[New possibilities in the treatment of Wilson-Konovalov disease (hepatolenticular degeneration)].
The favourable therapeutic effect of a new copper chelator--trientine (triethylenetetramine dihydrochloride) is reported. The drug was applied to 3 patients, 18 to 25 years of age, with Wilson-Konovalov disease (hepatolenticular degeneration) who in the course of chronic D-penicillamine treatment developed drug intolerance with signs of nephrotoxicity (in one patient) and myelotoxicity with leucopenia and thrombocytopenia (in two patients). The treatment was carried out orally with optimal daily dose of 1.8 gr trientine which led to a 4-6 times increase of the 24 hour urine copper excretion. The drug is well tolerated and no side effects of allergic reactions have been registered up to now in the patients treated.
V
V
Kolarski
D000293:Adolescent; D000328:Adult; D004341:Drug Evaluation; D004361:Drug Tolerance; D005260:Female; D006527:Hepatolenticular Degeneration / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D010396:Penicillamine / Q000008:administration & dosage / Q000009:adverse effects; D014266:Trientine / Q000008:administration & dosage / Q000009:adverse effects
D010396:Penicillamine; D014266:Trientine
Novi vŭzmozhnosti za lechenie na bolestta na Wilson-Konovalov (khepatolentikularna degeneratsiia).