bluuebunny/crossref_metadata_2025_split huggingface dataset:
Genotype does not predict severity of behavioural phenotype in juvenile neuronal ceroid lipofuscinosis (Batten disease)
Authors: ADAMS HEATHER R , BECK CHRISTOPHER A , LEVY ERIKA , JORDAN RACHEL , KWON JENNIFER M , MARSHALL FREDERICK J , VIERHILE AMY , AUGUSTINE ERIKA F , DE BLIECK ELISABETH A , PEARCE DAVID A , MINK JONATHAN W 
Published: February 2010

Aim  The primary aim of this investigation was to examine genotype and clinical phenotype differences in individuals with juvenile neuronal ceroid lipofuscinosis (JNCL) who were homozygous for a common disease‐causing deletion or compound heterozygous. The secondary aim was to cross‐validate the Child Behavior Checklist (CBCL) and the Unified Batten Disease Rating Scale (UBDRS), a disease‐specific JNCL rating scale.Method  Sixty individuals (28 males, 32 females; mean age 15y 1mo, SD 4y 9mo, range 5y 8mo–31y 1mo) with JNCL completed the UBDRS.Results  No significant genotype and clinical phenotype differences were identified when comparing individuals homozygous for the deletion with a heterogeneous group of compound heterozygous individuals. There were significant correlations among related behaviour items and scales on the CBCL and UBDRS (Spearman’s rho ranging from 0.39 [p<0.05] to 0.72 [p<0.01]). Behaviour and physical function ratings were uncorrelated, supporting divergent validity of these two constructs in JNCL.Interpretation  Previous reports of genotype and clinical phenotype differences were unsupported in this investigation, which did not find differences between individuals homozygous or heterozygous for the CLN3 deletion. The CBCL, an already validated measure of behaviour problems, appears valid for use in JNCL and cross‐validates well with the UBDRS.
The Ecosystem: Model or Metaphor?
Authors: Hess Gérald 
Published: February 2010

SummaryIndustrial ecology offers an original way of looking at economic activities. The approach is based on an analogy between certain objects studied by the science of ecology (ecosystems, metabolisms, symbiosis, biocenosis, etc.) and industrial systems. However, this analogical relationship raises difficulties due to the various interpretations to which it is open. Although there is agreement regarding its heuristic function, the analogy can nevertheless be understood either as a model or as a metaphor. The present article first attempts to show how models differ from metaphors. It then sets out to justify the epistemological relevance of this distinction for industrial ecology research. The reflection should thus contribute to clarifying the debate on the (supposed or desired) role of analogy in the field of industrial ecology and heighten the interest this field of investigation represents for implementing sustainable development.
Model System for Measuring the Effects of Position and Curvature of Fiber Reinforcement Within a Dental Composite
Authors: Rashidan Nayereh , Esmaeili Vahid , Alikhasi Marzieh , Yasini Sara 
Published: February 2010

Abstract Purpose: The aim of this study was to compare the effect of fiber curvature and position on flexural strength (FS), toughness, and elastic modulus in a dental flowable composite test specimen. Methods and Materials: Test specimens made of composite resin (Denfil Flow) were reinforced with preimpregnated glass fibers (Interlig). Control specimens (group A) did not contain fiber reinforcement. Fibers were placed with different positions and orientations into the test specimens (2 mm × 2 mm × 25 mm) (groups B, C, D). The test specimens (n = 10) were stored in distilled water for 3 days at 37°C before testing in a three‐point loading test (ISO 10477) at a crosshead speed of 1 mm/min to determine FS, flexural modulus (FM), and toughness. Data were analyzed with 1‐way analysis of variance and Tukey HSD (σ= 0.05). Results: The FM varied from 4.7 ± 0.5 to 6.7 ± 0.5 GPa. The lowest flexural strength and toughness values in reinforced specimens resulted from compression side fiber reinforcement (132 ± 12 MPa, 21 ± 4 MJ) and the highest from curved fiber reinforcement (174 ± 8 MPa, 83 ± 28 MJ), though this was not statistically significant from tension‐side reinforcement. Although the toughness of the curved reinforced group was significantly higher than other groups, the flexural strength of curved reinforcement was not significantly higher than tension‐side reinforcement. Conclusion: Position and fiber orientation influenced the flexural strength, FM, and toughness. The most effective in increasing toughness was curved placement of fibers.
Resident Selection Criteria for Advanced Education in Prosthodontic Programs: Program Directors’ Perspective
Authors: Yuan Judy Chia‐Chun , Lee Damian J. , Knoernschild Kent L. , Campbell Stephen D. , Sukotjo Cortino 
Published: February 2010

Abstract Purpose: The purpose of this study was to describe the criteria used by advanced education in prosthodontic program (AEPP) directors to select their residents, to rank them by perceived importance, and further assist prospective candidates with the application process for AEPP. Materials and Methods: Questionnaires were distributed to all prosthodontic program directors (N = 46). The program directors were requested to respond in five sections: (1) general information, (2) information obtained from applications and letters of recommendation, (3) interview process, (4) decision process, and (5) retrospective view of the selection process. Descriptive statistics were used to analyze the data. Data were collected and compiled into mean, standard deviation, and range. Results were tabulated and ranked. Results: Thirty‐eight responses (82.61%) were returned and analyzed. Most of the programs (75.77%) indicated that a combination of the program director, current residents, prosthodontic faculty, and staff members were involved in conducting the interview process. Factors considered very important when choosing applicants to the prosthodontic program were (1) interview process, (2) dental school class rank, (3) dental school grades (prosthodontics), (4) letters of recommendation, (5) dental school grades (clinical). Letters from the prosthodontic post‐doc program director and prosthodontic faculty were considered the most important source of recommendation. Honesty, organization, and energy were ranked as the most positive characteristics of the applicants during the interview. Almost all respondents (97%) were satisfied with the current selection process. When asked about the current applicant pool, most program directors (91.67%) were satisfied. Conclusions: The most and least important factors in selecting applicants by the program directors were described and ranked. This study was intended to provide the profession with some insight on how advanced Prosthodontic programs select their applicants. It may also serve as a valuable instrument for prospective applicants to AEPPs in the future.
Experience with a naphthylmedetomidine – ketamine – hyaluronidase combination in inducing immobilization in anthropoid apes
Authors: Hess L. , Votava M. , Schreiberová J. , Málek J. , Horáček M. 
Published: February 2010

AbstractBackground  The aim of the study was to compare the effect of naphthylmedetomidine to medetomidine on the behavior of orangutans and chimpanzees.Methods  The immobilization was performed as part of a medical examination in five chimpanzees and three orangutans. Following pre‐medication with midazolam (0.70–1.20 mg/kg p.o.), naphthylmedetomidine (50–70 μg/kg), or medetomidine (20–30 μg/kg) was given with ketamine (3 mg/kg) and hyaluronidase (150 M.U.) into musculus deltoideus.Results  We observed the distinct anti‐aggressive effect of naphthylmedetomidine. The immobilization with naphthylmedetomidine was shallower and the influence on cardiac frequency less substantial compared to medetomidine. The overall sedative effect of naphthylmedetomidine lasted for less time, and its effect was incompletely antagonized with atipamezole in comparison to medetomidine.Conclusions  Naphthylmedetomidine could replace medetomidine for inducing immobilization and sedation. A combination of naphthylmedetomidine–ketamine is suitable for relocating animals to other cages or for painless medical examinations.
Epizootic fatal amebiasis in an outdoor group of Old World monkeys
Authors: Ulrich Reiner , Böer Michael , Herder Vanessa , Spitzbarth Ingo , Hewicker‐Trautwein Marion , Baumgärtner Wolfgang , Wohlsein Peter 
Published: February 2010

AbstractBackground Entamoeba(E.)histolyticais an obligate parasite of humans and non‐human primates.Methods This report describes the pathomorphological, immunohistological, and microbiological findings of fatalE. histolyticainfection in two mantled guerezas (Colobus guereza) and one Hanuman langur (Semnopithecus entellus) from an epizootic outbreak of amebiasis in an open‐range recreation park.Results Pathomorphological examination revealed multifocal necrotizing and granulomatous hepatitis with intralesional protozoan trophozoites in all three cases. In addition, necrotizing and ulcerative gastritis was detected in both mantled guerezas. Furthermore, oligofocal acute pulmonary embolization was detected in one of these cases. No extra‐hepatic lesions were observed in the Hanuman langur. Immunohistological examination confirmed the etiologic diagnosis ofE. histolytica‐induced lesions.Conclusions AlthoughE. histolyticais a rarely diagnosed pathogen in Western European countries, veterinarians and animal keepers involved in handling and care taking of non‐human primates should be aware of the potential threat caused by this zoonotic parasite.
When to image neurologically normal children with headaches: development of a decision rule
Authors: Ahmed MAS , Martinez A , Cahill D , Chong K , Whitehouse WP 
Published: February 2010

AbstractAims:  The aim of this study was to develop and refine a decision rule on when to undertake brain imaging (BI) in neurologically normal children with headaches.Methods:  From the literature and a questionnaire study, a list of red flags (RFs) was drawn‐up. During the prospective 4‐year period, consecutive children with headache were classified according to RFs and the headache diagnosis.Result:  Three of 709 (0.4%) neurologically normal children had significant brain abnormalities. BI was carried out in 389 of 498 (78%) children with RFs. Significant abnormalities were found in three of 389 children (0.8%), all had unclassified headache (UH). BI was not arranged for the 211 children with no RFs. None of these developed RFs or abnormal signs on follow‐up for a mean of 13 months.Conclusion:  In addition to BI for those with neurological signs, we think BI should be considered for neurologically normal patients with UH and RFs. This would have saved imaging children needlessly: only 101 of 709 (14%) would have had scans arranged, instead of 389 of 709.
Utility of multidetector CT and virtual bronchoscopy in tracheobronchial obstruction in children
Authors: Sodhi Kushaljit Singh , Aiyappan Senthil Kumar , Saxena Akshay Kumar , Singh Meenu , Rao KLN , Khandelwal Niranjan 
Published: February 2010

AbstractPurpose:  The aim of this study was to evaluate the potential use of multidetector CT (MDCT) and virtual bronchoscopy (VB) in the evaluation of tracheobronchial patency in children with suspected bronchial obstruction and to compare its findings with fibreoptic/rigid bronchoscopy or surgery.Patients and methods:  A total of 43 children (15 girls, 28 boys) with clinically suspected bronchial obstruction underwent contrast enhanced MDCT, using an age‐ and weight‐ adjusted low dose protocol. Post‐processing was performed and VB and multiplanar reformations (MPR) were obtained at the same sitting. Findings obtained at MDCT and VB were compared with fibreoptic/rigid bronchoscopy and surgery.Results:  Obstructive pathology was found in 26 children, which included endoluminal foreign body, mucus plugs in 13 children, endobronchial tumour in three children and extrinsic compression (lymph node, aberrant Vessels, mediastinal cysts/tumours) of the tracheobronchial tree in 10 children. In 17 children, no obstructive lesion was identified. Excellent positive correlation was obtained, between MDCT‐VB and bronchoscopy/surgery, however, in one child with endobronchial obstruction caused by tracheitis, low dose MDCT‐VB was normal, but bronchoscopy revealed granularity and plaques.Conclusion:  MDCT‐Virtual bronchoscopy is useful in evaluating bronchial stenosis and obstruction caused by both endoluminal pathology and external compression and has the advantage of looking beyond stenosis. Its main application lies in providing the exact location of suspected foreign body, prior to bronchoscopy. However, it fails to disclose exact nature of obstructing pathology.
Performing Impartiality: Judicial Demeanor and Legitimacy
Authors: Mack Kathy , Roach Anleu Sharyn 
Published: February 2010

The demeanor exhibited by a judge is a key element in criminal proceedings, especially in direct interactions with the defendant, defense representatives, and prosecutors. The judicial demeanor(s) displayed must meet the normative confines of the judicial role—primarily impartiality—within the practical constraints of long case lists, when interactions are subject to considerable time pressure.
Drawing on findings from an extensive national court observation study, this article examines the varied demeanors displayed by magistrates—the presiding judicial officers—in Australia's lower courts. It explores the complex meanings of these demeanors, and considers how they may enhance or undermine the legitimacy of judicial authority.
CD40L induces inflammation and adipogenesis in adipose cells – a potential link between metabolic and cardiovascular disease
Authors: Platzer Isabel , Ernst Sandra , Walter Carina , Rudolf Philipp , Zirlik Katja , Köstlin Natascha , Willecke Florian , Münkel Christian , Schönbeck Uwe , Libby Peter , Bode Christoph , Missiou Anna , Wolf Dennis , Varo Nerea , Zirlik Andreas 
Published: February 2010

SummaryCD40L figures prominently in atherogenesis. Recent data demonstrate elevated levels of sCD40L in the serum of patients with the metabolic syndrome (MS). This study investigated the role of CD40L in pro-inflammatory gene expression and cellular differentiation in adipose tissue to obtain insight into mechanisms linking the MS with atherosclerosis. Human adipocytes and preadipocytes expressed CD40 but not CD40L. Stimulation with recombinant CD40L or membranes over-expressing CD40L induced a time- and dose-dependent expression of IL-6, MCP-1, IL-8, and PAI-1. Supernatants of CD40L-stimulated adipose cells activated endothelial cells, suggesting a systemic functional relevance of our findings. Neutralising antibodies against CD40L attenuated these effects substantially. Signalling studies revealed the involvement of mitogen-activated protein kinases and NFκB. Furthermore, stimulation with CD40L resulted in enhanced activation of C/EBPα and PPARγ and promoted adipogenesis of preadipose cells in the presence and absence of standard adipogenic conditions. Finally, patients suffering from the metabolic syndrome with high levels of sCD40L also displayed high levels of IL-6, in line with the concept that CD40L may induce the expression of inflammatory cytokines in vivo in this population. Our data reveal potent metabolic functions of CD40L aside from its known pivotal pro-inflammatory role within plaques. Our data suggest that CD40L may mediate risk at the interface of metabolic and atherothrombotic disease.
Stem cell and progenitor cell therapy in peripheral artery disease
Authors: Bramlage Peter , Amann Berthold , Lawall Holger 
Published: February 2010

SummaryAtherosclerotic peripheral artery disease (PAD) is a common manifestation of atherosclerosis. The occlusion of large limb arteries leads to ischaemia with claudication which can progress to critical limb ischaemia (CLI) with pain at rest, and to tissue loss. At present, common therapy for CLI is either surgical or endovascular revascularisation aimed at improving blood flow to the affected extremity. However, major amputation and death are still frequent complications. Exploring new strategies for revascularisation of ischaemic limbs is thus of major importance. Bone marrow (BM)-derived stem and progenitor cells have been identified as a potential new therapeutic option to induce therapeutic angiogenesis. Encouraging results of preclinical studies have rapidly led to several small clinical trials, in which BM-derived mononuclear cells were administered to patients with limb ischaemia. Clinical benefits were reported from these trials including improvement of ankle-brachial index (ABI), transcutaneous partial pressure of oxygen (TcPO2), re-duction of pain, and decreased need for amputation. Nonetheless, large randomised, placebo-controlled, double-blind studies are necessary and currently ongoing (BONMOT-CLI, JUVENTUS and NCT00498069). Further research relates to the optimal cell type and dosage, the isolation method, the role of colony-stimulating factors, administration route, and the supportive stimulation of cells with reduced functioning due to advanced PAD. Autologous stem cell therapy for ischaemic peripheral disease seems to be a promising new tool for the treatment of severe limb ischaemia. Preliminary evidence has established its safety, feasibility and effectiveness on several important endpoints. Several large endpoints studies are underway to further consolidate this evidence.
ALL-associated JAK1 mutations confer hypersensitivity to the antiproliferative effect of type I interferon
Authors: Hornakova Tekla , Chiaretti Sabina , Lemaire Muriel M. , Foà Robin , Ben Abdelali Raouf , Asnafi Vahid , Tartaglia Marco , Renauld Jean-Christophe , Knoops Laurent 
Published: February 2010

AbstractActivating mutations in JAK1 have been reported in acute lymphoblastic leukemias (ALLs). In this study, we found a type I interferon (IFN) transcriptional signature in JAK1 mutation-positive human ALL samples. This signature was recapitulated in vitro by the expression of JAK1 mutants in BW5147 and BaF3 hematopoietic cell lines. Binding of JAK1 to the IFN receptor was essential because mutations in the FERM domain abrogated this effect. Beside the constitutive activation of the type I IFN signaling cascade, JAK1 mutations also strongly potentiated the response to IFN in vitro. Typically, the proliferation of cell lines expressing JAK1A634D was abrogated by type I IFNs. Interestingly, we found that different JAK1 mutations differentially potentiate responses to type I IFNs or to interleukin-9, another cytokine using JAK1 to mediate its effects. This suggests that the type of mutation influences the specificity of the effect on distinct cytokine receptor signaling. Finally, we also showed in an in vivo leukemia model that cells expressing JAK1A634D are hypersensitive to the antiproliferative and antitumorigenic effect of type I IFN, suggesting that type I IFNs should be considered as a potential therapy for ALL with JAK1-activating mutations.
Structural classification by the Lipase Engineering Database: a case study of Candida antarctica lipase A
Authors: Widmann Michael , Juhl P Benjamin , Pleiss Jürgen 
Published: February 2010

Abstract

Background
The Lipase Engineering Database (LED) integrates information on sequence, structure and function of lipases, esterases and related proteins with the α/β hydrolase fold. A new superfamily for Candida antarctica lipase A (CALA) was introduced including the recently published crystal structure of CALA. Since CALA has a highly divergent sequence in comparison to other α/β hydrolases, the Lipase Engineering Database was used to classify CALA in the frame of the already established classification system. This involved the comparison of CALA to similar structures as well as sequence-based comparisons against the content of the LED.

Results
The new release 3.0 (December 2009) of the Lipase Engineering Database contains 24783 sequence entries for 18585 proteins as well as 656 experimentally determined protein structures, including the structure of CALA. In comparison to the previous release [1] with 4322 protein and 167 structure entries this update represents a significant increase in data volume. By comparing CALA to representative structures from all superfamilies, a structure from the deacetylase superfamily was found to be most similar to the structure of CALA. While the α/β hydrolase fold is conserved in both proteins, the major difference is found in the cap region. Sequence alignments between both proteins show a sequence similarity of only 15%. A multisequence alignment of both protein families was used to create hidden Markov models for the cap region of CALA and showed that the cap region of CALA is unique among all other proteins of the α/β hydrolase fold. By specifically comparing the substrate binding pocket of CALA to other binding pockets of α/β hydrolases, the binding pocket of Candida rugosa lipase was identified as being highly similar. This similarity also applied to the lid of Candida rugosa lipase in comparison to the potential lid of CALA.

Conclusion
The LED serves as a valuable tool for the systematic analysis of single proteins or protein families. The updated release 3.0 was used for the evaluation of α/β hydrolases. The HTML version of the database with new features is available at http://www.led.uni-stuttgart.de and provides sequences, structures and a set of analysis tools including phylogenetic trees and HMM profiles
Waist circumference cut-off values for the prediction of cardiovascular risk factors clustering in Chinese school-aged children: a cross-sectional study
Authors: Liu Ailing , Hills Andrew P , Hu Xiaoqi , Li Yanping , Du Lin , Xu Ying , Byrne Nuala M , Ma Guansheng 
Published: February 2010

AbstractBackgroundWaist circumference has been identified as a valuable predictor of cardiovascular risk in children. The development of waist circumference percentiles and cut-offs for various ethnic groups are necessary because of differences in body composition. The purpose of this study was to develop waist circumference percentiles for Chinese children and to explore optimal waist circumference cut-off values for predicting cardiovascular risk factors clustering in this population.MethodsHeight, weight, and waist circumference were measured in 5529 children (2830 boys and 2699 girls) aged 6-12 years randomly selected from southern and northern China. Blood pressure, fasting triglycerides, low-density lipoprotein cholesterol, high-density lipoprotein cholesterol, and glucose were obtained in a subsample (n = 1845). Smoothed percentile curves were produced using the LMS method. Receiver-operating characteristic analysis was used to derive the optimal age- and gender-specific waist circumference thresholds for predicting the clustering of cardiovascular risk factors.ResultsGender-specific waist circumference percentiles were constructed. The waist circumference thresholds were at the 90th and 84th percentiles for Chinese boys and girls respectively, with sensitivity and specificity ranging from 67% to 83%. The odds ratio of a clustering of cardiovascular risk factors among boys and girls with a higher value than cut-off points was 10.349 (95% confidence interval 4.466 to 23.979) and 8.084 (95% confidence interval 3.147 to 20.767) compared with their counterparts.ConclusionsPercentile curves for waist circumference of Chinese children are provided. The cut-off point for waist circumference to predict cardiovascular risk factors clustering is at the 90th and 84th percentiles for Chinese boys and girls, respectively.