Miracle-dz/UniFAR-FTUs huggingface dataset:
Optical coherence tomography and fundus autofluorescence in children with infantile nystagmus syndrome and early-onset retinal dystrophy

Background: The purpose of the study was to compare morphological retinal differences in children with infantile nystagmus syndrome (INS) and early-onset retinal dystrophies (EORD) with healthy controls in order to evaluate the diagnostic value of optical coherence tomography (OCT) and fundus autofluorescence (FAF). Material and methods: Twenty-six children (mean age 10.0 years) were ophthalmologically and electrophysiologically diagnosed as INS and EORD. Their SD-OCT and FAF imaging was prospectively performed and compared with 36 controls (mean age 7.2 years). Results: Optical coherence tomography imaging was successfully completed in 17/26 (mean age 9.7 years) and FAF in 15/26 (mean age 11.1 years) children with EORD. OCT imaging was completed in 29/36 (mean age 8.9 years) and FAF in 15/19 (mean age 8.7 years) control children. In all six children with Leber congenital amaurosis (LCA) photoreceptor layer abnormalities were seen in the peripheral retina using SD-OCT, but macular images could not be obtained. In 5/5 children with congenital stationary night blindness (CSNB) retinal thinning was found in all regions except the fovea. 2/6 children with achromatopsia showed ellipsoid zone disruption with macula thinning. In 1/1 child with blue cone monochromatism reduced macular thickness was observed. In 5/5 children with cone-rod dystrophy the outer retinal signal intensity was decreased. In 2/3 children with complete CSNB, slight foveal hypoautofluorescence was indicated. 1/4 children with achromatopsia showed a central hyperautofluorescent ring. In 1/1 child with blue cone monochromatism slight foveal hypoautofluorescence was observed. 2/7 children with cone-rod dystrophy showed a central hyperautofluorescent ring, and additionally in 1/7 a macular hypoautofluorescence in one eye and an additional patch of hyperautofluorescence centrally in the other eye were found. Conclusion: The study demonstrated OCT and FAF as a non-invasive and fast diagnostic tool that defines morphological changes in early-onset retinal dystrophies in young children with nystagmus.
Checkpoints inhibitors in first line therapy of metastatic non-small cell lung cancer patients

Lung cancer is still the leader cause of mortality due to cancer worldwide (1,2). Five-year overall survival (OS) in unresectable and metastatic non-small cell lung cancer (NSCLC) patients (stage IIIB/IV) is less than 5%, regardless of the number of cytotoxic chemotherapy lines given (3). NSCLC is characterized for having different genetic and epigenetic alterations, where high rates of somatic mutations generate a variety of tumor specific antigens that enhance immunogenicity (4). Cancer is the result of the failure of different immunological mechanisms that attempt to eliminate cancer antigens that finally escape the immune system allowing tumor growth (5). Immunotherapy in a very short period of time not only became a reality but also achieved the most spectacular results ever in different types of cancer including NSCLC patients.
Customer information sharing and new service development: is there a link?

The purpose of this paper is to establish the link between customer information sharing and new service development. ,Through a survey of tourism firms, 295 questionnaires were collected in three large tourism locations in Tanzania. Thereafter, the hypotheses were tested by structural equation modeling (SEM) after undertaking both factor analysis and confirmatory factor analysis. ,Results indicate that there is a positive association/link between customer information sharing and new service development. The link here expresses the association that exists as customers share information and the extent of use of this information for the firm benefit in facilitating new service development. Specifically, customers post service information and customer interaction behaviors positively support new service development; however, customers’ pre-service information revealed no link. ,These results offer practical evidence that post service information and interaction behaviors form the groundwork for development of new services in service-related organizations. ,These results evidence that customer post service information and customer interaction behaviors form the groundwork for development new services in tourism. Hence, the study strengthens the value co-creation and innovation views in the service arena by extending knowledge in the use of both the service and the customer environment for service improvement.
Highly thermally stable Au–Al bimetallic conductive thin films with a broadband transmittance between UV and NIR regions

Metallic transparent conductive electrodes with broadband transparency have great potential for optoelectronic devices, and a huge challenge is to simultaneously maintain the conductivity and transparency of the metallic thin films at high temperatures. We demonstrate a facile method to fabricate transparent conductive Au–Al bimetallic thin films with excellent thermal stability up to 500 °C. With the existence of 10 nm-thick Al cap layers, the dewetting of the 10 nm-thick Au thin films is successfully suppressed owing to the formation of Au–Al alloys induced by the inter-diffusion of Al atoms. Owing to the formation of the Au–Al alloys, the conductivity of the bimetallic ultra-thin films was improved up to 7.9 Ω □−1, which is even superior to that of the as-deposited 20 nm-thick Au thin films. The Au–Al bimetallic thin films stably exhibit a high transmittance with an average value of ∼63% over a wide range between 350 and 2500 nm, which is higher than that of the commercial indium tin oxide (ITO) thin films with an identical resistance, especially for the regions above 1200 nm. The bimetallic thin films also have outstanding reliability after several cycles of annealing up to 500 °C, offering an effective route to overcome the limitation of transparent conductive thin films for optoelectronic devices working under harsh conditions, i.e. vacuum and high temperatures.
Exopolysaccharide-Producing Lactic Acid Bacteria – Health-Promoting Properties And Application In The Dairy Industry

Abstract Exopolysaccharides (EPS) are one of the classes of extracellular biopolymers synthesized by bacteria. Some strains of lactic acid bacteria (LAB) used in the dairy industry are able to synthesize EPS (EPS(+) strains). EPS may be secreted by a cell in the form of capsule or slime. Our review describes the factors influencing the activity of EPS production by LAB, the impact of the use of EPS(+) strains on the quality of fermented milk products (yoghurt, cheeses, etc.) and pro-health properties of EPS produced by LAB. The capability to synthesize EPS by LAB depends on many factors, e.g., affiliation to species and characteristics of strain, growth stage, composition of culture medium (type of carbon and nitrogen sources, and presence of other nutrients), temperature, pH, and presence of adjuvant microflora. The presence of EPS synthesized by LAB strains has a significant effect on changes in various properties of dairy products, including: yoghurt, kefir and many other fermented milk drinks, sour cream and cheeses. The EPS act as thickening, emulsifying and gelling agents, hence the use of EPS(+) strains may become a certain alternative to the use of thickeners in, e.g., fermented milks. During formation of a casein milk curd, EPS are able to bind water and thus reduce syneresis. The high water holding capacity of EPS has a positive effect on increasing viscosity and improving texture of low-fat cheeses. EPS are claimed to have health-promoting properties, like: anticarcinogenic, antioxidative, immunomodulatory and reducing blood cholesterol. 1. Introduction. 2. General characteristics of exopolysaccharides. 3. Factors affecting exopolysaccharides synthesis by lactic acid bacteria. 4. Effect of exopolysaccharides on the quality of fermented milk products. 4.1. Effect of EPS on the quality of yoghurts. 4.2. Effect of EPS on the quality of other fermented milk drinks. 4.3. Effect of EPS on the quality of cheeses. 5. Health-promoting properties of exopolysaccharides. 6. Conclusions
Fenestrated Denture: A Grace Option To Edentulous Patient

Edentulism can lead directly to impairment, functional limitation, physical, psychological, and social disability, and handicap. Edentulism was found to have a significant effect on residual ridge resorption, which leads to a reduction in the height of alveolar bone and the size of the denture bearing area. Fenestrated denture is a concept which preserves remaining teeth. This case report discusses the advantages of exploitation few remaining teeth to minimize alveolar ridge resorption and improvement of retention and support through using fenestrated denture which overcomes many problems encountered by complete denture and removable partial denture patients.
Middle Miocene Palynoflora from the Adamów Lignite Deposit, Central Poland

ABSTRACT The first mid-Polish group is the youngest among the main Neogene lignite seams in Poland. Lignites of this group developed in the middle Miocene over almost the whole of Poland, and therefore they are an important correlation horizon throughout much of the Polish Lowlands. A total of 30 palynological samples from the 3-metre seam from the Adamów deposit (central Poland) were studied in detail. The results revealed the presence of wetland and mesophytic vegetation during the time of sedimentation. The study area was overgrown by palustrine wetland communities similar in their composition to modern pocosins. The climate was warm temperate and humid, which was inferred from the palynoflora composition, including frequency of palaeotropical and palaeotropical/warm-temperate taxa, and the presence of epihyllous fungi. The estimated mean annual temperature (MAT) for the lignite seam at Adamów is 15.7–18.0°C. Comparison with other palynofloras from the first mid-Polish lignite seam group shows that the climate was more or less homogenous within the entire Polish Lowlands during formation of the group of seams. The MAT ranges are also similar to other results from middle Miocene of Central Europe. The differences between the Adamów palynoflora and palynofloras from central and western Poland, dominated by swamp forests, most probably reflect the succession of plant communities in different hydrological and trophic conditions.
Dysferlin in skeletal and heart muscle: from trafficking to therapy

Dysferlinopathies are a heterogeneous group of congenital muscle disorders characterized by late-onset muscular dystrophy and increased muscle prowess before onset of symptoms. Mutations in the DYSF gene encoding the protein dysferlin have been identified as the common genetic cause of this rare disease spectrum. However, little is known about the pathomechanism leading to muscle degeneration and therefore, no curative treatment is available. Dysferlin is a tail-anchored multi-C2 domain protein belonging to the ferlin protein family. For a long time, it has mainly been implicated in membrane repair. In recent years, new evidence uncovered an additional and fundamental role for dysferlin: the formation of the T-tubule system in skeletal muscle. The aim of this study was to further elucidate the cellular role in skeletal and heart muscle and the trafficking of dysferlin. This would help to reveal the molecular pathomechanism of dysferlin-deficient muscular dystrophy and give the possibility to identify new targets for the development of therapeutic approaches. The finding of an abnormal T-tubule system in developing dysferlinopathy patient myotubes suggests that dysferlin is essential during a very early stage of T-tubule biogenesis in skeletal muscle. Not only the T-tubule system in skeletal muscle, but also the cardiac tubular system of dysferlin-deficient mice is altered with loss and axialization of membrane tubules. This finding, together with the localization and increased expression of dysferlin at the developing T-tubule system in cardiomyocytes, support the role of dysferlin as a key player of cardiac T-tubule biogenesis. Furthermore, these findings may explain the pathology of dysferlin-deficiency in skeletal and heart muscle. The investigation of the cellular trafficking shows that mutated dysferlin is retained in the endoplasmic reticulum or Golgi network and provides evidence that functional dysferlin follows the secretory pathway to reach the plasma and T-tubule membrane. Treatment with the chemical chaperone 4-phenylbutyrate (PBA) increases the steady-state protein levels and partially rescues functional deficits of dysferlin mutants. Thus, PBA might be a potential therapeutic for the treatment of dysferlinopathy. Another therapeutic approach is the induction of translational readthrough by aminoglycosides in patients harboring nonsense DYSF mutations. The systematic analysis of readthrough induction for all recurrent DYSF nonsense mutations revealed a high variety of readthrough efficiency and might provide a suitable prediction for the treatability of dysferlinopathy patients. This gives the possibility to selectively treat patients with high therapeutic potential as aminoglycosides are associated with severe adverse effects.
Managing technological innovation in the sports industry: a challenge for retail management

The purpose of this paper is twofold: first, to investigate the effects of the digitalization process on the demand and supply side in the sports world through the advent of mobile apps and online services, such as YouTube, which provide free (or almost) real fitness courses; and second, to investigate how technological innovation is managed in the sports sector. ,Drawing on a survey research approach, this study collected 150 questionnaires from consumers in the North of Italy. ,Based on a descriptive analysis of the data, the findings show that the digital channel becomes significant at an exponential rate and takes on a leading role in today’s era, especially among young people. However, the results also show that the traditional channel of use of the sports service (gyms/fitness centers) is privileged by consumers under the age of 35, even though there is a weak penetration of innovative tools offered by the innovation technology (mobile app and video tutorials on social networks such as YouTube) in the sports sector. ,The study provides an exhaustive overview of academic literature on technology and innovation management, and provides the contact point between the physical and digital world in the sports sector, and adds a significant scholarly and practical value in the exercise at home perspective of consumers. This research also contributes to the discussion on the competitiveness between the two channels (physical and digital one) in the sports sector, thus giving rise to further research concerning the offer of the type of sports service. ,This study is among the few that connect the technological advancements and management within the sports industry and provides the ground and theoretical basis for the scholar community to further build on.
Global Value Chains and External Adjustment: Do Exchange Rates Still Matter?

The paper explores how international integration through global value chains shapes the working of exchange rates to induce external adjustment both in the short and medium run. The analysis indicates that greater integration into international value chains reduces the exchange rate elasticity of gross trade volumes. This result holds both in the short and medium term, pointing to the rigidity of value chains. At the same time, greater value chain integration is associated with larger gross trade flows, relative to GDP, which tends to amplify the effect of exchange rate movements. Overall, combining these two results suggests that, for most countries, integration into global value chains does not materially alter the working of exchange rates and the benefits of exchange rate flexibility in facilitating external adjustment remain.
THE DEVELOPMENT OF ROLE-PLAYING LEARNING MODEL DESIGN ON ACCOUNTING SUBJECT

THE DEVELOPMENT OF ROLE-PLAYING LEARNING MODEL DESIGN ON ACCOUNTING SUBJECTAbstractThis study was aimed to generate a product of an effective role-playing learning model design on basic competences of practicing accounting cycles in service companies for the eleventh grade students. The research procedures were done based on ADDIE model. The developed product design was validated by the experts of learning materials, the experts of learning media, the experts of learning design, and the experts of characters building. The product was tested on Stella Duce 1 Senior High School students both in small groups which consisted of 12 people and a large group of 40 people. The instruments which was used to assess product validity included material aspects, media aspects, aspects of learning, and aspects of character building. The result of the experts’ validation and testing to students showed that it was a very good developed product. This meant that the role-playing learning model design on basic competences of practicing accounting cycles in service companies for the eleventh grade students was worth implementing in the learning activities.PENGEMBANGAN DESAIN PEMBELAJARAN MODEL ROLE-PLAYING PADA MATA PELAJARAN AKUNTANSIAbstrakPenelitian ini bertujuan untuk menghasilkan produk berupa desain pembelajaran model role-playing yang efektif untuk pembelajaran pada kompetensi dasar mempraktikkan siklus akuntansi perusahaan jasa untuk siswa kelas XI SMA. Prosedur pengembangan produk penelitian dilakukan berdasarkan Model ADDIE. Produk yang dikembangkan divalidasi oleh ahli materi pembelajaran, ahli media pembelajaran, ahli desain pembelajaran, dan ahli pendidikan karakter. Produk diujicobakan kepada siswa-siswa SMA Stella Duce 1 baik kelompok kecil yang terdiri dari 12 orang maupun kelompok besar yang terdiri dari 40 orang. Instrumen untuk menilai validitas produk mencakup aspek materi, aspek media, aspek pembelajaran, dan aspek pendidikan karakter. Hasil validasi dari para ahli dan hasil ujicoba pada siswa-siswa menunjukkan bahwa produk yang dikembangkan dinyatakan sangat baik. Artinya, desain model role-playing yang dikembangkan untuk pembelajaran pada kompetensi dasar mempraktikkan siklus akuntansi perusahaan jasa untuk siswa kelas XI SMA layak untuk diimplementasikan dalam praktik pembelajaran.
Prognostic value of chronic hepatitis B virus infection in patients with breast cancer in a hepatitis B virus endemic area

Background Except for hepatocellular carcinoma, chronic hepatitis B virus (HBV) infection has also been reported to be associated with increased morbidity and mortality of other cancers. However, the impact of chronic HBV infection on the prognosis of breast cancer (BC) remains unclear. Our study aimed to evaluate the prognostic value of HBV infection for BC in an endemic area of HBV in China. Methods There was a total of 1,904 patients with early BC who underwent mastectomy or breast-conserving surgery enrolled in our study. HBV infection on overall survival (OS) and hepatic metastasis-free survival (HMFS) was the main research indicator for this study. Results A total of 212 patients (11.1%) were identified with chronic HBV infection due to serum hepatitis B surface antigen (HBsAg) positive. HBsAg-positive patients had inferior OS (84.9% vs. 90.4%, P=0.005) and HMFS (92.5% vs. 97.1%, P=0.016) at 5 years than HBsAg-negative patients. Chronic HBV infection was an independent predictor of poor OS in patients with BC [multivariate analysis; hazard ratio (HR), 1.52; P=0.038], but not for HMFS. Subgroup analysis showed that chronic HBV infection was an unfavorable independent prognostic factor for OS in patients with stage II/III BC (HR, 1.59; P=0.025). The 5-year OS and HMFS rates of HBsAg-positive patients were 81.9% and 90.5% for patients with stage II/III BC, while those rates of HBsAg-negative patients were 88.5% and 96.3%, respectively. In stage I patients, there was no significant difference in 5-year OS (95.8% vs. 97.1%; P=0.629) and HMFS (100.0% vs. 99.0%; P=0.447). Conclusions In conclusion, chronic HBV infection predicts a worse prognosis in patients with stage II/III BC, but not stage I BC.
Patterns of polydrug use among pregnant substance abusers

Aim: Studies of drug use during pregnancy have generally focused on individual substances or specific combinations of drugs. The aim of this article is to increase our knowledge about polydrug use and pregnancy in a Nordic context by describing the sociodemographic characteristics of a clinical population of pregnant women with severe substance use, examining the scope and type of polydrug use and analysing factors associated with concurrent use of many, as opposed to a few, drugs. Method: A cross-sectional study of pregnant women on admission to compulsory care for substance abuse in Sweden between 2000 and 2009 (n = 119 women, representing 128 pregnancies). Data were retrieved from administrative registers and client records. Univariate links between demographic, social, obstetrical, treatment history variables and polydrug use were examined. Binary logistic regression was used to analyse the association between explanatory variables and polydrug use. Results: The average number of drugs being used concurrently was 2.65, and injection drug use was recorded in 73% of the pregnancies. Opiates and amphetamines were the most common primary drugs, followed by alcohol. The likelihood of polydrug use increased with first trimester pregnancy, planned (as opposed to emergency) committals, as well as the combination of partner substance abuse and injection drug use. Conclusions: Polydrug use was widespread among pregnant substance abusers. Policies, interventions and research often focus on individual drugs separately, but for clinical populations in particular there is a need to address drug use broadly, including a systematic recording of smoking habits. This also entails awarding more attention to those not eligible for established interventions, such as opiate maintenance treatment, and giving more consideration to a variety of life circumstances, such as partner drug use.
Characteristics, Main Impacts, and Stewardship of Natural and Artificial Freshwater Environments: Consequences for Biodiversity Conservation

In this overview (introductory article to a special issue including 14 papers), we consider all main types of natural and artificial inland freshwater habitas (fwh). For each type, we identify the main biodiversity patterns and ecological features, human impacts on the system and environmental issues, and discuss ways to use this information to improve stewardship. Examples of selected key biodiversity/ecological features (habitat type): narrow endemics, sensitive (groundwater and GDEs); crenobionts, LIHRes (springs); unidirectional flow, nutrient spiraling (streams); naturally turbid, floodplains, large-bodied species (large rivers); depth-variation in benthic communities (lakes); endemism and diversity (ancient lakes); threatened, sensitive species (oxbow lakes, SWE); diverse, reduced littoral (reservoirs); cold-adapted species (Boreal and Arctic fwh); endemism, depauperate (Antarctic fwh); flood pulse, intermittent wetlands, biggest river basins (tropical fwh); variable hydrologic regime—periods of drying, flash floods (arid-climate fwh). Selected impacts: eutrophication and other pollution, hydrologic modifications, overexploitation, habitat destruction, invasive species, salinization. Climate change is a threat multiplier, and it is important to quantify resistance, resilience, and recovery to assess the strategic role of the different types of freshwater ecosystems and their value for biodiversity conservation. Effective conservation solutions are dependent on an understanding of connectivity between different freshwater ecosystems (including related terrestrial, coastal and marine systems).