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91.
Recent increasing incidence and prevalence rates of Huntingtons disease (HD), a fatal neurodegenerative disorder, prompted us to investigate the epidemiological dynamic of HD in Austria during the period 1970–2001. Our study demonstrated a stable HD mortality rate throughout Austria of 0.125 per 100,000 individuals during the investigated period. The median age at death from HD was 56.5 years for both sexes and remained stable during the entire period observed, indicating no prolonged survival. An above average mortality rate from HD was observed in the north-eastern parts of Austria. Our data reflect the epidemiology of HD prior to and after the availability of genetic testing and provide a solid baseline for future investigations on the epidemiology of HD.This revised was published online in April 2005. In the previous version the article category was missing.  相似文献   
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Clinical measurement of the loudness discomfort level (LDL) historically has been part of the hearing aid fitting procedure, and this clinical practice remains popular today. LDL measurements also are recommended in contemporary hearing aid fitting protocols. Yet, surveys show that many hearing aid users are dissatisfied with the loudness of their hearing aids. In this evidence-based review article, we evaluate the effectiveness of clinical LDL measurements. Specifically, we asked the question "Are the clinical measurements of LDL for adult patients predictive of aided acceptance and satisfaction of loudness for high inputs in the real world?" Nearly 200 articles were reviewed; three met the criteria set forth in this review. The evidence supported using unaided LDLs for selecting the maximum real-ear output of hearing aids. No study using aided LDLs or aided loudness verification met the criteria. The level of the evidence for the three articles using unaided LDLs was low; no higher than Level 4. The limited number of studies, the level of evidence, and the statistical power of the studies prevents us from making a strong recommendation concerning the clinical use of LDL measures. Additional research in this area, especially research employing randomized controlled trials would be a useful addition to this body of literature.  相似文献   
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AIM: To study the changes in blood of human neutrophil lipocalin (HNL) and C-reactive protein (CRP) during the course of an acute infection in children. METHODS: Children (n=92) hospitalized with symptoms and signs of acute infections were included and categorized into five groups, i.e. bacterial infection, suspected bacterial infection, viral infection, suspected viral infection and others. Blood was taken at admittance and the following 3-4 d for the measurement of CRP and HNL. RESULTS: Both CRP and HNL were significantly raised at admittance in bacterial infection as compared to viral infection (p<0.001). After 25-48 h, 83% of the children with bacterial infections still had raised CRP levels in contrast to 11% having raised HNL levels. The levels of CRP, but not those of HNL, were significantly correlated to days of symptoms before admission. CONCLUSIONS: HNL is a promising diagnostic tool in the distinction of acute infections caused by bacteria or virus. The differences in the kinetics of CRP and HNL make HNL a better marker for monitoring antibacterial treatment, since HNL is probably elevated only when an active bacterial infection is at hand.  相似文献   
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Common confounding factors for polarimetric concentration measurements include additional optical rotations from unknown optically active molecules, linear birefringence of the medium, and path length variability. We show that by approximating Drude's equation and taking several measurements from the same sample at different wavelengths, the error due to confounding rotations in the measurements can theoretically be canceled. The analysis is developed with regard to glucose sensing in aqueous humor. First, we show that the optical rotatory dispersions of the known molecules in bovine aqueous humor could be represented by Drude's equations. Then, the total optical rotation is approximated by a function combining Drude's equations for the major contributors in the sample, i.e., glucose, glutamine, fructose, and phenylalanine. The concentration-related unknown coefficients in the approximating function are found by constrained nonlinear optimization of the function at different wavelengths. This technique is tested on a published data set and four alterations of those data: (1) concentrations randomly varied within narrow limits, (2) similar to alteration 1 but with significantly elevated glucose concentration, (3) similar to alteration 1 but with significantly decreased glucose concentration, and (4) concentrations randomly varied within wider limits than alteration 1. The method produces very accurate glucose-concentration estimates in all of these data sets. The relative error was smaller than 1% in all except the low-glucose sample (1.4%). This method may prove useful in noninvasive glucose measurement in humans.  相似文献   
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