bluuebunny/crossref_metadata_2025 huggingface dataset:
European atmosphere in 2050, a regional air quality and climate perspective under CMIP5 scenarios
Authors: Colette A. , Bessagnet B. , Vautard R. , Szopa S. , Rao S. , Schucht S. , Klimont Z. , Menut L. , Clain G. , Meleux F. , Curci G. , Rouïl L.
Published: August 2013

Abstract. To quantify changes in air pollution over Europe at the 2050 horizon, we designed a comprehensive modelling system that captures the external factors considered to be most relevant, and that relies on up-to-date and consistent sets of air pollution and climate policy scenarios. Global and regional climate as well as global chemistry simulations are based on the recent representative concentration pathways (RCP) produced for the Fifth Assessment Report (AR5) of the IPCC (Intergovernmental Panel on Climate Change) whereas regional air quality modelling is based on the updated emissions scenarios produced in the framework of the Global Energy Assessment. We explored two diverse scenarios: a reference scenario where climate policies are absent and a mitigation scenario which limits global temperature rise to within 2 °C by the end of this century. This first assessment of projected air quality and climate at the regional scale based on CMIP5 (5th Coupled Model Intercomparison Project) climate simulations is in line with the existing literature using CMIP3. The discrepancy between air quality simulations obtained with a climate model or with meteorological reanalyses is pointed out. Sensitivity simulations show that the main factor driving future air quality projections is air pollutant emissions, rather than climate change or intercontinental transport of pollution. Whereas the well documented "climate penalty" that weights upon ozone (increase of ozone pollution with global warming) over Europe is confirmed, other features appear less robust compared to the literature, such as the impact of climate on PM2.5. The quantitative disentangling of external factors shows that, while several published studies focused on the climate penalty bearing upon ozone, the contribution of the global ozone burden is somewhat overlooked in the literature.
Assessing the impact of late Pleistocene megafaunal extinctions on global vegetation and climate
Authors: Brault M.-O. , Mysak L. A. , Matthews H. D. , Simmons C. T.
Published: August 2013

Abstract. The end of the Pleistocene was a turning point for the Earth system as climate gradually emerged from millennia of severe glaciation in the Northern Hemisphere. The deglacial climate change coincided with an unprecedented decline in many species of Pleistocene megafauna, including the near-total eradication of the woolly mammoth. Due to an herbivorous diet that presumably involved large-scale tree grazing, the mammoth extinction has been associated with the rapid expansion of dwarf deciduous trees in Siberia and Beringia, thus potentially contributing to the changing climate of the period. In this study, we use the University of Victoria Earth System Climate Model (UVic ESCM) to simulate the possible effects of these extinctions on climate during the latest deglacial period. We have explored various hypothetical scenarios of forest expansion in the northern high latitudes, quantifying the biogeophysical effects in terms of changes in surface albedo and air temperature. These scenarios include a Maximum Impact Scenario (MIS) which simulates the greatest possible post-extinction reforestation in the model, and sensitivity tests which investigate the timing of extinction, the fraction of trees grazed by mammoths, and the southern extent of mammoth habitats. We also show the results of a simulation with free atmospheric CO2-carbon cycle interactions. For the MIS, we obtained a surface albedo increase and global warming of 0.006 and 0.175 °C, respectively. Less extreme scenarios produced smaller global mean temperature changes, though local warming in some locations exceeded 0.3 °C even in the more realistic extinction scenarios. In the free CO2 simulation, the biogeophysical-induced warming was amplified by a biogeochemical effect, whereby the replacement of high-latitude tundra with shrub forest led to a release of soil carbon to the atmosphere and a small atmospheric CO2 increase. Overall, our results suggest the potential for a small, though non-trivial, effect of megafaunal extinctions on Pleistocene climate.
Seismic hazards of the Iberian Peninsula – evaluation with kernel functions
Authors: Crespo M. J. , Martínez F. , Martí J.
Published: August 2013

Abstract. The seismic hazard of the Iberian Peninsula is analysed using a nonparametric methodology based on statistical kernel functions; the activity rate is derived from the catalogue data, both its spatial dependence (without a seismogenetic zonation) and its magnitude dependence (without using Gutenberg–Richter's law). The catalogue is that of the Instituto Geográfico Nacional, supplemented with other catalogues around the periphery; the quantification of events has been homogenised and spatially or temporally interrelated events have been suppressed to assume a Poisson process. The activity rate is determined by the kernel function, the bandwidth and the effective periods. The resulting rate is compared with that produced using Gutenberg–Richter statistics and a zoned approach. Three attenuation laws have been employed, one for deep sources and two for shallower events, depending on whether their magnitude was above or below 5. The results are presented as seismic hazard maps for different spectral frequencies and for return periods of 475 and 2475 yr, which allows constructing uniform hazard spectra.
Combined Silicone Oil Removal with Secondary Implantation of Anterior Chamber or Sclera-fixed Posterior Chamber Intraocular Lenses
Authors: Zhang Yan , Sun Dawei , Zhang Zhongyu , Xiao Huizhen , Peng Shaomin
Published: August 2013

Purpose

To compare the outcomes and complication rates of silicone oil removal (SOR) combined with secondary implantation of modern anterior chamber intraocular lenses (AC IOLs) versus scleral-fixated, posterior chamber IOLs (SF IOLs) in patients who lack capsular support.

Methods

A prospective, nonrandomized study was performed to compare 2 groups of patients between January 2006 and February 2011. Forty eyes of 40 patients undergoing AC IOL implantation (group 1) and 34 eyes of 34 patients undergoing SF IOL implantation (group 2) were included. The mean follow-up was 22.3 ± 6.3 (range 6–42) months.

Results

The mean postoperative corrected distance visual acuity (CDVA) recorded at the last follow-up in groups 1 and 2 was 0.70 ± 0.50 and 0.65 ± 0.55, respectively (p = 0.71). A total of 35 eyes (87.5%) and 29 eyes (85.3%) in groups 1 and 2 had an improved or unchanged CDVA; 5 eyes (12.5%) and 5 eyes (14.7%) had a deteriorated CDVA. The most frequent postoperative complications in the 2 groups were transient corneal edema, elevated intraocular pressure, hypotony, and retinal detachment. The differences in the number of eyes and the total number of complications were not statistically significant for early (p = 0.06) and late (p = 0.13) complications between the 2 groups.

Conclusions

Both secondary AC IOL and SF IOL implantation with simultaneous pars plana approach of SOR are suitable for treating silicone oil–filled, aphakic eyes. No significant difference in visual outcome or major complication rates was found between the 2 groups.
Large-area versus Small-area Application of Mitomycin C during Trabeculectomy
Authors: Matlach Juliane , Panidou Ermioni , Grehn Franz , Klink Thomas
Published: August 2013

Purpose

To compare 2 different application methods of mitomycin C (MMC) in patients undergoing trabeculectomy.

Methods

This retrospective trial compared outcomes of 191 eyes that underwent trabeculectomy with small-area (96 eyes; 50.3%) and large-area (95 eyes; 49.7%) MMC application. Main outcome measures were changes in intraocular pressure (IOP), required glaucoma medications, the frequency of complications, and postsurgical interventions.

Results

Within both treatment groups, a highly significant IOP reduction was seen during follow-up (p < 0.0001). Statistical analyses revealed a significant difference in IOP between both groups. Patients treated with a larger size of surface area had a higher IOP reduction within the first postoperative year. Choroidal detachment, shallow anterior chamber, and bleb leak were seen more often in the large-area group with more aggressive MMC use. In contrast, complications associated with bleb failure such as bleb scarring were higher in the small-area group. Success rate was higher in the large-area MMC application group after 12 months.

Conclusions

Large-area treatment seems to be a more efficient application method of MMC during trabeculectomy.
Epidemiology and Intermediate-Term Outcomes of Open- and Closed-Globe Injuries in Traumatic Childhood Cataract
Authors: Khokhar Sudarshan , Gupta Shikha , Yogi Rohit , Gogia Varun , Agarwal Tushar
Published: August 2013

Purpose

To study epidemiology and intermediate-term outcomes of open- and closed-globe injuries (CGI) in traumatic childhood cataract.

Methods

In this retrospective interventional case series, demographic parameters and history including type of injury of 57 children younger than 16 years with traumatic cataract were recorded; ocular examination included best-corrected visual acuity (BCVA), slit-lamp biomicroscopy, and posterior segment evaluation. Patients underwent cataract surgery with or without intraocular lens (IOL) implantation. Main surgical outcomes at 6 months comprised BCVA, residual refractive spherical error (SE), and postoperative complications, namely visual axis opacification (VAO) and amblyopia.

Results

Bow and arrow was the most common causal agent. Open-globe injury (OGI) was 3 times more frequent than CGI. There was a significant visual gain from baseline in both groups after cataract surgery (p<0.001); residual SE was greater in OGI (1.6 ± 0.95 SD) compared to blunt trauma (0.8 ± 0.55 SD; p = 0.001). Incidence of corneal scarring, iris distortion, posterior synechiae, and intraoperative posterior capsular tear was greater with OGI (p<0.05). A total of 86% of patients were rehabilitated with a primary/secondary IOL. Single-piece IOL implantation rate (p = 0.004) was significantly greater in CGI, with no statistical difference for in-the-bag IOL (p = 0.053) and IOL implantation rate (p = 0.16). Final BCVA was significantly better for in-the-bag IOL implantation compared to sulcus fixation. Postoperative complications included amblyopia (51%) and VAO (12%).

Conclusions

Bow and arrow injury caused the maximum cases of traumatic cataract; cataract extraction resulted in significant visual improvement; and CGI tended to have better prognosis in pediatric traumatic cataracts.
Continuous Blood Purification Ameliorates Endothelial Hyperpermeability in SAP Patients with MODS by Regulating Tight Junction Proteins via ROCK
Authors: Yang Mei , Chen Xue-mei , Du Xiao-Gang , Cao Fang-Fang , Luxmi Sicharam Vijaya , Shen Qing
Published: August 2013

Background

Excessive activation of inflammatory mediator cascade during severe acute pancreatitis (SAP) is a major cause of multiple organ dysfunction and is associated with a high mortality. Recently, more and more studies have shown that continuous blood purification (CBP) could improve the prognosis of patients with multiple organ dysfunction syndrome (MODS), but the exact mechanism is still unclear. Many researchers have found that the disruption of tight junction barrier was an important factor for endothelial hyperpermeability, which played a key role in the pathogenesis of MODS. Previously, we found CBP could attenuate endothelial hyperpermeability in SAP patients with lung injury through regulating cytoskeleton reorganization mediated by RhoA/ROCK. However, the effect of CBP on the change of tight junction proteins in SAP patients with MODS was still unknown. This study aimed to investigate the role of tight junctions in endothelial hyperpermeability in SAP patients with MODS using an in vitro model, and the effect of CBP on tight junction barrier.

Methods

Before CBP and after CBP, blood samples were collected to observe hepatic and renal function, and arterial blood gas, while the APACHE II score was calculated to evaluate the severity of patients. To test whether RhoA/ROCK signaling pathway was involved, human umbilical vein endothelial cells (HUVECs) were exposed to serum samples taken from patients at specific time points during CBP, or preincubated with ROCK inhibitor, Y-27632, followed by treatment with serum. Then, the changes in endothelial cell permeability and the expression and distribution of tight junction proteins occludin and claudin-1 were observed.

Results

Compared with before CBP, the APACHE II score, serum creatinine and alanine aminotransferase decreased significantly, while PaO2/FiO2 increased significantly after CBP. Meanwhile, endothelial permeability induced by serum from patients significantly increased, while the expression of tight junction proteins occludin and claudin-1 significantly decreased, and severe disruption of occludin and claudin-1 was found in these cells. However, pretreated with Rho-kinase inhibitor, Y-27632 could lessen all of these abnormalities, and in a dose-dependent way. Endothelial hyperpermeability, the abnormal expression and distribution of occludin and claudin-1 were attenuated in HUVECs treated with serum from patients after CBP treatment.

Conclusions

The abnormality of tight junctions mediated by ROCK was an important mechanism for endothelial hyperpermeability induced by serum from SAP patients with MODS. CBP could ameliorate the disorganization and redistribution of tight junction proteins, hence improve the endothelial permeability.
Central Closed Chest Implantation of Extracorporeal Membrane Oxygenation to Prevent Limb Ischemia
Authors: Deschka Heinz , Machner Matthias , El Dsoki Salah , Alken Aiman , Wimmer-Greinecker Gerhard
Published: August 2013

Purpose

Extracorporeal membrane oxygenation (ECMO) support is a widely accepted treatment option for patients with cardiogenic shock, but it is still related to a high incidence of severe complications and death. We present an alternative implantation technique to prevent life-threatening vascular complications.

Methods

Between January 2008 and January 2011, a total of 28 patients with acute myocardial failure and consecutive cardiogenic shock required ECMO as supportive treatment. Pre-implantation procedures were isolated CABG, CABG combined with mitral valve reconstruction or ventricular septal defect closure, respectively. The implantation of ECMO was performed by connecting the ascending aorta via an 8 mm Dacron prosthesis with the arterial line and percutaneous puncture of the femoral vein. The chest was closed after installation of ECMO was completed. The arterial line was directed subxyphoidally and removal was possible without thoracotomy.

Results

Average support duration was 8.7 ∓ 3.9 days. An additional intra-aortic balloon pump was used in 23 patients (89.3%). Cerebrovascular events occurred in 21.4% and gastrointestinal complications in 9.1%. Acute renal failure was treated with continuous renal replacement therapy in 64.3%. In eight cases a systemic infection had to be treated. One patient with pre-existing severe peripheral arterial disease suffered from limb malperfusion, requiring leg amputation. Twelve patients were successfully weaned from ECMO and 8 patients (28.6%) were discharged from hospital.

Conclusions

This alternative cannulation strategy offers effective cardiopulmonary support while minimizing the risk of limb hypo- or hyperperfusion without requiring reopening of the thorax.
Fleet Assignment with a Continuously Adjustable Flight Schedule
Authors: Wang Ta-Chung , Li Ching-Mei
Published: August 2013

Fleet assignment is one of the four major steps in airline planning. The objective of the fleet assignment problem (FAP) is to determine which type of aircraft should fly on each flight leg while considering different characteristics and costs associated with different fleet types. This article proposes a multiphase approach to solving the FAP. Backward arcs were used to solve basic FAPs. Then the backward arcs were eliminated by using linear programming. By allowing backward time, our formulation has a similar level of flexibility as the fleet assignment model with time-window (FAMTW), but the problem size grows much slower than FAMTW. Examples using real data obtained from a major airline are included in this article.
Risk Assessment and Operational Approaches to Manage Risk in Global Supply Chains
Authors: Kumar Sameer , Boice Brooke C. , Shepherd Mark J.
Published: August 2013

Global supply chains encounter low-occurrence/high-disruption risks that are not addressed adequately by adding inventory. Although many operational risk-management tools exist, these tools and methods have not been synthesized into a comprehensive management system. This article evaluates risk-assessment methodologies and operational approaches to manage risk in global supply chains, and proposes a closed-loop risk-management system with inputs, outputs, and feedback. A focal OEM company's global supply chain is used to analyze the feasibility of proposed risk assessment and an operational business model. A tool for determining supply chain root causes (supply chain failure modes and effects analysis—FMEA), and a tool for supply chain risk assessment (global supply chain risk-management scorecard) are presented. Results of completed supply chain FMEAs and global supply chain risk-management scorecards are reported. Supply chains need scalable risk-management tools to ensure supply continuity during major disruptions while balancing efficiency considerations during normal operations. The proposed management system includes scalable strategic and tactical tools to identify risks, determine root causes, assess change-management capabilities, monitor performance, and evaluate execution of the system.
No a la violencia contra la mujer
Authors: Jagger Bianca
Published: August 2013

Bianca Jagger, pensadora y activista mundial por la defensa de los derechos humanos, esta vez centra su análisis en la violencia contra la mujer en el mundo y en Nicaragua, su país natal, a la cual considera una verdadera pandemia que hay que detener. Ilustra la situación con abundante información en países como: Guatemala, India, Sudáfrica, la ex Yugoslavia, Ruanda, etc. DOI: http://dx.doi.org/10.5377/cultura.v19i60.1171 Cultura de Paz • Año XIX • N° 60 • Mayo - Agosto 2013 P / 5-9
Poesía de Milagros Terán
Authors: Martínez Rivas Carolos
Published: August 2013

La poesía de Milagros Terán, una de las poetas más importantes de Nicaragua, es presentada en Cultura de Paz. La belleza de lo cotidiano en la trascendencia de la palabra poética. Carlos Martínez Rivas, extraordinario y singular poeta de nuestra lengua, escribe una carta valorando textos de Milagros Terán. DOI: http://dx.doi.org/10.5377/cultura.v19i60.1176 Cultura de Paz • Año XIX • N° 60 • Mayo - Agosto 2013 P / 33-37
Active Sound Localization in a Symmetric Environment
Authors: Brindza Jordan , Thomas Ashleigh , Lee Spencer , McDermid William , He Yizheng , Lee Daniel D.
Published: August 2013

Localization for humanoid robots becomes difficult when events that disrupt robot positioning information occur. This holds especially true in symmetric environments because landmark data may not be sufficient to determine orientation. We propose a system of localizing humanoid robots in a known, symmetric environment using a Rao-Blackwellized particle filter and a sound localization system. This system was used in the RoboCup Standard Platform League, and has been found to reduce the amount of own-goals scored as compared with the previously used localization system without sound.
Kinematics and the Implementation of a Modular Caterpillar Robot in Trapezoidal Wave Locomotion
Authors: Wei Hongxing , Cui Yuanyang , Li Haiyuan , Tan Jindong , Guan Yong , Li Yong-Dong
Published: August 2013

With the development of bionic engineering, research into bionic robots has become a popular topic. In this field, the design of robotic mechanisms to realize the locomotion of insects forms a significant research branch. The current paper presents a caterpillar robotic mechanism that is composed of our newly-developed self-assembly modular robots (Sambot). A trapezoidal wave locomotion gait is planned for the caterpillar mechanism and the kinematics equations are established and solved analytically for such locomotion. The variations of the kinematics quantities are illustrated and discussed. The variation of the jump of the angular acceleration indicates that it is better to apply the trapezoidal wave gait to low velocity situations. Finally, the obtained data of the kinematics quantities is used to perform the gait control locomotion experiment and the errors of the experimental data are analysed in depth.
Vibration Control of Flexible Mode for a Beam-Type Substrate Transport Robot
Authors: Park Cheol Hoon , Park Dong Il , Park Joo Han
Published: August 2013

Beam-type substrate transport robots are widely used to handle substrates, especially in the solar cell manufacturing process. To reduce the takt time and increase productivity, accurate position control becomes increasingly important as the size of the substrate increases. However, the vibration caused by the flexible forks in beam-type robots interferes with accurate positioning, which results in long takt times in the manufacturing process. To minimize the vibration and transport substrates on the fork as fast as possible, the trajectories should be prevented from exciting the flexible modes of the forks. For this purpose, a fifth-order polynomial trajectory generator and input shaping were incorporated into the controller of the beam-type robot in this study. The flexible modes of the forks were identified by measuring the frequency response function (FRF), and the input shaping was designed so as not to excite the flexible modes. The controller was implemented by using MATLAB/xPC Target. In this paper, the design procedure of input shaping and its effectiveness for vibration attenuation in both “no load” and “load” cases is presented.
Comparison between Nano-and Micro-Sized Copper Particles as Fillers in NAO Friction Materials
Authors: Sharma Sanjeev , Bijwe Jayashree , Kumar Mukesh
Published: August 2013

In this study, the comparative role of nano-and micro-sized Cu powder in exploring the influence on the tribo-performance of friction materials in the form of brake-pads is discussed. Two friction composites were developed by keeping allthe ingredients constant and varying the size of the selected filler (copper powder). The developed micro-composite contained 10 % (wt) Cu (400–600 μm) while in the nano-composite only a part (2 %) of the micro-powder was replaced by a nano-powder (50–200 nm) since 10 % replacement was not possible because of the agglomeration problem. One more composite without any metallic filler was also developed for the sake of comparison. For tribo-evaluation, the brake-dynamometer testing was selected. The performance properties, such as friction, wear and sensitivity of friction towards load, speed and temperature were studied as per industrial practice. It was concluded that the inclusion of nano-Cu powder (just 2 %) improved the performance properties significantly. Micro-composite was a moderate performer while composite without Cu powder proved the poorest. The quality of a tribo-layer transferred to the disc as studied in SEM was confirmed to be one of the reasons for the improved performance of the nano-composite.
Taxonomy of the Crematogaster degeeri-species-assemblage in the Malagasy region (Hymenoptera: Formicidae)
Authors: Blaimer Bonnie B. , Fisher Brian L.
Published: August 2013

We revise the species-level taxonomy of the Crematogaster (Crematogaster) degeeri-species-assemblage, a group of related ants occuring in Madagascar and the wider Malagasy region, and further provide an identification key to all species-groups of the genus Crematogaster in this region. Within the C. degeeri-assemblage, we recognize twelve species based upon morphological data from worker, queen and male ants, as well as genetic data from the barcode region of cytochrome oxidase I. Seven new species are described: Crematogaster alafara Blaimer sp. nov., C. bara Blaimer sp. nov., C. mafybe Blaimer sp. nov., C. maina Blaimer sp. nov., C. malahelo Blaimer sp. nov., C. masokely Blaimer sp. nov., C. ramamy Blaimer sp. nov. Crematogaster tricolor Gerstäcker, 1859 (stat. rev.) and C. dentata Dalla Torre, 1893 (stat. nov.) are raised to species level, and the following new synonymies are proposed: Crematogaster degeeri lunaris Santschi, 1928 as a synonym of C. degeeri Forel, 1886; Crematogaster sewelli improba Forel, 1907 and C. sewelli mauritiana Forel, 1907 as synonyms of C. dentata Dalla Torre, 1893, and C. pacifica Santschi, 1919 as a synonym of C. lobata Emery, 1895. Species descriptions, images, and distribution maps and identification keys based on worker ants, as well as on queen ants where available, are presented for all twelve species. In addition, we present a molecular gene tree for cytochrome oxidase I and summarize levels of sequence divergence within and between species of the C. degeeri-species-assemblage. Our findings are discussed in the light of previous work on Malagasy Crematogaster ants.
Phase II Study of Irinotecan in Combination with Capecitabine on a 3-Weekly Schedule as First-Line Chemotherapy for Patients with Metastatic or Locally Advanced Colorectal Cancer
Authors: Antonieta Salud  , Vicente Alonso  , Pilar Escudero  , Miguel Burillo  , Cristina Martín  , Fernando Rivera  , Alfonso Yubero  , Carlos García-Girón  , Alberto Muñoz
Published: August 2013

Background: Capecitabine has demonstrated non inferiority efficacy and improved safety compared with 5-fluorouracil (5-FU)/leucovorin (LV) in metastatic colorectal cancer (mCRC) without the inconvenience of an infusional therapy. The aim of the present study was to evaluate the efficacy and safety of capecitabine plus irinotecan (CPT-11) given every 3 weeks (XELIRI regimen) as first-line treatment in locally advanced (LA) or mCRC, in order to improve patient tolerability and quality of life. Patients and methods: Patients with LA or mCRC received CPT-11 225 mg/m2(180 mg/m2 if > 65 years old) on day 1 and capecitabine 1000 mg/m2 (750 mg/m2 if > 65 years old) twice daily on days 2-15 every 3 week. Primary endpoints were objective response rate (ORR) and toxicity of the chemotherapeutic regimen. Secondary endpoints of overall survival (OS), progression-free survival (PFS), response duration and quality of life were also evaluated. Results: Ninety-one patients were included. In an intention-to-treat analysis, complete response was achieved in 3 patients and partial response in 27, for an ORR of 33%. The disease control rate (ORR + stable disease) was 72.5%. Median time to progression and OS were 9.3 and 17.1 months respectively. Grade 3/4 neutropenia and diarrhea were the most commonly reported adverse vents. Conclusion: The XELIRI regimen given every 3 weeks, as first-line therapy of LA or mCRC was effective and well tolerated, including elderly patients. Severe gastrointestinal toxicities and hematological events were manageable.
Urolithiasis in Children with Acute Lymphoblastic Leukemia
Authors: Mariane Gouvêa Monteiro de Camargo  , Ricardo Jordão Duarte  , Lilian Maria Cristofani  , Vicente Odone Filho  , Miguel Srougi
Published: August 2013

Background: Urinary tract lithiasis(UTL) requires careful intervention, especially when associated with other diseases. The purpose of this study was to assess the outcome of symptomatic UTL in children with acute lymphoblastic leukemia (ALL) and to evaluate its correlation with the anti-neoplastic treatment phase and drugs, and to assess the need for intervention, results, complications and follow-up.
Procedure: The charts of 350 patients with ALL (1990-2008) were retrospectively evaluated. Signs and symptoms, methods of diagnosis, complications, treatment approach, resolution, anti-leukemic treatment phase when the UTL was diagnosed, drugs used and interference on ALL treatment were recorded.
Results: A total of 12/350 patients (3.4%) had UTL (14 stones). The median age was 7.6 years. Pain was present in 60%, hematuria in 20% and both in 20%. The median stone size was 4mm (3- 13.8mm). Three patients required hospitalization (pain) and one had chemotherapy discontinued due to severe hematuria and blood support. At the time of diagnosis of UTL, four patients were in the induction phase with corticosteroids, four were in the maintenance phase with corticosteroids, two were in the maintenance phase without corticosteroids, and one had a recurrence after therapy, and two were off-therapy. 11/13 episodes of UTL had spontaneous resolution, two underwent successful extracorporeal shockwave lithotripsy, and one patient remains with an asymptomatic stone.
Conclusions: 3.4% of children with ALL presented symptomatic UTL. While the majority of cases have been resolved spontaneously, hospitalization and delay in chemotherapy have increased morbidity in these children.
Gleason Score Discrepancies Between Needle Biopsies and Radical Prostatectomy Specimens in an African Men: Clinical Implication
Authors: Abdellatif Janane  , Fouad Hajji  , Youssef Dakkak  , Mohamed Ghadouane  , Ahmed Ameur  , Mohamed Abbar  , Abderhman Albouzidi
Published: August 2013

Objective:Gleason scores, as determined by 18-gauge core needle biopsies (NB), were compared with both Gleason scores and the pathological staging of corresponding radical prostatectomy( RP) specimens. The goal was to evaluate the clinical implication and the prognostic impact of these discrepancies. Methods: Records of 234 consecutive patients undergoing a radical retro pubic prostatectomy between 2001 and 2012 were reviewed. In total, all our patients were enrolled, al1 of whom had been diagnosed with adenocarcinoma by transrectal needle biopsies using an 18-gauge automated spring-loaded biopsy gun. Results: Grading errors were greatest with wel1-differentiated tumors. The accuracy was 18 (23%) for Gleason scores of 2-4 on needle biopsy. Of the 108 evaluable patients with Gleason scores of 5-7 on needle biopsy, 84 (78%) were graded correctly. All of the Gleason scores of 8-10 on needle biopsy were graded correctly. 54 of 162 patients (33%), with a biopsy Gleason score of < 7 had their cancer upgraded to above 7. Tumors in 18 patients (60%) with both a Gleason score < 7 on the needle biopsy and a Gleason score of 7 for the prostatectomy specimen were confined to the prostate. Conclusion: The potential for grading errors is greatest with well-differentiated tumors and in patients with a Gleason score of < 7 on the needle biopsy. Predictions using Gleason scores are sufficiently accurate to warrant its use with all needle biopsies, recognizing that the potential for grading errors is greatest with well-differentiated tumors.
Hacia un marco constructivista realista para el análisis de las relaciones internacionales desde las periferias
Authors: Becerra Lucas
Published: August 2013

This article aims to systematize and articulate, within a theoretical proposal, the problems emerging from the absence of a conceptual framework that can account for international economy, politics and security dynamics and processes of peripheral countries. The article presents a proposal of a conceptual and methodological framework called peripheral realist constructivism. This framework arises from the triangulation, within a systemic approach, of notions of realism and constructivism, tailored from a South’s view. Beginning with a basic typology, the analysis deployed here finds that the international system is the relational outcome (material and notional) of its component units: the system is co-constructed in different senses for central and peripheral agents. Then, the framework allows analyze the behavior of the units by understanding how perceptions of threat and benefit are generated at the systemic level. Finally, two analytical models are constructed for understanding political and economic dynamics that streamline associative dynamics in the peripheries.
Una apertura del concepto de sociedad civil: desajustes, diálogos y contribuciones teóricas desde el Sur Global
Authors: Ballestrin Luciana , Losekann Cristiana
Published: August 2013

El concepto de sociedad civil ha sido rearticulado por la Teoría Política en las últimas décadas del siglo XX, vinculándose directamente en los contextos redemocratizados con las nociones de espacio público, ciudadanía, deliberación y participación política. En diferentes partes del mundo, varios estudios e investigaciones acerca de sociedades civiles (re)emergentes han desafiado el núcleo teórico/normativo elaborado por autores referenciales del Norte Global. Este artículo realiza una revisión crítica del concepto de sociedad civil, originalmente concebido y pensado por Europa. Si bien el concepto de sociedad civil ha sido informado y dinamizado por las manifestaciones en el Sur Global desde los ochenta, la gran mayoría de sus intérpretes con proyección mundial ha permanecido situada en el eje teórico del Norte. Nuestro principal objetivo es presentar algunos aportes sobre las especificidades y novedades que el Sur Global trae a la categoría, con vistas a su apertura conceptual para nuevos sentidos y significados. Defendemos que ésta ha sido capaz de romper con la geopolítica del conocimiento y con la colonialidad del saber en el nivel teórico global.
Inflammatory Cytokines Presented from Polymer Matrices Differentially Generate and Activate DCs In Situ
Authors: Ali Omar A. , Tayalia Prakriti , Shvartsman Dmitry , Lewin Sarah , Mooney David J.
Published: August 2013

During infection, inflammatory cytokines mobilize and activate dendritic cells (DCs), which are essential for efficacious T cell priming and immune responses that clear the infection. Here, macroporous poly(lactide‐co‐glycolide) (PLG) matrices are designed to release the inflammatory cytokines GM‐CSF, Flt3L, and CCL20 in order to mimic infection‐induced DC recruitment. The ability of these infection mimics to function as cancer vaccines is tested via induction of specific anti‐tumor T cell responses. All vaccine systems tested are able to confer specific anti‐tumor T cell responses and long‐term survival in a therapeutic B16‐F10 melanoma model. However, GM‐CSF and Flt3L vaccines result in similar survival rates and outperformed CCL20‐loaded scaffolds, even though they have differential effects on DC recruitment and generation. GM‐CSF signaling is identified as the most potent chemotactic factor for conventional DCs and significantly enhanced surface expression of MHC(II) and CD86(+), which are utilized for priming T cell immunity. In contrast, the use of Flt3L vaccines leads to greater numbers of plasmacytoid DCs, correlating with increased levels of T cell‐priming cytokines that amplify T cell responses. These results demonstrate that 3D polymer matrices modified to present inflammatory cytokines may be utilized to effectively mobilize and activate different DC subsets in vivo for immunotherapy.
Mechanically Controllable Break Junctions for Molecular Electronics
Authors: Xiang Dong , Jeong Hyunhak , Lee Takhee , Mayer Dirk
Published: August 2013

A mechanically controllable break junction (MCBJ) represents a fundamental technique for the investigation of molecular electronic junctions, especially for the study of the electronic properties of single molecules. With unique advantages, the MCBJ technique has provided substantial insight into charge transport processes in molecules. In this review, the techniques for sample fabrication, operation and the various applications of MCBJs are introduced and the history, challenges and future of MCBJs are discussed.
Phase‐Change Nanodot Material for an Optical Memory
Authors: Yamada Noboru , Kojima Rie , Hisada Kazuya , Mihara Takashi , Tsuchino Akio , Fujinoki Norihito , Birukawa Masahiro , Matsunaga Toshiyuki , Yasuda Nobuhiro , Fukuyama Yoshimitsu , Ito Kiminori , Tanaka Yoshihito , Kimura Shigeru , Takata Masaki
Published: August 2013

A sputtered phase‐change material, Ge10Sb90, processed into dots with a height and diameter of 50 nm, shows rapid crystallization triggered by 300 ps laser excitation. Crystallization takes place with a short time delay of approximately 70 ns for a sample with Sb seed layers. The delay becomes just 15–20 ns when a NiCr layer is provided to control the heating–cooling profile. The nanodot sample requires less energy for crystallization, with a large optical change equivalent to that of the blanket film. These results demonstrate that the nanodot phase‐change material could be a possible candidate for next‐generation “green” optical storage.
Targeted methylation testing of a patient cohort broadens the epigenetic and clinical description of imprinting disorders
Authors: Poole Rebecca L. , Docherty Louise E. , Al Sayegh Abeer , Caliebe Almuth , Turner Claire , Baple Emma , Wakeling Emma , Harrison Lucy , Lehmann Anna , Temple I. Karen , Mackay Deborah J.G. ,   On behalf of the International Clinical Imprinting Consortium
Published: August 2013

Imprinting disorders are associated with mutations and epimutations affecting imprinted genes, that is those whose expression is restricted by parent of origin. Their diagnosis is challenging for two reasons: firstly, their clinical features, particularly prenatal and postnatal growth disturbance, are heterogeneous and partially overlapping; secondly, their underlying molecular defects include mutation, epimutation, copy number variation, and chromosomal errors, and can be further complicated by somatic mosaicism and multi‐locus methylation defects. It is currently unclear to what extent the observed phenotypic heterogeneity reflects the underlying molecular pathophysiology; in particular, the molecular and clinical diversity of multilocus methylation defects remains uncertain. To address these issues we performed comprehensive methylation analysis of imprinted genes in a research cohort of 285 patients with clinical features of imprinting disorders, with or without a positive molecular diagnosis. 20 of 91 patients (22%) with diagnosed epimutations had methylation defects of additional imprinted loci, and the frequency of developmental delay and congenital anomalies was higher among these patients than those with isolated epimutations, indicating that hypomethylation of multiple imprinted loci is associated with increased diversity of clinical presentation. Among 194 patients with clinical features of an imprinting disorder but no molecular diagnosis, we found 15 (8%) with methylation anomalies, including missed and unexpected molecular diagnoses. These observations broaden the phenotypic and epigenetic definitions of imprinting disorders, and show the importance of comprehensive molecular testing for patient diagnosis and management. © 2013 Wiley Periodicals, Inc.
Severe hypospadias and its association with maternal‐placental factors
Authors: Huisma Felicity , Thomas Marion , Armstrong Linlea
Published: August 2013

The neonate born small for gestational age (SGA, birth weight <10th centile) with severe hypospadias, and without the presence of other malformations, is a presentation which clinical geneticists are asked to assess. However, this combination is not well addressed in the literature. Epidemiological studies point to an increased incidence and severity of hypospadias in SGA infants, but leave open the possibility that the association is attributable to the population subset who have multiple congenital abnormalities or genetic syndromes. Epidemiological studies unselected for birth weight have also identified an association between hypospadias with some risk factors tied to SGA, including prematurity, preeclampsia, and placental insufficiency. This study was developed after being involved in the care of several boys with SGA and severe hypospadias who were premature, with gestational histories of maternal hypertension and oligohydramnios. No underlying syndromes, hormonal abnormalities, or contributory family histories could be found. We hypothesized that severe hypospadias may in some cases be causally related to maternal‐placental factors rather than primary fetal abnormalities. A retrospective study of a cohort of singletons born SGA with unexplained hypospadias was conducted. We compared their gestational histories and hypospadias severities with those born at normal birth weights. The cohort with SGA had an increased ratio of severe to mild hypospadias. Further, within the cohort of SGA boys, there was a higher prevalence of prematurity, maternal hypertension, and oligohydramnios among those with severe vs mild hypospadias. Small sample sizes are limiting. © 2013 Wiley Periodicals, Inc.
Expanding the spectrum of microdeletion 4q21 syndrome: A partial phenotype with incomplete deletion of the minimal critical region and a new association with cleft palate and pierre robin sequence
Authors: Bhoj Elizabeth , Halbach Sara , McDonald‐McGinn Donna , Tan Christopher , Lande Rachel , Waggoner Darrel , Zackai Elaine
Published: August 2013

Microdeletion 4q21 syndrome has been described in about a dozen patients with deletions ranging from 3.2 to 15.1 MB with similar features including the distinctive facial characteristics of broad forehead, hypertelorism, and prominent front teeth, with severe growth delay, developmental delay, and neonatal hypotonia. A 1.37 MB minimal critical region has been described that accounts for this shared phenotype and includes five known genes: PRKG2, RASGEF1B, HNRNPD, HNRPDL, and ENOPH1. We report on two new patients found through single nucleotide polymorphism (SNP) microarray testing that expand the reported phenotype. Patient 1 has a novel deletion of 2.0 MB, the smallest reported deletion, which involves only a partial deletion of the minimal critical region, including the genes HNRNPD, HNRPDL, and ENOPH1. She shares much of the typical phenotype including moderate developmental delay, unusual facial features, small hands and feet, but not any growth delay or neonatal hypotonia. This patient allows further genotype–phenotype correlation of the genes in the minimal critical region, and supports that heterozygous loss of PRKG2 leads to the growth delay. Patient 2 has a novel 3.4 MB deletion that includes the entire critical region, and has typical features, but also presented with cleft palate and Pierre Robin sequence, which have not been previously described. A gene reported to be associated with inherited cleft palate, SCD5, is in the deleted region in this patient, which suggests it may be playing a role in palate formation. Taken together, these patients allow for an expansion of the microdeletion 4q21 syndrome and provide candidate genes for particular features of the phenotype. © 2013 Wiley Periodicals, Inc.
Long‐term follow‐up of four patients with langer–giedion syndrome: Clinical course and complications
Authors: Schinzel Albert , Riegel Mariluce , Baumer Alessandra , Superti‐Furga Andrea , Moreira Lilia M.A. , Santo Layla D.E. , Schiper Patricia P. , Carvalho José Henrique Dantas , Giedion Andres
Published: August 2013

Long‐term observations of individuals with the so‐called Langer–Giedion (LGS) or tricho–rhino‐phalangeal type II (TRPS2) are scarce. We report here a on follow‐up of four LGS individuals, including one first described by Andres Giedion in 1969, and review the sparse publications on adults with this syndrome which comprises ectodermal dysplasia, multiple cone‐shaped epiphyses prior to puberty, multiple cartilaginous exostoses, and mostly mild intellectual impairment. LGS is caused by deletion of the chromosomal segment 8q24.11–q24.13 containing among others the genes EXT1 and TRPS1. Most patients with TRPS2 are only borderline or mildly cognitively delayed, and few are of normal intelligence. Their practical skills are better than their intellectual capability, and, for this reason and because of their low self‐esteem, they are often underestimated. Some patients develop seizures at variable age. Osteomas on processes of cervical vertebrae may cause pressure on cervical nerves or dissection of cerebral arteries. Joint stiffness is observed during childhood and changes later to joint laxity causing instability and proneness to trauma. Perthes disease is not rare. Almost all males become bald at or soon after puberty, and some develop (pseudo) gynecomastia. Growth hormone deficiency was found in a few patients, TSH deficiency so far only in one. Puberty and fertility are diminished, and no instance of transmission of the deletion from a non‐mosaic parent to a child has been observed so far. Several affected females had vaginal atresia with consequent hydrometrocolpos. © 2013 Wiley Periodicals, Inc.
Further characterization of atypical features in auriculocondylar syndrome caused by recessive PLCB4 mutations
Authors: Kido Yasuhiro , Gordon Christopher T. , Sakazume Satoru , Bdira Eya Ben , Dattani Mehul , Wilson Louise C. , Lyonnet Stanislas , Murakami Nobuyuki , Cunningham Michael L. , Amiel Jeanne , Nagai Toshiro
Published: August 2013

Auriculocondylar syndrome (ACS) is a branchial arch syndrome typically inherited in an autosomal dominant fashion. Patients with ACS display the following core symptoms with varying severity: a specific malformation of the external ear, known as a “question mark ear,” micrognathia and mandibular condyle hypoplasia. Recently, phospholipase C, β 4 (PLCB4) mutations were identified as the major cause of autosomal dominant ACS, with mutations of the PLCB4 catalytic domain predicted to have a dominant negative effect. In addition, one ACS patient born to related parents harbored a homozygous partial deletion of PLCB4, and presented with ACS plus central apnea and macropenis; these features had not been previously reported in association with ACS. His parents, each with a heterozygous partial PLCB4 deletion, were phenotypically normal, suggesting autosomal recessive inheritance of ACS, with complete loss of function of PLCB4 predicted in the patient. We herein describe two brothers with ACS caused by compound heterozygous splice site mutations in PLCB4. The patients were born to the same unrelated and healthy parents, with each parent harboring one of the mutations, indicating autosomal recessive ACS. Both patients reported here had mixed apneas, gastrointestinal transit defects and macropenis, in addition to typical craniofacial features of ACS. This is the first example of ACS caused by compound heterozygous splice site mutations in PLCB4, the second autosomal recessive case of ACS confirmed by molecular analysis, and strengthens the link between complete loss of function of PLCB4 and extra‐craniofacial features. © 2013 Wiley Periodicals, Inc.