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...

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Bibliographic Details
Published in:The Lancet (British edition) 1991-06, Vol.337 (8755), p.1441-1442
Main Authors: Collinge, J., Palmer, M.S., Dryden, A.J.
Format: Article
Language:English
Subjects:
Alleles
Creutzfeldt-Jakob disease
Creutzfeldt-Jakob Syndrome - genetics
Creutzfeldt-Jakob Syndrome - transmission
Disease Susceptibility
Disease transmission
DNA - analysis
Encephalitis
Genetics
Gonadotropins, Pituitary - adverse effects
Growth Hormone - adverse effects
Homozygote
Hormones
Humans
Iatrogenic Disease
Medical research
Polymerase Chain Reaction
PrPSc Proteins
Valine - genetics
Viral Proteins - genetics
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Summary: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.
ISSN:0140-6736
1474-547X
DOI:10.1016/0140-6736(91)93128-V