bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Cleft Multidisciplinary Collaborative: Establishing a Network to Support Cleft Lip and Palate Research in the United Kingdom
Authors: Sainsbury David C. G. , Davies Amy , Wren Yvonne , Southby Lucy , Chadha Ambika , Slator Rona , Stock Nicola Marie ,   The Cleft Multidisciplinary Collaborative
Published: August 2018

Background: As a growing paradigm of health research, trainee collaboratives can influence clinical practice through the generation of cost-effective multicenter audit and research projects. The aims of the present article are to outline and discuss the establishment of a multidisciplinary collaborative in the context of cleft lip and/or palate (CL/P). Methods: The Cleft Multidisciplinary Collaborative (CMC) was formed in April 2016 under the overarching supervision of the National Institute for Health Research. Membership of the CMC is open to all members of the CL/P multidisciplinary team, who are encouraged to submit ideas for new research projects that will benefit clinical practice. Results: To date, 48 clinical participants are involved in the CMC. These participants represent all 17 cleft teams from the United Kingdom and encompass a wide range of disciplines. The CMC has undertaken 2 major projects so far. The first involved collection of phenotype data to support a national cohort study. The second, still in progress, is a systematic review investigating factors associated with outcomes for velopharyngeal competence following cleft palate repair. Conclusions: The concept of a multidisciplinary collaborative in CL/P has been demonstrated through the generation of a United Kingdom-wide network of committed clinicians and researchers and the effective undertaking of 2 large research projects. As the CMC gathers momentum, it hopes to attract funding to support its activities, to promote more involvement from the allied health and nursing professions, to encourage a more ingrained research culture within the CL/P community, and to promote the wider ambition of a global collaborative.
Impact of Virtual Planning on Surgical Reposition of the Premaxilla Using an Endonasal Approach and Simultaneous Alveoloplasty
Authors: Martínez Plaza Adoración , Martínez Elena Bullejos , Tara Miguel Pérez de Perceval , Fernandez Ana Belén Marín , Iglesias Fernando Monsalve , López Antonio España 
Published: August 2018

Objective: Describe the surgical repositioning of the premaxilla using an innovative minimally invasive endonasal approach and secondary bone graft at the same time. We want to emphasize the importance of virtual surgical planning in this technique. Material and Methods: A total of 6 patients with bilateral complete cleft lip and palate underwent a surgical repositioning of the premaxilla. Virtual surgical planning was performed in all cases. The ages varied between 8 and 12 years and all were male. Five patients were in the mixed dentition phase and 1 patient was in the definitive phase. Three of the patients had been prepared with presurgical nasoalveolar molding. The other 3 were not prepared for various reasons. All patients had primary repair of cleft lip and palate. Interventions: An innovative minimally invasive endonasal approach is presented that has allowed a safe 3-D reposition of the premaxilla in patients with bilateral cleft palate. A simultaneous secondary alveoloplasty with the use of absorbable osteosynthesis is a good choice to achieve symmetry and stability. Conclusions: Virtual surgical planning is an exceptional instrument to make an appropriate presurgical selection of the patients in which combine the 2 procedures.
Radiographic Evaluation of First Metatarsal and Medial Cuneiform Morphology in Juvenile Hallux Valgus
Authors: Kaiser Philip , Livingston Kristin , Miller Patricia E. , May Collin , Mahan Susan 
Published: August 2018

Background: Operative treatment of juvenile hallux valgus (JHV) has a high recurrence rate. The aim of this study was to better understand the pattern of radiographic deformity. Methods: Standing radiographs of 93 feet in 57 patients with JHV, and 50 feet in 36 normal patients were measured. Measurements included: hallux valgus angle (HVA), first metatarsal physis status (open or closed), intermetatarsal angle (IMA), distal metatarsal articular angle (DMAA), Meary’s angle, medial cuneiform angle (MCA), relative first to second metatarsal length ratio (1:2 MT ratio), cuneiform obliquity (CO), and congruency of the metatarsophalangeal joint (MTPJ). JHV groups were defined as normal (HVA 0-15 degrees), mild-moderate (HVA 15-35), or severe (HVA > 35) and were analyzed. Results: Seventy of 93 feet (75%) with JHV presented with mild-moderate JHV (average HVA of 26.2 ± 5.6 degrees), and 23 feet (25%, 23/93) presented with severe JHV (average HVA of 41.9 ± 5.3 degrees). Multivariable analysis determined that DMAA ( P < .001), MCA ( P = .04), and congruency ( P < .001) were independently associated with JHV and its severity (normal vs mild-moderate vs severe). Severe JHV cases had larger DMAA ( P = .01), larger IMA ( P = .01), larger 1:2 MT ratio ( P = .02), and were less frequently congruent ( P = .03) compared with mild-moderate JHV cases. Conclusion: Deformity in JHV was highly correlated with both the proximal and distal morphology of the first metatarsal and medial cuneiform. Severe JHV was associated with increased bony deformity and increased incongruity of the MTPJ. Treatment should be individualized, but JHV treatment algorithms can take this information into account. Level of Evidence: Level III, comparative study.
Predicting the profile of increasing disability in multiple sclerosis
Authors: Tomassini Valentina , Fanelli Fulvia , Prosperini Luca , Cerqua Raffaella , Cavalla Paola , Pozzilli Carlo 
Published: August 2018

Background: Effective therapeutic strategies to preserve function and delay progression in multiple sclerosis (MS) require early recognition of individual disease trajectories. Objectives: To determine the profiles of disability evolution, identify their early predictors and develop a risk score of increasing disability. Methods: We analysed demographic, clinical and magnetic resonance imaging (MRI) data from patients with relapsing MS, Expanded Disability Status Scale (EDSS) score of 3.0–4.0 and follow-up ≥ 2 years. Attaining EDSS = 6.0 defined increasing disability; relapses and/or MRI defined disease activity. Results: In total, 344 out of 542 (63.5%) patients reached EDSS ≥ 6.0; of these, 220 (64.0%) showed disease activity. In patients with activity, the number of relapses before reaching EDSS 3.0–4.0 predicted increasing disability; age > 45 at baseline predicted increasing disability without activity. Combining age and number of relapses increased the risk of and shortened the time to EDSS = 6.0. Conclusion: Increasing disability is frequently associated with persistent activity. The high number of relapses identifies early those patients worsening in the presence of activity. Age predicts increasing disability in the absence of activity. The presence of both factors increases the risk of developing severe disability. As this study likely describes the transition to progression, our findings contribute to improving patient management and stratification in trials on progressive MS.
How do facilitators of group programmes for long-term conditions conceptualise self-management support?
Authors: Hughes Stephen , Lewis Sophie , Willis Karen , Rogers Anne , Wyke Sally , Smith Lorraine 
Published: August 2018

Objectives Increasing self-management skills in people with long-term conditions is widely advocated in policies and guidelines. Group programmes are a common format; yet, how self-management support objectives are enacted in their delivery is poorly understood. Our aim is to explore the perspectives of group programme facilitators. Methods We undertook thematic analysis of transcribed data from in-depth semi-structured interviews with health professional facilitators (n = 13) from six diverse self-management support group programmes (of obesity, diabetes and chronic obstructive pulmonary disease). Results Facilitators viewed group programmes as responses to health system pressures, e.g. high patient demand. They focussed on providing in-depth education and instruction on physical health, risks and lifestyle behaviour change and emphasised self-responsibility for behaviour change whilst minimising goal setting and support amongst group participants. There were tensions between facilitators’ professional identity and group leader role. Discussion Group self-management support programmes may not be realising the broader aspirations advocated in long-term condition policy to support medical, emotional and social aspects of long-term conditions by minimising shared learning, problem solving, building of self-efficacy and goal setting. This suggests a disconnect at implementation. Increasing understandings of theoretical and practical self-management support in group programmes across both implementation and health professional (HCP) training will further the professional skills in this format.
Loneliness, social isolation and risk of cardiovascular disease in the English Longitudinal Study of Ageing
Authors: Valtorta Nicole K , Kanaan Mona , Gilbody Simon , Hanratty Barbara 
Published: August 2018

Background There is increasing evidence of an association between social relationships and morbidity in general, and cardiovascular disease in particular. However, recent syntheses of the evidence raise two important questions: is it the perceived quality or the more objective quantity of relationships that matters most; and what are the implications of changes in relationships over time? In this study, we investigate the cumulative effects of loneliness and social isolation on incident cardiovascular disease. Design A secondary analysis of prospective follow-up data from the English Longitudinal Study of Ageing (ELSA). Methods To assess the association between social isolation or loneliness and incident cardiovascular disease, lagged values of exposure to loneliness and isolation were treated as time-varying variables in discrete time survival models controlling for potential confounders and established cardiovascular disease risk factors. Results A total of 5397 men and women aged over 50 years were followed up for new fatal and non-fatal diagnoses of heart disease and stroke between 2004 and 2010. Over a mean follow-up period of 5.4 years, 571 new cardiovascular events were recorded. We found that loneliness was associated with an increased risk of cardiovascular disease (odds ratio 1.27, 95% confidence interval 1.01–1.57). Social isolation, meanwhile, was not associated with disease incidence. There was no evidence of a cumulative effect over time of social relationships on cardiovascular disease risk. Conclusions Loneliness is associated with an increased risk of developing coronary heart disease and stroke, independently of traditional cardiovascular disease risk factors. Our findings suggest that primary prevention strategies targeting loneliness could help to prevent cardiovascular disease.
Association Between Low-Molecular-Weight Heparin and Risk of Bleeding Among Hemodialysis Patients: A Retrospective Cohort Study
Authors: Lazrak Hind H. , René Emilie , Elftouh Naoual , Lafrance Jean-Philippe 
Published: August 2018

Background: Low-molecular-weight heparins (LMWH) replaced unfractionated heparin (UFH) in multiple indications. Although LMWH efficacy in hemodialysis was demonstrated through multiple studies, their safety remains controversial. The potential bioaccumulation in patients undergoing chronic hemodialysis raised the question of bleeding risk among this population. Objective: The aim of this study was to evaluate bleeding risk among patients with chronic hemodialysis receiving LMWH or UFH for the extracorporeal circuit anticoagulation. Design: We conducted a retrospective cohort study on data extracted from the Régie de l’assurance maladie du Québec (RAMQ) and Med-Echo databases from January 2007 to March 2013. Setting: Twenty-one hemodialysis centers in the province of Québec, Canada. Patients: Chronic hemodialysis patients. Measurements: Bleeding risk evaluated by proportional Cox model for time-dependent exposure using demographics, comorbidities, and drug use as covariates. Methods: Minor, major, and total bleeding events identified using International Classification of Diseases, Ninth Revision ( ICD-9)/ International Classification of Diseases, Tenth Revision ( ICD-10) codes in the RAMQ and Med-Echo databases. Exposure status to LMWH or UFH was collected through surveys at the facility level. Results: We identified 5322 prevalent and incident patients with chronic hemodialysis. The incidence rate for minor, major, and total bleeding was 9.45 events/1000 patient-year (95% confidence interval [CI]: 7.61-11.03), 24.18 events/1000 patient-year (95% CI: 21.52-27.08), and 32.88 events/1000 patient-year (95% CI: 29.75-36.26), respectively. We found similar risks of minor adjusted hazard ratio (HR: 1.04; 95% CI: 0.68-1.61), major (HR: 0.83; 95% CI: 0.63-1.10), and total bleeding (HR: 0.90; 95% CI: 0.72-1.14) when comparing LMWH with UFH. Limitations: Potential misclassification of patients’ exposure status and possible underestimation of minor bleeding risk. Conclusion: LMWH was not associated with a higher minor, major, or total bleeding risk. LMWH did not increase the risk of bleeding compared with UFH for the extracorporeal circuit anticoagulation in hemodialysis. The convenience of use and predictable effect made LMWH a suitable alternative to UFH in hemodialysis.
Superior Canal Dehiscence: A Comparative Postmortem Multislice Computed Tomography Study
Authors: Mittmann Philipp , Ernst Arne , Seidl Rainer , Skulj Anna‐Felicitas , Mutze Sven , Windgassen Marc , Buschmann Claas 
Published: August 2018

ObjectiveSuperior canal dehiscence is defined by missing bony coverage of the superior canal against the middle cranial fossa. The gold standard in diagnosis is high‐resolution computed tomography (CT). A false‐positive CT scan, identifying a dehiscence when one is not present, could lead to unnecessary surgical therapy. This study aims to compare postmortem CT scans with autopsy findings with regard to superior canal dehiscence.Study DesignPostmortem study.SettingTertiary referral center.Subjects and MethodsTwenty‐two nontraumatic death cases within a 3‐month period (January to March 2017) were included with 44 temporal bones. Each body underwent postmortem head CT prior to medicolegal autopsy. The middle fossa floor was exposed, and if present, the superior semicircular canal dehiscence was identified and measured. In each case, 3 comparable photographs were taken during the autopsy (left temporal bone, right temporal bone, overview).ResultsAutopsy findings revealed bony dehiscences in 11% of the temporal bones, whereas CT scan revealed bony dehiscences in 16%. The length of the dehiscences were longer when measured by CT imaging.ConclusionThe diagnosis of superior canal dehiscence syndrome requires high‐resolution CT with clinical symptoms and physiologic evidence of a third mobile window. Our study underlines a mismatch between multislice CT imaging in the coronal plane and the presence of a dehiscence on autopsy.
Separating two painting campaigns in Saul and David, attributed to Rembrandt, using macroscale reflectance and XRF imaging spectroscopies and microscale paint analysis
Authors: Dooley Kathryn A. , Gifford E. Melanie , van Loon Annelies , Noble Petria , Zeibel Jason G. , Conover Damon M. , Alfeld Matthias , Van der Snickt Geert , Legrand Stijn , Janssens Koen , Dik Joris , Delaney John K. 
Published: August 2018

Abstract
Late paintings of Rembrandt van Rijn (1606–1669) offer intriguing problems for both art historians and conservation scientists. In the research presented here, the key question addressed is whether observed stylistic differences in paint handling can be correlated with material differences. In Saul and David, in the collection of the Royal Picture Gallery Mauritshuis in The Hague, NL, the stylistic differences between the loose brushwork of Saul’s cloak and the more detailed depiction of his turban and the figure of David have been associated with at least two painting stages since the late 1960s, but the attribution of each stage has been debated in the art historical literature. Stylistic evaluation of the paint handling in the two stages, based on magnified surface examination, is further described here. One of the research goals was to determine whether the stylistic differences could be further differentiated with macroscale and microscale methods of material analysis. To address this, selected areas of the painting having pronounced stylistic differences were investigated with two macroscopic chemical imaging methods, X-ray fluorescence and reflectance imaging spectroscopies. The pigments used were identified and their spatial distribution was mapped. The mapping results show that the passages rendered in more detail and associated stylistically with the first painting stage, such as the orange-red color of David’s garment or the Greek key design in Saul’s turban, were painted with predominately red ochre mixed with vermilion. The regions of loose, bold brushwork, such as the orange-red slashing strokes in the interior of Saul’s cloak, associated with the second painting stage, were painted with predominately red ochre without vermilion. These macroscale imaging results were confirmed and extended with scanning electron microscopy with energy-dispersive X-ray spectroscopy (SEM–EDX) analysis of three cross-sections taken from regions of stylistic differences associated with the two painting stages, including one sample each from the right and left sleeve of David, and one from the interior of Saul’s cloak. SEM–EDX also identified a trace component, barium sulfate, associated with the red ochre of the second stage revisions. Combining mapping information from two spectroscopic imaging methods with localized information from microscopic samples has clearly shown that the stylistic differences observed in the paint handling are affiliated with differences in the chemical composition of the paints.
Are there any differences in the provided burn care between men and women? A retrospective study
Authors: Pompermaier Laura , Elmasry Moustafa , Abdelrahman Islam , Fredrikson Mats , Sjöberg Folke , Steinvall Ingrid 
Published: August 2018

Abstract

Background
Disparity between medical treatment for men and women has been recorded worldwide. However, it is difficult to find out if the disparities in both the use of resources and outcome depend entirely on sex-related discrimination. Our aim was to investigate if there are differences in burn treatments between the sexes.

Methods
All patients admitted with burns to Linköping University Hospital during the 16-year period 2000–2015 were included. Interventions were prospectively recorded using the validated Burn SCoring system (BSC). Data were analysed using a multivariable panel regression model adjusted for age, percentage total body surface area (%TBSA), and in-hospital mortality.

Results
A total of 1363 patients were included, who generated a total of 22,301 daily recordings while they were inpatients. Males were 70% (930/1363). Sex was not an independent factor for daily scores after adjustment for age, %TBSA, and mortality in hospital (model R2=0.60, p &lt; 0.001).

Conclusion
We found no evidence of inequity between the sexes in treatments given in our burn centre when we had adjusted for size of burn, age, and mortality. BSC seems to be an appropriate model in which to evaluate sex-related differences in the delivery of treatments.
BRAF V600E Confers Male Sex Disease-Specific Mortality Risk in Patients With Papillary Thyroid Cancer
Authors: Wang Fei , Zhao Shihua , Shen Xiaopei , Zhu Guangwu , Liu Rengyun , Viola David , Elisei Rossella , Puxeddu Efisio , Fugazzola Laura , Colombo Carla , Jarzab Barbara , Czarniecka Agnieszka , Lam Alfred K. , Mian Caterina , Vianello Federica , Yip Linwah , Riesco-Eizaguirre Garcilaso , Santisteban Pilar , O’Neill Christine J. , Sywak Mark S. , Clifton-Bligh Roderick , Bendlova Bela , Sýkorová Vlasta , Wang Yangang , Xing Mingzhao 
Published: August 2018

Purpose To test whether the prognostic risk of male sex in papillary thyroid cancer (PTC) is determined by BRAF V600E and can thus be stratified by BRAF status. Patients and Methods We retrospectively investigated the relationship between male sex and clinicopathologic outcomes in PTC, particularly mortality, with respect to BRAF status in 2,638 patients (male, n = 623; female, n = 2,015) from 11 centers in six countries, with median age of 46 years (interquartile range, 35-58 years) at diagnosis and median follow-up time of 58 months (interquartile range, 26-107 months). Results Distant metastasis rates in men and women were not different in wild-type BRAF PTC but were different in BRAF V600E PTC: 8.9% (24 of 270) and 3.7% (30 of 817; P = .001), respectively. In wild-type BRAF PTC, mortality rates were 1.4% (five of 349) versus 0.9% (11 of 1175) in men versus women ( P = .384), with a hazard ratio (HR) of 1.59 (95% CI, 0.55 to 4.57), which remained insignificant at 0.70 (95% CI, 0.23 to 2.09) after clinicopathologic multivariable adjustment. In BRAF V600E PTC, mortality rates were 6.6% (18 of 272) versus 2.9% (24 of 822) in men versus women ( P = .006), with an HR of 2.43 (95% CI, 1.30 to 4.53), which remained significant at 2.74 (95% CI, 1.38 to 5.43) after multivariable adjustment. In conventional-variant PTC, male sex similarly had no effect in wild-type BRAF patients; mortality rates in BRAF V600E patients were 7.2% (16 of 221) versus 2.9% (19 of 662) in men versus women ( P = .004), with an HR of 2.86 (95% CI, 1.45 to 5.67), which remained significant at 3.51 (95% CI, 1.62 to 7.63) after multivariable adjustment. Conclusion Male sex is a robust independent risk factor for PTC-specific mortality in BRAF V600E patients but not in wild-type BRAF patients. The prognostic risk of male sex in PTC can thus be stratified by BRAF status in clinical application.
Pheochromocytoma in Children and Adolescents With Multiple Endocrine Neoplasia Type 2B
Authors: Makri Angeliki , Akshintala Srivandana , Derse-Anthony Claudia , Del Rivero Jaydira , Widemann Brigitte , Stratakis Constantine A , Glod John , Lodish Maya 
Published: August 2018

Abstract

Context
Multiple endocrine neoplasia type 2B (MEN2B) is characterized by early-onset medullary thyroid cancer in virtually all cases and a 50% lifetime risk of pheochromocytoma (PHEO) development. The literature on PHEO in patients with MEN2B is limited with most data being reported from adult studies that primarily address MEN2A.

Objective
The aim of the current study is to describe PHEO development in a cohort of pediatric patients with MEN2B.

Design
Retrospective chart review of patients with MEN2B evaluated at the National Institutes of Health in the period between July 2007 and February 2018.

Results
A total of 38 patients were identified (21 males and 17 females). Mean age at MEN2B diagnosis was 10.6 ± 3.9 years. Eight patients (21%) developed PHEO in the course of follow-up to date, all of whom were sporadic cases with the classic M918T RET mutation. PHEO was diagnosed based on biochemical and/or imaging screening studies in five patients, whereas three patients presented with symptoms of excess catecholamines. PHEO was diagnosed at a mean age 15.2 ± 4.6 (range, 10 to 25) years and 4.0 ± 3.3 years after MEN2B diagnosis. Only one patient was diagnosed with PHEO as the initial manifestation of MEN2B after she presented with hypertension and secondary amenorrhea.

Conclusion
Undiagnosed PHEO can be associated with substantial morbidity. Current American Thyroid Association guidelines recommend PHEO screening starting at age 11 for the high-/highest risk group. The youngest patient diagnosed with PHEO in our cohort was an asymptomatic 10-year-old, suggesting that PHEO development may begin before the screening-recommended age of 11, though remains clinically undetectable and thus the current screening guidelines seem appropriate.