bluuebunny/crossref_metadata_2025_split huggingface dataset:
Consensus panel recommendations for the optimization of EPIDIOLEX® treatment for seizures associated with Lennox–Gastaut syndrome, Dravet syndrome, and tuberous sclerosis complex
Authors: Wechsler Robert T. , Burdette David E. , Gidal Barry E. , Hyslop Ann , McGoldrick Patricia E. , Thiele Elizabeth A. , Valeriano James 
Published: July 2024

AbstractFollowing the approval of Epidiolex® (cannabidiol; CBD) for the treatment of seizures associated with Lennox–Gastaut syndrome (LGS), Dravet syndrome (DS), and tuberous sclerosis complex (TSC), healthcare professionals (HCPs) have had substantial experience in treating patients with Epidiolex. However, confusion still remains among HCPs, caregivers, and patients regarding dosing, drug interactions, safety monitoring, and differentiation between Epidiolex and nonapproved CBD products. To establish consensus recommendations for Epidiolex treatment optimization in LGS, DS, and TSC, a panel of seven HCPs with expertise in epilepsy was convened. Panelists participated in a premeeting survey based on a literature review of Epidiolex for the treatment of LGS, DS, and TSC, and survey responses were compiled for discussion. A modified Delphi method was used to assess agreement among panelists regarding recommendation statements following two rounds of discussion. Panelists identified two broad themes – overcoming barriers to initiation and optimization of treatment for seizures associated with LGS, DS, and TSC – for consensus guidelines. Accurate identification of patients with these rare epilepsies is critical for optimization of Epidiolex treatment. Providers should differentiate Epidiolex from nonapproved CBD products and set expectations for the therapeutic effect and safety/tolerability of Epidiolex. Initial target dose and titration rate should be individualized by baseline variables, prior response to antiseizure medications, and therapeutic goals. Awareness of strategies to manage adverse events and concomitant medications, including drug–drug interactions, is critical. Tracking response to the maximum tolerated dose is an important measure of effectiveness. These consensus recommendations provide real‐world experience from neurology HCPs with experience in prescribing Epidiolex and can inform optimal use of Epidiolex for the treatment of seizures associated with LGS, DS, and TSC.Plain language summaryEpidiolex® (cannabidiol) is approved for treating seizures in Lennox–Gastaut syndrome, Dravet syndrome, and tuberous sclerosis complex. Although healthcare professionals have experience in treating patients with Epidiolex, there is a need for better understanding of dosing, drug interactions, and safety of this drug. Therefore, a group of epilepsy experts developed guidelines for best practices in Epidiolex treatment. Two main areas were identified: overcoming barriers to starting Epidiolex and considerations related to Epidiolex dosing. Within these areas, topics, including correct disease identification, managing adverse events, and determining individualized dose, were discussed. These guidelines provide real‐world experience to inform optimal Epidiolex use.
Energy needs within the rural community in Makueni County, Kenya
Authors: Kitetu Job , Thoruwa Thomas , Omosa Isaiah 
Published: July 2024

AbstractLiterature shows that only 56% of Kenyan households had access to electricity, with rural areas having the lowest access rate at percent. The high cost of extending the power grid to remote areas and power losses on distribution are significant challenges facing rural electrification. In addressing power accessibility problems, especially in rural areas, there is a need for tapping hydropower generation through the invention and implementation of in‐duct turbines to maximize the utilization of already existing pressurized water ducts that supply water in various parts of Kenya for hydropower generation. Makueni County is endowed with gravity‐fed water ducts with high potential for hydropower which can innovatively be produced by application of in‐duct turbines. This paper focuses on the assessment of energy needs and applications in rural areas. The research design was exploratory and experimental in nature. It was exploratory because, through an assessment, it sought to explore and identify the potential areas within the water supply lines for the production of hydropower to supply hydropower in Makueni County. It was experimental because the researcher developed (designed and fabricated) a hydro turbine for use in the production of hydropower from gravity water ducts of a diameter raging 100 mm. The research revealed that 62% (98) used solar power for lighting their homes, while 17% (28), 12% (20), and 8% (12) used lanterns, electricity, and kerosene lamps, respectively. Among the fuels assessed was firewood which was identified as the most used fuel at 89% (140). This was followed at a distance far by paraffin at 6% (9) of respondents. The households at 100% (158) identified electricity as a potential source of lighting for their household. The study recommends harnessing hydropower to enhance reach to 100% of the rural communities. The energy availability will provide opportunities for communities and institutions in rural areas to open their minds to business development and engage in income‐generating activities like the rearing of poultry and the development of light industries like the gridding of maize and other cereals.
Evaluation of the EUROSEM on soil erosion simulation for loess plateau, northern China
Authors: Su Chang , Wang Yu , Fu Xingtao , Wang Zhiyuan 
Published: July 2024

AbstractThe Loess Plateau region features fragmented landforms, sparse vegetation, and short but intense summer rainstorms that can easily cause severe soil erosion. To evaluate its applicability to a typical Loess Plateau in northern China, an indoor laboratory experimental study was conducted for events with different slope lengths (3, 4, and 5 m) and rainfall intensities (50, 60, 70, 80, 90, 100, 110, and 120 mm/h). EUROSEM (European Soil Erosion Model) was used to simulate the soil erosion process and predict both total runoff and sediment yield. The EUROSEM modeling results were compared with the observed experimental values and analysed to determine the performance of EUROSEM. The results and analysis showed that EUROSEM could effectively simulate the runoff processes. It produced better predictions for the total runoff yield than for the sediment yield, with Nash–Sutcliffe efficiencies of 0.9003 and 0.1820, respectively. The qualified rates of the RE (relative error) between simulated and observed values of the total runoff and sediment yield were 87.5% and 37.5%, respectively. Compared to the sediment yield under rainfall intensities of 50–80 mm/h, those under 90–110 mm/h had lower RE and higher NSE, which increased from −3.9 to 0.5. However, the modeling results deviated more from the experimental data at a rainfall intensity of 120 mm/h. According to this study, EUROSEM is a good fit for simulating the runoff and partial sediment yield processes of the Loess Plateau region's slopes. However, the accurate prediction of sediment yield still requires more research to achieve complete effectiveness using EUROSEM for typical and wide scenarios in China.
Molecular mechanism of acetylated distarch phosphate with pea protein isolate to improve the gel quality of myofibrillar protein gel
Authors: Jiang Wenting , Wu Jiulin , Yang Fujia , Chen Xu , Chen Meizhen , Huang Jianlian , Wu Jinhong , Wan Shuo , Cai Xixi , Wang Shaoyun 
Published: July 2024

AbstractIt is crucial to improve the gel properties of myofibrillar protein (MP) in the production of surimi products. This study investigated the effects of combining acetylated distarch phosphate (ADSP) with pea protein isolate (PPI) as exogenous additives on the physicochemical properties of Larimichthys crocea MP gel and elucidated the molecular mechanisms underlying the improvement of MP gel quality. The results showed that the mixture of 3% ADSP and 1% PPI increased the MP gel strength to 2.1 times and water holding capacity to 1.8 times. The rheological properties during thermal‐induced gelation were improved, and the surface roughness of gel microstructure was reduced. The protein conformation was stabilized by enhancing surface hydrophobicity and sulfhydryl content, and the gels showed trends of decreasing α‐helix and increasing random coils. Correlation and cluster analysis showed that physicochemical properties of MP gels were closely related to the changes of protein structure and the denaturation of active groups. The molecular interaction between ADSP, PPI, and MP and the mechanism of enhancing the properties and functions of MP gel were further clarified. These findings highlight the feasibility of ADSP–PPI as an effective strategy to improve the quality of fish MP gel.
Whey protein hydrolysate intervention ameliorates memory deficits in APP/PS1 mice: Unveiling gut microbe–short‐chain fatty acid–brain axis
Authors: Zhou Yongjie , Meng Hanxiu , Ding Ning , Hong Hui , Luo Yongkang , Wu Chao , Tan Yuqing 
Published: July 2024

AbstractThe intricate causes of Alzheimer's disease (AD) hinder effective, lasting treatment. Although the dietary modulation of the brain–gut axis was explored for AD therapy, the exact mechanism remains unclear. This study suggested that 140 days of the whey protein hydrolysate (WPH) intake could attenuate the AD pathologic symptoms in APP/PS1 transgenic mice via a bidirectional action of the gut microbe–SCFA (short‐chain fatty acid)–brain axis. Behavioral tests demonstrated that high‐dose WPH (WPH‐H, 100 mg/kg body weight [bw]) improved passive and recognition memory in mice. Furthermore, WPH‐H significantly reduced amyloid beta 1–42 (Aβ1–42) levels in serum (p < .05) and brain (p < .001) while enhancing serum superoxide dismutase (SOD) activity (p < .01). Brain acetylcholinesterase (p < .01) activity and pro‐inflammatory factors in serum were also reduced. Notably, WPH‐H remodeled gut microbiota composition by increasing Dubosiella and decreasing Bacteroides and norank_f__Ruminococcaceae while stimulating SCFA production. Proteomics indicated that WPH enhanced neurotoxic Aβ autophagy, synaptogenesis, neurotransmitter delivery, and antioxidative stress response via regulated protein expression. Correlation analysis revealed strong links between modified gut microbiota, elevated SCFA levels, and hippocampal protein up‐regulation (Atg4b, Nsfl1c, Tcf20, Nr2f1, and Trappc9) and down‐regulation (Krt1). Overall, the amelioration of memory deficits in APP/PS1 mice through WPH‐H consumption can be attributed to the interconnected interactions among gut microbes, SCFAs, and brain. Our study illuminated the intricate interplay between nutrition, gut health, and memory function, emphasizing WPH's potential in alleviating AD symptoms.
Incidence and patterns of lymph node metastases in head and neck rhabdomyosarcoma: One‐institution study
Authors: Wang Tian , Wang Jie , Li Qiang , Li Yi , Song Xinmao 
Published: July 2024

AbstractIntroductionHead and neck rhabdomyosarcoma (HNRMS) is an aggressive malignant soft tissue tumor that easily develops lymph node metastasis (LNM) and distant metastasis. No literature investigates the pattern of LNM in HNRMS.MethodsNinety‐five consecutive patients with HNRMS newly diagnosed at one institution between November 2011 and July 2023 were retrospectively reviewed. All the patients underwent head and neck contrast‐enhanced MRI and/or CT, PET‐CT if necessary. The associations between LNMs and clinical characteristics and histopathological parameters were discovered.Results44.2% of patients had evidence of LNM at diagnosis, and the most common LNM occurred in the ipsilateral retropharyngeal space. The primary tumor metastasizes to the retropharyngeal space, and then next to level II is the most common LN drainage basin. In multivariate analysis, only distant metastasis determines the prognosis, other than LN status.ConclusionsLNM has a high incidence in HNRMS and rarely causes contralateral metastasis for localized lesions or skip metastasis.
Recommendations for diagnosis, treatment, and prevention of iron deficiency and iron deficiency anemia
Authors: Iolascon Achille , Andolfo Immacolata , Russo Roberta , Sanchez Mayka , Busti Fabiana , Swinkels Dorine , Aguilar Martinez Patricia , Bou‐Fakhredin Rayan , Muckenthaler Martina U. , Unal Sule , Porto Graça , Ganz Tomas , Kattamis Antonis , De Franceschi Lucia , Cappellini Maria Domenica , Munro Malcolm G. , Taher Ali ,   from EHA‐SWG Red Cell and Iron
Published: July 2024

AbstractIron is an essential nutrient and a constituent of ferroproteins and enzymes crucial for human life. Generally, nonmenstruating individuals preserve iron very efficiently, losing less than 0.1% of their body iron content each day, an amount that is replaced through dietary iron absorption. Most of the iron is in the hemoglobin (Hb) of red blood cells (RBCs); thus, blood loss is the most common cause of acute iron depletion and anemia worldwide, and reduced hemoglobin synthesis and anemia are the most common consequences of low plasma iron concentrations. The term iron deficiency (ID) refers to the reduction of total body iron stores due to impaired nutrition, reduced absorption secondary to gastrointestinal conditions, increased blood loss, and increased needs as in pregnancy. Iron deficiency anemia (IDA) is defined as low Hb or hematocrit associated with microcytic and hypochromic erythrocytes and low RBC count due to iron deficiency. IDA most commonly affects women of reproductive age, the developing fetus, children, patients with chronic and inflammatory diseases, and the elderly. IDA is the most frequent hematological disorder in children, with an incidence in industrialized countries of 20.1% between 0 and 4 years of age and 5.9% between 5 and 14 years (39% and 48.1% in developing countries). The diagnosis, management, and treatment of patients with ID and IDA change depending on age and gender and during pregnancy. We herein summarize what is known about the diagnosis, treatment, and prevention of ID and IDA and formulate a specific set of recommendations on this topic.
Oncogenic and immunological targets for matched therapy of pediatric blood cancer patients: Dutch iTHER study experience
Authors: Boer Judith M. , Ilan Uri , Boeree Aurélie , Langenberg Karin P. S. , Koster Jan , Koudijs Marco J. , Hehir‐Kwa Jayne Y. , Nierkens Stefan , Rossi Corinne , Molenaar Jan J. , Goemans Bianca F. , den Boer Monique L. , Zwaan C. Michel 
Published: July 2024

AbstractOver the past 10 years, institutional and national molecular tumor boards have been implemented for relapsed or refractory pediatric cancer to prioritize targeted drugs for individualized treatment based on actionable oncogenic lesions, including the Dutch iTHER platform. Hematological malignancies form a minority in precision medicine studies. Here, we report on 56 iTHER leukemia/lymphoma patients for which we considered cell surface markers and oncogenic aberrations as actionable events, supplemented with ex vivo drug sensitivity for six patients. Prior to iTHER registration, 34% of the patients had received allogeneic hematopoietic cell transplantation (HCT) and 18% CAR‐T therapy. For 51 patients (91%), a sample with sufficient tumor percentage (≥20%) required for comprehensive diagnostic testing was obtained. Up to 10 oncogenic actionable events were prioritized in 49/51 patients, and immunotherapy targets were identified in all profiled patients. Targeted treatment(s) based on the iTHER advice was given to 24 of 51 patients (47%), including immunotherapy in 17 patients, a targeted drug matching an oncogenic aberration in 12 patients, and a drug based on ex vivo drug sensitivity in one patient, resulting in objective responses and a bridge to HCT in the majority of the patients. In conclusion, comprehensive profiling of relapsed/refractory hematological malignancies showed multiple oncogenic and immunotherapy targets for a precision medicine approach, which requires multidisciplinary expertise to prioritize the best treatment options for this rare, heavily pretreated pediatric population.
The reversed circular flow jet impingement (RCFJI) PV/T collector: Thermohydraulic and electrohydraulic analysis
Authors: Ishak Muhammad Amir Aziat Bin , Ibrahim Adnan 
Published: July 2024

AbstractSolar energy could be used to generate both electricity and heat with the aid of photovoltaic thermal (PV/T) systems. Although the systems have a variety of advantages, they nevertheless hold a significant constraint. The system suffers a susceptible constraint wherein the photovoltaic (PV) module experiences an increase in temperature due to exposure to solar irradiation. The integration of a cooling system is necessary to enhance its operational efficiency. A novel approach, known as the reversed circular flow jet impingement (RCFJI), was proposed as a means to improve the performance of a PV/T collector. The current work seeks to assess the thermohydraulic and electrohydraulic performance of the RCFJI PV/T collector. The experiment was conducted under an irradiance level of 500–900 W/m2. From the result obtained, the thermohydraulic efficiency reached its maximum value of 59.20% under 900 W/m2 at 0.14 kg/s. Conversely, the electrohydraulic efficiency attained the highest reading of 10.91% under 500 W/m2 at 0.13 kg/s. It was concluded that a higher flow rate reduces the friction coefficient while increasing the pressure drop. The thermohydraulic and electrohydraulic analyses emphasize the importance of assessing the friction coefficient and pressure drop to attain optimal performance. This study addresses the lack of research by presenting a new cooling approach that utilizes jet impingement. In addition, this study provides an understanding of the thermohydraulic and electrohydraulic performance of a RCFJI PV/T collector.
COVID‐19 vaccinated children, adolescents, and young adults with acute lymphoblastic leukemia show spike reactive antibodies and multifunctional T‐cells
Authors: Parker Rebecca S. , Le Justin , Villa Miguel , Luong Annie , Lin Tsen Yin , Lee Yesun , Doan Andrew , Aguayo‐Hiraldo Paibel , Pannaraj Pia S. , Yoon Seon‐Jae , Wallace William Dean , Armstrong April , O'Gorman Maurice R. , Bard Jennifer Dien , Parekh Chintan 
Published: July 2024

AbstractLittle is known about the efficacy of COVID‐19 vaccines during acute lymphoblastic leukemia therapy (ALL); data for COVID‐19 vaccine immune responses in pediatric leukemia remain sparse. We conducted a single center study of patients aged 5–25 years undergoing ALL chemotherapy who received COVID‐19 vaccination. Twenty‐one patients were enrolled; efficacy was evaluable in 20. Twenty were vaccinated while receiving chemotherapy. Twenty received the BNT162b2 mRNA vaccine. Spike reactive antibodies (S‐IgG) and/or T‐cells (SRT) were detected in 16 of 20 (80%) vaccinated patients; 13 (65%) and 9 (45%) were positive for S‐IgG and SRT, respectively. Six (30%) showed both spike reactive B and T‐cell responses. Eleven of the 13 with S‐IgG positivity were negative for anti‐Nucleocapsid IgG, an antibody profile consistent with a vaccine induced immune response. All 13S‐IgG+ patients showed neutralizing antibodies. SRT included CD4+ (7) and CD8+ (6) T‐cells; both CD4+ and CD8+ SRT were seen in 4. SRT were multifunctional (producing multiple cytokines) in most patients (8 of 9); 4 showed SRT with triple cytokine and B‐cell co‐stimulatory responses, indicating a multimodal adaptive immune response. Immune responses were seen among patients vaccinated in the settings of lymphopenia (6 of 12) intensive chemotherapy (3 of 4), and Peg allergy (6 of 8). Sequencing revealed public CD4+ and CD8+ TCR sequences reactive to epitopes across the spike protein. In conclusion, COVID‐19 vaccination induced B and/or T‐cell responses in a majority of children and young adults undergoing ALL chemotherapy.
A framework to examine the potential social influence of scientists and nongovernmental organizations in marine conservation
Authors: Li Minhui , Lee Tien M. , Zhang Xiong 
Published: July 2024

AbstractEffective marine conservation requires science‐based decisions and strong support from the public and local communities. Understanding the potential social influence of scientists and nongovernmental organizations (NGOs) in marine conservation is key, yet this aspect has been underexplored in conservation social science. In this study, we created a simple index of potential social influence (PSII) for scientists and NGOs, initiating an analytical framework to identify key variables correlated with the PSII and their underlying pathways. We illustrated this framework using China as a case study, where marine conservation advancement is urgently needed. Our results indicated that many of the variables we identified were correlated with the PSII, even though some are rarely mentioned in the related literature. Notably, both communication capability and annual funding positively impacted the potential social influence of scientists and NGOs, albeit through different pathways. This study provides valuable insights to enhance marine conservation efforts in China and addresses a critical research gap in assessing social influence on nature conservation.
Identification of novel variants in BRF1 gene from patient with developmental delay, hearing abnormality, and nervous system anomalies
Authors: Yin Hongwei , Yu Yonglin , Shen Yingying 
Published: July 2024

AbstractCerebellofaciodental syndrome characterized with dysmorphic features, intellectual disability, and brain anomalies. Now its clinical spectrum expanded more manifestations including bilateral sensorineural hearing impairment and inner ear malformation. Here, we report a 14‐month‐old boy with global developmental delay and hearing disorder. Whole exome sequencing (WES) revealed the compound heterozygous variants [NM_001519.4: c.652 T > G (p.W218G); c.915 + 1G > T] in the BRF1 gene which inherited from his parents, respectively. The MRI results showed hypoplastic cerebellar vermis, enlarged cisterna magna, and prominent fourth ventricle, the rehabilitation therapy failed to improve the symptoms for our patient. Our finding expands the genetic spectrum of BRF1 variants, which indicates patients with the developmental delay caused by BRF1 variants require other treatments instead of rehabilitation.