cssi/SciSciGPT-SciSciNet huggingface dataset:
Pinus nigra -Sphaeropsis sapinea as a model pathosystem to investigate local and systemic effects of fungal infection of pines

The Austrian pine/Sphaeropsis sapinea pathosystem was tested as a model to study the secondary metabolism of both localized and systemically induced defense responses in pine. Compared to wounding alone, fungal inoculations in lower stems of five-year-old potted Austrian pines induced significant ðP , 0:01Þ depletion or accumulation (0.4– 101-fold) in reaction zone phloem of several soluble and cell-wall-bound phenolics, and a 1.8-fold increase in lignin deposition. Inoculations also resulted in significant ðP , 0:05Þ accumulation (up to 3-fold) of several compounds in the phloem 25 cm above the inoculation site, indicating systemic induction of metabolic pathways. Taxifolin was among the induced metabolites, but was only observed in lower stems, irrespective of treatment. Another putative flavonoid was observed only when trees were inoculated with live mycelia, suggesting a phytoalexin-like role for this compound. In spite of these changes, lignin content and concentrations of several metabolites considered important in resistance (such as stilbenes) were either not correlated, or were positively correlated, with lesion size. Only three metabolites had negative correlations with lesion size, suggesting a potential role in resistance. Protein and lipid fractions of mycelial extracts, as well as killed mycelium of the pathogen, were also examined as potential elicitors. Among the treatments other than live inoculation, killed mycelium was the strongest inducer of host responses, followed by the protein extract. Lipids did not elicit host responses different from wounding alone. q 2004 Elsevier Ltd. All rights reserved.
IMPT1, an imprinted gene similar to polyspecific transporter and multi-drug resistance genes.

Human chromosome 11p15.5 and distal mouse chromosome 7 include a megabase-scale chromosomal domain with multiple genes subject to parental imprinting. Here we describe mouse and human versions of a novel imprinted gene, IMPT1 , which lies between IPL and p57 KIP2 and which encodes a predicted multi-membrane-spanning protein similar to bacterial and eukaryotic polyspecific metabolite transporter and multi-drug resistance pumps. Mouse Impt1 and human IMPT1 mRNAs are highly expressed in tissues with metabolite transport functions, including liver, kidney, intestine, extra-embryonic membranes and placenta, and there is strongly preferential expression of the maternal allele in various mouse tissues at fetal stages. In post-natal tissues there is persistent expression, but the allelic bias attenuates. An allelic expression bias is also observed in human fetal and post-natal tissues, but there is significant interindividual variation and rare somatic allele switching. The fact that Impt1 is relatively repressed on the paternal allele, together with data from other imprinted genes, allows a statistical conclusion that the primary effect of human chromosome 11p15.5/mouse distal chromosome 7 imprinting is domain-wide relative repression of genes on the paternal homolog. Dosage regulation of the metabolite transporter gene(s) by imprinting might regulate placental and fetal growth.
Electric field processing to control the structure of poly(vinylidene fluoride) composite proton conducting membranes

A novel method is reported for controlling the structure of poly(vinylidene fluoride) (PVdF) composite proton conducting membranes. When proton conducting Nafion or zirconium phosphate sulfophenylenphosphonate (ZrPSPP) particles are dispersed in a mixed colloidal suspension with PVdF particles, the proton conducting particles selectively respond to an applied electric field. Under appropriate conditions, the proton conducting particles are induced to assemble into chains that rapidly grow to span the gap between electrodes as the electric field is applied. By removing the solvent and melting the PVdF phase while applying the electric field, composite membranes were formed that have field-induced structure. In comparison to randomly structured composites, the electric field-processed Nafion/PVdF or ZrPSPP/PVdF composite membranes showed improved proton conductivity, water sorption, selectivity for protons over methanol, and controlled surface area changes upon swelling with water. The transport and mechanical properties of the electric field-processed composite membranes suggest the potential for improved performance in direct methanol fuel cells.
Performance of turbo codes over magnetic recording channels

We study the performance of turbo codes for magnetic recording channels using realistic channel models. The main objective is to provide a benchmark for the future studies on the subject. We examine a variety of schemes including the use of parallel concatenated turbo codes with and without turbo equalization and convolutional codes with an interleaver (can be regarded as a serial concatenation of convolutional codes with the partial response channel). We investigate the effect of the (turbo) code block length, the use of different code structures, and the effects of precoding and the media noise on the system performance. We assume that the partial response target is EPR4.
Comments on ''arithmetic coding as a non-linear dynamical system''

Abstract Nagaraj et al. [1] , [2] present a skewed-non-linear generalized Luroth Series (s-nGLS) framework. S-nGLS uses non-linear maps for GLS to introduce a security parameter a which is used to build a keyspace for image or data encryption. The map introduces non-linearity to the system to add an “encryption key parameter”. The skew is added to achieve optimal compression efficiency. s-nGLS used as such for joint encryption and compression is a weak candidate, as explained in this communication. First, we show how the framework is vulnerable to known plaintext based attacks and that a key of size 256 bits can be broken within 1000 trials. Next, we demonstrate that the proposed non-linearity exponentially increases the hardware complexity of design. We also discover that s-nGlS cannot be implemented as such for large bitstreams. Finally, we demonstrate how correlation of key parameter with compression performance leads to further key vulnerabilities.
Community ecology theory as a framework for biological invasions

Abstract Community ecology theory can be used to understand biological invasions by applying recent niche concepts to alien species and the communities that they invade. These ideas lead to the concept of ‘niche opportunity', which defines conditions that promote invasions in terms of resources, natural enemies, the physical environment, interactions between these factors, and the manner in which they vary in time and space. Niche opportunities vary naturally between communities but might be greatly increased by disruption of communities, especially if the original community members are less well adapted to the new conditions. Recent niche theory clarifies the prediction that low niche opportunities (invasion resistance) result from high species diversity. Conflicting empirical patterns of invasion resistance are potentially explained by covarying external factors. These various ideas derived from community ecology provide a predictive framework for invasion ecology.
Congenital Cholesteatoma: Theories, Facts, and 53 Patients

Congenital cholesteatoma has a different pathophysiology than acquired cholesteatoma in that these patients rarely have eustachian tube dysfunction. This likely accounts for their reasonable preoperative hearing and their lack of complications or recurrences postoperatively. The most important factor is early detection. Treatment remains surgical removal.
Predicting development of anticipatory nausea in cancer patients: prospective examination of eight clinical characteristics.

Abstract An algorithm based on learning theory and previous research for predicting which cancer patients would develop nausea in anticipation of chemotherapy treatments was tested. Patients with four or more of the following eight characteristics after their first treatment were predicted to develop anticipatory nausea (AN) by their fourth chemotherapy treatment: experienced nausea and/or vomiting after first treatment; nausea after treatment described as “moderate, severe, or intolerable;” vomiting after treatment described as “moderate, severe, or intolerable;” less than 50 yr of age; a susceptibility to motion sickness; feeling warm or hot all over after treatment; sweating following treatment; feelings of generalized weakness following treatment. The characteristics significantly predicted subsequent anticipatory nausea development (p
The War on Poverty: Measurement, Trends, and Policy.

We present a 50-year historical perspective of the nation's antipoverty efforts, describing the evolution of policy during four key periods since 1965. Over this half-century, the initial heavy reliance on cash income support to poor families has eroded; increases in public support came largely in the form of in-kind (e.g., Food Stamps) and tax-related (e.g., the Earned Income Tax Credit) benefits. Work support and the supplementation of earnings substituted for direct support. These shifts eroded the safety net for the most disadvantaged in American society. Three poverty-related analytical developments are also described. The rise of the Supplemental Poverty Measure (SPM)—taking account of noncash and tax-related benefits—has corrected some of the serious weaknesses of the official poverty measure (OPM). The SPM measure indicates that the poverty rate has declined over time, rather than being essentially flat as the OPM implies. We also present snapshots of the composition of the poor population in the United States using both the OPM and the SPM, showing progress in reducing poverty overall and among specific socioeconomic subgroups since the beginning of the War on Poverty. Finally, we document the expenditure levels of numerous antipoverty programs that have accompanied the several phases of poverty policy and describe the effect of these efforts on the level of poverty. Although the effectiveness of government antipoverty transfers is debated, our findings indicate that the growth of antipoverty policies has reduced the overall level of poverty, with substantial reductions among the elderly, disabled, and blacks. However, the poverty rates for children, especially those living in single-parent families, and families headed by a low-skill, low-education person, have increased. Rates of deep poverty (families living with less than one-half of the poverty line) for the nonelderly population have not decreased, reflecting both the increasing labor market difficulties faced by the low-skill population and the tilt of means-tested benefits away from the poorest of the poor.
Topiramate in the treatment of severe bulimia nervosa with comorbid mood disorders: a case series.

Objective To report observations on the efficacy and tolerability of topiramate in a sample of five patients with severe symptoms of bulimia nervosa and comorbid mood and/or anxiety disorders. Method Topiramate was added to other psychotropic medication under open-label conditions up to the maximum tolerated dose or until remission of the eating disorder was achieved. Results Topiramate almost completely eliminated binging and purging behavior in three of the five patients. Improvement was maintained throughout the period of follow-up for up to 18 months. One patient showed a partial, temporary response, and the fifth was intolerant of the drug and unable to complete an adequate trial. Discussion These results suggest strongly that the efficacy of topiramate in patients with bulimia nervosa with and without comorbid mood and anxiety disorders should be investigated more fully. © 2003 by Wiley Periodicals, Inc. Int J Eat Disord 33: 468–472, 2003.
ECONOMIC SEGMENTATION AND RACIAL ATTITUDES

This article explores the potential contributions of the economic segmentation framework in stratification research to the explanation of racial attitudes and beliefs. Employing data from a nationally representative telephone survey, the impact of sectoral location on whites’ views about the institutional production of racial inequality is examined. The findings indicate that, unlike the effects of traditional stratification variables, position in the segmented economy exerts no unique influence upon racial orientation.
An earthquake swarm on the Chagos-Laccadive Ridge and its tectonic implications

Summary. An unusual, isolated swarm of earthquakes occurred on the Chagos-Laccadive Ridge in the Central Indian Ocean between 1965 and 1970. Sixteen earthquakes were .located at approximately 6.0" S, 7 1.3" E on the steep west facing scarp of the Chagos Bank. This swarm forms the only major seismic activity in the period 1963-76 on the otherwise aseismic ridge. The mechanisms of the three largest earthquakes were studied using bodyand surface-wave data. All are extremely similar shallow normal fault events on an east-west fault plane. Such faulting is difficult to reconcile with that expected from either the trend of the Chagos-Laccadive Ridge or the spreading direction at the nearby Central Indian Ridge. The swarm may have occurred at depth on a cross fracture remaining from the breakup of the Chagos Bank and the Mascarene Plateau and the formation of the present Central Indian Ridge.
Microchondrules in two unequilibrated ordinary chondrites: Evidence for formation by splattering from chondrules during stochastic collisions in the solar nebula

The diversity of silicate, glassy spherules analogous to chondrules, called microchondrules, and the implications for their presence in unequilibrated ordinary chondrites (UOCs) were investigated using different electron microscope techniques. Our observations show that the abundance of microchondrules in UOCs is much larger than the values proposed by previous studies. We identified two different types of microchondrules, porous and nonporous, embedded within fine-grained matrices and type I chondrule rims. The porous microchondrules are characterized by distinctive textures and chemical compositions that have not been recognized previously. Additionally, we show detailed textures and chemical compositions of protuberances of silicate materials, connected to the chondrules and ending with microchondrules. We suggest that microchondrules and protuberances formed from materials splattered from the chondrules during stochastic collisions when they were still either completely or partially molten. The occurrence and distinct morphologies of microchondrules and protuberances suggest that rather than just a passive flash melting of chondrules, an additional event perturbed the molten chondrules before they underwent cooling. The bulk chemical compositions suggest that (1) nonporous microchondrules and protuberances were formed by splattering of materials that are compositionally similar to the bulk silicate composition of type I chondrules, and (2) the porous microchondrules could represent the splattered melt products of a less evolved, fine-grained dust composition. The preservation of protuberances and microchondrules in the rims suggests that the cooling and accretion rates were exceptionally fast and that they represent the last objects that were formed before the accretion of the parent bodies of OCs.
Brief exposure to secondhand smoke reversibly impairs endothelial vasodilatory function.

Introduction: We sought to determine the effects of brief exposures to low concentrations of tobacco secondhand smoke (SHS) on arterial flow-mediated dilation (FMD, a nitric oxide-dependent measure of vascular endothelial function), in a controlled animal model never before exposed to smoke. In humans, SHS exposure for 30min impairs FMD. It is important to gain a better understanding of the acute effects of exposure to SHS at low concentrations and for brief periods of time. Methods: We measured changes in FMD in rats exposed to a range of real-world levels of SHS for durations of 30min, 10min, 1min, and 4 breaths (roughly 15 s). Results: We observed a dose-response relationship between SHS particle concentration over 30min and post-exposure impairment of FMD, which was linear through the range typically encountered in smoky restaurants and then saturated at higher concentrations. One min of exposure to SHS at moderate concentrations was sufficient to impair FMD. Conclusions: Brief SHS exposure at real-world levels reversibly impairs FMD. Even 1min of SHS exposure can cause reduction of endothelial function.
Joining the Family: Experiences of Being and Becoming Ally Activists of LGBTQ People

Issues surrounding the full-inclusion and rights of lesbian, gay, bisexual, transgender, and queer (LGBTQ) people are at the forefront of many political discussions. People who are straight-identif...
Changing hafnium speciation in aqueous sulfate solutions: a high-energy X-ray scattering study.

The relationship of solution speciation and the structures of corresponding precipitates is examined for an aqueous Hf(4+) sulfate series. High-energy X-ray scattering (HEXS) and Raman spectroscopy data are used to probe atomic correlations in solutions. Hf(4+) in acidic perchlorate solution shows no evidence of a mononuclear metal species but instead has a peak in the pair-distribution function (PDF), generated from the HEXS data, at 3.55 A, indicating Hf(4+)-Hf(4+) solution correlations. The peak intensity is consistent with clusters that are, on average, larger than the tetramic unit [M4(OH)8(H2O)16](8+) usually attributed to Zr(4+) and Hf(4+) solution speciation under these conditions. Addition of sulfate results in a breakup of hydroxo-bridged oligomers into sulfate-capped dimers and, for higher concentrations, Hf-sulfate monomers. The bidentate coordination mode of sulfate dominates the dissolved precursors, although it is not found in the structure of the final crystallized product, which instead is comprised of bridging-bidentate sulfate ligation. Neither the PDF patterns nor the Raman spectra show any evidence of the larger oligomers, such as the octadecameric metal clusters, found in similar Zr(4+) solutions. The oligomeric units found in solution provide insights into possible assembly routes for crystallization. In addition to expanding our understanding of synthesis science this study also reveals differences in the aqueous chemistries between Hf and Zr, two elements with ostensibly very similar chemical behavior.
Computer Simulation of Thermoregulatory Responses to Heat Stress: A Future Work Design and Training Tool

A mathematical model of human thermoregulation was developed to simulate thermoregulatory responses of man exposed to heat stress environments. The model was validated with experimental results of a man pedalling in environments of 35 and 45°C dry-bulb and 33 mm Hg water vapor pressure.The model inputs of body segment temperatures, thermal characteristics, basal blood flows, basal heat production, and of environmental and task characteristics permitted the model to be “individualized” for a specific subject, environment, and task.The model outputs of body segment temperatures, blood flows, and sweat loss at designated intervals permitted observation of the dynamic thermoregulatory responses over a 60 minute exposure period.The model was successful in predicting the change in thermal responses of a clothed man working in heat stress environments. Over the 60 minute exposure at 45°C the mean absolute difference between experimental and simulated values of head skin temperature was 0.28°C. The difference for...
Becoming an ‘autonomous writer’: Epistemic stance displays and membership categorization in the writing conference

Although the members’ categories associated with institutional settings may seem obvious, more can be said about the actions that make them noticeable and persistent across contexts. This study inv...
Pneumococcal vaccine in the prevention of community-acquired pneumonia: a skeptical view of cost-effectiveness.

Streptococcus pneumoniae (pneumococcus) is an important pathogen that causes pneumonia, bacteremia, and meningitis in adults. In recent years, pneumococcal isolates resistant to penicillin have become increasingly prevalent in the U.S. For these reasons, wide use of the pneumococcal vaccine has been advocated. Pneumococcal vaccines have been proven to be effective in preventing the invasive complications of S pneumoniae infection. The efficacy of pneumococcal vaccines in preventing non-bacteremic pneumonia in high-risk subjects, however, has not been proven. The current pneumococcal vaccine is limited because it includes the antigens of the serotypes known to be associated with invasive infection, and it elicits a thymus-independent, B-cell response that evokes no memory and a poor immunologic response in many of the patients at greatest risk for pneumococcal infection. In the absence of data concerning efficacy of the pneumococcal vaccine against pneumonia, it is difficult to argue that it is cost-effective for this purpose. I concur with the recommendations for use of pneumococcal vaccine to prevent bacteremia, but I am skeptical about the vaccine's efficacy against pneumonia. More data and an improved vaccine are necessary.
Plasma stabilization conducting shells and their impact on TBR and activation in CLiFF design

Plasma stabilization and plasma elongation are best achieved by keeping a stabilizing shell as close as possible to the plasma. In CLiFF design, a 2-cm-thick flowing liquid layer is placed in front of a solid FW and is thought to be used as an active conduction shell if its conductance is relatively high such as with liquid lithium. On the other hand, higher conductance is achieved by solid shells (e.g. Cu, Al, FS, W, V alloy). In the present study, the adverse effect of this stabilizing shell (whether it is liquid or solid material) on tritium breeding ratio (TBR) is investigated. Among the design features that quantify this effect are: the type of breeder and structure, the degree of Li-6 enrichment, the material and thickness of the shell, and whether or not there is a front beryllium multiplying zone in the blanket. Additionally, the presence of a solid conducting shell near the FW will impose a safety concern in the case of LOCA. The present study addresses this concern and comparison of the level of decay heat and waste disposal rating is made among the candidate materials for the stabilizing shell.
LGBT Older Adults and Nurse Administrators: An Opportunity for Advocacy

One of the greatest health care challenges of the 21st century is the rapidly growing number of older adults in the United States. This aging population is also becoming increasingly diverse, and with this diversity comes an increased number of older adults who identify as lesbian, gay, bisexual, and transgender (LGBT). The needs and health outcomes of this specific subgroup of the older adult population cannot be extrapolated from the more general population of older adults. Nurses have the opportunity to lead health care providers in improving care for this vulnerable and sometimes invisible population. Leading this charge will require nurse executives who advocate, create care environments that are inclusive, and staff with nurses who can care for the specific needs of LGBT older adults. The purpose of this article is to raise awareness of the health needs of LGBT older adults and identify how nurse executives can advocate to improve care for this overlooked population.
Religion and Social Work Practice

Practitioners identified religious and spiritual issues that emerge in social work practice and that are salient factors during various life stages. The data suggest that attention should be direct...
Prognostic factors and patterns of failure in advanced stage Hodgkin lymphoma treated with combined modality therapy.

Abstract Background and purpose The role of irradiation to non-bulky and bulky sites of disease in advanced stage Hodgkin lymphoma is controversial. We aimed to review the long-term outcomes of patients treated with combined modality therapy to clarify the role of consolidative radiotherapy. Materials and methods Patients with stage III or IV Hodgkin lymphoma treated with Stanford V chemotherapy and consolidative radiotherapy to initial sites of disease ≥5 cm were analyzed retrospectively to determine patient outcomes, patterns of failure, and factors associated with treatment failure. Results A total of 170 patients were analyzed. Overall survival was 91.2%, freedom from progression was 80.6%, and progression-free survival was 78.9% at 10 years. 5 patients (2.9%) had refractory disease and 27 patients (15.9%) relapsed after treatment. Only an International Prognostic Score (IPS) greater than 2 predicted disease progression. 19 out of 27 relapses occurred exclusively outside of the radiation treatment field, and 17 out of 27 relapses occurred exclusively at original sites of disease. However, only 11 of 170 patients (6.5%) relapsed exclusively at original, non-bulky sites of disease not treated with radiation therapy. The cumulative incidence of local failure at 10 years was 4.6% for unirradiated sites and 2.6% for irradiated sites. Conclusion Patients with advanced stage Hodgkin lymphoma treated with combined modality therapy including consolidative radiotherapy to bulky disease sites had excellent long-term outcomes. Given the low frequency of isolated failures at initial sites, our results suggest that selective radiation therapy to sites at high risk of relapse may be feasible.
A Cross Section Estimate of Translog Production Function: Jordanian Manufacturing Industry

The objective of this paper is to estimate the Transcendental Logarithmic Production Function of manufacturing industry in Jordanian economy. Manufacturing industry can be considered as the fourth large one in Jordanian economy. In last decade, the growth rate in this sector is the second one after transport and communications. The gross fixed capital formation in manufacturing is the third one. In this paper we need a cross sectional data for cost shares, factor inputs, factor prices, and output. Main source of data for manufacturing industry in Jordan is the Industry Survey Department Statistics the Hashemite Kingdom of Jordan. The data for factor inputs and output were transferred to logarithm. A point around which production function should be estimated was chosen. Cost shares of inputs have been calculated by dividing compensations to employees, operating surplus, and value of materials by value manufacturing output. The theoretical framework in this research is the flexible production function. S0, the thee-input transcendental logarithmic (Translog) production function could be used. This function is approximated by second order Taylor series. The log of likelihood ratio test has been used to choose among different hypotheses. The symmetric translog hypothesis has been found as an appropriate one among other hypotheses. It is well behaved, where positivity and concavity of production function have been satisfied. After testing for nested hypotheses, the symmetry restrictions were be imposed. So, restricted and unrestricted functional forms are estimated. However, Iterative Zellner-Efficient Estimate has been used to get estimates equivalent to the maximum likelihood estimates. Allen partial elasticities of substitution, AES, and own and cross price elasticities for factor inputs have been calculated. AES measure the substitutability or complement among factors of production. On the other hand, AES can measure the curvature of isoquant. AES shows that capital-labor, capital–materials, and labor-materials are all substitutive. But, AES for labor-materials is more than a half of that for capital-labor. AES for capital-labor is one and three fourth of that for labor-materials. The Price elasticities of factor inputs show that capital and labor demand are more elastic than demand for materials. JEL Classification Codes: D2
The Patent Prosecution Highway: A Global Superhighway to Changing Validity Standards

This presentation explores the effect that work-share agreements amongst patent offices around the globe have on the validity of the resulting patents. The discussion seeks to survey the scope of current work-share programs, focusing on the Patent Prosecution Highway, and analyze the effect that such work-share programs have on the validity of patents issued through the corresponding processes. The analysis focuses on the presumption of validity in the United States and broadens to include Japan, Korea, and the member states of the European Union. Finally, we will go over the wide spectrum in quality of patent examiners throughout the PPH network and the relatively myopic tendencies of patent examiners, resulting in improvidently granted patents in the offices of second filing, as pertinent prior art will go unfound and unexamined in the push to issue patents quickly through the PPH programs.
Polymer film dewetting for fabrication of out-of-autoclave prepreg with high through-thickness permeability

Abstract Polymer film dewetting on a substrate (independent of fiber bed architecture) was explored, developed, and demonstrated as a method to produce out-of-autoclave, vacuum bag-only (OoA/VBO) prepregs with high transverse permeability and process robustness. The dimensions of the surface openings created by dewetting were measured, and the percent surface area exposed was calculated. Prepregs were fabricated with continuous and dewetted (discontinuous) films to produce trial laminates. The laminates were cured under both standard and sub-optimal conditions, and were characterized before, during, and after cure. Laminates fabricated with dewetted resin consistently achieved near-zero porosity. In contrast, laminates with continuous film displayed high levels of porosity, particularly during sub-optimal cure. The findings demonstrate that dewetting can be used effectively to produce OoA prepregs with high through-thickness permeability, which can yield porosity-free laminates via VBO processing. Furthermore, these results elucidate aspects of resin dewetting that are critical in the creation of robust OoA prepregs.
306 ARC SYNDROME: A RARE FORM OF ARTHROGRYPOSIS WITH MULTISYSTEM INVOLVEMENT.

ARC syndrome is a rare, autosomal recessive multisystem disorder characterized by arthrogryposis, renal tubular dysfunction, and neonatal cholestatic jaundice, which has been reported mainly in consanguineous families of Pakistani and Middle Eastern extraction. We present a 6-month-old male of Vietnamese extraction with ARC syndrome and a review of the literature. The patient initially presented with failure to thrive, contractures of the lower extremities bilaterally, and dry skin. Hypothyroidism had been noted on the newborn screen. Laboratory studies revealed giant platelets on a peripheral blood smear and a normal high-resolution chromosome study. Conjugated hyperbilirubinemia and renal tubular dysfunction were also identified, which prompted the diagnosis of ARC syndrome. The literature contains less than 100 ARC patients reported worldwide since the first report by Lutz-Richner and Landolt in 1973. Consistent findings include arthrogryposis of variable severity, renal dysfunction consisting of renal tubular acidosis and occasional nephrocalcinosis, and cholestatic liver disease. Less frequently reported features include ichthyosis, endocrine abnormalities, including hypothyroidism, and abnormal platelets, as demonstrated in our patient. Failure to thrive is an almost universal finding. Prognosis is poor, with most patients dying within the first year and the oldest reported patient surviving to 3 years. This diagnosis should be considered in a patient with arthrogryposis and multisystem involvement.
Achievement of Guided Growth in Children With Low-Tone Neuromuscular Early-Onset Scoliosis Using a Segmental Sublaminar Instrumentation Technique

Abstract Objectives/Summary of Background Data Segmental sublaminar spinal instrumentation without fusion results in guided growth and correction of deformity in early-onset scoliosis (EOS). The purpose of this study is to report the outcomes of a series of children with low-tone EOS that were treated surgically with a modification of a Luque trolley technique. Methods This study is a retrospective chart and radiographic review of a single-center series of 13 consecutive children who met inclusion criteria with documented progression of scoliosis greater than 25°. All children received surgical treatment with guided growth without fusion using a modified Luque trolley technique. The children's preoperative, postoperative, and most recent radiographs were assessed for Cobb angle, T1–T12 and T1–S1 height, and sagittal alignment including proximal junctional kyphosis. Surgimap spine software was used for calibration and measurement purposes. Complications and need for repeat and/or secondary surgical procedures were recorded. Results The mean age at surgery was 7.4 years (4.6–10.5). On average, 15 segments (13–16) were instrumented. None of the children went on to a spontaneous fusion, and the average growth rate per year from T1–T12 and T1–S1 was 0.9 cm/y and 1.5 cm/y, respectively. The mean total growth from T1–T12 and T1–S1 was 22.3 cm (16.6–30.2) and 37.5 cm (30.1–46.4). A total of three additional surgeries were needed in two children to address complications. There were no mortalities. Conclusions Sublaminar guided growth is a safe and effective treatment in the Low Tone Neuromuscular subset of EOS. Follow-up studies failed to show signs of auto fusion, and implant failure was not observed in our cohort. All children displayed growth postoperatively without the need for multiple distraction-based surgeries. Guided growth minimizes the risks associated with multiple surgical procedures while maintaining correction and allowing for near-normal rates of spinal growth.
Plastic deformation and material transfer on steel gage blocks during low force mechanical probing

Abstract Contact probing of gaging surfaces is used throughout dimensional metrology. Probe tips such as ruby, sapphire, or diamond are commonly employed as styli for universal length measuring machines (ULMs) and coordinate measuring machines (CMMs) due to the hardness, durability, and wear resistance. Gaging surfaces of gage blocks are precision ground or lapped, with very low surface roughness to enable wringing. Damage or contamination of these surfaces can prevent wringing and lead to measurement error. Experimental investigations using a horizontal ULM and CMM have revealed that even at low force settings (≤0.16 N), probe materials such as ruby and sapphire can cause plastic deformation to hardened carbon chrome steel (such as AISI 52,100) gage block surfaces at the microscale, likely attributed to fretting-associated wear. Under some conditions, permanent transfer of material from the probe stylus to the gaging surface is possible. Results demonstrate irreversible changes and damage to gaging surfaces with repeated probe contact on a ULM and CMM. Optical microscopy, optical profilometry, and scanning electron microscopy (SEM) provide a semi-quantitative assessment of microscale plastic deformation and material transfer. X-ray photoelectron spectroscopy (XPS), Auger electron spectroscopy (AES), and Raman techniques confirm chemical constituency of reference materials used (gage blocks and probes) and also identify makeup of deposits on gaging surfaces following probe contact.
Characterization of human cartilage-derived progenitor heterogeneity for cellular therapy strategies using time-lapse phase-contrast imaging

Background & Aim Cartilage tissue, one of the most popular sources of tissue for cartilage repair, contains a heterogeneous population of progenitors. However, only a subset of this population possess the significant biological potential to proliferate and differentiate to form cartilage. The goal of this study is to define the static and dynamic characteristics of these progenitors and progeny which can systematically serve as critical quality attributes (CQAs) for selection and expansion to obtain clonal cell populations with better quality and reproducibility to enable delivery of safe, effective and reliable cellular therapies. Methods, Results & Conclusion Articular cartilage (Outerbridge grade 1-2) obtained from nine knee arthroplasty patient's were enzymatically digested to isolate cells for 2-D cell culture assay. Large field of view phase contrast images were acquired hourly to capture images of the progenitors and their progeny (colony) for standardized ASTM-based automated colony-forming unit (CFU) analysis to define and quantify CQAs of the stem/progenitor cells and their progeny, which include: 1) lag-time for first division, 2) circularity, 3) area, 4) feret's diameter, 5) effective proliferation rate, and 6) colony density. Of the 225 colonies obtained from nine patients, 87 colonies (38.7%) were clonal (colony derived from single progenitor) and 138 colonies (61.3%) were non-clonal (colony derived from clumps of cells or multiple-single progenitors), as determined from the time-lapse video (Fig. 1). For the clonal colonies assessed, a wide variation was observed in morphological and biological characteristics of progenitor and their progeny as shown in Fig. 2: Lag time (median:3.8days; range:1.04-7days), circularity (median: 0.48; range:0.12-0.95), area (median: 534.4µm2; range:113.9-1988.9µm2), feret's diameter (median: 13.9µm; range:5.8-37.1µm) and colony density (median:16.9%;range:3.6-55%). An improved understanding of the heterogeneity of progenitor cells resident in adult cartilage will also improve the rigor of cell sourcing and targeting decisions for pharmacological and cellular therapies. The CQAs identified via this process can be reliably and reproducibly used for selection and expansion to obtain clonal cell populations to improve efficacy of cell therapy products.
An Information-processing Model of Understanding Speech

Simultaneous interpretation provides an ideal situation for a logical analysis of an information-processing model. To the external observer, the interpreter listens to a speech in one language, while simultaneously (or at least within a few seconds or so) articulating the same message in a second language. In terms of an information-processing description, the simultaneous interpreter must decode the surface structure of the original message, map it into some abstract representation, take this same abstract representation and map it into a new surface structure, and finally articulate the translated message.
Impact of short term oral steroid use for intravenous contrast media hypersensitivity prophylaxis in diabetic patients undergoing nonemergent coronary angiography or interventions.

Objectives Oral steroids are routinely administered in the United States for prophylaxis of iodinated contrast media hypersensitivity (ICMH). We studied the impact of short-term steroid use in diabetic patients with ICMH undergoing nonemergent coronary angiography. Methods We retrospectively analyzed records of diabetic patients with and without ICMH who underwent nonemergent coronary angiography at our center. Primary study endpoint was 30-day major adverse cardiac events (MACE) and secondary endpoints were pre- and postprocedure fasting blood glucose (FBG), highest in hospital blood glucose, pre- and postprocedure systolic blood pressure (SBP), and use of intravenous insulin and antihypertensive medications. Results A total of 88 diabetics with ICMH (study group) and 76 diabetics without ICMH (control group) undergoing angiography were enrolled. Demographics and hemoglobin A1c values were similar in both groups. Preprocedural FBG was significantly higher in the study group. The study group had significantly higher post angiography FBG (239.93 + 96.88 mg/dl vs. 156.6 + 59.88 mg/dl) and greater use of intravenous (IV) insulin (67.27% vs. 32.43%). Further, those who received steroids had significantly higher systolic SBP postprocedure (146.16 + 25.35 mmHg vs. 130.8 + 21.59 mmHg), a higher incidence of severe hypertension and use of IV antihypertensive medications (80.95% vs. 19.05%) periprocedurally. There were no differences in 30-day MACE between groups. Conclusion Short-term steroid use for ICMH results in a significant increase in surrogate markers for adverse clinical events after coronary procedures. Study findings highlight the need for better periprocedural management of these patients and to limit steroid prophylaxis to those with only true ICMH.
Maintaining Muscle Function Across the Lifespan: The State of Science

Sarcopenia is a geriatric syndrome currently defined as pathological loss of muscle mass and function. Sarcopenia is not only a major contributor to loss of physical function in older adults but is also associated with increased risk of morbidity, mortality, and increased healthcare costs. As a complex and multifactorial syndrome, sarcopenia has been associated with numerous degenerative changes during the aging process, but there is building evidence for significant contributions to the development of sarcopenia from neurodegenerative changes in the peripheral nervous system. A variety of interventions have been investigated for the treatment of sarcopenia, but current management is primarily focused on nutrition and therapeutic exercise interventions. Great strides have been made to improve screening procedures and diagnostic criteria for sarcopenia, but continued optimization of diagnostic and screening strategies is needed to better identify individuals with sarcopenia or at risk of developing sarcopenia. Understanding and addressing the major drivers of sarcopenia pathogenesis will help develop therapeutics that can reduce the impact of sarcopenia on affected individuals and society.
Clearing Time Considerations for DC ARC Flash Hazard Analysis of Battery Banks

Since empirical calculation methods of Arc Flash for DC systems are not yet available, several types of models and equation approaches are available for use with varying degrees of success in estimating a conservative thermal incident energy estimate for DC power systems. One of the undetermined variables for any of these calculations is the clearing time of any protective devices that may relate to the system configuration. The utility of the arcing currents derived from the DC arc flash calculation methods available does not always correlate well with estimating realistic clearing times for protective devices. This paper reviews the various commonly used calculation methods and proposes approaches for bracketing usable clearing times for calculations based on Time-Current Curves (TCC) for typical protective devices used in industry for battery banks. The methods used are directly applicable to other DC systems if the appropriate clearing time information and voltage current curves for non-linear are available.
Cancer cell-intrinsic expression of MHC II in lung cancer cell lines is actively restricted by MEK/ERK signaling and epigenetic mechanisms.

Background Programmed death 1/programmed death ligand 1 (PD-1/PD-L1) targeted immunotherapy affords clinical benefit in ~20% of unselected patients with lung cancer. The factor(s) that determine whether a tumor responds or fails to respond to immunotherapy remains an active area of investigation. We have previously defined divergent responsiveness of two KRAS-mutant cell lines to PD-1/PD-L1 blockade using an orthotopic, immunocompetent mouse model. Responsiveness to PD-1/PD-L1 checkpoint blockade correlates with an interferon gamma (IFNγ)-inducible gene signature and major histocompatibility complex class II (MHC II) expression by cancer cells. In the current study, we aim to identify therapeutic targets that can be manipulated in order to enhance cancer-cell-specific MHC II expression. Methods Responsiveness to IFNγ and induction of MHC II expression was assessed after various treatment conditions in mouse and human non-small cell lung cancer (NSCLC) cell lines using mass cytometric and flow cytometric analysis. Results Single-cell analysis using mass and flow cytometry demonstrated that IFNγ consistently induced PD-L1 and MHC class I (MHC I) across multiple murine and human NSCLC cell lines. In contrast, MHC II showed highly variable induction following IFNγ treatment both between lines and within lines. In mouse models of NSCLC, MHC II induction was inversely correlated with basal levels of phosphorylated extracellular signal-regulated kinase (ERK) 1/2, suggesting potential mitogen-activated protein (MAP) kinase-dependent antagonism of MHC II expression. To test this, cell lines were subjected to varying levels of stimulation with IFNγ, and assessed for MHC II expression in the presence or absence of mitogen-activated protein kinase kinase (MEK) inhibitors. IFNγ treatment in the presence of MEK inhibitors significantly enhanced MHC II induction across multiple lung cancer lines, with minimal impact on expression of either PD-L1 or MHC I. Inhibition of histone deacetylases (HDACs) also enhanced MHC II expression to a more modest extent. Combined MEK and HDAC inhibition led to greater MHC II expression than either treatment alone. Conclusions These studies emphasize the active inhibitory role that epigenetic and ERK signaling cascades have in restricting cancer cell-intrinsic MHC II expression in NSCLC, and suggest that combinatorial blockade of these pathways may engender new responsiveness to checkpoint therapies.
Temporally downscaling a precipitation intensity factor for soil erosion modeling using the NOAA-ASOS weather station network

Abstract Precipitation intensity is an important meteorological input for water erosion and runoff applications. A commonly used intensity factor is maximum 30-min intensity (I30), which represents the sustained intensity of a storm. Determining I30 is challenging for two reasons. First, intensity can vary significantly over time, even within very short durations of 5 min or less. Second, the majority of precipitation data sets are limited by their fixed-interval nature, and I30 may not be constant within fixed measurement intervals. When intensity is simply averaged given the accumulation of a measurement interval, the temporal resolution of the precipitation data set biases the result. Therefore, in this study, bias adjustments were determined for a range of selected temporal resolutions and Koppen-Geiger climate regions in the United States. In this case, the intensity factor was monthly mean maximum 30-minute intensity (MX.5P), which is a parameter used to generate stochastic meteorological inputs for models that include the Rangeland Hydrology and Erosion model (RHEM) and the Water Erosion Prediction Project model (WEPP). The adjustment factors were obtained by using linear regression of reference MX.5P values derived from breakpoint data against MX.5P values aggregated from the breakpoint data to represent lower temporal resolutions. The resulting slope coefficients were used to determine bias adjustment factors. In addition, multivariate machine learning regression was used to obtain more complex correlations involving a host of predictor variables that may each be determined from daily precipitation statistics and the spatial location of each station. In total, 609 stations and 16 climate classifications were represented in the regressions. Linear regressions for climate classifications gave RMSE for values of MX.5P derived from hourly data ranging from 0.98 to 3.46 mm hr−1 with an average of 2.18 mm hr−1. For daily data, the error range was 2.83–8.44 mm hr−1 with an average of 5.61 mm hr−1. The multivariate regression using machine learning algorithms improved regressions for coarser resolutions, reducing error to the 3–4 mm hr−1 range for downscaled daily values.