Miracle-dz/UniFAR-FTUs huggingface dataset:
A Transgenic Mouse Model of Cardiac AL Amyloidosis

Background: AL amyloidosis is the most frequent type of amyloidosis caused by the deposition in tissues of fibrillar aggregates composed of an abnormal immunoglobulin (Ig) light chain (LC) and leading to organ dysfunction. The most frequent and severe forms involve kidney and heart. Research on this disease suffers from the lack of reliable animal models, that could allow a better understanding of the disease and the design of new therapeutic strategies. In the present study, we aimed at reproducing AL amyloidosis in a mouse model. Methods: We developed an original transgenic approach using an insertion of a human pathogenic LC gene in the endogenous mouse kappa locus, such as the LC is produced by the naturally Ig producing B and plasma cells. Then, to avoid the association of human LCs with endogenous murine HCs, we backcrossed this strain with the DH-LMP2A mice, characterized by a high number of plasma cells devoid of endogenous HC. This strategy leads to a production of the human free LC similar or higher than in patients and proved efficient to reproduce in mice several monoclonal gammopathies of clinical significance (MGCS) (Fig.1A). Results: Despite strong LC production (Fig.1B), mice did not naturally develop AL amyloidosis. In vitro, the full length LC was resistant to amyloid formation at physiological conditions but the variable domain (IGLV6) showed high propensity to form fibrils. A single injection of amyloid fibrils and/or seeds, obtained from the variable domain (VL) of the human LC gene, led to amyloid deposits starting at 1 month post-injection, especially in the heart, spleen, liver and, to a lesser extent, in the kidney (Fig.1C). We confirmed that the deposits contain the full-length human LCs, which elongate VL fibrils in vivo (Fig.1D). Conclusions: This is, to our knowledge, the first transgenic mouse model of AL amyloidosis closely reproducing human lesions, especially in heart. Further studies are needed to better understand the early biochemical events leading to AL amyloidosis in vivo, but this model already shows that a partial degradation of the LC is likely required to initiate amyloid fibrils and that once seeded, the full length LC can elongate these fibrils. This mouse model opens new perspectives to better understand the toxicity of amyloid LC, their involvements in different biological processes and organ dysfunction and of course, to test new therapeutic approaches. Figure 1 Figure 1. Roussel: Takeda: Consultancy; Janssen: Membership on an entity's Board of Directors or advisory committees; GSK: Honoraria; BMS: Honoraria; Amgen: Consultancy. Jaccard: Janssen: Membership on an entity's Board of Directors or advisory committees; Pfizer: Honoraria; Abbvie: Honoraria. Bridoux: Janssen: Consultancy; AstraZeneca: Consultancy, Speakers Bureau. Sirac: Attralus, Inc: Patents & Royalties, Research Funding.
Discovery of Approximate Lexicographical Order Dependencies

Lexicographical order dependencies (LODs) specify orders between list of attributes, and are proven useful in optimizing SQL queries with order by clauses. To discover hidden dependencies from dirty data in practice, approximate dependency discoveries are actively studied, aiming at automatically discovering dependencies that hold on data with some exceptions. In this paper we study the discovery of approximate LODs. (1) We adapt two error measures, namely <inline-formula><tex-math notation="LaTeX">$g_1$</tex-math><alternatives><mml:math><mml:msub><mml:mi>g</mml:mi><mml:mn>1</mml:mn></mml:msub></mml:math><inline-graphic xlink:href="tan-ieq1-3130227.gif"/></alternatives></inline-formula> and <inline-formula><tex-math notation="LaTeX">$g_3$</tex-math><alternatives><mml:math><mml:msub><mml:mi>g</mml:mi><mml:mn>3</mml:mn></mml:msub></mml:math><inline-graphic xlink:href="tan-ieq2-3130227.gif"/></alternatives></inline-formula>, to LODs. We prove their desirable properties, present efficient algorithms for computing the measures and related lower and upper bounds, and study the relationship between the two measures. (2) We present an efficient approximate LOD discovery algorithm that is well suited to the two error measures, with a set of pruning rules, optimization techniques and ranking functions. (3) We study techniques for estimating <inline-formula><tex-math notation="LaTeX">$g_1$</tex-math><alternatives><mml:math><mml:msub><mml:mi>g</mml:mi><mml:mn>1</mml:mn></mml:msub></mml:math><inline-graphic xlink:href="tan-ieq3-3130227.gif"/></alternatives></inline-formula> by sampling, with high accuracy and far less time. (4) We conduct extensive experiments to verify the effectiveness and scalability of our methods, using both real-life and synthetic data.
Sickle Cell Disease Bias: An Early Opportunity for Intervention?

Background: Sickle cell disease (SCD) is a genetic condition leading to multi-organ dysfunction and chronic pain, with 1 out of 13 African Americans having the trait. Despite its prevalence and debilitating symptoms, SCD remains a disease with limited therapeutic interventions and decreased life expectancy. There is a clinical bias to undertreat the pain of patients with SCD with opioid medications, despite their effectiveness (Zempsky 2009). Early education about SCD may lead to improvements in provider perceptions and disease management. Methods: An anonymous survey was sent to first-year medical students at an academic institution prior to their hematology course. Data was collected about demographics, prior exposure to SCD, perceptions about opioid use disorder (OUD) in SCD, and baseline knowledge of management. Identical questions were asked for cancer-related pain. The survey was sent again after the completion of the hematology course to assess whether the curriculum was effective at characterizing misperceptions about patients with SCD. Results: Fifty-five medical students answered the survey prior to the course. Students were 38% male and 62% female. Seventy-five percent of students (N=41) had never encountered a patient with SCD. Forty percent (N=22) answered that OUD in patients with SCD is more likely, 60% as likely (N=33), and 0% less likely than the general population. Seventy-one percent (N=39) believed opiates should play a minor role, 5% (N=3) a major role, and 24% (N=13) said opioids should be avoided when managing SCD-related pain. After course completion, 35 students who had completed the survey prior to the course responded, 29% male and 71% female. Fifty-four percent (N=19) answered that OUD in patients with SCD is more likely, 43% (N=15) as likely, and 3% (N=1) less likely than the general population. Seventy-one percent (N=25) thought opiates should play a minor role, 9% (N=3) a major role, and 20% (N=7) said they should be avoided when managed SCD-related pain. Conclusion: After course completion, a higher percentage of medical students thought that OUD was more frequent in patients with SCD than the general population, with a similarly high percentage determining that opioid medications should play a minor role or be avoided in SCD. Opioid medications are an important part of treatment for SCD, especially for acute vaso-occlusive pain. Patients with SCD are no more likely to have OUD than the general population and have a lower rate of OUD than other patients with chronic pain conditions (Solomon 2008). This pilot study highlights that there is an opportunity to intervene in misperceptions of patients with SCD in early medical school education. Figure 1 Figure 1. No relevant conflicts of interest to declare.
Risk factors for adverse outcomes in women with high-risk pregnancy and their neonates, Haiti

ABSTRACT Objectives. To determine the prevalence of maternal death, stillbirth and low birthweight in women with (pre-)eclampsia and complicated pregnancies or deliveries in Centre de Références des Urgences Obstétricales, an obstetric emergency hospital in Port-au-Prince, Haiti, and to identify the main risk factors for these adverse pregnancy outcomes. Methods. We conducted a retrospective cohort study of pregnant women admitted to Centre de Référence des Urgences Obstétricales between 2013 and 2018 using hospital records. Risk factors investigated were age group, type of pregnancy (singleton, multiple), type of delivery and use of antenatal care services. Results. A total of 31 509 women and 24 983 deliveries were included in the analysis. Among these, 204 (0.6%) maternal deaths (648 per 100 000 women giving birth), 1962 (7.9%) stillbirths and 11 008 (44.1%) low birthweight neonates were identified. Of all admissions, 10 991 (34.9%) were women with (pre-)eclampsia. Caesarean section significantly increased the risk of maternal death in the women with a complicated pregnancy and women with (pre-)eclampsia, but reduced the risk of stillbirth in such women. Not attending antenatal care was associated with a significantly higher risk of stillbirth (odds ratio (OR) 4.82; 95% confidence interval (CI) 3.55–6.55) and low birthweight (OR 1.40; 95% CI 1.05–1.86) for women with complicated pregnancies. Conclusion. To prevent and treat pregnancy complications as early as possible, antenatal care attendance is crucial. Improving the quality of and access to antenatal care services and providing it free to all pregnant women in Haiti is recommended.
Changing roles of the clients, architects and contractors through BIM

Purpose – This paper aims to present a general review of the practical implications of building information modelling (BIM) based on literature and case studies. It seeks to address the necessity for applying BIM and re‐organising the processes and roles in hospital building projects. This type of project is complex due to complicated functional and technical requirements, decision making involving a large number of stakeholders, and long‐term development processes.Design/methodology/approach – Through desk research and referring to the ongoing European research project InPro, the framework for integrated collaboration and the use of BIM are analysed. Through several real cases, the changing roles of clients, architects, and contractors through BIM application are investigated.Findings – One of the main findings is the identification of the main factors for a successful collaboration using BIM, which can be recognised as “POWER”: product information sharing (P), organisational roles synergy (O), work proc.
Investigation of Flow Energy Dissipation over Different Stepped Spillways

In this study, to investigate energy dissipation of flow overstepped spillways of different step shapes, a physical wooden model has been built in the Shahid Bahonar Universitys hydraulics lab. This model with 41.41° slope has 84 cm height and 21 steps. Each step has 4 cm height, 4.5 cm width and 30 cm length. Experiments have been carried out for different types of step shapes (plain steps, end sill steps with thickness of 1, 2, 3 and 4 cm and steps with a bottom adverse slope of 15, 26, 36 and 45°). Overall, 131 experiments were done, the hydraulic parameters of flow over the model were measured and the energy dissipation of flow was calculated. Results show that the energy dissipation of flow on end sill and inclined stepped spillways are more than the plain one, it is increased by increasing the thickness of end sill or the adverse slope size. Comparison of flow energy dissipation over end sills stepped spillway and inclined type show that the inclined type has been dissipated more energy than the equivalent end sill type. Also for stepping spillway of different step shapes, flow energy dissipation decreases with increasing the flow rate. It is concluded that for a large stepped spillway with high flow rate the inclined type spillway is more effective than the other forms.
Automated biofabrication of anisotropic dense fibrin gels accelerate osteoblastic differentiation of seeded mesenchymal stem cells

Current challenges in the biofabrication of stable, cell-seeded fibrin gels are faced with a lack of microstructural and mechanical properties, limiting their use as bone extracellular matrix-mimicking constructs. While current approaches focus on generating fibrin gels of various fibrinogen and thrombin concentrations, i.e. 5–50 mg mL−1 and 1–250 units mL−1, respectively, processes that modulate gel fibrillar alignment and mechanical properties have yet to be explored. Herein, it was demonstrated that the automated gel aspiration-ejection (GAE) of precursor fibrin hydrogels of low concentration (i.e. 1 mg mL−1 fibrinogen and 0.5 units mL−1 thrombin) resulted in their immediate compaction while tailoring their fibrillar alignment and mechanical properties for potential target applications. Furthermore, this gel microenvironment accelerated the osteoblastic differentiation of seeded mesenchymal stem cells and their matrix mineralization, in vitro. Therefore, the ability to modulate the properties of dense fibrin constructs through automated GAE may be a novel biofabrication approach for cell delivery in bone regeneration.
Some Aspects of Micro-Fabrication Using Electro Discharge Deposition Process

The growing demand for micro-parts has led to the development of many processes for their production. One of the emerging processes in this category is the electric discharge deposition process in which a micro tool in the form of a wire is used. The tool wear is increased intentionally so that the worn out material gets deposited on the substrate. Experiments are designed using response surface methodology to identify the key operating parameters (voltage, current, duty cycle and pulse on-time) and then to study their effects on the structure’s height, and width. As a concluding part, micro parts (star and letters IITK) are fabricated on the substrate material and a method is proposed and demonstrated to detach micro parts from the metallic substrate material.Copyright © 2012 by ASME
The NICE OECD countries' geographic search filters: Part 2—validation of the MEDLINE and Embase (Ovid) filters

Objective: We previously developed draft MEDLINE and Embase (Ovid) geographic search filters for Organisation for Economic Co-operation and Development (OECD) countries to assess their feasibility for finding evidence about the countries. Here, we describe the validation of these search filters. Methods: We identified OECD country references from thirty National Institute for Health and Care Excellence (NICE) guidelines to generate gold standard sets for MEDLINE (n=2,065) and Embase (n=2,023). We validated the filters by calculating their recall against these sets. We then applied the filters to existing search strategies for three OECD-focused NICE guideline reviews (NG103 on flu vaccination, NG140 on abortion care, and NG146 on workplace health) to calculate the filters' impact on the number needed to read (NNR) of the searches. Results: The filters both achieved 99.95% recall against the gold standard sets. Both filters achieved 100% recall for the three NICE guideline reviews. The MEDLINE filter reduced NNR from 256 to 232 for the NG103 review, from 38 to 27 for the NG140 review, and from 631 to 591 for the NG146 review. The Embase filter reduced NNR from 373 to 341 for the NG103 review, from 101 to 76 for the NG140 review, and from 989 to 925 for the NG146 review. Conclusion: The NICE OECD countries' search filters are the first validated filters for the countries. They can save time for research topics about OECD countries by finding the majority of evidence about OECD countries while reducing search result volumes in comparison to no filter use.
Long-term follow-up of applying autologous bone grafts for reconstructing tympanomastoid defects in functional cholesteatoma surgery

Objectives This study investigated the long-term surgical outcomes of functional cholesteatoma surgery with canal wall reconstruction using autologous bone grafts as the primary material in patients with acquired cholesteatoma. Subjects and Methods Medical charts were retrospectively reviewed for all patients admitted to one institution for surgical intervention between 2010 and 2018. We analyzed 66 patients (66 ears) who underwent functional tympanomastoidectomy involving the use of autologous bone grafts for canal wall defect reconstruction. Surgical outcomes were evaluated by comparing preoperative audiometric results with follow-up data (at least 36 months after surgery). Logistic regression analyses were performed to determine prognostic factors related to long-term hearing success. These factors included classification and stage of cholesteatoma, stapes condition, ossicular chain damage, active infection of the middle ear, state of the contralateral ear, preoperative hearing thresholds, gender, and age. Results The mean follow-up period was 49.2 months. The recidivism rate was 6% (four of 66 ears). The pure-tone average significantly improved from 50.78 ± 19.98 to 40.81 ± 21.22 dB hearing level (HL; p < 0.001). Air–bone gaps significantly improved from 26.26 ± 10.53 to 17.58 ± 8.21 dB HL (p < 0.001). In multivariate logistic regression analysis, early-stage disease (p = 0.021) and pars flaccida cholesteatoma (p = 0.036) exhibited statistically significant correlations with successful hearing preservation. Conclusion Functional cholesteatoma surgery with autologous bone grafts reconstruction is an effective approach to significantly improve hearing with low recidivism rates. Localized disease and pars flaccida cholesteatoma were two independent predictors of successful hearing preservation.
DESIGN AND TEST OF A LONG-TERM FALL DETECTION SYSTEM INCORPORATED INTO A CUSTOM VEST FOR THE ELDERLY.

Falls in the elderly are a major problem for society. A serious consequence of falling is the "long-lie". A fall detection system and algorithm, incorporated into a custom designed garment has been developed which will automatically detect falls and potentially reduce the incidence of the "long-lie". The developed fall detection system consists of a tri-axial accelerometer, microcontroller, battery and Bluetooth module. This sensor is attached to a custom designed vest, designed to be worn by the elderly person under clothing. The fall detection algorithm was developed and incorporates both impact and posture detection capability. The vest was developed using feedback from elderly subjects donning, wearing and doffing a prototype vests and subsequently filling in a questionnaire. The re-designed vest and fall algorithm was tested in two clinical trials. Trial 1: young healthy subjects performing normal activities of daily living (ADL) and falls onto crash mats, while wearing the vest and sensor. Trial 2: The system was subsequently tested using 10 elderly subjects wearing the system over the course of 4 weeks, for 8 hours a day. Two teams of 5 elderly subjects wore the sensor system in turn for 2 week each. Results from trial 1, show that falls can be distinguished from normal activities with a sensitivity >90% and a specificity of >99%, from a total data set of 264 falls and 165 normal ADL. In trial 2, over 833 hours of monitoring was performed over the course of the four weeks from the elderly subjects, during normal daily activity. In this time no actual falls were recorded, however the system registered a total of the 42 fall-alerts. Further development of the system will include a more accurate fall-detection algorithm, more comfortable sensor attachment method, lighter and smaller sensor as well as, mobility monitoring and energy expenditure measurement. A fall detection system incorporated into a custom designed garment has been developed which will help reduce the incidence of the long-lie, when falls occur in the elderly population.
Use of Electronic Resources and its Impact: A Study of Dhaka University Library Users

This paper presents and analyses the status of electronic resources facilities and services provided by the Dhaka University Library (DUL). It also discusses the purpose of using e-resources, benefits, subject coverage status, overall user satisfactions, problems that are faced by DUL users while accessing e-resources and perceived impact of e-resources on users. Finally, it reports the results from questionnaire-based survey of e-resources use and its impact on DUL users. DOI: http://dx.doi. org/10.3329/el.v23i1. 12122 Eastern Librarian Vol.23(1) 2012 pp.74-90
Investigating Mechanisms for an Evaporative Cooled In-Wheel AFPM Machine

This paper investigated mechanisms that can be used to develop an evaporative cooled AFPM inwheel motor. Candidates for the working fluid and wicking materials were identified. Both immersion cooling and wick assisted cooling were proved to maintain stable pole piece temperatures. However it was shown that immersion cooling suffers from a slight temperature overshoot and requires the whole stator to be completely immersed in the working fluid. Conversely the wick assisted cooling mechanism avoids a temperature overshoot and makes use of less working fluid. This method however requires a more intricate design of the wick, which will be required to form an integral part of the machine.
A qualitative analysis of sacred moments in religiously integrated group therapy

ABSTRACT The aim of this study was to qualitatively explore sacred moments in religiously integrated group therapy for Danish Pentecostals who were experiencing a personal crisis (n = 18). This was a follow-up study based on data from 2009 and 2018. An interpretative phenomenological analytical approach was used to generate and analyze the data material. Three themes emerged: 1) Relationship with God, 2) Relationship with therapist, and 3) Relationship with group members. The participants’ experiences of sacred moments in these relationships were primarily facilitated by a strong therapeutic alliance, trust in the therapist, and therapeutic exercises. The study indicates that trust may be a prerequisite to clients’ experience of spiritual qualities in therapy. The interrelationships in group settings can facilitate sacred moments if led by a spiritually legitimated and trustful therapist. Our findings emphasise the value of working therapeutically with sacred relational aspects in psychotherapy in highly secularised countries as Denmark.
The Factor Determinants of Regional Origional Revenue in West Sumatra Province

This study aims to determine the effect of Labor Force Participation Rate, Number of Motorized Vehicles, Number of Small and Medium Enterprises, number of tourist Visits, average per capita expenditure, Room Occupancy Rate, and Regional Original Revenue in 19 Cities and Regencies in West Sumatra in 2005-2018. The model used is the OLS method using panel data and using the statistical program e-views 10 for Windows. The results of the study showed either partially or simultaneously that: Labor Force Participation Rate, Number of Motorized Vehicles, Number of Small and Medium Enterprises, Number of Tourist Visits, Average per capita expenditure and Room Occupancy Rate to Regional Original Income are positive and significant. The largest contribution to Regional Original Revenue is Motorized Vehicles, while the lowest is the Hotel Room Occupancy Rate. The coefficient of determination or goodness of fit is 0.742869. The contribution of all independent variables in explaining the dependent variable is 74.29 percent. Other variables outside the research model explain the remaining 25.71 percent.