Andersen-Tawil Syndrome

Andersen-Tawil syndrome (ATS) is a rare condition consisting of ventricular arrhythmias, periodic paralysis, and dysmorphic features. In 2001, mutations in KCNJ2, which encodes the α subunit of the potassium channel Kir2.1, were identified in patients with ATS. To date, KCNJ2 is the only gene implicated in ATS, accounting for approximately 60% of cases. ATS is a unique channelopathy, and represents the first link between cardiac and skeletal muscle excitability. The arrhythmias observed in ATS are distinctive; patients may be asymptomatic, or minimally symptomatic despite a high arrhythmia burden with frequent ventricular ectopy and bidirectional ventricular tachycardia. However, patients remain at risk for life-threatening arrhythmias, including torsades de pointes and ventricular fibrillation, albeit less commonly than observed in other genetic arrhythmia syndromes. The characteristic heterogeneity at both the genotypic and phenotypic levels contribute to the continued difficulties with appropriate diagnosis, risk stratification, and effective therapy. The initial recognition of a syndromic association of clinically diverse symptoms, and the subsequent identification of the underlying molecular genetic basis of ATS has enhanced both clinical care, and our understanding of the critical function of Kir2.1 on skeletal muscle excitability and cardiac action potentia

A comparison of the bacterial contamination of the surface of cleanroom operators’ garments following donning with and without gloves.

Background: Specialist cleanroom garments are a potential vector for transmission of microorganisms within these facilities. In order to maintain the low bioburden of such clothing it has been perceived best practice for operators to dress wearing sterile cleanroom gloves. However, the efficacy of such glove use upon the resultant bacterial contamination of the surface of cleanroom garments has not previously been evaluated. Aim: To compare surface bacterial contamination of cleanroom garments following their donning by operators dressing with or without gloves. Methods: Following prior handwashing and systematic donning of cleanroom clothing by operators dressing wearing either no gloves, non-sterile nitrile gloves or sterile cleanroom latex gloves, a direct agar contact method was immediately undertaken to test garment surfaces at 7 specific sites. Following incubation bacterial levels were suitably quantified. Findings: Comparing levels of growth displayed on plates used to test the surface of cleanroom garments worn by operators dressing with no gloves, non–sterile gloves or sterile cleanroom gloves, no significant difference was observed between the percentage of contact plates displaying growth and the levels of growth observed on plates, from any of the sites tested. Conclusion: Omission of gloves in line with a systematic handwashing procedure prior to the cleanroom garment donning process, may result in modest economic and environmental gain coupled with a slightly less burdensome procedure. However, this is reliant on rigorous adherence to handwashing protocol and assessment of associated risk factors

Gender influences bacterial contamination of reusable cleanroom operators’ garments following wear.

Background: Operators are the primary source of cleanroom contamination, with the majority of their detritus identified as skin squames and their associated microorganisms. To reduce contamination, operatives are required to wear a specific arrangement of specialist garments. However, bacteria can evade this clothing and tarnish outer surfaces whilst operators work, with adverse implication for cleanroom environment and product sterility. Gender plays a significant role in bacterial dispersion, with male rates being in excess of female counterparts. Currently there is a lack of published literature evaluating the effect of gender on contamination of cleanroom garments. Such information would assist cleanroom facilities to more robustly assess and mitigate operator-associated contamination risks. Aim: To compare bacterial contamination on the surface of cleanroom operators’ garments, specifically with respect to gender. Method: Levels of bacteria on garments worn by male and female operators working under two conditions (30 minutes: Grade A/B cleanroom and 60 minutes: Grade C cleanroom) were compared. Immediately following the operators’ exit from the cleanroom, a direct agar contact method was undertaken at several sites on the surface of their garments. Findings: Bacteria were recovered from the surface of garments worn by both genders. Bacterial levels on garments worn by male operators were almost always in excess of those worn by females at all sites tested (Percentage of plates displaying growth: Grade A/B – 83.9%/63.3% and Grade C – 86.1%/70.1%, respectively) [*p[less than]0.05]. Regardless of gender, bacterial levels at the chest and posterior cervicis region of suits were reduced with the donning of a hood, covering the head. Conclusions: Gender plays a significant role in bacterial contamination of cleanroom garment surfaces, with bacteria on the surface of clothing worn by males being in excess of that on garments worn by female counterparts. In addition, the donning of a hood reduces bacterial numbers on suits. These findings add to the limited body of knowledge examining bacterial contamination of cleanroom garments and contribute towards understanding operator-associated contamination risks within cleanroom facilities

A Chandra X-ray Study of Cygnus A - II. The Nucleus

We report Chandra ACIS and quasi-simultaneous RXTE observations of the nearby, powerful radio galaxy Cygnus A, with the present paper focusing on the properties of the active nucleus. In the Chandra observation, the hard (> a few keV) X-ray emission is spatially unresolved with a size \approxlt 1 arcsec (1.5 kpc, H_0 = 50 km s^-1 Mpc^-1) and coincides with the radio and near infrared nuclei. In contrast, the soft (< 2 keV) emission exhibits a bi-polar nebulosity that aligns with the optical bi-polar continuum and emission-line structures and approximately with the radio jet. In particular, the soft X-ray emission corresponds very well with the [O III] \lambda 5007 and H\alpha + [N II] \lambda\lambda 6548, 6583 nebulosity imaged with HST. At the location of the nucleus there is only weak soft X-ray emission, an effect that may be intrinsic or result from a dust lane that crosses the nucleus perpendicular to the source axis. The spectra of the various X-ray components have been obtained by simultaneous fits to the 6 detectors. The compact nucleus is detected to 100 keV and is well described by a heavily absorbed power law spectrum with \Gamma_h = 1.52^{+0.12}_{-0.12} (similar to other narrow line radio galaxies) and equivalent hydrogen column N_H (nuc) = 2.0^{+0.1}_{-0.2} \times 10^{23} cm^-2. (Abstract truncated).Comment: To be published in the Astrophysical Journal, v564 January 1, 2002 issue; 34 pages, 11 figures (1 color

US Renewable Futures in the GCAM

This project examines renewable energy deployment in the United States using a version of the GCAM integrated assessment model with detailed a representation of renewables, the GCAM-RE. Electricity generation was modeled in four generation segments and 12-subregions. This level of regional and sectoral detail allows a more explicit representation of renewable energy generation. Wind, solar thermal power, and central solar PV plants are implemented in explicit resource classes with new intermittency parameterizations appropriate for each technology. A scenario analysis examines a range of assumptions for technology characteristics, climate policy, and long-distance transmission. We find that renewable generation levels grow over the century in all scenarios. As expected, renewable generation increases with lower renewable technology costs, more stringent climate policy, and if alternative low-carbon technology are not available. The availability of long distance transmission lowers policy costs and changes the renewable generation mix

Obesity and the food environment: income and ethnicity differences among people with diabetes: the Diabetes Study of Northern California (DISTANCE).

ObjectiveIt is unknown whether any association between neighborhood food environment and obesity varies according to individual income and/or race/ethnicity. The objectives of this study were to test whether there was an association between food environments and obesity among adults with diabetes and whether this relationship differed according to individual income or race/ethnicity.Research design and methodsSubjects (n = 16,057) were participants in the Diabetes Study of Northern California survey. Kernel density estimation was used to create a food environment score for each individual's residence address that reflected the mix of healthful and unhealthful food vendors nearby. Logistic regression models estimated the association between the modeled food environment and obesity, controlling for confounders, and testing for interactions between food environment and race/ethnicity and income.ResultsThe authors found that more healthful food environments were associated with lower obesity in the highest income groups (incomes 301-600% and &gt;600% of U.S. poverty line) among whites, Latinos, and Asians. The association was negative, but smaller and not statistically significant, among high-income blacks. On the contrary, a more healthful food environment was associated with higher obesity among participants in the lowest-income group (&lt;100% poverty threshold), which was statistically significant for black participants in this income category.ConclusionsThese findings suggest that the availability of healthful food environments may have different health implications when financial resources are severely constrained

The CO\u3csub\u3e2\u3c/sub\u3e Concentration of Air Trapped in Greenland Ice Sheet Project 2 Ice Formed During Periods of Rapid Climate Change

The CO2 content of air occluded in Greenland Ice Sheet Project 2 (GISP2) ice formed over two separate intervals of rapidly changing climate, centered at approximately 46 and 63 kyr B. P., is as much as 90 ppm more during warm periods (interstadials) than during cold periods (stadials). These CO2 variations are superimposed on changes in annual layer thickness and δ18O of the ice and do not show the 200- to 700-year offsets which would be expected for concurrent variations in the atmosphere and the ice. The CO2 concentrations during the stadials are similar to the atmospheric values recorded by Antarctic ice of the same age, so processes occurring in the ice after bubble enclosure must be enriching the air trapped in GISP2 ice formed during the interstadials. This conclusion is supported by Ca content and electrical conductivity measurements of the ice, which show that adequate carbonate is present to produce these enrichments and that CO2 content is high only when the electrical conductivity (a proxy for H+ concentration) is high. High-resolution mapping of one 4-cm section of ice shows a 200-ppm increase in the CO2 content of the trapped air, from approximately 275 to 475 ppm. Analyses of the total inorganic carbon of ice from both the LGM and Holocene show that most of the Ca in the ice is from CaCO3 and that the δ13CO2 approaches that of soil and marine carbonates. These results show that the CO2 record preserved in ice can be altered by in situ decarbonation reactions and that only ice containing either abundant carbonate or essentially no carbonate contains a reliable record of paleoatmospheric CO2

The effect of boron diffusions on the defect density and recombination at the (111) silicon-silicon oxide interface

A combination of electron paramagnetic resonance(EPR) and minority carrier lifetime measurements is used to unambiguously demonstrate that the presence of a B diffusion layer at the surface of oxidized Si (111) wafers causes a significant increase in the interface defect density as well as interface recombination, compared to undiffused surfaces.EPRmeasurements show a nearly three-fold increase in the Pb center density, while the lifetime measurements indicate an increase in surface recombination activity by a factor of more than two, for B diffused samples with a sheet resistance of ∼250Ω∕□□

Superheating fields of superconductors: Asymptotic analysis and numerical results

The superheated Meissner state in type-I superconductors is studied both analytically and numerically within the framework of Ginzburg-Landau theory. Using the method of matched asymptotic expansions we have developed a systematic expansion for the solutions of the Ginzburg-Landau equations in the limit of small $\kappa$, and have determined the maximum superheating field $H_{\rm sh}$ for the existence of the metastable, superheated Meissner state as an expansion in powers of $\kappa^{1/2}$. Our numerical solutions of these equations agree quite well with the asymptotic solutions for $\kappa<0.5$. The same asymptotic methods are also used to study the stability of the solutions, as well as a modified version of the Ginzburg-Landau equations which incorporates nonlocal electrodynamics. Finally, we compare our numerical results for the superheating field for large-$\kappa$ against recent asymptotic results for large-$\kappa$, and again find a close agreement. Our results demonstrate the efficacy of the method of matched asymptotic expansions for dealing with problems in inhomogeneous superconductivity involving boundary layers.Comment: 14 pages, 8 uuencoded figures, Revtex 3.

THE EFFECTS OF DIET INDUCED OBESITY ON THE FORCE-LENGTH RELATIONSHIP IN RAT SOLEUS

INTRODUCTION Obesity is associated with chronic inflammation, which has been shown to affect the integrity of musculoskeletal tissues [1]. Previous data from our group suggests that obesity can result in intramuscular fat deposition [1]. It is unclear if this structural alteration has functional consequences, as the implications of obesity on muscle mechanics are not well understood. Therefore, the purpose of this study was to quantify the active force produced by soleus muscles of obese and non-obese rats at a range of muscle lengths. As the inclusion of fat into the muscle fibers will leave less room for contractile proteins, we hypothesized that obese rats will produce lower forces normalized to muscle mass at every length than non-obese control rats.   METHODS Fourteen rats were randomly allocated to a 12-week diet: either an obesity-inducing high fat high sucrose diet (DIO, 40% fat, 45% sucrose, n=8) or a standard chow diet (chow, 12% fat 0% sucrose, n=6). Prior to surgery, body composition was evaluated using dual energy X-ray absorptiometry. Custom-made tibial nerve cuffs were surgically attached to the right tibial nerve of each animal. The soleus was exposed, mechanically isolated, and clamped to a force transducer. The muscle was then stretched to a predetermined length and electrically stimulated at 3 times the motor unit threshold (50Hz) and the force output was measured [3]. Force tracings were digitized using WINDAQ® software. Passive, active, and total forces produced by the soleus were normalized to the maximum in vivo length of each animal. Forces were averaged into 5% length intervals within each animal. Students t-tests or a two-way ANOVA were conducted between groups, and a Bonferroni correction was used as needed, α=0.05. RESULTS DIO rats had increased body mass (DIO 816.4 ± 30.1g, chow 645.0 ± 28.3g; p&lt;0.05) and body fat (DIO 39.2 ± 1.3%, chow 21.8 ± 2.1%; p&lt;0.05) compared to chow-fed rats. Soleus mass (DIO: 0.28 ± 0.01 g, chow: 0.26 ± 0.11 g, p=0.32), was similar between the two groups. Absolute peak isometric force was similar between the two groups (DIO: 2.58 ± 0.10 N, chow: 2.18 ± 0.34 N, p=0.23). Active isometric force normalized to soleus mass was significantly higher in DIO group rats at every muscle length (Figure 1, p&lt;0.05). DISCUSSION AND CONCLUSIONS On average, DIO rats produced more active force at a given normalized length and soleus mass than chow rats, a finding that refutes our original hypothesis. Since optimal length occurs at the same relative muscle length for both groups, and since the decline in force from maximum is similar between groups, it appears that fascicle length, and an associated shift in the force-length relationship cannot explain our results. Results of differences in the force-velocity relationship (not shown here) suggest that the DIO rats may have a higher proportion of fast twitch fibres, but the relative force among slow and fast fibres is similar, and thus also should not affect these results. The results suggest that the force per cross-sectional area is higher in muscles from obese compared to lean rats, a finding that defies explanation at this time and needs thorough investigation in the future. Histology and tests looking at fibre and cell level muscle structures may provide more insight