Miracle-dz/UniFAR-FTUs huggingface dataset:
Critical aneurysmal dilatation of the thoracic aorta in young adolescents with variant hyperimmunoglobulin E syndrome

The autosomal‐dominant (AD) form of the hyperimmunoglobulin E syndrome (HIES) has been described as a multisystem disorder including immune, skeletal and dental abnormalities. Recently, the evaluation of patients from families in which HIES was inherited in a manner more consistent with autosomal‐recessive (AR) inheritance, showed that AR‐HIES is a clinically distinct disease entity. In addition to classical immunologic findings of AD‐HIES, the AR form presents with severe recurrent fungal and viral infections with herpes zoster, herpes simplex and characteristic mollusca contagiosa. Furthermore, cerebral vascular sequelae, including vasculitis, infarction and haemorrhage were noted. In this report, we describe the clinical picture of two patients who showed remarkable resemblance to the description of AR‐HIES, but also developed fatal aneurysmal dilatation of the thoracic aorta in adolescence. This finding may further consummate the clinical picture of AR‐HIES and emphasize the possibility to develop early aortitis, most likely preceding the critical aneurysm formation at older age. This process should be anticipated during childhood in cases with AR‐HIES.
Emperor’s New Clothes

Publication in top journals has become an indicator of academic performance that has huge influence on academic careers, on research funding, and on institutional rankings. Pressure to publish in these journals is immense. Pre-publication peer review, never without its critics, cannot cope. Yet, more depends on this peer review than ever before. The activities of academic publishers, governments, research councils, and universities are all ultimately supported by a system that is heavily gamed and cannot withstand scrutiny. The article compares the practice of peer review with its replacement, the peer review of myth. Myth, like religion, is dependent on faith, not logic. The article looks closely at the transition, focusing particularly on management studies. It explores how the practice of peer review, with all its deficiencies, has been superseded by the peer review of myth, with no deficiencies at all. Vested interests profit, but academic research suffers.
Reading private green space: Competing geographic identities at the level of the lawn

This paper focuses on private residential green space as a site of contested meanings. Recent research points to the emergence of an activism centered on ecological restoration and a shift away from the lawn as the only accepted landscape practice for private green space. However, it is clear that the lawn, a particularly powerful cultural landscape form in residential neighborhoods, still largely dominates this space across North America. This investigation examines the voices of two groups: traditional lawn owners and ecological activists. We observe two sets of discourses centered on private green space. Both groups construct residential green space as a site of identity politics-a site wherein the self is defined as pure and the other excluded as different and necessarily inferior. And both perceive their discourse as ''natural. '' The critical finding is that they are almost entirely oppositional discourses. The contest over what constitutes appropriate landscaping practices for this space provides a locus for bringing to a discursive level, the kinds of socio-cultural perspectives and practices that create and dominate our places in late capitalist society. We suggest that at present the lawn remains a barrier to alternative green space practices.
The changing face of familial colorectal cancer

Young patients with colorectal cancer should be assessed for genetic predispositions C olorectal cancer is predominantly a disease of elderly people in the developed world. The annual incidence of more than 35 000 cases in the United Kingdom means that many people have at least one affected relative. Colorectal cancer in elderly relatives may be due to shared environmental exposure rather than a true genetic predisposition, although the risk of developing colorectal cancer rises with the number of relatives affected. Genetic factors may have a role in up to 30% of cases,1 but only a small proportion (less than 5%) of colorectal cancers arise in families with strong histories in which tumours develop at a young age (less than 50 years) and represent truly high risk inherited predispositions. Even so, more than 1500 families per year need to be counselled and screened. Most hereditary colorectal cancers are attributable to two recognised syndromes. Familial adenomatous polyposis usually has a clear phenotype, characterised by numerous (more than 100) adenomatous polyps in the large bowel by the second to third decades of life, which inevitably progress into cancers. This autosomal dominant condition, caused by APC gene mutations, has been a paradigm for the …
The Issues Deserve More Credit

This paper explores the `issue-oriented' perspective on public involvement in politics opened up by recent research in Science and Technology Studies (STS). This research proposes that public controversy around techno-scientific issues is dedicated to the articulation of these issues and their eventual accommodation in society. It does not, however, fully answer the question of why issue formation should be appreciated as a crucial dimension of democratic politics. To address this question, I turn to the work of two early 20th-century American pragmatists: John Dewey and Walter Lippmann. In their work on democracy in industrial society, they conceived of public involvement in politics as being occasioned by, and providing a way to settle, controversies that existing institutions were unable to resolve. Moreover, Dewey developed a `socio-ontological' understanding of issues, which suggests that people's involvement in politics is mediated by problems that affect them. Dewey and Lippmann thus provide important argumentative resources for further elaborating the approach to public involvement developed in STS. STS research has also developed a `socio-ontological' approach, as it focuses on the `attachments' that people mobilize (and that mobilize people) in the performance of their concern with public affairs. Such an approach provides an alternative to discursivist analysis of the role of `issue framing' in the involvement of publics in politics.
RNA folding and combinatory landscapes.

In this paper we view the folding of polynucleotide (RNA) sequences as a map that assigns to each sequence a minimum-free-energy pattern of base pairings, known as secondary structure. Considering only the free energy leads to an energy landscape over the sequence space. Taking into account structure generates a less visualizable nonscalar landscape,'' where a sequence space is mapped into a space of discrete shapes. '' We investigate the statistical features of both types of landscapes by computing autocorrelation functions, as well as distributions of energy and structure distances, as a function of distance in sequence space. RNA folding is characterized by very short structure correlation lengths compared to the diameter of the sequence space. The correlation lengths depend strongly on the size and the pairing rules of the underlying nucleotide alphabet. Our data suggest that almost every minimum-free-energy structure is found within a small neighborhood of any random sequence. The interest in such landscapes results from the fact that they govern natural and artificial processes of optimization by mutation and selection. Simple statistical model landscapes, like Kauffman and Levin's [ital n]-[ital k] model [J. Theor. Biol. 128, 11 (1987)], are often used as a proxy for understanding realistic landscapes, likemore » those induced by RNA folding. We make a detailed comparison between the energy landscapes derived from RNA folding and those obtained from the [ital n]-[ital k] model. We derive autocorrelation functions for several variants of the [ital n]-[ital k] model, and briefly summarize work on its fine structure. The comparison leads to an estimate for [ital k]=7--8, independent of [ital n], where [ital n] is the chain length. While the scaling behaviors agree, the fine structure is considerably different in the two cases. « less
Serotonergic Drug Treatment of Deviant Sexual Interests

The clinical English language literature on sexual deviance and treatment with serotonergic medications is reviewed. Evidence is presented to support the hypothesis that serotonergic medications decrease deviant sexual interests without impairing non-deviant sexual interests. Criteria for selecting which medication to prescribe are reviewed and the clinical data on response to treatment, time to treatment response, and relapse are summarized. Specific recommendations for the treatment of sexual deviance with serotonergic medications are given. Finally, recommendations for future research are described.
Middle-Class Families and School Choice: Freedom versus Equity in the Context of a ‘local Education Market’

This article analyses the impact of social class on the process of school choice in Spain from the viewpoint of middle-class families. This practice must be seen in the framework of the new social context generated by the information society. The article begins by briefly describing changes in school choice policies in Spain. For a wider understanding, these changes are related to the broader process of the marketisation of education, which has been taking place in this country for the last 20 years. In the second part of the article, qualitative data are presented on how middle-class parents understand and experience the process of choosing a particular school for their children. In this study, 24 interviews were carried out in the city of Granada (southern Spain). They were fully transcribed and analysed using the methodology suggested by ‘grounded theory’ and Bourdieu's concept of social class. Aspects related to social and cultural capital are added to the traditional social class indicators, which are usually expressed in economic terms. Attention centres on the creation of reconversion strategies for these different forms of capital used by families to obtain positional advantage for their children. This research thus revisits the principles of social reproduction theories from the viewpoint of the ‘winners’, i.e. middle-class families.
Closed-Form Critical Conditions of Instabilities for Constant On-Time Controlled Buck Converters

A general instability critical condition in terms of the loop gain is derived for the constant on-time controlled buck converter. The instability is associated with a discrete-time pole at -1. Given an arbitrary control scheme, a systematic procedure is proposed to derive the critical condition, which also shows the required ramp slope to stabilize the converter. Different control schemes are shown to have similar forms of critical conditions. Some previously known critical conditions become special cases in this generalized framework, and their inaccuracies are identified.
Dead-time compensation method of SVPWM based on DSP

In direct torque control and vector control, the voltage source inverter (VSI) was controlled based on Space-vector pulse width modulation (SVPWM). The SVPWM is an excellent control strategy, but the relation between reference voltage and actual output voltage is nonlinear due to the dead time effect and the voltage drop of power devices. The distortion of voltage invokes serious problems such as current waveform distortion and deterioration of the systems. An improved method which reduces the distortion of voltage and current in induction motor control system is presented. The method can be implemented with software without any extra hardware. The experiment results verify this method has a good compensation effect.
Human Papillomavirus Is Associated with Breast Cancer in the North Part of Iran

We have analyzed the possible relevance of HPV infection for breast cancer risk among Iranian women from north part of Iran. Among women with breast cancer, 25.9% had positive test results for HPV DNA in breast tumor samples in contrast to 2.4% of women with noncancer status (P = 0.002). The infection of HPV has increased the risk of breast cancer (OR 14.247; 95% CI 1.558–130.284, P = 0.019). The high-risk HPV genotypes (types 16 and 18) in samples of breast cancer patients were the predominant types (53.34%). Other genotypes detected in breast cancer were HPV-6, HPV-11, HPV-15, HPV-23, and HPV-124, and one isolate could not be genotyped compared to HPV reference sequences. While the sole detected HPV in control specimens was HPV-124. Our study reveals that HPV infection and age are the risk factors in breast cancer development in the north part of Iran.
Foramen magnum cerebrospinal fluid flow characteristics in children with Chiari I malformation before and after craniocervical decompression.

OBJECT The Chiari I malformation presents significant challenges to clinicians because its pathophysiology is not well understood. In conducting cerebrospinal fluid (CSF) flow studies, investigators have attempted to correlate the clinical severity of these lesions with general flow velocity or bulk flow at the foramen magnum; however, these techniques have not allowed consistent prediction of symptomatology, explanation of the presence of syringomyelia, or the assessment of the hydrodynamic characteristics of the decompression. The authors used temporally and spatially resolved flow analyses to assess the characteristics of CSF flow in children with Chiari I malformation and the changes in these flow characteristics that occur after suboccipital decompression. METHODS The authors studied eight children with symptomatic Chiari I malformation with or without syringomyelia and two children without Chiari I malformation. All patients underwent phase-contrast magnetic resonance imaging before and after posterior fossa decompression. Velocity plots were displayed for each voxel. Several indices of CSF flow were developed to characterize the flow patterns associated with Chiari I malformation. In children with symptomatic Chiari I malformation, even though bulk flow or velocity is often normal, there was marked heterogeneity of flow at the foramen magnum. This was evident for several reasons: 1) an increase in cephalad and caudad peak velocities; 2) spatial inhomogeneity in velocities; 3) simultaneous bidirectional flow; and 4) substantial net craniad or caudad flows within particular voxels and subregions during the cardiac cycle. After posterior fossa decompression, the severity of these flow abnormalities decreased. CONCLUSIONS Foramen magnum CSF flow in children with symptomatic Chiari I malformations is spatially and temporally heterogeneous, and this heterogeneity improves postoperatively. The authors propose that relying on mean flow parameters in patients with Chiari I malformation is no longer sufficient; instead, more elaborate techniques to analyze foramen magnum CSF flow have become necessary.
Triggering Transference: Examining the Role of Applicability in the Activation and Use of Significant-Other Representations in Social Perception

Prior research has shown that different sources of knowledge activation may combine to trigger transference, the phenomenon whereby a significant-other representation is activated and used to interpret a new person, as assessed in terms of representation-consistent memory about the person (Andersen, Glassman, Chen, & Cole, 1995). The central prediction of the present study was that increasing levels of applicability sources of activation would produce corresponding increases in the extent to which significant-other representations are activated and used in social perception, combining with the previously documented chronic accessibility of these representations (Andersen et al., 1995). Applicability levels were manipulated in terms of the degree of featural overlap between a target person and a perceiver's significant-other representation. Across all six applicability levels examined, greater representation-consistent memory was seen on the basis of a significant-other representation relative to several c.
Mining Motifs from Human Motion

Mining frequently occurring temporal motion patterns (motion motifs) is importa nt for understanding, organizing and retrieving motion data. However, without any a priori knowledge of th e motifs, such as their lengths, contents, locations and total number, it remains a challenging problem due to the enor mous computational cost involved in analyzing huge motion databases. Moreover, since the same motion motif can exhibit different temporal and spatial variations, it prevents directly applying existing data mining methods tomotion data. In this paper, we propose an efficient motif discovery method which can handle both spatia l and temporal variations of motion data. We translate the motif discovery problem into finding continuous paths ina matching trellis, where each continuous path corresponds to an instance of a motif. A tree-growing me thod is introduced to search for the continuous paths constrained by a branching factor, and to accommodate intra-pattern variations of motifs. By using locality-sensitive hashing (LSH) to find the approximate matches and b the trellis, the overall complexity of our algorithm is only sub-quadratic to the size of the database, and is of lin ear memory cost. Experimental results on a data set of 32, 260 frames show that our method can effectively discover meaningful motion mo tifs regardless of their spatial and temporal variations.
miR-195 inhibits cell proliferation via targeting AEG-1 in hepatocellular carcinoma

Emerging evidence has indicated that microRNAs (miRNAs) are frequently dysregulated and are fundamental in the pathogenesis of hepatocellular carcinoma (HCC). However, the roles of miR-195 in HCC have not been well elucidated. In the present study, the expression of miR-195 was determined to be markedly downregulated in HCC tissues and cell lines, as compared with normal liver cells. Restoration of miR-195 expression resulted in significant inhibition of the proliferation and tumorigenicity of HCC cells in vitro and in vivo. Gene expression data and luciferase reporter assays revealed that miR-195 is able to directly inhibit the expression of astrocyte elevated gene 1 (AEG-1) through interaction with its 3′ untranslated region. Consistently, an inverse correlation between miR-195 and AEG-1 expression was observed in HCC tissues. Furthermore, the overexpression of AEG-1 was able to partially attenuate the miR-195-induced inhibition of cell growth and promotion of apoptosis. Taken together, these findings indicate that miR-195 functions as a tumor suppressor by inhibiting AEG-1. This pathway may provide new insights into the potential molecular mechanisms of HCC.
A double comparative study: Process models and student skills

We report on a recent experience in our Software Engineering laboratory course. More than one hundred students, from two different degrees, were divided in teams grouping diverse competences and skills, and had to develop four software products using a process model chosen among Waterfall, Spiral, and Extreme Programming. The two degrees involved were Computer Science and Informatics for Management. The former students have a standard, strong, background in programming, systems, and algorithms, while the latter students have a weaker training in programming compensated by some courses in Economics and Management. This situation allowed us to perform a double comparative study: on one hand, analyzing the laboratory results we obtained a comparison of the process models, which reproduced some results already known in literature. On the other hand we could compare “on the field” the different skills of students following different curricula, especially with respect to the requirements of the different process models. We discuss the results of this experience.
On distributed delay in linear control Laws-part I: discrete-delay implementations

This note proposes two approaches to approximate distributed delay in linear control laws and, furthermore, to implement it in the z-domain and in the s-domain. The H/sup /spl infin//-norm of the approximation error converges to 0 when the number N of approximation steps approaches +/spl infin/. Hence, the instability problem due to the approximation error, which has been widely studied in recent years, does not exist provided that N is large enough. Moreover, the static gain is guaranteed so that no extra efforts are needed to retain the steady-state performance. As by-products, two new formulas for the forward and backward rectangular rules are obtained. These formulas are more accurate than the conventional ones when the integrand has an exponential term.
Appendix E for "Generalized Method of Moments with Tail Trimming"

If > 4 it is easy to show the Jacobian and covariance terms are asymptotically bounded T and JT J , hence T = T = KT . We only consider 2 (2; 4) since the case = 4 follows similarly. Under the stated conditions and Theorem 3.1 of Basrak et al (2002: Theorem 3.1) each zt 2 fyt; htg has regularly varying tail P (jztj > z) = dzz z (1 + o (1)) : (A.1) Note h 2 t is bounded, and h 2 t y 2 t 1 is bounded if > 0 and otherwise has tail index =2 and is independent of t . Therefore the QML equations have tail (A.1) by the same convolution argument above (Cline 1986):
Powerful Pacifists: Democratic States and War

Democracies are less likely to fight wars with each other. They are also more likely to prevail in wars with autocratic states. I offer an explanation of this syndrome of powerful pacifism drawn from the microeconomic theory of the state. State rent seeking creates an imperialist bias in a country's foreign policy. This bias is smallest in democracies, where the costs to society of controlling the state are relatively low, and greatest in autocracies, where the costs are higher. As a result of this bias, autocracies will be more expansionist and, in turn, war-prone. In their relations with each other, where the absence of this imperialist bias is manifest, the relative pacifism of democracies appears. In addition, democracies, constrained by their societies from earning rents, will devote greater absolute resources to security, enjoy greater societal support for their policies, and tend to form overwhelming countercoalitions against expansionist autocracies. It follows that democracies will be more likely to win wars.
Situational differences in dialectical emotions: Boundary conditions in a cultural comparison of North Americans and East Asians

Past research generally suggests that East Asians tolerate opposing feelings or dialectical emotions more than North Americans. We tested the idea that North Americans would have fewer opposing emotions than East Asians in positive, but not in negative or mixed situations. Forty-seven European American, 40 Chinese, and 121 Japanese students reported the emotions that a protagonist of standardised positive, negative, and mixed situations would feel. Emotions were coded into three valence categories: pleasant, unpleasant, and neither-pleasant-nor-unpleasant. As predicted, cultural differences in opposing emotion associations were found in positive situations only. Moreover, East Asians reported more neither-pleasant-nor-unpleasant feelings, especially in mixed situations, possibly reflecting a deferral of valence appraisal due to expected change.