Corneal confocal microscopy : a novel noninvasive means to diagnose neuropathy in patients with Fabry disease

ABSTRACT: Neuropathy is a cause of significant disability in patients with Fabry disease, yet its diagnosis is difficult. In this study we compared the novel noninvasive techniques of corneal confocal microscopy (CCM) to quantify small-fiber pathology, and non-contact corneal esthesiometry (NCCA) to quantify loss of corneal sensation, with established tests of neuropathy in patients with Fabry disease. Ten heterozygous females with Fabry disease not on enzyme replacement therapy (ERT), 6 heterozygous females, 6 hemizygous males on ERT, and 14 age-matched, healthy volunteers underwent detailed quantification of neuropathic symptoms, neurological deficits, neurophysiology, quantitative sensory testing (QST), NCCA, and CCM. All patients with Fabry disease had significant neuropathic symptoms and an elevated Mainz score. Peroneal nerve amplitude was reduced in all patients and vibration perception threshold was elevated in both male and female patients on ERT. Cold sensation (CS) threshold was significantly reduced in both male and female patients on ERT (P < 0.02), but warm sensation (WS)and heat-induced pain (HIP) were only significantly increased in males onERT (P<0.01). However, corneal sensation assessed withNCCAwas significantly reduced in female (P < 0.02) and male (P < 0.04) patients on ERT compared with control subjects. According to CCM, corneal nerve fiber and branch density was significantly reduced in female (P < 0.03) and male (P < 0.02) patients on ERT compared with control subjects. Furthermore, the severity of neuropathic symptoms and the neurological component of the Mainz Severity Score Index correlated significantly with QSTand CCM. This study shows that CCM and NCCA provide a novel means to detect early nerve fiber damage and dysfunction, respectively, in patients with Fabry disease.

An examination of the relationship between workload and fatigue within and across consecutive days of work: Is the relationshiop static or dynamic?

Cognitive-energetical theories of information processing were used to generate predictions regarding the relationship between workload and fatigue within and across consecutive days of work. Repeated measures were taken on board a naval vessel during a non-routine and a routine patrol. Data were analyzed using growth curve modeling. Fatigue demonstrated a non-monotonic relationship within days in both patrols – fatigue was high at midnight, started decreasing until noontime and then increased again. Fatigue increased across days towards the end of the non-routine patrol, but remained stable across days in the routine patrol. The relationship between workload and fatigue changed over consecutive days in the non-routine patrol. At the beginning of the patrol, low workload was associated with fatigue. At the end of the patrol, high workload was associated with fatigue. This relationship could not be tested in the routine patrol, however it demonstrated a non-monotonic relationship between workload and fatigue – low and high workloads were associated with the highest fatigue. These results suggest that the optimal level of workload can change over time and thus have implications for the management of fatigue.

Modeling web search : preliminary results

This paper reports preliminary results from a study modeling the interplay between multitasking, cognitive coordination, and cognitive shifts during Web search. Study participants conducted three Web searches on personal information problems. Data collection techniques included pre- and post-search questionnaires; think-aloud protocols, Web search logs, observation, and post-search interviews. Key findings include: (1) users Web searches included multitasking, cognitive shifting and cognitive coordination processes, (2) cognitive coordination is the hinge linking multitasking and cognitive shifting that enables Web search construction, (3) cognitive shift levels determine the process of cognitive coordination, and (4) cognitive coordination is interplay of task, mechanism and strategy levels that underpin multitasking and task switching. An initial model depicts the interplay between multitasking, cognitive coordination, and cognitive shifts during Web search. Implications of the findings and further research are also discussed.

Estimation and compensation of clipping noise in OFDMA systems

We propose an efficient and low-complexity scheme for estimating and compensating clipping noise in OFDMA systems. Conventional clipping noise estimation schemes, which need all demodulated data symbols, may become infeasible in OFDMA systems where a specific user may only know his own modulation scheme. The proposed scheme first uses equalized output to identify a limited number of candidate clips, and then exploits the information on known subcarriers to reconstruct clipped signal. Simulation results show that the proposed scheme can significantly improve the system performance.

Random Indexing K-tree

Random Indexing K-tree is the combination of two algorithms suited for large scale document clustering.

Word Segmentation for Chinese Wikipedia Using N-Gram Mutual Information

In this paper, we propose an unsupervised segmentation approach, named "n-gram mutual information", or NGMI, which is used to segment Chinese documents into n-character words or phrases, using language statistics drawn from the Chinese Wikipedia corpus. The approach alleviates the tremendous effort that is required in preparing and maintaining the manually segmented Chinese text for training purposes, and manually maintaining ever expanding lexicons. Previously, mutual information was used to achieve automated segmentation into 2-character words. The NGMI approach extends the approach to handle longer n-character words. Experiments with heterogeneous documents from the Chinese Wikipedia collection show good results.

An extended CDIO syllabus framework with preparatory engineering proficiencies

The CDIO Initiative has been globally recognised as an enabler for engineering education reform. With the CDIO process, the CDIO Standards and the CDIO Syllabus, many scholarly contributions have been made around cultural change, curriculum reform and learning environments. In the Australasian region, reform is gaining significant momentum within the engineering education community, the profession, and higher education institutions. This paper presents the CDIO Syllabus cast into the Australian context by mapping it to the Engineers Australia Graduate Attributes, the Washington Accord Graduate Attributes and the Queensland University of Technology Graduate Capabilities. Furthermore, in recognition that many secondary schools and technical training institutions offer introductory engineering technology subjects, this paper presents an extended self-rating framework suited for recognising developing levels of proficiency at a preparatory level. The framework is consistent with conventional application to undergraduate programs and professional practice, but adapted for the preparatory context. As with the original CDIO framework with proficiency levels, this extended framework is informed by Bloom’s Educational Objectives. A proficiency evaluation of Queensland Study Authority’s Engineering Technology senior syllabus is demonstrated indicating proficiency levels embedded within this secondary school subject within a preparatory scope. Through this extended CDIO framework, students and faculty have greater awareness and access to tools to promote (i) student engagement in their own graduate capability development, (ii) faculty engagement in course and program design, through greater transparency and utility of the continuum of graduate capability development with associate levels of proficiency, and the context in which they exist in terms of pre-tertiary engineering studies; and (iii) course maintenance and quality audit methodology for the purpose of continuous improvement processes and program accreditation.

Scientific theories

Advanced Research Methods in the Built Environment addresses common topics raised by postgraduate level researchers rather than dealing with all aspects of the research process. Issues covered range from the practicalities of producing a journal article to the role of theory in research.

Deeply divergent mitochondrial lineages reveal patterns of local endemism in chironomids of the Australian Wet Tropics

The Wet Tropics bioregion of north-eastern Australia has been subject to extensive fluctuations in climate throughout the late Pliocene and Pleistocene. Cycles of rainforest contraction and expansion of dry sclerophyll forest associated with such climatic fluctuations are postulated to have played a major role in driving geographical endemism in terrestrial rainforest taxa. Consequences for the distributions of aquatic organisms, however, are poorly understood.The Australian non-biting midge species Echinocladius martini Cranston (Diptera: Chironomidae), although restricted to cool, well-forested freshwater streams, has been considered to be able to disperse among populations located in isolated rainforest pockets during periods of sclerophyllous forest expansion, potentially limiting the effect of climatic fluctuations on patterns of endemism. In this study, mitochondrial COI and 16S data were analysed for E. martini collected from eight sites spanning theWet Tropics bioregion to assess the scale and extent of phylogeographic structure. Analyses of genetic structure showed several highly divergent cryptic lineages with restricted geographical distributions. Within one of the identified lineages, strong genetic structure implied that dispersal among proximate (<1 km apart) streams was extremely restricted. The results suggest that vicariant processes, most likely due to the systemic drying of the Australian continent during the Plio-Pleistocene, might have fragmented historical E. martini populations and, hence, promoted divergence in allopatry.

Treadmill adaptation and verification of self-selected walking speed : a protocol for children

The term self-selected (i.e., individual or comfortable walking pace or speed) is commonly used in the literature (Frost, Dowling, Bar-Or, & Dyson, 1997; Jeng, Liao, Lai, & Hou, 1997; Wergel-Kolmert & Wohlfart, 1999; Maltais, Bar-Or, Pienynowski, & Galea, 2003; Browning & Kram, 2005; Browning, Baker, Herron, & Kram, 2006; Hills, Byrne, Wearing, & Armstrong, 2006) and is identified as the most efficient walking speed, with increased efficiency defined by lower oxygen uptake (VO^sub 2^) per unit mechanical work (Hoyt & Taylor, 1981; Taylor, Heglund, & Maloiy, 1982; Hreljac, 1993). [...] assessing individual and group differences in metabolic energy expenditure using oxygen uptake requires individuals to be comfortable with, and able to accommodate to, the equipment.

Within- and between-day repeatability and variability in children's physiological responses during submaximal treadmill exercise

The purpose of this study was to verify within- and between-day repeatability and variability in children's oxygen uptake (VO^sub 2^), gross economy (GE; VO^sub 2^ divided by speed) and heart rate (HR) during treadmill walking based on self-selected speed (SS). Fourteen children (10.1 ± 1.4 years) undertook three testing sessions over 2 days in which four walking speeds, including SS were tested. Within- and between-day repeatability were assessed using the Bland and Altman method, and coefficients of variability (CV) were determined for each child across exercise bouts and averaged to obtain a mean group CV value for VO^sub 2^, GE, and HR per speed. Repeated measures analysis of variance showed no statistically significant differences in within- or between-day CV for VO^sub 2^, GE, or HR at any speed. Repeatability within- and between-day for VO^sub 2^, GE, and HR for all speeds was verified. These results suggest that submaximal VO^sub 2^ during treadmill walking is stable and reproducible at a range of speeds based on children's SS.

Low-fat oxidation may be a factor in obesity among men with schizophrenia

Objective: Obesity associated with atypical antipsychotic medications is an important clinical issue for people with schizophrenia. The purpose of this project was to determine whether there were any differences in resting energy expenditure (REE) and respiratory quotient (RQ) between men with schizophrenia and controls. Method: Thirty-one men with schizophrenia were individually matched for age and relative body weight with healthy, sedentary controls. Deuterium dilution was used to determine total body water and subsequently fat-free mass (FFM). Indirect calorimetry using a Deltatrac metabolic cart was used to determine REE and RQ. Results: When corrected for FFM, there was no significant difference in REE between the groups. However, fasting RQ was significantly higher in the men with schizophrenia than the controls. Conclusion: Men with schizophrenia oxidised proportionally less fat and more carbohydrate under resting conditions than healthy controls. These differences in substrate utilisation at rest may be an important consideration in obesity in this clinical group.

Health-related quality of life in obese children and adolescents

Objective: This review addresses the effect of overweight and obese weight status on pediatric health-related quality of life (HRQOL). Method: Web of Science, Medline, CINAHL, Cochrane Library, EMBASE, AMED and PubMed were searched for peer-reviewed studies in English reporting HRQOL and weight status in youth (<21 years), published before March 2008. Results: Twenty-eight articles were identified. Regression of HRQOL against body mass index (BMI) using pooled data from 13 studies utilizing the Pediatric Quality of Life Inventory identified an inverse relationship between BMI and pediatric HRQOL (r=−0.7, P=0.008), with impairments in physical and social functioning consistently reported. HRQOL seemed to improve with weight loss, but randomized controlled trials were few and lacked long-term follow-up. Conclusions: Little is known about the factors associated with reduced HRQOL among overweight or obese youth, although gender, age and obesity-related co-morbidities may play a role. Few studies have examined the differences in HRQOL between community and treatment-seeking samples. Pooled regressions suggest pediatric self-reported HRQOL can be predicted from parent proxy reports, although parents of obese youths tend to perceive worse HRQOL than children do about themselves. Thus, future research should include both pediatric and parent proxy perspectives.

A role mining inspired approach to representing user behaviour in ERP systems

Despite all attempts to prevent fraud, it continues to be a major threat to industry and government. Traditionally, organizations have focused on fraud prevention rather than detection, to combat fraud. In this paper we present a role mining inspired approach to represent user behaviour in Enterprise Resource Planning (ERP) systems, primarily aimed at detecting opportunities to commit fraud or potentially suspicious activities. We have adapted an approach which uses set theory to create transaction profiles based on analysis of user activity records. Based on these transaction profiles, we propose a set of (1) anomaly types to detect potentially suspicious user behaviour and (2) scenarios to identify inadequate segregation of duties in an ERP environment. In addition, we present two algorithms to construct a directed acyclic graph to represent relationships between transaction profiles. Experiments were conducted using a real dataset obtained from a teaching environment and a demonstration dataset, both using SAP R/3, presently the most predominant ERP system. The results of this empirical research demonstrate the effectiveness of the proposed approach.

Resistance training for obese, type 2 diabetic adults: A review of the evidence

In both developed and developing countries, increased prevalence of obesity has been strongly associated with increased incidence of type 2 diabetes mellitus (T2DM) in the adult population. Previous research has emphasized the importance of physical activity in the prevention and management of obesity and T2DM, and generic exercise guidelines originally developed for the wider population have been adapted for these specific populations. However, the guidelines traditionally focus on aerobic training without due consideration to other exercise modalities. Recent reviews on resistance training in the T2DM population have not compared this modality with others including aerobic training, or considered the implications of resistance training for individuals suffering from both obesity and T2DM. In short, the optimal mix of exercise modalities in the prescription of exercise has not been identified for it benefits to the metabolic, body composition and muscular health markers common in obesity and T2DM. Similarly, the underlying physical, social and psychological barriers to adopting and maintaining exercise, with the potential to undermine the efficacy of exercise interventions, have not been addressed in earlier reviews. Because it is well established that aerobic exercise has profound effects on obesity and T2DM risk, the purpose of this review was to address the importance of resistance training to obese adults with T2DM.