NR ACXZ
AU Creange,A.; Gray,F.; Cesaro,P.; Adle-Biassette,H.; Duvoux,C.; Cherqui,D.; Bell,J.; Parchi,P.; Gambetti,P.; Degos,J.D.
TI Creutzfeldt-Jakob disease after liver transplantation
QU Annals of Neurology 1995 Aug; 38(2): 269-72
PT journal article
AB We report a 57-year-old woman who died from Creutzfeldt-Jakob disease 2 years after a liver transplantation. The liver donor had no history of neurological disease. In one albumin donor, possible Creutzfeldt-Jakob disease developed 3 years later. The patient initially had cerebellar symptoms. Neuropathology included "Kuru-type" plaques and prion protein (PrP) deposits involving the cerebellum predominantly. The patient was homozygote valine at codon 129 of the PrP gene while the liver was homozygote methionine. This observation raises the possibility of transmission of Creutzfeldt-Jakob disease by the graft itself or the associated albumin transfusions and, on a wider extent, by nonneural tissue.
IN Eine 57 Jahre alte Frau starb 2 Jahre nach einer Lebertransplatation an der Creutzfeldt-Jakob-Krankheit. Während vom Leberspender keine neurologische Erkrankung bekannt war, erkrankte ein Albuminspender anscheinend ebenfalls an der Creutzfeldt-Jakob-Krankheit. Die Frau besaß am Codon 129 des Prionproteins homozygot Valin, während die Leber homozygot Methionin am Codon 129 besaß. Die Krankheit scheint also durch eine transplantierte Leber von einem Spender mit langsamerem Verlauf, oder durch Albumin aus menschlichem Blut übertragen worden zu sein.
ZR 16
MH Case Report; Creutzfeldt-Jakob Syndrome/*etiology/pathology; Endopeptidase K; Female; Human; Iatrogenic Disease; *Liver Transplantation; Middle Age; Postoperative Complications; Prions/genetics; Serine Endopeptidases
AD Alain Créange, Françoise Gray, Pierre Cesaro, Homa Adle-Biassette, Jean-Denis Degos, Departement de Neurosciences, Centre Hospitalier Universitaire Henri Mondor, Creteil, France; Christophe Duvoux, Daniel Cherqui, Unité de Transplantation Hépatique, Centre Hospitalier Universitaire Henri Mondor, Créteil, France; Jeanne Bell, Neuropathology Laboratory, Western General Hospital, The University of Edinburgh, United Kingdom; Piero Parchi, Pierluigi Gambetti, Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, OH, USA
SP englisch
PO USA