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1.

Background

Residents face demanding situations on the job and have been found to perceive high levels of strain. Medical students also reported a high degree of strain and even depressive tendencies when entering their clinical rotations. The aim of this study was to explore the perceived strain of medical students from different undergraduate curricula and at different stages of academic advancement during different phases of an assessment simulating a resident’s first day in hospital.

Methods

Sixty-seven undergraduate medical students participated in the following three phases of the assessment in the role of a resident: a consultation hour with five simulated patients, a management phase with interprofessional contact, and a patient handover with a colleague. They completed the Strain Perception Questionnaire (STRAIPER) after each phase. Students from different undergraduate curricula (VI: vertically integrated, n =?35 versus non-VI: not vertically integrated, n =?26) and different academic advancement (semester 10, n =?26 versus final year, n =?41) were compared.

Results

All students showed the highest strain level after the management phase compared to the consultation hour and the handover. Medical students from a non-VI curriculum felt significantly more strain in the dimension of agitation (p <?.05) after the consultation hour compared to students from a VI curriculum and compared to the management phase and the handover. No significant difference in perceived strain was found between students from semester 10 compared to final year students.

Conclusions

During the consultation hour and the handover with a colleague medical students faced tasks which are familiar to them from undergraduate education. Their higher strain levels during the management phase might occur because they are confronted with unfamiliar tasks and decisions. Feeling responsible for the right actions in this phase of multitasking and professional interaction might have added to the strain students perceived during this phase. Patient management should be emphasized more in any type of undergraduate medical curriculum.
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2.

Background

One of the biggest challenges in the spirituality, religiosity, and health field is to understand how patients and physicians from different cultures deal with spiritual and religious issues in clinical practice.

Purpose

The present study aims to compare physicians’ perspectives on the influence of spirituality and religion (S/R) on health between Brazil, India, and Indonesia.

Method

This is a cross-sectional, cross-cultural, multi-center study carried out from 2010 to 2012, examining physicians’ attitudes from two continents. Participants completed a self-rated questionnaire that collected information on sociodemographic characteristics, S/R involvement, and perspectives concerning religion, spirituality, and health. Differences between physicians’ responses in each country were examined using chi-squared, ANOVA, and MANCOVA.

Results

A total of 611 physicians (194 from Brazil, 295 from India, and 122 from Indonesia) completed the survey. Indonesian physicians were more religious and more likely to address S/R when caring for patients. Brazilian physicians were more likely to believe that S/R influenced patients’ health. Brazilian and Indonesians were as likely as to believe that it is appropriate to talk and discuss S/R with patients, and more likely than Indians. No differences were found concerning attitudes toward spiritual issues.

Conclusion

Physicians from these different three countries had very different attitudes on spirituality, religiosity, and health. Ethnicity and culture can have an important influence on how spirituality is approached in medical practice. S/R curricula that train physicians how to address spirituality in clinical practice must take these differences into account.
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3.

Background

Comorbidities of cardiovascular diseases (CVDs), metabolic syndrome and autoimmune diseases with systemic inflammation are recent topics in medicine. Inflammatory skin diseases such as atopic dermatitis and psoriasis are an active source of diverse proinflammatory cytokines and chemokines, which are readily detectable in the circulation and are likely to be involved in developing comorbidities.

Evidence

Both atopic dermatitis and psoriasis are frequently comorbid with CVD, metabolic syndrome and autoimmune diseases, the consequence of which is called “inflammatory skin march”, “psoriatic march” or “march of psoriasis”.

Conclusion

In this review, we summarize the epidemiological evidence and pathogenetic concepts regarding inflammatory skin march in atopic dermatitis and psoriasis.
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4.

Background

Recent meta-analyses have found small-moderate positive associations between general performance in medical school and postgraduate medical education. In addition, a couple of studies have found an association between poor performance in medical school and disciplinary action against practicing doctors. The aim of this study was to examine if a sample of Danish residents in difficulty tended to struggle already in medical school, and to determine whether administratively observable performance indicators in medical school could predict difficulties in residency.

Methods

The study design was a cumulative incidence matched case–control study. The source population was all active specialist trainees, who were medical school graduates from Aarhus University, in 2010 to June 2013 in two Danish regions. Cases were doctors who decelerated, transferred, or dropped out of residency. Cases and controls were matched for graduation year. Medical school exam failures, grades, completion time, and academic dispensations as predictors of case status were examined with conditional logistic regression.

Results

In total 89 cases and 343 controls were identified. The total number of medical school re-examinations and the time it took to complete medical school were significant individual predictors of subsequent difficulties (deceleration, transferral or dropout) in residency whereas average medical school grades were not.

Conclusions

Residents in difficulty eventually reached similar competence levels as controls during medical school; however, they needed more exam attempts and longer time to complete their studies, and so seemed to be slower learners. A change from “fixed-length variable-outcome programmes” to “fixed-outcome variable-length programmes” has been proposed as a way of dealing with the fact that not all learners reach the same level of competence for all activities at exactly the same time. This study seems to support the logic of such an approach to these residents in difficulty.
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5.

Background

Medical students may not be able to identify the essential elements of situational awareness (SA) necessary for clinical reasoning. Recent studies suggest that students have little insight into cognitive processing and SA in clinical scenarios. Objective Structured Clinical Examinations (OSCEs) could be used to assess certain elements of situational awareness. The purpose of this paper is to review the literature with a view to identifying whether levels of SA based on Endsley’s model can be assessed utilising OSCEs during undergraduate medical training.

Methods

A systematic search was performed pertaining to SA and OSCEs, to identify studies published between January 1975 (first paper describing an OSCE) and February 2017, in peer reviewed international journals published in English. PUBMED, EMBASE, PsycINFO Ovid and SCOPUS were searched for papers that described the assessment of SA using OSCEs among undergraduate medical students. Key search terms included “objective structured clinical examination”, “objective structured clinical assessment” or “OSCE” and “non-technical skills”, “sense-making”, “clinical reasoning”, “perception”, “comprehension”, “projection”, “situation awareness”, “situational awareness” and “situation assessment”. Boolean operators (AND, OR) were used as conjunctions to narrow the search strategy, resulting in the limitation of papers relevant to the research interest. Areas of interest were elements of SA that can be assessed by these examinations.

Results

The initial search of the literature retrieved 1127 publications. Upon removal of duplicates and papers relating to nursing, paramedical disciplines, pharmacy and veterinary education by title, abstract or full text, 11 articles were eligible for inclusion as related to the assessment of elements of SA in undergraduate medical students.

Discussion

Review of the literature suggests that whole-task OSCEs enable the evaluation of SA associated with clinical reasoning skills. If they address the levels of SA, these OSCEs can provide supportive feedback and strengthen educational measures associated with higher diagnostic accuracy and reasoning abilities.

Conclusion

Based on the findings, the early exposure of medical students to SA is recommended, utilising OSCEs to evaluate and facilitate SA in dynamic environments.
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6.

Background

Dietary weight loss interventions most often result in weight loss, but weight maintenance on a long-term basis is the main problem in obesity treatment. There is a need for an increased understanding of the behaviour patterns involved in adopting a new dietary behavior and to maintain the behaviour over time.

Purpose

The purpose of this paper is to explore overweight and obese middle-aged women’s experiences of the dietary change processes when participating in a 2-year-long diet intervention.

Methods

Qualitative semi-structured interviews with 12 overweight and obese women (54–71 years) were made after their participation in a diet intervention programme. The programme was designed as a RCT study comparing a diet according to the Nordic nutrition recommendations (NNR diet) and a Palaeolithic diet (PD). Interviews were analysed according to Grounded Theory principles.

Results

A core category “Engagement phases in the process of a diet intervention” concluded the analysis. Four categories included the informants’ experiences during different stages of the process of dietary change: “Honeymoon phase”, “Everyday life phase”, “It’s up to you phase” and “Crossroads phase”. The early part of the intervention period was called “Honeymoon phase” and was characterised by positive experiences, including perceived weight loss and extensive support. The next phases, the “Everyday life phase” and “It’s up to you phase”, contained the largest obstacles to change. The home environment appeared as a crucial factor, which could be decisive for maintenance of the new dietary habits or relapse into old habits in the last phase called “Crossroads phase”.

Conclusion

We identified various phases of engagement in the process of a long-term dietary intervention among middle-aged women. A clear personal goal and support from family and friends seem to be of major importance for long-term maintenance of new dietary habits. Gender relations within the household must be considered as a possible obstacle for women engaging in diet intervention.
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7.

Background

Internationally, clinical ethics support has yet to be implemented systematically in community health and care services. A large-scale Norwegian project (2007–2015) attempted to increase ethical competence in community services through facilitating the implementation of ethics support activities in 241 Norwegian municipalities. The article describes the ethics project and the ethics activities that ensued.

Methods

The article first gives an account of the Norwegian ethics project. Then the results of two online questionnaires are reported, characterizing the scope, activities and organization of the ethics activities in the Norwegian municipalities and the ethical topics addressed.

Results

One hundred and thirty-seven municipal contact persons answered the first survey (55 % response rate), whereas 217 ethics facilitators from 48 municipalities responded to the second (33 % response rate). The Norwegian ethics project is vast in scope, yet has focused on some institutions and professions (e.g., nursing homes, home-based care; nurses, nurses’ aides, unskilled workers) whilst seldom reaching others (e.g., child and adolescent health care; physicians). Patients and next of kin were very seldom involved. Through the ethics project employees discussed many important ethical challenges, in particular related to patient autonomy, competence to consent, and cooperation with next of kin. The “ethics reflection group” was the most common venue for ethics deliberation.

Conclusions

The Norwegian project is the first of its kind and scope, and other countries may learn from the Norwegian experiences. Professionals have discussed central ethical dilemmas, the handling of which arguably makes a difference for patients/users and service quality. The study indicates that large (national) scale implementation of CES structures for the municipal health and care services is complex, yet feasible.
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8.

Purpose

To describe the course and configuration of the superficial temporal artery (STA) around the zygomatic arch.

Methods

Volume rendered 3D reconstructions of computed tomography angiography of 25 healthy patients were performed and analyzed at Duke University Hospitals.

Results

The STA coursed over the zygomatic arch or over the condylar process of the mandible in all cases (25/25 pts, 100 %). The STA courses over the posterior zygomatic arch in 23/25 pts (92 %), creating a characteristic “C” shape half-buttonhole configuration as it embraces the arch. When the STA travels posterior to the zygomatic arch, there is no C shape configuration (2/25 pts, 8 %). The STA bifurcates distal to the zygomatic arch in 24/25 pts (96 %).

Conclusions

The “C” shape half-buttonhole configuration is a useful identifying characteristic of the most common course of the STA—over the posterior zygomatic arch before it bifurcates.
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9.

Background

The emerging field, Lifestyle Medicine (LM), is the evidence-based practice of assisting individuals and families to adopt and sustain behaviors that can improve health. While competencies for LM education have been defined, and undergraduate curricula have been published, there are no published reports that address graduate level fellowship in LM. This paper describes the process of planning a LM fellowship curriculum at a major, academic teaching institution.

Methods

In September 2012 Harvard Medical School Department of Physical Medicine and Rehabilitation approved a “Research Fellowship in Lifestyle Medicine”. A Likert scale questionnaire was created and disseminated to forty LM stakeholders worldwide, which measured perceived relative importance of six domains and eight educational experiences to include in a one-year LM fellowship. Statistical procedures included analysis of variance and the Wilcoxon signed-rank test.

Results

Thirty-five stakeholders (87.5%) completed the survey. All domains except smoking cessation were graded at 4 or 5 by at least 85% of the respondents. After excluding smoking cessation, nutrition, physical activity, behavioral change techniques, stress resiliency, and personal health behaviors were rated as equally important components of a LM fellowship curriculum (average M?=?4.69, SD?=?0.15, p?=?0.12). All educational experiences, with the exception of completing certification programs, research experience and fund raising, were graded at 4 or 5 by at least 82% of the responders. The remaining educational experiences, i.e. clinical practice, teaching physicians and medical students, teaching other health care providers, developing lifestyle interventions and developing health promotion programs were ranked as equally important in a LM fellowship program (average M?=?4.23, SD?=?0.11, p?=?0.07).

Conclusions

Lifestyle fellowship curricula components were defined based on LM stakeholders’ input. These domains and educational experiences represent the range of competencies previously noted as important in the practice of LM. As the foundation of an inaugural physician fellowship, they inform the educational objectives and future evaluation of this fellowship.
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10.

Background

Diagnostic problems of thyroid cytology are frequently discussed, but relevance and causes of discrepant cytological and histological diagnoses are rarely studied in detail.

Objectives

Investigation of causes and relevance of discrepant diagnoses.

Materials and method

The analysis includes 297 patients who had thyroid resection after prior fine needle aspiration (FNA) and is based on the cytological and histological reports. In special cases, cytological and histological specimens were re-examined.

Results

Malignant tumors were found in 45 patients (15.1?%). In 5 patients the cytological diagnosis was “false negative”. Three of these 5 tumors were papillary carcinomas (PTC) of ≤10 mm, one an obviously nonmalignant papillary proliferation of the thyroidal epithelium and one a malignant lymphoma complicating autoimmune thyreoiditis (AIT). In 11 of the 35 patients with a FNA diagnosis “suspicious of malignancy” or “malignant,” 1 AIT, 4 goiter nodules, and 6 adenomas were diagnosed histologically. However, since distinct nuclear atypia was found in three of five false positive diagnoses, there still remains doubt in their benignity.

Conclusions

Carcinomas of ≤10 mm incidentally detected in the resected thyroid tissue may not be relevant to the patient and do not reduce the high negative predictive value of FNA. The final diagnosis on the resected tissue should include the cytological findings. Discrepant findings should be commented in the report to the clinician.
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11.

Background

Little is known about what is at stake at a subjective level for the oncologists and the advanced cancer patients when they face the question whether to continue, limit or stop specific therapies. We studied (1) the frequency of such questioning, and (2) subjective determinants of the decision-making process from the physicians’ and the patients’ perspectives.

Methods

(1) All hospitalized patients were screened during 1 week in oncology and/or hematology units of five institutions. We included those with advanced cancer for whom a questioning about the pursuit, the limitation or the withholding of specific therapies (QST) was raised. (2) Qualitative design was based on in-depth interviews.

Results

In conventional units, 12.8 % of cancer patients (26 out of 202) were concerned by a QST during the study period. Interviews were conducted with all physicians and 21 advanced cancer patients. The timing of this questioning occurred most frequently as physicians estimated life expectancy between 15 days and 3 months. Faced with the most frequent dilemma (uncertain risk-benefit balance), physicians showed different ways of involving patients. The first two were called the “no choice” models: 1) trying to resolve the dilemma via a technical answer or a “wait-and-see” posture, instead of involving the patients in the questioning and the thinking; and 2), giving a “last minute” choice to the patients, leaving to them the responsibility of the decision. In a third model, they engaged early in shared reflections and dialogue about uncertainties and limits with patients, proxies and care teams. These schematic trends influenced patients’ attitudes towards uncertainty and limits, as they were influenced by these ones. Individual and systemic barriers to a shared questioning were pointed out by physicians and patients.

Conclusions

This study indicate to what extent these difficult decisions are related to physicians’ and patients’ respective and mutually influenced abilities to deal with and share about uncertainties and limits, throughout the disease trajectory. These insights may help physicians, patients and policy makers to enrich their understanding of underestimated and sensitive key issues of the decision-making process.
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12.

Background

As one of the serious public health issues, vaccination refusal has been attracting more and more attention, especially for newly approved human papillomavirus (HPV) vaccines. Understanding public opinion towards HPV vaccines, especially concerns on social media, is of significant importance for HPV vaccination promotion.

Methods

In this study, we leveraged a hierarchical machine learning based sentiment analysis system to extract public opinions towards HPV vaccines from Twitter. English tweets containing HPV vaccines-related keywords were collected from November 2, 2015 to March 28, 2016. Manual annotation was done to evaluate the performance of the system on the unannotated tweets corpus. Followed time series analysis was applied to this corpus to track the trends of machine-deduced sentiments and their associations with different days of the week.

Results

The evaluation of the unannotated tweets corpus showed that the micro-averaging F scores have reached 0.786. The learning system deduced the sentiment labels for 184,214 tweets in the collected unannotated tweets corpus. Time series analysis identified a coincidence between mainstream outcome and Twitter contents. A weak trend was found for “Negative” tweets that decreased firstly and began to increase later; an opposite trend was identified for “Positive” tweets. Tweets that contain the worries on efficacy for HPV vaccines showed a relative significant decreasing trend. Strong associations were found between some sentiments (“Positive”, “Negative”, “Negative-Safety” and “Negative-Others”) with different days of the week.

Conclusions

Our efforts on sentiment analysis for newly approved HPV vaccines provide us an automatic and instant way to extract public opinion and understand the concerns on Twitter. Our approaches can provide a feedback to public health professionals to monitor online public response, examine the effectiveness of their HPV vaccination promotion strategies and adjust their promotion plans.
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13.

Introduction

Colorectal cancer (CRC) still represents the third most commonly diagnosed type of cancer in men and women worldwide. CRC is acknowledged as a heterogeneous disease that develops through a multi-step sequence of events driven by clonal selections; this observation is sustained by the fact that histologically similar tumors may have completely different outcomes, including a varied response to therapy.

Methods

In “early” and “intermediate” stage of CRC (stages II and III, respectively) there is a compelling need for new biomarkers fit to assess the metastatic potential of their disease, selecting patients with aggressive disease that might benefit from adjuvant and targeted therapies. Therefore, we review the actual notions on immune response in colorectal cancer and their implications for biomarker development.

Results

The recognition of the key role of immune cells in human cancer progression has recently drawn attention on the tumor immune microenvironment, as a source of new indicators of tumor outcome and response to therapy. Thus, beside consolidated histopathological biomarkers, immune endpoints are now emerging as potential biomarkers.

Conclusions

The introduction of immune signatures and cellular and molecular components of the immune system as biomarkers is particularly important considering the increasing use of immune-based cancer therapies as therapeutic strategies for cancer patients.
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14.

Background

The sleep related breathing disorder subtest of the pediatric sleep questionnaire (PSQ-SRBD) is widely recommended for the diagnosis of pediatric obstructive sleep apnea (OSA). It consists of 22 items and asks for symptoms in the areas of breathing, daytime sleepiness and behavior.

Objective

The aim of this study was to verify the suitability of the test in a cohort of children mainly suffering from hearing and speech developmental disorders.

Methodology

Retrospectively, the results of the PSQ-SRBD were collected over a period of 3.5 months from all patients above the age of 1 year. The results were evaluated on single item level and in relation to the total score.

Results

Of the 140 questionnaires completed, 12 were not evaluable. The mean age of the children was 5.6 ± 2.9 years and a majority suffered from language impairments, hearing and general developmental disorders. The rate of positive questionnaires with 30.5?% was unexpectedly high. The five most common “yes”- answers came from the behavioral field. Of children with a positive questionnaire, 20.5?% had no “yes” answers in the area of sleep-related respiratory symptoms.

Discussion

In the present cohort an unexpectedly high percentage of questionnaires was positive. A disproportionate number of responses from the behavioral domain are answered with “yes”. It can be assumed that in the above mentioned cohort the prevalence of pediatric SRBD is higher than in the general pediatric population. This could indicate an association between pediatric SRBD and specific language impairments. An alternative explanation would be that the questionnaire is not entirely suitable for this cohort, since it has a high proportion of questions in the behavioral area.
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15.

Background

Research activities for medical students and residents (trainees) are expected to serve as a foundation for the acquisition of basic research skills. Some medical schools therefore recommend research work as partial requirement for certification. However medical trainees have many difficulties concerning research, for which reason potential remedial strategies need to be constantly developed and tested. The views of medical trainees are assessed followed by their use and appraisal of a novel “self-help” tool designed for the purposes of this study with potential for improvement and a wider application.

Methods

This study was a cross-sectional survey of volunteering final-year medical students and residents of a medical school in Cameroon.

Results

This study surveyed the opinions of a total of 120 volunteers of which 82 (68%) were medical students. Three out of 82 (4%) medical students reported they had participated in research activities with a publication versus 10 out of 38 residents (26%). The reported difficulties in research for these trainees included referencing of material (84%), writing a research proposal (79%), searching for literature (73%) and knowledge of applicable statistical tests (72%) amongst others. All participants declared the “self-help” tool was simple to use, guided them to think and better understand their research focus.

Conclusion

Medical trainees require much assistance on research and some “self-help” tools such as the template used in this study might be a useful adjunct to didactic lectures.
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16.

Background

This study investigated perceived preparedness to practice, one year after graduation across osteopathic education institutions (OEIs) and explored possible differences between countries where osteopathy is regulated (Reg) and countries where it is not (Unreg).

Methods

Two hundred forty-five graduates from 7 OEIs in 4 European countries, already assessed in a previous study, were contacted one year after their graduation to complete the survey. Survey tools included a questionnaire to assess perceived preparedness to practice: Association of American Medical Colleges (AAMC) questionnaire, and a questionnaire to collect socio-demographic information and practice characteristics.

Results

One hundred sixty-eight graduates (68.6%) completed the survey. The AAMC mean score one year after the graduation (23.19; confidence interval 22.81–23.58) was significantly higher than in the previous study (17.58; 16.90–18.26) (p?<?0.001). A difference was also found between Reg (23.49; 23.03–23.95) and Unreg (22.34; 21.74–22.94) (p?=?0.004). Osteopaths with a previous healthcare degree scored significantly higher on AAMC score (25.53; 24.88–26.19) than osteopaths without a previous healthcare degree (22.33; 21.97–22.69) (p?<?0.001). Regulation and a previous degree were the only significant independent variables in the most predictive multivariate linear model. The model had an r2?=?0.33.

Conclusions

Graduates from OEIs where osteopathy is regulated felt significantly better prepared to practice than Unreg. Systematic information searches about graduates’ perception of preparedness to practice, may enable OEIs to strengthen their existing curricula to ensure their graduates are effectively prepared to practice.
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17.

Background

Chronotype manifestation often has a broad influence on sleep quality. One possible explanation for daytime impairments in evening types is the concept of “social jetlag”. Social jetlag is caused by an incompatibility between circadian preference and the socially accepted rhythm. This can be declared as a social stressor.

Objective

The association between chronotype, stress coping, and sleep quality was assessed in a pilot study.

Materials and methods

A total of 75 female adults aged 20–41 years participated in the study and completely answered all questions. Various questionnaires including sociodemographic data, information about sleep quality (Pittsburgh Sleep Quality Index, PSQI), chronotype (morningness–eveningness questionnaire, MEQ), and stress coping (Stress Coping Style Questionnaire, SVF78) were applied.

Results

Heightened use of maladaptive coping strategies is associated with a reduction in sleep quality. Chronotypes did not differ in terms of sleep quality and the coping strategies used.

Conclusion

Maladaptive coping strategy use seems to have a negative influence on sleep quality. Preventive education in adaptive stress coping strategies and avoidance of maladaptive stress coping thus seems useful to reduce these adverse influences on sleep quality.
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18.

Background

Interprofessional collaboration (IPC) primarily aims to enhance collaborative skills and to improve the awareness of teamwork and collaborative competencies of health care students. The Readiness for Interprofessional Learning Scale (RIPLS) was used to assess such skills. The aim of this study was to adapt a Chinese version of the RIPLS among Chinese health care students and to test the psychometric properties of the modified instrument.

Methods

The questionnaire was translated following a two-step process, comprising forward and backward translations and a pilot test. The Chinese version was tested on a group of students from various health care professions. Cronbach’s α coefficients were calculated for each of the four factors and also for the entire questionnaire in order to evaluate the internal consistency of the Chinese version of the RIPLS.

Results

Of the 295 health care students surveyed, 282 (96.5%) completed the questionnaire. Cronbach’s α coefficient for the overall scale was 0.842. Internal consistencies within each factor were good (α?>?0.70) except for the factor “Roles and Responsibilities”, where α?=?0.216. Confirmatory factor analysis showed that the data fit the four-factor structure.

Conclusion

The Chinese version of the RIPLS was an acceptable instrument for evaluating the attitudes of the health care students in China. The factor “Roles and Responsibilities” requires further scrutiny and development, at least in the Chinese context.
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19.

Purpose

The purpose of this study was to describe neurovascular structures-at-risk during establishment of five portals for access to distal biceps tendon (DBT) in cubital fossa, and to establish relative safety of these portal sites for such access. We hypothesized that all five portals are safe for endoscopic DBT exploration.

Methods

Ten fresh frozen cadaveric elbows were dissected after placement of portals at five potential sites (four anterior, one posterior). Nine neurovascular structures (CV, cephalic vein; LCN, lateral cutaneous nerve; LV, leash of vessels; RN, radial nerve; SRN, superficial radial nerve; PIN, posterior interosseous nerve; RA, radial artery; BA, brachial artery; MN, median nerve) were dissected, and their distances from portal sites were measured. Statistical analysis was performed to determine relative portal safety, and risk of injury to neurovascular structures in relation to each portal was analyzed.

Results

Structures that were significantly “at risk” were RA (p = 0.006), SRN (p = 0.002), and PIN (p = 0.004). RA was significantly “at risk” of injury from portal 4 (p = 0.009). Similarly, SRN was “at risk” from portal 3 (p = 0.036), and the PIN was “at risk” from portal 2 (p = 0.003).

Conclusions

Portal 1 (parabiceps portal) was safe for all neurovascular structures, however, portals 2–4 were significantly closer to neurovascular structures. RA, SRN, and PIN were significantly “at risk” as compared to other structures amongst the portals studied. Portal 5 was relatively safe for SRN and PIN.

Clinical relevance

Portals 1 (parabiceps portal) and 5 (distal posterior) can be safely placed for endoscopic access to the DBT. Portal 4 (open distal anterior) may be used after careful open dissection and under direct vision. Portals 2 and 3 are not recommended for elbow endoscopy.
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20.

Background

X-linked inhibitor of apoptosis protein (XIAP) deficiency is a rare immunodeficiency that is characterized by recurrent hemophagocytic lymphohistiocytosis (HLH) and splenomegaly and sometimes associated with refractory inflammatory bowel disease (IBD). Although hematopoietic stem cell transplantation (HSCT) is the only curative therapy, the outcomes of HSCT for XIAP deficiency remain unsatisfactory compared with those for SLAM-associated protein deficiency and familial HLH.

Aim

To investigate the outcomes and adverse events of HSCT for patients with XIAP deficiency, a national survey was conducted.

Methods

A spreadsheet questionnaire was sent to physicians who had provided HSCT treatment for patients with XIAP deficiency in Japan.

Results

Up to the end of September 2016, 10 patients with XIAP deficiency had undergone HSCT in Japan, 9 of whom (90%) had survived. All surviving patients had received a fludarabine-based reduced intensity conditioning (RIC) regimen. Although 5 patients developed post-HSCT HLH, 4 of them survived after etoposide administration. In addition, the IBD associated with XIAP deficiency improved remarkably after HSCT in all affected cases.

Conclusion

The RIC regimen and HLH control might be important factors for successful HSCT outcomes, with improved IBD, in patients with XIAP deficiency.
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