NR AOBB
AU Billette de Villemeur,T.B.
TI [Prion diseases]
OT Maladies a prion
QU Archives de Pediatrie 2003 Jan 1; 10(1): 54-7
PT journal article; review; review, tutorial
AB Prion is an ubiquitous membrane protein in mammals, which is mainly synthesized in central nervous system. Prion diseases are the result of an accumulation of prions having acquired a resistance to the physiological degradation and an infectious capacity. Human prion diseases are very rare diseases including sporadic Creutzfeldt-Jakob disease (the most frequent form manifesting as a presenile dementia), familial transmissible spongiform encephalopathies and two juvenile transmissible forms: iatrogenic Creutzfeldt-Jakob secondary to treatment with human extractive growth hormone and variant Creutzfeldt-Jakob disease resulting from bovine spongiform encephalopathy food transmission. Knowledge of the underlying prion biology has led to preventive measures which offer today a reasonable guarantee against the juvenile forms.
ZR 6
MH Diagnosis, Differential; English Abstract; Human; Incidence; *Prion Diseases/drug therapy/pathology/transmission; Prognosis
AD Service de neuropediatrie, hopital Trousseau, 26, avenue Docteur-A.-Netter, 75012 Paris, France. thierry.billette@trs.ap-hop-paris.fr
SP französisch
PO Frankreich