bluuebunny/crossref_metadata_2025_split huggingface dataset:
Trends of testing for and diagnosis of α1-antitrypsin deficiency in the UK: more testing is needed
Authors: Soriano Joan B. , Lucas Sarah J. , Jones Rupert , Miravitlles Marc , Carter Victoria , Small Iain , Price David , Mahadeva Ravi 
Published: May 2018

α1-antitrypsin deficiency (AATD) significantly increases the risk of developing chronic obstructive pulmonary disease (COPD), and testing of all COPD patients for AATD is recommended by the World Health Organization, European Respiratory Society and Global Initiative for Chronic Obstructive Lung Disease (GOLD). We aimed to determine trends for testing and diagnosing AATD from 1990 to 2014.This study analysed all patients diagnosed with COPD from about 550 UK Optimum Patient Care Research Database general practices, including a subgroup of those diagnosed before the age of 60 years.We identified 107 024 COPD individuals, of whom 29 596 (27.6%) were diagnosed before 60 years of age. Of them, only 2.2% (95% CI 2.09–2.43%) had any record of being tested for AATD. Of those tested, 23.7% (95% CI 20.5–27.1%) were diagnosed with AATD. Between 1994 and 2013 the incidence of AATD diagnosis generally increased. A diagnosis of AATD was associated with being male, being an ex-smoker, more severe COPD with a lower forced expiratory volume in 1 s % pred and higher GOLD 2017 stages (all p<0.05).Despite an increase in the frequency of AATD testing since 1990, only 2.2% of patients diagnosed with COPD before the age of 60 years were tested. AATD prevalence was 23.7% in those tested. Thus, it appears that AATD remains markedly underdiagnosed in COPD patients.
The mucus bundles responsible for airway cleaning are retained in cystic fibrosis and by cholinergic stimulation
Authors: Ermund Anna , Meiss Lauren N. , Dolan Brendan , Bähr Andrea , Klymiuk Nikolai , Hansson Gunnar C. 
Published: May 2018

The beneficial effect of anticholinergic therapy for chronic lung diseases such as chronic obstructive pulmonary disease (COPD) is well documented, although cholinergic stimulation paradoxically inhibits liquid absorption, increases ciliary beat frequency and increases airway surface liquid transport.Using pig tracheobronchial explants, we quantified basal mucus transport before as well as after incubation with the clinically used antimuscarinic compound ipratropium bromide (Atrovent) and stimulation with acetylcholine.As expected, surface liquid transport was increased by acetylcholine and carbachol. In contrast, the mucus bundles secreted from the submucosal glands normally transported on the cilia were stopped from moving by acetylcholine, an effect inhibited by ipratropium bromide. Interestingly, in pigs lacking a functional cystic fibrosis (CF) transmembrane conductance regulator (CFTR) channel, the mucus bundles were almost immobile. As in wild-type pigs, CF surface liquid transport increased after carbachol stimulation. The stagnant CF mucus bundles were trapped on the tracheal surface attached to the surface goblet cells.Pseudomonas aeruginosabacteria were moved by the mucus bundles in wild-type but not CF pigs.Acetylcholine thus uncouples airway surface liquid transport from transport of the surface mucus bundles as the bundles are dynamically inhibited by acetylcholine and the CFTR channel, explaining initiation of CF and COPD, and opening novel therapeutic windows.
Reptin drives tumour progression and resistance to chemotherapy in nonsmall cell lung cancer
Authors: Mikesch Jan-Henrik , Schwammbach Daniela , Hartmann Wolfgang , Schmidt Lars H. , Schliemann Christoph , Angenendt Linus , Wiewrodt Rainer , Marra Alessandro , Thoennissen Nils H. , Wardelmann Eva , Köhler Gabriele , Lenz Georg , Müller-Tidow Carsten , Berdel Wolfgang E. , Arteaga Maria-Francisca 
Published: May 2018

While targeted nonsmall cell lung cancer (NSCLC) therapies have improved the outcome of defined disease subtypes, prognosis for most patients remains poor. We found the AAA+ ATPase Reptin to be highly expressed in the vast majority of 278 NSCLC tumour samples. Thus, the objective of the study was to assess the role of Reptin in NSCLC.Survival analyses of 1145 NSCLC patients revealed that high RNA expression levels of Reptin are associated with adverse outcome. Knockdown of Reptin in human NSCLC cells impaired growthex vivoand eliminated engraftment in a xenograft model. Reptin directly interacted with histone deacetylase 1 (HDAC1) as the critical mechanism driving NSCLC tumour progression. Pharmacological disruption of the Reptin/HDAC1 complex resulted in a substantial decrease in NSCLC cell proliferation and induced significant sensitisation to cisplatin.Our results identify Reptin as a novel independent prognostic factor and as a key regulator mediating proliferation and clonal growth of human NSCLC cellsex vivoandin vivo. We unveil a Reptin/HDAC1 protein complex whose pharmacological disruption sensitises NSCLC cells to cisplatin, suggesting this approach for application in clinical trials.
Challenges and perspectives in obstructive sleep apnoea
Authors: Randerath Winfried , Bassetti Claudio L. , Bonsignore Maria R. , Farre Ramon , Ferini-Strambi Luigi , Grote Ludger , Hedner Jan , Kohler Malcolm , Martinez-Garcia Miguel-Angel , Mihaicuta Stefan , Montserrat Josep , Pepin Jean-Louis , Pevernagie Dirk , Pizza Fabio , Polo Olli , Riha Renata , Ryan Silke , Verbraecken Johan , McNicholas Walter T. 
Published: May 2018

Obstructive sleep apnoea (OSA) is a major challenge for physicians and healthcare systems throughout the world. The high prevalence and the impact on daily life of OSA oblige clinicians to offer effective and acceptable treatment options. However, recent evidence has raised questions about the benefits of positive airway pressure therapy in ameliorating comorbidities.An international expert group considered the current state of knowledge based on the most relevant publications in the previous 5 years, discussed the current challenges in the field, and proposed topics for future research on epidemiology, phenotyping, underlying mechanisms, prognostic implications and optimal treatment of patients with OSA.The group concluded that a revision to the diagnostic criteria for OSA is required to include factors that reflect different clinical and pathophysiological phenotypes and relevant comorbidities (e.g.nondipping nocturnal blood pressure). Furthermore, current severity thresholds require revision to reflect factors such as the disparity in the apnoea–hypopnoea index (AHI) between polysomnography and sleep studies that do not include sleep stage measurements, in addition to the poor correlation between AHI and daytime symptoms such as sleepiness. Management decisions should be linked to the underlying phenotype and consider outcomes beyond AHI.
The identification and appraisal of assessment tools used to evaluate metatarsus adductus: a systematic review of their measurement properties
Authors: Marshall Nicole , Ward Emily , Williams Cylie M. 
Published: June 2018

AbstractBackgroundMetatarsus adductus is the most common congenital foot deformity in newborns. It involves adduction of the metatarsals at the Lisfranc joint. A systematic literature review was conducted to investigate the following question: What tools are used to identify and quantify metatarsus adductus and how reliable, valid and responsive are they?MethodsThe following electronic databases were searched for studies describing tools for the identification and quantification of metatarsus adductus in adults and children published from inception to June 2016: Ovid MEDLINE, Embase, CINAHL, Scopus, Web of Science and AMED. Two researchers initially searched all articles by screening titles and abstracts. If there was any doubt as to an article's eligibility, the full text paper was retrieved. Reference lists and citations of all retained studies were examined in an attempt to locate further studies. Articles were excluded if they were not in English or described other congenital foot conditions that did not include metatarsus adductus. Studies included in the review reporting measurement properties of measurement tools were critically appraised using the Consensus‐based Standards for the selection of health Measurement Instruments (COSMIN) critical appraisal tool.ResultsThere were 282 articles screened by title and abstract and 28 articles screened from full text. Fifteen articles were included and nine had data that were extractable for appraisal using the COSMIN critical appraisal tool. Techniques to measure metatarsus adductus included the heel bisector method, photocopies, ultrasound, footprints, dynamic foot pressure and radiographs. There was a paucity of quality data reporting the reliability, validity or responsiveness for measuring metatarsus adductus. Several radiographic angles showed good reliability (intraclass correlation (ICC) – 0.84, 0.97) in adults during pre‐operative planning.ConclusionThere have been multiple assessment techniques proposed for quantification of metatarsus adductus, but there is paucity of reliability, validity or responsiveness to measurement data about these techniques, especially in relation to the paediatric population. Further consideration of measurement testing is required to determine if the most common non‐radiographic measures of metatarsus adductus are acceptable for clinical use.
Treatment of Relapsed Myeloma in a Patient With Renal Insufficiency
Authors: Bladé Joan , Rosiñol Laura , Cibeira María Teresa , Fernández de Larrea Carlos 
Published: May 2018

The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors’ suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice. A 45-year-old man was diagnosed in March 2010 with stage III immunoglobulin G kappa multiple myeloma (MM) after presenting with bone pain as a result of multiple lytic bone lesions and T12 vertebral collapse. Laboratory work-up showed a serum M protein of 72 g/L and a 24-hour kappa light-chain urine protein excretion of 730 mg, hemoglobin of 10.2 g/dL, serum albumin of 49 g/L, serum β2-microglobulin of 6.4 mg/L, serum creatinine level of 1.6 mg/dL with an estimated glomerular filtration rate (eGFR) of 47 mL/min/1.73 m2, and normal serum calcium and lactate dehydrogenase (LDH) levels. His bone marrow contained 58% plasma cells, which showed the 17p deletion abnormality (Fig 1). He was treated with vertebroplasty and alternating chemotherapy with carmustine, vincristine, cyclophosphamide, melphalan, and prednisone and vincristine, carmustine, doxorubicin and dexamethasone. Because of progressive disease, salvage therapy with bortezomib and dexamethasone was administered with no response. The patient was then switched to lenalidomide and dexamethasone, which yielded minimal response. He underwent autologous stem-cell transplantation (ASCT) with melphalan 200 mg/m2 as high-dose therapy in February 2011, which led to a partial response, but in December 2011, progressive disease was documented, and the patient was enrolled in a clinical trial of carfilzomib monotherapy, with stable disease for 33 cycles. In October 2014 serum M protein rose to 38.6 g/L, with 24-hour kappa light-chain urine protein excretion of 840 mg, serum creatinine of 2.1 mg/dL, and an eGFR of 41 mL/min/1.73 m2. He presented to discuss ongoing treatment options.
The LDIFLARE and CCM Methods Demonstrate Early Nerve Fiber Abnormalities in Untreated Hypothyroidism: A Prospective Study
Authors: Sharma Sanjeev , Tobin Victoria , Vas Prashant R J , Rayman Gerry 
Published: May 2018

AbstractContextRecent studies using skin biopsy suggest presence of small-fiber neuropathy in subclinical hypothyroidism. This study uses two noninvasive methods—the laser Doppler imager flare technique (LDIFLARE) and corneal confocal microscopy (CCM)—to assess small-fiber function (SFF) and small-fiber structure (SFS), respectively, in newly diagnosed hypothyroidism (HT) before and after adequate treatment.Design and SettingSingle-center, prospective, intervention-based cohort study.Patients and ParticipantsTwenty patients with newly diagnosed HT (15 with primary HT and 5 with post-radioiodine HT) along with 20 age-matched healthy controls (HCs).InterventionsPatients with HT and HCs were assessed neurologically at diagnosis and baseline, respectively. The HT group was reassessed after optimal replacement (defined as TSH level of 0.27 to 4.20 mIU/L) with levothyroxine (LT4) and HCs were reviewed after 1 year.Main Outcome MeasuresNeurologic assessment for small fibers was performed by using LDIFLARE for SFF and CCM for SFS; large fibers were studied by sural nerve conduction velocity (SNCV) and sural nerve amplitude (SNAP).ResultsAt baseline, both LDIFLARE (mean ± SD) (6.74 ± 1.20 vs 8.90 ± 1.75 cm2; P = 0.0002) and CCM nerve fiber density (CNFD) (expressed as number of fibers per mm2: 50.77 ± 6.54 vs 58.32 ± 6.54; P = 0.002) were significantly reduced in the HT group compared with HCs whereas neither SNCV nor SNAP was different (P ≥ 0.05). After optimal LT4 treatment, both LDIFLARE (7.72 ± 1.12 vs 6.74 ± 1.20 cm2; P ≤ 0.0001) and CNFD (54.43 ± 5.70 vs 50.77 ± 6.54 no./mm2; P = 0.02) improved significantly but remained significantly reduced compared to HCs (P = 0.008 and P = 0.01, respectively) despite normalization of TSH.ConclusionsThis study demonstrates that dysfunction of small fibers precedes large neural fiber abnormalities in early HT. This can be reversed by replacement therapy to achieve a biochemically euthyroid state, but small-fiber neural outcomes continued to remain low compared with values in HCs.
Memory Dysfunction
Authors: Gliebus G. Peter 
Published: June 2018

ABSTRACTPURPOSE OF REVIEWThis article reviews the current understanding of memory system anatomy and physiology, as well as relevant evaluation methods and pathologic processes.RECENT FINDINGSOur understanding of memory formation advances each year. Successful episodic memory formation depends not only on intact medial temporal lobe structures but also on well-orchestrated interactions with other large-scale brain networks that support executive and semantic processing functions. Recent discoveries of cognitive control networks have helped in understanding the interaction between memory systems and executive systems. These interactions allow access to past experiences and enable comparisons between past experiences and external and internal information. The semantic memory system is less clearly defined anatomically. Anterior, lateral, and inferior temporal lobe regions appear to play a crucial role in the function of the semantic processing system. Different but tightly interconnected cortical regions, such as the prefrontal region, may play a controlling role in this system. The presentation of clinical disease affecting memory is the result of the selective vulnerability of the memory system. An understanding of current concepts of memory anatomy, physiology, and evaluation plays a central role in establishing an accurate diagnosis.SUMMARYDifferent memory systems rely on separate but overlapping distributed brain networks. Certain pathologic processes preferentially affect memory systems. An understanding of memory formation stages will enable more accurate diagnosis.
Editorial
Authors: Thomas Joby 
Published: June 2018

Atna, Journal of Tourism Studies (AJTS) has offered a platform for the publication of quality articles on research and learning in tourism over the years, thus reading out to researchers and professionals in the field. The Journal has continued to make serious inroads to understand the varied aspects and issues related to the study of tourism. Scholars, with the submission of excellent articles and reviewers through their critical feedback have helped to enhance the quality of the articles published. We are grateful to all authors and reviewers for helping AJTS to build a quality journal among the travel and tourism community in our country. The scholarly contributions featured in the current issue range across such wide areas, as Tourism Development, Local Community and Tourism, Coastal Tourism, Human Resource Management in Tourism, Tourism Management and Heritage Tourism. “A Study on Creation and Development of Wine Tourism Circuits in Maharashtra, by Anupama Kotur Kaddi examines the most important considerations for the creation of wine tourism circuits and analyses the opinions of wine tourism service providers on the creation and development of wine tourism circuits in the wine regions of Maharashtra. Local Community Involvement in Tourism: A Content Analysis of Websites of Wildlife Resorts, by Nagarjuna G reports on the various activities and practices adopted by wildlife resorts for the development of the local community. “Sustainable Coastal Tourism: A Community Perspective,” by Joseph P D and Pakkeerappa P examines the indicators of coastal sustainability from the perspective of the local community along the selected beaches of Dakshina Kannada. Cheryl Venan Dias and Venan Bonaventure Dias try to identify the human resource issues which are prevalent in the micro, small and medium travel, tourism and hospitality enterprises in their article “A Shift from Work-Life Balance to Work-Life Choices: A Study on Tourism Professionals in Goa”.
Book Reviews
Authors: Lollar Karen , Zlatic Thomas D. , Logan Robert K. , Jacobson Ron , Nayman Ira , Hunt III Arthur W. , Petkanas Bill 
Published: May 2018

Abstract

More Sensible Thinking, Martin H. Levinson (2012)
New York: Institute of General Semantics, 210 pp.,
ISBN: 9780982755969, p/bk, $15.95

Walter Ong’s Contributions to Cultural Studies: The Phenomenology of the Word and I-Thou Communication, Thomas J. Farrell (2015)
Revised edition, with an introduction by Robert A. White and a new afterword and bibliography, New York: Hampton Press, 354 pp.,
ISBN: 981572739505, h/bk, $41.95

Sapiens: A Brief History of Humankind, Yuval Noah Harari (2014)
Toronto: Signal: McClelland & Stewart (Canada) (2015), New York: Harper Collins (US), 464 pp.,
ISBN-13: 9780062316097, h/bk, $35.00, ISBN-13: 9780062316110, p/bk, $22.99

Writing Myself into Existence: Notes on a Literary Life and Other Adventures, Arthur Asa Berger (2016)
Seattle, WA: NeoPoiesis Press, 174 pp.,
ISBN: 9780990356561, p/bk, $16.95

McLuhan in an Age of Social Media, Paul Levinson
Connected Editions, 63 pp.,
ISBN: 9781561780501, p/bk, $9.99

The Benedict Option: A Strategy for Christians in a Post-Christian Nation, Rod Dreher (2017)
New York: Sentinel Press, 262 pp.,
ISBN: 9780735213296, h/bk, $25.00

Confronting Technopoly: Charting a Course Towards Human Survival, Phil Rose (ed.) (2017)
Chicago, IL: University of Chicago Press, 252 pp.,
ISBN: 9781783206889, p/bk, $100
An analysis of creative effect on interdisciplinary practices in art education
Authors: Kim Hyungsook 
Published: May 2018

Abstract
The conventional belief that arts education is individual-based and pure no longer holds any meaning. An interdisciplinary approach in this field is becoming more widespread. The consilience between the arts, humanities and social sciences, the arts and the natural sciences, and the arts and art education is recognized as enabling more creativity and thinking skills that are more integrated. Thus, art education has emerged as a core pillar in producing future talents. This study emphasizes the need for interdisciplinary practices for ensuring the creativity enhancement of the learner through art education, which aims to produce creative talents. Therefore, we developed and deployed interdisciplinary practices designed for sixth graders from August 2013 to January 2014. We assessed the learner’s creativity before and after using Language A and figures from the Torrance Tests of Creative Thinking (TTCT) in order to verify its effect on the correlation among five lower creativity factors. The results of the research questions showed a significant effect of interdisciplinary practices on the development of learners’ creativity. Figure A of the TTCT Type A language consisted of five lower factors of creativity: fluency, originality, abstractness of the title, delicacy and premature closure. The five resistance causes to these factors demonstrated a significant difference. Finally, this study maintained that the interdisciplinary practice of an art-based curriculum could promote students’ capacity by fostering creativity and providing the basis for an assessment method in contemporary art education.
Solidarity rebooted: Trust and the rise of civil society in times of crisis
Authors: Winter Lisa , Korbiel Izabela , Koukou Asimina , Sarikakis Katharine 
Published: May 2018

Abstract
Citizens’ distrust in political institutions has reached an all-time high in the European Union in recent years. Since the outbreak of the European sovereign debt crisis in 2009, the public’s faith in the community of nations has slumped, and EU institutions have made slow progress in regaining their citizens’ trust. What began as an economic crisis has sparked off mediated debates about European integration and therein about processes of exclusion and inclusion on multiple levels. Gradually, the media have turned their attention to national and supranational decisions as a means of solving the crisis. Not only the effectiveness and legitimacy of political decisions, but also the European Union per se, and any claims for European unity, were challenged in this process. Under these circumstances, we argue, there is a struggle over whose ‘voice’ is to be heard. Elites, political and financial in particular, have historically found in media a platform to be represented. Citizens’ voices, however, have not had this access. They pursue this by circumventing established media practices to generate and circulate counter-narratives to those of governments, financial corporations and the media. This article discusses the ways in which falling trust in institutions and institutions’ failure to maintain a welfare state, to resolve the crisis and to demonstrate solidarity as a matter of obligation to the EU constitutional underpinnings have reinforced the rise of civil society connectedness. Although, as we discuss, this is a wider European phenomenon, we examine the situation in a crisis hot zone, Greece, and the ways in which civil society has aimed to counterbalance a failing welfare state. The article aims to take stock of on-the-ground strategies of solidarity, which need to be understood not only against the backdrop of distrust and institutional failure, but also by the side of lack of acknowledgement and recognition and depoliticization of citizens’ dissent.
Documentary is as documentary does: The production of realities in The Reunion
Authors: von Schantz Miriam 
Published: May 2018

Abstract
This article argues that the film Återträffen (The Reunion) (Odell, 2013) offers an ethical event of reconfiguring the partition of the sensible as distributing boundaries between the factual and the fictive, what the author calls an event of realing. By turning the tables of expectations and context, this film, marketed as a feature fiction film, is seen to cause disruption to the spectatorial practice of parting the sensible through a contraction of foreknowledge, instead ushering the viewing subject into a missing contract, which is to say, making possible a responsible embrace of the act of viewing as an ethico-onto-epistemological practice.