NR ADHK

AU Deslys,J.P.

TI [Biological aspects of Creutzfeldt-Jakob disease related to the extractive growth hormone]

OT Aspects biologiques de la maladie de Creutzfeldt-Jakob liee a l'hormone de croissance extractive

QU Pathologie Biologie 1995 Feb; 43(2): 97-103

PT journal article; review; review, tutorial

AB Today 34 young patients have been infected by the Creutzfeldt-Jakob Disease (CJD) agent in France after treatment with pituitary derived growth hormone. 974 individuals received potentially contaminated batches of hormone. The authors then investigated the genetic structure of the prion protein (PrP) gene at the codon 129 in 32 of the 34 CJD patients. Their results show that all are homozygous, 50% of the individuals of the control group being heterozygous. These data strongly suggest that it may exist a genetic susceptibility to the early development of CJD in infected individuals.

IN In Frankreich wurden 974 junge Menschen mit potentiell mit Creutzfeldt-Jakob-Erregern kontaminiertem Wachstumshormon behandelt. Bisher erkrankten 34 davon an der Creutzfeldt-Jakob-Krankheit. davon wurden 32 untersucht und erwiesen sich alle als homozygot bezüglich des Prionproteincodons 129. In einer Kontrollgruppe waren 50% heterozygot.

ZR 31

MH Adolescent; Child; Creutzfeldt-Jakob Syndrome/*etiology/genetics; *Drug Contamination; English Abstract; Growth Substances/*adverse effects; Human; Prions/genetics/*toxicity

AD Laboratoire de Neuropathologie Experimentale et Neurovirologie, DSV/DPTE/SSA, Commissariat a l'Energie Atomique, Fontenay-aux-Roses, France.

SP französisch

PO Frankreich

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