论文部分内容阅读
A collaborative study of human transmissible spongiform encephalopathies has b een carried out from 1993 to 2000 and includes data from 10 national registries, the majority in Western Europe. In this study, we present analyses of predictor s of survival in sporadic (n = 2304), iatrogenic (n = 106) and variant Creutzfel dt Jakob disease (n = 86) and in cases associated with mutations of the prion p rotein gene (n=278), including Gerstmann Strussler Scheinker syndrome (n = 24) and fat al familial insomnia (n = 41). Overall survival for each disease type was assess ed by the .Kaplan Meier method and the multivariate analyses by the Cox proport ional hazards model. In sporadic disease, longer survival was correlated with yo unger age at onset of illness, female gender, codon 129 heterozygosity, presence of CSF 14-3-3 protein and type 2a prion protein type. The ability to predict survival based on patient covariates is important for diagnosis and counselling, and the characterization of the survival distributions, in the absence of thera py, will be an important starting point for the assessment of potential therapeu tic agents in the future.
A collaborative study of human transmissible spongiform encephalopathies has b een carried out from 1993 to 2000 and includes data from 10 national registries, the majority in Western Europe. In this study, we present analyzes of predictor s of survival in sporadic (n = 2304) , iatrogenic (n = 106) and variant Creutzfel dt Jakob disease (n = 86) and in cases associated with mutations of the prion p rotein gene (n = 278), including Gerstmann Ströussler Scheinker syndrome al familial insomnia (n = 41). Overall survival for each disease type was assessed ed by the. Kaplan Meier method and the multivariate analyzes by the Cox proportional models. In sporadic disease, longer survival was correlated with yo unger age at onset of illness, female gender, codon 129 heterozygosity, presence of CSF 14-3-3 protein and type 2a prion protein type. The ability to predict survival based on patient covariates is important for diagnosis and counseling, and the characterization of the survival distributions, in the absence of the py py, will be an important starting point for the assessment of potential therapeu tic agents in the future.