Author/Editor     Beović, Bojana
Title     Creutzfeldt-Jacobsova bolezen
Translated title     Creutzfeldt-Jacobs disease
Type     članek
Source     Zdrav Vars
Vol. and No.     Letnik 40, št. 11-12
Publication year     2001
Volume     str. 380-3
Language     slo
Abstract     Prion diseases represent a novel group of infectious diseases. They are most probably caused by prions, proteinaceous particles, capable of their own replication. Prion diseases may occur spontaneously, may be inherited, or linked to a known source of infection. Some of them are known for centuries, but in the last two decades appearance of novel prion diseases has been witnessed. New prion diseases seem to be linked to food supplements containing animal proteins. In the eighties, bovine spongiform encephalopathy developed in the cattle and was followed by a similar disease in some other domestic animals and animals in the captivity In the nineties a novel variant of Creutzfeldt Jacob (vCJD) disease was described, differing from the sporadic fprrn of CJD in age of the patients, duration of illness, symptoms and signs, electroencephalographic features and neuropathology. Results of many stuplies support the hypothesis of BSE being the cause of vCJD. The number of cases of vCJD is still growing and the prognosis of the epidemics is unclear. In the last few years many studies are underway looking after appropriate diagnostic methods and treatment of prion diseases, but new concerns have risen about secondary transmission of the vCJD.
Summary     Prionske bolezni so nova skupina nalezljivih bolezni. Povzročajo jih prioni, najverjetneje beljakovinski delci, ki so sposobni razmnoževanja. Prionske bolezni se pojavljajo sporadično, so dedne ali pa so posledica znane okužbe. Nekatere med njimi so znane že stoletja, v zadnjih dveh desetletjih pa smo priča pojavu novih prionskih bolezni, ki so najverjetenje povezane med seboj in so posledica hranjenja govedi s krmo živalskega izvora. Pri govedu se je v osemdesetih letih pojavila bovina spongiformna encefalopatija (BSE) epizootičnih razsežnosti. Sledili so posamezni primeri pri drugih domačih živalih in živalih v ujetništvu. Sredi devetdesetih let smo bili priča pojavu nove prionske bolezni tudi pri človeku. Variantna Creutzfeldt Jacobova (vCJB) bolezen se razllkuje od dosedaj znanih oblik po starosti obolelih, poteku, klinični sliki, elektronecefalografskih in nevropatoloških spremembah. Številni dokazi povezujejo vCJB z BSE. Število primerov vCJB se v zadnjih letih povečuje, napovedi o obsegu epidemije pri človeku so še vedno črnoglede. V zadnjem času se hitro razvija diagnosticiranje v CJB, raziskave potekajo tudi na področju zdravljenja. Skrb zbuja možnost prenosa bolezni s človeka na človeka.
Descriptors     CREUTZFELDT-JAKOB SYNDROME
PRION DISEASES
ENCEPHALOPATHY, BOVINE SPONGIFORM