Hundred cases of hepatolenticular degeneration: Croatian experience (CROSBI ID 480845)
Prilog sa skupa u zborniku | sažetak izlaganja sa skupa | međunarodna recenzija
Podaci o odgovornosti
Relja, Maja ; Petrović, Zrinka
engleski
Hundred cases of hepatolenticular degeneration: Croatian experience
Hepatolenticular degeneration or Wilson's disease (WD), is an autosomal recessive disorder of copper metabolism. The systemic symptoms of WD are secondary to excessive accumulation of copper in the brain, liver, cornea and other organs. WD is one of the very few chronic neurological disease for which effective and specific therapy is available. But usually a misdiagnosis apperas early in the course of the disease. The aim of present study was to report the neurological and medical evaluation of 95 patients with WD. Most patients at diagnosis had neurologic form of the illness (95%). Most of our patients were treated with d-penicillamine and/or zinc salts. In conclusion, we can say thet the diagnosis can usaually be made provided that it is suspected.
Hepatolenticular degeneration; copper; brain; liver; d-penicillamine; zinc salts
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Podaci o prilogu
33-33-x.
2000.
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objavljeno
Podaci o matičnoj publikaciji
Symposium on movement disorders "The Alpine Basal Ganglia Club " - Proceedings
Pirtošek, Zvezdan
Ljubljana: University Medical Centre, Ljubljana
Podaci o skupu
"The Alpine Basal Ganglia Club" - Symposium on Movement Disorders
pozvano predavanje
13.09.2000-14.09.2000
Ljubljana, Slovenija