Histopathology and fibrillin-1 distribution in severe early onset Marfan syndrome

Marfan syndrome (MFS) is an autosomal dominant condition which may involve the cardiovascular, ocular, skeletal, and other systems. Mutations causing MFS are found in the FBN1 gene, encoding fibrillin-1, an extracellular matrix protein involved in microfibril formation. In the most severe cases, mutations are generally found in exons 24-32, and children with these mutations usually die in the first years of life, of cardiopulmonary failure. We present clinical, molecular and histopathological studies on a patient with severe early onset MFS. He has a mutation in exon 25 of FBN1, a G > A transition at nucleotide position 3131 that converts the codon TGC, coding for cysteine at position 1044, to TAC, coding for tyrosine (C1044Y). This has resulted in abnormalities of the extracellular matrix and a severe clinical phenotype, although he has survived to the age of 14 years. (c) 2005 Wiley-Liss, Inc.

Rotary atomizer drop size distribution database

Wind tunnel measurements of drop Size distributions from Micronair A U4000 and A U5000 rotary atomizers were collected to develop a database for model use. The measurements varied tank mix, flow rate, air speed, and blade angle conditions, which were correlated by multiple regressions (average R-2 = 0.995 for A U4000 and 0.988 for AU5000). This database replaces an outdated set of rotary atomizer data measured in the 1980s by the USDA Forest Service and fills in a gap in data measured in the 1990s by the Spray Drift Task Force. Since current USDA Forest Service spray projects rely on rotary atomizers, the creation of the database (and its multiple regression interpolation) satisfies a need seen for ten years.

Correlation between anammox activity and microscale distribution of nitrite in a subtropical mangrove sediment

The distribution of anaerobic ammonium oxidation (anammox) in nature has been addressed by only a few environmental studies, and our understanding of how anammox bacteria compete for substrates in natural environments is therefore limited. In this study, we measure the potential anammox rates in sediment from four locations in a subtropical tidal river system. Porewater profiles of NOx- (NO2- plus NO3-) and NO2- were measured with microscale biosensors, and the availability of NO2- was compared with the potential for anammox activity. The potential rate of anammox increased with increasing distance from the mouth of the river and correlated strongly with the production of nitrite in the sediment and with the average concentration or total pool of nitrite in the suboxic sediment layer. Nitrite accumulated both from nitrification and from NOx- reduction, though NOx- reduction was shown to have the greatest impact on the availability of nitrite in the suboxic sediment layer. This finding suggests that denitrification, though using NO2- as a substrate, also provides a substrate for the anammox process, which has been suggested in previous studies where microscale NO2- profiles were not measured.

Adsorption in slit pores and pore-size distribution: A molecular layer structure theory

A new approach is developed to analyze the thermodynamic properties of a sub-critical fluid adsorbed in a slit pore of activated carbon. The approach is based on a representation that an adsorbed fluid forms an ordered structure close to a smoothed solid surface. This ordered structure is modelled as a collection of parallel molecular layers. Such a structure allows us to express the Helmholtz free energy of a molecular layer as the sum of the intrinsic Helmholtz free energy specific to that layer and the potential energy of interaction of that layer with all other layers and the solid surface. The intrinsic Helmholtz free energy of a molecular layer is a function (at given temperature) of its two-dimensional density and it can be readily obtained from bulk-phase properties, while the interlayer potential energy interaction is determined by using the 10-4 Lennard-Jones potential. The positions of all layers close to the graphite surface or in a slit pore are considered to correspond to the minimum of the potential energy of the system. This model has led to accurate predictions of nitrogen and argon adsorption on carbon black at their normal boiling points. In the case of adsorption in slit pores, local isotherms are determined from the minimization of the grand potential. The model provides a reasonable description of the 0-1 monolayer transition, phase transition and packing effect. The adsorption of nitrogen at 77.35 K and argon at 87.29 K on activated carbons is analyzed to illustrate the potential of this theory, and the derived pore-size distribution is compared favourably with that obtained by the Density Functional Theory (DFT). The model is less time-consuming than methods such as the DFT and Monte-Carlo simulation, and most importantly it can be readily extended to the adsorption of mixtures and capillary condensation phenomena.

Socio-economic distribution of environmental risk factors for childhood injury

Objective: Childhood injury remains the single most important cause of mortality in children aged between 1-14 years in many countries. It has been proposed that lower socio-economic status (SES) and poorer housing contribute to potential hazards in the home environment. This study sought to establish whether the prevalence of observed hazards in and around the home was differentially distributed by SES, in order to identify opportunities for injury prevention. Methods: This study was a cross-sectional, random sample survey of primary school children from 32 schools in Brisbane. Interviews and house audits were conducted between July 2000 and April 2003 to collect information on SES (income, employment and education) and previously identified household hazards. Results: There was evidence of a relationship between prevalence of household environmental hazards and household SES; however, the magnitude and direction of this relationship appeared to be hazard-specific. Household income was related to play equipment characteristics, with higher SES groups being more likely to be exposed to risk. All three SES indicators were associated with differences in the home safety characteristics, with the lower SES groups more likely to be exposed to risk. Conclusion:The differential distribution of environmental risk factors by SES of household may help explain the SES differential in the burden of injury and provides opportunities for focusing efforts to address the problem.

Recurrence interval statistics of cellular automaton seismicity models

The recurrence interval statistics for regional seismicity follows a universal distribution function, independent of the tectonic setting or average rate of activity (Corral, 2004). The universal function is a modified gamma distribution with power-law scaling of recurrence intervals shorter than the average rate of activity and exponential decay for larger intervals. We employ the method of Corral (2004) to examine the recurrence statistics of a range of cellular automaton earthquake models. The majority of models has an exponential distribution of recurrence intervals, the same as that of a Poisson process. One model, the Olami-Feder-Christensen automaton, has recurrence statistics consistent with regional seismicity for a certain range of the conservation parameter of that model. For conservation parameters in this range, the event size statistics are also consistent with regional seismicity. Models whose dynamics are dominated by characteristic earthquakes do not appear to display universality of recurrence statistics.

On sensor network segmentation for urban water distribution monitoring

Identifying water wastage in forms of leaks in a water distribution network of any city becomes essential as droughts are presenting serious threats to few major cities. In this paper, we propose a deployment of sensor network for monitoring water flow in any water distribution network. We cover the issues related with designing such a dedicated sensor network by considering types of sensors required, sensors' functionality, data collection, and providing computation serving as leak detection mechanism. The main focus of this paper is on appropriate network segmentation that provides the base for hierarchical approach to pipes' failure detection. We show a method for sensors allocation to the network in order to facilitate effective pipes monitoring. In general, the identified computational problem belongs to hard problems. The paper shows a heuristic method to build effective hierarchy of the network segmentation.

When electronic management tools work - and don't work - in social-based distribution channels:a study of IT manufacturers and resellers

Electronic information tools have become increasingly popular with channel manufacturers in their efforts to manage resellers. Although these tools have been found to increase the efficiency of communications, researchers and practitioners alike have questioned their effectiveness. To investigate how top-down electronic information affects social channel relationships we consider the use of such tools in information technology distribution channels. Using electronic communications theory and channel governance theory we hypothesize that the usefulness of the tools is a function of the type of information inherent in each tool (demand creation information or supply fulfillment information) and the particular communications characteristics of this information.

Computational mechanics of molecular systems:quantifying high-dimensional dynamics by distribution of Poincaré recurrence times

A framework that connects computational mechanics and molecular dynamics has been developed and described. As the key parts of the framework, the problem of symbolising molecular trajectory and the associated interrelation between microscopic phase space variables and macroscopic observables of the molecular system are considered. Following Shalizi and Moore, it is shown that causal states, the constituent parts of the main construct of computational mechanics, the e-machine, define areas of the phase space that are optimal in the sense of transferring information from the micro-variables to the macro-observables. We have demonstrated that, based on the decay of their Poincare´ return times, these areas can be divided into two classes that characterise the separation of the phase space into resonant and chaotic areas. The first class is characterised by predominantly short time returns, typical to quasi-periodic or periodic trajectories. This class includes a countable number of areas corresponding to resonances. The second class includes trajectories with chaotic behaviour characterised by the exponential decay of return times in accordance with the Poincare´ theorem.

Size frequency distribution of senile plaques in post-mortem brains of five patients with senile dementia of the Alzheimer type (SDAT)

Numerous senile plaques are one of the most characteristic histological findings in SDAT brains. Large classical plaques may develop from smaller uncored forms. There is no strong evidence that, once formed, plaques disappear from the tissue. We have examined cresyl-violet stained sections of the parahippocampal gyrus (PHG), hippocampus, frontal lobe and temporal lobe of five SDAT patients. The frequency of various sizes of plaques were determined in each of these brain regions. Statistical analysis showed that the ratio of large plaques to small plaques was greater in the hippocampal formation (especially the PHG) than in the neocortex. One explanation of these results is that plaques grow more rapidly in the hippocampal formation than elsewhere. Alternatively, if the rate of plaque growth is much the same in different brain regions, the data suggest that plaques develop first in the hippocampal formation (especially the PHG) and only later spread to the neocortex. This interpretation is also consistent with the theory that the neuropathology of SDAT spreads from the olfactory cortex via the hippocampal formation to the neocortex. Further development of this technique may help identify the site of the primary lesion in SDAT.

The visual cortex in Alzheimer disease:laminar distribution of the pathological changes in visual areas V1 and V2

Alzheimer’s disease (AD) is an important neurodegenerative disorder causing visual problems in the elderly population. The pathology of AD includes the deposition in the brain of abnormal aggregates of ?-amyloid (A?) in the form of senile plaques (SP) and abnormally phosphorylated tau in the form of neurofibrillary tangles (NFT). A variety of visual problems have been reported in patients with AD including loss of visual acuity (VA), colour vision and visual fields; changes in pupillary responses to mydriatics, defects in fixation and in smooth and saccadic eye movements; changes in contrast sensitivity and in visual evoked potentials (VEP); and disturbances in complex visual tasks such as reading, visuospatial function, and in the naming and identification of objects. In addition, pathological changes have been observed to affect the eye, visual pathway, and visual cortex in AD. To better understand degeneration of the visual cortex in AD, the laminar distribution of the SP and NFT was studied in visual areas V1 and V2 in 18 cases of AD which varied in disease onset and duration. In area V1, the mean density of SP and NFT reached a maximum in lamina III and in laminae II and III respectively. In V2, mean SP density was maximal in laminae III and IV and NFT density in laminae II and III. The densities of SP in laminae I of V1 and NFT in lamina IV of V2 were negatively correlated with patient age. No significant correlations were observed in any cortical lamina between the density of NFT and disease onset or duration. However, in area V2, the densities of SP in lamina II and lamina V were negatively correlated with disease duration and disease onset respectively. In addition, there were several positive correlations between the densities of SP and NFT in V1 with those in area V2. The data suggest: (1) NFT pathology is greater in area V2 than V1, (2) laminae II/III of V1 and V2 are most affected by the pathology, (3) the formation of SP and NFT in V1 and V2 are interconnected, and (4) the pathology may spread between visual areas via the feed-forward short cortico-cortical connections.

A structural investigation of the alkali metal site distribution within bioactive glass using neutron diffraction and multinuclear solid state NMR

The atomic-scale structure of Bioglass and the effect of substituting lithium for sodium within these glasses have been investigated using neutron diffraction and solid state magic angle spinning (MAS) NMR. Applying an effective isomorphic substitution difference function to the neutron diffraction data has enabled the Na-O and Li-O nearest-neighbour correlations to be isolated from the overlapping Ca-O, O-(P)-O and O-(Si)-O correlations. These results reveal that Na and Li behave in a similar manner within the glassy matrix and do not disrupt the short range order of the network former. Residual differences are attributed solely to the variation in ionic radius between the two species. Successful simplification of the 2

Factors determining the size frequency distribution of β-amyloid (Aβ) deposits in Alzheimer's disease

The size frequency distributions of discrete β-amyloid (Aβ) deposits were studied in single sections of the temporal lobe from patients with Alzheimer's disease. The size distributions were unimodal and positively skewed. In 18/25 (72%) tissues examined, a log normal distribution was a good fit to the data. This suggests that the abundances of deposit sizes are distributed randomly on a log scale about a mean value. Three hypotheses were proposed to account for the data: (1) sectioning in a single plane, (2) growth and disappearance of Aβ deposits, and (3) the origin of Aβ deposits from clusters of neuronal cell bodies. Size distributions obtained by serial reconstruction through the tissue were similar to those observed in single sections, which would not support the first hypothesis. The log normal distribution of Aβ deposit size suggests a model in which the rate of growth of a deposit is proportional to its volume. However, mean deposit size and the ratio of large to small deposits were not positively correlated with patient age or disease duration. The frequency distribution of Aβ deposits which were closely associated with 0, 1, 2, 3, or more neuronal cell bodies deviated significantly from a log normal distribution, which would not support the neuronal origin hypothesis. On the basis of the present data, growth and resolution of Aβ deposits would appear to be the most likely explanation for the log normal size distributions.

What determines the size frequency distribution of β-amyloid (Aβ) deposits in Alzheimer's disease patients?

The factors determining the size of individual β-amyloid (A,8) deposits and their size frequency distribution in tissue from Alzheimer's disease (AD) patients have not been established. In 23/25 cortical tissues from 10 AD patients, the frequency of Aβ deposits declined exponentially with increasing size. In a random sample of 400 Aβ deposits, 88% were closely associated with one or more neuronal cell bodies. The frequency distribution of (Aβ) deposits which were associated with 0,1,2,...,n neuronal cell bodies deviated significantly from a Poisson distribution, suggesting a degree of clustering of the neuronal cell bodies. In addition, the frequency of Aβ deposits declined exponentially as the number of associated neuronal cell bodies increased. Aβ deposit area was positively correlated with the frequency of associated neuronal cell bodies, the degree of correlation being greater for pyramidal cells than smaller neurons. These data suggested: (1) the number of closely adjacent neuronal cell bodies which simultaneously secrete Aβ was an important factor determining the size of an Aβ deposit and (2) the exponential decline in larger Aβ deposits reflects the low probability that larger numbers of adjacent neurons will secrete Aβ simultaneously to form a deposit. © 1995.

An examination of the calcium and strontium site distribution in bioactive glasses through isomorphic neutron diffraction, X-ray diffraction, EXAFS and multinuclear solid state NMR.

Strontium has been substituted for calcium in the glass series (SiO2)49.46(Na2O)26.38(P2O5)1.07(CaO)23.08x(SrO)x (where x = 0, 11.54, 23.08) to elucidate their underlying atomic-scale structural characteristics as a basis for understanding features related to the bioactivity. These bioactive glasses have been investigated using isomorphic neutron and X-ray diffraction, Sr K-edge EXAFS and solid state 17O, 23Na, 29Si, 31P and 43Ca magic-angle-spinning (MAS) NMR. An effective isomorphic substitution first-order difference function has been applied to the neutron diffraction data, confirming that Ca and Sr behave in a similar manner within the glass network, with residual differences attributed to solely the variation in ionic radius between the two species. The diffraction data provides the first direct experimental evidence of split Ca–O nearest-neighbour correlations in these melt quench bioactive glasses, together with an analogous splitting of the Sr–O correlations; the correlations are attributed to the metal ions correlated either to bridging or to non-bridging oxygen atoms. Triple quantum (3Q) 43Ca MAS NMR corroborates the split Ca–O correlations. Successful simplification of the 2 < r (A) < 3 region via the difference method has also revealed two distinct Na environments. These environments are attributed to sodium correlated either to bridging or to nonbridging oxygen atoms. Complementary multinuclear MAS NMR, Sr K-edge EXAFS and X-ray diffraction data supports the structural model presented. The structural sites present will be intimately related to their release properties in physiological fluids such as plasma and saliva, and hence the bioactivity of the material. Detailed structural knowledge is therefore a prerequisite for optimising material design.