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A rare disease put an end to the life of a Slovenian



LJUBLJANA – One person died of Creutzfeldt-Jakob disease (CJB) in Slovenia this year (May data). Many people associate this disease with mad cow disease, but when it comes to CJB, it is not necessary to think about this form exactly. The National Institute of Public Health (NIJZ) taught us that there are four forms of CJB:

– up to 80 percent of all cases are sporadic CJB form (sCJB)which arises for an unexplained reason;
familial or genetic CJB (dCJB) is a very rare form that results from a gene mutation and is a hereditary autosomal dominant;
CJB variant (vCJB) is caused by the introduction of prions into the body by ingestion of mechanically-treated beef from diseased animals for bovine spongiform encephalopathy (BSE) or by transfusion of the patient's blood;
Iatrogenic CJB Form (iCJB) is due to the accidental transfer of prions in health care to the treatment of growth and gonadotropic hormones derived from corpus pituitary carcasses after transplantation of the cornea or dura, resulting from death by CJB through contaminated surgical materials during neurosurgical procedures.

According to the NHRI, in the past five years (from early 2015 to May 2019), the autopsy confirmed 26 cases of sporadic CJB in death suspected of the disease, of which one case of sCJB has been confirmed to date. "We have not yet discovered the variants or iatrogenic forms of CJB in Slovenia."

It is important to note that CJB is transmitted through contaminated food of animal origin and from person to person, as well as' diagnostic and therapeutic procedures, namely treatment with growth hormone, less commonly gonadotropic hormone, corneal transplantation and solid brain transplantation, neurosurgical instruments and in vCJB by blood transfusion. "


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