NR ABJF

AU Billette de Villemeur,T.B.; Gourmelen,M.; Beauvais,P.; Rodriguez,D.; Vaudour,G.; Deslys,J.P.; Dormont,D.; Richard,P.; Richardet,J.M.

TI [Creutzfeldt-Jakob disease in 4 children treated with growth hormone]

OT Maladie de Creutzfeldt-Jakob chez quatre enfants traites par hormone de croissance

QU Revue Neurologique 1992; 148(5): 328-34

PT journal article; review; review of reported cases

AB Creutzfeldt-Jakob disease was diagnosed in four growth hormone recipients at the age of 10, 11, 18 and 19 years. To our knowledge, the two first cases are the first instances of Creutzfeldt-Jakob disease recorded in children. Three of them were still being treated with synthetic hormone at the onset of the disease. Neurological disorders: ataxia and diplopia, appeared first, dementia and myoclonus appeared later. Eighteen cases of Creutzfeldt-Jakob disease in growth hormone recipients are now recorded, and the present risk of Creutzfeldt-Jakob disease in pituitary growth recipients is estimated to be 1/300. Because of the long incubation period, new cases are to be feared. Other causes of iatrogenic Creutzfeldt-Jakob disease are reviewed. These facts incite to consider carefully using products of human origin in human therapy. The interactions between growth hormone, prion and host's genomic make-up are still not clear.

IN In Frankreich erkrankten 4 Empfänger von Wachstumshormonen mit 10, 11, 18 bzw. 19 Jahren an der Creutzfeldt-Jakob-Krankheit.

ZR 48

MH Adolescent; Case Report; Child; Creutzfeldt-Jakob Syndrome/diagnosis/*etiology/genetics; Drug Contamination; Electroencephalography; English Abstract; Female; Growth Disorders/therapy; Growth Hormone/*adverse effects/therapeutic use; Human; *Iatrogenic Disease; Male; Prions/pathogenicity; Time Factors

AD Service de Pediatrie, Hopital Trousseau, Paris.

SP französisch

PO Frankreich

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