Lafora Disease Masquerading as Hepatic Dysfunction

Lafora disease is fatal intractable progressive myoclonic epilepsy. It is frequently characterized by epileptic seizures, difficulty walking, muscle spasms, and dementia in late childhood or adolescence. We chronicle here an unusual case of an asymptomatic young male soccer player who presented with elevated liver enzymes. Neurological examination was unremarkable. The diagnostic workup for hepatitis, infectious etiologies, autoimmune disorders, hemochromatosis, Wilson\u27s disease, alpha-1 antitrypsin deficiency, and other related diseases was inconclusive. He subsequently underwent an uneventful percutaneous liver biopsy. Based on the pathognomonic histopathological findings, Lafora disease was considered the likely etiology. The present study is a unique illustration of this rare disorder initially manifesting with abnormal liver enzymes. It underscores the importance of clinical suspicion of Lafora disease in cases with unexplained hepatic dysfunction. Prompt liver biopsy and genetic testing should be performed to antedate the onset of symptoms in these patients

Endoscopic Submucosal Tunnel Dissection as a Novel Therapeutic Technique in Patients With Barrett’s Esophagus

© 2020 American Federation for Medical Research. With the ameliorated resectability prowess of endoscopic techniques, a myriad of diseases previously treated by major ablative surgeries are now endoscopically curable. Endoscopic submucosal tunnel dissection (ESTD) is a relatively new technique that has diversified endoscopic application. Although ESTD has frequently been used for the resection of esophageal neoplastic lesions, the clinical evidence pertaining to its efficacy in the treatment of circumferential Barrett’s esophagus remains sparse. In this study, we evaluated ESTD as a potential therapeutic technique in patients with Barrett’s esophagus-related high-grade dysplasia. The tunneling strategy helped achieve complete en bloc resection at an increased dissection speed, without any procedural complications. This article illustrates that ESTD can be a feasible, safe, and effective treatment for dysplastic Barrett’s esophagus. Future research should aim to stratify the potential risks and complications associated with this optimization of endoscopic submucosal dissection in patients with superficial esophageal lesions

Value-Based Fuzzy Approach for Non-functional Requirements Prioritization

Non-functional requirements (NFRs) are often addressed late in a project and, in turn, can get less attention in the requirements prioritization (RP) process. For various reasons, RP may happen based on functional requirements (FRs) only. While many approaches for prioritizing NFRs have been published, these are known also for some limitations, e.g. not being scalable, being domain-specific and not able to cope with changing requirements. In this paper, we proposes a value-based fuzzy approach for prioritizing NFRs together with FRs. Our proposed approach takes into account (1) the relationships of NFRs with FRs using experts’ evaluations and fuzzy logic, and (2) the dependencies among both types of requirements and also the interdependencies that particularly exist among the NFRs themselves. We evaluated our proposal by conducting a real-world case study of an ATM system. We also compared the list of prioritized NFRs with the list of NFRs prioritized by different stakeholders on the basis of classification factors. The results of applying the proposed approach on NFRs of ATM system show that the approach produces a conflict-free and consistent list of prioritized NFRs.</p

Mutations in phosphodiesterase 6 identified in familial cases of retinitis pigmentosa.

To delineate the genetic determinants associated with retinitis pigmentosa (RP), a hereditary retinal disorder, we recruited four large families manifesting cardinal symptoms of RP. We localized these families to regions on the human genome harboring the α and β subunits of phosphodiesterase 6 and identified mutations that were absent in control chromosomes. Our data suggest that mutations in PDE6A and PDE6B are responsible for the retinal phenotype in these families

Fuzzy modeling and parameters optimization for the enhancement of biodiesel production from waste frying oil over montmorillonite clay K-30

Transesterification is a promising technology for the biodiesel production to provide an alternative fuel that considers the environmental concerns. From the economic and environmental protection points of view, utilization of waste frying oil for the production of biodiesel addresses very beneficial impacts. Production of higher yield of biodiesel is a challenging process in order to commercialize it with a lower cost. The current study focuses on the influence of different parameters such as reaction temperature (°C), reaction period (min), oil to methanol ratio and amount of catalyst (wt%) on the production of biodiesel. The main objective of this work is to develop a model via fuzzy logic approach in order to maximize the biodiesel produced from waste frying oil using montmorillonite Clay K-30 as a catalyst. The optimization for the operating parameters has been performed via particle swarm optimization (PSO) approach. During the optimization process, the decision variables were represented by four different operating parameters: temperature (40–140 °C), reaction period (60–300 min), oil/methanol ratio (1:6–1:18) and amount of catalyst (1–5 wt%). The model has been validated with the experimental data and compared with the optimal results reported based on other optimization techniques. Results showed the increment of biodiesel production by 15% using the proposed strategy compared to the earlier study. The obtained biodiesel production yield reached 93.70% with the optimal parameters for a temperature at 69.66 °C, a reaction period of 300 min, oil/methanol ratio of 1:9 and an amount of catalyst of 5 wt%

Development of Reaction Kinetics Model for the Production of Synthesis Gas from Dry Methane Reforming

The energy supply systems dependent on fossils and municipal solid waste (MSW) materials are primarily responsible for releasing greenhouse (GHG) gases and their related environmental hazards. The increasing amount of methane (CH4) and carbon dioxide (CO2) is the scientific community's main concern in this context. Reduction in the emission amount of both gases combined with the conversion technologies that would convert these total threat gases (CO2 and CH4) into valuable feedstocks will significantly lower their hazardous impact on climate change. The conversion technique known as dry methane reforming (DMR) utilizes CO2 and CH4 to produce a combustible gas mixture (CO+H2), popularly known as synthesis gas/or syngas. Therefore, this research study aims to explore and enlighten the characteristics of the DMR mechanism. The conversion behaviour of CO2 and CH4 was studied with modelling and simulation of the DMR process using MATLAB. The results showed that inlet gas flow has a significant impact on the reactions. In contrast, the inlet molar composition ratio of the reactions was found to have no substantial effect on the mechanism of DMR. Copyright © 2021 by Authors, Published by BCREC Group. This is an open access article under the CC BY-SA License (https://creativecommons.org/licenses/by-sa/4.0).

Influence of Surfactant-Mediated Interparticle Contacts on the Mechanical Stability of Supraparticles

[Image: see text] Colloidal supraparticles are micron-scale spherical assemblies of uniform primary particles, which exhibit emergent properties of a colloidal crystal, yet exist as a dispersible powder. A prerequisite to utilize these emergent functionalities is that the supraparticles maintain their mechanical integrity upon the mechanical impacts that are likely to occur during processing. Understanding how the internal structure relates to the resultant mechanical properties of a supraparticle is therefore of general interest. Here, we take the example of supraparticles templated from water/fluorinated oil emulsions in droplet-based microfluidics and explore the effect of surfactants on their mechanical properties. Stable emulsions can be generated by nonionic block copolymers consisting of a hydrophilic and fluorophilic block and anionic fluorosurfactants widely available under the brand name Krytox. The supraparticles formed in the presence of both types of surfactants appear structurally similar, but differ greatly in their mechanical properties. While the nonionic surfactant induces superior mechanical stability and ductile fracture behavior, the anionic Krytox surfactant leads to weak supraparticles with brittle fracture. We complement this macroscopic picture with Brillouin light spectroscopy that is very sensitive to the interparticle contacts for subnanometer-thick adsorbed layers atop of the nanoparticle. While the anionic Krytox does not significantly affect the interparticle bonds, the amphiphilic nonionic surfactant drastically strengthens these bonds to the point that individual particle vibrations are not resolved in the experimental spectrum. Our results demonstrate that seemingly subtle changes in the physicochemical properties of supraparticles can drastically impact the resultant mechanical properties

Allelopathic effect of Ficus benjamina leaf extract, litter, and mulch on germination and growth of sunflower

Allelopathy is an important biological process, which has direct or indirect effects on the germination and growth potentials of plants. Awareness about the allelopathic properties of plants which prevail in agricultural systems can help growers to amend crop cultivation patterns accordingly. In this study, we evaluated the allelopathic effects of Ficus benjamina on germination and early seedling growth of four hybrids of sunflower (Oliver, Parsun-3, SFH-80 and NK- S- 278). Ethanolic and hot-water aqueous extracts from leaves, while litter and mulches of the test allelopathic plant significantly reduced germination, radicle and hypocotyle growth of sunflower. Germination percentage was drastically reduced in all the four sunflower hybrids by ethanolic, hot-water and litter extracts; however, compared to control, mulching assay significantly increased germination in hybrids Oliver (76%), Parsun-3 (42%), SFH-80 (78%) and NK-S-278 (30%) at 2, 4, 8 and 12g extract concentration, respectively. Hypocotyle and radicle length of test hybrids were significantly reduced in each assay type. Among tested assays, ethanolic extracts revealed more drastic effects on the studied parameters than hot-water, litter, and mulching. Sunflower hybrid NK-S-278 was more severely affected, while Parsun-3 exhibited resistance to the allelopathic stress. Inhibitory effects were more prominent with increasing concentration of the extracts. The order of the phytotoxic effects of tested bioassays was ethanolic extract˃ hot-water˃ litter˃ mulching. The study suggested that Ficus leaves may possess potent allelochemicals with growth inhibitory effects on sunflower seedlings. It is suggested that further study might be required to check the allelopathic effect of Ficus benjamina on germination and growth of these sunflower hybrids in field conditions

Structural and Ion Dynamics in Fluorine-Free Oligoether Carboxylate Ionic Liquid-Based Electrolytes

Here, we investigate the physicochemical and electrochemical properties of fluorine-free ionic liquid (IL)-based electrolytes with two different cations, tetrabutylphosphonium, (P-4,4,4,(4))(+), and tetrabutylammonium, (N-4,N-4,N-4,N-4)(+), coupled to a new anion, 2-[2-(2-methoxyethoxy)ethoxylacetate anion (MEEA), for both neat and (P-4,P-4,P-4,P-4) (MEEA)(-), also doped with 10-40 mol % of Li(MEEA). We find relatively weaker cation-anion interactions in (P-4,P-4,P-4,P-4)(MEEA) than in (N-4,N-4,N-4,N-4)(MEEA), and for both ILs, the structural flexibility of the oligoether functionality in the anion results in low glass transition temperatures, also for the electrolytes made. The pulsed field gradient nuclear magnetic resonance (PFG NMR) data suggest faster diffusion of the (MEEA)(-) anion than (P-4,P-4,P-4,P-4)(+) cation in the neat IL, but the addition of a Li salt results in slightly lower mobility of the former than the latter and lower ionic conductivity. This agrees with the combined Li-7 NMR and attenuated total reflection-Fourier transform infrared (ATR-FTIR) spectroscopy data, which unambiguously reveal preferential interactions between the lithium cations and the carboxylate groups of the IL anions, which also increased as a function of the lithium salt concentration. In total, these systems provide a stepping stone for further design of fluorine-free and low glass transition temperature IL-based electrolytes and also stress how crucial it is to control the strength of ion-ion interactions

Germination, growth and yield performance of flax (Linum Usitatissimum L.) nnder gamma irradiation stress

Gamma irradiation is a widely manipulated mutation breeding approach in agriculture for producing crops with desired agronomic traits. The technique is particularly advantageous to conventional breeding methods because of minimal labor and time requirement. Under laboratory and field experiments during 2013, seeds of Linum usitatissimum L. were irradiated with 2, 4, 8, 12, 16, 20, 24, 28 and 32 krad of gamma irradiation doses from Co-60 source for evaluating their effects on germination, seedling survival, radicle and plumule lengths, vegetative growth and productivity. It was noted that radiation doses caused significant changes in the studied traits of test plant. Germination, radicle and plumule lengths in lab study while shoot length, number of leaves and leaf area, number of fruits plant-1, number of seeds fruit-1, husk weight fruit-1, number of branches plant-1, fresh and dry biomass and moisture content of shoots under pot culture varied significantly under the applied radiation stress. In general, radiation doses up to 8 kr had stimulatory effects on the studied parameters while doses exceeding 8 kr negatively influenced germination, growth and productive attributes of flax. Results observed both stimulatory and inhibitory effects of the irradiation doses. The study suggests that radiation doses above 32 krad induced lethal effects on general growth of flax