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N Delasnerie-Laupretre

Publications and source records attributed to N Delasnerie-Laupretre.

14 recordsLinked to original sources
PubMed2005

Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada.

BACKGROUND: An international study of the epidemiologic characteristics of Creutzfeldt-Jakob disease (CJD) was established in 1993 and included national registries in France, Germany, Italy, the Netherlands, Slovakia, and the United Kingdom. In 1997, the study was extended to Australia, Austria, Canada, Spain, and Switzerland. METHODS: Data were pooled from all participating countries for the years 1993 to 2002 and included deaths from definite or probable CJD of all etiologic subtypes. RESULTS: Four thousand four hundred forty-one cases were available for analysis and included 3,720 cases of sporadic CJD, 455 genetic cases, 138 iatrogenic cases, and 128 variant cases. The overall annual mortality rate between 1999 and 2002 was 1.67 per million for all cases and 1.39 per million for sporadic CJD. Mortality rates were similar in all countries. There was heterogeneity in the distribution of cases by etiologic subtype with an excess of genetic cases in Italy and Slovakia, of iatrogenic cases in France and the UK, and of variant CJD in the UK. CONCLUSIONS: This study has established overall epidemiologic characteristics for Creutzfeldt-Jakob disease (CJD) of all types in a multinational population-based study. Intercountry comparisons did not suggest any relative change in the characteristics of sporadic CJD in the United Kingdom, and the evidence in this study does not suggest the occurrence of a novel form of human bovine spongiform encephalopathy infection other than variant CJD. However, this remains a possibility, and countries currently unaffected by variant CJD may yet have cases.

Adolescent↗
PubMed2004

Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies.

A collaborative study of human transmissible spongiform encephalopathies has been 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 predictors of survival in sporadic (n = 2304), iatrogenic (n = 106) and variant Creutzfeldt-Jakob disease (n = 86) and in cases associated with mutations of the prion protein gene (n = 278), including Gerstmann-Sträussler-Scheinker syndrome (n = 24) and fatal familial insomnia (n = 41). Overall survival for each disease type was assessed by the Kaplan-Meier method and the multivariate analyses by the Cox proportional hazards model. In sporadic disease, longer survival was correlated with younger 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 therapy, will be an important starting point for the assessment of potential therapeutic agents in the future.

Adolescent↗
PubMed1998

Descriptive epidemiology of Creutzfeldt-Jakob disease in six European countries, 1993-1995. EU Collaborative Study Group for CJD.

After the occurrence of bovine spongiform encephalopathy (BSE), there has been concern that transmission of BSE to the human population might result in a change in the epidemiological characteristics of Creutzfeldt-Jakob disease (CJD). A collaborative study of CJD in the European Union was performed from 1993 to 1995, to compare data from national registries for CJD in France, Germany, Italy, The Netherlands, Slovakia, and the United Kingdom. Five hundred seventy-five patients with definite or probable CJD died in the study period with an overall annual mortality rate of 0.71 cases per million. The incidence rates for CJD were similar in all participating countries despite variations in postmortem rates, and age-specific incidence rates were also relatively consistent, with the exception of an increased incidence of CJD in patients younger than 39 years of age in the United Kingdom. In relation to etiological subtypes of CJD, 87% of cases were sporadic, 8% genetic, and 5% iatrogenic. Genetic forms of CJD comprised 80% of all cases in Slovakia, and iatrogenic forms of CJD occurred most frequently in France and the United Kingdom. The statistical data reported here do not provide evidence of a causal link between BSE and CJD in Europe as a whole. However, the study has established baseline epidemiological parameters for CJD in participating European countries, which may be important in the assessment of any future change in the characteristics of CJD as a result of the epidemic of BSE.

Adult↗
PubMed1995

[Epidemiology of Creutzfeldt-Jakob disease].

Creutzfeldt-Jakob disease is a rare disease (incidence < 1 case per million inhabitants), reported in all continents. Certain characteristics are constant from one country to another: mean age of onset: 65 years, duration of the disease: about 6 months, sex ratio close to 1 and approximately 10% of familial cases. The geographical sites of this disease identified in Chile, Israel and Czechoslovakia suggest certain genetic and/or environmental risk factors. The principal environmental factor implicated is scrapie in sheep, especially in these regions. Genetic studies have demonstrated the presence of apparently causal mutations of the PrP gene and a high risk of CJD associated with certain normal polymorphisms of this gene (codon 129). Certain grafts (dura mater, cornea) and treatment by extracted growth hormone are responsible for iatrogenic transmission. Multicentre epidemiological studies recruiting a large number of cases are needed to define the risk factors of this disease.

Creutzfeldt-Jakob Syndrome↗
PubMed1993

Sleep, snoring and smoking in high school students.

The relationship between snoring, sleep and smoking was investigated in young students from a survey by self-administered questionnaire on sleep habits, conducted in high schools in the educational district of Lyons (France). Our sample consisted of 11,417 boys and 13,265 girls between 15 and 20 years of age. We found that 28.4% of girls and 38.8% of boys reported that they snored. In both sexes, snoring was associated with short (<7 h) and long (>10 h) sleep length, nocturnal awakenings and daytime sleepiness. Smoking was a risk factor for snoring. Multiple logistic regression showed that the odds-ratio for habitual snoring increased when tobacco consumption increased from a 1.30 (moderate smokers) to 2.26 (heavy smokers) in girls and from 1.38 to 2.09 in boys. This dose-effect relationship between smoking and snoring suggests that a substantial proportion of snoring cases is attributable to tobacco consumption in young students.

Journal Article↗
PubMed1986

Study of endemic scrapie in a flock of "Ile de France" sheep.

An "Ile de France" flock was studied over a period of 5 years. The authors found similar results concerning incidence and age of onset of scrapie to those found in the English literature. A close relationship was established between the age of the dam at parturition and the age of the progeny at onset of scrapie. In the later years of our study, scrapie was detected in younger animals than in the earlier years, suggesting a phenomenon of genetic "anticipation". Increased fecundity in this endemically affected flock might represent a form of selection which favorises the occurrence and maintenance of the disease within the flock.

Animals↗
PubMed1986

[Familial predisposition to multiple sclerosis].

Seven families were studied to evaluate the influence of genetic factors in MS and its transmissibility. The disease was associated with some B7 and/or DR2 alleles in relation to the susceptibility gene in all but one case. This association was always transmissible conjointly. The carrier haplotype conditions disease manifestations and its prognosis, favorable in this series, but it is not sufficient to promote the onset of the disease. This requires a triggering factor with sometimes disorders affecting 3 generations as demonstrated by the analysis of one family. Therefore there is not a genic heredity but a predisposition heredity. Possible associations of MS with other morbid states such as insulin-dependent diabetes must be considered when counselling families.

Adolescent↗
PubMed1982

Cerebro spinal fluid C2 and HLA system in multiple sclerosis.

C3 hemolytic activity has been measured in serum and C.S.F. of 67 multiple sclerosis (M.S.) patients classified in three evolutionary groups according to the index of progression of the disease and tested in HLA-A, B, C, DR and Bf. The median level of serum C2 is normal whether it is of the evolution or the HLA type. The average level of C.S.F. C2 is the same in the evolutionary groups and is low in patients bearing B 18 allele or B 18, DR2 haplotype. It seems that the median level of C.S.F. C2 is linked to the HLA types associated to the susceptibility gene(s) of the disease.

Alleles↗
PubMed1981

Serum and cerebrospinal fluid C2 in multiple sclerosis.

C2 hemolytic activity was quantitated in the serum and cerebrospinal fluid of 46 MS patients studied twice at a 1-year interval. whereas serum C2 levels were found within the normal range, CSF C2 values were decreased (55 and 59% of normal) in patients with severe active disease. In contrast CSF C2 levels were normal in patients with stable or recently improved condition. CSF C2 fluctuations in individuals were found to closely parallel changes in the clinical course.

Complement C2↗