bluuebunny/crossref_metadata_2025_split huggingface dataset:
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC/D): A Systematic Literature Review
Authors: Romero Jorge , Mejia-Lopez Eliany , Manrique Carlos , Lucariello Richard 
Published: May 2013

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetic form of cardiomyopathy (CM) usually transmitted with an autosomal dominant pattern. It primary affects the right ventricle (RV), but may involve the left ventricle (LV) and culminate in biventricular heart failure (HF), life threatening ventricular arrhythmias and sudden cardiac death (SCD). It accounts for ll%-22% of cases of SCD in the young athlete population. Pathologically is characterized by myocardial atrophy, fibrofatty replacement and chamber dilation. Diagnosis is often difficult due to the nonspecific nature of the disease and the broad spectrum of phenotypic variations. Therefore consensus diagnostic criteria have been developed and combined electrocardiography, echocardiography, cardiac magnetic resonance imaging (CMRI) and myocardial biopsy. Early detection, family screening and risk stratification are the cornerstones in the diagnostic evaluation. Implantable cardioverter-defibrillator (ICD) implantation, ablative procedures and heart transplantation are currently the main therapeutic options.
Using Museum Objects to Improve Wellbeing in Mental Health Service Users and Neurological Rehabilitation Clients
Authors: Ander Erica E , Thomson Linda JM , Blair Kathryn , Noble Guy , Menon Usha , Lanceley Anne , Chatterjee Helen J 
Published: May 2013

Introduction: The study investigated the impact of museum object handling sessions on hospital clients receiving occupational therapy in neurological rehabilitation and in an older adult acute inpatient mental health service. Methods: The research used a qualitative approach based on objectivist and constructionist methods, from which themes typical of the object handling sessions were derived. Results: Themes emerging from detailed analysis of discourse involving clients (n = 82) and healthcare staff (n = 8) comprised: distraction and decreasing negative emotion; increasing vitality and participation; tactile stimulation; conversational and social skills; increasing a sense of identity; novel perspectives and thoughts; learning new things; enjoyment and positive emotion. Critical success factors included good session facilitation for mitigating insecurity, ward staff support and the use of authentic heritage objects. Conclusion: Museums and their collections can be a valuable addition to cultural and arts occupations, in particular for long-stay hospital clients.
The Executive Dysfunctions Most Commonly Associated with Multiple Sclerosis and Their Impact on Occupational Performance
Authors: Preston Jenny , Hammersley Richard , Gallagher Helen 
Published: May 2013

Introduction: The relationship between executive dysfunction and functional status in people with multiple sclerosis (MS) is not fully understood. This study attempts to provide empirical evidence of the executive dysfunctions most commonly associated with MS and to develop an understanding of the impact of this on occupational performance. Method: This cross-sectional study explores the relationships between an MS group (n = 69) and a healthy control group (n = 30) using the Behavioural Assessment of Dysexecutive Syndrome and the Dysexecutive Questionnaire. Results: More than a third of people with MS experienced some impairment of executive function sufficient to interfere with occupational performance. The most commonly occurring problems included planning, problem solving, behavioural regulation, lack of insight, apathy and lack of motivation. Executive function deficits were evident from the early stages of the disease, although participants with a secondary progressive form of MS appeared to demonstrate greater difficulties. Conclusion: While the Behavioural Assessment of Dysexecutive Syndrome may reliably identify the presence of executive dysfunction within this clinical population it does not inform clinicians of the impact on occupational performance. Self-reported executive function demonstrates heightened awareness of occupational performance deficits and may lead to more accurate reporting of executive dysfunction. The impact of depression, fatigue and anxiety, however, requires further investigation.
The Feasibility of Reactive Dye in PVA Films as High Dosimeter
Authors: Shaheen Akhtar  , Taqmeem Hussain  , Aamir Shahzad  , Qamar-ul-Islam  
Published: May 2013

The gamma dosimetric response of the Methylene Blue (MB) in Polyvinyl Alcohol (PVA) films was investigated. The spectrophotometric analysis was done to observe the changes in these films at pre and post irradiation. The PVA based films were irradiated using Cs137 γ-source within dose range (100-200) kGy. The effect of concentration of dyes, pH of the dyed solutions and thickness of dyed films were employed as the dosimetric parameters in this study. The stability of MB-PVA films before and after irradiation was examined at ambient temperature. MB-PVA films were found to be light sensitive. Owing to simple preparation and absence of any toxic solvents during preparation, PVA based films were found to be docile for large-scale production and application for routine irradiation processes of medical equipments.
Louis Dantin et l’écriture autobiographique
Authors: Hayward Annette 
Published: May 2013

Un des aspects les plus fascinants de ce Québécois surdoué qu’on connaît sous le nom de Louis Dantin est l’histoire de sa vie. Il en était conscient, comme en témoigne son souci de livrer des éléments autobiographiques, que ce soit dans sa correspondance, ses oeuvres lyriques, son « esquisse autobiographique » ou dans Les enfances de Fanny, qu’il qualifiait de « roman autobiographique ». Même s’il n’a pas osé publier plusieurs de ces textes de son vivant, son exemple a inspiré beaucoup d’écrivains de l’entre-deux-guerres. Cet article examine les différents volets de son discours autobiographique tout en suggérant que la complexité de cet homme, qui était en avance sur son temps, dépasse nettement l’image qu’il a osé livrer dans ses écrits.
Se préfacer, dans l’oubli de soi-même
Authors: Bonenfant Luc 
Published: May 2013

Louis Dantin est-il l’auteur des vers de Nelligan ? En répondant provisoirement oui à cette question, posée par Valdombre en 1938 et depuis reprise par de nombreux critiques, cet article s’intéresse au problème de l’allonymie chez Dantin. L’étude comparée de textes poétiques et narratifs « signés » par Nelligan, Silvio et Eugène Seers permet en effet de dégager une allégorèse de type biographique où l’écrivain se ferait le fossoyeur de lui-même et de ses masques. Les allonymes et autres noms propres qui désignent provisoirement l’écrivain participent ainsi d’une axiologie euphorique où, en s’absorbant tous dans celui de Louis Dantin, ils signent en définitive la naissance possible et efficace de Dantin lui-même, ce nom devenant d’office la marque persistante de l’homme, c’est-à-dire celle sous laquelle l’histoire le retiendra tout autant comme poète que comme critique.
Les enfances de Fanny : un roman américain
Authors: Morency Jean 
Published: May 2013

Publié à titre posthume en 1951, Les enfances de Fanny s’avère beaucoup plus qu’un roman autobiographique dans lequel Louis Dantin aurait courageusement bravé les interdits de son époque. Cette oeuvre constitue en fait l’aboutissement de l’exil de Dantin aux États-Unis et de son intégration socioculturelle à son milieu d’accueil. Rien de « canadien » en effet dans Les enfances de Fanny, si ce n’est l’usage du français, Dantin s’inspirant plutôt de ses lectures américaines, qui forment la matière de ses chroniques sur le livre américain publiées dans Le Jour et qui contribuent à tisser, dans le roman, tout un réseau thématique et intertextuel qui contribue à faire de cette oeuvre un grand roman américain.
Portrait de Réjean Ducharme en messie des lettres québécoises 1
Authors: Chabot Julien-Bernard 
Published: May 2013

Cet article propose une étude de la réception des oeuvres de Réjean Ducharme dans le contexte de la décennie 1960, époque où prévaut un imaginaire du temps qui appelle le changement à s’incarner, à prendre corps et visage humains. Ce désir d’emblématisation se traduit, dans le domaine des lettres québécoises, par l’attente du « grand écrivain » qui officialiserait la réussite du projet de modernisation propre à la Révolution tranquille. L’accueil réservé aux premières oeuvres de Ducharme, qui a donné lieu à ce que l’on appelle désormais « l’affaire Ducharme », a été directement influencé par ce climat de messianisme littéraire, et plus particulièrement par l’écart mesurable entre le désir de faire de l’auteur un emblème du renouveau national et la volonté de l’auteur de se retirer de la sphère publique.
Insertion en emploi et TED : analyse des retombées et des enjeux politiques d’un projet pilote
Authors: Dumais Lucie , Prohet Alexandra , Archambault Léonie 
Published: May 2013

L’équipe de recherche a évalué un projet pilote visant l’insertion professionnelle de personnes ayant un trouble envahissant du développement (TED). Au terme du projet de 36 mois, 29 % des 45 participants occupaient un emploi rémunéré, souvent au-delà du salaire minimum. L’analyse des données a permis de constater que les méthodes d’intervention ont évolué en cours de projet, que plusieurs objectifs ont été atteints, mais que le rodage du modèle n’a pas été possible. L’analyse des processus a permis de cerner les enjeux d’une dynamique partenariale ainsi que ceux associés aux intérêts politiques en contexte d’expérimentation. Dans l’ensemble, le projet a servi à mieux cerner les critères d’une intégration en emploi réussie et à saisir comment s’élaborent des services et programmes d’insertion.
Asymptotically stable detection of a weak signal
Authors: Shevlyakov Georgy , Shin Vladimir , Lee Seokhyoung , Kim Kiseon 
Published: May 2013

SUMMARYTo design highly efficient and robust detectors of a weak signal, an asymptotic approach to stable estimation exploiting redescending score functions is used. Two new indicators of robustness of detection, the detection error sensitivity and detection stability, are introduced. The optimal Neyman–Pearson rules maximizing detection efficiency under the guaranteed level of detection stability are written out. Under heavy‐tailed noise distributions, the proposed asymptotically stable detectors based on redescending score functions, namely, the minimum error sensitivity and the radical ones, outperform conventional linear bounded Huber's and redescending Hampel's detectors both on small and large samples. Copyright © 2013 John Wiley & Sons, Ltd.
A Graphene Oxide and Copper‐Centered Metal Organic Framework Composite as a Tri‐Functional Catalyst for HER, OER, and ORR
Authors: Jahan Maryam , Liu Zhaolin , Loh Kian Ping 
Published: May 2013

AbstractA composite made from the assembly of graphene oxide (GO) and copper‐centered metal organic framework (MOF) shows good performance as a tri‐functional catalyst in three important electrocatalysis reactions, namely: the hydrogen evolution reaction (HER), oxygen evolution reaction (OER), and oxygen reduction reaction (ORR). One of the challenges in the area of electrocatalysis is to find an effective catalyst that will reduce, as well as generate, oxygen at moderate temperatures. The enhanced electrocatalytic properties and stability in acid of the GO‐MOF composite is due to the unqiue porous scaffold structure, improved charge transport and synergistic interactions between the GO and MOF. In polymer electrolyte membrane fuel cell testing, the GO‐incorporated Cu‐MOF composite delivers a power density that is 76% that of the commercial Pt catalyst.
Report of a patient with developmental delay, hearing loss, growth retardation, and cleft lip and palate and a deletion of 7q34‐36.1: Review of distal 7q deletions
Authors: Rush Eric T. , Stevens Jadd M. , Sanger Warren G. , Olney Ann H. 
Published: May 2013

AbstractThe use of aCGH has improved our ability to find subtle cytogenetic abnormalities as well as to find more precise information in patients with previously known abnormalities. In addition, it has allowed more specific genotype–phenotype correlation. In this report we describe a patient with a chromosomal deletion initially diagnosed with conventional cytogenetic analysis which was redemonstrated and more specifically described upon aCGH analysis. Our patient is a 12‐year‐old female born to a 26‐year‐old G1P0 mother. She was noted as a neonate to have a bilateral cleft lip and cleft palate, abnormal external ears, dysmorphic facies, and moderate to severe hearing loss. She has subsequently shown developmental delay, hyperreflexia, seizures, hyperactivity, and absence of speech. Chromosomal analysis showed deletion of 7q34q36.1. FISH studies confirmed the deletion was interstitial. Parental chromosomes were performed and did not show any cytogenetic abnormalities. aCGH was recently performed for the patient to further characterize the breakpoints of the deletion and confirmed the deletion was interstitial and of 13.2 Mb in size. Both proximal and terminal 7q deletion show a different phenotype than that of our patient. A number of patients with similar deletions have been found and while significant variability is observed, a number of findings appear to be common to deletions in this region. Therefore, we feel that distal interstitial deletions of chromosome 7q represent a recognizable phenotype and could be considered a separate deletion syndrome. © 2013 Wiley Periodicals, Inc.
Focal dermal hypoplasia (goltz–gorlin syndrome): A new case with a novel variant in the PORCN gene (c.1250T>C:p.F417S) and unusual spinal anomaly
Authors: Garavelli Livia , Simonte Graziella , Rosato Simonetta , Wischmeijer Anita , Albertini Enrico , Guareschi Elisa , Longo Caterina , Albertini Giuseppe , Gelmini Chiara , Greco Chiara , Errico Stefania , Savino Gustavo , Pavanello Marco , Happle Rudolf , Unger Sheila , Superti‐Furga Andrea , Grzeschik Karl‐Heinz 
Published: May 2013

AbstractFocal dermal hypoplasia (FDH; Goltz–Gorlin syndrome; OMIM 305600) is a disorder that features involvement of the skin, skeletal system, and eyes. It is caused by loss‐of‐function mutations in the PORCN gene. We report a young girl with FDH, microphthalmos associated with colobomatous orbital cyst, dural ectasia and cystic malformation of the spinal cord, and a de novo variant in PORCN. This association has not been previously reported, and based on these observations the phenotypic spectrum of FDH might be broader than previously appreciated. It would be prudent to alter the suggested surveillance for this rare disorder. © 2013 Wiley Periodicals, Inc.
The value of the clinical geneticist caring for adults with congenital heart disease: Diagnostic yield and patients' perspective
Authors: van Engelen Klaartje , Baars Marieke J.H. , Felix Joyce P. , Postma Alex V. , Mulder Barbara J.M. , Smets Ellen M.A. 
Published: May 2013

AbstractFor adult patients with congenital heart disease (CHD), knowledge about the origin and inheritance of their CHD is important. Clinical geneticists may play a significant role in their care. We explored the diagnostic yield of clinical genetic consultation of adult CHD patients, patients' motivations for the consultation, implications for reproductive decisions, patients' evaluation of the impact of provided information, and satisfaction with counseling. Chart review was performed on all adult patients referred for CHD to our clinical genetics department between 2000 and 2011 (n = 90). Additionally, a questionnaire was sent to those patients referred between 2005 and 2011 (n = 64), of which 46 useful questionnaires were returned (72% response). Of patients without an etiological diagnosis at referral (n = 83), 17 (20%) were eventually diagnosed with syndromic CHD, 6 (7%) with nonsyndromic monogenetic CHD and 45 (54%) with nonsyndromic multifactorial CHD. The diagnosis remained undetermined in 15 (18%) patients. Half of patients who returned the questionnaire had purposefully postponed having children until after genetic consultation and 13% had changed their mind about having children or not after the consultation. Counseling was valued positively. In this study, we showed the added value of clinical genetic consultation in the care for adult CHD patients: it improves diagnostics by establishing an etiological diagnosis and associated recurrence risk in a substantial proportion of patients and leads to more informed reproductive decisions. With new genetic testing technologies an etiological diagnosis may be established in an increasing number of patients in the near future. © 2013 Wiley Periodicals, Inc.
Thermal integration of heat transfer fluid systems
Authors: Bade Mukund H. , Bandyopadhyay Santanu 
Published: May 2013

ABSTRACTAppropriate integration of subsystems results in an energy‐efficient and cost‐effective design of the overall system. In many industrial applications, heat transfer fluid (HTF) is used to supply required amount of heat to multiple heat demands from a single heat source. Appropriate integration of fired heater with a process leads to overall reduction in energy requirement as well as capital investment. In this paper, four methodologies are developed to integrate fired heater appropriately with a process. Firstly, a targeting methodology is proposed to determine the minimum HTF flow rate to supply heat from single heater to multiple heat demands. Secondly, a methodology to integrate radiant fired heater with a process through HTF is discussed. Thirdly, indirect integration of fired heater, incorporating energy recovery from flue gas through the air preheater, is developed. Finally, improvement of overall energy efficiency by utilizing process heat below pinch through air preheating is demonstrated. It is also illustrated that the number of fired heater can be reduced, and hence, the total cost of the system can also be reduced by indirect integration. © 2013 Curtin University of Technology and John Wiley & Sons, Ltd.
Association between operative approach and complications in patients undergoing Hartmann's reversal
Authors: Cellini C , Deeb A-P , Sharma A , Monson J R T , Fleming F J 
Published: May 2013

AbstractBackgroundComplications following reversal of Hartmann's procedure are common, with morbidity rates of up to 50 per cent, and a mortality rate as high as 10 per cent. This is based on case series with heterogeneous data collection and analysis. This study determined risk factors for complications following Hartmann's reversal.MethodsPatients who underwent elective open and laparoscopic Hartmann's reversal were identified from the American College of Surgeons National Surgical Quality Improvement Program database (2005–2010). The programme collects patient demographics, preoperative medical history, clinical findings and laboratory investigations. Postdischarge data were obtained by a certified reviewer. Complications were categorized as major, septic or incisional. Risk-adjusted 30-day outcomes were assessed by univariable and multivariable analyses, adjusting for patient characteristics, co-morbidity and operative approach.ResultsDuring the study period 7996 patients had a Hartmann's procedure and 2567 cases of Hartmann's reversal were identified, including 336 laparoscopic procedures (13·1 per cent). Major, septic and incisional complication rates were 13·3, 8·5 and 15·7 per cent respectively, with a mortality rate of 0·5 per cent. A laparoscopic approach was found to be independently associated with fewer major (odds ratio (OR) 0·53, 95 per cent confidence interval 0·34 to 0·81), septic (OR 0·48, 0·27 to 0·83) and incisional (OR 0·54, 0·37 to 0·80) complications. A history of chronic obstructive pulmonary disease (OR 1·78–2·00), steroid use (OR 1·75), body mass index at least 30 kg/m2 (OR 1·48), diabetes (OR 1·40), smoking (OR 1·33–1·40), American Society of Anesthesiologists fitness grade III and IV (OR 1·46–1·48) and prolonged operating time (OR 1·02) were other factors associated with complications.ConclusionA laparoscopic approach to Hartmann's reversal was associated with fewer complications than open surgery in this highly selected group of patients.
Catheter-directed thrombolysis for iliofemoral deep vein thrombosis
Authors: Saunders J H , Arya P H , Abisi S , Yong Y P , MacSweeney S , Braithwaite B , Altaf N 
Published: May 2013

Abstract

Background
Recent international guidance recommends the use of catheter-directed thrombolysis (CDT) in selected patients with symptomatic iliofemoral deep vein thrombosis (DVT). The aim of this study was to estimate the potential increase in workload as a result of this recommendation.

Methods
Using the radiology database, a review was performed of all DVTs diagnosed between August 2010 and February 2012 at a large tertiary referral hospital. The National Institute for Health and Clinical Excellence and American College of Chest Physicians guidance was applied retrospectively to this cohort, using case-note review by two independent clinicians to determine which patients would have been suitable for CDT.

Results
Some 563 patients had DVT confirmed radiologically over the 18-month interval. Fifty-three of the 128 patients with iliofemoral DVT would have been eligible for intervention with CDT, equivalent to 4·4 patients per 100 000 per year. Only eight (15 per cent) of the 53 were actually referred to vascular services for treatment. All eight patients had successful CDT, which involved a stay in critical care for monitoring (median 2 (range 1–3) sessions).

Conclusion
Vascular units should be prepared for a major increase in the requirement for CDT for iliofemoral DVT. This increase will affect inpatient beds, the interventional radiology suite, critical care and interhospital referrals.
Randomized clinical trial on enhanced recovery versus standard care following open liver resection
Authors: Jones C , Kelliher L , Dickinson M , Riga A , Worthington T , Scott M J , Vandrevala T , Fry C H , Karanjia N , Quiney N 
Published: May 2013

Abstract

Background
Enhanced recovery programmes (ERPs) have been shown to reduce length of hospital stay (LOS) and complications in colorectal surgery. Whether ERPs have the same benefits in open liver resection surgery is unclear, and randomized clinical trials are lacking.

Methods
Consecutive patients scheduled for open liver resection were randomized to an ERP group or standard care. Primary endpoints were time until medically fit for discharge (MFD) and LOS. Secondary endpoints were postoperative morbidity, pain scores, readmission rate, mortality, quality of life (QoL) and patient satisfaction. ERP elements included greater preoperative education, preoperative oral carbohydrate loading, postoperative goal-directed fluid therapy, early mobilization and physiotherapy. Both groups received standardized anaesthesia with epidural analgesia.

Results
The analysis included 46 patients in the ERP group and 45 in the standard care group. Median MFD time was reduced in the ERP group (3 days versus 6 days with standard care; P &lt; 0·001), as was LOS (4 days versus 7 days; P &lt; 0·001). The ERP significantly reduced the rate of medical complications (7 versus 27 per cent; P = 0·020), but not surgical complications (15 versus 11 per cent; P = 0·612), readmissions (4 versus 0 per cent; P = 0·153) or mortality (both 2 per cent; P = 0·987). QoL over 28 days was significantly better in the ERP group (P = 0·002). There was no difference in patient satisfaction.

Conclusion
ERPs for open liver resection surgery are safe and effective. Patients treated in the ERP recovered faster, were discharged sooner, and had fewer medical-related complications and improved QoL. Registration number: ISRCTN03274575 (http://www.controlled-trials.com).