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Introduction. Shwachman-Diamond Syndrome (SDS) is an autosomal-recessive disorder characterized by neutropenia, pancreatic exocrine insufficiency, skeletal dysplasia, and an increased risk for leukemic transformation. Biallelic mutations in the SBDS gene have been found in about 90% of patients. The clinical spectrum of SDS in patients is wide, and variability has been noticed between different patients, siblings, and even within the same patient over time. Herein, we present two SDS siblings (UPN42 and UPN43) carrying the same SBDS mutations and showing relevant differences in their phenotypic presentation. Study aim. We attempted to understand whether other germline variants, in addition to SBDS, could explain some of the clinical variability noticed between the siblings. Methods. Whole-exome sequencing (WES) was performed. Human Phenotype Ontology (HPO) terms were defined for each patient, and the WES data were analyzed using the eVai and DIVAs platforms. Results. In UPN43, we found and confirmed, using Sanger sequencing, a novel de novo variant (c.10663G > A, p.Gly3555Ser) in the KMT2A gene that is associated with autosomal-dominant Wiedemann–Steiner Syndrome. The variant is classified as pathogenic according to different in silico prediction tools. Interestingly, it was found to be related to some of the HPO terms that describe UPN43. Conclusions. We postulate that the KMT2A variant found in UPN43 has a concomitant and co-occurring clinical effect, in addition to SBDS mutation. This dual molecular effect, supported by in silico prediction, could help to understand some of the clinical variations found among the siblings. In the future, these new data are likely to be useful for personalized medicine and therapy for selected cases. [ABSTRACT FROM AUTHOR]

Background: Diabetic retinopathy (DR) is a complication associated with uncontrolled DM. It is a leading preventable cause of visual impairment in the world and a cause of blindness in those under 75 years old in developing countries. We aimed to explore the prevalence and associated risk factors of DR among diabetic patients in the West Bank. Materials and Methods:A quantitative multicenter cross-sectional study was conducted in all West Bank cities. Nearly, 385 patients underwent a comprehensive eye examination in addition to blood and urine tests. A previously validated questionnaire for ocular examination classification was used together with a socio-demographic and past medical history information sheet. Results: The prevalence of all DR in the West Bank was 41.8%. The prevalence of non-proliferative diabetic retinopathy (NPDR) was 50.3% (38.5% for mild NPDR, 10.6% for moderate NPDR and 1.2% for severe NPDR). The prevalence of proliferative diabetic retinopathy (PDR) was 9.9% and 39.7% for diabetic macular edema (DME) (17.4% for mild, 15.5% for moderate and 6.8% for severe DME). The prevalence of vision-threatening PDR and DME was 49.7% for both. In a univariate analysis, DR was significantly associated with body mass index; BMI (p = 0.035), DM duration (p = 0.002), Low-density lipoprotein (LDL) (p = 0.034), glutamic-oxaloacetic transaminase (GOT) level (p = 0.016) andblood urea (BU) (p = 0.044). A multivariate analysis showed a strong significant association between DR andpatients who had DM for 10-19years (adjusted odds ratio; AOR (95%CI); 1.843 (1.05–3.22)), abnormal levels of LDL (AOR (95%CI); 0.50 (0.30–0.83)), abnormal levels of GOT (AOR (95%CI); 0.49 (0.27–0.89)), and overweight (AOR (95%CI); 0.39 (0.19–0.80)). Conclusions: We found that the prevalence of DR in Palestine was higher than the global prevalence. Referral coordination between ophthalmologists and internal physicians is necessary to better follow up with DR patients. An interventional educational program by clinicians and public health professionals is recommended. [ABSTRACT FROM AUTHOR]

The strawberry has a very short postharvest life due to its fast softening and decomposition. The goal of this research is to see how well a starch-silver nanoparticle (St-AgNPs) coating affects the physical, chemical, and microbiological qualities of strawberries during postharvest life. Additionally, the effect of washing with running water on silver concentration in coated strawberry fruit was studied by an inductively coupled plasma-optical emission spectrometer (ICP-OES). Furthermore, the shelf-life period was calculated in relation to the temperature of storage. Fourier transform infrared-attenuated total reflectance (FTIR-ATR), UV-Visible, and Transmission Electron Microscopic (TEM) were used to investigate the structure of starch-silver materials, the size and shape of AgNPs, respectively. The AgNPs were spherical, with an average size range of 12.7 nm. The coated samples had the lowest weight loss, decay, and microbial counts as compared to the uncoated sample. They had higher total acidity and anthocyanin contents as well. The washing process led to the almost complete removal of silver particles by rates ranging from 98.86 to 99.10%. Finally, the coating maintained strawberry qualities and lengthened their shelf-life from 2 to 6 days at room storage and from 8 to 16 days in cold storage. [ABSTRACT FROM AUTHOR]

The use of corrugated webs increases web shear stability and eliminates the need for transverse stiffeners in steel beams. Optimised regression learner techniques (ORLTs) are rarely used for calculating shear capacity in steel beam research. This study proposes a new approach for calculating the maximum shear capacity of steel beams with trapezoidal corrugated webs (SBCWs) by using ORLTs. A new shear model is proposed using ORLTs in accordance with plate buckling theory and previously developed formulas for predicting the shear strength of SBCWs. The proposed ORLT models are implemented using the regression learner toolbox of MATLAB software (2020b). The available data of more than 125 test results from different specimens prepared by previous researchers are used to create the model. In this study, web geometry and relevant web steel grades determine the shear capacity of SBCWs. Four regression methods are adopted. Results are compared with those of an artificial neural network model. The model output factor represents the ratio of the web vertical shear stress to the normalised shear stress. Shear capacity can be estimated on the basis of the resulting factor from the model. The proposed model is verified using two methods. In the first method, a series of tests are performed by the authors. In the second method, the results of the model are compared with the shear values obtained experimentally by other researchers. On the basis of the test results of previous studies and the current work, the proposed model provides an acceptable degree of accuracy for predicting the shear capacity of SBCWs. The results obtained using Gaussian process regression are the most appropriate because its recoded mean square error is 0.07%. The proposed model can predict the shear capacity of SBCWs with an acceptable percentage of error. The recoded percentage of error is less than 5% for 93% of the total specimens. By contrast, the maximum differential obtained is ±10%, which is recorded for 3 out of 125 specimens. [ABSTRACT FROM AUTHOR]

Nowadays, water pumping systems based on photovoltaics as a source of electricity have widely increased. System cost and efficiency still require enhancement in order to spread their application. Perovskite solar cells (PSCs) are the most hopeful third-generation photovoltaic for replacing the silicon-based photovoltaic thanks to their high power conversion efficiency, reaching 25.8%; tunable band-gap; long diffusion length; low fabrication temperature; and low cost. In this work, for the first time, we proposed a high-power-density hybrid perovskite solar cell thermoelectric generator (TEG) array for feeding a synchronous reluctance motor (SynRM) driving a water pump for use in an irrigation system. A control technique was used to achieve two functions. The first function was driving the motor to obtain the maximum torque/ampere. The second was harvesting the maximum perovskite solar cell array output power on the basis of the maximum power point tracking (MPPT) algorithm using the perturbation and observation approach. Thus, the proposed hybrid perovskite solar cell–thermoelectric generator feeds the motor via an inverter without DC–DC converters or batteries. Accordingly, the short life problems and the high replacement cost are avoided. The proposed complete system was simulated via the MATLAB package. Moreover, a complete laboratory infrastructure was constructed for testing the proposed high-power-density hybrid perovskite solar cell–TEG array for the water pumping system. The results revealed that using the high-power-density hybrid perovskite solar cell–TEG array, both the motor's output power and the pump's flow rate were improved by 11% and 14%, respectively, compared to only using the perovskite solar cell array. Finally, both the simulation and experimental results proved the high-performance efficiency of the system in addition to showing its system complexity and cost reduction. [ABSTRACT FROM AUTHOR]

Pomegranate is one of the most important and widely distributed trees. Boron and zinc are important nutrients for plant growth and fruit quality. Nanotechnology has emerged as one of the most innovative scientific fields in agriculture. This study was conducted to describe the changes in the physiochemical characteristics (weight, diameter, length, firmness and color), as well as the phytochemicals attributes (total phenolics, total flavonoids, ascorbic acid, anthocyanin and antioxidant %) and minerals contents, of pomegranates fruits of the 'Wonderful' cultivar as a result of spraying pomegranate trees using nanomaterials (zinc oxide (ZnONPs) and boron oxide (B2O3NPs)). In three successive developmental stages (full bloom, 6 weeks after full bloom and one month before harvest time), the trees were sprayed with 0.25, 0.5 and 1 g/L ZnONPs, as well as 0.25, 0.5 and 1 g/L B2O3NPs during the 2021 and 2022 seasons. The application of ZnONPs and B2O3NPs influenced the qualitative characteristics of the fruits in the studied seasons. The highest marketable % was observed for the 0.50 and 1 g/L ZnONPs and 1 g/L B2O3NPs compared to the other treatments. Also, a positive effect was recorded for the ZnONPs and B2O3NPs on the fruits' physical properties. All of the ZnONP and B2O3NP treatments resulted in increasing the total phenolic, flavonoid, anthocyanin and ascorbic acid contents and the antioxidant activity in the pomegranate juices. In conclusion, our results suggest that spraying pomegranate trees with ZnONPs and B2O3NPs improves the marketable fruit, enhances the fruit quality and increases the bioactive components and antioxidant activity. [ABSTRACT FROM AUTHOR]

Apricots are a fragile fruit that rots quickly after harvest. Therefore, they have a short shelf-life. The purpose of this work is to determine the effect of coatings containing chitosan (CH) as well as its nanoparticles (CHNPs) as thin films on the quality and shelf-life of apricots stored at room (25 ± 3 °C) and cold (5 ± 1 °C) temperatures. The physical, chemical, and sensorial changes that occurred during storage were assessed, and the shelf-life was estimated. Transmission electron microscopy was used to examine the size and shape of the nanoparticle. The nanoparticles had a spherical shape with an average diameter of 16.4 nm. During the storage of the apricots, those treated with CHNPs showed an obvious decrease in weight loss, decay percent, total soluble solids, and lipid peroxidation, whereas total acidity, ascorbic acid, and carotenoid content were higher than those in the fruits treated with CH and the untreated fruits (control). The findings of the sensory evaluation revealed a significant difference in the overall acceptability scores between the samples treated with CHNPs and the other samples. Finally, it was found that CHNP coatings improved the qualitative features of the apricots and extended their shelf-life for up to 9 days at room temperature storage and for 30 days in cold storage. [ABSTRACT FROM AUTHOR]

This paper presents the results of research and development of capacitive-based sensors of rotating shaft vibration for fault diagnostic systems of powerful turbines and hydro generators. It showed that diagnostic systems with special sensors are the key to increasing the reliability of powerful turbines and hydro generators. The application of sensors in monitoring systems was considered, and the requirements for the sensors used were analyzed. Structures of concentric capacitive-based sensors of rotating shaft vibration based on the measurement of the capacitance value from the distance to the metal surface were proposed. The design scheme was created for determining electrode dimensions of the rotating shaft vibration capacitive-based sensors with concentric electrodes, and analytical dependences were obtained. The calculation results allow the selection of optimal parameters of the active and guard electrodes. Analytical and computer simulation methods determined the response functions of the capacitive sensors. Analytical calculations and simulation results using 3D FEM were used to find the response functions of the sensors. The calculation of the characteristics of the capacitive-based sensors of rotating shaft vibration is presented. The study of the influence of fringe effects was carried out using the obtained results of the modeling and analytical calculations. [ABSTRACT FROM AUTHOR]