bluuebunny/crossref_metadata_2025 huggingface dataset:
The Network Society: A Shift in Cognitive Ecologies?
Authors: Wall-Smith Mat
Published: May 2013

By examining the psychodynamic effects on human cognition of the adoption of the technology of writing we can logically assess and contextualize the potential effect of the massification of networked information systems on our day-to-day thought processes. The identification of congruent, parallel and differential affect between writing and network technologies demands that their development be considered above and beyond the dictates and imperatives of consumer capitalism, it demands that the Internet be thought of in terms of public infrastructure rather than saleable capital.
The Usability of Open Source Software
Authors: Nichols David , Twidale Michael
Published: May 2013

Open source communities have successfully developed a great deal of software although most computer users only use proprietary applications. The usability of open source software is often regarded as one reason for this limited distribution. In this paper we review the existing evidence of the usability of open source software and discuss how the characteristics of open source development influence usability. We describe how existing human-computer interaction techniques can be used to leverage distributed networked communities, of developers and users, to address issues of usability.
A Critical Analysis of the Adoption and Utilization of the Internet in Thailand for Educational Purposes
Authors: Prammanee Noppadol
Published: May 2013

Although the use of the Internet in Thailand has a short history, it continues to develop at a rapid rate. This paper presents how the Thai government adopted the Internet into their educational systems by looking at the diffusion-of-innovation theory. Also, it will briefly describe the evolution as well as criticize the adoption of the Internet in Thai education with an emphasis on academic uses. Moreover, it will explain the benefits, obstacles, and future plans of Internet usage in Thailand for education.
Combat Power and Enterprise Competitiveness
Authors: Quarterman John , Harker Ken , Salus Peter
Published: May 2013

If the Spanish Armada had had distributed information for shared awareness, we'd all be speaking Spanish now.

Companies such as GE that do have it are among the most successful. The U.S. military has spent millions of dollars demonstrating that shared situational awareness improves combat effectiveness. Let's look at some examples, and then let's apply the same ideas to intranets and to corporate use of the Internet. Does better distribution of corporate information lead to shared awareness and increased business competitiveness?
Open Networks, Closed Regimes: The Impact of the Internet on Authoritarian Rule (Table of Contents)
Authors: Kalathil Shanthi , Boas Taylor
Published: May 2013

None available
Cyborg logs and collective stream of (de)consciousness capture for producing attribution-free informatic content such as cyborglogs
Authors: Mann Steve
Published: May 2013

Various forms of apparatus for a new kind of wiki or blog (weblog) are described. In particular, ways of bringing together a collective deconsciousness are presented. The systems works with CyborgLogs (cyborglogs or "glogs") from a community of portable computer users, or it can also be used with a mixture of portable (handheld or wearable), mobile (automotive, boat, van, or utility vehicle), or base-station (home, office, public space, etc.) systems. The system enables a community to exist without conscious thought or effort on the part of the individual participants. Because of the participants' ability to constantly experience the world through the apparatus, the apparatus can behave as a true extension of the participants' mind and body, giving rise to a new kind of collective experience. In other embodiments, the system may operate without the need for participants to bear any kind of technological prosthesis.
Opening the sources of accountability
Authors: David Shay
Published: May 2013

This paper scrutinizes the concept of accountability in light of free and open source software. On the view that increasing accountability grants value to society by motivating those most likely and able to prevent risk and harm to do so, I argue that while developing software collaboratively, licensing it openly, and distributing its source code freely are promising first steps in the long journey to rehabilitate accountability in our highly computerized society, our very understanding of what accountability is changes too. This paper analyzes the concept of accountability in an open environment and explores the implications in two mission–critical application fields in which software plays a significant role — electronic voting, and electronic medical records. It further considers the potential remedies to accountability’s erosion that free and open source software offer, and the ways in which accountability can be generalized to collective action if we understand it less as punishability and more as a culture that encourages the prevention of risk and harm. With such reconceptualized accountability in mind, I find that code visibility, a self–imposed standard of care and sensible licensing arrangements, are a potent, practical, and effective alternative to the strict liability standards offered as a solution to the accountability problem by earlier scholars.
FLOSS developers as a social formation
Authors: Lehmann Frauke
Published: May 2013

Developers of Free/Libre and Open Source Software (FLOSS) are often referred to as a community or as a scene. But so far this seems mostly just a rough expression. This paper takes a closer look at FLOSS developers and their projects to find out how they work, what holds them together and how they interact. Community and social movement seem not to apply as descriptors. Looking at norms, values, structures, and conflict resolution, a hacker subculture appears which is compartmentalised into differently organised projects. By testing empirical findings against various theoretical approaches, ideas for further research are identified.
The realities of Free/Libre/Open Source Software developers in Japan and Asia
Authors: Shimizu Hiroyuki , Iio Jun , Hiyane Kazuo
Published: May 2013

A variety of individuals around the world are furthering development of Free/Libre/Open Source Software (FLOSS) through the Internet. Why do they participate in developers’ communities and continue to develop FLOSS? Is their treatment enough to sustain their activities? Surveys, using online questionnaires, were conducted to answer these questions to analyze the FLOSS movement sociologically. However these surveys tend to focus on developers in the West. We decided to see if there are regional differences in FLOSS development. To that end, we conducted two surveys, the FLOSS–JP survey in Japanese and the FLOSS–ASIA survey in other Asian languages. In this paper, we describe regional differences, especially among Asian and Japanese FLOSS developers and compare the results to those from Western FLOSS surveys. Detailed reports of FLOSS–JP/ASIA are available at our Web site (MRI, 2004)
Does deinstitutionalisation work? Relationships between psychiatric outpatient and inpatient care provision in a rural German catchment area
Authors: Valdes-Stauber Juan , Cranach Michael Von , Putzhammer Albert , Kilian Reinhold
Published: May 2013

Objective: We intended to find out whether an intensification of outpatient care could have an influence on hospitalisations, readmissions, length of stay, coercive measures and involuntary admissions. Method: We investigated the development of some care variables within a district psychiatric hospital responsible for a rural catchment area of 320,000 inhabitants. Associations between inpatient care variables and outpatient activity were assessed by means of multivariate Prais-Winsten regression models for time series. Results: There was a dramatic reduction of mean LOS figures, associated with the activity level of the outpatient clinic. After the conclusion of the deinstitutionalisation process, total number of admissions, cumulative LOS, quotas of involuntary admissions and number of coercive measures did not increase when the number of beds and mean length of stay decreased. Readmissions decreased significantly when outpatient activity increased. Conclusions: Community-oriented ambulatory care on the basis of multi-disciplinary outreach teams seems to be able to reduce high-frequent readmissions and control mean LOS while at the same time a number of coercive measures keep the number of admissions stable. Economic and clinical effects on real inpatient care, however, cannot be definitively evaluated as long as bed provision does not decrease proportionally with the increase of ambulatory activity.
The Potential of Baobab (Adansonia digitata L.) Extracts as Biocontrol on the Growth and Aflatoxin Production by Aspergillus flavus and A. parasiticus
Authors: El-Nagerabi Saifeldin A. F. , Elshafie Abdulkadir E. , AlKhanjari Suleiman S. , Al-Bahry Saif N. , Elamin Mohamed R.
Published: May 2013

Moulds and associated mycotoxins, especially aflatoxins, are important factors that advesely affect food and feed produced from contaminated plant and animal prodcuts. They are lethal to humans and animals, which emphasizes the great concern in food and feed production. In this study, the effects of baobab (Adansonia digitata) extracts on the vegetative growth and aflatoxin secretion by A. flavus (SQU21) and A. parasiticus (CBS921.7) strains were exzmined. Different concentrations of baobab fruit extract (1.5, 3, 5, and 7% w/v) and essential oil (0.5, 1, 3 and 5% v/v) was used. Fruit extract of baobab apparently inhibited the total aflatoxin secretion up to 20.4-68.5% for A. flavus and 11.9-69.1% for A. parasiticus, whereas the inhibition of aflatoxin B1 production ranged between 29.9-79.2% and 13-68% for the two strains, respectively. The highest inhibition levels of total aflatoxin and aflatoxin B1 secretion by A. flavus (47.2-95.7%; 28.1-89.7%) and A. parasiticus (42.7-93.3%; 25.9-80.2%) were obtained with essential oil extracted from baobab seeds. The two extracts significantly reduced the vegetative growth and the mycelial dry weights of selected fungi. This indicates the antifungal activity and inhibitory effect of baobab on the growth and aflatoxin production by the two toxigenic strains. Thus, fruit extract and essential oil of A. digitata can be suggested as potentially effective biocontrol and biopreservative substrates against food and feed contamination by aflatoxigenic moulds.
Characterization of Innovation Situation in a Remote Rural Region
Authors: Sarkkinen Minna , Kässi Tuomo
Published: May 2013

Innovation debates dealing with regional economic development have generally focused on densely populated, technologically advanced areas and areas around university cities, undervaluing rural regions and their integration in national innovation systems. However, some statistics indicate that remote and rural regions may also have good innovation potential. This study examines more closely the innovation situation of the firms located in a remote rural region in Finland. The study is based on a structured survey including four main questions describing the innovation situation in the firms. The study outlines the most important features regarding the innovation, correlations between the main results and differences between the firm size categories and sectors. In general, the results indicate, e.g., high importance of a firm's own innovation activities, and reveal some marketing goals of innovation and financial barriers. A correlation analysis reveals several significant correlations between the main results of the survey.
The Foundations of Digital Signal Processing Using Signal Wizard Systems®
Authors: Gaydecki Patrick
Published: May 2013

Signal Wizard Systems® is a digital signal processing (DSP) research venture within the School of EEE at the University of Manchester, UK. It specialises in the development and supply of real-time DSP products for audio signal analysis and processing. The unique and underpinning philosophy of these products is their ease of use. The systems require minimal knowledge of DSP theory on the part of the user and none of the mathematics associated with digital filter design. Filters and other algorithms can be designed in seconds, downloaded and executed in real time with just a few mouse clicks. Since 2004 Signal Wizard products have been sold all over the world for applications ranging from noise suppression, adaptive filtering and system modelling to musical instrument research. In particular, their ease of use ensures that they are ideally suited for teaching simple and more advanced concepts in DSP both at undergraduate and postgraduate level. For this purpose, a DSP laboratory teaching package has been developed using the Signal Wizard range of devices, and has proven an invaluable tool for training our student cohort in the practical aspects of DSP engineering design and programming.
Application of a DSP-Based Control System in a Course in Synchronous Machines and Excitation Systems
Authors: Sumina Damir , Bulić Neven , Mišković Mato
Published: May 2013

The paper presents educational tools for students of an undergraduate course in synchronous generators and excitation systems. Laboratory exercises are based on the work with a laboratory model which consists of a digital control system and a synchronous generator connected to the power network. The code development is supported by two software tools where the code can be developed in the C or C++ programming language or in a graphical environment. In the Electrical Machines Laboratory, the presented laboratory model allows students to develop the code for excitation control, to learn how a conventional excitation control structure and various types of power system stabilisers function, to carry out experiments with a step change in the active power reference or voltage reference, and to record generator responses. The proposed approach offers students the opportunity to apply the knowledge they have acquired in lectures through practical work in the laboratory.
A DSP and FPGA Testbed for Teaching and Research of Wireless Communications
Authors: Webber Julian , Nishimura Toshihiko , Ohgane Takeo , Ogawa Yasutaka
Published: May 2013

This paper describes the teaching and research of signal processing and communications systems that took place during the development of a real-time transceiver and radio channel testbed at Hokkaido University, Japan. Digital signal processing (DSP) concepts were taught and learnt during both the testbed system development and also the results gathering and analysis stages. The performance of a modern multiple antenna communications system is dependent on a number of key parameters, and the student interaction with such a real-time system can assist in the understanding of key but often abstract theoretical concepts. The communications algorithm and architecture overview on a signal processing board containing a Xilinx field programmable gate array (FPGA) and Analog Devices TigerSharc DSP is detailed. The lessons learned and potential uses of the testbed in both teaching and research are also described.
Overcoming the Challenges of Porting OpenCV to TI's Embedded ARM + DSP Platforms
Authors: Coombs Joseph , Prabhu Rahul , Peake Greg
Published: May 2013

The growing performance and decreasing price of embedded processors are opening many doors, for both developers in the industry and in academia. However, the complexities of these systems can create serious developmental bottlenecks. Sophisticated software packages such as OpenCV can assist in both the functional development and educational aspects of these otherwise complex applications; such tools lend themselves very well to use by the academic community, in particular in providing examples of algorithm implementation. However the task of migrating this software to embedded platforms poses its own challenges. This paper will review how to mitigate some of these issues, including C++ implementation, memory constraints, floating-point support, and opportunities to maximise performance using vendor-optimised libraries and integrated accelerators or co-processors. Finally, we will introduce a new effort by Texas Instruments to optimise vision systems by running OpenCV on the C6000™ digital signal processor architecture. Benchmarks will show the advantage of using the DSP by comparing the performance of a DSP+ARM® system-on-chip (SoC) processor against an ARM-only device.
Influenza: Propagation, Quantification, and Storage
Authors: Balish Amanda L. , Katz Jacqueline M. , Klimov Alexander I.
Published: May 2013

Influenza viruses are negative‐sense, single‐stranded, enveloped RNA viruses belonging to the family Orthomyxoviridae. Three types exist, influenza A, B, and C. All infect humans, but only A and B are major human pathogens. Influenza type A viruses are divided into subtypes based on genetic and antigenic differences in the two surface spike proteins, hemagglutinin (HA) and neuraminidase (NA). The appropriate cell lines to be used for isolation of influenza A or B viruses depend on the clinical information and the host of origin. MDCK cells are the preferred cell line for isolation of human influenza viruses from clinical specimens. Curr. Protoc. Microbiol. 29:15G.1.1‐15G.1.24. © 2013 by John Wiley & Sons, Inc.
Utility of the PedsQL Rheumatology Module as an Outcome Measure in Juvenile Fibromyalgia
Authors: Joffe Naomi E. , Lynch‐Jordan Anne , Ting Tracy V. , Arnold Lesley M. , Hashkes Philip J. , Lovell Daniel J. , Passo Murray H. , Powers Scott W. , Schikler Kenneth N. , Kashikar‐Zuck Susmita
Published: May 2013

Objective

The PedsQL rheumatology module is currently the only available measure of disease‐specific quality of life for children and adolescents with juvenile fibromyalgia (FM), but limited information has been published about the psychometric properties of the instrument, specifically in juvenile FM. The objective of this study was to assess the reliability, validity, and sensitivity to change of the 5 scales (pain and hurt, daily activities, treatment, worry, and communication) of the patient and parent proxy versions of the PedsQL rheumatology module in the context of a randomized controlled trial in juvenile FM.

Methods

The entire PedsQL rheumatology module was administered as a supplementary outcome measure at baseline, posttreatment, and 6‐month followup assessments of 114 children and adolescents with juvenile FM enrolled in a trial testing the efficacy of cognitive–behavioral therapy.

Results

Internal consistency reliabilities for the scales were adequate to strong (Cronbach's α = 0.68–0.86). Parent proxy and child reports on most scales (except for daily activities and communication) showed moderate correlations (Spearman's r = 0.33–0.45). Support for construct validity was found by comparing child and parent reports with other related measures of pain and functioning (visual analog scale pain ratings and the Functional Disability Inventory). Finally, sensitivity to change was demonstrated by significant changes in 4 of the 5 scales (excluding the daily activities scale) after treatment.

Conclusion

The PedsQL rheumatology module generally appears to have good utility for use in juvenile FM patients, but there are some caveats to the interpretation of specific scales in this population.
Readability and Suitability Assessment of Patient Education Materials in Rheumatic Diseases
Authors: Rhee Rennie L. , Von Feldt Joan M. , Schumacher H. Ralph , Merkel Peter A.
Published: May 2013

Objective

Web‐based patient education materials and printed pamphlets are frequently used by providers to inform patients about their rheumatic disease. Little attention has been given to the readability and appropriateness of patient materials. The objective of this study was to examine the readability and suitability of commonly used patient education materials for osteoarthritis (OA), rheumatoid arthritis, systemic lupus erythematosus, and vasculitis.

Methods

Five or 6 popular patient resources for each disease were chosen for evaluation. Readability was measured using the Flesch‐Kincaid reading grade level and suitability was determined by the Suitability Assessment of Materials (SAM), a score that considers characteristics such as content, graphics, layout/topography, and cultural appropriateness. Three different reviewers rated the SAM score and means were used in the analysis.

Results

Twenty‐three resources written on the 4 diseases were evaluated. The education material for all 4 diseases studied had readability above the eighth‐grade level and readability did not differ among the diseases. Only 5 of the 23 resources received superior suitability scores, and 3 of these 5 resources were written for OA. All 4 diseases received adequate suitability scores, with OA having the highest mean suitability score.

Conclusion

Most patient education materials for rheumatic diseases are written at readability levels above the recommended sixth‐grade reading level and have only adequate suitability. Developing more appropriate educational resources for patients with rheumatic diseases may improve patient comprehension.
Visible‐Light Photocatalytic Reduction of Sulfonium Salts as a Source of Aryl Radicals
Authors: Donck Simon , Baroudi Abdulkader , Fensterbank Louis , Goddard Jean‐Philippe , Ollivier Cyril
Published: May 2013

Triarylsulfonium salts are prompted to undergo efficient homolytic reduction by single electron transfer under mild photocatalytic conditions. The liberated aryl radical can then participate in carbon‐carbon bond formation processes with allyl sulfones and activated olefins. Triarylsulfonium salts emerge as a valuable and alternative source of aryl radicals for synthesis.
Synthesis of N‐Arylamides by Copper‐Catalyzed Amination of Aryl Halides with Nitriles
Authors: Xiang Shi‐Kai , Zhang Dong‐Xue , Hu Hao , Shi Jiang‐Ling , Liao Li‐Guo , Feng Chun , Wang Bi‐Qin , Zhao Ke‐Qing , Hu Ping , Yang Hua , Yu Wen‐Hao
Published: May 2013

A copper‐catalyzed amination of aryl halides with nitriles has been developed. The use of nitriles as nitrogen nucleophiles can make the synthesis of N‐arylamides more simple than that using amides through in‐situ hydrolysis. A variety of N‐arylamides and benzoxazole derivatives can be synthesized according to this approach.
The Copper‐Catalyzed Oxidative N‐Acylation of Sulfoximines
Authors: Wang Long , Priebbenow Daniel L. , Zou Liang‐Hua , Bolm Carsten
Published: May 2013

An oxidative cross‐coupling reaction between aldehydes and sulfoximines involving dual CH/NH functionalization has been developed. This reaction process is facilitated by a simple copper catalyst (1 mol% loading) and tert‐butyl hydroperoxide (TBHP) as the oxidant and proceeds under mild reaction conditions to afford a series of valuable N‐acylated sulfoximine derivatives in excellent yields.
Building Functionality through Sequential CB and CF Bond Formation
Authors: Palau‐Lluch Gerard , Fernández Elena
Published: May 2013

α′‐Fluoro β‐boryl ketones can be efficiently synthesised from a sequential organocatalytic β‐boration pathway and consecutive electrophilic fluorination reaction in an acidic medium. Alternatively, the regioisomers of α‐fluoro β‐boryl ketones can be diastereoselectively obtained from Cu(I)‐mediated β‐boration followed by in situ fluorination of the boron enolate. Enantioenriched mixtures of the major anti‐diastereomer of vicinal CB and CF bonds are found in the presence of the chiral ligand QuinoxP*.
Nitrones as Trapping Reagents of α,β‐Unsaturated Carbene Intermediates – [1,2]Oxazino[5,4‐b]indoles by a Platinum‐ Catalyzed Intermolecular [3+3] Cycloaddition
Authors: Yang Weibo , Wang Tao , Yu Yang , Shi Shuai , Zhang Tuo , Hashmi A. Stephen K.
Published: May 2013

Some readily available Boc‐protected 2‐(3‐methoxy‐1‐propynyl)anilines and nitrones in platinum‐catalyzed reactions deliver [1,2]oxazino[5,4‐b]indoles. Twelve examples with yields of 41–95% are reported. Different substituents like nitro, trifluoromethyl, fluoro, bromo, and ester groups are tolerated. With regard to the mechanism, this reaction probably combines an initial intramolecular cyclization/elimination to vinylcarbenoid species and a subsequent stepwise intermolecular [3+3] cycloaddition with the nitrones.
Mortality of congenital osteochondrodysplasias: A nationwide registry‐based study
Authors: Morales‐Piga Antonio , Alonso‐Ferreira Verónica , Villaverde‐Hueso Ana , Posada de la Paz Manuel , López‐Abente Gonzalo
Published: May 2013

Osteochondrodysplasias are a heterogeneous group of more than 200 entities, characterized by abnormalities of cartilage, bone growth, and skeletal development. The aim of this study was to assess temporal and spatial changes in overall mortality due to these disorders in Spain, using data from a nationwide registry. Annual deaths showing osteochondrodysplasias as the underlying cause of death were selected using the International Classification of Diseases‐9th revision (ICD‐9) codes for the period 1981 through 1998, and ICD‐10 codes for the period 1999 through 2008. Age‐adjusted mortality rates were calculated by sex, and geographic analysis was performed by municipality. A total of 679 deaths were recorded (53% men). Age‐adjusted mortality rates went from 0.09 (0.06, 0.12) per 100,000 population in 1981 to 0.05 (0.03, 0.08) per 100,000 population in 2008. A changing trend in the age‐standardized mortality rate was in evidence, with an annual increase of 2.4% (−0.4, 5.2) from 1981 to 1994, and an annual decrease of −7.3% (−10.9, −3.5) from 1995 onwards. Geographic analysis showed some places situated in the west and south of Spain with greater risk of mortality. There is a need to identify risk factors and to increase overall knowledge about the life expectancy and epidemiology of osteochondrodysplasias. (c) 2013 Wiley Periodicals, Inc.
6p21.3 microdeletion involving the SYNGAP1 gene in a patient with intellectual disability, seizures, and severe speech impairment
Authors: Writzl Karin , Knegt Alida C.
Published: May 2013

The chromosome 6p21.3 microdeletion phenotype was recently identified through array comparative genomic hybridization. The main features are developmental delay with severe speech impairment, seizures, and behavioral abnormalities. Three patients have been reported with deletion sizes ranging from 100 to 800 kb. We report on a 9‐year‐old boy with an apparently de novo, 50 kb deletion, and global developmental delay, severe speech impairment, and generalized epilepsy well‐controlled by medication. There were four genes identified in this deletion, of which SYNGAP1 is considered to be responsible for speech impairment and epilepsy. We compared the clinical features of this patient with previously reported patients with 6p21.3 and patients with SYNGAP1 mutations. © 2013 Wiley Periodicals, Inc.
De novo trisomy 12p in twin girls with different levels of mosaicism
Authors: Lim Eileen C.P. , Ng Ivy S.L. , Yong Min‐Hwee , Yon Hui‐Yi , Brett Maggie S.Y. , Tan Ene‐Choo
Published: May 2013

We report on a pair of twins with trisomy 12p diagnosed postnatally. The girls were referred for dysmorphism and global developmental delay and have been followed from 10 months of age. They have different levels of mosaicism for both buccal cells and lymphocytes. Although their phenotypic features were similar, there were different degrees of severity which correlate with the different levels of mosaicism. © 2013 Wiley Periodicals, Inc.
Progressive extreme heterotopic calcification
Authors: Silengo Margherita , Defilippi Claudio , Belligni Elga , Biamino Elisa , Flex Elisabetta , Brusco Alfredo , Ferrero Giovanni Battista , Tartaglia Marco , Hennekam Raoul C.
Published: May 2013

The formation of ectopic calcifications in soft tissues can occur either sporadically or as a genetically determined condition, and is seen only infrequently. We report on a girl in whom widespread, rapidly progressive ectopic calcifications were detected shortly after birth. Calcifications became present around all joints, tendons and ligaments, but did not involve internal organs and skin, and eventually caused almost complete immobility of the child at 2 years. There were no other health problems and cognitive development was normal. We compare the manifestations in the child to the characteristics of known entities causing ectopic calcifications and conclude the child differs to each. Laboratory evaluation failed to identify autoimmune phenomena as well as calcium metabolism or other biochemical abnormalities; molecular studies did not identify occurrence of mutations in disease genes known to be involved in ectopic calcifications. We conclude the manifestations in the child represent an unreported entity of hitherto unknown etiology. © 2013 Wiley Periodicals, Inc.
Recurrence of urorectal septum malformation sequence spectrum anomalies in siblings: Time to explore the genetics
Authors: Aggarwal Shagun , Phadke Shubha R.
Published: May 2013

Urorectal septum malformation sequence (URSM) is a pattern of malformation which encompasses abnormalities of the perineal orifices, external genitalia, genitourinary system, and anorectum. The spectrum ranges from a complete form with absence of perineal openings and persistent cloaca to milder/partial forms usually with one perineal opening and internal abnormalities of anorectum, urethra, and Müllerian structures. URSM is felt to arise due to abnormalities of the caudal mesoderm, which constitutes the urorectal septum. Here, we report two male siblings, affected with a spectrum of anomalies simulating URSM. This is the first report of recurrence of URSM in sibs. It suggests the existence of hitherto unknown genetic mechanisms for this pattern of malformation. © 2013 Wiley Periodicals, Inc.
Characterization of X‐linked hypohidrotic ectodermal dysplasia (XL‐HED) hair and sweat gland phenotypes using phototrichogram analysis and live confocal imaging
Authors: Jones Kyle B. , Goodwin Alice F. , Landan Maya , Seidel Kerstin , Tran Dong‐Kha , Hogue Jacob , Chavez Miquella , Fete Mary , Yu Wenli , Hussein Tarek , Johnson Ramsey , Huttner Kenneth , Jheon Andrew H. , Klein Ophir D.
Published: May 2013

Hypohidrotic ectodermal dysplasia (HED) is the most common type of ectodermal dysplasia (ED), which encompasses a large group of syndromes that share several phenotypic features such as missing or malformed ectodermal structures, including skin, hair, sweat glands, and teeth. X‐linked hypohidrotic ectodermal dysplasia (XL‐HED) is associated with mutations in ectodysplasin (EDA1). Hypohidrosis due to hypoplastic sweat glands and thin, sparse hair are phenotypic features that significantly affect the daily lives of XL‐HED individuals and therefore require systematic analysis. We sought to determine the quality of life of individuals with XL‐HED and to quantify sweat duct and hair phenotypes using confocal imaging, pilocarpine iontophoresis, and phototrichogram analysis. Using these highly sensitive and non‐invasive techniques, we demonstrated that 11/12 XL‐HED individuals presented with a complete absence of sweat ducts and that none produced sweat. We determined that the thin hair phenotype observed in XL‐HED was due to multiple factors, such as fewer terminal hairs with decreased thickness and slower growth rate, as well as fewer follicular units and fewer hairs per unit. The precise characterization of XL‐HED phenotypes using sensitive and non‐invasive techniques presented in our study will improve upon larger genotype–phenotype studies and the assessment of future therapies in XL‐HED. © 2013 Wiley Periodicals, Inc.
Maritime adaptations and dietary variation in prehistoric Western Alaska: Stable isotope analysis of permafrost‐preserved human hair
Authors: Britton Kate , Knecht Rick , Nehlich Olaf , Hillerdal Charlotta , Davis Richard S. , Richards Michael P.
Published: May 2013

The reconstruction of diet and subsistence strategies is integral in understanding early human colonizations and cultural adaptations, especially in the Arctic—one of the last areas of North America to be permanently inhabited. However, evidence for early subsistence practices in Western Alaska varies, particularly with regards to the emergence, importance, and intensity of sea mammal hunting. Here, we present stable carbon and nitrogen isotope data from permafrost‐preserved human hair from two new prehistoric sites in Western Alaska, providing a direct measure of diet. The isotope evidence indicates a heavy reliance on sea mammal protein among the earlier Norton‐period group (1,750 ± 40 cal BP), confirming that the complex hunting technologies required to intensively exploit these animals were most likely already in place in this region by at least the beginning of 1st millennium AD. In contrast, analysis of the more recent Thule‐period hair samples (650 ± 40 cal BP; 570 ± 30 cal BP) reveals a more mixed diet, including terrestrial animal protein. Sequential isotope analysis of two longer human hair locks indicates seasonal differences in diet in a single Norton‐period individual but demonstrates little dietary variation in a Thule‐period individual. These analyses provide direct evidence for dietary differences among Alaska's early Eskimo groups and confirm the antiquity of specialized sea mammal hunting and procurement technologies. The results of this study have implications for our understanding of human adaptation to maritime and high‐latitude environments, and the geographical and temporal complexity in early Arctic subsistence. Am J Phys Anthropol 151:448–461, 2013.© 2013 Wiley Periodicals, Inc.
Brief communication: 5‐HTTLPR genetic diversity and mode of subsistence in Native Americans
Authors: Bisso‐Machado Rafael , Ramallo Virginia , Tarazona‐Santos Eduardo , Salzano Francisco M. , Bortolini Maria Cátira , Hünemeier Tábita
Published: May 2013

The relationship between the “individualism‐collectivism” and the serotonin transporter functional polymorphism (5‐HTTLPR), suggested in the previous reports, was tested in Native South Amerindian populations. A total of 170 individuals from 21 populations were genotyped for the 5‐HTTLPR alleles. For comparative purposes, these populations were classified as individualistic (recent history of hunter–gathering) or collectivistic (agriculturalists). These two groups showed an almost identical S allele frequency (75 and 76%, respectively). The analysis of molecular variance showed no structural differences between them. Behavioral typologies like those suggested by JY Chiao and KD Blizinsky (Proc R Soc B 277 () 529–537) are always a simplification of complex phenomena and should be regarded with caution. In addition, classification of a whole nation in the individualist/collectivist dichotomy is controversial. The focus on modes of subsistence in preindustrial societies, as was tested here, may be a good alternative although the postulated association between the 5‐HTTLPR S allele and the collectivist societies was not confirmed. Am J Phys Anthropol 151:492–494, 2013.© 2013 Wiley Periodicals, Inc.
Frequency‐comb spectroscopy of the hydrogen 1S‐3S and 1S‐3D transitions
Authors: Peters Elisabeth , Yost Dylan C. , Matveev Arthur , Hänsch Theodor W. , Udem Thomas
Published: May 2013

An observation of the 1S‐3S and 1S‐3D two‐photon transitions in an atomic hydrogen beam using direct frequency‐comb spectroscopy in a Doppler‐free arrangement is reported. The measurements of these transition frequencies may be used for an improved determination of the Rydberg constant and the 1S Lamb shift and could shed light on the current discrepancy in the determination of the proton charge radius. Concurrently, a frequency comb well‐suited for high‐precision, Doppler‐free spectroscopy in the deep ultraviolet (DUV) wavelength region is demonstrated.
Validation of the Methotrexate‐First Strategy in Patients With Early, Poor‐Prognosis Rheumatoid Arthritis: Results From a Two‐Year Randomized, Double‐Blind Trial
Authors: O'Dell James R. , Curtis Jeffrey R. , Mikuls Ted R. , Cofield Stacey S. , Bridges S. Louis , Ranganath Veena K. , Moreland Larry W. ,   for the TEAR Trial Investigators
Published: May 2013

Objective

Methotrexate (MTX) taken as monotherapy is recommended as the initial disease‐modifying antirheumatic drug for rheumatoid arthritis (RA). The purpose of this study was to examine outcomes of a blinded trial of initial MTX monotherapy with the option to step‐up to combination therapy as compared to immediate combination therapy in patients with early, poor‐prognosis RA.

Methods

In the Treatment of Early Rheumatoid Arthritis (TEAR) trial, 755 participants with early, poor‐prognosis RA were randomized to receive MTX monotherapy or combination therapy (MTX plus etanercept or MTX plus sulfasalazine plus hydroxychloroquine). Participants randomized to receive MTX monotherapy stepped‐up to combination therapy at 24 weeks if the Disease Activity Score in 28 joints using the erythrocyte sedimentation rate (DAS28‐ESR) was ≥3.2.

Results

Attrition at 24 weeks was similar in the MTX monotherapy and combination groups. Of the 370 evaluable participants in the initial MTX group, 28% achieved low levels of disease activity and did not step‐up to combination therapy (MTX monotherapy group). The mean ± SD DAS28‐ESR in participants continuing to take MTX monotherapy at week 102 was 2.7 ± 1.2, which is similar to that in participants who were randomized to immediate combination therapy (2.9 ± 1.2). Participants who received MTX monotherapy had less radiographic progression at week 102 as compared to those who received immediate combination therapy (mean ± SD change in modified Sharp score 0.2 ± 1.1 versus 1.1 ± 6.4). Participants assigned to initial MTX who required step‐up to combination therapy at 24 weeks (72%) demonstrated similar DAS28‐ESR values (3.5 ± 1.3 versus 3.2 ± 1.3 at week 48) and radiographic progression (change in modified Sharp score 1.2 ± 4.1 versus 1.1 ± 6.4 at week 102) as those assigned to immediate combination therapy. The results for either of the immediate combination approaches, whether triple therapy or MTX plus etanercept, were similar.

Conclusion

These results in patients with early, poor prognosis RA validate the strategy of starting with MTX monotherapy. This study is the first to demonstrate in a blinded trial that initial MTX monotherapy with the option to step‐up to combination therapy results in similar outcomes to immediate combination therapy. Approximately 30% of patients will not need combination therapy, and the 70% who will need it are clinically and radiographically indistinguishable from those who were randomized to receive immediate combination therapy.
Premature Cell Senescence and T Cell Receptor–Independent Activation of CD8+ T Cells in Juvenile Idiopathic Arthritis
Authors: Dvergsten Jeffrey A. , Mueller Robert G. , Griffin Patricia , Abedin Sameem , Pishko Allyson , Michel Joshua J. , Rosenkranz Margalit E. , Reed Ann M. , Kietz Daniel A. , Vallejo Abbe N.
Published: May 2013

Objective

CD8+ T cells lacking CD28 were originally reported to be a characteristic feature of juvenile idiopathic arthritis (JIA), but the relevance of these unusual cells to this disease remains to be elucidated. Because of recent evidence that loss of CD28 cells is typical of terminally differentiated lymphocytes, the aim of this study was to examine functional subsets of CD8+ T cells in patients with JIA.

Methods

Blood and/or waste synovial fluid samples were collected from children with a definite diagnosis of JIA (n = 98). Deidentified peripheral blood (n = 33) and cord blood (n = 13) samples from healthy donors were also collected. CD8+ and CD4+ T cells were screened for novel receptors, and where indicated, bioassays were performed to determine the functional relevance of the identified receptor.

Results

JIA patients had a naive T cell compartment with shortened telomeres, and their entire T cell pool had reduced proliferative capacity. They had an overabundance of CD31+CD28nullCD8+ T cells, which was a significant feature of oligoarticular JIA (n = 62) as compared to polyarticular JIA (n = 36). CD31+ CD28nullCD8+ T cells had limited mitotic capacity and expressed high levels of the senescence antigens histone γH2AX and/or p16. Ligation of CD31, which was independent of the T cell receptor (TCR), sufficiently induced tyrosine phosphorylation, vesicle exocytosis, and production of interferon‐γ and interleukin‐10.

Conclusion

These data provide the first evidence of cell senescence, as represented by CD31+CD28null CD8+ T cells, in the pathophysiology of JIA. Activation of these unusual cells in a TCR‐independent manner suggests that they are maladaptive and could be potential targets for immunotherapy.
Sonographic Joint Assessment in Rheumatoid Arthritis: Associations With Clinical Joint Assessment During a State of Remission
Authors: Gärtner Miriam , Mandl Peter , Radner Helga , Supp Gabriela , Machold Klaus P. , Aletaha Daniel , Smolen Josef S.
Published: May 2013

Objective

Sonography, as compared with clinical assessment, is a sensitive tool for evaluating synovitis in rheumatoid arthritis (RA). However, differences between these assessment tools may depend on how joint activity (i.e., an active joint) is defined. The present study was undertaken to compare clinically active joints with sonographically active joints in patients with RA, applying different sonographic definitions of an active joint.

Methods

Sonographic assessment of the finger and wrist joints (total of 11 joints) of each hand was performed in RA patients whose disease was in remission (Clinical Disease Activity Index ≤2.8; n = 60). Gray‐scale (GS) and power Doppler (PD) ultrasound signals for synovitis were evaluated on a 4‐point scale (grade 0 = none, grade 3 = severe). The sensitivity and specificity of swollen joint counts were investigated using, as reference, increasingly stringent sonographic definitions of an active joint. Sonographic findings were also assessed for correlations with other clinical variables, including the Health Assessment Questionnaire (HAQ) disability index (DI). Followup analyses were performed after 6–12 months.

Results

GS ultrasound signals yielded positive findings for synovitis in 67.2% of the 1,320 joints assessed, and PD ultrasound signals indicated signs of synovitis in 20.4% of the joints assessed. Clinical identification of joint swelling was 100% specific for sonographic joint activity, independent of the stringency of the sonographic definition used; maximum sensitivity of the swollen joint counts was 25% for the most stringent definition (i.e., GS grade 3 and PD grade 3). Furthermore, patients with a higher‐grade PD signal (grade 3) showed a higher HAQ DI score compared to patients with lower‐grade PD signals (mean ± SD HAQ DI 0.45 ± 0.62 versus 0.20 ± 0.35). A higher grade of PD signal at baseline was found in joints that were assessed as clinically swollen at the consecutive followup visit.

Conclusion

Low‐grade PD and GS ultrasound signals may not necessarily reflect the presence of active synovitis in RA joints. High‐grade PD signals correlate well with the presence of clinical joint swelling and clinical disease activity, and a higher grade of PD signal is associated with higher degrees of functional impairment.
Site‐Selective Functionalization of Periodic Mesoporous Organosilica (PMO) with Macrocyclic Host for Specific and Reversible Recognition of Heavy Metal
Authors: Ye Gang , Leng Yuxiao , Bai Feifei , Wei Jichao , Wang Jianchen , Chen Jing
Published: May 2013

A novel kind of macrocyclic‐host‐functionalized periodic mesoporous organosilica (PMO) with excellent and reversible recognition of PbII was developed. The macrocyclic host molecule cis‐dicyclohexano[18]crown‐6, with strong affinity to PbII, was carefully modified as a bridged precursor to build the PMO material. To break down the limit of the functionalization degree for PMOs incorporated with large‐sized moieties, a site‐selective post‐functionalization method was proposed to further decorate the external surface of the PMO material. The selective recognition ability of the upgraded PMO material towards PbII was remarkably enhanced without destroying the mesoporous ordering. Solid‐state 13C and 29Si NMR spectroscopy, X‐ray photoelectron spectroscopy (XPS), XRD, TEM, and nitrogen adsorption–desorption isotherm measurements were utilized for a full characterization of the structure, micromorphology, and surface properties. Reversible binding of PbII was realized in the binding–elution cycle experiments. The mechanism of the supramolecular interaction between the macrocyclic host and metal ion was discussed. The synthetic strategy can be considered a general way to optimize the properties of PMOs as binding materials for practical use while preserving the mesostructure.
Multiple bands near fronts in VHF wind‐profiling radar and radiosonde data
Authors: Lawson John , Schultz David M. , Vaughan Geraint , Kirshbaum Daniel J.
Published: May 2013

Fronts passing over the Mesosphere–Stratosphere–Troposphere radar near Aberystwyth, Wales, UK, possessed multiple bands in returned power and vertical shear of the horizontal wind. Warm fronts tended to have more bands than cold fronts. Of 82 bands in power with an associated band in the radiosonde data, 45% were associated with a decrease in specific humidity and an increase in stability (drying stable bands) and 21% were associated with an increase in humidity and a decrease in stability (moistening unstable bands). The radar detects airstream boundaries near fronts, some of which are not part of the front.
An ultrasensitive chemiluminescent method for the tanshinol borneol ester determination and its pharmacokinetic analysis
Authors: Nie Fei , Bu Min , Wu Lihong , Zheng Jianbin
Published: May 2013

Tanshinol borneol ester (DBZ), a chemical combination of danshensu and borneol, is an experimental drug that exhibits efficacious anti‐ischemic activity in animal models. In this work, an ultrasensitive chemiluminescence (CL) method for the determination of DBZ was established based on the inhibitory effect of DBZ on the CL signal produced from the reaction between potassium permanganate and luminol in alkaline solution. The CL intensity responded linearly to the concentration of DBZ in the range 2.0 × 10‐10 to 4.0 × 10‐8 g/mL with a detection limit of 7 × 10−11 g/mL. The relative standard deviation (RSD) was 3.8% for 4.0 × 10−9 g DBZ (n = 11). The proposed method showed characteristics of high sensitivity, simple device and quick. In addition, this proposed method had been applied satisfactorily to the analysis of DBZ in blood. The pharmacokinetics of DBZ in rat has also been studied using the CL method. Copyright © 2013 John Wiley & Sons, Ltd.
Bis(4,6‐dimethoxy‐1,3,5‐triazin‐2‐yl) Ether as Coupling Reagent for Peptide Synthesis
Authors: Jastrzabek Konrad , Bednarek Przemyslaw , Kolesinska Beata , Kaminski Zbigniew J.
Published: May 2013

Bis(4,6‐dimethoxy‐1,3,5‐triazin‐2‐yl) ether (4) was prepared by treatment of 2‐hydroxy‐4,6‐dimethoxy‐1,3,5‐triazine with 2‐chloro‐4,6‐dimethoxy‐1,3,5‐triazine in 61% yield. Ether 4, isoelectronic with pyrocarbonates, was found capable to activate carboxylic acids in the presence of 1,4‐diazabicyclo[2.2.2]octane (DABCO) to yield, under mild reaction conditions, superactive triazine esters. Versatility of this new coupling reagent was confirmed by condensation of lipophilic and sterically hindered carboxylic acids with amines in 71–98% yield, and by synthesis of peptides, including those containing Aib‐Aib sequence, in solution with high yield and high enantiomeric purity.