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Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease.

The spongiform encephalopathy Creutzfeldt-Jakob disease (CJD) has been transmitted to man via administration of growth hormone and gonadotropin extracted from large pooled batches of human cadaveric pituitary glands. In the UK, 1908 individuals were exposed to potentially contaminated growth hormone, of whom 6 have so far manifested CJD. Examination of the prion protein genes of all these cases and of a single case of gonadotropin-related CJD showed that 4 had the uncommon valine 129 homozygous genotype indicating genetic susceptibility to prion infection. Such genetic susceptibility may be important in the aetiology of sporadic CJD disease.

Alleles↗

[Prion, from crazy cows to iatrogenic Creutzfeldt-Jakob disease. Which risk in laboratory or in hospital?].

The long latency time, without any characteristic clinical sign, of transmissible degenerative encephalopathies, the transmissibility of the called "prion" infectious agent, associated with its exceptional resistance to normal inactivation methods, are resulting in accidental transmissions, both human (Creutzfeldt-Jakob disease), and animal (bovine spongiform encephalopathy). Among data about physical and chemical inactivation methods tested, we retain, to avoid professional or iatrogenic transmissions in the laboratory or in hospital, steam autoclaving and sodium hypochlorite or hydroxide treatment. But inactivation shall not be performed using the current processes as regarding parameters such as temperature, concentration and duration of exposure.

Animals↗