[A study of trientine therapy in Wilson's disease with neurological symptoms].

Abstract

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.

• Authors: M Suda, J Kubota, Y Yamaguchi, Y Fujioka, Y Saito, T Aoki
• Journal: No to hattatsu = Brain and development (No To Hattatsu) Vol. 25 Issue 5 Pg. 429-34 (Sep 1993) ISSN: 0029-0831 [Print] Japan
• PMID: 8398232 (Publication Type: Case Reports, English Abstract, Journal Article)

Chemical References

• Penicillamine
• Trientine

Topics

• Adolescent
• Adult
• Dysarthria (physiopathology)
• Dystonia (physiopathology)
• Female
• Hepatolenticular Degeneration (drug therapy, physiopathology, urine)
• Humans
• Male
• Penicillamine (adverse effects)
• Penicillin Resistance
• Tremor (physiopathology)
• Trientine (therapeutic use, urine)