Miracle-dz/UniFAR-FTUs huggingface dataset:
Congenital Corneal Endothelial Dystrophies Resulting From Novel De Novo Mutations

Purpose: To describe 2 cases of congenital corneal endothelial edema resulting from novel de novo mutations. Methods: Case A patient was a 15-month-old white child and case B patient was a 3-year-old Hispanic child presenting with bilateral cloudy corneas since birth. Clinicopathologic findings are presented. DNA samples were screened for mutations in candidate genes by Sanger sequencing. Results: Slit-lamp examination of case A patient revealed stromal edema and haze. Histology of the keratoplasty button showed stromal thickening with loss of endothelium and thin Descemet membrane. Sanger sequencing established the diagnosis of congenital hereditary endothelial dystrophy by detection of a compound heterozygous mutation in SLC4A11. The proband displayed a novel de novo frameshift mutation in one SLC4A11 allele, p.(Pro817Argfs*32), in conjunction with a maternally inherited missense mutation in SLC4A11, p.(Arg869His). Case B patient similarly presented with stromal edema and stromal haze. Histopathologic analysis revealed a spongy epithelium, focal discontinuities in Bowman layer, stromal thickening with areas of compacted posterior stroma, variable thickness of Descemet membrane, and regional multilayered endothelium. Sanger sequencing found a novel de novo nonsense mutation in the first exon of ZEB1, p.(Cys7*). Conclusions: To the authors' knowledge, we report the earliest clinical presentation of posterior polymorphous corneal dystrophy resulting from a de novo mutation in ZEB1. Additionally, we present a congenital hereditary endothelial dystrophy case with a thin Descemet membrane with a novel compound heterozygous SLC4A11 mutation. In the absence of a family history or consanguinity, de novo mutations may result in congenital corneal endothelial dystrophies.
Emerging Endotypes of Chronic Rhinosinusitis and Its Application to Precision Medicine

Chronic rhinosinusitis (CRS) is a heterogeneous inflammatory disease with various underlying pathophysiologic mechanisms which translate to endotypes, in contrast to clinical phenotypes or histological subtypes. Defining endotypes can help clinicians predict disease prognosis, select subjects suitable for a specific therapy, and assess risks for comorbid conditions, including asthma. Therefore, with recent advancement of biologicals in CRS clinical trials, endotyping can be a breakthrough in treating recalcitrant CRS. CRS is caused by dysregulated immunologic responses to external stimuli, which induce various inflammatory mediators from inflammatory cells, including innate lymphoid cells (ILCs) and T lymphocytes as well as epithelial cells. Thymic stromal lymphopoietin (TSLP), interleukin (IL)-25, and IL-33, which are mainly secreted by epithelial cells in response to external stimuli, act on type 2 ILCs and T helper 2 (Th2) cells, inducing IL-4, IL-5, and IL-13. Local immunoglobulin E (IgE) production is also a signature event in nasal polyps (NP). These inflammatory mediators are novel potential therapeutic targets for recalcitrant CRS. This article reviews recent publications regarding endotypes and endotype-based therapeutic strategies in CRS and NP.
Development and psychometric properties of the Healthy Aging Activity Engagement Scale (HAAE)

ABSTRACT Objectives: Accumulating research indicates that engaging in healthy lifestyle behaviors (e.g. exercise, cognitive and social engagement, stress reduction) can prevent illness and disability as people age and improve mental health. The Healthy Aging Activity Engagement (HAAE) scale was developed to holistically assess an individual's level of engagement in healthy aging behaviors across multiple health domains. Methods: Participants were 275 healthy younger, midlife and older adults. Participants rated engagement in 38 healthy aging lifestyle behaviors over the past 7 days on a scale from 1 (strongly disagree) to 5 (strongly agree). Results: Traditional measurement analysis and Rasch modelling techniques resulted in a 32-item scale with three subscales representing biologic health, social and cognitive strategies, and health safeguard behaviors. With the exception of the health safeguard subscale, the HAAE total score and subscales demonstrated good internal consistency, test-retest reliability, convergent and discriminant validity, and responsiveness to a program designed to promote engagement in healthy brain aging behaviors. Discussion: With further validation, the HAAE may be useful for holistic clinical assessment of behaviors that promote healthy aging, support brain and mental health, and for treatment planning. Furthermore, the HAAE can be used to measure the outcome of multi-domain lifestyle interventions.
Femtogram scale nano-optomechanical resonators in water

A femtogram scale nanobeam optomechanical crystal (OMC) resonator operating in water is designed and demonstrated. The thermomechanical motion of the highly damped mechanical resonance in water can be resolved by the sensitive cavity optomechanical readout. The present device has an estimated mass sensitivity at the attogram level (1.33 ag/VHz) in water. A silicon nitride nanobeam OMC resonator with high optical Q-factor operating at visible wavelength is designed to improve the mass sensitivity.
Effect of pulsed light on structural and physiological properties of Listeria innocua and Escherichia coli

The application of broad‐spectrum intense light pulses is an innovative nonthermal technology for the decontamination of packaging materials, liquids or foodstuffs. The objective of this study was the fundamental investigation of the cellular impact of a pulsed light treatment on Listeria innocua and Escherichia coli.
Public Support for Sale

Federal agencies award thousands of grants and contracts each year. Although we have considerable insight into the implications of these expenditures for politicians, we know little about their consequences for agencies. I develop a theory that links agency spending in citizens’ states to their support of agency performance, with the expectation that this relationship is conditioned by citizen–agency ideological congruence. I contend that agency spending serves as an information shortcut for citizens when evaluating agencies because it is often visible in a way that other agency activities, such as regulatory decisions, are not. When a citizen fundamentally supports the policies and projects an agency is funding, the positive effect of spending may be enhanced. I find support for these expectations. This article contributes to our understanding of the ways in which expenditures shape opinion toward agencies, which is important given the significance of public sentiment for an agency’s political survival.
DC breakdown characteristics of high temperature polymer films

This paper shows the effect of electrode area on dielectric strength for different polymeric materials. In the investigation, four electrodes of different diameters are selected in the range of 12.5-50 mm. To develop a base of understanding, several commercially available dielectric materials of different thickness that have been studied are aramid paper (Nomexreg) polyimide film (Kaptonreg) Mylarreg polyester film, PTFE, Nomex-Mica, and Nomex-polyester-Nomex composites. The DC breakdown strength of these high temperature dielectric materials is investigated by using the two-parameter Weibull distribution. It is noticed that the lower thickness films are more susceptible to the mechanical damage during handling process, thus increasing the chances of having lower breakdown strength. This lower breakdown strength cannot be ignored for effective insulation design of electric power apparatus and for the high reliability that is desired in the sensitive devices such as microelectronics, fiber optics, and in outer space applications
To define service level in an integrated model for warehouse and inventory planning by utilizing heuristic solution: An example

The main objective of this study is to define service level equation with a different approach in the forward-reserve models. In this article within the forward-reserve model, in order to ensure service level optimization, a heuristic sequential solution procedure is proposed. A global model is presented and solved for evaluating and decision making of warehouse and inventory decisions. The proposed method is related to service level and inventory decisions as well as warehouse decisions and shortage cost. Two stages are followed with the proposed method. While in the first stage inventory decisions along with the constant service level decision being taken in the second part the results obtained in the first stage being kept constant and with this regard warehouse decisions being taken. Since the main purpose in this article is to develop a new equation for a service level, the results of the second stage are not shown in detail. It has been shown with an application of a real example that we could reach the solution in less time for the new developed service level equation comparing to simple service level search procedure. The main contribution of this study to literature is to develop a new service level equation for forward-reserve models.
CELLULAR IMMUNITY TO CYTOMEGALOVIRUS IN A PATIENT FOLLOWING BONE MARROW TRANSPLANTATION

SUMMARY Cell-mediated immunity to cytomegalovirus (CMV) was studied in a bone marrow transplant patient with evidence of active CMV infection. The lymphocytes from this patient were found to specifically recognize and respond in vitro by transformation to CMV-infected Wistar-38 fibroblasts and by production of macrophage migration inhibition factor to CMV antigen. In addition, plasma and spinal fluid from the patient were found to contain blocking factor that specifically inhibited the lymphocyte response in the above assays. Biochemical, biophysical, and immunological studies indicate that the blocking factor may be an antigen-antibody complex. The immunosuppression required for bone marrow transplantation results in an increased number of severe and often life-threatening viral infections (4, 11). Immunosuppressive regimens utilized in patients undergoing renal transplantation also result in a high incidence of viral infections, particularly infection with cytomegalovirus (CMV) (3, 8). Increasing complement-fixing antibody titers to CMV have been reported in both renal and bone marrow transplant recipients with clinical evidence of CMV infection (3, 4, 8, 9, 11). Thus, despite immunosuppression, a specific antibody response is seen and a vigorous serological response may indicate a more favorable prognosis (11). Depression of cellular immunity, as measured by blasto-genic response to phytohemagglutinin, has been described in renal transplant patients infected with CMV (9). However, the presence of specific cellular immune response to CMV in infected patients is poorly documented. This report characterizes the cell-mediated immune response to CMV in a patient who developed evidence of acute CMV infection following bone marrow transplantation. Lymphoid cells from this patient were found to specifically recognize and respond in vitro by transformation to a CMV-infected cell line and by production of macrophage migration inhibition factor (MIF) to CMV antigen. Furthermore, plasma and spinal fluid from this patient were found to contain blocking factor that specifically inhibited lymphocyte transformation and MIF production in response to CMV antigen. Partial characterization of the blocking factor indicates that it may be an antigen-antibody complex.
Sudden Death as the Initial Manifestation of Primary Pulmonary Hypertension: Report of Four Cases

&NA; Four autopsy cases are presented in which sudden death was the initial manifestation of primary pulmonary hypertension. The arteriopathy was plexogenic in two cases (a 3‐year‐old girl and a 16‐year‐old boy) and was thrombotic in two other cases (55‐ and 59‐year‐old women). The diagnosis of primary pulmonary hypertension, particularly in forensic cases, requires that the pathologist be especially aware of the possibility and that a careful evaluation of multiple sections of lung be performed. Determination of the histopathologic type also is important because some forms of the disease may be familial and may be treatable in other family members if they are detected early.
Sensorimotor integration on a rapid time scale

Significance Humans and other animals continuously monitor sensory information to inform the selection of motor commands for adaptive behaviors. Acoustic communication, for example, utilizes auditory feedback to fine-tune vocal production parameters. Because most animal species produce vocalizations that last several hundred milliseconds, it is difficult to dissect the temporal dynamics of audio-vocal feedback control. Here we took advantage of the brief echolocation signals of bats and mapped out the time course of vocal adjustments to background noise on a millisecond time scale. The high temporal resolution data provided the foundation for the model of audio-vocal volume control. We discovered that temporal summation, a shared auditory process across the animal kingdom, lies at the core of adaptive vocal volume control. Sensing is fundamental to the control of movement: From grasping objects to speech production, sensing guides action. So far, most of our knowledge about sensorimotor integration comes from visually guided reaching and oculomotor integration, in which the time course and trajectories of movements can be measured at a high temporal resolution. By contrast, production of vocalizations by humans and animals involves complex and variable actions, and each syllable often lasts a few hundreds of milliseconds, making it difficult to infer underlying neural processes. Here, we measured and modeled the transfer of sensory information into motor commands for vocal amplitude control in response to background noise, also known as the Lombard effect. We exploited the brief vocalizations of echolocating bats to trace the time course of the Lombard effect on a millisecond time scale. Empirical studies revealed that the Lombard effect features a response latency of a mere 30 ms and provided the foundation for the quantitative audiomotor model of the Lombard effect. We show that the Lombard effect operates by continuously integrating the sound pressure level of background noise through temporal summation to guide the extremely rapid vocal-motor adjustments. These findings can now be extended to models and measures of audiomotor integration in other animals, including humans.
Bir Meslekî Egitim Kurumu Olarak Polis Meslek Yüksek Okulları

Bir mesleki egitim kurulusu olan Polis Meslek Yuksekokullari incelemenin temelini olusturmaktadir. Calismada, Polis kavrami, polis egitimi, ulkemizde polis egitiminin tarihi gelisimi aciklanmakta ve 2001 yilinda kurulan Polis Meslek Yuksekokullari cesitli yonleriyle incelenmektedir
Management of acute asthma in children using metered dose inhaler and small volume nebulizer.

OBJECTIVE To determine whether the administration of beta2-agonist by Metered Dose inhaler (MDI) with accessory device (AD) is a as effective as the administration of beta2-agonist by small volume nebulizers (SVN) for the treatment of acute asthma. METHODS A cross sectional study was conducted at Emergency Room (ER) of National Institute of Child Health (NICH), Karachi, between October 2000 to March 2001. This study included 150 children, 6 months and older with a history of wheeze and presenting with an acute asthma exacerbation. Children were categorized into mild, moderate and severe asthma according to medical scoring system. Children were assigned randomly into group A and B to receive standard dose of beta2-agonist (salbutamol) by MDI/AD (group A) or SVN (group B). Baseline characteristics and asthma severity were recorded. All variables (dyspnoea, use of accessory muscles, cyanosis, respiratory rate, heart rate, blood pressure, oxygen saturation, pulsus paradoxus, and wheeze) and Peak Expiratory Flow Rate (PEFR) in children 5 years and older, were determined at pre and post inhalation therapy. RESULTS Both groups did not differ in demographic characteristics. There were no significant differences in outcome measures. In children treated with MDI/ADs and SVNs. PEFR increased significantly in both the groups after completion of treatment, but PEFR was not statistically significant when compared in between groups. CONCLUSION The data suggested that MDI/AD is an effective alternative to nebulizer for the treatment of children with acute asthma exacerbation in the ER.
Spectral Learning from a Single Trajectory under Finite-State Policies

We present spectral methods of moments for learning sequential models from a single trajec-tory, in stark contrast with the classical literature that assumes the availability of multiple i.i. d. trajectories. Our approach leverages an efficient SVD-based learning algorithm for weighted au-tomata and provides the first rigorous analysis for learning many important models using dependent data. We state and analyze the algorithm under three increasingly difficult scenarios: proba-bilistic automata, stochastic weighted automata, and reactive predictive state representations controlled by a finite-state policy. Our proofs include novel tools for studying mixing properties of stochastic weighted automata.
GATA-3 protects against severe joint inflammation and bone erosion and reduces differentiation of Th17 cells during experimental arthritis.

OBJECTIVE Rheumatoid arthritis is associated with the infiltration of T helper cells into the joints. It is unclear whether interferon-gamma (IFNgamma)-producing Th1 cells or the novel T helper subset, interleukin-17 (IL-17)-producing Th17 cells, are the pathogenic mediators of joint inflammation in chronic nonautoimmune arthritis. Therefore, this study was aimed at examining whether the Th2-specific transcription factor GATA-3 can regulate arthritis, in an experimental murine model, by modulating Th1 and/or Th17 cell polarization. METHODS Arthritis was induced with methylated bovine serum albumin (mBSA) in both wild-type and CD2 T cell-specific GATA-3 (CD2-GATA-3)-transgenic mice. At days 1 and 7 after the induction of arthritis, knee joints were scored macroscopically for arthritis severity and for histologic changes. Single-cell suspensions were generated from the spleens, lymph nodes, and inflamed knee joints. Cytokine expression by CD4+ T cells was determined using flow cytometry, and IL-17 expression in the inflamed knee joints was determined by enzyme-linked immunosorbent assay. Analyses of gene expression were performed for Th17-associated factors. RESULTS Wild-type mice developed severe joint inflammation, including massive inflammatory cell infiltration and bone erosion that increased significantly over time, reaching maximal arthritis scores at day 7. In contrast, only mild joint inflammation was observed in CD2-GATA-3-transgenic mice. This mild effect was further accompanied by systemic and local reductions in the numbers of IL-17+IFNgamma- and IL-17+IFNgamma+, but not IL-17-IFNgamma+, CD4+ T cells, and by induction of Th2 cytokine expression. Moreover, GATA-3 overexpression resulted in reduced gene expression of the Th17-associated transcription factor retinoic acid-related orphan receptor gammat. CONCLUSION These results indicate that enforced GATA-3 expression protects against severe joint inflammation and bone erosion in mice, accompanied by reduced differentiation of Th17 cells, but not Th1 cells, during mBSA-induced arthritis.
Angiographic follow-up after placement of a self-expanding coronary-artery stent.

BACKGROUND The placement of stents in coronary arteries after coronary angioplasty has been investigated as a way of treating abrupt coronary-artery occlusion related to the angioplasty and of reducing the late intimal hyperplasia responsible for gradual restenosis of the dilated lesion. METHODS From March 1986 to January 1988, we implanted 117 self-expanding, stainless-steel endovascular stents (Wallstent) in the native coronary arteries (94 stents) or saphenous-vein bypass grafts (23 stents) of 105 patients. Angiograms were obtained immediately before and after placement of the stent and at follow-up at least one month later (unless symptoms required angiography sooner). The mortality after one year was 7.6 percent (8 patients). Follow-up angiograms (after a mean [+/- SD] of 5.7 +/- 4.4 months) were obtained in 95 patients with 105 stents and were analyzed quantitatively by a computer-assisted system of cardiovascular angiographic analysis. The 10 patients without follow-up angiograms included 4 who died. RESULTS Complete occlusion occurred in 27 stents in 25 patients (24 percent); 21 occlusions were documented within the first 14 days after implantation. Overall, immediately after placement of the stent there was a significant increase in the minimal luminal diameter and a significant decrease in the percentage of the diameter with stenosis (changing from a mean [+/- SD] of 1.88 +/- 0.43 to 2.48 +/- 0.51 mm and from 37 +/- 12 to 21 +/- 10 percent, respectively; P less than 0.0001). Later, however, there was a significant decrease in the minimal luminal diameter and a significant increase in the stenosis of the segment with the stent (1.68 +/- 1.78 mm and 48 +/- 34 percent at follow-up). Significant restenosis, as indicated by a reduction of 0.72 mm in the minimal luminal diameter or by an increase in the percentage of stenosis to greater than or equal to 50 percent, occurred in 32 percent and 14 percent of patent stents, respectively. CONCLUSIONS Early occlusion remains an important limitation of this coronary-artery stent. Even when the early effects are beneficial, there are frequently late occlusions or restenosis. The place of this form of treatment for coronary artery disease remains to be determined.
Molecular basis of the activation of basal histone H1(0) gene expression.

Histone H1(0) is encoded by a gene that is expressed only in cells committed to differentiation. We have previously cloned the Xenopus laevis H1(0) gene and studied elements involved in the regulation of its expression in transfected Xenopus laevis A6 cells, and in microinjected embryos. In this work, in order to understand the basis of the action of these elements, we used an A6 cell nuclear extract and showed that the H1(0) promoter is able to direct efficient in vitro transcription, which is highly dependent on a functional TATA box. However, in contrast to what we observed in vivo, in transfected A6 cells, the in vitro transcription was independent of major regulatory elements, defined in vivo. We then used this in vitro system to reconstitute H1(0) gene regulation. The creation of a repressive environment by the addition of purified histone H1 to the in vitro transcription system allowed us to obtain transcription dependent on the integrity of the regulatory elements. Investigating the basis of this regulation we found that protein-DNA interaction on the proximal promoter region was dependent on the integrity of proximal elements, and moreover the distal regulatory element, the UCE, was able to modulate this interaction. We conclude that the role of these regulatory elements is to maintain the basal TATA-dependent transcription of H1(0) under repressive condition: i.e., H1-mediated repression of transcription, or chromatin assembly in general.