Miracle-dz/UniFAR-FTUs huggingface dataset:
Implications for Gene Therapy-Limiting Expression of IL-2Rγc Delineate Differences in Signaling Thresholds Required for Lymphocyte Development and Maintenance

X-linked SCID patients are deficient in functional IL-2Rγc leading to the loss of IL-2/IL-4/IL-7/IL-9/IL-15/IL-21 signaling and a lack of NK and mature T cells. Patients treated with IL-2Rγc gene therapy have T cells develop; however, their NK cell numbers remain low, suggesting antiviral responses may be compromised. Similarly, IL-2Rγc−/− mice reconstituted with IL-2Rγc developed few NK cells, and reconstituted T cells exhibited defective proliferative responses suggesting incomplete recovery of IL-2Rγc signaling. Given the shift toward self-inactivating long terminal repeats with weaker promoters to control the risk of leukemia, we assessed NK and T cell numbers and function in IL-2Rγc−/− mice reconstituted with limiting amounts of IL-2Rγc. Reconstitution resulted in lower IL-2/-15–mediated STAT5 phosphorylation and proliferation in NK and T cells. However, TCR costimulation restored cytokine-driven T cell proliferation to wild-type levels. Vector modifications that improved IL-2Rγc levels increased cytokine-induced STAT5 phosphorylation in both populations and increased NK cell proliferation demonstrating that IL-2Rγc levels are limiting. In addition, although the half-lives of both NK and T cells expressing intermediate levels of IL-2Rγc are reduced compared with wild-type cells, the reduction in NK cell half-live is much more severe than in T cells. Collectively, these data indicate different IL-2Rγc signaling thresholds for lymphocyte development and proliferation making functional monitoring imperative during gene therapy. Further, our findings suggest that IL-2Rγc reconstituted T cells may persist more efficiently than NK cells due to compensation for suboptimal IL-2Rγc signaling by the TCR.
A Clinico-Anatomical Study of Strabismus in a Tertiary Care Hospital

This prospective study was conducted about the clinical profile, extraocular muscle involvement, epidemiology and the etiology of 162 cases of manifest strabismus attending the Ophthalmology outpatient department from July 2009 to July-2011. Incidence of manifest strabismus was found to be 0.63%, of which 63% were paralytic and 37% non paralytic, 66% were esotropia and 34% exotropia. Paralytic esotropia was common in the age group of 41-50 years (46.4%) and nonparalytic in 0-10 years (47.4%). Males dominated over females whether esotropia or exotropia. Paralytic exotropia was common in 41-50 years (45.6%) and non paralytic in 0-10 years (45.5%). Paralytic strabismus was common in the lower socioeconomic group (52.9%) and non paralytic in middle and higher class (86.7%). Abduction defect was found in 73.5% due to lateral rectus muscle involvement. Abducent nerve palsy was the most common cause of paralytic strabismus (56.8%) followed by oculomotor nerve (21.6%). No isolated 4 th nerve involvement was encountered. Involvement of more than one cranial nerves was found in 21.6%. Most of the patients presented with deviation of eye followed by diplopia and defective vision. Vascular involvement was the commonest etiology in the paralytic group. Ophthalmologists need to be trained and well equipped strabismus clinics should be established in this part of the country.
A flexible interface design for Web directories to accommodate different cognitive styles

Search engines are very popular tools for collecting information from distributed resources. They provide not only search facilities, but they also offer directories for users to browse content divided into groups. In this paper, we've adopted an individual differences approach to explore user's attitudes towards various interface features provided by existing Web directories. Among a variety of individual differences, cognitive style is a particularly important characteristic that influences the effectiveness of information seeking. Empirical results indicate that users' cognitive styles influence their reactions to the organization of subject categories, presentation of the results, and screen layout. We developed a set of design guidelines on the basis of these results, and propose a flexible interface that adopts these guidelines to accommodate the preferences of different cognitive style groups.
Retroperitoneal Sarcoma - A Clinico-Pathological Study

Retroperitoneal sarcomas are rare tumours that comprise approximately 15% of all soft tissue sarcomas in adults 1 . Most of the retroperitoneal tumours are malignant and approximately one third is soft tissue sarcoma. Although significant advances in our understanding of extremity soft tissue sarcoma have resulted in improved treatments and outcomes, similar progress has not been achieved in our understanding and treatment of retroperitoneal soft tissue sarcomas. Here we present a retrospective analysis of 30 cases of retroperitoneal sarcoma from a tertiary care centre from 2006 to 2010. The incidence of retroperitoneal sarcoma in our study was around 0.10% of the total surgical admissions. Peak incidence was seen between 5060 years and >85% patients were above 40 years. There was a definite male preponderance, with a male to female ratio of 2.75: 1. The most useful and economical investigative modality is contrast enhanced CT scan of the abdomen Most of the patients present at a later stage of the disease with adjacent organ involvement and distant metastasis. These factors contribute to the bad prognostic nature of the disease. Potentially curative resection in the form of en-bloc resection/ complete excision was possible only in 50% of cases. The role of adjuvant treatment in retroperitoneal sarcoma still remains unsettled. The most common histopathological type of retroperitoneal sarcoma was liposarcoma. Majority of patients developed local recurrence and distant metastasis within a short period, especially in margin compromising resections. Margin negative resections are associated with good prognosis.
Silica optical fiber technology for devices and components

WBDG | WBDG Whole Building Design Guide Sun, 28 Apr 2019 07:14:00 GMT With over 500,000 users downloading 3 million documents per month, the WBDG is the only web-based portal providing government and industry practitioners with one-stop access to current information on a wide range of building-related guidance, criteria and technology from a 'whole buildings' perspective. Resolve a DOI Name Sat, 27 Apr 2019 14:10:00 GMT [? ] korics.co.kr Science and technology of polymeric ablative materials for . Sun, 28 Apr 2019 17:37:00 GMT The protective heat shield of the vehicle must work as an aerodynamic body, as a structural component, and as a thermal insulator , . When non ablative TPS materials are used to manufacture the heat shield, re-radiation is the main phenomenon exploited to insulate the body re-entering in the atmosphere; in this case, NA-TPS are produced using ceramics or particular metals (such as tungsten or . 3D printing of ceramics: A review ScienceDirect Sun, 28 Apr 2019 09:58:00 GMT The SL technique is believed to be the most prominent and popular 3D printing technology and has been extensively used worldwide [].It was first proposed and developed by Hull in 1986 [] and was later commercialised by 3D Systems Inc. SL is a process in which a light source of a certain wavelength (usually in the ultraviolet range) is used to selectively cure a liquid surface in a vat . Ultrapure water Wikipedia Sat, 27 Apr 2019 23:29:00 GMT Ultrapure water is used extensively in the semiconductor industry; this is the highest grade of UPW quality.The consumption of electronic-grade or molecular-grade water by the semiconductor industry can be compared to the water consumption of a small city; a single factory can utilize ultrapure water (UPW) at a rate of 2 MGD, or ~5500 m 3 /day. The use of UPW varies; it may be used to rinse .
Effect of smokeless tobacco (snus) on smoking and public health in Sweden

Objective: To review the evidence on the effects of moist smokeless tobacco (snus) on smoking and ill health in Sweden. Method: Narrative review of published papers and other data sources (for example, conference abstracts and internet based information) on snus use, use of other tobacco products, and changes in health status in Sweden. Results: Snus is manufactured and stored in a manner that causes it to deliver lower concentrations of some harmful chemicals than other tobacco products, although it can deliver high doses of nicotine. It is dependence forming, but does not appear to cause cancer or respiratory diseases. It may cause a slight increase in cardiovascular risks and is likely to be harmful to the unborn fetus, although these risks are lower than those caused by smoking. There has been a larger drop in male daily smoking (from 40% in 1976 to 15% in 2002) than female daily smoking (34% in 1976 to 20% in 2002) in Sweden, with a substantial proportion (around 30%) of male ex-smokers using snus when quitting smoking. Over the same time period, rates of lung cancer and myocardial infarction have dropped significantly faster among Swedish men than women and remain at low levels as compared with other developed countries with a long history of tobacco use. Conclusions: Snus availability in Sweden appears to have contributed to the unusually low rates of smoking among Swedish men by helping them transfer to a notably less harmful form of nicotine dependence.
Whole‐Mount Imaging Demonstrates Hypervascularity of the Pancreatic Ducts and Other Pancreatic Structures

Confocal microscopy in combination with commercial software is frequently used to generate three‐dimensional images of tissue architecture. Here we report a novel, whole‐mount imaging protocol technique that allows detailed three‐dimensional imaging of adult pancreatic structures. This technique provides an improved appreciation of the anatomical detail of pancreatic structures and of the relationship between the pancreatic ducts and islets. In addition, imaging of the pancreatic ducts revealed a previously unappreciated high degree of hypervascularity. Anat Rec, 2012. © 2012 Wiley Periodicals, Inc.
Dislocation structures in copper single crystals fatigued at low amplitudes

Abstract Single crystals cycled over a wide range of strains, below and up to those of the plateau in the cyclic stress-strain curve, have been prepared by ultrasonic fatigue. The dislocation structures of these specimens have been examined by transmission electron microscopy (TEM) and details of the structures, including the dimensions and varieties of the structures and the identities of the dislocations, have been documented. Special attention has been given to the occurrence of secondary dislocations and their role in the dislocation mechanisms, such as the transformation of loop patches into persistent slip bands.
How Unique is Your .onion?: An Analysis of the Fingerprintability of Tor Onion Services

Recent studies have shown that Tor onion (hidden) service websites are particularly vulnerable to website fingerprinting attacks due to their limited number and sensitive nature. In this work we present a multi-level feature analysis of onion site fingerprintability, considering three state-of-the-art website fingerprinting methods and 482 Tor onion services, making this the largest analysis of this kind completed on onion services to date. Prior studies typically report average performance results for a given website fingerprinting method or countermeasure. We investigate which sites are more or less vulnerable to fingerprinting and which features make them so. We find that there is a high variability in the rate at which sites are classified (and misclassified) by these attacks, implying that average performance figures may not be informative of the risks that website fingerprinting attacks pose to particular sites. We analyze the features exploited by the different website fingerprinting methods and discuss what makes onion service sites more or less easily identifiable, both in terms of their traffic traces as well as their webpage design. We study misclassifications to understand how onion services sites can be redesigned to be less vulnerable to website fingerprinting attacks. Our results also inform the design of website fingerprinting countermeasures and their evaluation considering disparate impact across sites.
Fast inhomogeneous plane wave algorithm for scattering from objects above the multilayered medium

A previously developed fast inhomogeneous plane wave algorithm is adapted to the analysis of the scattering from the objects above a multilayered medium. Based on the similarity of the inhomogeneous plane wave expansion of the Green's function encountered in the layered medium study to its free space counterpart, the proper steepest descent path can be defined. By using the interpolation and extrapolation techniques, the translation matrix is diagonalized. The contributions of the pole and branch point, resulting from the deformation of the integration path, can be handled easily. Comparing with the previously developed methods in this area, this algorithm is simpler, more efficient, and more general. The multilevel fast inhomogeneous plane wave algorithm has been implemented and the numerical results show that O(N log N) computational complexity of the algorithm is achieved. The algorithm is validated by comparing the results from a conventional method of moments program.
Identification, sequencing, and localization of a new carbonic anhydrase transcript from the hydrothermal vent tubeworm Riftia pachyptila

The vestimentiferan annelid Riftia pachyptila forms dense populations at hydrothermal vents along the East Pacific Rise at a depth of 2600 m. It harbors CO2‐assimilating sulfide‐oxidizing bacteria that provide all of its nutrition. To find specific host transcripts that could be important for the functioning of this symbiosis, we used a subtractive suppression hybridization approach to identify plume‐ or trophosome‐specific proteins. We demonstrated the existence of carbonic anhydrase transcripts, a protein endowed with an essential role in generating the influx of CO2 required by the symbionts. One of the transcripts was previously known and sequenced. Our quantification analyses showed a higher expression of this transcript in the trophosome compared to the branchial plume or the body wall. A second transcript, with 69.7% nucleotide identity compared to the previous one, was almost only expressed in the branchial plume. Fluorescent in situ hybridization confirmed the coexpression of the two transcripts in the branchial plume in contrast with the trophosome where only one transcript could be detected. An alignment of these translated carbonic anhydrase cDNAs with vertebrate and nonvertebrate carbonic anhydrase protein sequences revealed the conservation of most amino acids involved in the catalytic site. According to the phylogenetic analyses, the two R. pachyptila transcripts clustered together but not all nonvertebrate sequences grouped together. Complete sequencing of the new carbonic anhydrase transcript revealed the existence of two slightly divergent isoforms probably coded by two different genes.
Clinical warning criteria in evaluation by computed tomography the secondary neurological headaches in adults

Our aims were to investigate the frequency of intracranial lesions detected by cranial computed tomography (CT‐scan) amongst adult patients who had clinical warning criteria (CWC) for secondary neurological headaches and to determine the importance of CWC in predicting a possible lesion on CT‐scan. Seventy consecutive patients with headache exhibiting CWC were included in this prospective study. The CWC included: (i) increase in the intensity and frequency of headache; (ii) abrupt onset of headache; (iii) persistence of headache despite analgesics; (iv) alteration of the characteristics of headache; and (v) presence of focal neurological symptoms or findings. The mean age of the patients was 46.5 years; the female‐to‐male ratio was 1.5. Of the patients, 35.7% had a neurological cause identified by CT‐scan, and 64.3% had normal CT‐scan. In the patients without lesion, of headaches, 64.4% were primary, and 35.6% were from undefined headache group. Although, of the above criteria, only the 5th was different markedly in the patients with lesion than the patients without lesion, in evaluation by CT‐scan the secondary neurological headaches in adults, all CWC should look for absolutely in their history and physical examination.
Effectiveness of a pay-for-performance intervention to improve maternal and child health services in Afghanistan: a cluster-randomized trial.

BACKGROUND A cluster randomized trial of a pay-for-performance (P4P) scheme was implemented in Afghanistan to test whether P4P could improve maternal and child (MCH) services. METHODS All 442 primary care facilities in 11 provinces were matched by type of facility and outpatient volume, and randomly assigned to the P4P or comparison arm. P4P facilities were given bonus payments based on the MCH services provided. An endline household sample survey was conducted in 72 randomly selected matched pair catchment areas (3421 P4P households; 3427 comparison). The quality of services was assessed in 81 randomly sampled matched pairs of facilities. Data collectors and households were blinded to the intervention assignment. MCH outcomes were assessed at the cluster level. RESULTS There were no substantial differences in any of the five MCH coverage indicators (P4P vs comparison): modern contraception(10.7% vs 11.2% (P = 0.90); antenatal care: 56.2% vs 55.6% (P = 0.94); skilled birth attendance (33.9% vs 28.5%, P = 0.17); postnatal care (31.2% vs 30.3%, P = 0.98); and childhood pentavalent3 vaccination (49.6 vs 52.3%, P = 0.41), or in the equity measures. There were substantial increases in the quality of history and physical examinations index (P = 0.01); client counselling index (P = 0.01); and time spent with patients (P = 0.05). Health workers reported limited understanding about the bonuses. CONCLUSIONS The intervention had minimal effect, possibly due to difficulties communicating with health workers and inattention to demand-side factors. P4P interventions need to consider management and community demand issues.
Long-term treatment with sildenafil in a thalassemic patient with pulmonary hypertension.

Pulmonary hypertension (PHT), defined as Doppler peak systolic tricuspid gradient (TG) higher than 30 mmHg, develops in a high percentage of patients with β-thalassemia (10% in thalassemia major and greater than 50% in thalassemia intermedia [TI]). Recent studies correlate PHT with age and high
Cerebral correlates of psychotic symptoms in Alzheimer's disease

BACKGROUND Psychotic symptoms are produced by distributed neuronal dysfunction. Abnormalities of reality testing and false inference implicate frontal lobe abnormalities. OBJECTIVES To identify the functional imaging profile of patients with Alzheimer's disease manifesting psychotic symptoms as measured by single photon emission computed tomography (SPECT). METHODS Twenty patients with Alzheimer's disease who had SPECT and clinical evaluations were divided into two equal groups with similar mini mental status examination (MMSE), age, sex, and the range of behaviours documented by the neuropsychiatric inventory (NPI), except delusions and hallucinations. SPECT studies, registered to a probabilistic anatomical atlas, were normalised across the combined group mean intensity level, and subjected to a voxel by voxel subtraction of the non-psychotic minus psychotic groups. Subvolume thresholding (SVT) corrected random lobar noise to produce a three dimensional functional significance map. RESULTS The significance map showed lower regional perfusion in the right and left dorsolateral frontal, left anterior cingulate, and left ventral striatal regions along with the left pulvinar and dorsolateral parietal cortex, in the psychotic versus non-psychotic group. CONCLUSION Patients with Alzheimer's disease who manifest psychosis may have disproportionate dysfunction of frontal lobes and related subcortical and parietal structures.
Hospital-Based Study of the Care and Cost of Acute Ischemic Stroke in Japan

Background and Purpose— To evaluate the current status of care and cost of acute ischemic stroke in Japan, we performed a hospital-based analysis at a tertiary emergency hospital with a 24-hour neurology-neurosurgery team and care unit. Methods— During the 12-month period of October 2000 to September 2001, we collected data on 179 patients consecutively hospitalized with acute ischemic stroke within 7 days of onset. We examined demographic data, in-hospital care, length of hospital stay, outcome at discharge, and hospital costs. The medical cost data were collected from official hospital medical cost charts, which calculated direct medical costs for beds, staff, examinations, medications, and rehabilitation. Results— The mean age was 70 years, and 69% were male. Hospital arrival was within 3 hours of onset in 30% of the patients. A history of stroke was present in 37%. The mean initial National Institutes of Health Stroke Scale score was 8.3 points (median, 6 points). Using the Trial of Org 10172 in Acute Stroke Treatment classification, 25% were lacunar, 27% were atherothrombotic, 33% were cardioembolic, and 15% were of unknown origin. All patients underwent neuroimaging studies during hospitalization; 96% and 92% underwent CT and MRI with MR angiography, respectively. Antithrombotic medications were given in 94%, none of whom received thrombolysis. A newly licensed neuroprotective agent, edaravone, was given in 16%. More than half of the patients (55%) were initially admitted to the neurological intensive care unit. Overall, 64% received in-hospital rehabilitation. Mean length of stay was 33 days. In-hospital mortality rate was 3%. On the modified Rankin Scale (mRS), 63% were independent (mRS, 0 to 2) and 34% were dependent (mRS, 3 to 5) at discharge. Two thirds of the patients (65%) went directly back home. The mean hospital cost per patient was $6887 ($209/d), of which 69% was attributable to the costs for beds and staff, 12% for medications, 7% for rehabilitation, 6% for imaging studies, 5% for laboratory examinations, and 1% for other costs. Conclusions— Despite the single hospital-based analysis, this study provided current, precise data on short-term inpatient care and costs of acute ischemic stroke in Japan. Because stroke often carries a permanent dependence, long-term cost-effective stroke care should be established.
The Protective Effects of Isoliquiritigenin and Glycyrrhetinic Acid against Triptolide-Induced Oxidative Stress in HepG2 Cells Involve Nrf2 Activation

Triptolide (TP), an active ingredient of Tripterygium wilfordii Hook f. , possesses a wide range of biological activities. Oxidative stress likely plays a role in TP-induced hepatotoxicity. Isoliquiritigenin (ISL) and glycyrrhetinic acid (GA) are potent hepatoprotection agents. The aim of the present study was to investigate whether Nrf2 pathway is associated with the protective effects of ISL and GA against TP-induced oxidative stress or not. HepG2 cells were treated with TP (50 nM) for 24 h after pretreatment with ISL and GA (5, 10, and 20 μM) for 12 h and 24 h, respectively. The results demonstrated that TP treatment significantly increased ROS levels and decreased GSH levels. Both ISL and GA pretreatment decreased ROS and meanwhile enhanced intracellular GSH content. Additionally, TP treatment obviously decreased the protein expression of Nrf2 and its target genes including HO-1 and MRP2 except NQO1. Moreover, both ISL and GA displayed activities as inducers of Nrf2 and increased the expression of HO-1, NQO1, and MRP2. Taken together the current data confirmed that ISL and GA could activate the Nrf2 antioxidant response in HepG2 cells, increasing the expression of its target genes which may be partly associated with their protective effects in TP-induced oxidative stress.