Miracle-dz/UniFAR-FTUs huggingface dataset:
Molecular mechanism of intraductal papillary mucinous neoplasm and intraductal papillary mucinous neoplasm‐derived pancreatic ductal adenocarcinoma

Pancreatic ductal adenocarcinoma (PDA) is one of the most lethal human malignancies. Dissecting the mechanisms underlying PDA development is important for developing early detection methods and effective prevention and therapies for the disease. PDA is considered to arise from distinct precursor lesions, including pancreatic intraepithelial neoplasia (PanIN) and intraductal papillary mucinous neoplasia (IPMN). However, little is known about molecular mechanisms of development of IPMN and IPMN‐derived PDA. We have recently reported that loss of Brg1, a core subunit of SWI/SNF chromatin remodeling complexes, cooperates with oncogenic Kras to form cystic neoplastic lesions that resemble human IPMN and progress to PDA. Brg1 null IPMN‐PDA is less lethal compared to PanIN‐derived PDA (PanIN‐PDA) driven by mutant Kras and hemizygous p53 deletion, mirroring prognostic trends in PDA patients. Brg1 null IPMN‐PDA possesses a distinct molecular signature that supports less malignant potential compared to PanIN‐PDA. Furthermore, Brg1 deletion inhibits Kras‐dependent PanIN development from adult acinar cells, but promotes Kras‐driven preneoplastic transformation in adult duct cells. Therefore, Brg1 is a determinant of context‐dependent Kras‐driven pancreatic tumorigenesis and chromatin remodeling may underlie the development of distinct PDA subsets. Understanding molecular mechanism of IPMN and IPMN‐derived PDA could provide critical clues for novel diagnostic and therapeutic strategies of the disease.
Obesity and associated factors in adults with intellectual disability.

BACKGROUND We examined the prevalence of obesity in adults with intellectual disabilities (ID) compared with the general population, and the factors associated with obesity and weight management status, comparing individuals with ID who were overweight or obese to those who were not. METHODS We analysed baseline data (n = 1450) from the ongoing 4-year Longitudinal Health and Intellectual Disabilities Study (LHIDS) using a multivariate approach. Measures included body mass index (BMI), demographics, level of ID, diagnoses related to ID, health behaviours (i.e. physical activity, dietary habits, smoking, and alcohol consumption), various health parameters (e.g. mobility limitation, medications), and residential type and location. RESULTS Compared with the general population, adults (≥ 18 years) with ID had a higher prevalence of obesity (38.3% vs. 28%) and morbid obesity (7.4% vs. 4.2%). Being female (AOR = 1.40, 95% CI = 1.09-1.81), having Down syndrome (AOR = 2.53, 95% CI = 1.86-3.45), taking medications that cause weight gain (AOR = 1.80, 95% CI = 1.38-2.37), engaging in less moderate physical activity (AOR = 0.89, 95% CI = 0.79-0.99), and drinking greater amounts of soda (AOR = 1.20, 95% CI = 1.02-1.42) were associated with higher rates of obesity. CONCLUSION Adults with ID, in general, have a high risk of developing obesity, and women with ID have a high risk of developing morbid obesity. Health promotion initiatives should target individuals with the greatest risk.
Itinerant magnetic metals

In this review, an overview of itinerant magnets without magnetic elements is presented, beginning with a comparison of the local and itinerant moment pictures, the two extremes of magnetism. Then, the theoretical developments leading up to the self-consistent renormalization theory of spin fluctuations will be discussed, followed by an introduction to quantum criticality and the experimental signatures associated with systems near a quantum critical point. Three itinerant magnets without magnetic elements, ZrZn2, Sc3.1In, and TiAu are the focus of this review, as their empty d shells set them apart in their purely itinerant character, while several enhanced Pauli paramagnets and intermediate moment magnets are also discussed to put the overall comparison into perspective.
Nature, level and type of networking for individuals with dual diagnosis: A European perspective

Aim: To obtain more detailed information on service availability and appropriateness and interagency networking for individuals with dual diagnosis (DD) at seven European centres. Methods: Data was collected using two parts of the three-part Treatment of Dual Diagnosis tool in seven European centres as part of the Integrated Services Aimed at Dual Diagnosis and Optimal Recovery from Addiction (ISADORA) study between 2002 and 2005 focusing on the nature, level and type of networking for individuals with co-morbid mental health and substance misuse problems (DD). A multi-level process of qualifying networking was used. Findings: Findings show that 50–90% of the listed centres across the ISADORA sites reported some level of networking but only 30% had a joint care agreement in place or shared patient/client records with at least one other agency. Barriers and facilitators to interagency collaboration are described. Conclusion: This pan-European study highlighted the need for a more integrated and focused approach to DD service delivery.
A Phase II Trial on Alpha-Interferon α IFN) Effect in Patients with Monoclonal IgM Gammopathy

Waldenstrom's macroglobulinemia (WM) is an incurable disorder of B cells. Following occasional reports of response to α interferon (IFN) and in view of its effectiveness in hairy cell leukemia, we tested this agent in a relatively large group (n = 88) of patients who had an IgM monoclonal component (MC) greater than 10 g/1. Thirty eight patients had a MC >30 g/1 and were classified as Waldenstrom's macroglobulinemia (WM), while fifty had either WM in an early stage or an IgM monoclonal gammopathy of undeterminated significance (all of them operationally classified as IgM-MGUS). All patients received IFN 3 MU/day for one month and then 3 times/week. Response to treatment was mainly based on MC reduction in two consecutive determinations (>50%: major response; 25-50%: minor response). Of 36 evaluable WM patients, 12 had a major and 6 a minor response; of 41 evaluable IgM-MGUS patients, 2 had a major and 6 a minor response. In WM patients with a major response, MC reduction was associated with disappearance .
Fragile X syndrome: economic burden and health-related quality of life of patients and caregivers in France.

BACKGROUND Fragile X syndrome (FXS) is the main hereditary cause of intellectual disability. Although the associated burden appears to be considerable, to date no study has comprehensively assessed the cost incurred because of FXS, including its specific impact on health-related quality of life and the burden on caregivers using standardised quantitative tools. The aim of this article is to provide data in order to increase awareness of the repercussions of FXS on patients and caregivers as well as on the health and social care systems in France. METHODS A retrospective cross-sectional study was carried out on 145 patients recruited through Le Goëland X-Fragile and Mosaïques, the French FXS patient associations. Data on their demographic characteristics and resource use were obtained from an online questionnaire, and costs were estimated by a bottom-up approach. The EQ-5D health questionnaire was used to measure patients' and caregivers' health-related quality of life. Perceived burden of care was measured using the Zarit Caregiver Burden Interview. The Barthel index, a non-utility-based assessment, was used to measure patients' level of dependence. RESULTS The annual total direct cost of FXS was estimated at €25 800 per patient. The main contributors were informal care provided by the main caregiver (€10 500) and social services (€8400). Healthcare costs, estimated at €2700, represented only a minor share. Mean EQ-5D utility scores were 0.49 for patients and 0.75 for caregivers. The mean burden for caregivers as measured by the Zarit Caregiver Burden Interview was 39.9. CONCLUSIONS Fragile X syndrome requires significant resources that are mainly of a non-medical nature and are higher for children than for adults. Compared with related diseases, it constitutes a particularly high burden for caregivers. Using a bottom-up approach and a wide range of standardised measures, this study underscores the need for greater awareness of the burden of FXS as well as an assessment of new and existing interventions to address it.
Impact of knee osteoarthritis on the quality of life among Saudi elders: A comparative study

Background: The population sector of elders is progressively increasing, and it is estimated that it will reach almost one third of the total population in 2025. Large number of elders is suffering from knee osteoarthritis (OA) as the most prevalent rheumatic diseases worldwide. Recently there is a growing concern among researchers and health professionals regarding the impact of knee OA on elders and their quality of life (QOL). Objectives: To explore the impact of knee OA on QOL among Saudi elders aged between 60-70 years and to define which of the Short from survey (SF-36) domain (s) and which component, physical or mental, are mostly affected by this knee OA. In addition to study the presence of correlation between scores of SF-36 domains and components and the following factors; patient′s age, gender, weight, height, body mass index (BMI), disease duration and severity, educational level, stability of the income source, income, housing type, housing ownership, and care giver. Subjects and Methods: This study was carried out during (December/ 2010 - March/ 2011). One hundred and twenty Saudi elders, (58, 48.3% male), aged between 60-70 years with mean± SD (64.5 ± 3.03) participated in this comparative study. Half of them had severe knee OA, as defined by knee society score (KSS), they were recruited from King Saud Medical City. The other half were without knee OA, were recruited from Prince Salman Social Center and the Saudi association for the Cooperative Retirees. Arabic version of Short Form -36 questionnaire was used to measure participants QOL domains scores as well as the overall scores of the physical and mental components of the questionnaire. Data collected were statistically treated and presented in tables. Results: Current study showed a better significant scores for Saudi elders without knee OA on all SF-36 domains (p = 0.0001). Calculating the patients′ domain′s scores as percentage from the maximum domain′s scores which showed that, the mental health component of elders patients was more affected than physical health component and that role limitation due to emotional problems domain (RLEP) was the most affected domain (22.7%). Physical component showed positive correlation with income (r= 0.320, P= 0.013). While mental component showed negative correlation with disease duration (r = -0.341, P=0.008). Conclusion: Knee OA has negative impact on Saudi elders′ QOL. Mental health component was more affected than physical health component. Consequently RLEP domain was the most affected domain. Patients′ QOL was positively correlated to income and negatively correlated to disease duration.
Thermoelectric performance and the role of anti-site disorder in the 24-electron Heusler TiFe2Sn

Heusler compounds XY2Z with 24 valence electrons per formula unit are potential thermoelectric materials, given their thermal and chemical stability and their relatively earth-abundant constituent elements. We present results on the 24-electron compound TiFe2Sn here. First principles calculations on this compound suggest semiconducting behavior. A relatively flat conduction band that could be associated with a high Seebeck coefficient upon electron doping is found. A series of compounds have been prepared and characterized using a combination of synchrotron x-ray and neutron diffraction studies to understand the effects of site order/disorder phenomena and n-type doping. Samples fabricated by a three step processing approach were subjected to high temperature Seebeck and electrical resistivity measurements. Ti:Fe anti-site disorder is present in the stoichiometric compound and these defects are reduced when starting Ti–rich compositions are employed. Additionally, we investigate control of the Seebeck coefficient through the introduction of carriers through the substitution of Sb on the Sn site in these intrinsically p-type materials.
Advanced carbon nanotubes functionalization

Similar to graphene, carbon nanotubes are materials made of pure carbon in its sp2 form. Their extended conjugated π-network provides them with remarkable quantum optoelectronic properties. Frustratingly, it also brings drawbacks. The π–π stacking interaction makes as-produced tubes bundle together, blurring all their quantum properties. Functionalization aims at modifying and protecting the tubes while hindering π–π stacking. Several functionalization strategies have been developed to circumvent this limitation in order for nanotubes applications to thrive. In this review, we summarize the different approaches established so far, emphasizing the balance between functionalization efficacy and the preservation of the tubes’ properties. Much attention will be given to a functionalization strategy overcoming the covalent–noncovalent dichotomy and to the implementation of two advanced functionalization schemes: (a) conjugation with molecular switches, to yield hybrid nanosystems with chemo-physical properties that can be tuned in a controlled and reversible way, and; (b) plasmonic nanosystems, whose ability to concentrate and enhance the electromagnetic fields can be taken advantage of to enhance the optical response of the tubes.
Mortality in people with intellectual disabilities in England

BACKGROUND People with intellectual disabilities (IDs) die at younger ages than the general population, but nationally representative and internationally comparable mortality data about people with ID, quantifying the extent and pattern of the excess, have not previously been reported for England. METHOD We used data from the Clinical Practice Research Datalink database for April 2010 to March 2014 (CPRD GOLD September 2015). This source covered several hundred participating general practices comprising roughly 5% of the population of England in the period studied. General practitioner (GP) records identified people diagnosed by their GP as having ID. Linked national death certification data allowed us to derive corresponding mortality data for people with and without ID, overall and by cause. RESULTS Mortality rates for people with ID were significantly higher than for those without. Their all-cause standardised mortality ratio was 3.18. Their life expectancy at birth was 19.7 years lower than for people without ID. Circulatory and respiratory diseases and neoplasms were the three most common causes of death for them. Cerebrovascular disease, thrombophlebitis and pulmonary embolism all had standardised mortality ratios greater than 3 in people with ID. This has not been described before. Other potentially avoidable causes included epilepsy (3.9% of deaths), aspiration pneumonitis (3.6%) and colorectal cancer (2.4%). Avoidable mortality analysis showed a higher proportion of deaths from causes classified as amenable to good medical care but a lower proportion from preventable causes compared with people without ID. International comparison to areas for which data have been published in sufficient detail for calculation of directly standardised rates suggest England may have higher death rates for people with ID than areas in Canada and Finland, and lower death rates than Ireland or the State of Massachusetts in the USA. CONCLUSIONS National data about mortality in people with ID provides a basis for public health interventions. Linked data using GP records to identify people with ID could provide comprehensive population-based monitoring in England, unbiased by the circumstances of illnesses or death; to date information governance constraints have prevented this. However, GPs in England currently identify only around 0.5% of the population as having ID, suggesting that individuals with mild, non-syndromic ID are largely missed. Notably common causes of death suggest control of cardiovascular risk factors, epilepsy and dysphagia, management of thrombotic risks and colorectal screening are important areas for health promotion initiatives.
Occupational Therapy and the Childhood Obesity Epidemic: Research, Theory and Practice

Childhood obesity is a public health epidemic in the United States. Children who are overweight or obese experience multiple physical, emotional, and social challenges in daily life activity (occupational) participation. The profession of occupational therapy addresses children’s current and future health and occupational needs through the ability to participate in everyday life. Presented in this paper is an overview of the role occupational therapy can and does play in meeting the challenges posed by childhood obesity with all children, including those with disabilities. In the context of the research and prevalence data, examples of occupational therapy interventions for school- and community-based settings are examined. Case studies illustrate occupational therapists’ contribution in prevention of and intervention for childhood obesity. Future research and strategies for prevention and health promotion advocating for the occupational health of children are discussed.
Prevalence of psychotropic medication use and association with challenging behaviour in adults with an intellectual disability. A total population study

BACKGROUND There is a high prevalence of psychotropic medication use in adults with Intellectual Disabilities (ID), often in the absence of psychiatric disorder, also associated with challenging behaviour. Previous research has focused on specific sample frames or data from primary care providers. There is also a lack of consistency in the definition of challenging behaviour used. METHODS We adopted a total population sampling method. Medication data on 265 adults with ID were classified according to the Anatomical Therapeutic Chemical classification system. The Behaviour Problems Inventory - short form classified challenging behaviours. We examined the association between challenging behaviour and the use of psychotropic medication, and whether any association would still be present after accounting for socio-demographic and clinical characteristics. RESULTS 70.57% of adults with ID were prescribed at least one class of any medication (mean per person =2.62; range 0-14). Psychotropic medications were used by 37.73% of participants with antipsychotics the commonest type used by 21.89% of individuals. Polypharmacy and high dosages were common. Generalised Linear Models indicated significant associations between psychotropic medication and the presence of a psychiatric diagnosis, challenging behaviour, older age and type of residence. Male gender was additionally associated with antipsychotic medication. CONCLUSIONS The use of a total population sample identified via multiple routes is less likely to overestimate prevalence rates of medication use. Current challenging behaviour was a predictor of medication use after controlling for other variables. Data indicate that there may be differences in prescribing patterns associated with different topographies of challenging behaviours.
The nature and prevalence of catatonic symptoms in young people with autism

BACKGROUND A proportion of young people with autism are reported to show catatonic-like symptoms in adolescence. The aetiology and prevalence of such presentations is unknown but include a set of behaviours that can best be described as attenuated. METHOD The current study empirically investigated the presence and nature of such attenuated behaviours in children and adolescents with autism using a newly developed 34-item third party report measure, the Attenuated Behaviour Questionnaire. Caregivers or parents of young people with autism reported on the presentation of symptoms via the online completion of the Attenuated Behaviour Questionnaire and two established clinical measures of repetitive behaviour and depression. RESULTS Initial results indicate that the Attenuated Behaviour Questionnaire is a workable clinical measure in this population with a degree of discriminant validity with regard to catatonia. Attenuated behaviour indicative of catatonia was relatively common in young people with autism with up to 20.2% having an existing diagnosis of catatonia and evidence of a relationship between attenuated behaviours and measures of depression and repetitive and restricted behaviours. CONCLUSION Catatonic symptoms are more prevalent in young people with autism than previously thought, and the Attenuated Behaviour Questionnaire has potential as a clinical and research tool.
Current understanding of the concept of frailty in later life

Frailty has attracted increasing attention in the medical literature over the last 20 years. What is it? Why is it important? How does it arise? Can it be recognized? What is recommended for its management? In this review we try to address all these questions and also assess what has to be done to further our understanding of frailty and its management.
The use of care pathways as tools to support the implementation of evidence-based practice

This article presents some of the data from a study exploring the experiences and views of a range of professional staff using care pathways in their everyday practice. It focuses on the views of doctors, nurses, and therapists. Within the context of delivering integrated care, several themes are explored in relation to the successful implementation of evidence-based care pathways.
Comparison of Institutional Costs for Laparoscopic Preperitoneal Inguinal Hernia Versus Open Repair and Its Reimbursement in an Ambulatory Surgery Center

Objective To compare institutional costs for open versus laparoscopic inguinal hernia repair and its relationship to reimbursement in an ambulatory surgery center in the United States. Method Analysis of institutional costs in US$ of 2006 for all nonreusables used in a laparoscopic total extraperitoneal (TEP) hernia repair using a polyester mesh compared with open hernia repair using polypropylene mesh. A comparison of the institution's disposable costs related to reimbursement at an ambulatory surgery center in Southeastern United States was performed to identify the most cost-effective procedure for the outpatient facility. Results As fixed and indirect costs of the ambulatory surgery center are similar for both procedures, a cost difference can only be found in direct disposable costs with that being US$ 235.57 for the procedure-specific disposables in the laparoscopic hernia repair as compared with US$ 117.15 for the open hernia repair. Cost for identical disposables used in both procedures amounted to US$ 32.57. Laparoscopic TEP hernia repair has a higher cost for procedure related disposables versus the open hernia repair at +US$ 118.42 mainly being due to the more costly polyester mesh. A flat rate reimbursement of US$ 1800 for a laparoscopic procedure compared with only US$ 950 for the open procedure minus all disposable cost results in a higher institutional income of +US&$ 731.58 (US$ 1531.86 vs. US$ 800.28), from which other institutional costs can be paid. Conclusions Despite marginally higher procedure-related disposable costs for laparoscopic TEP hernia repair, the institutional income is remarkably higher owing to a better reimbursement for this procedure in ambulatory surgery centers. From the institution's point of view, laparoscopic hernia repair is by far the more cost-effective procedure when compared with an open hernia procedure at the present time.