bluuebunny/crossref_metadata_2025 huggingface dataset:
Assessing Children’s Credibility in Courtroom Investigations of Alleged Child Sexual Abuse: Suggestibility, Plausibility, and Consistency
Authors: Denne Emily , Sullivan Colleen , Ernest Kyle , Stolzenberg Stacia N.
Published: September 2019

As children’s testimonies of child sexual abuse (CSA) often lack concrete evidence to corroborate a child’s claims, attorneys devote a substantial amount of time to establishing a child as credible during the course of a trial. Examining 134 CSA victim testimonies for children aged 5–17 ( M = 12.48, SD = 3.34; 90% female), we explored how attorneys assess child credibility through specifically targeting children’s suggestibility/honesty, plausibility, and consistency. Results revealed that while prosecutors examine plausibility more often to establish credibility, defense attorneys focus their assessments on suggestibility/honesty and potential inconsistency. However, both attorneys asked many more questions about children’s consistency than any other area of potential credibility. Furthermore, while prosecutors ask proportionally more credibility-challenging questions of older children, the defense do not. These results suggest that prosecutors may be missing an opportunity to establish children as honest and consistent and elucidate a need to train attorneys on the implications of children’s inconsistencies, suggestibility, and plausible abuse dynamics.
Uncompensated Care and the Collapse of Hospital Payment Regulation: An Illustration of the Tinbergen Rule
Authors: Clemens Jeffrey , Ippolito Benedic
Published: September 2019

The primary objective of “all-payer” rate setting—regulatory regimes through which states set hospital payment rates for all insurers—was to control costs through consistent, centrally regulated payments. These regimes were often linked, however, to an ancillary goal of financing care for the uninsured. We show that the surcharge mechanism used to achieve this secondary objective decreased the stability of these payment regimes. This instability reflected a feedback loop from surcharge rates to insurance coverage and back to the quantities of uncompensated care in need of financing. Instability was exacerbated when Health Maintenance Organizations were exempted from surcharge collections, creating a regulatory arbitrage opportunity. Legal challenges connected to the incidence of uncompensated care surcharges contributed to the abandonment of all-payer rate regulation by several states. These developments illustrate the wisdom of the Tinbergen Rule, which recommends that independent policy objectives be met with independent policy instruments.
Intracameral bevacizumab role in trabeculectomy: A 1-year prospective randomized controlled study
Authors: Rabina Gilad , Barequet Dana , Mimouni Michael , Kurtz Shimon , Shemesh Gabi , Rosenblatt Amir , Rosenfeld Eldar
Published: September 2019

Purpose:

To evaluate the effect of intracameral Bevacizumab on trabeculectomy success rates.

Methods:

A prospective, randomized, interventional clinical trial. Patients with primary open-angle glaucoma were randomly assigned to two groups: trabeculectomy with mitomycin C and trabeculectomy with mitomycin C and intracameral bevacizumab. Complete success is defined as an intraocular pressure (IOP) reduction of at least 30% from baseline IOP to a measured pressure of between 5 and 18 mm Hg without the use of IOP lowering medications. Qualified success is defined as same achievement of reduced IOP, but with the use of IOP lowering medications. Overall success is defined as same achievement of reduced IOP with or without the use of IOP lowering medications.

Results:

Thirty-three patients in the mitomycin C group and 36 patients in the mitomycin C and bevacizumab group were included in final analyses. The IOP at presentation was 28.3 ± 8 and 28.4 ± 8.6 mm Hg, compared to 10.8 ± 3.4 and 12.3 ± 3.7 mm Hg at 12 months (p < 0.0001) for the mitomycin C group and the mitomycin C and bevacizumab group, respectively. Complete success at 12 months was achieved in 65% of the mitomycin C group compared to 60% of the mitomycin C and bevacizumab group (p = 0.77). Overall success was achieved in 82% compared to 80% of patients at 12 months (p = 0.78). Both groups showed a statistically significant reduction in IOP after 6 and 12 months (p ⩽ 0.001). There were no statistically significant differences in visual acuity and complications.

Conclusion:

Intracameral bevacizumab during mitomycin C trabeculectomy in patients with primary open-angle glaucoma apparently does not improve success rates. The adjuvant use of intracameral bevacizumab is therefore not justified.
Jury verdicts and outcomes of malpractice cases involving arteriovenous hemodialysis access
Authors: Phair John , Carnevale Matthew , Wilson Eelin , Koleilat Issam
Published: September 2019

Objective:

To analyze malpractice cases involving hemodialysis access to prevent future litigation and improve physician education.

Methods:

Jury verdict reviews from the WESTLAW database from 1 January 2005 to 1 January 2015 were reviewed. The search terms “hemodialysis,” “dialysis,” “graft,” “fistula,” “AVG,” “AVF,” “arteriovenous,” “catheter,” “permacatheter,” and “shiley” were used to compile data on the demographics of the defendant, plaintiff, allegation, complication, and verdict.

Results:

Sixty-six cases involving the litigation pertaining to hemodialysis catheter, arteriovenous fistula (AVF) or arteriovenous grafts (AVGs) were obtained. Of these, 55% involved catheter-based hemodialysis access, 18% involved AVF, and 27% involved AVG. The most frequent physician defendants were vascular surgeons (36%), internists (14%), nephrologists (14%), general surgeons (9%), and interventional radiologists (6%). Of the patients, 38% involved were male and the average patient age was 56.3 (standard deviation (SD) = 20.1) years. Region of injury was 50% in the neck or chest, 42% in the arm, and 8% in the groin. Injury was listed as death in 79% of cases. Of the deaths, 95% involved bleeding at some point in the chain of events. The most common claims related to the cases were failure to perform the surgery or procedure safely (44%), failure to diagnose and treat in a timely manner (30%), and negligent hemodialysis treatment (11%). The most common complications cited were hemorrhage (62%), loss of function of limb (15%), and ischemia due to steal syndrome (11%). A total of 26 cases (39%) were found for the plaintiff or settled. The median award was US$463,000 with a mean of US$985,299 (SD = US$1,314,557).

Conclusion:

While popular opinion may indicate that steal syndrome is a commonly litigated complication, our data reveal that the most common injury litigated is death which may frequently be the result of a hemorrhagic episode. In addition to hemorrhage, the remaining most common complications included steal syndrome and loss of limb function. Therefore, steps to better prevent, diagnose and treat bleeding, nerve injury, and steal syndrome in a timely manner are critical to preventing hemodialysis-access-associated litigation.
Hemodialysis access cost comparisons among incident tunneled catheter patients
Authors: Wagner Jason Kane , Fish Larry , Weisbord Steven D , Yuo Theodore H
Published: September 2019

Background:

Arteriovenous fistula is the ideal hemodialysis access, but most patients start with tunneled dialysis catheter. Arteriovenous fistula and arteriovenous graft surgery may reduce tunneled dialysis catheter use and also increase procedural expenses. We compared Medicare costs associated with arteriovenous fistula, arteriovenous graft, and tunneled dialysis catheter.

Methods:

Using the US Renal Data System, we identified incident hemodialysis patients in 2008 who started with tunneled dialysis catheter, survived at least 90 days, and had adequate Medicare records for analysis. We followed them until death or end of 2011; access modality was based on billing evidence of arteriovenous fistula or arteriovenous graft creation. We assumed patients without such records remained with tunneled dialysis catheter. We generated multivariate linear regression models predicting Medicare expenditures, censoring costs when patients died; we included all payments to physicians and institutions. We also created algorithms to identify access-related costs.

Results:

There were 113,505 patients in the US Renal Data System who started hemodialysis in 2008, of whom 51,002 Medicare patients met inclusion criteria. Of that group, 41,532 (81%) began with tunneled dialysis catheter; 27,064 patients were in the final analysis file. In the first 90 days after hemodialysis initiation, 6100 (22.5%) received arteriovenous fistula, 1813 (6.7%) arteriovenous graft, and 19,151 (70.8%) stayed with tunneled dialysis catheter. Annualized access costs by modality were tunneled dialysis catheter US$13,625 (95% confidence interval: US$13,426–US$13,285); arteriovenous fistula US$16,864 (95% confidence interval: US$16,533–US$17,194); and arteriovenous graft US$20,961 (95% confidence interval: US$20,967–US$21,654; p < .001). Multivariate linear regression demonstrated that staying with tunneled dialysis catheter had lowest access-related costs, arteriovenous fistula was intermediate, and those who underwent arteriovenous graft surgery were highest (p < .021). Access type was not significantly associated with total costs. Additional arteriovenous fistula and arteriovenous graft creation (US$3525 and US$3804 per access per year, respectively) and open and endovascular access-related interventions (US$3102 and US$3569 per procedure per year, respectively; all p < .001) were important predictors of increased cost.

Conclusions:

Among patients starting hemodialysis with tunneled dialysis catheter, continued tunneled dialysis catheter use is associated with lowest access-related cost. Both endovascular and open interventions are associated with significant additional costs. Further investigation is warranted to develop efficient patient-centered strategies for hemodialysis access.
Controlled networking: Organizational cohesion and programmatic coherence of Swiss parties on Twitter
Authors: Wüest Bruno , Mueller Christian , Willi Thomas
Published: September 2019

Political parties are under increasing pressure to extend and activate their voter bases by employing more innovative communication strategies. This article focuses on the social media platform Twitter to explore how well Swiss parties performed in terms of employing digital communication during the 2015 federal election campaign. As such, it uses the follower network as an indicator of organizational cohesion, along with two indicators of programmatic coherence based on Twitter message content. Computing density and centrality statistics allow for the quantification of these two aspects in the party networks, while the nonparametric bootstrap introduces uncertainty of the account sampling process into the analysis. Our results suggest that smaller and newer parties, as well as the Social Democrats, tend to exhibit disproportionally high levels of organizational cohesion. At the same time, most parties show comparable—and also disproportionately low—levels of programmatic coherence compared to those displayed by the Social Democrats.
Continuous versus intermittent aerobic exercise in the improvement of quality of life for women with polycystic ovary syndrome: A randomized controlled trial
Authors: Ribeiro Victor Barbosa , Lopes Iris Palma , dos Reis Rosana Maria , Silva Rafael Costa , Mendes Maria Célia , Melo Anderson Sanches , de Souza Hugo Celso Dutra , Ferriani Rui Alberto , Kogure Gislaine Satyko , Lara Lúcia Alves da Silva
Published: September 2019

Polycystic ovary syndrome predisposes alterations which contribute to the reduction of quality of life. This randomized controlled clinical trial study was to evaluate the effect of two protocols of aerobic exercise on quality of life in women with polycystic ovary syndrome. Women were allocated to three groups: continuous aerobic training ( n = 28), intermittent aerobic training ( n = 29), and control group (no training; n = 30). Testosterone levels, body composition indices, and quality of life were assessed at baseline and after 16 weeks of intervention. Both protocols were effective to improve testosterone levels, anthropometric indices, and quality of life in polycystic ovary syndrome women. Thus, these protocols should be included in the clinical environment to improve clinical parameters psychological, biological and social health to this population.
Spousal influence: A study of women with eating and body image concerns
Authors: Birmingham Wendy C , Cavallini Adriane Q , Sgro Jordan
Published: September 2019

Eating disorder literature often overlooks those exhibiting eating and body image concerns without an eating disorder diagnosis. Supportive spouses may ameliorate negative body image and eating behavior, but spouses who exhibit both supportive and non-supportive behaviors concurrently (ambivalent) may send mixed messages. Eating disorder behaviors and spousal interactions were assessed in 61 women who demonstrated eating disordered behavior and body dissatisfaction but were not clinically diagnosed with an eating disorder. Spouses mostly provided messages of reassurance. However, some women were unable to overcome their internalized negative body image. Feelings of social comparison were seen with ambivalent spouses. Supportive relationships may be protective, but actual interventions may be needed to change negative body image.
Paediatric nurses’ knowledge and experience of autism spectrum disorders: An Italian survey
Authors: Corsano Paola , Cinotti Mariaelena , Guidotti Laura
Published: September 2019

Hospitalization of children with autism spectrum disorders (ASDs) is a challenge for paediatric nurses. The literature reveals a lack of knowledge among the clinical and behavioural characteristics of autism. The aim of the study is to investigate the knowledge of paediatric nurses about ASDs and their experience with children with ASD. Ninety-three nurses completed Knowledge About Childhood Autism Among Healthcare Workers questionnaire, a sociodemographic questionnaire and an open-ended questionnaire to evaluate the experience of nurses. Findings showed that nurses have a basic knowledge of ASD, except for comorbidity and onset of the ASD, in which they obtained lower scores. Older nurses and those who had more experience in a paediatric ward or with children with ASD showed higher scores. Analysis of the content of open-ended questionnaire showed that nurses have difficulties in managing the relationship with children with ASD, and that they wonder about how to improve it, seeking useful strategies for this purpose. During interactions, nurses experience mixed feelings such as sadness, suffering, uneasiness, inadequacy, displeasure, embarrassment and tenderness. They declare the need for more knowledge about ASD. They would like to improve their own capacity for interactions with these children, also through training.
Incredible years for whom? Governing early years parenting as a site of colonisation in Aotearoa New Zealand
Authors: Bae Shil
Published: September 2019

This article conducts a critical analysis of the Incredible Years parenting programme through the lens of post-colonial and post-structural theories. Drawing from Foucault’s concept of ‘governmentality’ and ‘discursive normalisation’, the author questions the norms and definitions constructed by the implementation of Incredible Years in New Zealand, and attempts to disrupt taken-for-granted values and assumptions in modern parenting. The analysis of this study shows that the discourses in Incredible Years (re)produce colonising values and assumptions, reinforcing the privileged knowledge of the West in parenting. The author points out how this approach to parenting constructs those who do not fit into the norm as ‘the Other’ and normalises/reinforces conformity to the dominant culture in this context.
Climate change in China: A study of news diversity in party-sponsored and market-oriented newspapers
Authors: Duan Ran , Miller Serena
Published: September 2019

Chinese news publications potentially play a crucial role in mitigating global climate change. Presently, the majority of scholars treat the Chinese news media system as one single entity. We expect, however, Chinese party-sponsored and market-oriented newspapers differ in their representation of climate change. Using the conceptual framework of news diversity, we examined how Chinese journalists reported on climate change by examining their use of media frames, source types, and multiple viewpoints in news articles. The results revealed that market-oriented newspapers were indeed significantly different by including more diverse viewpoints, conflict frames, and environmental nongovernmental organizational sources, while party-sponsored newspapers employed more domestic political, science, and scientific uncertainty frames. The results suggest that researchers should be cautious about generalizing past findings to the entire Chinese media ecosystem because it is unique, diverse, and complex.
Balancing personal wishes and caring capacity in future planning for adults with an intellectual disability living with family carers
Authors: McCausland Darren , Brennan Damien , McCallion Philip , McCarron Mary
Published: September 2019

Most people with intellectual disabilities (IDs) live at home with family, and most carers and care recipients wish to continue this arrangement. However, despite worry about what will happen when carers are unable to continue caring, most families do not plan for the future. The Future Care Road Map (FCRM) pilot study sought to enhance future care planning for families of adults with ID. Twelve families were facilitated through the FCRM process to establish future care plans, including future living and care arrangements. The pilot successfully improved future planning, but readiness of families and facilitation were important to outcomes. Continued care within the family was the most common future wish of care recipients. However, caring capacity emerged as a pervasive theme. Ultimately, it may be the capacity of the family, services or individual with ID themselves to provide future care which determines how feasibly future wishes translate into future plans.
Civilianising specialist units: Reflections on the policing of cyber-crime
Authors: Whelan Chad , Harkin Diarmaid
Published: September 2019

Civilianisation refers to utilising non-sworn personnel to perform certain roles within police organisations. While the civilianisation of policing has been examined in a variety of contexts, it has generally been in relation to attempts to improve police efficiency. The current literature is much less focused on efforts to intentionally seek out civilians to improve police effectiveness, which, we suggest, is likely to apply in the case of police responses to cyber-crime. Using empirical data collected with three specialist cyber-crime units in Australia, we explore the arguments for and against civilianising cyber-crime units as a strategy to improve police capacity, as reported by police and civilian members of these units. We consider these arguments in relation to a broader debate as to whether it is better to improve police capacity by employing civilian experts or attempt to develop greater expertise on cyber-crime among police.
Prophylaxis for postoperative atrial fibrillation: A quality initiative study exploring adherence to NICE guidance in a UK tertiary cardiothoracic intensive care unit
Authors: Zochios Vasileios , Chandan Joht Singh , Taverner Thomas , Babu Aswin , Singh Harjot
Published: September 2019

Background

Acute onset atrial fibrillation is a common dysrhythmia experienced by patients following cardiac surgery which can often cause morbidity and extended hospital length of stay. The primary aim of the study was to explore adherence to National Institute for Health and Care Excellence (NICE) guidance which suggests the need for prophylaxis for postoperative atrial fibrillation (POAF). Secondary aims were to explore factors contributing to the development POAF and the impact of POAF on patient-centred outcomes.

Methods

An analysis consisting of descriptive statistics and regression models was conducted using 138 patient’s records who underwent cardiac surgery between January and March 2017.

Results

We identified 83 (62%) patients on prophylactic rate control medications prior to surgery. During the study period, a total of 50 patients (36%) developed POAF, of which 28 were on prophylactic medication prior to surgery. Patients who developed POAF had significantly prolonged hospital length of stay compared to those who did not develop POAF.

Conclusion

Our study identified a significant proportion of patients not being offered prophylactic rate control prior to cardiac surgery. It is clear that poor patient outcomes are associated with the development of POAF and therefore there is an important need to ensure preventative measures are implemented in guidance relating to the management of these patients. Our results also suggest that tight management of clinical and physiological risk factors prior and during cardiac surgery may improve outcomes in this group of patients and could be considered in future enhanced recovery after cardiac surgery protocols.
Mostly Vegetarian, But Flexible About It: Investigating How Meat-Reducers Express Social Identity Around Their Diets
Authors: Rosenfeld Daniel L. , Rothgerber Hank , Tomiyama A. Janet
Published: September 2019

Beyond indicating that one does not eat meat, the decision to identify as vegetarian signals social identity. Yet many people limit their meat intake without giving up meat entirely: These people are called flexitarians (a term combining the words, “flexible” and “vegetarian”). Some flexitarians, despite eating meat, consider themselves to be vegetarian. Through a preregistered study ( N = 837), we investigated how flexitarians express social identity around their diets—namely, how they self-identify on a continuous scale ranging from meat-eater (i.e., omnivorous) to vegetarian. Over and above actual eating behavior, two psychosocial variables emerged as significant predictors of flexitarians’ levels of vegetarian identification: the centrality of meat-reduced dieting to their identity and their beliefs about carnism (the ideology of eating animals). These results suggest that greater consideration of meat-reduced eating behaviors offers promise for elucidating the intersections of social identity and moral judgment.
Playing the long surveillance ball game: Metachronous testicular tumor developing after treatment and remission of extragonadal germ cell tumor
Authors: Chow Alexander K , Hoeksema Jerome , Wang Dian
Published: September 2019

A 32-year-old man with vague abdominal pain was found to have enlarged para-aortic and mediastinal lymph nodes on computed tomography. He was diagnosed with retroperitoneal mixed germ cell tumor as confirmed on percutaneous biopsy. At the time of diagnosis, lactic dehydrogenase, human beta-chorionic gonadotropin, and alpha-fetoprotein were elevated. He completed four cycles of platinum-based chemotherapy with excellent response and no clinical disease progression. Three years later, he presented to the Urology clinic with a right testicular mass. His tumor markers remained negative. He was taken for a right radical orchiectomy with the pathology resulting in pure seminoma (pT1Nx).
Long‐term results of the DelIVery for Pulmonary Arterial Hypertension trial
Authors: Gomberg‐Maitland Mardi , Bourge Robert C. , Shapiro Shelley M. , Tarver James H. , Zwicke Dianne L. , Feldman Jeremy P. , Chakinala Murali M. , Frantz Robert P. , Torres Fernando , Bag Remzi , Murphy Jeffrey A. , Lautenbach Amy A. , Morris Marty , Peterson Leigh , Waxman Aaron B.
Published: September 2019

Background

The DelIVery for Pulmonary Arterial Hypertension clinical trial was a multi‐center, prospective, single arm, Investigational Device Exemption study utilizing a fully implantable, programmable intravascular delivery system consisting of a pump and a catheter for intravenous treprostinil. The study met its primary endpoint and demonstrated that the intravascular delivery system significantly reduced catheter related complications at 22,000 subject‐days of follow‐up compared with a predefined objective performance criterion. Here we summarize the results obtained during a 6.4‐year follow‐up period.

Methods

Throughout study follow‐up, participants had clinic visits and medication refills at least every 12 weeks (dependent on the subjects’ dose). All adverse events and intravascular delivery system complications were evaluated and recorded.

Results

Sixty pulmonary arterial hypertension subjects were followed post device implantation for approximately 282 patient‐years (range 87 days to 6.4 years). Of the 60 subjects, 14 died (1 related to intravascular delivery system pump failure), 2 withdrew after lung transplants, and 2 withdrew due to pump pocket infection. No catheter‐related bloodstream infections, catheter thrombosis or occlusions, or catheter kinks occurred through 282 patient‐years. Two participants had adverse events of abdominal pain, rash, due to subcutaneous treprostinil “leaks” after one catheter puncture and one catheter laceration during pump refill and replacement, respectively. Eight pump failure events occurred: seven pump motor stalls and one early replacement (faulty battery).

Conclusion

Delivery of treprostinil with an intravascular delivery system is a safe alternative to an external delivery system, while providing enhanced life experiences. To preserve the risk–benefit ratio, treatment at specialized pulmonary arterial hypertension centers is recommended until training is disseminated at other sites.
Engineered landscapes of the southern Murray–Darling Basin: Anthropocene archaeology in Australia
Authors: Davies Peter , Lawrence Susan
Published: September 2019

Human activities over the past 200 years have fundamentally transformed the shape of Australia’s southern Murray–Darling Basin. The arrival of British colonists in the 19th century disrupted millennia of human management of the region and brought widespread changes to biota and soils. The subsequent development of mining, transport and irrigation infrastructure re-engineered the region’s landscapes to meet human objectives and ambitions. This article offers an integrated regional history of anthropogenic change across the southern Murray–Darling Basin, identifying historical processes driving complex ongoing interactions between human activities and the natural environment. We examine three broad domains of engineering and geo-disturbance in the region, including the development of transport corridors, micro- and macro-scale water management and landforms remade by erosion and sedimentation. We use the archaeology of the recent past to integrate insights drawn from physical geography, fluvial geomorphology and related research into the enduring landscape changes of modern Australia’s food bowl.
Modified Single-Patch versus Two-Patch Repair for Atrioventricular Septal Defect: A Systematic Review and Meta-Analysis
Authors: Loomba Rohit S. , Flores Saul , Villarreal Enrique G. , Bronicki Ronald A. , Anderson Robert H.
Published: September 2019

Background:

We performed a meta-analysis of studies to determine whether the modified single-patch technique offers benefits when compared to the two-patch repair. The postoperative outcomes examined in this study were cardiopulmonary bypass time, cross-clamp time, duration of mechanical ventilation, intensive care unit length of stay, total hospital length of stay, need for reoperation, need for reoperation for left ventricular outflow tract obstruction or left atrioventricular valve regurgitation, need for pacemaker implantation, and mortality during follow-up.

Methods:

A review was conducted to identify studies comparing a modified single-patch repair versus two-patch repair. A fixed-effects model was utilized for end points with low heterogeneity and a random-effects model for end points with significant heterogeneity. Meta-regression was also performed to determine the influence of other factors on the variables of interest.

Results:

A total of 964 unique manuscripts were screened, with 10 being included in the final analyses. There were a total of 724 patients, with 353 (49%) having undergone repair utilizing a modified single-patch repair. Mean age at repair for modified single-patch repair and two-patch repair was 8.81 and 9.03 months, respectively. Significant differences were noted in cardiopulmonary bypass time and cross-clamp time with mean difference of −28.53 and −22.69 minutes, respectively. In comparison to the two-patch repair, both times were decreased in modified single-patch repair. No significant difference was noted in any other variables.

Conclusions:

Modified single-patch repair for atrioventricular septal defects requires less cardiopulmonary bypass and cross-clamp time but does not significantly impact the examined postoperative outcomes.
Lecompte Procedure for Relief of Severe Airway Compression in Children With Congenital Heart Disease
Authors: Greene Christina L. , Mainwaring Richard D. , Sidell Douglas , Palmon Michal , Hanley Frank L.
Published: September 2019

Purpose:

Children with congenital heart disease may present with severe airway compression prior to any surgical procedure or may develop airway compression following their surgical procedure. This combination of congenital heart defect and airway compression poses a significant management challenge. The purpose of this study was to review our experience with the Lecompte procedure for relief of severe airway compression.

Methods:

This was a retrospective review of ten patients who underwent a Lecompte procedure for relief of severe airway compression over the past nine years (2010-2018). Three patients with absent pulmonary valve syndrome presented with severe symptoms prior to any surgical procedure. Seven patients presented with symptoms of airway compression following repair of their congenital heart defects (one with absent pulmonary valve syndrome, three patients had repair of pulmonary atresia with ventricular septal defect, and three patients had undergone aortic arch surgery). The median age at presentation was two years (range: one day to seven years).

Results:

The ten patients underwent a Lecompte procedure without any significant complications or operative mortality. The median interval between the surgical procedure and extubation was 9.5 days. No patients have required any further interventions for relief of airway obstruction.

Conclusions:

The Lecompte procedure is a surgical option for young children who present with severe airway compression. The patients in this series responded well to the Lecompte procedure as evidenced by clinical relief of airway compression.
Abnormal Pulmonary Artery Bending Correlates With Increased Right Ventricular Afterload Following the Arterial Switch Operation
Authors: Loke Yue-Hin , Capuano Francesco , Mandell Jason , Cross Russell R. , Cronin Ileen , Mass Paige , Balaras Elias , Olivieri Laura J.
Published: September 2019

Purpose:

In transposition of great arteries, increased right ventricular (RV) afterload is observed following arterial switch operation (ASO), which is not always related to pulmonary artery (PA) stenosis. We hypothesize that abnormal PA bending from the Lecompte maneuver may affect RV afterload in the absence of stenosis. Thus, we sought to identify novel measurements of three-dimensional cardiac magnetic resonance (CMR) images of the pulmonary arteries and compare with conventional measurements in their ability to predict RV afterload.

Methods:

Conventional measurements and novel measurements of the pulmonary arteries were performed using CMR data from 42 ASO patients and 13 age-matched controls. Novel measurements included bending angle, normalized radius of curvature ( Rc), and normalized weighted radius of curvature ( Rc- w). Right ventricular systolic pressures (as the surrogate for RV afterload) were measured by either recent echocardiogram or cardiac catheterization.

Results:

Conventional measurements of proximal PA size correlated with differential pulmonary blood flow ( r = 0.49, P = .001), but not with RV peak systolic pressures ( r = −0.26, P = .18). In ASO patients, Rc- w correlated with higher RV systolic pressures ( r = −0.57, P = .002). Larger neoaortic areas and rightward bending angles correlated with smaller right pulmonary artery Rc ( r = −0.48, P = .001; r = 0.41, P = .01, respectively). Finally, both pulmonary arteries had significantly smaller Rc compared to normal controls.

Conclusions:

Pulmonary arteries exhibit abnormal bends following ASO that correlate with increased RV afterload, independent of PA stenosis. Future work should focus on clinical and hemodynamic contributions of these shape parameters.
McCune-Albright Syndrome With Unremitting Hyperthyroidism at Early Age: Management Perspective for Early Thyroidectomy
Authors: Merchant Nadia , Viau-Colindres Johanna M. , Hicks Kelly A. , Balazs Andrea E. , Wesson David E. , Lopez Monica E. , Karaviti Lefkothea
Published: September 2019

Background. McCune-Albright syndrome (MAS) is characterized by hyperpigmented macules, endocrinopathies, and fibrous dysplasia. Hyperthyroidism is the second most common endocrinopathy in MAS and its management is challenging, particularly among infants and toddlers. Traditionally, young infants have been treated with antithyroid medications, but remission is likely and these medications have severe side effects and affect the control of other endocrinopathies. Thus, it is reasonable to consider permanent treatment options at an earlier age. In this article, we performed a retrospective chart review and describe 3 children who underwent thyroidectomy at an early age due to complex presentation. Case Descriptions. Case 1 was a female patient who underwent bilateral adrenalectomy due to adrenal hyperplasia and subsequently underwent thyroidectomy at 5 months of age due to unremitting hyperthyroidism with fibrous dysplasia, multiple fractures, and ovarian cysts with vaginal bleeding. Case 2 was a 20-month-old female on methimazole who acquired influenza A, precipitating a thyroid storm, and subsequently developed central precocious puberty. Case 3 was a 4-year-old female who underwent thyroidectomy because of unremitting hyperthyroidism after methimazole cessation due to declining neutrophils. All 3 children experienced no complications from thyroidectomy. Conclusions. Early thyroidectomy by an experienced surgeon is an option for managing MAS-associated hyperthyroidism, even in very young patients, with excellent results.
Organizational Readiness to Implement System Changes in an Alaskan Tribal Dental Care Organization
Authors: Randall C.L. , Hort K. , Huebner C.E. , Mallott E. , Mancl L. , Milgrom P. , Nelson L. , Senturia K. , Weiner B.J. , Cunha-Cruz J.
Published: September 2019

Introduction:

Tribal health care systems are striving to implement internal changes to improve dental care access and delivery and reduce health inequities for American Indian and Alaska Native children. Within similar systems, organizational readiness to implement change has been associated with adoption of system-level changes and affected by organizational factors, including culture, resources, and structure.

Objectives:

The objectives of this study were to assess organizational readiness to implement changes related to delivery of evidence-based dental care within a tribal health care organization and determine workforce- and perceived work environment–related factors associated with readiness.

Methods:

A 92-item questionnaire was completed online by 78 employees, including dental providers, dental assistants, and support staff (88% response rate). The questionnaire queried readiness for implementation (Organizational Readiness for Implementing Change), organizational context and resources, workforce issues, organizational functioning, and demographics.

Results:

Average scores for the change commitment and change efficacy domains (readiness for implementation) were 3.93 (SD = .75) and 3.85 (SD = .80), respectively, where the maximum best score was 5. Perceived quality of management, a facet of organizational functioning, was the only significant predictor of readiness to implement change (B = .727, SE = .181, P < .0002) when all other variables were accounted for.

Conclusion:

Results suggest that when staff members (including dentists, dental therapists, hygienists, assistants, and support staff) from a tribal health care organization perceive management to be high quality, they are more supportive of organizational changes that promote evidence-based practices. Readiness-for-change scores indicate an organization capable of institutional adoption of new policies and procedures. In this case, use of more effective management strategies may be one of the changes most critical for enhancing institutional behaviors to improve population health and reduce health inequities.

Knowledge Transfer Statement:

The results of this study can be used by clinicians and other leaders implementing changes within dental care organizations. To promote organizational readiness for change and, ultimately, more expedient and efficient adoption of system-level changes by stakeholders, consideration should be given to organizational functioning generally and quality of management practices specifically.
CD137 deficiency causes immune dysregulation with predisposition to lymphomagenesis
Authors: Somekh Ido , Thian Marini , Medgyesi David , Gülez Nesrin , Magg Thomas , Gallón Duque Alejandro , Stauber Tali , Lev Atar , Genel Ferah , Unal Ekrem , Simon Amos J. , Lee Yu Nee , Kalinichenko Artem , Dmytrus Jasmin , Kraakman Michael J. , Schiby Ginette , Rohlfs Meino , Jacobson Jeffrey M. , Özer Erdener , Akcal Ömer , Conca Raffaele , Patiroglu Türkan , Karakukcu Musa , Ozcan Alper , Shahin Tala , Appella Eliana , Tatematsu Megumi , Martinez-Jaramillo Catalina , Chinn Ivan K. , Orange Jordan S. , Trujillo-Vargas Claudia Milena , Franco José Luis , Hauck Fabian , Somech Raz , Klein Christoph , Boztug Kaan
Published: September 2019

Somekh and colleagues identify CD137, a member of the tumor necrosis factor superfamily, as a novel cause of immunodeficiency associated with a risk of autoimmunity and lymphoid malignancy.
Mechanism of platelet α-granule biogenesis: study of cargo transport and the VPS33B-VPS16B complex in a model system
Authors: Ambrosio Andrea L. , Di Pietro Santiago M.
Published: September 2019

Platelet α-granules play important roles in platelet function. They contain hundreds of proteins that are synthesized by the megakaryocyte or taken up by endocytosis. The trafficking pathways that mediate platelet α-granule biogenesis are incompletely understood, especially with regard to cargo synthesized by the megakaryocyte. Vacuolar-protein sorting 33B (VPS33B) and VPS16B are essential proteins for α-granule biogenesis, but they are largely uncharacterized. Here, we adapted a powerful method to directly map the pathway followed by newly synthesized cargo proteins to reach α-granules. Using this method, we revealed the recycling endosome as a key intermediate compartment in α-granule biogenesis. We then used CRISPR/Cas9 gene editing to knock out VPS33B in pluripotent stem cell–derived immortalized megakaryocyte cells (imMKCLs). Consistent with the observations in platelets from patients with VPS33B mutation, VPS33B-knockout (KO) imMKCLs have drastically reduced levels of α-granule proteins platelet factor 4, von Willebrand factor, and P-selectin. VPS33B and VPS16B form a distinct and small complex in imMKCLs with the same hydrodynamic radius as the recombinant VPS33B-VPS16B heterodimer purified from bacteria. Mechanistically, the VPS33B-VPS16B complex ensures the correct trafficking of α-granule proteins. VPS33B deficiency results in α-granule cargo degradation in lysosomes. VPS16B steady-state levels are significantly lower in VPS33B-KO imMKCLs, suggesting that VPS16B is destabilized in the absence of its partner. Exogenous expression of green fluorescent protein–VPS33B in VPS33B-KO imMKCLs reconstitutes the complex, which localizes to the recycling endosome, further defining this compartment as a key intermediate in α-granule biogenesis. These results advance our understanding of platelet α-granule biogenesis and open new avenues for the study of these organelles.
Real-world experience of first-line afatinib in patients with EGFR-mutant advanced NSCLC: a multicenter observational study
Authors: Ho Gwo-Fuang , Chai Chee-Shee , Alip Adlinda , Wahid Mohd Ibrahim A. , Abdullah Matin Mellor , Foo Yoke-Ching , How Soon-Hin , Zaatar Adel , Lam Kai-Seng , Leong Kin-Wah , Low John-Seng-Hooi , Yusof Mastura Md , Lee Erica Chai-Yong , Toh Yok-Yong , Liam Chong-Kin
Published: September 2019

Background

This study aimed to evaluate the efficacy, side-effects and resistance mechanisms of first-line afatinib in a real-world setting.

Methods

This is a multicenter observational study of first-line afatinib in Malaysian patients with epidermal growth factor receptor (EGFR)-mutant advanced non-small cell lung cancer (NSCLC). Patients’ demographic, clinical and treatment data, as well as resistance mechanisms to afatinib were retrospectively captured. The statistical methods included Chi-squared test and independent t-test for variables, Kaplan-Meier curve and log-rank test for survival, and Cox regression model for multivariate analysis.

Results

Eighty-five patients on first-line afatinib from 1st October 2014 to 30th April 2018 were eligible for the study. EGFR mutations detected in tumors included exon 19 deletion in 80.0%, exon 21 L858R point mutation in 12.9%, and rare or complex EGFR mutations in 7.1% of patients. Among these patients, 18.8% had Eastern Cooperative Oncology Group performance status of 2–4, 29.4% had symptomatic brain metastases and 17.6% had abnormal organ function.

Afatinib 40 mg or 30 mg once daily were the most common starting and maintenance doses. Only one-tenth of patients experienced severe side-effects with none having grade 4 toxicities. The objective response rate was 76.5% while the disease control rate was 95.3%. At the time of analysis, 56 (65.9%) patients had progression of disease (PD) with a median progression-free survival (mPFS) of 14.2 months (95% CI, 11.85–16.55 months). Only 12.5% of the progressed patients developed new symptomatic brain metastases. The overall survival (OS) data was not mature. Thirty-three (38.8%) patients had died with a median OS of 28.9 months (95% CI, 19.82–37.99 months). The median follow-up period for the survivors was 20.0 months (95% CI, 17.49–22.51 months).

Of patients with PD while on afatinib, 55.3% were investigated for resistance mechanisms with exon 20 T790 M mutation detected in 42.0% of them.

Conclusions

Afatinib is an effective first-line treatment for patients with EGFR-mutant advanced NSCLC with a good response rate and long survival, even in patients with unfavorable clinical characteristics. The side-effects of afatinib were manageable and T790 M mutation was the most common resistance mechanism causing treatment failure.
Endoscopic ultrasound staging in patients with gastro-oesophageal cancers: a systematic review of economic evidence
Authors: Yeo Seow Tien , Bray Nathan , Haboubi Hasan , Hoare Zoe , Edwards Rhiannon Tudor
Published: September 2019

Background

The sensitivity of endoscopic ultrasound (EUS) in staging gastro-oesophageal cancers (GOCs) has been widely studied. However, the economic evidence of EUS staging in the management of patients with GOCs is scarce. This review aimed to examine all economic evidence (not limited to randomised controlled trials) of the use of EUS staging in the management of GOCs patients, and to offer a review of economic evidence on the costs, benefits (in terms of GOCs patients’ health-related quality of life), and economic implications of the use of EUS in staging GOCs patients.

Methods

The protocol was registered prospectively with PROSPERO (CRD42016043700; http://www.crd.york.ac.uk/PROSPERO/display_record.php?ID=CRD42016043700). MEDLINE (ovid), EMBASE (ovid), The Cochrane Collaboration Register and Library (including the British National Health Service Economic Evaluation Database), CINAHL (EBSCOhost) and Web of Science (Core Collection) as well as reference lists were systematically searched for studies conducted between 1996 and 2018 (search update 28/04/2018). Two authors independently screened the identified articles, assessed study quality, and extracted data. Study characteristics of the included articles, including incremental cost-effectiveness ratios, when available, were summarised narratively.

Results

Of the 197 articles retrieved, six studies met the inclusion criteria: three economic studies and three economic modelling studies. Of the three economic studies, one was a cost-effectiveness analysis and two were cost analyses. Of the three economic modelling studies, one was a cost-effectiveness analysis and two were cost-minimisation analyses. Both of the cost-effectiveness analyses reported that use of EUS as an additional staging technique provided, on average, more QALYs (0.0019–0.1969 more QALYs) and saved costs (by £1969–£3364 per patient, 2017 price year) compared to staging strategy without EUS. Of the six studies, only one included GOCs participants and the other five included oesophageal cancer participants.

Conclusions

The data available suggest use of EUS as a complementary staging technique to other staging techniques for GOCs appears to be cost saving and offers greater QALYs. Nevertheless, future studies are necessary because the economic evidence around this EUS staging intervention for GOCs is far from robust. More health economic research and good quality data are needed to judge the economic benefits of EUS staging for GOCs.

PROSPERO Registration Number

CRD42016043700.