NR ABUB
AU Brown,P.; Preece,M.; Brandel,J.P.; Sato,T.; McShane,L.; Zerr,I.; Fletcher,A.; Will,R.G.; Pocchiari,M.; Cashman,N.R.; Huillard d'Aignaux,J.N.; Cervenakova,L.; Fradkin,J.; Schonberger,L.B.; Collins,S.J.
TI Iatrogenic Creutzfeldt-Jakob disease at the millennium
QU Neurology 2000 Oct 24; 55(8): 1075-81
KI Neurology. 2001 Apr 10;56(7):987. PMID: 11294952
PT journal article; review; review, tutorial
AB The causes and geographic distribution of 267 cases of iatrogenic Creutzfeldt-Jakob disease (CJD) are here updated at the millennium. Small numbers of still-occurring cases result from disease onsets after longer and longer incubation periods following infection by cadaveric human growth hormone or dura mater grafts manufactured and distributed before the mid-1980s. The proportion of recipients acquiring CJD from growth hormone varies from 0.3 to 4.4% in different countries, and acquisition from dura mater varies between 0.02 and 0.05% in Japan (where most cases occurred). Incubation periods can extend up to 30 years, and cerebellar onsets predominate in both hormone and graft recipients (in whom the site of graft placement had no effect on the clinical presentation). Homozygosity at codon 129 of the PRNP gene is over-represented in both forms of disease; it has no effect on the incubation period of graft recipients, but may promote shorter incubation periods in hormone cases. Knowledge about potential high-risk sources of contamination gained during the last quarter century, and the implementation of methods to circumvent them, should minimize the potential for iatrogenic contributions to the current spectrum of CJD.
IN Bis zum Juli 2000 wurden schon 114 Dura mater und 139 Wachstumshormonfälle gezählt, wobei bei Dura mater Japan mit 67 und bei Wachstumshormonen Frankreich mit 74 Fällen dominierten
ZR 19
MH Creutzfeldt-Jakob Syndrome/*epidemiology/*genetics; Human; Iatrogenic Disease/*epidemiology; Risk Factors
AD Paul Brown (brownp@ninds.nih.gov), Laboratory of CNS Studies, NINDS, NIH, Bethesda, MD 20892, USA
SP englisch
PO USA
EA pdf-Datei und HTML-Version