NR AKZZ
AU Sol-Caubel,I.; Castela,F.; Brousse,V.; Faivre,L.; Guigonis,V.; Thiriez,G.; Billette de Villemeur,T.B.
TI [Prion diseases in pediatrics]
OT Les maladies a prions en pediatrie
QU Archives de Pediatrie 1999 Mar; 6(3): 293-301
PT journal article; review; review, tutorial
AB Prion diseases are rare neurologic affections with a poor prognosis, occurring in both humans and animals. Creutzfeldt-Jakob disease (CJD) secondary to human extracted growth hormone treatment is the most frequent condition in pediatrics. In 1994, a new type of CJD (variant CJD) was described in young adults in the United Kingdom, only 10 years after the bovine spongiform encephalopathy epidemic, with recent works showing a direct relationship between the bovine epidemic and the human cases. An accumulation of a single protein called the prion protein (PrP) has been discovered in the brain in all of these cases, animal and human, leading to the hypothesis that a new infectious agent could proceed without any nuclear acid information; another hypothesis is that of a still unknown viral agent. The PRNP gene encoding for this PrP protein is well described: some mutations and a polymorphism in the 129th codon have been shown to be implicated in many cases of CJD. PrP is a ubiquitous protein, with yet unknown physiological function. There are still many questions to be answered: shall we expect new pediatric cases of variant CJD? Assuming that animal-human contamination is related to alimentation, are there other ways of contamination.
ZR 36
MH Adult; Animal; Cattle; Child; English Abstract; Food Microbiology; Human; Mutation/genetics; Pediatrics; Polymorphism (Genetics)/genetics; *Prion Diseases/classification/etiology/transmission; Prions/genetics
AD Service de neuropediatrie, hopital Armand-Trousseau, Paris, France.
SP französisch
PO Frankreich