NR AQVI
AU Peden,A.H.; Head,M.W.; Ritchie,D.L.; Bell,J.E.; Ironside,J.W.
TI Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient
QU Lancet 2004 Aug 7; 364(9433): 527-9
KI Lancet. 2004 Aug 7;364(9433):477-9. PMID: 15302172
PT journal article
AB We report a case of preclinical variant Creutzfeldt-Jakob disease (vCJD) in a patient who died from a non-neurological disorder 5 years after receiving a blood transfusion from a donor who subsequently developed vCJD. Protease-resistant prion protein (PrPres) was detected by western blot, paraffin-embedded tissue blot, and immunohistochemistry in the spleen, but not in the brain. Immunohistochemistry for prion protein was also positive in a cervical lymph node. The patient was a heterozygote at codon 129 of PRNP, suggesting that susceptibility to vCJD infection is not confined to the methionine homozygous PRNP genotype. These findings have major implications for future estimates and surveillance of vCJD in the UK.
MH Blood Transfusion/*adverse effects; Codon/genetics; Creutzfeldt-Jakob Syndrome/diagnosis/metabolism/*transmission; Heterozygote; Human; *PrPsc Proteins/blood/*genetics/isolation & purification; Support, Non-U.S. Gov't
AD Alexander H. Peden (a.peden@ed.ac.uk), Mark W. Head (m.w.head@ed.ac.uk), Diane L. Ritchie (diane.ritchie@ed.ac.uk), Jeanne E Bell, James W. Ironside (james.ironside@ed.ac.uk), National Creutzfeldt-Jakob Disease Surveillance Unit, Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, UK
SP englisch
PO England