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Synopses Is Creutzfeldt-Jakob Disease Transmitted in Blood?

By Maura N. Ricketts, Neil R. Cashman, Elizabeth E. Stratton and Susie Elsaadany


Creutzfeldt-Jakob disease (CJD) has been considered infectious since the mid-1960s, but its transmissibility through the transfusion of blood or blood products is controversial. The causative agent’s novel undefined nature and resistance to standard decontamination, the absence of a screening test, and the recognition that even rare cases of transmission may be unacceptable have led to the revision of policies and procedures worldwide affecting all facets of blood product manufacturing from blood collection to transfusion. We reviewed current evidence that CJD is transmitted through blood. Creutzfeldt-Jakob disease (CJD), a rare neurodegenerative disorder, affects 0.5 to 1 persons per million population worldwide each year (1-8). CJD is a human spongiform encephalopathy; others are kuru, which is associated with ritualistic cannibalism in the Fore tribe of Papua New Guinea; Gerstmann-Sträussler-Scheinker syndrome, an inherited disorder; and fatal familial insomnia, inherited as an autosomal-dominant trait. Animal spongiform encephalopathies include scrapie, bovine spongiform encephalopathy (BSE), transmissible mink encephalopathy, and wasting disease of elk most frequently referred to as transmissible spongiform encephalopathies; other names such as prion dementias, transmissible degenerative encephalopathies, and infectious cerebral amyloidoses are also used. The classic clinical symptoms of CJD are rapidly progressive presenile dementia, myoclonus, and progressive motor dysfunction. No treatment is available, and survival averages less than 1 year (most often 2 to 6 months) (9). Diagnosis is based on symptoms, electroencephalograms, and neuropathologic tests (10,11). The pathophysiology of CJD is incompletely understood, although it is known that in person

Year: 2013
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