternative copper chelating agent in the treatment of Wilson's disease in patients with penicillamine-induced neutropenia, thrombocytopenia, SLE, and nephrosis. No serious untoward side effects were noted.
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PMID:Treatment of Wilson's disease with triethylene tetramine hydrochloride (Trientine). 232 83

Twenty patients with Wilson's disease in whom severe penicillamine intolerance developed have been managed with the orally active chelating agent trientine dihydrochloride (trien). The stage of illness of the patients ranged from the presymptomatic through severe neurological or hepatic disease to the "decoppered" postsymptomatic cases. Trien has proved to be a safe and highly effective treatment both for reversing symptoms and for maintaining patients previously successfully decoppered with penicillamine. There has been evidence of depletion of the body stores of copper by trien coinciding with the clinical improvement. In most of the patients the toxic symptoms which forced a change of therapy were reversed on trien therapy; however, elastosis perforans did not seem to benefit, and two patients with penicillamine-induced systemic lupus erythematosus were not helped by the change. No other toxic signs or symptoms were observed. There was no evidence of teratogenicity either in animals or in the six patients who became pregnant while taking trien; all six infants have developed normally. Trien is a satisfactory alternative therapy for Wilson's disease; its usefulness, is however, severely limited by the lack of a product license.
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PMID:Treatment of Wilson's disease with trientine (triethylene tetramine) dihydrochloride. 612 64