[A study of trientine therapy in Wilson's disease with neurological symptoms].
D-penicillamine, an orally-administered chelating agent, is effective for Wilson's disease (WD). However 25% of WD patients showed serious adverse reactions to D-penicillamine cause this drug to be discontinued after months or years of treatment. For these cases, trientine-2HCl and trientine-4HCl, less toxic agents, are investigated. Three patients with WD, associated with neurological symptoms, were given either trientine-2HCl or trientine-4HCl. These patients had been on therapy with D-penicillamine. Severe adverse reactions had developed during the course of therapy, and D-penicillamine was discontinued, pancytopenia in case 1, nephrotic syndrome in case 2, and myasthenia gravis in case 3. Trientine-2HCl for case 1, and trientine-4HCl for cases 2 and 3 were instituted and continued. The neurological findings in all patients were extremely improved without side effects by trientine therapy. Though the chelating action on copper is weaker than that of D-penicillamine, it is efficient in improvement of the clinical neurological symptoms.
Second Department of Pediatrics, Toho University School of Medicine, Tokyo.
M
M
Suda
J
J
Kubota
Y
Y
Yamaguchi
Y
Y
Fujioka
Y
Y
Saito
T
T
Aoki
D000293:Adolescent; D000328:Adult; D004401:Dysarthria / Q000503:physiopathology; D004421:Dystonia / Q000503:physiopathology; D005260:Female; D006527:Hepatolenticular Degeneration / Q000188:drug therapy* / Q000503:physiopathology / Q000652:urine; D006801:Humans; D008297:Male; D010396:Penicillamine / Q000009:adverse effects; D010403:Penicillin Resistance; D014202:Tremor / Q000503:physiopathology; D014266:Trientine / Q000627:therapeutic use* / Q000652:urine
D010396:Penicillamine; D014266:Trientine