Miracle-dz/UniFAR-FTUs huggingface dataset:
Cardiac and Ocular Pathologies in a Mouse Model of Mucopolysaccharidosis Type VI

Mucopolysaccharidosis type VI (MPS VI) is a lysosomal storage disease caused by a deficiency of arylsulfatase B (ASB) which has its function in the sequential degradation of glycosaminoglycans (GAG). Targeted disruption of the ASB gene resulted in a mouse model of MPS VI that has been closely investigated for skeletal and chondral dysplasia. As ocular and cardiac impairment are also clinically important manifestations of the MPS VI syndrome, the present study was initiated for detailed biochemical, histologic and functional analysis of cornea, optic nerve and heart in ASB-deficient mice. Biochemical evidence for GAG-storage could be obtained for liver, kidney, spleen and myocardium as well as for heart valves, cornea and optic nerve from ASB-deficient mice. In MPS VI mice, histology revealed structural impairment of corneal stroma and epithelium as well as a thickening of the heart valves. According to histologic investigations, the optic nerve appeared not to be altered. However, GAG-storage in the dura mater could be demonstrated in MPS VI mice. Heart function was assessed by echocardiography. While the dimensions of MPS VI hearts were not altered, these hearts clearly showed decreased myocardial contraction and a 50% reduction of cardiac output. In addition, insufficiencies in the mitral and aortic valves were detected. Thus, ASB-deficient mice resemble the phenotype of human MPS VI not only in the skeletal but also in the ocular and cardiac symptoms. To our knowledge, these in vivo evaluations of heart function represent the first respective investigation of a MPS VI animal model and should provide a valuable measure for therapy studies in the MPS VI mouse.
Predictors of error in estimates of blood alcohol concentration: a replication.

OBJECTIVE To identify predictors of error in estimated blood alcohol concentration (eBAC) in a sample of bar patrons. METHOD Six hundred sixty-six patrons (43.6% female) were randomly sampled from 32 bars. Patrons were asked to provide a breath sample into a handheld breath alcohol concentration test unit upon entrance and exit from the bar. Patrons also completed a brief survey at entrance and exit. For analyses, this sample was stratified by whether patrons consumed alcohol before attending the bar. Estimates of BAC were calculated using Matthews and Miller's formula (1979). A three-category dependent variable was created based on the estimation accuracy of eBAC relative to breath alcohol concentration: accurate (within .02), underestimate of BAC, and overestimate of BAC. RESULTS Of those that drank before arriving at the bar, 29% of eBACs were accurate, 32.3% were underestimates, and 38.8% were overestimates. For those who drank only at the bar, 42.0% were accurate, 20.8% were underestimates, and 37.7% were overestimates. Among those who drank before attending the bar, the number of drinks consumed before attending the bar was significantly related to eBAC underestimate. Among those who drank only at the bar, predictors of overestimate included being female, drinking more, and drinking longer. CONCLUSIONS The accuracy of eBAC is poor at best. In an earlier study of parties, eBACs were often underestimated; in the bar setting, eBACs were often overestimated. More research is needed to understand the role of setting on eBAC calculations.
Behavioral Assessment and Treatment of Pediatric Feeding Disorders

Pediatric feeding disorders are estimated to occur in as many as one in every four infants and children, and when serious can require numerous, costly and sustained interventions. For over a decade research has cumulated evidence on the contributions of Behavior Analysis in understanding and remediating some types of pediatric feeding disorders. The systematic use of this body of evidence in conjunction with other approaches (medical, nutrition, occupational therapy, physical therapy, and so forth) is being carried out on an inpatient treatment unit at the Kennedy Krieger Institute. Key aspects are described here, including direct observation behavior assessment, approaches for increasing and decreasing feeding behavior, skill acquisition, transfer of treatment gains, and parent training. The results based on case studies and overall program evaluation indicate that medically complicated, severe feeding disorders can be treated successfully in a few months with a multidisciplinary approach which incorporates behavioral procedures. J Dev Behav Pediatr 15:278–291, 1994. Index terms: pediatric feeding disorders, food refusal, failure to thrive, tube dependence.
A Knock‐in Foxj1CreERT2::GFP mouse for recombination in epithelial cells with motile cilia

The transcription factor Foxj1 is expressed by cells destined to differentiate into epithelial cells projecting motile cilia into fluid‐ or air‐filled cavities. Here, we report the generation of an inducible knock‐in Foxj1CreERT2::GFP mouse, which we show reliably induces Cre‐mediated recombination for genetic studies in epithelial cells with motile cilia throughout embryonic and postnatal development. Induction during embryonic stages revealed efficient recombination in the epithelial component of the choroid plexus in the developing brain as early as E12.5. Induction during late embryonic stages showed confined recombination not only in the choroid plexus but also in the ventricular walls of the brain. Recombination induced during postnatal periods expanded to include epithelia of the lungs, testis, oviduct, and brain. Using these mice, we confirmed our recent discovery of a perinatally derived neuronal population in the mouse olfactory bulbs, which is derived from the Foxj1 lineage. Our Foxj1CreERT2::GFP knock‐in mouse will be a powerful tool for studying molecular mechanisms associated with the continuum of cells that form the Foxj1 lineage, and for assessing their physiological significance during development and aging. genesis 52:350–358, 2014. © 2014 Wiley Periodicals, Inc.
Designing a report card for nursing facilities: what information is needed and why.

PURPOSE This article presents a rationale and conceptual framework for making comprehensive consumer information about nursing facilities available. Such information can meet the needs of various stakeholder groups, including consumers, family/friends, health professionals, providers, advocates, ombudsman, payers, and policy makers. DESIGN AND METHODS The rationale and framework are based on a research literature review of key quality indicators for nursing facilities. RESULTS The findings show six key areas for information: (a) facility characteristics and ownership; (b) resident characteristics; (c) staffing indicators; (d) clinical quality indicators; (e) deficiencies, complaints, and enforcement actions; and (f) financial indicators. This information can assist in selecting, monitoring, and contracting with nursing facilities. IMPLICATIONS Model information systems can be designed using existing public information, but the information needs to be enhanced with improved data.
Endothelial Dysfunction in Patients with Asthma: The Role of Polymorphisms of ACE and Endothelial NOS Genes

Objectives. Polymorphisms of the angiotensin converting enzyme (ACE) and endothelial nitric oxide (eNOS) genes have been implicated in asthma pathogenesis. Angiotensin II and NO have important roles in maintaining vascular tone. In this study, the relationship between endothelial dysfunction and ACE and eNOS gene polymorphisms was investigated in patients with asthma. Methods. This cross‐sectional, controlled study was conducted at the Yedikule Chest Disease Hospital and Cardiology Center in a University Hospital. Forty‐nine patients with asthma (18 male, 31 female; mean age: 33 ± 12 years) and 49 age‐ and sex‐matched healthy controls (20 male, 29 female; mean age: 30 ± 8 years) were included. Pulmonary function tests and flow‐mediated dilatation of the brachial artery [endothelium dependent dilatation (EDD)] were examined by high‐resolution ultrasonography. The ACE and eNOS genotypes were determined by PCR. Results. Asthma patients showed lower EDD (12 ± 6% vs. 22 ± 6%, p < 0.001) as compared to controls. The EDD was correlated with both predicted value of FEV1 (r = 0.31, p = 0.04) and predicted value of FVC (r = 0.37, p = 0.013). Conversely, EDD values in patients with moderate asthma were significantly lower than those in patients with mild asthma (10.1 ± 5.2% vs. 14.1 ± 5.7%, p = 0.017). However, the ACE and eNOS genotype distribution was not significantly different between controls and asthma groups. Furthermore, EDD was not associated with both gene polymorphism of ACE and eNOS. Conclusion. Patients with asthma have decreased vasodilatatory response to shear stress (EDD). Decreased EDD is correlated with the severity of asthma, but not with the distribution of ACE and eNOS genotypes.
THE ECONOMIC IMPACT OF SOVEREIGN DEFAULTS IN LATIN AMERICA 1870-2012*

ABSTRACT This article analyzes sovereign debt defaults in four Latin American countries—Argentina, Brazil, Chile and Mexico—for the period 1870-2012. The impact of sovereign defaults on real GDP growth is generally short-lived, while the impact in terms of output losses is deep and lasts long. Defaults in the period 1972-2012 show a deep and long-lasting impact compared to defaults in earlier periods. Moreover, the length of the contraction that follows a default is associated with favourable international conditions in the run-up to a default, while the depth of the contraction is associated with an expansive domestic economy in the run-up to a default. The results fit with boom–bust theories and sudden stop models. RESUMEN Este artículo analiza impagos de deuda soberana en cuatro países Latinoamericanos— Argentina, Brasil, Chile y México— en el periodo 1870-2012. El impacto sobre el crecimiento del PIB real es generalmente de corta duración, mientras que las pérdidas acumuladas del producto son más profundas y duraderas. Los impagos durante el periodo 1972-2012 tienen un impacto más profundo y más duradero comparado con impagos en periodos anteriores. Adicionalmente, la duración de la contracción posterior al impago es asociado con favorables condiciones internacionales, mientras que la profundidad de la contracción es asociada con una expansión económica doméstica en el periodo antes de la crisis. Los resultados son consistentes con las teorías de boom-bust y los modelos de sudden stop.
Interactions between different generation HIV‐1 fusion inhibitors and the putative mechanism underlying the synergistic anti‐HIV‐1 effect resulting from their combination

We previously reported that the combinatorial use of T20 and T1144, the first and next generations of HIV fusion inhibitors, containing different functional domains resulted in synergistic anti‐HIV‐1 effect, but this effect diminished when T20 and T1144 were covalently linked together. To elucidate the mechanism underlying this synergistic anti‐HIV‐1 effect, we studied the interactions between T20 and T1144 either in a mixture state or in a covalently linked state. T20 alone in solution was largely featureless, while T1144 alone was in α‐helical trimeric conformation. When mixed in solution, T20 and T1144 showed a loose and transient interaction, with a moderate 10% α‐helical content increase, but this interaction was greatly enhanced in the linked state, and T20 and T1144 showed ~100% α‐helical content. These results suggested that the loose and transient interaction between T20 and T1144 may destabilize the T1144 trimer, which makes its otherwise shielded binding sites more accessible to N‐terminal heptad repeat (NHR) and increases its associating rate, thus increasing its anti‐HIV‐1 potency against the temporarily exposed target in NHR and causing the synergistic anti‐HIV‐1 effect. However, the strong interaction between T20 and T1144 in the covalently linked state may shield their NHR‐binding sites, resulting in reduction of the synergistic effect. —Cai, L. , Pan, C. , Xu, L. , Shui, Y. , Liu, K. , Jiang, S. Interactions between different generation HIV‐1 fusion inhibitors and the putative mechanism underlying the synergistic anti‐HIV‐1 effect resulting from their combination. FASEB J. 26, 1018‐1026 (2012). www.fasebj. org
Life stress, the dopamine receptor gene, and emerging adult drug use trajectories: A longitudinal, multilevel, mediated moderation analysis

Abstract This study was designed to examine the prospective relations of life stress and genetic status with increases in drug use. African Americans (N = 399) in rural Georgia (Wave 1 mean age = 17 years) provided three waves of data across 27.5 months and a saliva sample from which the dopamine receptor D4 (DRD4) gene was genotyped. Multilevel growth curve modeling analysis indicated that emerging adults manifested the highest escalations in drug use when they reported high life stress and carried an allele of DRD4 with 7 or more repeats (7 + R allele). In addition, emerging adults who reported high life stress and carried the 7 + R allele evinced the largest increases in two proximal risk factors for drug use: affiliations with drug-using companions and drug use vulnerability cognitions. Furthermore, when the Gene × Environment interaction effects on the increases in affiliations with drug-using companions and vulnerability cognitions were entered into the model forecasting drug use, the Life Stress × DRD4 Status interaction on drug use became nonsignificant in the presence of the risk mechanisms. This finding provides an example of “second generation” Gene × Environment interaction research in which the interaction's effects on proximal risk mechanisms account for its effects on outcomes.
Aging, Pension Reform and the Current Account

Declining fertility and increasing life expectancy put pressure on the financing of social security in developed countries. Economists and policy makers have considered options to address this financing challenge, most of which contain reforms of the pension system. No pension reform appears unambiguously superior: reforms typically fare good in some dimensions, but not all. Because of trade-offs, evaluation criteria play a major role in the selection of the preferred policy reform. Typically, analysts consider financial sustainability of the system, evolution of output per capita and minimum income in old age. In this paper we add one criterion, current account imbalances. Using an overlapping-generations model calibrated for Austria with a multi-pillar pension system and an aging population, we compare pension reforms with pay-as-you-go financing and capital-funded financing. In contrast to a number of previous studies, the capital-funded pillar in our model contains both a tax component and insurance against the longevity risk, both realistic. Neglecting current accounts, we find that increases in retirement age with the current pay-as-you-go pension system achieve a good balance between output maintenance, pension finance sustainability and old-age anti-poverty, consistent with previous studies. Such arrangements however increase the dependence on net foreign assets, as the need to save for consumption after retirement is reduced. Adding a capital-funded pillar to the pension system helps achieve (and in some cases improve on) the same goals without increasing the dependence on net foreign assets, over the long run. Savings placed in the pension funds are indeed available for domestic investment. As in previous studies, there are however transitional costs.
The Rates and Clinical Characteristics of Pneumolabyrinth in Temporal Bone Fracture

Background Pneumolabyrinth is a rare inner ear clinical manifestation. To date, only about 50 cases have been reported—all as case reports. Consequently, the rate and clinical characteristics of pneumolabyrinth have not been evaluated. Methods Of the 38, 568 patients who visited our emergency department for head trauma, 466 underwent temporal bone computed tomography (CT). One hundred seventy-five patients had temporal bone fracture (13 bilateral temporal bone fractures; 188 ears with temporal bone fractures), and 14 patients had pneumolabyrinth (15 ears with pneumolabyrinth; 1 bilateral case). A retrospective review of their medical records and radiologic findings was performed. Temporal bone fractures were classified by two different systems: the traditional classification and an otic capsule–based classification. Results Pneumolabyrinth occurred in 8.0% of all temporal bone fractures, 4.0% of longitudinal temporal bone fractures, 16.1% of transverse or mixed temporal bone fractures, and 48.4% of otic capsule–violating temporal bone fractures. In all cases, pneumolabyrinth was found on CT, which was performed within 3 days, but not on follow-up CT performed 5 days or longer after head trauma. All patients complained of hearing loss and dizziness. Hearing in most patients (83.3%) did not improve, whereas dizziness improved in 91.7% of patients. Air was located only in the vestibule or semicircular canal in 53.3% and in the vestibular or semicircular canal and cochlea in 46.6% of ears with pneumolabyrinth. The initial hearing threshold and recovery rate using pure-tone audiometry were not different according to the air location in the inner ear. Conclusion Pneumolabyrinth was more common than expected; we believe that the timing of evaluation affects its rarity. Pneumolabyrinth was detected in nearly 50% of patients with otic capsule–violating temporal bone fractures when CT scanning was performed early after trauma.
How Much Do Idiosyncratic Bank Shocks Affect Investment? Evidence from Matched Bank-Firm Loan Data

We show that supply-side financial shocks have a large impact on firms’ investment. We develop a new methodology to separate firm borrowing shocks from bank supply shocks using a vast sample of matched bank-firm lending data. We decompose aggregate loan movements in Japan for the period 1990–2010 into bank, firm, industry, and common shocks. The high degree of financial institution concentration means that individual banks are large relative to the size of the economy, which creates a role for granular shocks as in Gabaix’s (2011) study. We show that idiosyncratic granular bank supply shocks explain 30–40 percent of aggregate loan and investment fluctuations.
Co-operatives, credit unions and principles : a post 2008 critique

This thesis examines the adherence of credit unions to their co-operative principles. Noting the financial crisis of 2008 and the calls for change to a more ethical, transparent and fairer financial industry co-operatives appear to be a plausible way forward. The analysis of the corporate governance of co-operatives indicates that they are founded on co-operative principles, the cornerstone being the active participation by its members. The study of the previous literature demonstrates instances where co-operatives did not adhere to their principles as well as instances where members were not very active. Credit unions in Cyprus were selected as the specific co-operative industry and geographic location for the collection of data; one of the reasons being that in March 2013, six months prior to the first collection of data, Cyprus suffered a second financial crisis, giving the opportunity to investigate these two issues in a time of scrutiny and pressure. Structured interviews with thirty credit union employees, documents (statistics) from the ex-supervisory body and three focus groups with thirty members (in total) were carried out. The findings show that credit unions do not seem to adhere to their principles and members do not seem to be actively involved with their credit unions. As the warning bells for their long-term survival may have already sounded, credit unions must encourage their members to get involved, rebuild their trust amongst them, among membership and the society in general, provide co-operative-related training and restrict the involvement of the political parties. Finally, ways must be found so that credit unions remain closely involved with the societies they serve. To this extent, Service-Dominant Logic is proposed as a possible way in assisting credit unions to re-focus on their membership.
Molecular physiology and pharmacology of HERG. Single-channel currents and block by dofetilide.

BACKGROUND The human ether-a-go-go-related gene (HERG) is one locus for the hereditary long-QT syndrome. A hypothesis is that HERG produces the repolarizing cardiac potassium current IKr with the consequence that mutations in HERG prolong the QT interval by reducing IKr. The elementary properties of HERG are unknown, and as a test of the hypothesis that HERG produces IKr, we compared their elementary properties. METHODS AND RESULTS We injected HERG cRNA into Xenopus oocytes and measured currents from single channels or current variance from the noise produced by ensembles of channels recorded from macro patches. Single-channel conductance was dependent on the extracellular potassium concentration ([K]o). At physiological [K]o, it was 2 picosiemens (pS), and at 100 mmol/L [K]o, it was 10 pS. Openings occurred in bursts with a mean duration of 26 ms at -100 mV. Mean open time was 3.2 ms and closed times were 1.0 and 26 ms. In excised macro patches, HERG currents were blocked by the class III antiarrhythmic drug dofetilide, with an IC50 of 35 nmol/L. Dofetilide block was slow and greatly attenuated at positive potentials at which HERG rectifies. CONCLUSIONS The microscopic physiology of HERG and IKr is similar, consistent with HERG being an important component of IKr. The pharmacology is also similar; dofetilide appears to primarily block activated channels and has a much lower affinity for closed and inactivated channels.
Developing urban space: the changing role of NGOs in Bangladesh

ABSTRACT Rapid urbanisation has been an emerging issue in recent decades, particularly for developing countries such as Bangladesh. As one of world’s largest international NGOs, BRAC, based in Bangladesh, is transiting towards a more integrated approach for alleviating urban poverty. This article presents BRAC’s current journey for eradicating urban poverty and outlines the characteristics of urban poverty, key issues faced by the urban poor, and learning that BRAC has accumulated through its urban interventions. Introducing the concept of “coproduction”, the article identifies BRAC’s priorities to eradicate urban poverty within the context of an overall urban strategy.