bluuebunny/crossref_metadata_2025_split huggingface dataset:
Influence of the ionic form of a cation-exchange adsorbent on chromatographic separation of galactooligosaccharides
Authors: Moravčík Juraj , Gramblička Michal , Wiśniewski Łukasz , Vaňková Katarína , Polakovič Milan 
Published: March 2012

AbstractSeparation performance of sodium, lithium, potassium and hydrogen ionic forms of a commercial cation exchanger, Diaion UBK 530, based on a styrene-divinylbenzene copolymer resin support was studied. The adsorbents were applied to the separation of galactooligosaccharides from their mixture with lactose, glucose and galactose. Separation performance of the hydrogen ionic form of the adsorbent was superior to that of other adsorbent forms. A much higher yield of galactooligosaccharides of the requested purity was achieved for this adsorbent form owing to its better kinetic properties. Adsorption properties of individual adsorbents were discussed in relation to possible adsorption mechanisms.
FDI(R) for satellites: How to deal with high availability and robustness in the space domain?
Authors: Olive Xavier 
Published: March 2012

FDI(R) for satellites: How to deal with high availability and robustness in the space domain?The European leader for satellite systems and at the forefront of orbital infrastructures, Thales Alenia Space, is a joint venture between Thales (67%) and Finmeccanica (33%) and forms with Telespazio a Space Alliance. Thales Alenia Space is a worldwide reference in telecoms, radar and optical Earth observation, defence and security, navigation and science. It has 11 industrial sites in 4 European countries (France, Italy, Spain and Belgium) with over 7200 employees worldwide. Satellite evolution and the wish to design more autonomous missions imply the enhancement of the satellite architecture and special attention paid to fault management (i.e., Fault Detection, Isolation and Recovery, or FDIR, in space). Nevertheless, the constraints on FDIR techniques and strategies remain the same as for standard missions: robustness, reactive detection, quick isolation/identification and validation. This paper gives an introduction to Fault Tolerance (FT) in the space domain and some principles for the coming FT architectures. The current context of FDIR is presented by describing the approach implemented on telecommunication satellites and, more precisely, on one of the most FDIR sensible subsystems: the AOCS (Attitude and Orbit Control System). Following the current state of FDIR in the space domain, some perspectives are given such as a centralized distributed FDIR strategy for the next generation of autonomous satellites as well as some research tracks and hybrid diagnosis.
An LPV pole-placement approach to friction compensation as an FTC problem
Authors: Patton Ron , Chen Lejun , Klinkhieo Supat 
Published: March 2012

An LPV pole-placement approach to friction compensation as an FTC problemThe concept of combining robust fault estimation within a controller system to achieve active Fault Tolerant Control (FTC) has been the subject of considerable interest in the recent literature. The current study is motivated by the need to develop model-based FTC schemes for systems that have no unique equilibria and are therefore difficult to linearise. Linear Parameter Varying (LPV) strategies are well suited to model-based control and fault estimation for such systems. This contribution involves pole-placement within suitable LMI regions, guaranteeing both stability and performance of a multi-fault LPV estimator employed within an FTC structure. The proposed design strategy is illustrated using a nonlinear two-link manipulator system with friction forces acting simultaneously at each joint. The friction forces, regarded as a special case of actuator faults, are estimated and their effect is compensated within a polytope controller system, yielding a robust form of active FTC that is easy to apply to real robot systems.
Robust multisensor fault tolerant model-following MPC design for constrained systems
Authors: Yetendje Alain , Seron Maria , De Doná José 
Published: March 2012

Robust multisensor fault tolerant model-following MPC design for constrained systemsIn this paper, a robust fault-tolerant control strategy for constrained multisensor linear systems, subject to sensor faults and in the presence of bounded state and output disturbances, is proposed. The scheme verifies that, for each sensors-estimator combination, suitable residual variables lie inside pre-computed sets and selects a more appropriate combination based on a chosen criterion. An active fault tolerant output feedback controller yields an MPC-based control law and, by means of the notion of a "tube" of trajectories, we ensure robust closed-loop exponential stability and good performance in the fault-free case and under the occurrence of abrupt sensor faults.
Delayed complication after Gamma Knife surgery for mesial temporal lobe epilepsy
Authors: Usami Kenichi , Kawai Kensuke , Koga Tomoyuki , Shin Masahiro , Kurita Hiroki , Suzuki Ichiro , Saito Nobuhito 
Published: March 2012

Object
Despite the controversy over the clinical significance of Gamma Knife surgery (GKS) for refractory mesial temporal lobe epilepsy (MTLE), the modality has attracted attention because it is less invasive than resection. The authors report long-term outcomes for 7 patients, focusing in particular on the long-term complications.

Methods
Between 1996 and 1999, 7 patients with MTLE underwent GKS. The 50% marginal dose covering the medial temporal structures was 18 Gy in 2 patients and 25 Gy in the remaining 5 patients.

Results
High-dose treatment abolished the seizures in 2 patients and significantly reduced them in 2 others. One patient in this group was lost to follow-up. However, 2 patients presented with symptomatic radiation necrosis (SRN) necessitating resection after 5 and 10 years. One patient who did not need necrotomy continued to show radiation necrosis on MRI after 10 years. One patient died of drowning while swimming in the sea 1 year after GKS, before seizures had disappeared completely.

Conclusions
High-dose treatment resulted in sufficient seizure control but carried a significant risk of SRN after several years. Excessive target volume was considered as a reason for delayed necrosis. Drawbacks such as a delay in seizure control and the risk of SRN should be considered when the clinical significance of this treatment is evaluated.
Activation of the Classical Complement Pathway by Bacillus anthracis Is the Primary Mechanism for Spore Phagocytosis and Involves the Spore Surface Protein BclA
Authors: Gu Chunfang , Jenkins Sarah A. , Xue Qiong , Xu Yi 
Published: March 2012

Abstract
Interactions between spores of Bacillus anthracis and macrophages are critical for the development of anthrax infections, as spores are thought to use macrophages as vehicles to disseminate in the host. In this study, we report a novel mechanism for phagocytosis of B. anthracis spores. Murine macrophage-like cell line RAW264.7, bone marrow-derived macrophages, and primary peritoneal macrophages from mice were used. The results indicated that activation of the classical complement pathway (CCP) was a primary mechanism for spore phagocytosis. Phagocytosis was significantly reduced in the absence of C1q or C3. C3 fragments were found deposited on the spore surface, and the deposition was dependent on C1q and Ca2+. C1q recruitment to the spore surface was mediated by the spore surface protein BclA, as recombinant BclA bound directly and specifically to C1q and inhibited C1q binding to spores in a dose-dependent manner. C1q binding to spores lacking BclA (ΔbclA) was also significantly reduced compared with wild-type spores. In addition, deposition of both C3 and C4 as well as phagocytosis of spores were significantly reduced when BclA was absent, but were not reduced in the absence of IgG, suggesting that BclA, but not IgG, is important in these processes. Taken together, these results support a model in which spores actively engage CCP primarily through BclA interaction with C1q, leading to CCP activation and opsonophagocytosis of spores in an IgG-independent manner. These findings are likely to have significant implications on B. anthracis pathogenesis and microbial manipulation of complement.
Antigen Recognition Is Facilitated by Invadosome-like Protrusions Formed by Memory/Effector T Cells
Authors: Sage Peter T. , Varghese Laya M. , Martinelli Roberta , Sciuto Tracey E. , Kamei Masataka , Dvorak Ann M. , Springer Timothy A. , Sharpe Arlene H. , Carman Christopher V. 
Published: March 2012

Abstract
Adaptive immunity requires that T cells efficiently scan diverse cell surfaces to identify cognate Ag. However, the basic cellular mechanisms remain unclear. In this study, we investigated this process using vascular endothelial cells, APCs that possess a unique and extremely advantageous, planar morphology. High-resolution imaging revealed that CD4 memory/effector T cells dynamically probe the endothelium by extending submicron-scale, actin-rich “invadosome/podosome-like protrusions” (ILPs). The intimate intercellular contacts enforced by ILPs consistently preceded and supported T cell activation in response to endothelial MHC class II/Ag. The resulting calcium flux stabilized dense arrays of ILPs (each enriched in TCR, protein kinase C-θ, ZAP70, phosphotyrosine, and HS1), forming what we term a podo-synapse. Similar findings were made using CD8 CTLs on endothelium. Furthermore, careful re-examination of both traditional APC models and professional APCs suggests broad relevance for ILPs in facilitating Ag recognition. Together, our results indicate that ILPs function as sensory organelles that serve as actuators of immune surveillance.
Assessing Absolute and Relative Pro-poor Growth, with an Application to Selected African Countries
Authors: Bibi Sami , Duclos Jean-Yves , Verdier-Chouchane Audrey 
Published: March 2012

Abstract
This paper proposes a multidimensional procedure for jointly assessing the absolute and relative pro-poorness of growth. It is also a procedure for testing whether poverty comparisons can be made over classes of indices that incorporate both absolute and relative views of poverty. Besides being robust to whether pro-poor judgements should be absolute or relative, the procedure is also robust to choosing over a class of weights to aggregate the impact of growth on the poor as well as over ranges of absolute and relative poverty lines. The test is applied to distributional changes in five middle- and four lower-income African countries, countries that have witnessed different impacts of growth in the last two decades.
Preventing the Reconstruction of the Polar Discontinuity at Oxide Heterointerfaces
Authors: Boschker H. , Verbeeck J. , Egoavil R. , Bals S. , van Tendeloo G. , Huijben M. , Houwman E. P. , Koster G. , Blank D. H. A. , Rijnders G. 
Published: March 2012

AbstractPerovskite oxide heteroepitaxy receives much attention because of the possibility to combine the diverse functionalities of perovskite oxide building blocks. A general boundary condition for the epitaxy is the presence of polar discontinuities at heterointerfaces. These polar discontinuities result in reconstructions, often creating new functionalities at the interface. However, for a significant number of materials these reconstructions are unwanted as they alter the intrinsic materials properties at the interface. Therefore, a strategy to eliminate this reconstruction of the polar discontinuity at the interfaces is required. We show that the use of compositional interface engineering can prevent the reconstruction at the La0.67Sr0.33MnO3/SrTiO3 (LSMO/STO) interface. The polar discontinuity at this interface can be removed by the insertion of a single La0.33Sr0.67O layer, resulting in improved interface magnetization and electrical conductivity.
Multicomponent Organic Nanoparticles for Fluorescence Studies in Biological Systems
Authors: McDonald Tom O. , Martin Philip , Patterson Joseph P. , Smith Darren , Giardiello Marco , Marcello Marco , See Violaine , O'Reilly Rachel K. , Owen Andrew , Rannard Steve 
Published: March 2012

AbstractThe formation of dual‐component organic nanoparticles by a modified emulsion‐templated freeze‐drying approach leads to aqueous nanosuspensions showing fluorescence (Förster) resonance energy transfer (FRET) from within a distribution of single nanoparticles. The combination of both FRET dyes within dual‐component nanoparticles (<200 nm) allows the spatial and physical monitoring of the particles, as the FRET signal is lost on dissolution and breakdown of the nanoparticles. The monitoring of accumulation by Caco‐2 cells and macrophages shows very limited internalization within the non‐phagocytic cells. Conservation of FRET within the macrophages confirms extensive whole‐particle internalization. The cellular permeability through Caco‐2 monolayers is also assessed and movement of intact dual‐component particles is observed, suggesting a mechanism for enhanced pharmacokinetics in vivo.
State of the Art of Carbon Nanotube Fibers: Opportunities and Challenges
Authors: Lu Weibang , Zu Mei , Byun Joon‐Hyung , Kim Byung‐Sun , Chou Tsu‐Wei 
Published: March 2012

AbstractThe superb mechanical and physical properties of individual carbon nanotubes (CNTs) have provided the impetus for researchers in developing high‐performance continuous fibers based upon CNTs. The reported high specific strength, specific stiffness and electrical conductivity of CNT fibers demonstrate the potential of their wide application in many fields. In this review paper, we assess the state of the art advances in CNT‐based continuous fibers in terms of their fabrication methods, characterization and modeling of mechanical and physical properties, and applications. The opportunities and challenges in CNT fiber research are also discussed.
The face in congenital melanocytic nevus syndrome
Authors: Kinsler Veronica , Shaw Adam C. , Merks Johannes H. , Hennekam Raoul C. 
Published: March 2012

AbstractCongenital melanocytic nevi (CMN) are known to be associated with neurological abnormalities and melanoma, but have not been considered to be part of a developmental syndrome. The objective of this study was to test our clinical observation that children with CMN show more facial similarities than might be expected by coincidence. We selected facial photographs of 95 white Caucasian children with CMN from our database only on the basis of good neutral views, allowing careful evaluation of facial morphology. These were scored independently by two clinical geneticists using standardized categories and definitions for facial morphology. Prevalence of age‐independent features was compared to established norms in a large population, and associations with cutaneous phenotype were investigated. CMN were found to be associated with characteristic facies, and 74% of children in this series had at least three typical features. The characteristic features were: wide or prominent forehead, apparent hypertelorism, eyebrow variants, periorbital fullness, small/short nose, narrow nasal ridge, broad nasal tip, broad or round face, full cheeks, prominent pre‐maxilla, prominent/long philtrum, and everted lower lip. No association was found with the severity of cutaneous phenotype. We conclude that children with CMN often have a characteristic face. We propose the term “congenital melanocytic nevus syndrome” to describe this association. © 2012 Wiley Periodicals, Inc.
Cytogenetic and epidemiological findings in Down syndrome: England and Wales 1989–2009
Authors: Morris Joan K. , Alberman Eva , Mutton David , Jacobs Patricia 
Published: March 2012

AbstractThis study describes the characteristics of karyotypes leading to phenotypic Down syndrome (trisomy 21) in 29,256 cases diagnosed between 1989 and 2009 in England and Wales included in the National Down Syndrome Cytogenetic Register (NDSCR). The frequency of occurrence of the different karyotypes, proportions diagnosed prenatally, sex ratios, mean maternal age, and proportions of mothers with recurrences were analyzed. Nearly 97% of all cases were free trisomy 21; 2.9% contributory trisomy 21, 0.3% double or triple aneuploidies; 1% of all were mosaics. Mean maternal age of free trisomy 21 cases was 35 years, 54% were male, and 1% of mothers had recurrences. Free trisomy 21 mosaics had a lower mean maternal age (33 years), a lower proportion of males (39.5%), and 2.5% of mothers had recurrences. The majority of contributory translocations were Robertsonian or rea (21;21). Their mothers were younger, particularly those of Robertsonian translocations (28 years). Of the Robertsonian der (14;21) translocations of known parental origin, 54% were de novo, 41% maternal and 5% paternal and 15.8% of mothers of those of maternal origin had recurrences. Multiple aneuploidies have the highest proportion of males (67%), highest proportion of mosaics (40%), a mean maternal age of 37 years, and no mothers had a recurrence. The size of this national register allowed the frequency of occurrence of the rarer karyotypes of Down syndrome to be estimated and their epidemiology described. © 2012 Wiley Periodicals, Inc.
Smith–Lemli–Opitz syndrome: Objective assessment of facial phenotype
Authors: Nowaczyk Małgorzata J.M. , Tan Mattea , Hamid Jemila S. , Allanson Judith E. 
Published: March 2012

AbstractSmith–Lemli–Opitz syndrome (SLOS), is an autosomal recessive condition caused by cholesterol synthesis deficiency which results in a wide phenotypic spectrum which includes multiple malformations, distinctive facial appearance, and intellectual disability. This anthropometric and observational study was carried out to define the key facial characteristics of individuals with SLOS and to evaluate evolution of the facial phenotype with age. Clinical photographs were obtained on 51 subjects with SLOS and standardized facial anthropometry was performed on 42; the ages ranged from 6 months to 20 years. For each individual, 22 standardized cranial and facial measurements were obtained and compared to published age‐ and sex‐matched controls. Craniofacial pattern profiles were compared between sexes, various age groups, plasma cholesterol concentration at the time of diagnosis, and physical severity score. Mean‐Z, a measurement of overall facial size, and craniofacial variability index (CVI), a summary anthropometric measure of craniofacial deviation from the norm, were calculated and compared according to methods published previously. A characteristic craniofacial pattern profile was universally present: narrow forehead, brachycephaly, short palpebral fissures, short nasal ridge, anteverted nares, flat face, normal jaw width, and retrognathia. The facial measurements of subjects with SLOS had higher deviations from the norm with mean CVI of 2.11; SD = 0.47 (controls: Mean = 0.76, SD = 0.19; <0.0001) than age‐ and sex‐matched controls. Their faces were also smaller than controls: 70% of subjects had mean‐Z values 2 SD below the mean for controls; average Z‐score was −1.64; SD = 0.85 (controls: mean = 0, SD = 0.64; <0.0001). There were no significant differences in the craniofacial pattern profile between the sexes or clinical severity as measured by either plasma cholesterol level at the time of diagnosis or the physical severity score. Patients with a lower weight at the time of assessment and patients with higher physical severity score had higher CVI measures (P < 0.001 and P < 0.002, respectively), suggesting that the degree of deviation from craniofacial norms is a function of the overall physical severity. © 2012 Wiley Periodicals, Inc.
Café‐au‐lait macules and intertriginous freckling in piebaldism: Clinical overlap with neurofibromatosis type 1 and Legius syndrome
Authors: Stevens Cathy A. , Chiang Pei‐Wen , Messiaen Ludwine M. 
Published: March 2012

AbstractPiebaldism is an autosomal dominant disorder characterized by congenital hypopigmented patches of skin and hair and has been found to be associated with mutations in theKITorSLUGgenes. Café‐au‐lait macules (CALM) may occasionally be seen in piebaldism. There are four reports describing six patients who were said to have both piebaldism and neurofibromatosis type 1 (NF1) due to the presence of multiple CALM and intertriginous freckling, but none of these patients had undergone comprehensiveNF1mutation analysis. We describe a large family with piebaldism in which two members meet diagnostic criteria for NF1 based on the presence of >5 CALM and intertriginous freckling. Interestingly, only these two family members are of mixed race, which could be of importance. A novel complex mutation in theKITgene was identified in several family members affected with piebaldism; the proband meeting diagnostic criteria for NF1 also underwent comprehensiveNF1andSPRED1testing with no mutations detected. These findings suggest that piebaldism may occasionally include CALM and intertriginous freckling, which may create diagnostic confusion especially in the absence of a family history of piebaldism. However, careful clinical evaluation and molecular testing if necessary should distinguish these two disorders. © 2012 Wiley Periodicals, Inc.
A microdeletion proximal of the critical deletion region is associated with mild Wolf–Hirschhorn syndrome
Authors: Hannes Femke , Hammond Peter , Quarrell Oliver , Fryns Jean‐Pierre , Devriendt Koenraad , Vermeesch Joris R. 
Published: March 2012

AbstractIt is generally accepted that the facial phenotype of Wolf–Hirschhorn syndrome is caused by deletions of either Wolf–Hirschhorn critical regions 1 or 2 (WHSCR 1–2). Here, we identify a 432 kb deletion located 600 kb proximal to both WHSCR1–2 in a patient with a WHS facial phenotype. Seven genes are underlying this deletion region including FAM193a, ADD1, NOP14, GRK4, MFSD10, SH3BP2, TNIP2. The clinical diagnosis of WHS facial phenotype was confirmed by 3D facial analysis using dense surface modeling. Our results suggest that the WHSCR1–2 flanking sequence contributes directly or indirectly to the severity of WHS. Sequencing the Wolf–Hirschhorn syndrome candidate 1 and 2 genes did not reveal any mutations. Long range position effects of the deletion that could influence gene expression within the WHSCR were excluded in EBV cell lines derived from patient lymphoblasts. We hypothesize that either (1) this locus harbors regulatory sequences which affect gene expression in the WHSCR1–2 in a defined temporal and spatial developmental window or (2) that this locus is additive to deletions of WHSCR1–2 increasing the phenotypic expression. © 2012 Wiley Periodicals, Inc.