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The objective of this study was to evaluate, during the drying of spent coffee grounds, the application of pretreatments with ethanol and the application of ultrasound assisting the refractive window (RW) drying, and to compare with convective drying by hot air (AC). The effect on the kinetics parameters of the Fick and Page models were evaluated, as well as on the content of total phenolics and antioxidant capacity. For AC drying, samples of spent ground coffee were prepared in the form of fixed rectangular beds 0.7 cm high, which were placed on polyester sheets and pretreated up to 10 times sprayed with ethanol, then dried by AC at 80 °C and 0.8 m/s. For RW drying, the samples prepared in the same way as for AC were used. For this case, the application of ethanol as pretreatment and the use of ultrasound during process were tested. As results, regardless of the conditions applied, drying by RW was up to 50 % faster, evidenced in the highest values of effective diffusivity (from the Fick model) and the kinetic parameter (from the Page model). Regarding the treatments applied and their effect on the drying kinetics, any treatment had a significant effect on AC drying. On the contrary, the strategies applied in RW drying had significant effects, both the application of pretreatment with ethanol as well as the application of ultrasound assisting the process accelerated the drying kinetics. However, the treatment with ethanol and RW drying was the one that best preserved the phenolic compounds and the antioxidant capacity in the samples. Therefore, these strategies could be a good option to improve RW drying by accelerating the process and preserving the bioactive compounds in the spent coffee grounds for subsequent utilization., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Introduction: Open spina bifida (OSB) is the most common congenital anomaly of the central nervous system. It is associated with severe neurodevelopmental delay, motor impairment, hydrocephalus, and bowel and bladder dysfunction. In selected cases, intrauterine spina bifida repair has been shown to improve neonatal outcomes. Rarely, the spine can have a double defect compromising two different segments and there is a lack of evidence on the feasibility and benefits of intrauterine repair in these cases., Case Presentation: We present a case with both cervicothoracic and lumbosacral myelomeningocele, Arnold-Chiari malformation type II and bilateral ventriculomegaly, that was treated successfully at 25 weeks with open micro-neurosurgery. Double myelomeningocele was successfully treated through a single 2-cm micro-hysterotomy, by performing external versions to sequentially expose and repair both defects. Weekly postoperative follow-up showed no progression of ventriculomegaly or complications attributable to the procedure. Preterm rupture of membranes prompted a conventional cesarean delivery at 32 weeks of gestation. Neurodevelopmental outcome at 20 months was within normal ranges, having achieved ambulation without orthopedic support and with no need for ventriculoperitoneal shunting., Conclusion: This report demonstrates for the first time the feasibility of double OSB repair through a single 2-cm micro-hysterotomy, suggesting that selected isolated cases of double myelomeningocele could be candidates for fetal intervention. Further prospective studies should be carried out to assess the potential benefit of double OSB intrauterine open repair., (© 2024 The Author(s). Published by S. Karger AG, Basel.)

StarD7 is a member of the START protein family required for phosphatidylcholine delivery to the mitochondria, thus key to maintain mitochondrial structure. Its deficiency has been associated with an impairment of cellular processes, such as proliferation and migration, and it has also been reported that it is needed in myogenic differentiation. Here, we show that StarD7 deficiency in C2C12 muscle cells results in the accumulation of abnormal mitochondria, a reduced number of mitochondria per cell area and increased glycolysis. In addition, StarD7-deficient cells undergo an increase in mitochondria-ER contact sites, reduced connexin 43 expression, and disturbances in lipid handling, evidenced by lipid droplet accumulation and decreased levels in phosphatidylserine synthase 1 and 2 expression. Interestingly, StarD7-deficient cells showed alterations in mitophagy markers. We observed accumulation of LC3B-II and BNIP3 proteins in mitochondria-enriched fractions and accumulation of autophagolysosomal and lysosomal vesicles in StarD7-deficient cells. Furthermore, live-cell imaging experiments of StarD7 knockdown cells expressing mitochondria-targeted mKeima indicated an enhanced mitochondria delivery into lysosomes. Importantly, StarD7 reconstitution in StarD7-deficient cells restores LC3B-II expression in mitochondria-enriched fractions at similar levels to those observed in control cells. Collectively, these findings suggest that StarD7-deficient C2C12 myoblasts are associated with altered cristae structure, disturbances in neutral lipid accumulation, glucose metabolism, and increased mitophagy flux. The alterations mentioned above allow for the maintenance of mitochondrial function., (© 2023 Federation of European Biochemical Societies.)

This study aimed to evaluate the effects of ultrasound (US) on the drying acceleration of potentially probiotic guava, including its impact on drying kinetics, probiotic (Lacticaseibacillus rhamnosus GG) viability, and functional quality of the product during drying. To perform US pre-treatments, one group of samples were first pre-treated by US (38 W/L, 25 kHz) for 15 and 30 min and then immersed in the probiotic solution for 15 or 30 min, and another group of samples were submerged in the probiotic solution simultaneously applying US (US-assisted) for 15 and 30 min. After pre-treatments, the samples were convectively dried at 60 °C. Based on the results, all US pre-treatments improved the drying rate (up to 59%) and reduced the drying time (up to 31%) to reach 25% moisture compared to non-sonicated samples. The reduction in drying time (from ∼6 h to ∼4 h for US pre-treated samples) was crucial for maintaining the probiotic viability in the dehydrated guavas. These samples showed counts of 6.15 to 7.00 CFU∙g -1 after 4 h, while the control samples reached counts of 4.17 to 4.45 CFU∙g -1 after 6 h. US pre-treatment did not affect the color parameters of the samples before drying (p > 0.05). The functional compounds were reduced during drying (p < 0.05), however, all US pre-treated samples had lower reductions in vitamin C content (up to 20%), phenolic compounds (up to 41%) and antioxidant capacity (up to 47%) compared to control samples (up to 52%, 81% and 61%, respectively). Therefore, US pre-treatment (highlighting the US-assisted probiotic incorporation for 30 min) reduced the drying time for guava slices and minimized the thermal impact on probiotic viability and functional compounds, being a strategy to produce potentially probiotic dehydrated guava., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Mitochondrial network architecture plays a critical role in cellular physiology. Indeed, alterations in the shape of mitochondria upon exposure to cellular stress can cause the dysfunction of these organelles. In this scenario, mitochondrial dynamics proteins and the phospholipid composition of the mitochondrial membrane are key for fine-tuning the modulation of mitochondrial architecture. In addition, several factors including post-translational modifications such as the phosphorylation, acetylation, SUMOylation, and o-GlcNAcylation of mitochondrial dynamics proteins contribute to shaping the plasticity of this architecture. In this regard, several studies have evidenced that, upon metabolic stress, mitochondrial dynamics proteins are post-translationally modified, leading to the alteration of mitochondrial architecture. Interestingly, several proteins that sustain the mitochondrial lipid composition also modulate mitochondrial morphology and organelle communication. In this context, pharmacological studies have revealed that the modulation of mitochondrial shape and function emerges as a potential therapeutic strategy for metabolic diseases. Here, we review the factors that modulate mitochondrial architecture.

The prenatal approach from a preventive perspective is necessary to reduce perinatal complications. A perinatal care model with a holistic and horizontal approach is required. Mexico is currently considered an emerging market economy with inequality and an economic gap that impacts the accessibility and distribution of healthcare services. Guanajuato is one of the 32 states of Mexico and represents 1.6% of the country's surface. Strategies during the prenatal approach allow prediction, diagnosis, and anticipation of the principal causes of morbidity and mortality. Combining data from maternal characteristics and history with findings of biophysical and biochemical tests at 11 to 13 weeks of gestation can define the patient-specific risk for a large spectrum of complications that include miscarriage and fetal death, preterm delivery, preeclampsia, congenital disorders, and fetal growth abnormalities. We aim to describe the care model designed and implemented in the State Center for Timely Prenatal Screening of the Maternal and Child Hospital of Leon, Guanajuato, Mexico. Previous research showed there is a lack of information for low and middle-income countries regarding how to integrate prenatal screening strategies in the absence of resources to perform cell-free fetal DNA or biochemical serum markers in countries with emergent economies. This care model is carried out through horizontal processes where the screening is provided by trained and certified general practitioners who identify the population at risk in a timely manner for specialized care, and could help guide other Mexican states, and other countries with emergent economies with limited financial, professional, and infrastructural resources to improve prenatal care with a sense of equity, equality, and social inclusion as well as the timely evaluation of specialized perinatal care of high-risk patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that may be construed as a potential conflict of interest., (Copyright © 2023 Bermudez Rojas, Medina Jimenez, Manzanares Cuadros, Diaz Martínez, Padilla Raygoza and Lara Lona.)

Introduction: Acquired brain injury (ABI) is defined as a neurological injury, acutely occurred, at some point in life causing impairment or loss of functional capacity. In 2019, a specific document was created by the Ombudsman pointing out the relevance of attention to this entity in the pediatric age., Patients and Method: The process of creation and the casuistry of care of one of the first comprehensive care units for subacute ACD in pediatric age within the public health system is presented., Results: Different clinical guidelines have been prepared on the admission and care process within the unit, both for patients and their relatives. Twenty-four patients ≤18 years old, admitted to the subacute phase ACD unit from November 2019 to July 2021, 12 coming from the Community of Madrid, were attended. The median age was 6.97 years. Traumatic mechanism was the most frequent, with iatrogenic causes predominating, followed by precipitation and vehicle-related accidents. On admission to the unit, 8 maintained a minimally conscious/vegetative state. The collaboration of up to 14 different specialists was required due to the complexity of the patients. The overall evolution was favorable in 23 cases, with sequelae in all of them., Conclusion: The creation of units specialized in pediatric ACD care with specific action protocols and coordinated trans- and multidisciplinary work is of vital importance., (Copyright © 2022 Sociedad Española de Rehabilitación y Medicina Física. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: A proportion of monochorionic diamniotic (MCDA) twin pregnancies complicated by twin-to-twin transfusion syndrome (TTTS) can present after 26 weeks of gestation. The aim of this study was to compare perinatal outcomes of late TTTS treated by fetoscopic laser coagulation versus traditional management with amniodrainage and/or emergency preterm cesarean delivery (CD)., Methods: Retrospective cohort from January 2012 to January 2023 of consecutive MCDA twin pregnancies complicated by TTTS after 26 weeks and evaluated in our referring centers. We analyzed perinatal outcomes of cases treated with fetoscopic laser surgery at our national referral fetal surgery center in Queretaro, Mexico, and compared them with those managed with traditional management (amniodrainage and/or emergency preterm CD). The primary outcome was survival at discharge and the secondary outcome was gestational age (GA) at birth., Results: Among the study population, 46 TTTS cases were treated by fetoscopy at 27+6 (26+0-31+0) weeks+days and were compared with a group of 39 cases who underwent emergency preterm CD. In comparison to the group who underwent traditional management, the group treated by laser fetoscopy showed a significantly higher GA at birth (32+3 vs. 29+1 weeks+days, p &lt; 0.001), lower frequency of preterm delivery below 37 weeks (91.3% vs. 100%, p = 0.06), 34 weeks (63.0% vs. 100%, p &lt; 0.001), 32 weeks (50% vs. 74.4%, p = 0.02), or 30 weeks (28.3% vs. 53.8%, p = 0.01), and significantly higher perinatal survival (89.1% vs. 71.8%, p &lt; 0.05 of at least one twin; and 65.2% vs. 38.5%, p = 0.01 of both twins, respectively)., Conclusion: MCDA twins complicated with TTTS can be treated with fetoscopic laser surgery between 26 and 31 weeks of gestation, which is a feasible and safe option, and such cases are associated with a higher GA at birth and better perinatal survival than those managed with amniodrainage and/or emergency preterm CD., (© 2023 S. Karger AG, Basel.)

StarD7 belongs to START protein family involved in lipid traffic, metabolism, and signaling events. Its precursor, StarD7.I which is important for mitochondrial homeostasis, is processed to the StarD7.II isoform that lacks the mitochondrial targeting sequence and is mainly released to the cytosol. StarD7 knockdown interferes with cell migration by an unknown mechanism. Here, we demonstrate that StarD7 silencing decreased connexin 43 (Cx43), integrin β1, and p-ERK1/2 expression in the non-tumoral migratory HTR-8/SVneo cells. StarD7-deficient cells exhibited Golgi disruption and reduced competence to reorient the microtubule-organizing center. The migratory capacity of StarD7-silenced cells was reestablished when Cx43 level was resettled, while p-ERK1/2 expression remained low. Importantly, ectopic expression of the StarD7.II isoform not only restored cell migration but also ERK1/2, Cx43, and integrin β1 expression. Thus, StarD7 is implicated in cell migration through an ERK1/2/Cx43 dependent mechanism but independent of the StarD7.I function in the mitochondria., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2022 Cruz Del Puerto et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Background: COVID-19 outbreak has been associated with a wide variety of psychiatric manifestations such as panic, anxiety, and depression. We aim to assess the impact of the COVID - 19 pandemic on the levels of stress and depression of pregnant women in Mexico., Methods: A cross-sectional web survey was carried out in pregnant women in 10 states of the Mexican Republic during the COVID-19 pandemic among public and private hospitals. The perception of stress was assessed using the Perceived Stress Scale, while depressive symptoms were evaluated using the Edinburgh Postnatal Depression Scale., Results: A total of 549 surveys were applied, of which 96.1% ( n  = 503) were included in the data analysis. The mean participant's age was 28.1 years old. The mean perceived stress scale score was 24. 33.2% ( n  = 167) of participants had a score equal to 27 points or more and were considered highly stressed. The mean depression score was 9. A total of 17.5% ( n  = 88) participants had more than 14 points on the Edinburgh's depression scale, and were considered depressed. Stress levels were higher at later gestational ages ( p  = .008)., Conclusions: COVID-19 pandemic has caused mental health issues in pregnant women reflected by high perceived stress levels and depression.

Introduction: Placental extravillous trophoblasts play a crucial role in the establishment of a healthy pregnancy. Reactive oxygen species (ROS) may contribute to their differentiation and function as mediators in signaling processes or might cause oxidative stress resulting in trophoblast dysfunction. The krüppel-like transcription factor 6 (KLF6) regulates many genes involved in essential cell processes where ROS are also involved. However, whether KLF6 regulates ROS levels has not been previously investigated., Materials and Methods: KLF6 was silenced by siRNAs in HTR8-SV/neo cells, an extravillous trophoblast model. Total and mitochondrial ROS levels, as well as mitochondrial membrane potential and apoptosis were analyzed by flow cytometry. The expression of genes and proteins of interest were analyzed by qRT-PCR and Western blot, respectively. Cell response to oxidative stress, proliferation, viability, morphology, and migration were evaluated., Results: KLF6 downregulation led to an increase in ROS and NOX4 mRNA levels, accompanied by reduced cell proliferation and increased p21 protein expression. Catalase activity, 2-Cys peroxiredoxin protein levels, Nrf2 cytoplasmic localization and hemoxygenase 1 expression, as well as mitochondrial membrane potential and cell apoptosis were not altered suggesting that ROS increase is not associated with cellular damage. Instead, KLF6 silencing induced cytoskeleton modifications and increased cell migration in a ROS-dependent manner., Discussion: Present data reveal a novel role of KLF6 on ROS balance and signaling demonstrating that KLF6 downregulation induces an increase in ROS levels that contribute to extravillous trophoblast cell migration., Competing Interests: Declaration of competing interest None., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Trophoblast cell differentiation is of paramount importance for successful pregnancy. Krüppel-like factor 6 (KLF6), a transcription factor with diverse roles in cell physiology and tumor biology, is required for trophoblast differentiation through the syncytial pathway. Herein, we demonstrate that extravillous trophoblast (EVT) cell migration and mesenchymal phenotype are increased upon KLF6 downregulation or the expression of a deletion mutant lacking its transcriptional regulatory domain (KΔac). Raman spectroscopy revealed molecular modifications compatible with increased differentiation in cells stably expressing the KΔac mutant. Moreover, abnormally invasive placenta showed lower KLF6 immunostaining compared with the normal placenta. Thus, impaired KLF6 expression or function stimulates EVT migration and differentiation in vitro and may contribute to the physiopathology of the abnormally invasive placenta., (© 2022 Federation of European Biochemical Societies.)

Ultrasound is an emerging technology, which has been highly explored in the food area to improve processes and products. When ultrasound is applied to a product with solid or fluid characteristics, the passage of acoustic waves and acoustic cavitation generates different mechanisms responsible for modifications in the original matrix of the sample. These effects of ultrasound can also be used to take advantage of by-products, for example by extracting compounds of interest, including natural pigments. Natural pigments or colorants are being highly demanded by different industries not only for color purposes but also due to their healthy properties, the greater demands in regulations and new consumer preferences. This review presents an updated critical analysis of the application of ultrasound-assisted extraction (UAE) to obtain natural pigments from food processing by-products. Initially, the ultrasound effects and mechanisms that improve the extraction of natural pigments in a fluid medium, as well as the factors that influence the extraction and the energy consumption of UAE are analyzed and described. Subsequently, the UAE application to obtain pigments belonging to the groups of carotenoids, chlorophyll, anthocyanins and betalains is evaluated. These sections detail the processing conditions, positive and negative effects, as well as possible applications of the extracted pigments. This review presents relevant information that may be useful to expand and explore new applications of ultrasound technology as well as promote the revaluation of by-products to obtain pigments that can be used in food, pharmaceutical or cosmetic industries., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Linares and Rojas.)

The TuberOus SClerosis registry to increase disease Awareness (TOSCA) Post-Authorization Safety Study (PASS) was a non-interventional, multicenter, safety substudy that assessed the long-term safety of everolimus in patients with tuberous sclerosis complex (TSC) receiving everolimus for its licensed indications in the European Union (EU). This substudy also aimed to address TSC-associated neuropsychiatric disorders (TAND), sexual development, and male infertility. Eligible patients were enrolled from 39 sites across 11 countries in the EU. Outcomes of interest included the incidence of adverse events (AEs), serious adverse events (SAEs), treatment-related AEs (TRAEs), AEs leading to everolimus discontinuation, AEs of special interest (AESIs), the observed relationship between everolimus blood levels and incidence of AESIs, TAND, and reproductive clinical features. Herein, we present the final analysis results from this substudy (data cutoff date: 22 January 2020). At data cutoff, 179 patients were enrolled (female, 59.2%; age ≥18 years, 65.9%), of which the majority completed the study (76%). Overall, 121 patients (67.6%) had AEs regardless of causality. The most frequent TRAEs (≥5%) were stomatitis (7.8%), aphthous ulcer (6.7%), and hypercholesterolemia (6.1%). The most common treatment-related SAEs (>1%) were pneumonia (3.4%), influenza, pyelonephritis, aphthous ulcer, stomatitis, dyslipidemia, and hypercholesterolemia (1.1% each). Ten patients (5.6%) reported AEs leading to everolimus discontinuation. The common psychiatric disorders ( N = 179) were autism spectrum disorder (21.8%), anxiety disorder (12.8%), "other" psychiatric disorders (8.9%), attention-deficit hyperactivity disorder, and depressive disorder (7.8% each). Of 179 patients, 88 (49.2%) had ≥1 behavioral problem. Of these ( n = 88), the most common (>20%) were sleep difficulties (47.7%), anxiety (43.2%), mood swings (37.5%), depression mood (35.2%), impulsivity (30.7%), severe aggression (23.9%), and overactivity (22.7%). Of 179 patients, four (2.2%) reported abnormal puberty onset, and three (1.7%) reported other reproductive disorders. Of 106 females, 23 (21.7%) reported menstrual cycle disorders and 10 (9.4%) reported amenorrhea. Available data did not show delays in sexual maturation or an association between sexual development and infertility. The results demonstrate that everolimus has a manageable long-term safety profile in the TSC treatment setting. No new safety signals emerged. This substudy also contributed to the mapping of TAND and reproductive clinical features in patients with TSC., Competing Interests: Authors RM and YH are employees at Novartis and report stock ownership at Novartis. Author AR is an employee at Novartis. MF reports consulting fees for being part of the scientific advisory board and TOSCA working committee; received speaker honoraria, support for travel, and payment for expert testimony from Novartis; and reports leadership or fiduciary role in the following: ILAE task force epilepsy surgery, ERN EpiCARE, ÖGKN, and ÖGfE. AM received honoraria and support for travel from Novartis. BW reports honoraria for a lecture from Takeda. JK received honoraria, support for travel, and research grant from Novartis. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Ruiz-Falcó Rojas, Feucht, Macaya, Wilken, Hahn, Maamari, Hirschberg, Ridolfi and Kingswood.)

Introduction: Villous cytotrophoblast (vCTB) cells fuse to generate and maintain the syncytiotrophoblast layer required for placental development and function. Krüppel-like factor 6 (KLF6) is a ubiquitous transcription factor with an N-terminal acidic transactivation domain and a C-terminal zinc finger DNA-binding domain. KLF6 is highly expressed in placenta, and it is required for proper placental development. We have demonstrated that KLF6 is necessary for cell fusion in human primary vCTBs, and in the BeWo cell line., Materials and Methods: Full length KLF6 or a mutant lacking its N-terminal domain were expressed in BeWo cells or in primary vCTB cells isolated from human term placentas. Cell fusion, gene and protein expression, and cell proliferation were analyzed. Moreover, Raman spectroscopy and atomic force microscopy (AFM) were used to identify biochemical, topography, and elasticity cellular modifications., Results: The increase in KLF6, but not the expression of its deleted mutant, is sufficient to trigger cell fusion and to raise the expression of β-hCG, syncytin-1, the chaperone protein 78 regulated by glucose (GRP78), the ATP Binding Cassette Subfamily G Member 2 (ABCG2), and Galectin-1 (Gal-1), all molecules involved in vCTB differentiation. Raman and AFM analysis revealed that KLF6 reduces NADH level and increases cell Young's modulus. KLF6-induced differentiation correlates with p21 upregulation and decreased cell proliferation. Remarkable, p21 silencing reduces cell fusion triggered by KLF6 and the KLF6 mutant impairs syncytialization and decreases syncytin-1 and β-hCG expression., Discussion: KLF6 induces syncytialization through a mechanism that involves its regulatory transcriptional domain in a p21-dependent manner., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Mitochondria are dynamic organelles crucial for cell function and survival implicated in oxidative energy production whose central functions are tightly controlled by lipids. StarD7 is a lipid transport protein involved in the phosphatidylcholine (PC) delivery to mitochondria. Previous studies have shown that StarD7 knockdown induces alterations in mitochondria and endoplasmic reticulum (ER) with a reduction in PC content, however whether StarD7 modulates mitochondrial dynamics remains unexplored. Here, we generated HTR-8/SVneo stable cells expressing the precursor StarD7.I and the mature processed StarD7.II isoforms. We demonstrated that StarD7.I overexpression altered mitochondrial morphology increasing its fragmentation, whereas no changes were observed in StarD7.II-overexpressing cells compared to the control (Ct) stable cells. StarD7.I (D7.I) stable cells were able to transport higher fluorescent PC analog to mitochondria than Ct cells, yield mitochondrial fusions, maintained the membrane potential, and produced lower levels of reactive oxygen species (ROS). Additionally, the expression of Dynamin Related Protein 1 (Drp1) and Mitofusin (Mfn2) proteins were increased, whereas the amount of Mitofusin 1 (Mfn1) decreased. Moreover, transfections with plasmids encoding Drp1-K38A, Drp1-S637D or Drp1-S637A mutants indicated that mitochondrial fragmentation in D7.I cells occurs in a fission-dependent manner via Drp1. In contrast, StarD7 silencing decreased Mfn1 and Mfn2 fusion proteins without modification of Drp1 protein level. These cells increased ROS levels and presented donut-shape mitochondria, indicative of metabolic stress. Altogether our findings provide novel evidence indicating that alterations in StarD7.I expression produce significant changes in mitochondrial morphology and dynamics., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Introduction: The KCNB1 gene encodes a voltage-dependent potassium channel that regulates transmembrane currents in pyramidal neurons. Heterozygous variants have recently been associated with early-onset epileptic encephalopathies and intellectual disability, but their clinical characterisation has not yet been fully defined., Aim: To describe the clinical spectrum associated with variants of KCNB1 in paediatric patients., Patients and Methods: Retrospective study of four patients from three families with KCNB1 encephalopathy, including an analysis of the clinical and electroencephalographic features of epilepsy, associated neurological manifestations and neurodevelopmental pattern., Results: In two of them, the mutation in KCNB1 was de novo; the other two, who were sisters, inherited the variant from a parent with germline mosaicism. All had mild-to-moderate intellectual disability, two patients had autistic spectrum disorder and two had attention deficit hyperactivity disorder. Only case 2 displayed alterations in the MRI brain scan: progressive cortical atrophy. Three of them developed epilepsy (cases 1-3). Case 1: onset at 9.5 months with West syndrome that was well controlled with vigabatrine and zonisamide. Case 2: onset at 13 months with West syndrome, evolutionary development of polymorphic seizures (atonic, hypermotor, dysautonomic and tonic) that were refractory to 10 antiepileptic drugs and corticosteroids. Accompanied by a movement disorder characterised by ataxia, dyskinesias and tremor. Case 3: onset at 14.5 years with atonic seizures, multifocal EEG pattern and adequate control with levetiracetam., Conclusions: KCNB1 encephalopathy has a heterogeneous natural history, mainly with respect to epilepsy, ranging from patients with refractory epilepsy to patients without any epileptic seizures. All had neurodevelopmental disorders, such as intellectual disability or autism spectrum disorder, independent of epilepsy.

This work studied three emerging approaches to improve the convective drying (50 °C, 0.8 m/s) of celery. Celery slices of 2 mm thick were pretreated for 5 min using ultrasound (32 W/L, 40 kHz), vacuum (75 kPa vacuum pressure) and ethanol (99.8% v/v, as drying accelerator) applied individually or in combination. To evaluate individual effects of ultrasound and vacuum, the treatments were also performed with distilled water or air medium, respectively. Moreover, the cavitational level was characterized in each condition. Drying kinetics was evaluated tending into account the drying time required by each treatment and the Page's model parameters. In addition, microstructural effects and shrinkage were evaluated. As results, ethanol combined with ultrasound significantly improved drying kinetics reducing drying time by around 38%. However, vacuum pretreatment did not affect drying kinetics even in combination with ethanol and/or ultrasound. Microstructural evaluation did not evidence cell disruption, suggesting changes in intercellular spaces, pores and/or cell wall permeability. The use of ethanol and vacuum showed a greater effect on shrinkage after pretreatment and after drying, respectively. In conclusion, at the studied conditions, the drying acceleration by vacuum and ultrasound is lower compared to the effect produced using ethanol., (Copyright © 2021 The Authors. Published by Elsevier B.V. All rights reserved.)

Introduction: Tuberous sclerosis complex (TSC) displays great phenotypic variability. Increasingly early diagnosis, including prenatal identification, entails the need for the paediatrician and neuropaediatrician to establish early suspicion and identification of factors that may influence prognosis and treatment., Aim: To determine the clinical criteria for early diagnosis, initial complementary tests, actions and treatments to prevent different comorbidities, so as to improve the prognosis of these patients., Patients and Methods: Descriptive, retrospective study of = 18-year-olds with a definitive diagnosis of TSC in a tertiary hospital from 1998 to 2019. We collected variables referring to epidemiological data, multisystem involvement, complementary tests and genetics., Results: Ninety-four patients were analysed. The main diagnostic reasons were epilepsy and rhabdomyomas. The frequency of occurrence of clinical criteria was determined, and neuropathological findings were the main findings, followed by cutaneous stigmata, rhabdomyomas and renal lesions. Statistical relationships were found between clinical, radiological and genetic aspects, the influence of preventive activities on the occurrence of epilepsy and the relevance of everolimus use were tested., Conclusions: Rhabdomyomas and skin stigmata in patients and parents are major diagnostic signs in infants. Tubers and subependymal nodules are statistically associated with the development of epilepsy. Early epileptic spasms, refractory to treatment in the first months, increase the risk of cognitive deficits and autism spectrum disorder. Epileptic abnormalities need to be closely monitored in the first year of life. Everolimus is an alternative treatment for several comorbidities, but its early use (< 3 years) requires further study.

Objective: The purpose of this article was to describe our experience with the prenatal diagnosis of CHD in patients referred to our Fetal Cardiology Unit., Methods: Prospective cohort study of consecutive fetuses referred for advanced fetal echocardiography to our Fetal Cardiology Unit during a 3-year period (September 2015-September 2018)., Results: Totally 809 fetuses were evaluated, with 1263 fetal advanced echocardiographies performed. Suspected cardiac abnormality was the most common indication for referral (62.2%). Only 7.3% of patients had known morbidities or risk factors for CHD. Mean gestational age at first examination was 25.6 ± 6.4 weeks. A total of 528 (65.3%) fetuses were found to have a cardiac defect: 40.7% had isolated CHD while 24.6% had associated anomalies. The most common defects found were ventricular septal defects (20.3%), followed by conotruncal defects (9.7%), hypoplastic left heart syndrome (9.3%), fetal arrhythmias (8.9%), and venous anomalies (8.7%). 31.6% presented abnormal genetic studies, the most frequent being Down syndrome (23/212, 10.8%), followed by DiGeorge syndrome (11/212, 5.2%)., Conclusions: Prenatal screening and diagnosis of CHD in Mexico are feasible, with suspected cardiac abnormality being the main reason for referral to a specialized Fetal Cardiology Unit. Efforts must be made to make screening available to the general population in the first and second trimesters of pregnancy by fetal medicine or trained specialists, in order to identify fetal CHD and offer advanced echocardiography, genetic studies, timely fetal cardiac intervention in selected cases, and delivery in tertiary centers, to improve overall survival.

Background: An interesting approach to improve dried foods nutritional properties, functionality, and sensorial attributes, is by taking advantage of pre-treatments for incorporating components into the food matrix. Based on this, this work studied the incorporation of black carrot anthocyanins in apple tissue by using ethanol (concentrations 0-300 mL L -1 ) as a pre-treatment to ultrasound-assisted convective drying. Samples were pre-treated in acidified ethanol solutions, with and without anthocyanins, and then dried (50 °C, 1 m s -1 ) by convective and ultrasound-assisted convective (21.77 kHz, 20.5 kW m -3 ) drying. Both the drying process improvement and the obtained product properties were studied., Results: The anthocyanins did not influence the drying kinetics. In contrast, time reduction was > 50% by using both ethanol pre-treatments and ultrasound. Ethanol pre-treatments decreased the external resistance to mass transfer, while ultrasound decreased both internal and external resistances. The impregnation increased the anthocyanins (above 947%), which were retained after drying. Colour modifications after pre-treatments and after drying (L*, b*, h° decrease, and a* increase), and antioxidant capacity retention were observed in samples with anthocyanin addition., Conclusion: The results point that ethanol pre-treatments and ultrasound application can accelerate drying, and through the natural colouring incorporation during pre-treatments, the nutritional properties of dried samples were better retained. © 2020 Society of Chemical Industry., (© 2020 Society of Chemical Industry.)

Ultrasound was combined with ethanol to improve different aspects of carrot convective drying, evaluating both processing and product quality. The ultrasound in water treatment resulted in cellular swelling and small impact on texture. Differently, the ultrasound in ethanol and ethanol treatments modified both carrot microstructure (cell wall modifications of parenchymatic tissue) and macrostructure (shrinkage and resistance to perforation). Pre-treatments with ultrasound in ethanol and ethanol improved the drying kinetics, reducing the processing time (~50%) and the energy consumption (42-62%). These pre-treatments also enhanced rehydration, whose initial rate and water retention were higher than the control. In addition, the carotenoid content was preserved after drying, for all the treatments. Any impact on shrinkage was observed. A mechanistic discussion, based on structural modification (microstructure and macrostructure) and physical properties of water and ethanol, was provided. As conclusion, this work not only described positive aspects of combining the technologies of ultrasound and ethanol as pre-treatments to convective drying, but also proposed mechanisms to explain the phenomena., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Purpose: Rasmussen's encephalitis (RE) is a chronic neurological disorder characterized by inflammation of the cerebral cortex, mainly unilateral, that leads to drug-resistant epilepsy and progressive neurological impairment. Central Precocious Puberty (CPP) is uncommon, albeit increased in frequency in patients with neurological conditions and the physiopathological bases of these associations remains unclear in most cases. Epilepsy has been proposed to play a role, as well as the accumulation of substances produced as a result of metabolism or tissue degeneration in some neurodegenerative diseases. However, CPP has not been previously described in patients with RE., Methods: From a series of patients affected by RE followed-up at a referral center, an in-depth review of the characteristics of those who developed CCP was carried out., Results: Three cases were identified, representing a relative frequency of 21.4 % for CPP. They were girls, of Caucasian ethnicity, without family history of CPP or any image-identified abnormalities in the hypothalamic area. In two cases CPP manifested immediately before the onset of the epilepsy (prior to the diagnosis of RE) and in the other, after epilepsy onset but coinciding with a worsening of the seizures. A GnRH test with pubertal response confirmed CPP in the three cases., Conclusion: The high proportion of CPP in patients affected by RE suggested a plausible relationship between these two entities. Various factors involved, including neuroinflammation, are hypothesized in the present study. However, further studies are needed to elucidate the pathophysiological bases, which could provide insight in the understanding of both entities., (Copyright © 2020. Published by Elsevier Ltd.)

Introduction: Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome with typical clinical features including seizures, chronic hemiplegia, hemianopsia and intellectual impairment. Progressive clinical decline may be attributable, at least in part, to progressive venous ischemia. Transcranial Doppler (TCD) ultrasonography could be useful to monitor the degree of hemodynamic involvement and its progression., Purpose: To determine whether there is an association between the degree of asymmetry in TCD and intensity of clinical and radiological involvement and whether there is a correlation between clinical changes and changes in serial TCD., Methods: In fourteen SWS pediatric patients and two "possible cases" (infants younger than two years old without previously known brain involvement, but with other typical signs of SWS) mean flow velocity in the middle cerebral arteries (MCA) was measured by TCD in both hemispheres. The percent difference between hemispheres (asymmetry) was calculated. Clinical and radiological severity was scored using scales. The correlation between TCD asymmetry and SWS clinical and radiological scores was analyzed at baseline, as well as the correlation between the changes in the different variables (TCD asymmetry, clinical and radiological cores) during evolution and in relation to the changes due to therapy., Results: The percentage of MCA velocity asymmetry was positively correlated with the clinical severity score (p = 0.04), and with seizure frequency (p = 0.014). Throughout evolution, therapeutic and clinical changes were associated with noticeable changes in transcranial doppler asymmetry in some cases., Conclusions: TCD may provide a noninvasive method to assess the severity of blood flow abnormalities at baseline and a method to monitor children for progressive changes over time., Competing Interests: Declaration of competing interest None., (Copyright © 2020 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

Background & Aims: Gut microbiota are affected by diet and ethnicity, which impacts cognition and hospitalizations in cirrhosis., Aim: Study interactions of diet with microbiota and impact on hospitalizations and cognition in American and Mexican cohorts., Methods: Controls and age-balanced patients with compensated/decompensated cirrhosis were included and followed for 90-day hospitalizations. A subset underwent minimal hepatic encephalopathy (MHE) testing. Parameters such as dietary, salivary and faecal microbiota (diversity, taxa analysis, cirrhosis dysbiosis ratio CDR:high = good) between/within countries were analysed. Regression analyses for hospitalizations and MHE were performed., Results: In all, 275 age-balanced subjects (133 US [40 Control, 50 Compensated, 43 Decompensated] and 142 Mexican [41 Control, 49 Compensated, 52 Decompensated]) were enrolled. MELD/cirrhosis severity was comparable. Diet showed lower protein and animal fat intake in all decompensated patients, but this was worse in Mexico. Diversity was lower in stool and saliva in decompensated patients, and worse in Mexican cohorts. Prevotellaceae were lower in decompensated cirrhosis, particularly those with lower animal fat/protein consumption across countries. Hospitalizations were higher in Mexico vs the USA (26% vs 14%, P = .04). On regression, Prevotellaceae, Ruminococcaceae and Lachnospiraceae lowered hospitalization risk independent of MELD and ascites. MHE testing was performed in 120 (60/country and 20/subgroup) subjects and MHE rate was similar. MELD and decompensation increased while CDR and Prevotellaceae decreased the risk of MHE., Conclusions: Changes in diet and microbiota, especially related to animal fat and protein intake and Prevotellaceae, are associated with MHE and hospitalizations in Mexican patients with cirrhosis compared to an American cohort. Nutritional counselling to increase protein intake in cirrhosis could help prevent these hospitalizations., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

There is growing evidence for inflammation as a cause and/or consequence of seizures in epilepsy as certain inflammatory biomarkers are elevated. Interleukin (IL)-6, with pro-inflammatory and epileptogenic effects, can perpetuate seizures. Clinical and experimental data support its involvement in acute refractory situations, with some cases responding to treatment with tocilizumab, a humanized monoclonal antibody against the IL-6 receptor. We describe 2 pediatric cases of refractory epilepsy with an abrupt debut responding to tocilizumab. Advances in the knowledge of inflammatory biomarkers involved in epilepsy and the targeted treatment could have important benefits, especially in cases that are refractory to usual treatments., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Primary trochlear headache is a little-known cause of periorbital headache described in adults. It can involve very disabling pain. In addition, it can be associated with other types of headaches, making them even more difficult to identify. To diagnose this pathology, it is necessary that the examination of the trochlea be incorporated into the usual clinical practice of the patient with headache, which will allow the establishment of an adequate treatment. The case is presented of an adolescent patient with a diagnosis of migraine, who was admitted with a disabling headache secondary to a primary trochlear headache., (Publicado por Elsevier España, S.L.U.)

The present work proposes using the ultrasound technology to incorporate microencapsulated nutrients during pre-treatments for drying of food products. Both hydrophilic and lipophilic nutrients were evaluated: incorporation of microcapsules of iron (obtained by spray drying using maltodextrin as wall material) and carotenoids (obtained by hot emulsification and solidification using hydrogenated palm oil as wall material). The ultrasound pre-treatment was applied in water and ethanol, where the microcapsules were dispersed, and food samples were immersed. Pumpkin and apple were selected as suitable food material to perform the iron and carotenoid incorporation, respectively. Ultrasound allowed more homogeneous iron incorporation in pumpkin. The iron content increased more than 1000% in pre-treated samples compared to control. In the same manner, carotenoid content increased in about 430% when ultrasound was applied. After drying, the carotenoid content decreased by 65% in control samples. However, better carotenoid retention was obtained after drying in ultrasound processed samples. The results show that pre-treatment with ultrasound can be used to incorporate nutrients into the food matrix, increasing not only the incorporated quantity but also promoting their preservation. Nevertheless, future studies must be performed to determine the nutrient bioavailability and bioaccessibility., (Copyright © 2019 Elsevier B.V. All rights reserved.)

This work aimed to demonstrate the internal and external structural changes in potato cylinders caused by different times of ultrasound pretreatment. In addition, the structural changes were associated with the viscoelasticity and mass transfer. For which, potato cylinders were immersed in distilled water and pretreated with and without ultrasound (ultrasonic bath of 91 W/L and 25 kHz) up to 120 min. Then, the microstructure was evaluated by stereoscopic observation as a direct method, and by viscoelasticity and mass transfer evaluation (pigment diffusion and drying) as indirect methods. Both external and internal structure of the material were evaluated. As results, it was demonstrated the formation of microchannels inside the potato tissue as well as the surface erosion caused by ultrasound, especially after 60 min of pretreatment. Further, it was observed that the product viscoelasticity is affected by the ultrasound pretreatment reducing the elasticity. In addition, the mass transfer on the cylinders was improved by ultrasound pretreatment. The pigment transfer was enhanced, demonstrating the acoustic erosion at the sample border. Furthermore, the drying process was accelerated by ultrasound, demonstrating the reduction of the internal resistance to water transfer. Finally, it was observed that high structural changes on the potato cylinders can bring some disadvantages such as changes on the color and severe shrinkage. This work concluded that the structural changes caused by ultrasound can be evidenced directly and indirectly. Furthermore, different structural changes took place, on both inside and outside of the vegetable sample. However, despite ultrasound improves further process, especially those that involve mass transfer, the parameters time and ultrasonic power must be optimized to obtain desirable results without reducing the product quality., (Copyright © 2018 Elsevier B.V. All rights reserved.)

The present work evaluated the effect of high-power ultrasonication on the structure and properties of guava juice. The microstructure, concentration of lycopene, in vitro accessibility of lycopene and physical properties (pulp sedimentation, turbidity and colour) were evaluated. The results of this study demonstrate that the ultrasonication disrupts the guava cells, releasing their content and altering the juice properties. Although this processing decreases the total amount of lycopene in guava juice, the release of lycopene from the cells increased its in vitro accessibility. Furthermore, the size reduction of the dispersed pulp particles improved the physical stability of the juice, avoiding pulp sedimentation without significant colour changes during storage. In conclusion, it is suggested that ultrasonication is an interesting alternative to improve the physical and nutritional properties of fruit juices., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

StarD7 is a lipid binding protein involved in the delivery of phosphatidylcholine to the mitochondria whose promoter is activated by Wnt/β-catenin signaling. Although the majority of glucose enters glycolysis, ~ 2-5% of it can be metabolized via the hexosamine biosynthetic pathway (HBP). Considering that HBP has been implicated in the regulation of β-catenin we explored if changes in glucose levels modulate StarD7 expression by the HBP in trophoblast cells. We found an increase in StarD7 as well as in β-catenin expression following high-glucose (25 mM) treatment in JEG-3 cells; these effects were abolished in the presence of HBP inhibitors. Moreover, since HBP is able to promote unfolded protein response (UPR) the protein levels of GRP78, Ire1α, calnexin, p-eIF2α and total eIF2α as well as XBP1 mRNA was measured. Our results indicate that a diminution in glucose concentration leads to a decrease in StarD7 expression and an increase in the UPR markers: GRP78 and Ire1α. Conversely, an increase in glucose is associated to high StarD7 levels and low GRP78 expression, phospho-eIF2α and XBP1 splicing, although Ire1α remains high when cells are restored to high glucose. Taken together these findings indicate that glucose modulates StarD7 and β-catenin expression through the HBP associated to UPR, suggesting the existence of a link between UPR and HBP in trophoblast cells. This is the first study reporting the effects of glucose on StarD7 in trophoblast cells. These data highlight the importance to explore the role of StarD7 in placenta disorders related to nutrient availability.

Introduction: Fifty million people are affected by epilepsy. Up to 30% are not controlled with the aid of antiepileptic drugs. The vagus nerve stimulator (VNS) is a therapeutic alternative that must be taken into account., Aims: To determine the effect of the VNS in a cohort of paediatric patients with refractory epilepsy., Patients and Methods: A retrospective study of children with a VNS implanted between 2008 and 2017 in a tertiary hospital. Epidemiological, aetiological, clinical and electrophysiological data, along with VNS parameters were analysed., Results: The study included 35 patients, with a mean age when the VNS was implanted of 12.84 years (range: 3.1-18.7 years) and a mean time between onset of epilepsy and implantation of 7.2 years (range: 1.3-17.7 years). The causation was structural in 62.9% of cases. The most frequent epileptic conditions were: Lennox-Gastaut syndrome and focal epilepsy, with a predominance of tonic seizures (57.1%). The video electroencephalogram showed multifocal anomalies (54%) and a pattern of epileptic encephalopathies (34.3%). Intellectual disability was associated in 94% of the cases. The mean of previous antiepileptic drugs was 9.6 ± 3 (range: 4-16). 43% responded to treatment (>= 50% reduction in number of seizures), with a mean reduction of 67.3%, which improved with higher ages of onset of epilepsy. Three patients were seizure-free (8.5%). The number of seizures decreased by 33% at six months and by 47.4% at 24 months. There was also a notable degree of cognitive (57%) and behavioural improvement (53%). In 28% of cases there were some side effects, but in general they were mild., Conclusions: The VNS is a valid option in refractory epilepsy, with improvements not only in terms of seizures but also regarding cognitive-behavioural aspects, this being very important for the paediatric population.

An agroindustrial by-product (cactus pear peel) and whole grains flour (brown rice and amaranth) were used to present a gluten-free snack proposal. The effect of 5% (F1), 7% (F2), and 10% (F3) substitution of brown-rice flour for yellow cactus pear peel powder ( Opuntia ficus-indica ) on the snack physical, sensorial, and nutritional properties was evaluated. In addition, 20% of amaranth flour (Amaranthus caudatus) was used for all formulations. As the percentage of substitution increased, the a⁎ value increased, while the L⁎ decreased. The control snacks presented higher hardness, while the snacks with 10% substitution presented a greater crispness. The sensorial properties (overall liking, colour, crispness, and oiliness) reported that the samples containing cactus pear peel powder were the most accepted. The fat content decreased as the substitution percentage increased. The F3 formulation presented the best physical and sensorial properties and when compared with other commercial snack brands, it presented low fat and an adequate protein and fibre content. Therefore, snacks based on brown rice, amaranth, and cactus pear by-product could be considered as a good option of gluten-free product, contributing to reducing the lack of gluten-free products on the markets.

Biological materials are characterized by complex structures and compositions, leading to viscoelastic behavior. Their viscoelastic characterization is important for the evaluation, design, and optimization of processes to ensure high quality products. Proposals of methodologies of analysis and modeling are critical steps in studying the rheological properties of these materials. In this context, a new model, the Guo-Campanella Model, was recently proposed to describe the stress-relaxation behavior of biological material. This work is an independent and impartial evaluation of this new model. It considers 10 different samples, comprising in natura and processed foods, from both plant and animal bases. For comparison, a Generalized Maxwell Model and the Peleg Model were also evaluated. The Guo-Campanella Model fitted the stress-relaxation data of evaluated products well, demonstrating its validity for describing the viscoelastic behavior of biological materials with different structures, sources, and processing. Finally, the Guo-Campanella Model parameters were evaluated and their interpretations and possible uses described. It was shown that the Guo-Campanella Model can be successfully used for future studies., Practical Applications: The stress-relaxation assay is a common technique for characterizing the viscoelastic properties of biological materials. The results obtained are generally evaluated using such compound models as the Generalized Maxwell model. Although this approach is interesting from a fundamental point of view, it results in many parameters to evaluate, thus increasing the complexity and limiting the interpretation. In this sense, the Guo-Campanella Model has only two parameters, which facilitates interpretation, especially for practical applications. This work validated this model, also contributing to its interpretation by discussing the meaning of its parameters. Consequently, this is potentially useful for future studies on food properties and process design., (© 2017 Wiley Periodicals, Inc.)

In this work the effects of pre-drying and frying time on colour, oil, texture and sensorial acceptability (overall liking) of potato chips were evaluated. Potato chips were pre-dried for 0, 10, 20 and 30 min at 60 °C and fried in soybean oil at 190 °C for 60, 70 and 80 s. The colour parameters (L*, a* and b*) increased or decreased depending on the pre-drying and frying time. Hardness increased as the pre-drying and frying time increased. On the other hand, the water initially removed by pre-drying decrease the gradient of mass transfer (water-oil). The oil content reduced to (about 21%) in pre-dried samples when compared to control sample. Finally, sensorial evaluation showed that samples without pre-drying and/or fried for very short or very long times had low acceptance levels. The pre-drying and frying times influenced the colour, texture, water and oil content, and resulted into fried potato chips with better acceptance scores.

Objectives: Shigella sonnei is a globally important diarrhoeal pathogen tracked through the surveillance network PulseNet Latin America and Caribbean (PNLA&C), which participates in PulseNet International. PNLA&C laboratories use common molecular techniques to track pathogens causing foodborne illness. We aimed to demonstrate the possibility and advantages of transitioning to whole genome sequencing (WGS) for surveillance within existing networks across a continent where S. sonnei is endemic., Methods: We applied WGS to representative archive isolates of S. sonnei (n = 323) from laboratories in nine PNLA&C countries to generate a regional phylogenomic reference for S. sonnei and put this in the global context. We used this reference to contextualise 16 S. sonnei from three Argentinian outbreaks, using locally generated sequence data. Assembled genome sequences were used to predict antimicrobial resistance (AMR) phenotypes and identify AMR determinants., Results: S. sonnei isolates clustered in five Latin American sublineages in the global phylogeny, with many (46%, 149 of 323) belonging to previously undescribed sublineages. Predicted multidrug resistance was common (77%, 249 of 323), and clinically relevant differences in AMR were found among sublineages. The regional overview showed that Argentinian outbreak isolates belonged to distinct sublineages and had different epidemiologic origins., Conclusions: Latin America contains novel genetic diversity of S. sonnei that is relevant on a global scale and commonly exhibits multidrug resistance. Retrospective passive surveillance with WGS has utility for informing treatment, identifying regionally epidemic sublineages and providing a framework for interpretation of prospective, locally sequenced outbreaks., (Crown Copyright © 2017. Published by Elsevier Ltd. All rights reserved.)

Introduction: Sturge-Weber syndrome is a congenital vascular disorder characterised by facial capillary malformation (port-wine stain) associated with venous and capillary malformations in the brain and eye. Neurological symptoms and alterations in other locations may also be observed., Objectives: This study describes the clinical and epidemiological characteristics and different treatments in a cohort of patients diagnosed with Sturge-Weber syndrome in a tertiary hospital., Material and Methods: This comparative, retrospective and cross-sectional study was conducted by reviewing the medical records of patients diagnosed with Sturge-Weber syndrome between 1998 and 2013., Results: The study included 13 patients (54% male, 46% female) diagnosed with Sturge-Weber syndrome. The mean age at diagnosis was 15 months. Leptomeningeal angiomatosis was present in 100% of cases: right hemisphere (46%), left hemisphere (38%), and bilateral (15%). Facial angioma was present in 61% of the cases: right (23%), left (38%) and bilateral (7%). Other skin disorders were found in 23% of the cases, including 2 with hemilateral involvement on the side where facial and leptomeningeal angiomatosis was present and one case of generalised cutis marmorata. Ocular disease was found in 77% of patients; the most common conditions were glaucoma (46%), strabismus (23%) and choroidal angioma (23%). Epilepsy was present in 100% of the cases, with partial seizures (simple or complex) being the most frequent (62%). Seizure control was highly variable; 31% of the patients had needed to try more than 3 drugs, 15% 3 drugs, and 31% 2 drugs, while 23% experienced good seizure control with monotherapy. One patient required surgery for epilepsy (left hemispherectomy) and has been seizure-free since then. The most frequent observations in electroencephalograms were spikes, polyspikes, and wave spikes in the lobes affected by leptomeningeal angiomatosis (46%). Other neurological symptoms were hemiparesis (39%), recurrent headaches (39%), stroke-like episodes (23%), psychomotor retardation (46%), and mental retardation (46%). Leptomeningeal calcifications could be seen in 85% of patient MRIs, as well as increased calcification in 70%; 54% of the patients had been treated with aspirin., Conclusions: There are multiple clinical manifestations of Sturge-Weber syndrome. Being familiar with all of them is vitally important for diagnosing and for monitoring and treating the condition correctly, which will improve the quality of life of these patients., (Copyright © 2016 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.)

Green coconut water has unique nutritional and sensorial qualities. Despite the different technologies already studied, its enzymatic stability is still challenging. This study evaluated the use of ultrasound technology (US) for inactivating/sensitizing coconut water peroxidase (POD). The effect of both US application alone and as a pre-treatment to thermal processing was evaluated. The enzyme activity during US processing was reduced 27% after 30min (286W/L, 20kHz), demonstrating its high resistance. The thermal inactivation was described by the Weibull model under non-isothermal conditions. The enzyme became sensitized to heat after US pre-treatment. Further, the use of US resulted in more uniform heat resistance. The results suggest that US is a good technology for sensitizing enzymes before thermal processing (even for an enzyme with high thermal resistance). Therefore, the use of this technology could decrease the undesirable effects of long times and/or the high temperatures of the conventional thermal processing., (Copyright © 2016 Elsevier B.V. All rights reserved.)