An assessment of the quality of the I-DSD and the I-CAH registries - international registries for rare conditions affecting sex development

With the proliferation of rare disease registries, there is a need for registries to undergo an assessment of their quality against agreed standards to ensure their long-term sustainability and acceptability.This study was performed to evaluate the I-DSD and I-CAH Registries and identify their strengths and weaknesses. The design and operational aspects of the registries were evaluated against published quality indicators. Additional criteria included the level of activity, international acceptability of the registries and their use for research. The design of the I-DSD and I-CAH Registries provides them with the ability to perform multiple studies and meet the standards for data elements, data sources and eligibility criteria. The registries follow the standards for data security, governance, ethical and legal issues, sustainability and communication of activities. The data have a high degree of validity, consistency and accuracy and the completeness is maximal for specific conditions such as androgen insensitivity syndrome and congenital adrenal hyperplasia. In terms of research output, the external validity is strong but the wide variety of cases needs further review. The internal validity of data was condition specific and highest for conditions such as congenital adrenal hyperplasia. The shift of the registry from a European registry to an international registry and the creation of a discrete but linked CAH registry increased the number of users and stakeholders as well as the international acceptability of both registries. The I-DSD and I-CAH registries comply with the standards set by expert organisations. Recent modifications in their operation have allowed the registries to increase their user acceptability

Filtering and scalability in the ECO distributed event model

Event-based communication is useful in many application domains, ranging from small, centralised applications to large, distributed systems. Many different event models have been developed to address the requirements of different application domains. One such model is the ECO model which was designed to support distributed virtual world applications. Like many other event models, ECO has event filtering capabilities meant to improve scalability by decreasing network traffic in a distributed implementation. Our recent work in event-based systems has included building a fully distributed version of the ECO model, including event filtering capabilities. This paper describes the results of our evaluation of filters as a means of achieving increased scalability in the ECO model. The evaluation is empirical and real data gathered from an actual event-based system is used

Methods of editing cloud and atmospheric layer affected pixels from satellite data

Subvisible cirrus clouds (SCi) were easily distinguished in mid-infrared (MIR) TIROS-N daytime data from south Texas and northeast Mexico. The MIR (3.55-3.93 micrometer) pixel digital count means of the SCi affected areas were more than 3.5 standard deviations on the cold side of the scene means. (These standard deviations were made free of the effects of unusual instrument error by factoring out the Ch 3 MIR noise on the basis of detailed examination of noisy and noise-free pixels). SCi affected areas in the IR Ch 4 (10.5-11.5 micrometer) appeared cooler than the general scene, but were not as prominent as in Ch 3, being less than 2 standard deviations from the scene mean. Ch 3 and 4 standard deviations and coefficients of variation are not reliable indicators, by themselves, of the presence of SCi because land features can have similar statistical properties

Assessing the SEU resistance of CMOS latches using alpha-particle sensitive test circuits

The importance of Cosmic Rays on the performance of integrated circuits (IC's) in a space environment is evident in the upset rate of the Tracking and Data Relay Satellite (TDRS) launched in Apr. 1983. This satellite experiences a single-event-upset (SEU) per day which must be corrected from the ground. Such experience caused a redesign of the Galileo spacecraft with SEU resistant IC's. The solution to the SEU problem continues to be important as the complexity of spacecraft grows, the feature size of IC's decreases, and as space systems are designed with circuits fabricated at non-radiation hardened foundries. This paper describes an approach for verifying the susceptibility of CMOS latches to heavy-ion induced state changes. The approach utilizes alpha particles to induce the upsets in test circuits. These test circuits are standard cells that have offset voltages which sensitize the circuits to upsets. These results are then used to calculate the upsetability at operating voltages. In this study results are presented for the alpha particle upset of a six-transistor static random access memory (SRAM) cell. Then a methodology is described for the analysis of a standard-cell inverter latch

Methods of editing cloud and atmospheric layer affected pixels from satellite data

The location and migration of cloud, land and water features were examined in spectral space (reflective VIS vs. emissive IR). Daytime HCMM data showed two distinct types of cloud affected pixels in the south Texas test area. High altitude cirrus and/or cirrostratus and "subvisible cirrus" (SCi) reflected the same or only slightly more than land features. In the emissive band, the digital counts ranged from 1 to over 75 and overlapped land features. Pixels consisting of cumulus clouds, or of mixed cumulus and landscape, clustered in a different area of spectral space than the high altitude cloud pixels. Cumulus affected pixels were more reflective than land and water pixels. In August the high altitude clouds and SCi were more emissive than similar clouds were in July. Four-channel TIROS-N data were examined with the objective of developing a multispectral screening technique for removing SCi contaminated data

Plasmon Evolution and Charge-Density Wave Suppression in Potassium Intercalated Tantalum Diselenide

We have investigated the influence of potassium intercalation on the formation of the charge-density wave (CDW) instability in 2H-tantalum diselenide by means of Electron Energy-Loss Spectroscopy and density functional theory. Our observations are consistent with a filling of the conduction band as indicated by a substantial decrease of the plasma frequency in experiment and theory. In addition, elastic scattering clearly points to a destruction of the CDW upon intercalation as can be seen by a vanishing of the corresponding superstructures. This is accompanied by a new superstructure, which can be attributed to the intercalated potassium. Based on the behavior of the c-axis upon intercalation we argue in favor of interlayer-sites for the alkali-metal and that the lattice remains in the 2H-modification

The longitudinal conductance of mesoscopic Hall samples with arbitrary disorder and periodic modulations

We use the Kubo-Landauer formalism to compute the longitudinal (two-terminal) conductance of a two dimensional electron system placed in a strong perpendicular magnetic field, and subjected to periodic modulations and/or disorder potentials. The scattering problem is recast as a set of inhomogeneous, coupled linear equations, allowing us to find the transmission probabilities from a finite-size system computation; the results are exact for non-interacting electrons. Our method fully accounts for the effects of the disorder and the periodic modulation, irrespective of their relative strength, as long as Landau level mixing is negligible. In particular, we focus on the interplay between the effects of the periodic modulation and those of the disorder. This appears to be the relevant regime to understand recent experiments [S. Melinte {\em et al}, Phys. Rev. Lett. {\bf 92}, 036802 (2004)], and our numerical results are in qualitative agreement with these experimental results. The numerical techniques we develop can be generalized straightforwardly to many-terminal geometries, as well as other multi-channel scattering problems.Comment: 13 pages, 11 figure

Pressure ulcer related pain in community populations: a prevalence survey.

BACKGROUND: Pressure ulcers are costly to the healthcare provider and can have a major impact on patient's quality of life. One of the most distressing symptoms reported is pain. There is very little published data on the prevalence and details of pain experienced by patients with pressure ulcers, particularly in community populations. The study was conducted in two community NHS sites in the North of England. METHODS: The aim was to estimate the prevalence of pressure area related pain within a community population. We also explored the type and severity of the pain and its association with pressure ulcer classification. A cross-sectional survey was performed of community nurses caseloads to identify adult patients with pressure ulcers and associated pain. Consenting patients then had a full pain assessment and verification of pressure ulcer grade. RESULTS: A total of 287 patients were identified with pressure ulcers (0.51 per 1000 adult population). Of the 176 patients who were asked, 133 (75.6%) reported pain. 37 patients consented to a detailed pain assessment. Painful pressure ulcers of all grades and on nearly all body sites were identified. Pain intensity was not related to number or severity of pressure ulcer. Both inflammatory and neuropathic pain were reported at all body sites however the proportion of neuropathic pain was greater in pressure ulcers on lower limbs. CONCLUSIONS: This study has identified the extent and type of pain suffered by community patients with pressure ulcers and indicates the need for systematic and regular pain assessment and treatment

Linking highway improvements to changes in land use with quasi-experimental research design: A better forecasting tool for transportation decision-making

An important issue for future improvement and extensions of highways will be the ability of projects to sustain challenges to Environmental Impact Statements based upon forecasts of regional growth. A legal precedent for such challenges was established in 1997 when a U.S. District Court judge ruled that the EIS for a proposed Illinois toll road was deficient because the growth projections were the same in the build and no-build scenarios. This paper incorporates popular regional growth forecasting models into a quasi-experimental research design that directly relates new highway investments in three California counties to changes in population and employment location, while controlling for no-build historical counterfactuals. The authors model simultaneous employment and population growth from 1980 to 2000 in Merced, Orange, and Santa Clara counties, three California counties that received substantive highway improvements during the mid-1990s. The strategy permits a comparison of the before-and-after tests for effects of investments on economic growth and land use in three regions that contrast how increased highway access affects development patterns: (1) for an urban center in Santa Clara County, (2) for an exurban region in Orange County, and (3) for a small town in Merced County. We find that traditional forecast approaches, which lack explicit control selection, can lead to erroneous conclusions about an impact. Our integrated form of the lagged adjustment model confirms results from a conventional form of the model that includes all cross-sectional units as observations; in both forms of the model we estimate a statistically significant increase in employment development in the exurban region in Orange County where new toll roads were constructed. In the case of Santa Clara County, neither our quasi-experimental integrated approach nor the conventional lagged adjustment approach estimates a significant effect on population or employment growth that can be attributed to the new highways constructed in the urban center. For the small town environment in Merced County, the conventional simultaneous growth regressions produce a materially different estimate than the approach we develop and examine in this paper. Isolating effects to local spatial units where the intervention occurred and their no-build counterfactual produces estimates of a statistically significant decrease in employment growth in the small town near the newly constructed highway bypass