[Clinico-diagnostic, therapeutic and prognostic studies of Wilson-Konovalov disease. The results of 25 years' experience].
The author's 25 years experience in clinical, diagnostic, therapeutic and prognostic study of 24 patients with Wilson-Konovalov's disease (hepatilenticular degeneration) is presented. The great diagnostic importance of copper metabolism (plasma copper level, urine copper), plasma ceruloplasmin concentration, histochemical and quantitative determination of copper in liver cells (after a liver biopsy) is pointed out. These are of special importance in young patients (under 25 years) and in children with chronic parenchymal liver disease. In 1/4 of the patients the early stage of the disease resembled chronic active hepatitis (predominantly liver type of the disease). The basic therapeutic drug is D-penicillamine hydrochloride and when it is not tolerated the drug Trien may be used. Very good therapeutic results and considerable improvement of prognosis, social and working rehabilitation of the patients have been achieved. The preconditions for good therapeutic and prognostic results are the early discovery, exact diagnosis and prolonged purposeful pathogenetic treatment (chelatory and hepatoprotective). Zinc sulfate and potassium sulfide are only secondary therapeutic means in patients with hepatolenticular degeneration.
Kolarski
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D000293:Adolescent; D000328:Adult; D002648:Child; D002908:Chronic Disease; D003131:Combined Modality Therapy / Q000379:methods; D003937:Diagnosis, Differential; D005260:Female; D006521:Hepatitis, Chronic / Q000175:diagnosis; D006527:Hepatolenticular Degeneration / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D006801:Humans; D008297:Male; D011379:Prognosis
Klinikodiagnostichni, lechebni i prognostichni prouchvaniia pri bolestta na Wilson-Konovalov. Izvodi ot 25-godishen opit.