Miracle-dz/UniFAR-FTUs huggingface dataset:
Laboratory and Molecular Characteristics of Recessive von Willebrand Disease Type 2C (2A Subtype IIC) of Variable Severity due to Homozygous or Double Heterozygous Mutations in the D1 and D2 Domains

The detection of even tiny amounts of von Willebrand factor (VWF):antigen after desmopressin treatment or in hidden sites like platelets allows the differentiation between patients with recessive von Willebrand disease (VWD) type 3, severe type 1, and 2C (2A subtype IIC). Recessive VWD 2C of various severity displays a characteristic multimeric pattern with pronounced dimer band, absence of triplet structure and lack of large multimers not due to increased proteolysis. Recessive VWD type 2C (2A subtype IIC) is caused by homozygosity or double heterozygosity of missense mutations in the D1 and D2 domains of the VWF propeptide (pp) that catalyzes the multimerization in the D3 domain at the N terminus of mature VWF. In expression studies of recombinant mutant VWF, secretion of VWF mainly consisted of dimers which failed to form intermediate- and high-molecular-weight multimers consistent with the clinical diagnosis of VWD 2C (2A subtype IIC). Carriers of a heterozygous missense mutation in the VWFpp region (D1–D2 domain) of the VWF gene may present mild VWD type 1 and show a typical multimeric pattern with a heavy predominance of VWF dimers.
On the Robust Control of DC-DC Converters: Application to a Hybrid Power Generation System

Abstract In this paper a complete robust control synthesis is performed for a hybrid power generation structure composed by a Fuel Cell and a Supercapacitor. The control strategies are applied to the DC-DC boost power converters associated to each power source. Multivariable PI control with H ∞ performance, H ∞ full and reduced order controllers are designed and compared. The multivariable PI controller is designed through an optimization procedure based on solving some Linear Matrix Inequalities. A μ-analysis and frequency/time response performances results shows the advantages of the different proposed control strategies.
The fleeting nature of the prediction of college academic success.

REPORTS DATA RELATING FRESHMEN PREDICTORS TO INDEPENDENTLY COMPUTED GRADE-POINT AVERAGES FOR EACH OF THE 8 SEMESTERS OF UNDERGRADUATE COLLEGE RESIDENCE. A SUBSTANTIAL AMOUNT OF INSTABILITY OF INTELLECTUAL PERFORMANCE OVER THIS 4-YR TIME SPAN IS REVEALED. IMPLICATIONS FOR COLLEGE ADMISSION RESEARCH AND FOR POLICIES GOVERNING FAILURE AND PROBATION ARE DISCUSSED. (PsycINFO Database Record (c) 2006 APA, all rights reserved)
Chattering-free robust nonlinear controller for an interior permanent magnet synchronous motor speed drive system

A chattering-free robust nonlinear controller is proposed for the speed control of an IPMSM drive system to compensate for the parameter uncertainties and load disturbances. First, the input-output feedback linearization technique is used with a load torque estimator. Next, the upper bounds of the uncertainties are defined in design of the chattering-free robust controller. After that, in order to derive the chattering-free nonlinear robust control laws without reducing the benefits of the sliding mode control, a new method using the sliding manifold information is proposed. Asymptotic stability of the proposed control method is proven by Lyapunov stability theory and Barbalat's lemma. A digital signal processor, TMS320LF2407, is used to implement the proposed control scheme. The experimental results show that the proposed system can effectively reduce the chattering phenomenon and has fast transient responses, good load disturbance rejection responses, and good tracking responses.
Humidity Detection Using Metal Organic Framework Coated on QCM

Quartz crystal microbalance (QCM) coated with poly-4-vinylpyridine (PVP) and metal organic framework HKUST-1 are investigated and compared for humidity sensing. Drop casting method is employed to coat the PVP and HKUST-1 solutions onto the surface of a quartz crystal microbalance. The resonance frequencies of these sensors with varying relative humidity (RH) from 22% RH to 69% RH are measured using impedance analysis method. The sensitivity, humidity hysteresis, response, and recovery times of these sensors are studied. The sensitivities of uncoated, PVP, and HKUST-1 coated QCM sensors are 7 Hz, 48 Hz, and 720 Hz, respectively, in the range of 22% RH–69% RH. The extraction of desorption rate and adsorption energy associated with the adsorption and desorption of water molecules on these surfaces reveals that HKUST-1 has better sensing properties than PVP and uncoated QCM sensors. In this work, the HKUST-1 coated QCM is shown to be a promising material for moisture detection.
Runtime Assertion Checking with the XJML tool

Since the conception of the Java Modeling Language (JML), many tools, applications and implementations have been done for its support. In this context, the users or developers who want to use JML seem surrounded by many of these tools, applications and so on. Looking for a common infrastructure and an independent language to provide a bridge between these tools and JML, it was developed an approach to embedded contracts in the eXtensible Markup Language (XML) for Java: XJML. XJML claims to offer you the ability to separate preconditions, posconditions and class invariants using JML and XML, and then execute Runtime Assertion Checking (RAC), Extended Static Checking (ESC) and/or Full Static Program Verification (FSPV). In this work, we present some experiments and results with XJML and Runtime Assertion Checking, using one Java class.
PSEISMIC: A personalized self-exciting point process model for predicting tweet popularity

Social networking websites allow users to create and share a variety of items. Big information cascades of post resharing can be generated because users of these sites reshare each other's posts with their friends and followers. In this work, we aim at predicting the final number of reshares for any given post. We build on the theory of self-exciting point processes to develop a statistical model, PSEISMIC, which leads to accurate predictions of popularity. Moreover, we perform cluster analysis to group all tweets so that the coefficient of memory kernel in PSEISMIC can be estimated for every cluster, rather than using the same memory kernel. Experiments conducted on a large-scale retweet dataset show that the proposed PSEISMIC model outperforms the state-of-the-art approach, SEISMIC in predicting the popularity of a given post.
Ethanol formation in adh0 mutants reveals the existence of a novel acetaldehyde-reducing activity in Saccharomyces cerevisiae

A strain of Saccharomyces cerevisiae has been constructed which is deficient in the four alcohol dehydrogenase (ADH) isozymes known at present. This strain (adh0), being irreversibly mutated in the genes ADH1, ADH3, and ADH4 and carrying a point mutation in the gene ADH2 coding for the glucose-repressible isozyme ADHII, still produces up to one third of the theoretical maximum yield of ethanol in a homofermentative conversion of glucose to ethanol. Analysis of the glucose metabolism of adh0 cells shows that the lack of all known ADH isozymes results in the formation of glycerol as a major fermentation product, accompanied by a significant production of acetaldehyde and acetate. Treatment of glucose-growing adh0 cells with the respiratory-chain inhibitor antimycin A leads to an immediate cessation of ethanol production, demonstrating that ethanol production in adh0 cells is dependent on mitochondrial electron transport. Reduction of acetaldehyde to ethanol in isolated mitochondria could also be demonstrated. This reduction is apparently linked to the oxidation of acetaldehyde to acetate. Preliminary data suggest that this novel type of ethanol formation in S. cerevisiae is associated with the inner mitochondrial membrane.
Factor Structure of Social Support and Its Relationship To Minor Psychiatric Disorders Among Japanese Adolescents

Although social support has been one of the preferred topics in health psychology for the past two decades, there still remains controversy about its construct and the measurement model for it. The People In Your Life (PIYL) scale, a self-report adaptation of Henderson et al.'s Interview Schedule for Social Interactions (1980), appears to be an outgrowth of the recent developments in this domain. We administered PIYL to two samples, 550 and 434 each, of Japanese adolescents and examined its factor structure, its psychometric properties and its concurrent relationship to minor psychiatric disorders. Of the original four subscales, the presence of two subscales called availability of social integration and availability of attachment was confirmed, while the remaining subscales called adequacy of social integration and adequacy of attachment were found to form one general factor of perceived adequacy. Such a three-factor model appeared to fit the data better, psychometrically more sound and externally more valid in accounting for the minor psychiatric disorders.
Combined Administration of a Mutant TGF-β1/Fc and Rapamycin Promotes Induction of Regulatory T Cells and Islet Allograft Tolerance

The critical roles of TGF-β in the reciprocal differentiation of tolerance-promoting CD4+Foxp3+ regulatory T cells (Tregs) and proinflammatory Th17 effector cells affect alloimmune reactivity and transplant outcome. We reasoned that a strategy to harness TGF-β and block proinflammatory cytokines would inhibit the differentiation of Th17 cells and strengthen the cadre of Tregs to promote tolerance induction and long-term allograft survival. In this study, we report the development of a long-lasting autoactive human mutant TGF-β1/Fc fusion protein that acts in conjunction with rapamycin to inhibit T cell proliferation and induce the de novo generation of Foxp3+ Treg in the periphery, while at the same time inhibiting IL-6–mediated Th17 cell differentiation. Short-term combined treatment with TGF-β1/Fc and rapamycin achieved long-term pancreatic islet allograft survival and donor-specific tolerance in a mouse model. This effect was accompanied by expansion of Foxp3+ Tregs, enhanced alloantigen-specific Treg function, and modulation of transcript levels of Foxp3, IL-6, and IL-17. Our strategy of combined TGF-β1/Fc and rapamycin to target the IL-6–related Tregs and Th17 signaling pathways provides a promising approach for inducing transplant tolerance and its clinical application.
The effect of comorbidity on hospital mortality in patients with SLE from an Asian tertiary hospital

Objectives The objective of the study was to assess the disease burden of systemic lupus erythematosus (SLE) and the usefulness of the Charlson Comorbidity Index (CCI) as risk-adjusted hospital mortality predictors in patients with SLE using a hospital administrative database. Methods A historical cohort study of a hospital discharge database from 2004 to 2011 was used to identify cases with SLE and comorbidity using the International Statistical Classification of Diseases and Related Health Problems, ninth revision, Australian modification (ICD-9-AM) codes. Results Over the eight years, 841 patients met the criteria of SLE with a hospital mortality rate of 9.2%. The hospital mortality rates (2.4%, 15.7%, 25.0%, and 30.4%, respectively, p < 0.001) and hospital length of stay (geometric mean, 3.5, 5.6, 8.8, and 7.5 days, respectively, p < 0.001) were consistently increased for patients with CCI ranging from none, low, moderate to high grade, respectively. Cox proportional hazards model analysis showed that CCI (hazard ratio (HR) 7.8 high vs. none, p < 0.001) and infectious disease (HR 2.0, p = 0.016) were significant and independent predictors of hospital mortality. Similar results were also seen with hospital length of stay by zero-truncated negative binomial regression model analysis. Conclusion The SLE burden is high in this population. Comorbidities and infectious disease were some of the most important contributors to hospital mortality and resource utilization.
Microwave radiation: An alternative method to sinter utilitarian porcelain

Microwave heating has been widely used in the industry to synthesize dielectric materials. The electromagnetic field distribution is the major factor that influences how the materials are sintered in a microwave oven. To calculate the different distributions is then crucial to understand the complex phenomenon of the dielectric heating under a microwave radiation. In this work we demonstrate the viability to use microwave radiation to sinter utilitarian porcelain at 2.45 GHz, in a multimode cavity oven, with a nominal power of 1 kW. To characterize the quality of the final product we measured different physical properties, as rupture energy, impact resistance, porosity, water absorption and concentration of undesired elements, and we compared the results with that obtained with sintering by conventional methods. Finally, we observed that the sintering process is faster, with lower costs and lower emissions of harmful gases into the atmosphere.
MESFET Noise Model based on Three Equivalent Temperatures

A completed model for the prediction of MESFET noise parameters by using the standard microwave circuit simulator is proposed in this paper. Starting from the noise model with two noise sources and two corresponding equivalent temperatures, the correlation between the noise sources in the model is taken into account in a new way, by assigning an additional complex parameter named equivalent correlation temperature. A set of equation describing the noise parameters as the function of equivalent circuit elements as well as three equivalent temperatures is derived. The proposed procedure is implemented within the circuit simulator Libra and applied to a number of commercially available MESFETs. The simulated noise parameters agree very well with referent data.
Threshold levels of Flt3‐ligand are required for the generation and survival of lymphoid progenitors and B‐cell precursors

The generation of B‐cell precursors (BCP) from lymphohematopoietic progenitors (LHP) in bone marrow is dependent on signals provided by the receptor tyrosine kinase Flt3 and its ligand, Flt3‐ligand (FL). Mice deficient in FL exhibit striking reductions in LHP and BCP. Currently, the mechanism by which Flt3 regulates lymphoid lineage/B‐cell development is unknown. Here, we show that haploinsufficiency of FL (FL+/−) reduced the numbers of LHP, common lymphoid progenitors, and pro‐B cells, suggesting that FL levels set a threshold for B lymphopoiesis. Limiting dilution analysis confirmed reduced BCP frequency in FL+/− mice. Real‐time PCR of LHP from FL+/− animals showed increased transcripts of the B lineage inhibitor id1. However, targeted deletion of id1 did not restore the lymphoid/B lineage deficiencies in FL−/− mice, supporting Id1‐independent mechanisms. BrdU incorporation studies established that FL is not essential for the proliferation of Flt3+ multipotential progenitors. Analysis of FL−/− progenitors expressing low levels of Flt3 revealed decreased levels of the pro‐survival factor Mcl1. Consequently, the Flt3+ LHP progeny of Flt3low LSK+ cells exhibited increased Annexin V staining. Together, these data suggest that Flt3 signaling initiates a cascade of events in Flt3low precursors that promote the survival of LHP from which BCP are derived.
The Development of Hospital Library Consortia and the Legacy of the Chicago and South Consortium

Consortia have a special role in the development of hospital libraries and the development of hospital librarians. Holst and Bensing, and later Lessick, discussed the importance of consortia in Interlibrary Loan services in health care libraries in the 1st and 2nd editions of The Medical Library Association Guide to Managing Health Care Libraries, but discussions of hospital library consortia have generally dropped out of sight in the literature in the past 30 years (1, 2). Beyond the original mission of sharing resources, hospital library consortia have evolved to fill other needs and goals, influenced by technological advances in health care and the publishing worlds. The Chicago and South Consortium (CSC) is a great example of a successful hospital library consortium that has evolved with the changing times. Founded in 1972, the Consortium is now a certain age and still going strong.
Attribution of Blame for Criminal Acts and its Relationship with Type of Offence

ABSTRACT: This paper examines the relationship between type of offence and attribution of blame by offenders for their criminal activity. The Gudjonsson Blame Attribution Inventory (GBAI) was administered to 139 prisoners at Grendon Psychiatric Prison, and 37 psychiatric patients with a forensic history. As predicted, a significant relationship with type of offence was found. Subjects who had committed sexual offences reported the strongest remorse for their offence, and those who had committed violent offences (e.g. homicide, grevious bodily harm) were more likely to attribute their offence to mental factors (e.g. loss of self-control, depression).
SUBSTRATE SPECIFICITY OF A BACTERIAL UV ENDONUCLEASE AND THE OVERLAP WITH IN VITRO PHOTOENZYMATIC REPAIR

Abstract— The action of an endonuclease from Micrococcus luteus, that operates on ultraviolet (UV) radiation damage, overlaps greatly with that of the yeast photoreactivating enzyme: homo and hetero cyclobutyl pyrimidine dimers in DNA are substrate for both enzymes, but pyrimidine adducts or the ‘spore photoproduct’ in DNA are not.
Recovery from Sevoflurane Anesthesia: A Comparison to Isoflurane and Propofol Anesthesia

Backgroud Sevoflurane has a lower blood:gas partition coefficient than isoflurane, which may cause a more rapid recovery from anesthesia; it also might cause faster emergence times than for propofol‐based anesthesia. We evaluated a database that included recovery endpoints from controlled, randomized, prospective studies sponsored by Abbott Laboratories that compared sevoflurane to isoflurane or propofol when extubation was planned immediately after completion of elective surgery in adult patients. Methods Sevoflurane was compared to isoflurane in eight studies (N = 2,008) and to propofol in three studies (N = 436). Analysis of variance was applied using least squares method mean values to calculate the pooled mean difference in recovery endpoints between primary anesthetics. The effects of patient age and case duration also were determined. Results Sevoflurane resulted in statistically significant shorter times to emergence (‐3.3 min), response to command (‐3.1 min), orientation (‐4.0 min) and first analgesic (‐8.9 min) but not time to eligibility for discharge (‐1.7 min) compared to isoflurane (mean difference). Times to recovery endpoints increased with increasing case duration with isoflurane but not with sevoflurane (patients receiving isoflurane took 4‐5 min more to emerge and respond to commands and 8.6 min more to achieve orientation during cases longer than 3 hr in duration than those receiving sevoflurane). Patients older than 65 yr had longer times to orientation, but within any age group, orientation was always faster after sevoflurane. There were no differences in recovery times between sevoflurane and propofol. Conclusions Recovery from sevoflurane was 3‐4 min faster than with isoflurane in all age groups, and the difference was magnified in longer‐duration surgical cases (> 3 hr).
Regulation of Hepatic Drug Transporter Activity and Expression by Organochlorine Pesticides

Organochlorine (OC) pesticides constitute a major class of persistent and toxic organic pollutants, known to modulate drug‐detoxifying enzymes. In the present study, OCs were demonstrated to also alter the activity and expression of human hepatic drug transporters. Activity of the sinusoidal influx transporter OCT1 (organic cation transporter 1) was thus inhibited by endosulfan, chlordane, heptachlor, lindane, and dieldrine, but not by dichlorodiphenyltrichloroethane isomers, whereas those of the canalicular efflux pumps MRP2 (multidrug resistance‐associated protein 2) and BCRP (breast cancer resistance protein) were blocked by endosulfan, chlordane, heptachlor, and chlordecone; this latter OC additionally inhibited the multidrug resistance gene 1 (MDR1)/P‐glycoprotein (P‐gp) activity. OCs, except endosulfan, were next found to induce MDR1/P‐gp and MRP2 mRNA expressions in hepatoma HepaRG cells; some of them also upregulated BCRP. By contrast, expression of sinusoidal transporters was not impaired (organic anion‐transporting polypeptide (OATP) 1B1 and OATP2B1) or was downregulated (sodium taurocholate co‐transporting polypeptide (NTCP) and OCT1). Such regulations of drug transporter activity and expression, depending on the respective nature of OCs and transporters, may contribute to the toxicity of OC pesticides.
Review of Rate Regions for Interference Channels

Interference affects many types of communication channels including digital subscriber lines and wireless links. A basic model for studying coding for such scenarios is known as the interference channel (IC). Several of the fundamental results known about ICs are reviewed with special emphasis on Gaussian channels. We further discuss four recent results including an improvement of a standard achievable rate region
Clinicopathological study on hepatocellular carcinoma with lymphocytic infiltration

We examined the clinicopathologic features of 11 surgically resected hepatocellular carcinomas (HCCs) less than 3 cm in diameter with marked inflammatory cell infiltration (LHCCs). In comparison with the other 152 HCCs without such an infiltration (controls), there were no significant differences in male/female ratio, age, serum α‐fetoprotein levels, and laboratory and imaging findings. All the 11 LHCC cases were hepatitis B surface antigen (HBsAg) negative and hepatitis C virus antibody positive. Among the 152 controls, 116 cases were also HBsAg negative and HCVAb positive and were referred to as HCV‐only controls. The clinical features were not significantly different between the LHCC and the HCV‐only controls. The LHCC group tended to have higher numbers of lymphocytes and monocytes in pre‐ and post‐operative peripheral blood, but there were no significant group differences. Recurrence rate was 9.1% in the LHCC group, 47.7% in the controls and 47.5% in the HCV‐only controls (P < .01). Five‐year survival rate was 100% in the LHCC group, 65.1% in the controls and 68.1% in the HCV‐only controls (P < .01). Histologically, remarkable inflammatory cell infiltration, mostly lymphocytic, was observed in the cancerous tissue of the LHCC group. Varying degrees of piecemeal necrosis of cancer nests produced by infiltrating lymphocytes were observed in all the 11 cases. Lymph follicle formation was also found in 10 of 11 cases (90.9%). Liver cirrhosis was associated in 6 LHCC cases (54.5%), in 117 control cases (77.0%), and in 91 HCV‐only controls (78.4%). Tumor invasion into the portal vein in the vicinity of the tumor was found in 1 LHCC case (9.1%), in 54 controls (35.5%), and in 34 HCV‐only controls (29.3%). Immunohistochemically, most of the infiltrating lymphocytes, other than those in the lymph follicle, were identified as T lymphocyte, and CD8+ T lymphocyte was more predominant than CD4+ T lymphocyte. Better prognosis of the LHCC group could attribute to the anti‐tumor effect induced by cellular immunity of CD8+ and CD4+ T lymphocytes, and partly by humoral immunity of B cells which formed lymph follicles.