bluuebunny/crossref_metadata_2025_split huggingface dataset:
THE KNOWN UK CASES OF ATAXIA TELANGIECTASIA-LIKE DISORDER (ATLD)
Authors: Westwood Katy , Ealing John , Taylor Malcolm , Worth Paul , Nemeth Andrea 
Published: October 2015

Ataxia telangiectasia-like disorder (ATLD) is a very rare autosomal recessive disease with only 25 patients recognised worldwide. ATLD is likened to Ataxia telangiectasia (A-T) due to an overlap of clinical presentations and cellular characteristics. The clinical hallmark of A-T and ATLD is progressive young onset cerebellar ataxia. Variably present characteristics include dysarthria, oculomotor apraxia, ocular telangiectasia, immunodeficiency, spontaneous chromosome abnormalities and a predisposition to malignancy. In contrast to A-T, ocular telangiectasia is absent. Furthermore, ATLD patients tend to have a later onset and slower progression of neurological signs than A-T. The ATM gene, that has a key role in genome stability, is mutated in A-T resulting in an increase cancer predisposition. In ATLD, gene MRE11 is mutated leading to deficient activation of ATM. A functional consequence of the MRE11 mutation is raised chromosomal radiosensitivity because functional ATM kinase is required to rejoin chromosome breaks. This poster/presentation will describe the clinical features and genetic analysis of the ATLD cases with progressive ataxia known in the UK.
Undergraduate Prize Winner 2015
DIGGING DEEPER
Authors: Witherick Jonathan , Bovill Begoña , Dong Anni , Albur Mahableshwar , Bosnell Rose 
Published: October 2015

BackgroundMultiple ring-enhancing brain lesions have a wide differential. We report a case with a rare cause that was successfully treated.Case reportAn 18 year old presented with a short history of headache, dry cough and fevers. His past medical history included Aspergers, childhood epilepsy and bilateral hearing loss (with identified chromosomal translocation) and a recent diagnosis of trans-bronchial biopsy-positive sarcoidosis for which he was taking prednisolone. Radiology showed left upper zone consolidation; despite initial improvement with piperacillin-tazobactam, his headaches intensified and contrast-enhanced brain imaging demonstrated multiple ring-enhancing lesions; lesions were also identified in the liver and spleen. CSF analysis confirmed active inflammation but cultures (including liver biopsy) were negative. Subsequently liver tissue 16S rDNA rtPCR analysis identified rDNA of the pseudomonas genus with a 99% sequence homology for P.luteola and P.zeshuii. These soil-dwelling organisms may have been acquired by the patient whilst working on his grandfather's allotment. The patient was successfully treated with Meropenem.ConclusionThis case involved input from multiple clinical specialties and highlights the collaboration needed to diagnose and manage such a rare organism. The case highlights unusual pathogens that may cause opportunistic infection in the immunocompromised host and the importance of a thorough occupational history.
IS THE APPLAUSE SIGN USEFUL FOR DIAGNOSIS OF DEMENTIA AND MCI?
Authors: Bonello Michael , Larner Andrew 
Published: October 2015

ObjectiveTo examine the diagnostic utility of the applause sign for dementia and MCI in consecutive referrals to cognitive disorders clinic.ResultsOver a 10–month period (January–November 2014) 243 consecutive new outpatients (F:M=124:119; age range 18–91 years, median 61) were administered the applause test (“clap 3 times”). Of the 240 with interpretable results, 46 were diagnosed with dementia (DSM–IV criteria), 66 with MCI (Petersen criteria), and 128 with no cognitive impairment. Using the categorization of applause sign from Luzzi et al. (2011), the percentage of patients with any cognitive impairment who clapped 3–10, and >10 times was 38.9, 60, 70.8, and 87.5 respectively. The null hypothesis that the proportion of patients with cognitive impairment did not differ significantly between groups was rejected (χ2=21.4, df=3, p<0.001). Abnormal scores (>3 claps) had sensitivities for dementia, MCI, and any cognitive impairment of 0.52, 0.24, and 0.36 respectively, and specificities of 0.84, 0.88, and 0.88.ConclusionsThis pragmatic study confirms and extends the results of a previous smaller study (n=100) of applause sign in the cognitive clinic, showing the applause sign to be a high specificity low sensitivity test for cognitive impairment.
Sex Disparities in Post-Acute Myocardial Infarction Pharmacologic Treatment Initiation and Adherence
Authors: Smolina Kate , Ball Laura , Humphries Karin H. , Khan Nadia , Morgan Steven G. 
Published: October 2015

Background—
The prevalence of the use of secondary prevention cardiovascular medications is lower among women than men, but it is unclear if this is a result of lower treatment initiation among women or lower treatment adherence. We aimed to map the treatment pathway for survivors of acute myocardial infarction (AMI) by sex and age.

Methods and Results—
This retrospective population-based cohort study used linked administrative data sets in British Columbia (2004–2011), which include health care, prescription drugs, sociodemographic, and mortality information. The study cohort included all individuals admitted to hospital for AMI in 2007–2009 and survived for 1 year after hospital discharge. Patients were evaluated for whether they initiated and then subsequently filled prescriptions angiotensin-converting enzyme inhibitors, β-blockers, and statins. More than two thirds of AMI survivors initiated treatment on all appropriate medications, given their contraindications, within 2 months of discharge. Younger men were significantly more likely than younger women to initiate appropriate treatment (adjusted odds ratio, 1.38; 95% confidence interval, 1.10–1.75). By the end of 1 year after discharge, only one third of all AMI survivors filled all appropriate prescriptions for at least 80% of the year. There was no significant difference in adherence to medication therapy between women and men.

Conclusions—
The majority of AMI survivors either discontinue treatment or do not refill their prescriptions consistently. Women <55 years are significantly less likely to be on optimal therapy by the end of 1 year after discharge, which is driven by a sex disparity in treatment initiation and not treatment adherence.
Description of Bursaphelenchus piceae sp. n. (Nematoda: Parasitaphelenchinae) from larval galleries of the six-toothed spruce bark beetle, Pityogenes chalcographus (L.) (Coleoptera: Curculionidae: Scolytinae), in Norway spruce, Picea abies (L.) Karsten
Authors: Tomalak Marek , Pomorski Jan J. 
Published: October 2015

Bursaphelenchus piceae sp. n. is described from the bark of Norway spruce, Picea abies. Adults and propagative juveniles of the nematode are found in larval galleries of the six-toothed spruce bark beetle, Pityogenes chalcographus, while its dauer juveniles aggregate in Malpighian tubules of older larvae, pupae and adult beetles. The new species is characterised by the body length of 719 (530-945) μm in female and 632 (474-808) μm in male, its moderately slender body (a = 41.4 (35.4-50.2) and 42.6 (35.1-47.2) in female and male, respectively), and small spicules (16.0 (14.0-18.3) μm along arc and 13.7 (12.2-15.5) μm along chord). The extended anterior vulval lip in female, lateral fields with four incisures, long post-uterine sac extending for 67-91% of vulva-anus distance, and number (7) and arrangement of male caudal papillae suggest that B. piceae sp. n. is closely related to the xylophilus-group yet it differs by the relatively small, claw-like spicules with no cucullus at the tip. The close relation of B. piceae sp. n. to the xylophilus-group has been confirmed by DNA sequencing and phylogenetic analysis. Morphologically B. piceae sp. n. most closely resembles B. tokyoensis and B. fagi but can be separated from both by the unique shape of the spicules. The taxonomic separation of the new species is also confirmed by the unique molecular profile of the ITS region (ITS-RFLP). The presence of dauer juveniles of B. piceae sp. n. in Malpighian tubules of adult P. chalcographus may lead to extensive damage to this organ as shown by local expansion of the tubule basement membrane and degradation of its cellular epithelium. Detailed phylogenetic analysis revealed that B. piceae sp. n. together with five other bark beetle-associated Bursaphelenchus species, namely B. trypophloei, B. masseyi, B. tiliae, B. tokyoensis and B. fagi, constitutes a small phylogenetic clade that is most closely located to, but separate from, the xylophilus-group.
CalWater Field Studies Designed to Quantify the Roles of Atmospheric Rivers and Aerosols in Modulating U.S. West Coast Precipitation in a Changing Climate
Authors: Ralph F. M. , Prather K. A. , Cayan D. , Spackman J. R. , DeMott P. , Dettinger M. , Fairall C. , Leung R. , Rosenfeld D. , Rutledge S. , Waliser D. , White A. B. , Cordeira J. , Martin A. , Helly J. , Intrieri J. 
Published: October 2015

Abstract
The variability of precipitation and water supply along the U.S. West Coast creates major challenges to the region’s economy and environment, as evidenced by the recent California drought. This variability is strongly influenced by atmospheric rivers (ARs), which deliver much of the precipitation along the U.S. West Coast and can cause flooding, and by aerosols (from local sources and transported from remote continents and oceans) that modulate clouds and precipitation. A better understanding of these processes is needed to reduce uncertainties in weather predictions and climate projections of droughts and floods, both now and under changing climate conditions.
To address these gaps, a group of meteorologists, hydrologists, climate scientists, atmospheric chemists, and oceanographers have created an interdisciplinary research effort, with support from multiple agencies. From 2009 to 2011 a series of field campaigns [California Water Service (CalWater) 1] collected atmospheric chemistry, cloud microphysics, and meteorological measurements in California and associated modeling and diagnostic studies were carried out. Based on the remaining gaps, a vision was developed to extend these studies offshore over the eastern North Pacific and to enhance land-based measurements from 2014 to 2018 (CalWater-2). The dataset and selected results from CalWater-1 are summarized here. The goals of CalWater-2, and measurements to date, are then described.
CalWater is producing new findings and exploring new technologies to evaluate and improve global climate models and their regional performance and to develop tools supporting water and hydropower management. These advances also have potential to enhance hazard mitigation by improving near-term weather prediction and subseasonal and seasonal outlooks.
The Threat to Weather Radars by Wireless Technology
Authors: Saltikoff Elena , Cho John Y. N. , Tristant Philippe , Huuskonen Asko , Allmon Lynn , Cook Russell , Becker Erik , Joe Paul 
Published: October 2015

Abstract
Wireless technology, such as local area telecommunication networks and surveillance cameras, causes severe interference for weather radars because they use the same operational radio frequencies. One or two disturbances can be removed from the radar image, but the number and power of the interfering wireless devices are growing all over the world, threatening that one day the radars could become useless for weather observations. Some agencies have already changed or are considering changing frequency bands, but now even other bands are under threat.
Use of equipment at radio frequencies is regulated by laws and international agreements. Technologies have been developed for peaceful coexistence. If wireless devices use these technologies to protect weather radars, their data transmission capabilities become limited, so it is tempting to violate the regulations. Hence, it is an important task for the worldwide weather community to involve themselves in the radio frequency management process and work in close contact with their national radio authorities to ensure that meteorological interests be duly taken into account in any decision-making process toward the future usage of wireless devices.
The Effect of the Balcones Escarpment on Three Cases of Extreme Precipitation in Central Texas
Authors: Nielsen Erik R. , Schumacher Russ S. , Keclik Alexandra M. 
Published: October 2015

Abstract
The proximity to the Gulf of Mexico and local topography makes central Texas particularly prone to heavy precipitation and deadly flood events. Specifically, the Balcones Escarpment, located in central Texas, creates extremely favorable hydrologic characteristics for damaging floods. Urban centers such as San Antonio and Austin, Texas, are located along this terrain feature and have suffered at times, even with mitigation strategies, catastrophic flood damage. While the hydrologic effects of the Balcones Escarpment are well known, the meteorological impacts are uncertain. The purpose of this study is to evaluate the effect of the Balcones Escarpment in three cases of extreme precipitation in which the rainfall was maximized near the escarpment. Numerical simulations for each event were run at convection-allowing grid spacing using the Weather Research and Forecasting (WRF) Model and were used as control runs. Then, the Balcones Escarpment was removed by moving the associated terrain gradient to the north and west. The removal of the Balcones Escarpment did not change the overall characteristics of any of the three rainfall events, with the spatial pattern and magnitude of precipitation similar between the control and terrain-modified simulations. However, the location of the maximum precipitation was slightly, but consistently, shifted to the north and west. These results show that the overall atmospheric conditions are much more important for determining the intensity and occurrence of extreme rainfall in central Texas than the local topography, but the Balcones Escarpment can cause subtle hydrologically important changes in the location of the maximum accumulation.
Growth and Obesity Among Older Single Ventricle Patients Presenting for Fontan Conversion
Authors: Freud Lindsay R. , Webster Gregory , Costello John M. , Tsao Sabrina , Rychlik Karen , Backer Carl L. , Deal Barbara J. 
Published: October 2015

Introduction: Long-term growth outcomes and the prevalence of obesity among older single ventricle (SV) patients have not been well characterized. We investigated these parameters, as well as the impact of obesity on survival, in an older cohort of SV patients presenting for Fontan conversion. Methods: We analyzed preoperative height, weight, and body mass index (BMI) of patients who underwent Fontan conversion. Overweight and obese were defined as BMI ≥85 percentile and ≥95 percentile for patients <20 years and BMI 25 to 30 kg/m2 and ≥30 kg/m2 for patients ≥20 years, respectively. Postoperative transplant-free survival was assessed among obese, overweight, and normal weight patients. Results: We evaluated 139 patients presenting for Fontan conversion at a median age of 23.2 years. Patients had shorter stature compared to the normal population (mean Z score −0.6, P < .001). Younger patients had lower BMI compared to the normal population (<20 years: mean Z score −0.5, P = .02), while older patients had elevated BMI (≥20 years: mean Z score +0.4, P < .001). The mean BMI among older patients approached overweight at 24.6 kg/m2. The prevalence of obesity increased with advancing age, with 36% overweight and 14% obese at >30 years. At a median of 8.2 years following Fontan conversion, obesity and overweight status were not associated with transplant-free survival. Conclusion: Older SV patients presenting for Fontan conversion had shorter stature compared to the normal population as well as a high prevalence of overweight and obesity. Although there was no relationship between weight status and early postoperative survival, further investigation of long-term outcomes is warranted.
A common language to assess allergic rhinitis control: results from a survey conducted during EAACI 2013 Congress
Authors: Hellings Peter W. , Muraro Antonella , Fokkens Wytske , Mullol Joaquim , Bachert Claus , Canonica G. Walter , Price David , Papadopoulos Nikos , Scadding Glenis , Rasp Gerd , Demoly Pascal , Murray Ruth , Bousquet Jean 
Published: October 2015

AbstractBackgroundThe concept of control is gaining importance in the field of allergic rhinitis (AR), with a visual analogue scale (VAS) score being a validated, easy and attractive tool to evaluate AR symptom control. The doctors’ perception of a VAS score as a good tool for evaluating AR symptom control is unknown, as is the level of AR control perceived by physicians who treat patients.Methods307 voluntarily selected physicians attending the annual (2013) European Academy of Allergy and Clinical Immunology (EAACI) meeting completed a digital survey. Delegates were asked to (1) estimate how many AR patients/week they saw during the season, (2) estimate the proportion of patients they considered to have well‐, partly‐ and un‐controlled AR, (3) communicate how they gauged this control and (4) assess how useful they would find a VAS as a method of gauging control. 257 questionnaires were filled out completely and analysed.ResultsEAACI delegates reported seeing 46.8 [standard deviation (SD) 68.5] AR patients/week during the season. They estimated that 38.7 % (SD 24.0), 34.2 % (SD 20.2) and 20.0 % (SD 16.34) of their AR patients had well‐controlled (no AR symptoms), partly‐controlled (some AR symptoms), or un‐controlled‐(moderate/severe AR symptoms) disease despite taking medication [remainder unknown (7.1 %)]. However, AR control was assessed in many ways, including symptom severity (74 %), frequency of day‐ and night‐time symptoms (67 %), activity impairment (57 %), respiratory function monitoring (nasal and/or lung function; 40 %) and incidence of AR exacerbations (50 %). 91 % of delegates felt a simple VAS would be a useful tool to gauge AR symptom control.ConclusionsA substantial portion of patients with AR are perceived as having uncontrolled or partly controlled disease even when treated. A simple VAS score is considered a useful tool to monitor AR control.
Structural Change of Methyltrimethoxysilane in the Early Stage of Sol–Gel Coating via Silsesquioxane
Authors: Ando Hideyo , Suzuki Masahiro , Muramatsu Ryo , Nakazawa Masaki , Matsuda Yasuhiro , Tasaka Shigeru 
Published: October 2015

Abstract
Structural change of methyltrimethoxysilane (MTMS), which can form sol–gel coating films via silsesquioxane, was investigated to elucidate the mechanism of coating at the early stage. Although films that can be used for sol–gel coating can be formed only after heating at about 100 °C for an hour, growth of nanoparticles was detected in the MTMS solutions at room temperature by static and dynamic light scattering. The existence and growth of nanoparticles were also confirmed by other measurements.
Calixarene-polymers via Simple Polymerization of t-Butylcalix[8]arenes (A8-type Monomer) with Hexamethylene Diisocyanate (B2-type Monomer); Molecular Bunch of Grapes (Botryosin)
Authors: Kudo Hiroto , Shizuma Motohiro , Kubo Kosuke , Hayashi Tomohiro 
Published: October 2015

Abstract
Reaction of t-butylcalix[8]arene (BCA[8]) as an A8-type monomer with hexamethylene diisocyanate as a B2-type monomer was examined to afford only soluble polymers in high yields. Analysis by 1H NMR, IR, MALDI-TOF mass spectroscopy, and AFM indicated that the synthesized polymers consist of nanoscale-gel-shaped structures that contain fixed holes and cavities. We designated this class of polymers as botryosin, after the Greek word botrys, meaning a bunch of grapes. Examination of host–guest chemistry of the synthesized polymers revealed complexation with alkali metal ions.
The Mycenean golden kylix of the Benaki Museum: A dubitandum?
Authors: Papageorgiou Irini 
Published: October 2015

Η χρυσή κύλικα µε αρ. ευρ. 2108 αποκτήθηκε από το Μουσείο Μπενάκη το 1939 και, χρονολογούµενη στους µυκηναϊκούς χρόνους, αποτέλεσε µαζί µε τα κοσµήµατα του «Θησαυρού της Θήβας» τον πυρήνα της προϊστορικής συλλογής του Μουσείου. Προερχόµενη από αγορά, άρα εξ ορισµού ύποπτη, δεν κίνησε παρά ελάχιστα το ενδιαφέρον του επιστηµονικού κοινού σε τέτοιο µάλιστα σηµείο ώστε να λησµονηθεί, αν και ιδιότυπη, ή µάλλον εξαιτίας αυτού. Υποψίες για την αυθεντικότητά της έχουν πάντως διατυπωθεί, αλλά µόνον ανεπισήµως. Η κύλικα έχει κατασκευαστεί από τέσσερα φύλλα χρυσού ενωµένα µεταξύ τους µε κόλληση, εκτός από το έλασµα της λαβής, το οποίο έχει στερεωθεί µε καρφιά. Από τυπολογική άποψη, παρουσιάζει στα επιµέρους µορφολογικά της στοιχεία αρκετές συνάφειες µε χρυσά µυκηναϊκά αγγεία, καθώς και µε κεραµικά δείγµατα της ΥΕΙΙ-ΙΙΙΑ1 περιόδου. Ως προς την τεχνική της κατασκευής της, είναι συµβατή σε πολλά µε όσα δεδοµένα έχουν συλλεγεί για τη µεταλ-λοτεχνία της Ύστερης Εποχής του Χαλκού. Τη χαρακτηρίζουν, ωστόσο, κάποιες τεχνικές ιδιαιτερότητες που σπανίζουν ανάµεσα στα αγγεία από πολύτιµα µέταλλα δεν είναι όµως ανύπαρκτες. Εξαίρεση συνιστούν τόσο ο τρόπος µε τον οποίο έχει προσηλωθεί η λαβή στο χείλος, όσο και η κατασκευή της από τέσσερα ελάσµατα. Επιπλέον, σε σηµαντικό βαθµό από τα χρυσά αιγαιακά σκεύη της 2ης χιλιετίας π.Χ. αποκλίνει ως προς την ανάλυση του µετάλλου κατασκευής της. Πρόκειται για καθαρό χρυσό, στην πρωτοφανή αναλογία του 98,8% µε ελάχιστα ποσοστά αργύρου και χαλκού, ο οποίος είτε συνιστά µια σπάνια εξαίρεση αυτοφυούς χρυσού, είτε ανήκει στην κατηγορία του εξευγενισµένου. Εάν ισχύει η δεύτερη υπόθεση, η κύλικα συγκαταλέγεται µάλλον στα κίβδηλα, δεδοµένου ότι ο εξευγενισµός του χρυσού φαίνεται πως λαµβάνει χώρα µετά την εµφάνιση της νοµισµατοκοπίας. Σε κάθε περίπτωση θα πρέπει, πάντως, να υπογραµµιστεί ότι δεν διαθέτουµε πλούσια βάση δεδοµένων µε αναλύσεις επί του συνόλου των αιγαιακών χρυσών.
A plan to present the Greeks abroad through the Project method
Authors: Tsantali Calliope , Nikolidakis Symeon 
Published: October 2015

<p class="NoSpacing">The present paper is concerned with students’ sensitization about the Greeks abroad who are introduced through the Project method to reinforce national awareness, especially during an economic crisis period when the economic choices followed by Greece are disapproved. The dynamics of the Greek communities abroad towards the economic and cultural development of the countries of reception and the concurrent enhancement of their bonds with Greece are pinpointed. The contact between the Greek native students and the offspring of the Greeks abroad is pursued towards an exchange of viewpoints, considerations and information about issues of politics, economy and culture. As a result, they all have a sense of solidarity in an expanded Greece encompassing the Greeks’ communities abroad. History and the History of Civilization are utilized to present this topic through the Project method. This way, emphasis is placed on migration, national identity, solidarity and the dynamics developed by the population mobility in the globalized societies. The Project method forms the suitable ground to cultivate and develop such objectives. Thus, students will formulate perceptions and examine the specific issues from a different perspective. A combination of theoretical issues of nationalism with a post-modern narration about nationalism in the globalized societies is conducted, where the national identity is reinforced and the citizens’ role in the globalized societies is not overlooked. </p>