Reliability Assessment

Substrate reduction therapy (SRT) is considered to be a potential therapeutic option for juvenile GM2 gangliosidosis (jGM2g). We evaluated the efficacy of SRT in jGM2g, assessing neurological, neuropsychological and brain magnetic resonance imaging (MRI) outcomes over a 24-month period of treatment. In an open-label and single-center study, five jGM2g patients (mean age 14.6+/-4.5 years) received oral miglustat at doses of 100-200mg t.i.d. adjusted to body surface area. Patients underwent general and neurological examinations, neuropsychological, electrophysiological, and brain MRI studies. All patients showed neurological deterioration over the period of the study, with particularly notable worsening of gait, speech and coordination. One patient experienced acute psychosis, and another showed worsening of pre-existing epilepsy. Some neuropsychological tests showed no evidence of deterioration in the three patients with high enough cognitive functioning for reliable assessment. Profound cognitive impairment in two children precluded neuropsychological evaluation. In four patients, evaluation of brain MRI showed no changes in white matter signal abnormalities and cerebellar atrophy noted at baseline, while one patient showed progression of cerebellar and supratentorial brain atrophy. Transmission electron microscopy analysis of peripheral mononuclear cells showed reduction of intracytoplasmatic inclusions with treatment. SRT with miglustat of patients with jGM2g failed to ameliorate progressive neurological deterioration, but apparently no worsening of some areas of cognitive function tested and brain MRI lesions was noted over 24 months of treatment. The results must be interpreted with care owing to the small sample of patients and the lack of a control-arm.

Assessing the learning style preferences of adult workers (consumers) with moderate to severe intellectual disabilities and accommodating the consumer's learning style preference during the job training process is a critical feature of quality supported employment services and an under-explored area of applied research. However, before evaluating the effects of learning style preferences on job training outcomes, consumers must first demonstrate the skills to express learning preferences. This research sought to demonstrate a method of assessing the learning style preference of consumers with moderate to severe intellectual disabilities and to investigate which of two instructional interventions, when matched with the consumers preferred learning style, would have the greatest impact on consumer job performance. Three independent variables were included in this study: (1) the consumer's learning style preference, (2) auditory instructional cues, and (3) visual instructional cues. The dependent variable was the accuracy of job task performance for each consumer under the alternating instructional interventions. ^ Four participants participated in the exploratory phase (phase I) of the study and three participants participated in the empirical phase (phase II) of the study. Phase I involved a descriptive analysis. Consumer learning style was assessed utilizing the Learning Style Inventory-Primary (Perrin, 1991), which was modified for use with the participant group. Phase II involved single-subject research design principles. Specifically, an alternating treatment withdrawal design was utilized to determine which instructional strategy (auditory or visual cues) when matched with the consumer's preferred learning style, had the greatest impact on the dependent variable. An Independent t-Test and a Helmert Contrast were used to determine statistical significance between conditions. An alpha level of .05 was set for determining significance. ^ Results indicate that learning style preferences for consumers with moderate to severe intellectual disabilities can be reliably assessed. Analysis of the data indicated that there was a significant difference in the effect of the two instructional interventions on consumer job performance. When the instructional intervention was congruent with the consumer's learning style, the greater the improvement in job performance. When the intervention was incongruent to the consumer's preferred learning style, a lesser degree of improved job performance would result. ^

The efficient and reliable assessment of general community health requires the development of comprehensive and parsimonious measures of proven validity. The Nottingham Health Profile (NHP) has been demonstrated to be a reliable indicator of common expressions of discomfort and stress in the general population. The present paper describes its linguistic adaptation into French, the derivation of item weights by Thurstone's method of paired comparisons and the comparison of item weights across various sociodemographic groups. There is more similarity than variation on the valuation of the state of health explored by the NHP between the French and the British population as little inter-cultural or inter-linguistic variations were found. The differences in judgement of severity elicited across sociodemographic groups in the French sample cast some doubts on the relevance of general weights for use in population surveys.

This paper presents a framework for assessing the impact of the data collection process on the validity of key measures in reliability. The quality of data is affected by many organisational and behavioural factors. The aims of developing this framework are to (1) identify inputs/steps that have the most significant impact on the quality of key performance indicators such as MTTF (mean time to failure) and MTTR (mean time to repair), (2) identify ‘weak’ links in the data collection process, and (3) identify potential remedial actions. Development of this framework will assist the understanding of assumptions used in reliability calculations and improve the quality of underlying data and the data collection process. Consequently, this is a vital step in the continued development and use of data based decision-making models for reliability assessment.

The present paper deals with the application of several chemometrical methods (cluster and principal components analysis, source apportioning on absolute principal components scores) to an aerosol data collection from Unterloibach, Austria. It is shown that seven latent factors explaining almost 80% of the total variance are responsible for the data structure and are conditionally identified as “secondary aerosol”, “mineral dust”, “oil burning”, “lead smelter”, “coal burning”, “salt” and “fertilizer” emission sources. Furthermore, the contribution of each identified source to the formation of the particle total mass and chemical compounds total concentration is calculated. Thus, a reliable assessment of the air quality in the region is performed. The requirements of the sustainability concept for ecological indicators in this case is easily transformed into a multivariate statistical problem taking into account not separate indicators but the specific multivariate nature of aerosol pollution.

The performance of a tracking filter can be evaluated in terms of the filterpsilas optimality conditions. Testing for optimality is necessary because the estimation error covariance as provided by the filter is not a reliable indicator of performance, which is known to be ldquooptimisticrdquo (inconsistent) particularly when there are model mismatches and target maneuvers. The conventional root-mean square (RMS) error value and its variants are widely used for performance evaluation in simulation and testing but it is not feasible for real-time operations where the ground truth is hardly available. One approach for real-time reliability assessment is optimality self online monitoring (OSOM) investigated in this paper. Statistical tests for optimality conditions are formulated. Simulation examples are presented to illustrate their possible use in evaluation and adaptation.

To illustrate the process of developing a projective drawing test, this paper describes the experience of creating and working with the Face Stimulus Assessment (FSA). This tool comprises a series of three stimulus images: Picture 1 consists of a standardized image of a human face; Picture 2 contains an outline of the face only; and Picture 3 is a blank page. Six samples of the FSA completed by clients with multiple disabilities (including communication disorders and autism) are presented. As the FSA is a work in progress and has yet to be established as a valid and reliable assessment, implications for further research are also discussed.

Abstract 1. In research on trust in the organizational context, there is some agreement evolving that trust should be measured with respect to various foci. The Workplace Trust Survey (WTS) by Ferres (2002) provides reliable assessment of coworker, supervisor, and organizational trust. By means of a functionally equivalent translation, we developed a German version of the questionnaire (G-WTS) comprising 21 items. A total of 427 employees were surveyed with the G-WTS and questionnaires concerning several work-related ...

The Hamilton Rating Scale for Depression (HAMD) is the de facto international gold standard for the assessment of depression. There are some criticisms, however, especially with regard to its inter-rater reliability, due to the lack of standardized questions or explicit scoring procedures. The GRID-HAMD was developed to provide standardized explicit scoring conventions and a structured interview guide for administration and scoring of the HAMD. We developed the Japanese version of the GRID-HAMD and examined its inter-rater reliability among experienced and inexperienced clinicians (n=70), how rater characteristics may affect it, and how training can improve it in the course of a model training program using videotaped interviews. The results showed that the inter-rater reliability of the GRID-HAMD total score was excellent to almost perfect and those of most individual items were also satisfactory to excellent, both with experienced and inexperienced raters, and both before and after the training. With its standardized definitions, questions and detailed scoring conventions, the GRID-HAMD appears to be the best achievable set of interview guides for the HAMD and can provide a solid tool for highly reliable assessment of depression severity.

This study assesses the measurement properties of a scale that measures the key internal organizational factors that influence middle managers to initiate corporate entrepreneurship activities. In this study, corporate entrepreneurship is used in a broad sense to include the development and implementation of new ideas into the organization. Using this definition, this study describes an instrument used to empirically identify