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Mutations in the human PCDH19 gene lead to epileptic encephalopathy of early childhood. It is characterized by the early onset of serial seizures, cognitive impairment and behavioral disorders (including autistic personality traits). In most cases, difficulties arise in selecting therapy due to pharmacoresistance. The pathogenesis of the disease is complex. The data available to us at the moment from numerous studies present the pathogenesis of «PCDH19 syndrome¼ as multi-level, affecting both the epigenetic support of cell life, and development of stem cells and progenitor cells in the process of neuroontogenesis, and the influence on the neurotransmitter mechanisms of the brain, and disruption of the formation of neural networks with an inevitable increase in the excitability of the cerebral cortex as a whole, and local changes in the highly labile regulatory structures of the hippocampal region. And it is not surprising that all these changes entail not only (and perhaps not so much) epileptization, but a profound disruption of the regulation of brain activity, accompanied by autism spectrum disorders, more profound disorders in the form of schizophrenia or cyclothymia, and the formation of delayed psychomotor development. A «side branch¼ of these pathogenetic processes can also be considered the participation of PCDH19 dysfunctions in certain variants of oncogenesis. The need for polypharmacy (in most cases) confirms the diversity of mechanisms involved in the pathogenesis of the disease and makes the prospects for the development of effective and rational treatment regimens very vague. Cautious optimism is caused only by attempts at relatively specific treatment with ganaxolone.

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Non-photochemical laser-induced nucleation (NPLIN) in supersaturated potassium bromide (KBr) solutions with the addition of acidic polymers is reported here for the first time. Upon absorbing the incident laser, crystallites are immediately induced along the laser pathway in the solution, eventually growing into needle-shaped crystals of varying sizes. When comparing induction time, nucleation probability, and crystal habits with spontaneous nucleation, the results suggest that NPLIN creates a distinct morphological pathway, transforming cubic crystals into needle-like structures. Additionally, it improves crystallization probability and growth rate. This paper aims to realize control from crystal nucleation to crystal growth by adding acidic polymers to the process of laser-induced nucleation, potentially influencing crystal morphology modification in NPLIN. With 19 wt% acidic polymers added to the solution as additives, control over both crystal growth and morphological modifications was observed: cubic KBr crystals with square patterns were produced through laser irradiation, and there was a varying reduction in both the number and growth rate of the crystals. The influence of acidic polymers on the solution environment was analyzed to determine the reasons for the variations in crystal quantity and growth speed. The underlying mechanisms responsible for the changes in crystal shape were also discussed.

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Directional control of the process of doughs with nutrition fortification is challenging. Thus, this study aimed to develop non-starch polysaccharides that can modify the quality of flour products. Polysaccharides were extracted from three different garlic cultivars, evaluated for physicochemical properties and used to enrich doughs for microstructure and mesoscopic characteristics analysis. We assessed the moisture distribution, texture characteristics, thermodynamic properties, dynamic viscoelastic properties, protein structure, microstructure and molecular interaction of the doughs and demonstrated a relatively high molecular weight, lower steric hindrance of molecular chains and higher cross-linking ability with the dough network in the supernatant polysaccharide from Yunnan single-clove-garlic (SGSOS) fraction. These features of SGSOS fraction improved the rheological, thermodynamic, texture characteristics, and water distribution of doughs. These findings provide information on the use of garlic polysaccharides during the processing and manufacturing of foods to enhance their processing adaptability and qualities.

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BACKGROUND: Altered trace element status is associated with epilepsy in humans and dogs with idiopathic epilepsy (IE). OBJECTIVES: Compare hair element concentrations in epileptic and healthy dogs. ANIMALS: Sixty-three dogs with IE (53 treated, 10 untreated) and 42 controls. METHODS: Case-control study using ICP-MS to determine hair calcium, magnesium, phosphorus, sodium, potassium, iron, copper, manganese, zinc, selenium, chromium, lead, mercury, cadmium, arsenic, aluminum, and nickel concentration. Groups were compared using nonparametric tests. Results were controlled for diet, sex, age, and hair color using generalized linear mixed models. RESULTS: Compared to healthy controls, dogs with IE had lower hair phosphorus (mean ± SD; IE: 286.19 ± 69.62 µg/g, healthy: 324.52 ± 58.69 µg/g; P = .001), higher hair copper (IE: 10.97 ± 3.51 µg/g, healthy: 8.41 ± 1.27 µg/g; P < .001), zinc (IE: 158.25 ± 19.64 µg/g, healthy: 144.76 ± 32.18 µg/g; P < .001), copper/zinc ratio (IE: 0.07 ± 0.02, healthy: 0.06 ± 0.01; P = .003), selenium (IE: 1.65 ± 0.43 µg/g, healthy: 0.94 ± 0.73 µg/g; P < .001), and arsenic (IE: 0.40 ± 0.78 µg/g, healthy: 0.05 ± 0.08 µg/g; P < .001). When comparing treated and untreated epileptic dogs with healthy dogs, the differences in phosphorus and selenium remained significant for both groups, whereas the differences in copper, zinc, and arsenic were significant only for treated dogs. Potassium bromide treatment was strongly associated with high hair arsenic (P = .000). CONCLUSIONS AND CLINICAL IMPORTANCE: Altered trace element status could be involved in the pathophysiology of IE in dogs. Antiseizure drugs might affect trace element and arsenic metabolism.

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Low thawing rates affects the quality of the product, which necessitates the use of new thawing technologies. In this study, the effect of alternating magnetic field (MF) (1-80 Hz) on the thawing rate, physicochemical properties, protein denaturation, and structure of rainbow trout fillet was studied and compared with refrigerator thawing (RT) (4 °C). Thawing time remarkably decreased in MF thawing as compared to RT. The results revealed that the water holding capacity of RT was significantly lower than that of MF, which was consistent with the results of structural investigation characterized by Scanning electron microscopy. Particle size distribution, Fourier transform infrared spectroscopy, and Differential scanning calorimetry indicated that MF could unfold and aggregate the protein structure at 40 and 80 Hz. The findings of this study indicated the potential of the MF in accelerating the thawing process. However, more studies are needed to clarify all aspects of the process.

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Physico-chemical properties of potato starch-based foods (PSBF) interacted with milk protein (MP), and soybean oil (SBO) were investigated. Microstructures, rheological properties, and chemical bonding among those ingredients were determined. An emulsion-filled gel, in which oil droplets stabilized by MP and/or amylose-lipid complex (ALC) dispersed in a starch gel structure of PSBF was revealed by confocal laser scanning microscopy. Starch-starch, protein-oil, and protein-protein played the dominant interactions while ALC and starch-protein interaction were subordinates. Rheological data showed that MP induced a thinning effect on starch gel, while SBO seemed to reinforce the solid-like properties of the gel. The chemical interactions analyzed by FTIR, Raman, and X-ray diffraction suggested that these foods were lack in non-covalent crosslinks and were dominated by diverse physical interactions. However, the different preparation of such foods could induce chemical binding in a different way and MP and SBO could affect the properties of PSBF in this study.

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Potassium bromide is a frequently used antiseizure medication with a half-life time of over 25 days. Oral intake of sodium chloride as well as renal function influence this half-life time and may have an influence on the needed dose to get proper serum levels. The hypothesis is that dogs living close to coastal areas require a greater potassium bromide dose than dogs living more inland. The main study objective was to determine the relationship between bromide dose, serum bromide concentration, treatment duration, type of food, concurrent therapies and the proximity of the dog's residency to a coastal area. A retrospective cross-sectional study was performed. A total of 658 bromide serum measurements were retrieved from the veterinary faculty's laboratory archive, Utrecht University, The Netherlands. Information on the bromide dose, renal function, treatment period, type of food, concurrent therapies and residence was obtained retrospectively from owners of all dogs using a postal survey. A dataset of 220 unique study units was created. The dogs were grouped based on their residence (proximity to the coast > or <50 km). Differences between the groups of dogs regarding bromide dose, serum bromide concentration, treatment duration, type of food and concurrent therapies were analyzed to evaluate the effect of residence on bromide dose and serum concentration. Results: Although not statistically significant there is a trend that dogs living in close proximity to the sea may require a higher dose of potassium bromide to maintain therapeutic concentrations compared to dogs living more inlands. Additional studies are needed to further explore this observation.

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This study evaluated the effect of molecular weight of chitosan (3 kDa,150 kDa, 400 kDa, and 600 kDa) on zein-nisin-chitosan nanocomplexes. The formation mechanism, physicochemical and antibacterial properties of the nanocomplexes (ZNC0.3, ZNC15, ZNC40, and ZNC60) were assessed. The nanocomplexes were characterized by DLS, ζ-potential, atomic force microscopy, scanning electron microscopy, circular dichroism, fourier transform infrared and UV-Vis spectroscopy. The results showed that the lowest molecular weight chitosan (LMWC, 3 kDa) formed nanocomplexes with nisin and zein structurally differed from the higher molecular weights chitosan (HMWC, >3 kDa). LMWC was doped on the surface of the nanocomplexes. HMWC linked and formed a network to adsorb zein and nisin. The antibacterial activity against Staphylococcus aureus showed that the minimum inhibitory concentration of ZNC0.3, ZNC15, ZNC40, and ZNC60 was 7.0625, 14.125, 14.125, and 28.25 µg/mL. ZNC0.3 could be a suitable nisin delivery system for its high encapsulation efficiency (85.38%) and antibacterial properties.

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BACKGROUND: Bromide is a halide ion of the element bromine usually administered in the form of potassium salt as monotherapy or add-on treatment in epileptic dogs. It is excreted unchanged in the urine and undergoes tubular reabsorption in competition with chloride. Thus, dietary chloride content affects serum bromide concentrations. This is the first published clinical report of bromide toxicosis secondary to a dietary modification of chloride content in an epileptic dog treated with potassium bromide. CASE PRESENTATION: A 3-year-old 55-kg neutered male Tibetan Mastiff was evaluated because of a 1-month history of progressive signs including ataxia, lethargy and behaviour changes. The dog was successfully treated for idiopathic epilepsy since the age of 1-year-old with phenobarbital and potassium bromide. Two months prior to presentation, the owners decided to change the dog's diet without veterinary advice. Physical examination was unremarkable. A 12-kg weight gain was recorded since last follow-up (8 months). Neurological examination revealed severe symmetric 4-limbs ataxia with altered vigilance and intermittent episodes of hyperactivity and aggressive behaviour without significant abnormality of cranial nerves. Serum bromide concentration was high and increased by 103 % since last follow-up. Nutritional evaluation revealed a 53 % decrease of chloride content in the diet before and after dietary transition. Bromide toxicosis was suspected, due to bromide reduced clearance secondary to the decreased dietary chloride content. Potassium bromide treatment was lowered by 15 % without further dietary changes. Neurologic signs progressively improved over the next month, without any seizure. After two months, the serum bromide concentration lowered to the same level measured before dietary modification. After four months, neurological examination was unremarkable. CONCLUSIONS: Dietary chloride content can directly influence serum bromide concentrations, therefore affecting seizure control or contributing to unexpected adverse effects. In the present case, a reduction in chloride intake markedly increased serum bromide concentrations causing bromism. Dietary changes should be avoided in dogs treated with potassium bromide to maintain stable serum bromide levels.

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Colloidal all-inorganic perovskites nanocrystals (NCs) have emerged as a promising material for display and lighting due to their excellent optical properties. However, blue emissive NCs usually suffer from low photoluminescence quantum yields (PLQYs) and poor stability, rendering them the bottleneck for full-color all-perovskite optoelectronic applications. Herein, a facile approach is reported to enhance the emission efficiency and stability of blue emissive perovskite nano-structures via surface passivation with potassium bromide. By adding potassium oleate and excess PbBr2 to the perovskite precursor solutions, potassium bromide-passivated (KBr-passivated) blue-emitting (≈450 nm) CsPbBr3 nanoplatelets (NPLs) is successfully synthesized with a respectably high PLQY of 87%. In sharp contrast to most reported perovskite NPLs, no shifting in emission wavelength is observed in these passivated NPLs even after prolonged exposures to intense irradiations and elevated temperature, clearly revealing their excellent photo- and thermal-stabilities. The enhancements are attributed to the formation of K-Br bonding on the surface which suppresses ion migration and formation of Br-vacancies, thus improving both the PL emission and stability of CsPbBr3 NPLs. Furthermore, all-perovskite white light-emitting diodes (WLEDs) are successfully constructed, suggesting that the proposed KBr-passivated strategy can promote the development of the perovskite family for a wider range of optoelectronic applications.

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Herein, the synthesis of a novel polymeric conjugate N,O-CMCS-Dopamine (DA) based on an amide linkage is reported. The performances of this conjugate were compared with those of an analogous N,O-CMCS-DA ester conjugate previously studied (Cassano et al., 2020) to gain insight into their potential utility for Parkinson's disease treatment. The new amide conjugate was synthesized by standard carbodiimide coupling procedure and characterized by FT-IR, 1H NMR spectroscopies and thermal analysis (Differential Scanning Calorimetry). In vitro mucoadhesive studies in simulated nasal fluid (SNF) evidenced high adhesive effect of both ester and amide conjugates. Results demonstrated that the amide conjugate exerted an important role to prevent DA spontaneous autoxidation both under stressed conditions and physiological mimicking ones. MTT test indicated cytocompatibility of the amide conjugate with Olfactory Ensheating Cells (OECs), which were shown by cytofluorimetry to internalize efficiently the conjugate. Overall, among the two conjugates herein studied, the N,O-CMCS-DA amide conjugate seems a promising candidate for improving the delivery of DA by nose-to-brain administration.

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Seizure threshold-2 (SZT2) gene variants have been associated with a decrease in seizure threshold resulting in variable phenotypic expressions ranging from mild-moderate intellectual disabilities without seizures, to an early-onset epileptic encephalopathy with severe cognitive impairment. In addition, hypotonia and distinctive facial dysmorphism, including a high forehead and to a lesser extent ptosis and down-slanting palpebral fissures, were present in the majority. We herein report a novel SZT2 variant in one of two siblings both diagnosed with epilepsy of infancy with migrating focal seizures (EIMFS). This report is the fourth to document a possible familial case in EIMFS, a condition that was not previously associated with SZT2 variant. This report expands the phenotypic expression of SZT2, corroborates the importance of genetic counseling in some cases of EIMFS, and highlights the efficacy of potassium bromide in controlling the seizures associated with this condition.

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Current work focuses on the synthesis and characterization of novel pH-sensitive biocompatible gelatin/carboxymethyl cellulose based hydrogels by free radical polymerization technique cross-linked with glutaraldehyde. Effect of pH, polymer ratio and variable crosslinking concentrations on dynamic swelling, equilibrium swelling, porosity, sol-gel analysis and in vitro release pattern was investigated. Hydrogel structure was confirmed by FTIR, XRD, and DSC. Moreover scanning electron microscopy confirmed the porous structure of gel network. Various structure property relationships like average molecular weight between crosslinks (Mc), solvent interaction parameters, volume fraction of polymer (V2,s) and diffusion coefficient (D) that affect the release behaviour were determined. Results showed that maximum swelling and highest release of drug occurred at pH 1.2. Porosity and gel fraction increased by increasing polymer load. The invivo absorption and pharmacokinetics evaluation in rabbit's models revealed the controlled nature of hydrogels. MTT assay confirmed the biocompatible nature of blank hydrogels against Vero cell lines and cytotoxic potential against HeLa cell lines. The preliminary safety evaluation and oral tolerability revealed that the hydrogel solution is safe up to 4000 mg/kg body weight without causing any hematological or histopathological changes in rabbits.

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Milk proteins and polyphenols are increasingly being studied as functional ingredients due to the epidemiologically-proved health benefits. In this study, composite ß-lactoglobulin (ß-lg) or ß-lactoglobulin nanoparticles (ß-lgNPs)-3,5-di-O-caffeoylquinic acid (3,5diCQA) with superior physicochemical and antioxidant activity (AA) were produced using ß-lg and 3,5-di-O-caffeoylquinic acid. The main interactions between ß-lg or ß-lgNPs with 3,5diCQA were hydrogen bonding and hydrophobic effects. The 3,5diCQA caused a decrease in α-helix and ß-sheet structure with a corresponding increase in unordered structure. Compared to ß-lg alone, composite ß-lg or ß-lgNPs-3,5diCQA slightly decreased the particle size but increased their negative surface potentials especially for ß-lg or ß-lgNPs at a molar ratio of 5:1. The addition of 3,5diCQA appreciably improved the AA in a dose-dependent manner. These results shed light on the structural, physicochemical, and AA of composite ß-lg or ß-lgNPs-3,5diCQA non-covalent complexes, important for application as functional ingredients in food solutions as well as in the pharmaceutical industry.

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Preparation and characterization of novel encapsulation system based on calcium alginate hydrogels filled with cumin essential oil has been investigated. Firstly, the effect of sodium alginate concentration, CaCl2 level, hardening time, encapsulation and emulsion fabrication methods was studied on loading capacity of the hydrogels using a Resolution-V fractional factorial design (2 V5-1 FFD), followed by response surface methodology (RSM). At the optimum point, the in-vitro release of phenolic compounds in simulated gastric and intestinal mediums were 96.02 ± 0.96% and 10.65 ± 1.23% after 180 min, respectively. The Scanning electron microscopy (SEM) images demonstrated a relatively smooth surface with small pore size. Based on SEM images and Fourier-transform infrared spectrums, the cumin essential oil was encapsulated successfully in calcium alginate beads. Thus, calcium alginate hydrogel could be introduced as a promising carrier for encapsulating biochemical active compounds with favorable features.

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We investigated the effect of temporary rearing in brackish water on the taste quality in meat of crab cooked. The main salinity-responsive factors included 5'-nucleotides and free amino acids (FAAs) in crab meat that were identified using tri-step infrared spectroscopy. Compared to the fresh water group, the contents of 5'-adenosine monophosphate and 5'-inosine monophosphate in the brackish water group significantly increased in the 2nd week and decreased in the 6th week, respectively. The contribution ratio of umami FAAs increased from 8.1 to 13.5% in the 4th week in the brackish water group, showing maximum value of equivalent umami concentration. Moreover, Ca2+ and Cl- contents significantly increased in the 4th and 6th weeks, respectively (P < 0.05). Infrared spectroscopy was an effective method to identify the taste components. With respect to the taste quality, four weeks were determined as the best period for temporary rearing of the crab in brackish water.

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Vincent van Gogh (1853-1890) is one of the most famous artists in the world. During his 10-year career as an artist, he created more than 850 paintings. These works of art are now displayed in museums around the globe. It is therefore even more surprising that van Gogh sold just one painting during his lifetime. Van Gogh is also well-known for his mental illness. In 1888, at the age of 35, he famously sliced off his left ear. This was followed by multiple mental collapses in early 1889, leading to his admission to a mental hospital. Despite living in the asylum, van Gogh continued to paint and created some of his most beautiful works of art during the year at Saint-Rémy. Tragically, he committed suicide in 1890 at the age of 37. Over the 130 years since his death, there has been much speculation about the underlying illness of Vincent van Gogh. Many of his contemporary physicians felt that he had a form of epilepsy as the cause of his sudden "attacks". By the last quarter of the 19th century, science and medicine were moving rapidly forward, and there were many medical conditions that had effective treatments. One example is the use of digoxin for the treatment of heart failure, and another is the discovery of potassium bromide for seizures. This paper provides an overview of van Gogh's mental illness, the treatments that were offered by his contemporaneous physicians, and the role that these factors may have influenced his paintings.

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Due to the poor solubility and permeability of rifaximin (RFX), it is not effective against intracellular pathogens although it shows strong activity against most bacteria. To develop an effective mucoadhesive drug delivery system with a targeted release in bacterial infection site, RFX-loaded chitosan (CS)/carboxymethyl-chitosan (CMCS) nanogel was designed and systematically evaluated. FTIR, DSC, and XRD demonstrated that the nanogel was formed by interactions between the positively charged NH3+ on CS and CMCS, and the negatively charged COO on CMCS. RFX was encapsulated into the optimized nanogel in amorphous form. The nanogel was a uniform spherical shape with a mean diameter of 171.07 nm. It had excellent sustained release, strong mucin binding ability, and pH-responsive properties of quicker swelling and release at acidic pH. It showed low hemolytic ratio and high antioxidant activity. The present investigation indicated that the CS-nanogel could be potentially used as a promising bacterial responsiveness drug delivery system.

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A previously undescribed polysaccharide, GMP70-1, was isolated from the rinds of Garcinia mangostana Lin. Physicochemical characterization analysis showed that GMP70-1 (absolute molecular weight: 2.01 × 104 g/mol) is a multi-branched acidic heteropolysaccharide with a compact coil chain conformation in sodium chloride solution. The repeated unit of GMP70-1 was mainly composed of (1 → 5)-linked α-L-Araf, (1 → 3, 5)-linked α-L-Araf, (1 → 2, 4)-linked α-L-Rhap, (1 → 4)-linked ß-D-Galp, terminating with t-α-L-Araf, t-α-D-GalpA, and t-ß-D-Galp. To explore the medicinal potential responsible for the bioactivity of G. mangostana, an immunomodulatory assay was performed. The in vitro cell test showed that GMP70-1 possessed a prominent immunoregulatory activity by enhancing the phagocytic uptake of neutral red and promoting the secretion of nitric oxide (NO), reactive oxygen species (ROS), tumor necrosis factor-α (TNF-α), interleukin (IL)-6, and IL-1ß of macrophages. Furthermore, an in vivo zebrafish evaluation revealed that the production of ROS and NO was significantly increased after treated with GMP70-1.

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New-onset refractory status epilepticus (NORSE) is a rare neurological emergency condition with poor prognosis. A 30-year-old male suddenly had tonic-clonic convulsions seven days after a preceding fever and diarrhea. MRI showed a reversible splenial lesion, and he developed refractory multifocal and generalized seizures in spite of anticonvulsant medication. He was diagnosed with NORSE and received a combination treatment with immunotherapy and targeted temperature management (TTM), which effectively decreased his seizures. This case suggests that even for patients with reversible splenial lesions, NORSE should be considered, and that treatment with immunotherapy and TTM may be effective.