bluuebunny/crossref_metadata_2025 huggingface dataset:
A new Bernoulli–Euler beam model based on a reformulated strain gradient elasticity theory
Authors: Zhang GY , Gao X-L
Published: November 2019

A new non-classical Bernoulli–Euler beam model is developed using a reformulated strain gradient elasticity theory that incorporates both couple stress and strain gradient effects. This reformulated theory is first derived from Form I of Mindlin’s general strain gradient elasticity theory. It is then applied to develop the model for Bernoulli–Euler beams through a variational formulation based on Hamilton’s principle, which leads to the simultaneous determination of the equation of motion and the complete boundary conditions and provides a unified treatment of the strain gradient, couple stress and velocity gradient effects. The new beam model contains one material constant to account for the strain gradient effect, one material length scale parameter to describe the couple stress effect and one coefficient to represent the velocity gradient effect. The current non-classical beam model reduces to its classical elasticity-based counterpart when the strain gradient, couple stress and velocity gradient effects are all suppressed. In addition, the newly developed beam model includes the models considering the strain gradient effect only or the couple stress effect alone as special cases. To illustrate the new model, the static bending and free vibration problems of a simply supported beam are analytically solved by directly applying the general formulas derived. The numerical results reveal that the beam deﬂection predicted by the current model is always smaller than that by the classical model, with the difference being large for very thin beams but diminishing with the increase of the beam thickness. Also, the natural frequency based on the new beam model is found to be always higher than that based on the classical model.
The Social Origins of ESG: An Analysis of Innovest and KLD
Authors: Eccles Robert G. , Lee Linda-Eling , Stroehle Judith C.
Published: November 2019

This article uses the study of two environmental, social, and governance (ESG) data vendors—KLD and Innovest—to exemplify the “social origins of ESG issues” argument made by Eccles and Stroehle in their 2018 working paper “Exploring Social Origins in the Construction of ESG Measures.” Based on in-depth interviews with the organizations’ founders and historical document analysis, we recap the history of the cases and show how different origins, philosophies, and “purposes” of ESG issues shaped the methods and data characteristics of two of the most important data vendors of their time. We discuss why MSCI chose to continue with the financial value–oriented methodology of Innovest while discontinuing the values-driven KLD methodology. Through an in-depth literature analysis, we further show that not only the creation but also the use of “nonfinancial performance” concepts rely on processes of social construction. We also show that investors use different ESG data from those used by academics, potentially leading to misaligned narratives. Finally, with this article we join the call for more explicit contextualization of ESG data, highlighting that both practitioners and academics need to better understand the social construction that underlies analyses that use different concepts of ESG.
Social–Behavioral Interventions for Secondary-Level Students With High-Functioning Autism in Public School Settings: A Meta-Analysis
Authors: McKeithan Glennda K. , Sabornie Edward J.
Published: November 2019

The number of secondary-level students with high-functioning autism (HFA) served in public school settings has increased in recent years, and many of these students have difficulty with social–behavioral expectations in such settings. Instructional specialists must know which interventions have been shown to be effective, so they can make informed decisions about service delivery and instructional support (professional development) that maximize the potential for student success. The intent of the present meta-analysis was to examine the efficacy of social–behavioral interventions used with adolescent students with HFA served in public school settings. Thirteen studies employing single-case research designs published in peer-reviewed journals between 1987 and 2018 were found through an extensive literature search. The analyses indicated that specific social skill and behavioral interventions had high effect sizes and, likewise, improved outcomes for students with HFA. Implications for special education practice and future research are provided.
The Relationships of High-Sensitivity C-Reactive Protein and Homocysteine Levels With Disease Activity, Damage Accrual, and Cardiovascular Risk in Systemic Lupus Erythematosus
Authors: Pocovi-Gerardino Gabriela , Correa-Rodríguez Maria , Rubio José-Luis Callejas , Fernández Raquel Ríos , Amada María Martín , Caparros María-Gracia Cruz , Rueda-Medina Blanca , Ortego-Centeno Norberto
Published: November 2019

Chronic inflammation coupled with cardiovascular disease (CVD) risk factors influences the progression of atherosclerosis in systemic lupus erythematosus (SLE). High-sensitivity C-reactive protein (hs-CRP) and homocysteine (Hcy) are associated with the risk of CVD in the general population, but their associations with CV risk and disease activity in SLE are unclear. In this cross-sectional study ( N = 139 SLE patients, mean age = 45.27 ± 13.18 years), we investigated associations between hs-CRP and Hcy levels and disease activity, damage accrual, and CVD risk in SLE. Disease activity and damage accrual were measured with the SLE Activity Index 2000 (SLEDAI-2K), the Systemic Lupus Erythematosus International Collaborating Clinics Group/American College of Rheumatology damage index (SDI), and anti-double-stranded DNA antibodies (anti-dsDNA). CVD risk factors of obesity, diabetes mellitus, hypertension, blood lipids, and ankle–brachial index were collected. Linear regression analysis and one-way analysis of variance were used to analyze relationships of hs-CRP and Hcy with SLE activity, damage accrual, and CVD risk factors.

Results:

hs-CRP correlated significantly with SLEDAI-2K ( p = .036), SDI ( p = .00), anti-dsDNA titers ( p = .034), diabetes ( p = .005), and obesity ( p = .027). hs-CRP and Hcy correlated with triglyceride (TG) levels ( p = .032 and p < .001, respectively), TG/high-density lipoprotein cholesterol index ( p = .020 and p = .001, respectively), and atherogenic index of plasma ( p = .006 and p = .016, respectively). hs-CRP levels >3 mg/L correlated with SDI score ( p = .012) and several CVD risk factors.

Discussion:

Findings suggest SLE patients with elevated hs-CRP and/or Hcy have a higher prevalence of CVD risk factors.
A rare association of blepharophimosis–ptosis–epicanthus inversus case with congenital nasolacrimal duct obstruction
Authors: Gupta Nishi , Ganesh Suma , Singla Poonam , Kumar Sunil
Published: November 2019

Purpose:

The aim of reporting this case is to describe a rare combination of blepharophimosis–ptosis–epicanthus inversus syndrome with congenital nasolacrimal duct obstruction. A variety of lacrimal anomalies have been seen in blepharophimosis–ptosis–epicanthus inversus syndrome but the occurrence of nasolacrimal duct obstruction is rare.

Method:

The blepharophimosis–ptosis–epicanthus inversus syndrome is an autosomal dominant rare genetic defect with clinical manifestation of dysplasia of the eyelids, palpebral fissures, flat nasal bridge, and ptosis. A 20-month-old boy was referred with the complaints of watering and discharge from his right eyes since birth. On examination, the child had all the features of blepharophimosis–ptosis–epicanthus inversus syndrome with right congenital nasolacrimal duct obstruction in line with the published reports.

Result:

On endoscopic probing and irrigation, the probe could not be visualized into the inferior meatus. On dacryoendoscopy, the membranous part of the nasolacrimal duct was found to be completely obliterated with no light transmission into the nose indicating a malformed nasolacrimal duct. The child was managed by endoscopic dacryocystorhinostomy. We could find only one case report published so far on the combination of congenital nasolacrimal duct obstruction with blepharophimosis–ptosis–epicanthus inversus syndrome. This study adds one more case of blepharophimosis–ptosis–epicanthus inversus syndrome with congenital nasolacrimal duct obstruction and adjuvant use of dacryoendoscopy.
Efficacy of alemtuzumab in relapsing-remitting MS patients who received additional courses after the initial two courses: Pooled analysis of the CARE-MS, extension, and TOPAZ studies
Authors: Comi Giancarlo , Alroughani Raed , Boster Aaron L , Bass Ann D , Berkovich Regina , Fernández Óscar , Kim Ho Jin , Limmroth Volker , Lycke Jan , Macdonell Richard AL , Sharrack Basil , Singer Barry A , Vermersch Patrick , Wiendl Heinz , Ziemssen Tjalf , Jacobs Alan , Daizadeh Nadia , Rodriguez Claudio E , Traboulsee Anthony , Baker Darren P. , Bueno Ericka M. , Mitchell Colin , Orndorff Rebecca L. , Zediak Valerie P.
Published: November 2019

Background:

Alemtuzumab is given as two annual courses. Patients with continued disease activity may receive as-needed additional courses.

Objective:

To evaluate efficacy and safety of additional alemtuzumab courses in the CARE-MS (Comparison of Alemtuzumab and Rebif® Efficacy in Multiple Sclerosis) studies and their extensions.

Methods:

Subgroups were based on the number of additional alemtuzumab courses received. Exclusion criteria: other disease-modifying therapy (DMT); <12-month follow-up after last alemtuzumab course.

Results:

In the additional-courses groups, Courses 3 and 4 reduced annualized relapse rate (12 months before: 0.73 and 0.74, respectively; 12 months after: 0.07 and 0.08). For 36 months after Courses 3 and 4, 89% and 92% of patients were free of 6-month confirmed disability worsening, respectively, with 20% and 26% achieving 6-month confirmed disability improvement. Freedom from magnetic resonance imaging (MRI) disease activity increased after Courses 3 and 4 (12 months before: 43% and 53%, respectively; 12 months after: 73% and 74%). Safety was similar across groups; serious events occurred irrespective of the number of courses.

Conclusion:

Additional alemtuzumab courses significantly improved outcomes, without increased safety risks, in CARE-MS patients with continued disease activity after Course 2. How this compares to outcomes if treatment is switched to another DMT instead remains unknown.
Causative factors of eating problems among adolescents with type 1 diabetes mellitus: A qualitative study
Authors: Sien Pamela Lim Ming , Jamaludin Nur Iryani Amirah , Samrin Siti Nur Adila , S Nik Shanita , Ismail Rokiah , Anuar Zaini Azriyanti , Sameeha Mohd Jamil
Published: November 2019

Adolescents with type 1 diabetes mellitus are prone to have eating problems. This study aimed to determine factors of eating problems among this population in University Malaya Medical Centre. Fifteen adolescents who scored more than 20 marks in the Diabetes Eating Problem Survey – Revised questionnaire were invited for an in-depth interview. Questions were asked based on their questionnaire’s response. The interview sessions were recorded, transcribed verbatim, and thematic analysis was used. Five main themes emerged: pressure, physiological factor, psychological factor, patient’s low compliances to insulin intake and food control and fear. Early referral to child psychologist would prevent it from developing.
The Empower Action Model: A Framework for Preventing Adverse Childhood Experiences by Promoting Health, Equity, and Well-Being Across the Life Span
Authors: Srivastav Aditi , Strompolis Melissa , Moseley Amy , Daniels Kelsay
Published: November 2019

The empower action model addresses childhood adversity as a root cause of disease by building resilience across multiple levels of influence to promote health, equity, and well-being. The model builds on the current evidence around adverse childhood experiences and merges important frameworks within key areas of public health—the socio-ecological model, protective factors, race equity and inclusion, and the life course perspective. The socio-ecological model is used as the foundation for this model to highlight the multilevel approach needed for improvement in public health. Five key principles that build on the protective factors literature are developed to be applied at each of the levels of the socio-ecological model: understanding, support, inclusion, connection, and growth. These principles are developed with actions that can be implemented across the life span. Finally, actions suggested with each principle are grounded in the tenets of race equity and inclusion, framing all actionable steps with an equity lens. This article discusses the process by which the model was developed and provides steps for states and communities to implement this tool. It also introduces efforts in a state to use this model within county coalitions through an innovative use of federal and foundation funding.
Gaps in knowledge and future directions for the use of faecal microbiota transplant in the treatment of inflammatory bowel disease
Authors: Yalchin Mehmet , Segal Jonathan P. , Mullish Benjamin H. , Quraishi Mohammed Nabil , Iqbal Tariq H. , Marchesi Julian R. , Hart Ailsa L.
Published: November 2019

Faecal microbiota transplant (FMT) has now been established into clinical guidelines for the treatment of recurrent and refractory Clostridioides difficile infection (CDI). Its therapeutic application in inflammatory bowel disease (IBD) is currently at an early stage. To date, there have been four randomized controlled trials for FMT in IBD and a multitude of observational studies. However, significant gaps in our knowledge regarding optimum methods for FMT preparation, technical aspects and logistics of its administration, as well as mechanistic underpinnings, still remain. In this article, we aim to highlight these gaps by reviewing evidence and making key recommendations on the direction of future studies in this field. In addition, we provide an overview of the current evidence of potential mechanisms of FMT in treating IBD.
Targeted therapies for advanced bladder cancer: new strategies with FGFR inhibitors
Authors: Casadei Chiara , Dizman Nazli , Schepisi Giuseppe , Cursano Maria Concetta , Basso Umberto , Santini Daniele , Pal Sumanta K. , De Giorgi Ugo
Published: November 2019

Inhibitors of fibroblast growth factor receptor (FGFR) represent an outstanding treatment approach for selected patients with urothelial cancer (UC). These agents are changing the clinical approach to a subgroup of UC, the luminal-papillary subtype, characterized by FGFR mutations, fusions, or amplification. In this review, we provide an overview of the results of recent clinical trials on FGFR tyrosine kinase inhibitors (TKIs) currently in clinical development for the treatment of UC: erdafitinib, rogaratinib, infigratinib, and the monoclonal antibody vofatamab. The Food and Drug Administration recently granted accelerated approval to erdafitinib for patients with advanced UC with alterations of FGFR2 or FGFR3 after progression on platinum-based chemotherapy. We also look at future therapeutic options of combination regimens with immune-checkpoint inhibitors as strategies for improving the antitumor effects of this class of drug, and for preventing or delaying the development of resistance.
An other geography
Authors: Oswin Natalie
Published: November 2019

The marginalization, sidelining, erasure and dismissal of ‘othered’ people and epistemologies persist within the discipline of geography today. In the present article, I discuss this fact as a source of harm for many individuals, a result of centuries of white supremacist heteropatriarchal grounding and a failure of the collective critical geographical imagination. A new turn is underway, however, one that turns away from the mainstream of the discipline and toward each ‘other’. Solidarities across modes of difference are building in scholarship that inhabits an epistemological elsewhere, and these can and must be harnessed in this time of serious threats to academic freedom and social justice.
High geographic prevalence of pulmonary artery hypertension: associations with ethnicity, drug use, and altitude
Authors: Dubroff Jason , Melendres Lana , Lin Yan , Beene Daniel Raley , Ketai Loren
Published: November 2019

While estimates of pulmonary arterial hypertension incidence and prevalence commonly range from 1–3/million and 15–25/million, respectively, clinical experience at our institution suggested much higher rates. We sought to describe the disease burden of pulmonary arterial hypertension in the geographic area served by our Pulmonary Hypertension Clinic and compare it to the REVEAL registry. Our secondary objectives were to document pulmonary arterial hypertension prevalence in minorities underrepresented in REVEAL (Hispanics and Native Americans) and to address the association of pulmonary arterial hypertension with exposure to drugs and moderately increased residential altitude in this population. Retrospective review of pulmonary arterial hypertension clinic patients alive during 2016 identified 154 patients. Hispanic patients made up 35.7% of the cohort, a much greater percentage than REVEAL, p < .001 but smaller than the percentage of Hispanic patients (48.4%) in geographic area served by the clinic. Pulmonary arterial hypertension due to drug exposure was more common and idiopathic pulmonary arterial hypertension was less common than in REVEAL (p < .001). Overall, pulmonary arterial hypertension incidence was 14 cases per million, greater than the REVEAL registry, odds ratio 6.3 (95% CI: 4.2–9.5), (p < .001). Annual period prevalence of pulmonary arterial hypertension was 93 cases per million, also greater than the REVEAL, odds ratio = 7.5 (95% CI: 6.4–8.8) and remained greater when the clinic cohort was constrained to patients with hemodynamic severity comparable to REVEAL, odds ratio = 3.8 (95% CI: 3.0–4.6), (p < .001). There was a strong association between pulmonary arterial hypertension prevalence and residence at altitude > 4000 ft, odds ratio = 26.6 (95% CI: 8.5–83.5), p < .001; however, this was potentially confounded by pulmonary arterial hypertension treatment referral patterns. These findings document a much higher local pulmonary arterial hypertension incidence and prevalence than previously reported in REVEAL. While population ethnicity differed markedly from REVEAL, the disease burden was not driven by these differences. The possible association of moderately increased residential altitude with pulmonary arterial hypertension warrants further evaluation.
Epidemiology of feline urolithiasis in Mexico (2006–2017)
Authors: Mendoza-López Claudia I , Del-Angel-Caraza Javier , Aké-Chiñas María A , Quijano-Hernández Israel A , Barbosa-Mireles Marco A
Published: November 2019

Objectives

The objectives of this study were to identify the proportions of different types of uroliths, characterize the population of cats that present with urolithiasis and determine possible predisposing factors in a population of Mexican cats.

Methods

This study analyzed clinical specimens of feline urolithiasis submitted to our laboratory in the period from 2006 to 2017. The mineral composition of the uroliths was determined by qualitative and quantitative mineral analyses, performed by stereoscopic microscopy and infrared spectroscopy.

Results

In the population studied, 54.3% of all uroliths were calcium oxalate, followed by 32.1% struvite and 7.4% purine (urate and xanthine) uroliths, with other types accounting for 6.2% of submissions. The male:female ratio was 1.2:1. Calcium oxalate submissions were predominantly from males and struvite submissions were predominantly from females. The age of the cats with stone submissions ranged from 6 months to 17 years. In cats with calcium oxalate uroliths, 52.3% were aged 7 years or older. Cats with struvite uroliths were younger, with 65.4% younger than 6 years of age. Almost 90% of all submitted uroliths were from domestic shorthair cats.

Conclusions and relevance

This is the first epidemiologic study of urolithiasis in cats in Mexico. Age and sex predispositions to common uroliths were identified, as males aged ≥7 years primarily presented with calcium oxalate uroliths and females aged <6 years primarily presented struvite uroliths. Cases of urolithiasis of genetic origin, including xanthinuria and cystinuria, were also detected, in addition to silicate uroliths.
Weapons of the Chic: Instagram Influencer Engagement Pods as Practices of Resistance to Instagram Platform Labor
Authors: O’Meara Victoria
Published: November 2019

This article examines the phenomenon of Instagram influencer “engagement pods” as an emergent form of resistance that responds to the reconfigured working conditions of platformized cultural production. Engagement pods are grassroots communities that agree to mutually like, comment on, share, or otherwise engage with each other’s posts, no matter the content, to game Instagram’s algorithm into prioritizing the participants’ content and show it to a broader audience. I argue that engagement pods represent a response to the material conditions of platformized cultural production on Instagram, where proprietary curation algorithms wrest knowledge and control of the labor process from producers. Cooperative algorithm hacking of this sort, although quite distinct from traditional organizing strategies, responds to the coercive force of the “threat of invisibility” that necessitates constant data production. They represent a collective attempt to exert some control over their “conditions of presence-to-others” and, in so doing, combat precarity and protect wages in the field. In a post-industrial economy where traditional models of labor organizing have struggled to address the conditions of platformized cultural work, I argue that the unusual phenomenon of Instagram engagement pods represents an organic form of worker resistance that responds to the unique conditions of these workers.
Populist and Popular: An Experiment on the Drivers of User Reactions to Populist Posts on Facebook
Authors: Blassnig Sina , Wirz Dominique S.
Published: November 2019

Populist politicians are often associated with the successful use of social networking sites (SNS). However, it is still unclear whether the popularity of populist posts is driven by the nature of the messages, by the populist actors as the source, or by the interaction of both factors. By following a 2 × 2 experimental design ( N = 647) and by manipulating populist versus nonpopulist messages in a Facebook post and a typically populist versus mainstream politician as the source, this study contributes to the existing literature in two ways. First, it shows that both populist messages and populist actors foster the perception of a Facebook post as populist but that only populist messages are drivers of user reactions. Second, complementing content analyses on populism and user reactions, the study demonstrates that the effect of populist communication on user reactions is moderated by recipients’ populist attitudes. Users with strong populist attitudes share populist messages more often than they share nonpopulist messages.
Remarkably Still Repairable Large Aortopulmonary Window in an Adult Patient
Authors: Zografos Panagiotis M. , Protopapas Eleftherios M. , Hakim Nikolaos I. , Alexopoulos Chrysanthos , Sarris George E.
Published: November 2019

Aortopulmonary window (APW) is an abnormal congenital connection between the main pulmonary artery (MPA) and the ascending aorta, with intact aortic and pulmonary valves, leading to heart failure or, if not repaired early, to pulmonary vascular obstructive disease. We report the rare case of an asymptomatic adult with an unrestrictive APW, whose pulmonary arterial hypertension was remarkably still reversible, permitting successful repair.
An Unusual Complication of the “Supported” Ross Procedure
Authors: Cedars Ari , Jobanputra Kamlesh , Bichell David
Published: November 2019

We present a unique surgical complication in a 19-year-old patient who underwent a “supported” Ross procedure for congenital aortic stenosis. In the present case, herniation of pulmonary autograft material through coronary fenestrations in the Dacron supporting material can be appreciated. This case suggests a possible need to modify surgical technique to ensure that all autograft tissue remains contained within the Dacron bolster.
Sliding Sheet Use in Nursing Practice: An Intervention Study
Authors: Alperovitch-Najenson Deborah , Weiner Chava , Ribak Joseph , Kalichman Leonid
Published: November 2019

Background: Previous studies have discovered that the utilization of sliding sheets in patient care is a valuable technique for repositioning bedridden patients compared with traditional cotton sheets or carriers. Our aim was to examine the effects of sliding sheet usage on work-related musculoskeletal disorders and disability, perceived workload, burnout, and job satisfaction, among nurses and nursing assistants. Method: This repeated measurement study included 41 female nurses and nursing assistants from three internal medicine departments who provided direct patient care. Participants completed an eight-part questionnaire (demographics, Neck Disability Index, Quick Disability of the Arms, Shoulder and Hand Questionnaire, Modified Oswestry Low Back Pain Disability Questionnaire, BackAche Disability Index workload, burnout, and job satisfaction) 4 times during the study period: 3 months prior to the intervention, on the first day of the intervention, and 3 and 6 months after commencement of the intervention. Findings: After 3 and 6 months of sliding sheet usage, pain and disability decreased in the neck ( p < .001); arms, shoulders, hands ( p = .041); and lower back ( p < .001), with an increase in job satisfaction ( p < .001). Discussion/Application to Practice: The findings of our study indicate a clear influence of reducing work-related musculoskeletal pain and disability while increasing job satisfaction when sliding sheets are introduced into nursing practice. Occupational health nurses in health care should consider this type of low-cost intervention as a method for reducing musculoskeletal injury among direct patient care providers.
Direction of Dependence Between Specific Symptoms of Depression: A Non-Gaussian Approach
Authors: García-Velázquez Regina , Jokela Markus , Rosenström Tom Henrik
Published: November 2019

Psychopathology could arise from direct interactions between symptoms. Evidence suggests that the mechanisms underlying somatic and cognitive-affective symptoms of depression are different. The aim of this study was to explore dynamic associations among cognitive-affective depression criteria. We used distribution-based direction of dependence models, which estimate whether the presence of symptom A is more likely to depend on the presence of symptom B than vice versa. We analyzed six large samples of adults from the United States ( N = 34,963) and conducted a simulation study to test the performance of the algorithm with ordinal variables and a second simulation study focusing on Type I error. Our results were consistent with the literature: Depressed mood and anhedonia were reactive to changes in other symptoms, whereas suicidality may reinforce other symptoms or reflect factors doing so. We discuss the results in the context of other empirical findings and theories of depression, reflect on the potential of these methods in psychopathology, and consider some practical implications.
Attitude and Perception of Physicians and Nurses Toward the Role of Clinical Pharmacists in Riyadh, Saudi Arabia: A Qualitative Study
Authors: Alsuhebany Nada , Alfehaid Lama , Almodaimegh Hind , Albekairy Abdulkareem , Alharbi Shmeylan
Published: November 2019

Clinical pharmacists are responsible for guiding pharmacotherapy and ensuring medication safety along with other health-care providers. This study highlighted barriers that physicians and nurses encounter when interacting with clinical pharmacists. Twenty-seven physicians and nurses were randomly invited to participate in focus-group discussions. Five focus-group discussions were recorded and then transcribed, and the transcripts were reviewed and coded. Three major themes were identified, which were the role of clinical pharmacists, interprofessional communication, and competency. The greatest challenge reported in this study was lack of consistent understanding of the role of clinical pharmacists which to some extent caused communication deficiencies and affected the level of involvement with multidisciplinary teams. Despite that, majority of the participants perceived pharmacists as beneficial in optimizing pharmacotherapy and improving quality of care. Clinical pharmacy services are perceived positively in impacting quality of care as expressed by majority of the study participants. However, there is a lack of common understanding of the role of clinical pharmacists by other health-care providers.
Patient-reported outcome instruments in clinical trials of systemic sclerosis
Authors: Pauling John D , Caetano Joana , Campochiaro Corrado , De Luca Giacomo , Gheorghiu Ana Maria , Lazzaroni Maria Grazia , Khanna Dinesh
Published: November 2019

Patient-reported outcome instruments provide valuable insight into disease-related morbidity known only to the patient and complement more objective outcome tools in the clinical trial setting. They are of particular importance in systemic sclerosis owing to the challenges around defining disease activity, the episodic nature of many disease-specific manifestations and the paucity of validated objective surrogate outcome measures for use in clinical trials. Early clinical trials of systemic sclerosis often incorporated legacy patient-reported outcome instruments, but the last 20 years has witnessed the emergence of several scleroderma-specific instruments that are now being routinely used alongside other outcomes in systemic sclerosis clinical trials. More recently, the value of patient-reported outcomes has been highlighted by their prominence in the American College of Rheumatology Combined Response Index for Systemic Sclerosis that has been utilized as the primary endpoint of recent clinical trials of early diffuse systemic sclerosis. This review considers the role and performance of the various patient-reported outcome instruments utilized in systemic sclerosis clinical trials, the current positioning of patient-reported outcome instruments within clinical trial endpoint models across the range of systemic sclerosis disease manifestations and, where applicable, we shall highlight areas for future research.
Sonographic Evaluation of Fetal Cardiac Anatomy in the Late First Trimester
Authors: Hildebrand Emily
Published: November 2019

Objective:

Congenital heart defects (CHDs) are the most common cause of infant death in the United States. Severe, complex cardiac anomalies make up approximately half of the cases and carry significant morbidity and mortality. The purpose of this literature review was to define a CHD screening protocol to be used in the first trimester.

Methods:

Literature was pulled that discussed the impact of color Doppler and spatiotemportal image correlation on image quality, explored the benefits and limitations of early screening, and analyzed the risk of bioeffects to the developing fetus.

Results:

A simplified cardiac screening protocol has been shown to be a feasible addition to the first trimester nuchal translucency scan.

Conclusion:

Color Doppler and three-dimensional sonography can be utilized during the first trimester to evaluate the fetal heart for major anomalies between 11 weeks and 13 weeks 6 days. Recommendations for future research and guidance from accrediting bodies are provided.
Impact of Arteriovenous fistula creation on estimated glomerular filtration rate decline in Predialysis patients
Authors: Bénard Valérie , Pichette Maude , Lafrance Jean-Philippe , Elftouh Naoual , Pichette Vincent , Laurin Louis-Philippe , Nadeau-Fredette Annie-Claire
Published: November 2019

Background

Arteriovenous fistula (AVF) is the vascular access of choice for patients on hemodialysis. Recent evidence suggests that AVF creation may slow estimated glomerular filtration rate (eGFR) decline. The study objective was to assess the impact of the AVF creation on eGFR decline, after controlling for key confounding factors.

Methods

This retrospective cohort study included adult patients followed in a single-center predialysis clinic between 1999 and 2016. Patients with a patent AVF were followed up to 2 years pre- and post-AVF creation. Estimated GFR trajectory was reported using linear mixed models adjusted for demographic characteristics, comorbidities and use of renin-angiotensin-aldosterone blockade.

Results

A total of 146 patients were studied with a median age 68.7 (60.5–75.4) years and a median eGFR at time of AVF creation of 12.8 (11.3–13.9) mL/min/1.73m2. The crude annual eGFR decline rates were − 3.60 ± 4.00 mL/min/1.73 m2 pre- and − 2.28 ± 3.56 mL/min/1.73 m2 post-AVF, resulting in a mean difference of 1.28 mL/min/1.73 m2 (95% CI 0.49, 2.07). In a mixed effect linear regression model, monthly eGFR decline was − 0.63 (95% CI -0.81, − 0.46; p <  0.001) mL/min/1.73m2/month. The period after AVF creation was associated with a relatively higher eGFR (β 0.94, 95% CI 0.61–1.26, p <  0.001). There was a significant association between follow-up time and the period pre/post AVF (β 0.19, 95% CI 0.16, 0.22; p <  0.001) such that eGFR decline was more attenuated each month after AVF creation.

Conclusions

In this cohort, AVF creation was associated with a significant reduction of eGFR decline. Further prospective studies are needed to confirm this association.
Two novel mutations in the ALPL gene of unrelated Chinese children with Hypophosphatasia: case reports and literature review
Authors: Mao Xiaojian , Liu Sichi , Lin Yunting , Chen Zhen , Shao Yongxian , Yu Qiaoli , Liu Haiying , Lu Zhikun , Sheng Huiyin , Lu Xinshuo , Huang Yonglan , Liu Li , Zeng Chunhua
Published: November 2019

Objective

Hypophosphatasia (HPP) is an inherited disorder of defective skeletal mineralization caused by mutations in the ALPL gene that encodes the Tissue Non-specific Alkaline Phosphatase (TNSALP). It is subdivided into six forms depending on the age of onset: perinatal lethal, prenatal benign, infantile, childhood, adult, and odonto HPP. Among these, infantile HPP is characterized by early onset and high frequency of lethal outcome. Few studies have reported the phenotype and genetic characteristics of HPP in Chinese children.

Case presentation

Three forms of HPP were identified in four unrelated patients from four different Chinese families, including one lethal infantile (patient 1), two childhood (patient 2 and 3) and one odonto HPP (patient 4). Six variants in the ALPL gene were identified, including five missense mutations and one frameshift mutation. Of which, none were reported previously in the Chinese population, and two were novel (c.359G > C: p.G120A and c.1017dupG: p.H340AfsX3). Patient 1 carrying a novel homozygous (c.359G > C) mutation showed respiratory distress and pneumonia at first day of his life. He presented nearly negligible level of serum ALP activity, overall skeletal hypominaralization and died at 3 months old. Patient 2, 3 and 4 were compound heterozygotes with decreased serum ALP activity. Patient 2 and 3 presented premature loss of deciduous teeth, muscle weakness and bone pain, whereas patient 4 had early loss of deciduous teeth only. All four pedigrees exhibited autosomal recessive pattern of inheritance.

Conclusions

In this study, six mutations in the ALPL gene were found in four Chinese HPP patients, two of which were novel: c.359G > C in exon 5 and c.1017dupG in exon 10. Our results strongly indicated that the novel mutation c.359G > C might be disease-causing and associated with severe infantile form of HPP.
Oral health of children and adolescents with or without attention deficit hyperactivity disorder (ADHD) living in residential care in rural Rhineland-Palatinate, Germany
Authors: Ehlers Vicky , Callaway Angelika , Wantzen Sophia , Patyna Michael , Deschner James , Azrak Birgül
Published: November 2019

Background

Attention deficit hyperactivity disorder (ADHD) is defined as childhood neurobehavioural disorder. Due to short attention span, oral hygiene and dental treatment of such individuals can be challenging. Aim of this study was to evaluate the oral health of children and adolescents with and without ADHD living in residential care in rural Rhineland-Palatinate, Germany.

Methods

Included in the study were 79 participants (male/female:58/21, age 9–15 years) living in residential care: 34 participants with ADHD and 45 participants without ADHD (control). Oral examination included the following parameters decayed, missing, filled teeth in the primary dentition (dmft), decayed, missing, filled surfaces/teeth in the secondary dentition (DMFS/DMFT), approximal plaque index (API), bruxism and orthodontic treatment. Additionally, oral hygiene, last dental visit and treatment performed, and dietary habits were assessed by questionnaire.

Results

There were no significant differences in dmft, API, bruxism and oral hygiene habits between groups. However, participants with ADHD tended to have higher DMFS/DMFT values than the control group. Ongoing orthodontic treatment was found more often in the control group. The ADHD group tended to consume acidic/sugary beverages and sweet snacks more often than the controls. Different treatments (control visit/prophylaxis, dental therapy, orthodontic treatment) were performed at the last dental visit in the two groups.

Conclusions

Within the limitations of this study,oral health was similar in children and adolescents with or without ADHD from the same residential care setting. Parents/guardians need instructions for better supervision of oral hygiene and dietary habits to improve the poor oral health of children with or without ADHD.
A bootstrapping approach for generating an inverse distance weight matrix when multiple observations have an identical location in large health surveys
Authors: Kim Sung Wook , Achana Felix , Petrou Stavros
Published: November 2019

Spatial weight matrices play a key role in econometrics to capture spatial effects. However, these constructs are prone to clustering and can be challenging to analyse in common statistical packages such as STATA. Multiple observations of survey participants in the same location (or cluster) have traditionally not been dealt with appropriately by statistical packages. It is common that participants are assigned Geographic Information System (GIS) data at a regional or district level rather than at a small area level. For example, the Demographic Health Survey (DHS) generates GIS data at a cluster level, such as a regional or district level, rather than providing coordinates for each participant. Moreover, current statistical packages are not suitable for estimating large matrices such as 20,000 × 20,000 (reflective of data within large health surveys) since the statistical package limits the N to a smaller number. In addition, in many cases, GIS information is offered at an aggregated level of geographical areas. To alleviate this problem, this paper proposes a bootstrap approach that generates an inverse distance spatial weight matrix for application in econometric analyses of health survey data. The new approach is illustrated using DHS data on uptake of HIV testing in low and middle income countries.
The impact of endometrial thickness change after progesterone administration on pregnancy outcome in patients transferred with single frozen-thawed blastocyst
Authors: Bu Zhiqin , Yang Xinhong , Song Lin , Kang Beijia , Sun Yingpu
Published: November 2019

Background

The aim of this study was to explore the impact of endometrial thickness change after progesterone administration on pregnancy outcome in patients transferred with single frozen-thawed blastocyst.

Methods

This observational cohort study included a total of 3091 patients undergoing their first frozen-thawed embryo transfer (FET) cycles between April 2015 to March 2019. Endometrial thickness was measured by trans-vaginal ultrasound twice for each patient: on day of progesterone administration, and on day of embryo transfer. The change of endometrial thickness was recorded.

Results

Regardless of endometrial preparation protocol (estrogen-progesterone/natural cycle), female age, body mass index (BMI), and infertility diagnosis were comparable between patients with an increasing endometrium on day of embryo transfer and those without. However, clinical pregnancy rate increases with increasing ratio of endometrial thickness. Compared with patients with Non-increase endometrium, those with an increasing endometrium on day of embryo transfer resulted in significantly higher clinical pregnancy rate (56.21% vs 47.13%, P = 0.00 in estrogen-progesterone cycle; 55.15% vs 49.55%, P = 0.00 in natural cycle).

Conclusions

In most patients, endometrial thickness on day of embryo transfer (after progesterone administration) increased or kept being stable compared with that on day of progesterone administration. An increased endometrium after progesterone administration was associated with better pregnancy outcome.
The pathogenesis of renal injury and treatment in light chain deposition disease
Authors: Wang Qi , Jiang Fang , Xu Gaosi
Published: November 2019

Light chain deposition disease (LCDD) is a rare clinical disorder. The deposition of light chain immunoglobulins mainly affects the kidneys, which have different characteristics than other tissues. To date, the therapeutic approach for the treatment of LCDD has no evidence-based consensus, and clinical experience of reported cases guides current disease management strategies. The present systematic review investigates and summarizes the pathological mechanisms of renal injury and the subsequent treatments for LCDD.
Simultaneous, radiation-free registration of the dentoalveolar position and the face by combining 3D photography with a portable scanner and impression-taking
Authors: Ritschl Lucas M. , Wolff Klaus-Dietrich , Erben Pia , Grill Florian D.
Published: November 2019

Objectives

Simultaneous, radiation-free registration of the teeth and the upper and lower jaw positions in relation to the extraoral soft tissue could improve treatment planning and documentation. The purpose of this study is to describe a workflow to solve this form of registration and surface acquisition with a mobile device.

Methods

Facial scans of ten healthy participants were taken using a blue-light LED 3D scanner (Artec® Space Spider; Artec® Group; Luxembourg). An impression of the maxillary dentoalveolar arch was taken simultaneously to the 3D photo using a modified impression tray with two different extraoral registration geometries (sphere vs. cross). Following, an impression of the mandibular dentoalveolar arch was taken once. Both impressions were scanned with the 3D scanner. All resulting standard tesselation language (.stl) files of the geometries were compared to the original, virtual .stl files and the root mean square errors (RMSE) were calculated for each surface (Artec Studio 13 Professional × 64; Artec® Group; Luxembourg) to determine which geometry serves as a better reference for intra-extraoral registration.

Results

The RMSE between the original geometries and the scanned counterfeits were statistically lower for spherical geometries (p < 0.008). Once scanned and aligned, both geometries enabled an alignment of the intra- and extraoral scan. However, the spherical geometries showed virtually better results without significance (p = 0.70).

Conclusions

The presented study provides a radiation-free solution for simultaneous dentoalveolar correlations in relation to the extraoral soft tissue. Spherical geometries achieved more precise and easier intra-extraoral alignments using the applied mobile 3D scanner and workflow.
FGF-induced LHX9 regulates the progression and metastasis of osteosarcoma via FRS2/TGF-β/β-catenin pathway
Authors: Li Shuang-Qing , Tu Chao , Wan Lu , Chen Rui-Qi , Duan Zhi-Xi , Ren Xiao-Lei , Li Zhi-Hong
Published: November 2019

Background

Fibroblast growth factor (FGF) and tumor growth factor-β (TGFβ) have emerged as pivotal regulators during the progression of osteosarcoma (OS). LHX9 is one crucial transcription factor controlled by FGF, however, its function in OS has not been investigated yet.

Methods

The expression of LHX9, FRS2, BMP4, TGF-beta R1, SMAD2, beta-catenin and metastasis-related proteins was measured by real-time quantitative PCR (RT-qPCR) and Western blot. CCK-8 assay and colony formation assay were employed to determine the proliferation of OS cells, while scratch wound healing assay and transwell assay were used to evaluate their migration and invasion, respectively. In vivo tumor growth and metastasis were determined by subcutaneous or intravenous injection of OS cells into nude mice.

Results

LHX9 expression was evidently up-regulated in OS tumor tissues and cell lines. Knockdown of LHX9 impaired the proliferation, migration, invasion and metastasis of OS cells. Mechanistically, LHX9 silencing led to the down-regulation of BMP-4, β-catenin and metastasis-related proteins, which was also observed in beta-catenin knockdown OS cells. By contrast, FRS2 knockdown conduced to the up-regulation of LHX9, BMP4, β-catenin and TGF-βR1, while TGF-beta inhibition repressed the expression of LHX9 and metastasis-related proteins. Additionally, let-7c modulates LHX9 and metastasis-related proteins by suppressing TGF-beta R1 expression on transcriptional level.

Conclusions

This study revealed LHX9 was essential for the proliferation, migration, invasion, and metastasis of OS cells via FGF and TGF-β/β-catenin signaling pathways.
Copy number variations in candidate genomic regions confirm genetic heterogeneity and parental bias in Hirschsprung disease
Authors: Lantieri Francesca , Gimelli Stefania , Viaggi Chiara , Stathaki Elissavet , Malacarne Michela , Santamaria Giuseppe , Grossi Alice , Mosconi Manuela , Sloan-Béna Frédérique , Prato Alessio Pini , Coviello Domenico , Ceccherini Isabella
Published: November 2019

Background

Hirschsprung Disease (HSCR) is a congenital defect of the intestinal innervations characterized by complex inheritance. Many susceptibility genes including RET, the major HSCR gene, and several linked regions and associated loci have been shown to contribute to disease pathogenesis. Nonetheless, a proportion of patients still remains unexplained. Copy Number Variations (CNVs) have already been involved in HSCR, and for this reason we performed Comparative Genomic Hybridization (CGH), using a custom array with high density probes.

Results

A total of 20 HSCR candidate regions/genes was tested in 55 sporadic patients and four patients with already known chromosomal aberrations. Among 83 calls, 12 variants were experimentally validated, three of which involving the HSCR crucial genes SEMA3A/3D, NRG1, and PHOX2B. Conversely RET involvement in HSCR does not seem to rely on the presence of CNVs while, interestingly, several gains and losses did co-occur with another RET defect, thus confirming that more than one predisposing event is necessary for HSCR to develop. New loci were also shown to be involved, such as ALDH1A2, already found to play a major role in the enteric nervous system. Finally, all the inherited CNVs were of maternal origin.

Conclusions

Our results confirm a wide genetic heterogeneity in HSCR occurrence and support a role of candidate genes in expression regulation and cell signaling, thus contributing to depict further the molecular complexity of the genomic regions involved in the Enteric Nervous System development. The observed maternal transmission bias for HSCR associated CNVs supports the hypothesis that in females these variants might be more tolerated, requiring additional alterations to develop HSCR disease.