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The performance of broiler chickens is directly influenced by temperature changes and the occurrence of heat stress, whether it is cold or hot. The present study aims to assess the cooling and heating degree days in different stages of broiler chicken production in Khorasan Razavi province. To achieve this objective, daily average temperature data were collected from 13 synoptic stations during the statistical period of 1988 -2018. Cooling and heating degree days were calculated for each week of production using specific thresholds, and their spatial distribution was analyzed. Furthermore, the relationship between cooling and heating degree days and geographical features was evaluated. The findings indicate a decrease in cooling degree days and an increase in heating degree days as latitude increases. The northern and western regions of the province exhibited a greater need for heating throughout all stages of broiler chicken production, whereas the eastern and southern regions had higher cooling requirements at different production stages. Overall, the northeastern, southern, and western marginal areas of the province, including Khaf, Gonabad, Kashmar, Sarakhs, and Sabzevar, exhibited the lowest cooling and heating needs during the 6 -week period of broiler chicken production, making them suitable climates for this economic activity. [ABSTRACT FROM AUTHOR]

Spatiotemporal variations in drought events were examined through multiple drought indices in the Upper Indus Basin (UIB) during the 1980–2020 period using observed climate data of precipitation, temperature (T max., T min., and T mean), and potential evapotranspiration (PET) from 16 meteorological stations. Software like ClimPACT2 (to quality check data and generate SPI, SPEI, and CDD drought indices), DrinC (to generate AI, eRDI and PD drought indices), CMhyd (to bias correct NASA POWER gridded data), and ArcGIS (to map extreme drought years) were used in this study. It was revealed that precipitation decreased at 30 mm/decade and maximum and minimum temperatures increased at 0.132 °C/decade and 0.23 °C/decade, aridity increased by decreasing aridity index at 0.15/decade, and number of consecutive dry days increased at 2 days/decade. All drought indices reflected decreasing trends, indicating a warming and drying climatic regime. The eRDI drought index analysis revealed that droughts have occurred for 12.6 years in the last four decades, and mild droughts (15%) are more common, followed by moderate droughts (9.4%), severe droughts (4.7%), and extreme droughts (2.4%). The study provides comparisons of drought patterns under changing climate in three distinct climatic and physical regions of UIB, i.e., Jammu, Kashmir, and Ladakh, and reveals that the UIB is not free from droughts. [ABSTRACT FROM AUTHOR]

Objectives: This study aims to determine the current state of CDD diagnosis and epilepsy treatment in an upper‐middle‐income country. Methods: Forty‐seven families of the Brazilian CDD Association were invited to participate in an online survey to gather information about the diagnosis and treatment of epilepsy. Results: Forty‐three families (91.5%) of unrelated patients with confirmed genetic diagnosis of CDD participated. The median age was 7 years (ranging from 1.3–25 years) and the male: female ratio was 1:6. Early and severe epilepsy started during infancy in 74.4%. Seizures occurred daily in 61.9% and 83.7% had clusters of seizures. The mean age of diagnosis was 3.3 years (ranging from 37 days to 16 years), and younger patients had an earlier diagnosis (p < 0.001). Patients were seen by an average of 4.4 physicians (1–15) before the diagnosis. The most relevant obstacles to genetic testing were cost (55.8%) and late requests by physicians (27.9%). At the moment of the assessment, patients received a mean of 3.6 ASMs/day (ranging from 1 to 5). Thirty‐four (79.1%) caregivers reported side effects throughout life, including life‐threatening events in 16.3%. Significance: Based on our findings, a sense of urgency for genetic assessment implementation is evident since the delay in the diagnosis with unnecessary use of resources and excessive polytherapy with serious side effects cause a higher burden to the healthcare system, caregivers, and patients. Plain Language Summary: In this study, we assessed the diagnosis and treatment of patients with genetically confirmed DEE‐CDKL5 from the Brazilian Association of CDD with an online survey. Caregivers reported a long delay in the diagnosis associated with cost and late referral to genetic testing, considered the last resource for one‐third of the patients. Patients received a high number of ASM, mainly under polytherapy, with serious side effects. Although it is promising that younger patients received earlier diagnosis, public policies for genetic testing are needed to improve CDD patients' care. [ABSTRACT FROM AUTHOR]

Abstract Objectives This study aims to determine the current state of CDD diagnosis and epilepsy treatment in an upper‐middle‐income country. Methods Forty‐seven families of the Brazilian CDD Association were invited to participate in an online survey to gather information about the diagnosis and treatment of epilepsy. Results Forty‐three families (91.5%) of unrelated patients with confirmed genetic diagnosis of CDD participated. The median age was 7 years (ranging from 1.3–25 years) and the male: female ratio was 1:6. Early and severe epilepsy started during infancy in 74.4%. Seizures occurred daily in 61.9% and 83.7% had clusters of seizures. The mean age of diagnosis was 3.3 years (ranging from 37 days to 16 years), and younger patients had an earlier diagnosis (p

Pediatric genetic epilepsies, such as CDKL5 Deficiency Disorder (CDD), are severely debilitating, with early-onset seizures occurring more than ten times daily in extreme cases. Existing antiseizure drugs frequently prove ineffective, which significantly impacts child development and diminishes the quality of life for patients and caregivers. The relaxation of cannabis legislation has increased research into potential therapeutic properties of phytocannabinoids such as cannabidiol (CBD) and Δ9-tetrahydrocannabinol (THC). CBD's antiseizure properties have shown promise, particularly in treating drug-resistant genetic epilepsies associated with Lennox–Gastaut syndrome (LGS), Dravet syndrome (DS), and Tuberous Sclerosis Complex (TSC). However, specific research on CDD remains limited. Much of the current evidence relies on anecdotal reports of artisanal products lacking accurate data on cannabinoid composition. Utilizing model systems like patient-derived iPSC neurons and brain organoids allows precise dosing and comprehensive exploration of cannabinoids' pharmacodynamics. This review explores the potential of CBD, THC, and other trace cannabinoids in treating CDD and focusing on clinical trials and preclinical models to elucidate the cannabinoid's potential mechanisms of action in disrupted CDD pathways and strengthen the case for further research into their potential as anti-epileptic drugs for CDD. This review offers an updated perspective on cannabinoid's therapeutic potential for CDD. [ABSTRACT FROM AUTHOR]

Cyclin‐dependent kinase‐like 5 (CDKL5) deficiency disorder (CDD) is a neurodevelopmental disease caused by mutations in the X‐linked CDKL5 gene and characterized by early‐onset epilepsy, intellectual disability, and autistic features. To date, the etiological mechanisms underlying CDD are largely unknown and no effective therapies are available. The Cdkl5 knock‐out (KO) mouse has been broadly employed in preclinical studies on CDD; Cdkl5‐KO mice display neurobehavioral abnormalities recapitulating most CDD symptoms, including alterations in motor, sensory, cognitive, and social abilities. However, most available preclinical studies have been carried out on adult Cdkl5‐KO mice, so little is known about the phenotypic characteristics of this model earlier during development. Furthermore, major autistic‐relevant phenotypes, for example, social and communication deficits, have been poorly investigated and mostly in male mutants. Here, we assessed the autistic‐relevant behavioral phenotypes of Cdkl5‐KO mice during the first three post‐natal weeks and in adulthood. Males and females were tested, the latter including both heterozygous and homozygous mutants. Cdkl5 mutant pups showed qualitative and quantitative alterations in ultrasonic communication, detected first at 2 weeks of age and confirmed later in adulthood. Increased levels of anxiety‐like behaviors were observed in mutants at 3 weeks and in adulthood, when stereotypies, reduced social interaction and memory deficits were also observed. These behavioral effects of the mutation were evident in both sexes, being more marked and varied in homozygous than heterozygous females. These findings provide novel evidence for the autistic‐relevant behavioral profile of the Cdkl5 mouse model, thus supporting its use in future preclinical studies investigating CDD pathology and autism spectrum disorders. Lay Summary: Cyclin‐dependent kinase‐like 5 (CDKL5) deficiency disorder (CDD) is a rare neurodevelopmental disease characterized by several symptoms, including autistic features. Here we demonstrate the presence of a variety of autistic‐relevant behavioral alterations in the preclinical model of CDD, i.e., the Cdkl5 knock‐out (Cdkl5‐KO) mouse, taking into account sex and age differences. Our results support the validity of this genetic mouse line for modeling CDD and its use for future preclinical pharmacological and molecular studies on this disorder. [ABSTRACT FROM AUTHOR]

Nowadays, electricity is increasing rapidly. This increase is caused by several factors, one of which is meteorological factors. Meteorological parameters have various types, but this research uses three types in the form of temperature, humidity, and wind speed. The selection of these three types is due to the fact that they have a very close relationship with human life. In line with that, this research uses datasets obtained from the official websites of BMKG (Meteorology, Climatology and Geophysics Agency) and PLN (State Electricity Company). On this occasion, researchers used several methods, namely Cross-Industry Standard Process for Data Mining (CRISP-DM), Cooling Degree Days (CDD), and Support Vector Machine (SVM). The CRISP-DM method is useful for describing the data mining cycle so that the process can be more organized. The SVM algorithm is useful for predicting electricity consumption based on meteorological parameters in January to April 2024, while the CDD method is useful for knowing the correlation of meteorological parameters to electricity consumption in winter. In line with this, this research produces predictions of electricity consumption based on meteorological parameters in January 2024 to April 2024 with an average range of 20.9 Watts per day. In addition, trends and predictions during model evaluation obtained a precision value of 0.796, recall of 0.793, F1 score of 0.793, MAPE of 17.2%, RMSE of 0.41, MAE of 0.167 and accurate of 0.98. These values indicate that the performance of the accuracy model is very high.

CDKL5 deficiency disorder (CDD) is a genetically caused developmental epileptic encephalopathy that causes severe communication impairments. Communication of individuals with CDD is not well understood in the literature and currently available measures are not well validated in this population. Accurate and sensitive measurement of the communication of individuals with CDD is important for understanding this condition, clinical practice, and upcoming interventional trials. The aim of this descriptive qualitative study was to understand how individuals with CDD communicate, as observed by caregivers. Participants were identified through the International CDKL5 Disorder Database and invited to take part if their child had a pathogenic variant of the CDKL5 gene and they had previously completed the Communication and Symbolic Behavior Checklist (CSBS‐DP ITC). The sample comprised caregivers of 23 individuals with CDD, whose ages ranged from 2 to 30 years (median 13 years), 15 were female, and most did not use words. Semistructured interviews were conducted via videoconference and analyzed using a conventional content analysis. Three overarching categories were identified: mode, purpose and meaning, and reciprocal exchanges. These categories described the purposes and mechanism of how some individuals with CDD communicate, including underpinning influential factors. Novel categories included expressing a range of emotions, and reciprocal exchanges (two‐way interactions that varied in complexity). Caregivers observed many communication modes for multiple purposes. Understanding how individuals with CDD communicate improves understanding of the condition and will guide research to develop accurate measurement for clinical practice and upcoming medication trials. [ABSTRACT FROM AUTHOR]

The present study investigates the energy efficiency of different container house configurations across thirty European locations. By employing Heating Degree Days (HDDs) and Cooling Degree Days (CDDs), the research delves into climatic zone exploration, providing a simplified climatic classification for residential purposes and comparing it with the Köppen–Geiger model. The authors use specific hourly climatic data for each location, obtained through dynamic simulations with TRNSYS v.18 software. Initially, the CDDs are calculated by using different base temperatures (comfort temperatures that minimize energy demand) tailored to the specific conditions of each case. Then, the thermal loads of container houses are evaluated in different climatic scenarios, establishing a direct correlation between climatic conditions and the energy needs of these innovative and modular housing solutions. By comparing stacked and adjacent modular configurations in container housing, particularly in post-disaster scenarios, the study underscores the importance of adaptive design to optimize energy efficiency. The analysis conducted by the authors has allowed them to propose a climate characterization model based on HDDs, CDDs, and solar irradiance, obtaining an effective novel correlation with the Köppen–Geiger classification, especially in extreme climates. The present model emerges as a powerful tool for climate characterization in residential applications, offering a new perspective for urban planning and housing design. Furthermore, the results reveal a significant correlation between climate classification and the specific energy needs of container houses, emphasizing the direct influence of regional climatic characteristics on energy efficiency, particularly in small-sized dwellings such as container houses. [ABSTRACT FROM AUTHOR]

The change in heating and cooling needs of Greece in the near future due to the climate change is assessed in the present study. Global and regional climate models and two different representative concentration pathways (RCPs) are used to simulate the expected change in temperature. A widely used methodology of computation of heating degree days (HDDs) and cooling degree days (CDDs) is employed with a base temperature of 18 °C. In agreement with the expected temperature rise in the near future, an HDD decrease and CDD increase under both RCPs is also expected. The changes under RCP8.5 are stronger compared to those under RCP4.5. Differences related to topography are noted. The HDD decrease is stronger than CDD increase but the relative increase in CDDs is higher than the relative increase in HDDs. The highest absolute decreases in HDDs are expected for February and March while the highest absolute increases in CDDs are expected during the three summer months. [ABSTRACT FROM AUTHOR]

Context: Organic semiconductors (OSCs) have attracted a great deal of interest in recent days. There are various types of OSCs, among which small molecules have various inherent benefits. Further research is needed to advance this new kind of material because the field is still developing, and the current focus is on creating small molecules that exist naturally for OSCs. OSCs with nonlinear optical (NLO) characteristics offer a significant advantage over others. Thus, this study theoretically investigates naturally occurring anthraquinones such as chrysophanol and rhein as potential OSCs, as well as their NLO properties. The calculated properties include the ionization potential (IP), electron affinity (EA), and bandgap (Eg). The FMO energy levels together with the Eg, IP (8.17–8.53 eV), and EA (1.87–2.44 eV) suggest the semiconductor nature of the studied compounds. The calculated values of reorganization energy (λ) and transfer integrals (V) suggest the p-type character of both molecules. Rhein has the lowest λh (0.19 eV) and Eg (3.28 eV) and the highest Vh, predominantly because of its better p-type character. The polarizability increases due to the presence of an electron-withdrawing substituent, leading to better NLO performance for Rhein, which is supported by its lower LUMO and Eg values. Methods: The studied molecules were optimized with the DFT/B3LYP-GD3/6-31+G(d,p) method using Gaussian 16 software. The crystal structure was simulated with Materials Studio 7.0, and the V values were calculated with the ADF package. The CDD and DOS plots were obtained with the Multiwfn 3.8 program. [ABSTRACT FROM AUTHOR]

This study evaluates the effect of complete nationwide lockdown in 2020 on residential electricity demand across 13 Indian cities and the role of digitalisation using a public smart meter dataset. We undertake a data-driven approach to explore the energy impacts of work-from-home norms across five dwelling typologies. Our methodology includes climate correction, dimensionality reduction and machine learning-based clustering using Gaussian Mixture Models of daily load curves. Results show that during the lockdown, maximum daily peak demand increased by 150-200% as compared to 2018 and 2019 levels for one room-units (RM1), one bedroom-units (BR1) and two bedroom-units (BR2) which are typical for low- and middle-income families. While the upper-middle- and higher-income dwelling units (i.e., three (3BR) and more-than-three bedroom-units (M3BR)) saw night-time demand rise by almost 44% in 2020, as compared to 2018 and 2019 levels. Our results also showed that new peak demand emerged for the lockdown period for RM1, BR1 and BR2 dwelling typologies. We found that the lack of supporting socioeconomic and climatic data can restrict a comprehensive analysis of demand shocks using similar public datasets, which informed policy implications for India's digitalisation. We further emphasised improving the data quality and reliability for effective data-centric policymaking.

In this work, the physical mechanisms of three highly efficient circularly polarized luminescent materials are introduced. The UV–vis spectra are plotted; the transition properties of their electrons at the excited states are investigated using a combination of the transition density matrix (TDM) and the charge difference density (CDD); combining the distribution of electron clouds, the essence of charge transfer excitation in three structures is explained. The resonance Raman spectrum of the three structures at the S1 and S2 excited states are calculated. The M, M-4 and M, M-5 structures are found to produce novel chirality by electronic circular dichroism (ECD) spectrum, and the reasons for the chirality of the M, M-4 and M, M-5 structures are discussed by analyzing the density of transition electric/magnetic dipole moments (TEDM/TMDMs) in different orientations. Finally, the Raman optical activity (ROA) of M, M-4, and M, M-5 are calculated, and the spectra are plotted. This study will provide guidance for the application of carbon-based nanomaterials in organic electronic devices, solar cells, and optoelectronics. [ABSTRACT FROM AUTHOR]

近年來，我國在洗錢防制（Anti-Money Laundering, AML）的立法及執 行上逐漸邁入正軌並與先進國家逐步看齊，國內金融機構多數都已建置基本洗錢防制系統，並配合商業資料庫提供的名單進行認識你的客戶（Know Your Customer, KYC）與客戶盡職調查（Customer Due Diligence, CDD）中的 姓名檢核工作。然而，在實際作業中，金融機構因姓名檢核缺失被主管機關糾正或裁罰案例層出不窮，此皆肇因於姓名檢核作業的困難性以及工作的繁重，法遵洗防單位苦於沒有科技協助只能大量仰賴人力所致。 本文依據國內實際實施的困難並針對現況進行分析，並參照國外的實際 案例作法，以及符合國內情境方式引入新技術來提出建議，期望能為金融機構在採用AI 大數據等新技術執行洗錢防制負面新聞檢核時作為參考。 In recent years, our country has gradually stepped on the right track in the legislation and implementation of anti-money laundering (AML), and has gradually lined up with advanced countries. Most domestic financial institutions have established basic anti-money laundering systems, and perform the name screening operation of know your customers (KYC) and customer due diligence (CDD) process with the sanction and watchlists provided by the commercial database. However, in practice, financial institutions have been corrected or punished by the FSC (Financial Supervisory Commission R.O.C. (Taiwan)) due to the lack and missing of name screening. This is due to the difficulty and heavy workload of name screening operation. The legal compliance team suffers from the lack of technological assistance. It can only rely heavily on manpower.

Objective: We aimed to assess the treatment response of infantile‐onset epileptic spasms (ES) in CDKL5 deficiency disorder (CDD) vs other etiologies. Methods: We evaluated patients with ES from the CDKL5 Centers of Excellence and the National Infantile Spasms Consortium (NISC), with onset from 2 months to 2 years, treated with adrenocorticotropic hormone (ACTH), oral corticosteroids, vigabatrin, and/or the ketogenic diet. We excluded children with tuberous sclerosis complex, trisomy 21, or unknown etiology with normal development because of known differential treatment responses. We compared the two cohorts for time to treatment and ES remission at 14 days and 3 months. Results: We evaluated 59 individuals with CDD (79% female, median ES onset 6 months) and 232 individuals from the NISC database (46% female, median onset 7 months). In the CDD cohort, seizures prior to ES were common (88%), and hypsarrhythmia and its variants were present at ES onset in 34%. Initial treatment with ACTH, oral corticosteroids, or vigabatrin started within 1 month of ES onset in 27 of 59 (46%) of the CDD cohort and 182 of 232 (78%) of the NISC cohort (p <.0001). Fourteen‐day clinical remission of ES was lower for the CDD group (26%, 7/27) than for the NISC cohort (58%, 106/182, p =.0002). Sustained ES remission at 3 months occurred in 1 of 27 (4%) of CDD patients vs 96 of 182 (53%) of the NISC cohort (p <.0001). Comparable results were observed with longer lead time (≥1 month) or prior treatment. Ketogenic diet, used within 3 months of ES onset, resulted in ES remission at 1 month, sustained at 3 months, in at least 2 of 13 (15%) individuals with CDD. Significance: Compared to the broad group of infants with ES, children with ES in the setting of CDD often experience longer lead time to treatment and respond poorly to standard treatments. Development of alternative treatments for ES in CDD is needed. [ABSTRACT FROM AUTHOR]

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Copyright of Retos: Nuevas Perspectivas de Educación Física, Deporte y Recreación is the property of Federacion Espanola de Asociaciones de Docentes de Educacion Fisica and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Purpose: To assess community-directed treatment coverage with Ivermectin (CDTI) in Onchocerciasis-endemic communities in Birnin Kudu local government area (LGA) Jigawa state. Subjects and Methods: This was a community-based multistaged cross-sectional survey based on probability proportional to size. The study involved the administration of a questionnaire on 2021 respondents from 207 households. Also, 30 Community Leaders and Community Directed Distributors (CDDs) were purposively selected for interview from the communities visited. Results: Overall, 2021 respondents from the 2031 sampled population took part in the study giving a response rate of 99.6%. Slightly above half, 1130 (55.9%) were males. The geographic and therapeutic coverage of mass drug administration of Ivermectin achieved in the LGA was 100% and 79.9%, respectively. The key factors affecting coverage includes the unavailability of drugs (48.8%), absenteeism of some of the household members (31%), and inadequate incentives to the CDDs by the government and poor record keeping by the CDDs. Conclusion: This study found that the minimum geographic and therapeutic coverage of Ivermectin distribution was achieved by CDD as recommended by the World Health Organization for the control of onchocerciasis. For this to be sustained and to achieve elimination, there must be an adequate supply of ivermectin, training of CDDs, retraining of CDDs, adequate supervision in record keeping, and health education to the community. [ABSTRACT FROM AUTHOR]

Modelling rainfall extremes and dry periods over the Southeast Asia (SEA) region is challenging due to the characteristics of the region, which consists of the Maritime Continent and a mountainous region; it also experiences monsoonal conditions, as it is located between the Asian summer monsoon and the Australian summer monsoon. Representing rainfall extremes is important for flood and drought assessments in the region. This paper evaluates extreme rainfall climatic indices from regional climate models from CORDEX Southeast Asia and compares them with the results of high‐resolution global climate models with a comparable spatial resolution from the HighResMIP experiment. Observations indicate a high intensity of rainfall over areas affected by tropical cyclones and long consecutive dry day periods over some areas in Indochina and the southern end of Indonesia. In the model simulations, we find that both coupled and sea surface temperature‐forced HighResMIP model experiments are more similar to the observations than CORDEX model results. However, the models produce a poorer simulation of precipitation intensity‐related indices due to model biases in the rainfall intensity. This bias is higher in CORDEX than in HighResMIP and is evident in both the low‐ and high‐resolution HighResMIP model versions. The comparable performances of HighResSST (atmosphere‐only runs) and Hist‐1950 (coupled ocean–atmosphere runs) demonstrate the accuracy of the ocean model. Comparable performances were also found for the two different resolutions of HighResMIP, suggesting that there is no improvement in the performance of the high‐resolution HighResMIP model compared to the low‐resolution HighResMIP model. [ABSTRACT FROM AUTHOR]

CDKL5 deficiency disorder (CDD) is a rare developmental and epileptic encephalopathy, developing in the first months of life, caused by a mutation in the cyclin-dependent kinase-like 5 (CDKL5) gene. Children with CDD often have sleep (90%) and breathing disorders in wake (50%). Sleep disorders may have a significant impact emotional wellbeing and quality of life of caregivers of children with CDD and are challenging to treat. The outcomes of these features are unknown in children with CDD. We retrospectively evaluated sleep and respiratory function changes over 5–10 years in a small cohort of Dutch children with CDD, using video-EEG and/or polysomnography (3 × 24 h) and a parental questionnaire, the Sleep Disturbance Scale for Children (SDSC). The present study is a follow-up sleep and PSG study to evaluate if sleep and breathing disturbances persist in children with CDD previously studied. Sleep disturbances persisted during the study period (5.5–10 years). All five individuals had long sleep latency (SL, range 32–174.5 min) and frequent arousals and awakenings (14–50/night), unrelated to apneas/seizures, corresponding to the SDSC findings. Low sleep efficiency (SE, 41–80%) was present and did not improve. In our participants, total sleep time (TST, 3h52min-7h52min) was short and remained so. Time in bed (TIB) was typical for children aged 2–8 years, but did not adjust with ageing. Low duration (4.8–17.4%) or even absent REM sleep persisted over time. No sleep apneas were noted. Central apneas due to episodic hyperventilation were reported during wakefulness in two of the five. Sleep disturbances were present and persisted in all. The decreased REM sleep and sporadic breathing disturbances in wake may indicate failure of brainstem nuclei. Sleep disturbances can severely affect the emotional wellbeing and quality of life of the caregivers and the individuals with CDD and are challenging to treat. Hopefully our polysomnographic sleep data contribute to find the optimal treatment of the sleep problems in CDD patients. • Sleep disturbances were present and persistent in people with CDD. • Sleep disorders may have significant impact on the emotional wellbeing and quality of life of caregivers and CDD individuals. • With ageing, respiratory disturbances in wake remained present in two of five people with CDD. • Decreased REM sleep and breathing disturbances in wakefulness may indicate failure of brainstem nuclei. [ABSTRACT FROM AUTHOR]

Copyright of NCTU Law Review is the property of National Yang Ming Chiao Tung University, School of Law & Taiwan Technology Law Institute and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

[Display omitted] • TM@Al 12 P 12 nanoclusters as single atom catalysts (SAC) toward OER. • Catalytic efficiency is investigated via DFT calculations. • Best catalytic efficiency is shown by Co@Al 12 P 12 complex. Global warming is an unparalleled challenge to the globe. The increased usage of fossil fuel has significantly increased the CO 2 level in the environment. Thus, scientists have diversified their attention towards other energy resources. Hydrogen energy represents a sustainable resource of green energy, where hydrogen and oxygen gases are converted into high amounts of energy without emitting greenhouse gases. In this work, we evaluated the potential use of transition metal (TM) doped Al 12 P 12 single atom catalysts (SACs) toward the oxygen evolution reaction (OER) on the basis of the density functional theory (DFT) calculations. Results have shown that the highest activity is realized by the Co@Al 12 P 12 SAC. This study represents another milestone in our quest of exploring the applicability of different TM@Nanocage systems as SACs for water electrolysis. We hope that this study will assist in the full realization of the water splitting reaction to produce hydrogen energy fuel. [ABSTRACT FROM AUTHOR]

CDKL5 deficiency disorder (CDD) is an epileptic encephalopathy associated with medically refractory epilepsy. We sought to determine whether prolonged corrected QT interval (QTc) or other cardiac conduction abnormalities were seen in CDD in a clinical cohort. A cohort of individuals with CDD was evaluated in the Children's Hospital Colorado's International Foundation for CDKL5 Research designated Center of Excellence clinic with routine electrocardiograms obtained as part of routine clinical care. Retrospective review of electrocardiograms was completed. ECGs from 44 individuals (7 male, 37 female, age range 0–34.5 years) with pathogenic mutations and findings consistent with CDD were evaluated. Multiple ECGs were available from the 44 individuals obtained from 1996 to 2020. Prolonged QTc was found in two individuals (4.5%) and either resolved or was not confirmed on Holter monitor; no additional interventions were performed. A total of 11 individuals had echocardiograms for a variety of indications including unexplained tachycardia and ECG abnormalities; all were normal. Two individuals in the cohort died during the study with no abnormal findings on ECG. The incidence of prolonged QTc or other significant actionable cardiac abnormalities was rare in a cohort of individuals with CDD though was higher than the prevalence seen within the general population. Further studies in a larger, confirmatory cohort over a longer period are needed. [ABSTRACT FROM AUTHOR]

Background: CDKL5 Deficiency Disorder (CDD) is a rare developmental and epileptic encephalopathy characterized by dominant X-linked inheritance and early infantile onset. To date, more than 300 pathogenic variants of the CDKL5 gene have been reported with different phenotypes. As a rare genetic disease, data on CDD are still limited, making the diagnostic and therapeutic process very challenging. The objective of our study was to provide a comprehensive overview of CDD, including those aspects of the disease for which there is unfortunately still limited knowledge., Materials and Methods: The presence of a CDKL5 variant, cognitive impairment/delayed psychomotor development, and onset of epilepsy within the first year of life were screened for the diagnosis. Comprehensive clinical assessment, laboratory and radiological investigations were performed., Results: Fifteen (n=15) patients were enrolled in the study. In most cases, concordance was found between our data and those already present in the literature. In contrast, some other features, including the development of macrocephaly and the presence of congenital gastrointestinal malformations and spinal cord abnormalities, differ from previous findings., Conclusions: Our study provides an overview on CDD, including those features for which we still have limited knowledge and, albeit on a limited sample, several insights on this rare condition., (Copyright © 2024. Published by Elsevier B.V.)

Early epilepsy is a prominent feature in patients with CDKL5-deficiency disorder (CDD). The underlying mechanism for excessive excitability in CDD is largely unknown. The brain organoid model has been recently developed to resemble many critical features of early human brain development. Here, we used a brain organoid model to investigate the cellular electrophysiological basis for hyper-excitability in CDD patients. Our study employed cortical organoids derived from two CDD patients harboring the same CDKL5 mutation (R59X) and two controls from their healthy parents. Whole-cell patch-clamp recordings revealed higher action potential (AP) firing rate and lower rheobase in both CDD organoids, indicating increased intrinsic neuronal excitability. We further found dysfunction of voltage-gated ion channels in CDD neurons that leads to hyperexcitability, including higher Na+ and K+ current densities and a negative shift in Na+ channel activation. In contrast to neuronal properties, we found that glutamatergic neurotransmission and the electrophysiological properties of glial cells were not altered in CDD organoids. In support of our CDD findings, we further discovered similar electrophysiologic properties in cortical organoids derived from a Rett syndrome (RTT) patient, including alterations in AP firings and Na+ and K+ channel function suggesting a convergent mechanism. Together, our study suggests a critical role of intrinsic neuronal hyperexcitability and ion channel dysfunction, seen in early brain development in both CDD and RTT disorders. This investigation provides potential novel drug targets for developing treatments of early epilepsy in such disorders.

Heating and Cooling Degree Days (HDD, CDD) are indicative of the energy needs of buildings and also are associated with agriculturalism, tourism and other outdoor activities. Under the changing climate, future modifications of HDD and CDD are of primary importance. In the present work, monthly and annual HDD and CDD are computed and analyzed for the present and near future climate conditions. Elevation and sea proximity were found to be crucial in the formulation of energy requirements. Summer energy needs for cooling are expected to increase due to global warming while winter needs for heating are expected to decrease.

Childhood Disintegrative Disorder (CDD) is a rare condition characterized by regression of developmental and behavioral functioning after a period of apparently normal development, with an age of onset around 4 years. CDD is not included within the latest edition of the Diagnostic and Statistical Manual of Mental Disorders. We present a case report of an 11-year-old male who achieved normal development for up to 7 years followed by a deterioration of previously acquired linguistic, intellectual, and social skills. Following treatment with lithium carbonate combined with risperidone, the patient experienced a reduction in irritability and aggression. CDD is a rare condition; therefore, the data presented may be useful to investigate its characteristics of the onset, to improve the understanding of the aspects of differentiation from the Autism Spectrum Disorder and finally to propose the possibility of treatment [ABSTRACT FROM AUTHOR]

Purpose: The optimal timing of elective surgery in patients with the colonic diverticular disease remains controversial. We aimed to analyze the timing of sigmoidectomy in patients with diverticular disease and its influence on postoperative course with respect to the classification of diverticular disease (CDD). Methods: Patients who underwent elective laparoscopic sigmoidectomy were retrospectively enrolled and subdivided into two groups based on the time interval between the last attack and surgery: group A, early elective (≤ 6 weeks), and group B, elective (> 6 weeks). Multivariate regression models were used to identify factors which predict conversion to laparotomy, postoperative course, and length of hospital stay. Results: A total of 133 patients (group A (n = 88), group B (n = 45)) were included. Basic demographic data did not differ between groups except for a higher rate of diabetes in group B (p = 0.009). The conversion rate was significantly higher in group A in comparison to group B (group A vs. group B: n = 23 (26.1%) vs. n = 3 (6.7%), p = 0.007). Logistic regression analysis revealed the timing of surgery and CDD stage as significant predictors for intraoperative conversion. Moreover, the postoperative course was influenced by high age as well as intraoperative conversion and length of hospital stay by conversion, preoperative CRP levels, and elective surgery. Conclusions: Both, timing of surgery and the disease stage, influence the conversion rates in laparoscopic sigmoidectomy for diverticular disease. Accordingly, patients with complicated acute or chronic sigmoid diverticulitis should be operated in the inflammation-free interval. [ABSTRACT FROM AUTHOR]

Childhood Disintegrative Disorder (CDD) is a rare and little researched developmental disorder characterised by regression in language and social skills after a period of seemingly normal development until at least the age of 2 years. The study contacted all parents of CDD patients in Norway to assess patient symptomatology and parents' experiences of regression via questionnaire or interview. There were 12 participants. Symptomatology was in-line with previous studies, with universal regression in language and social skills and onset predominantly at 2–4 years. Regression was connected to feelings of 'loss' and uncertainty over the prognosis for CDD patients. The study supported CDD diagnostic criteria and showed that CDD patient regression has profound implications for parental well-being. [ABSTRACT FROM AUTHOR]

Cyclin-dependent kinase-like 5 (CDKL5) deficiency disorder (CDD) is a rare neurodevelopmental disorder caused by a mutation in the X-linked CDKL5 gene. CDKL5 is a serine/threonine kinase that is critical for axon outgrowth and dendritic morphogenesis as well as synapse formation, maturation, and maintenance. This disorder is characterized by early-onset epilepsy, hypotonia, and failure to reach cognitive and motor developmental milestones. Because the disease is monogenic, delivery of the CDKL5 gene to the brain of patients should provide clinical benefit. To this end, we designed a gene therapy vector, adeno-associated virus (AAV)9.Syn.hCDKL5, in which human CDKL5 gene expression is driven by the synapsin promoter. In biodistribution studies conducted in mice, intracerebroventricular (i.c.v.) injection resulted in broader, more optimal biodistribution than did intra-cisterna magna (i.c.m.) delivery. AAV9.Syn.hCDKL5 treatment increased phosphorylation of EB2, a bona fide CDKL5 substrate, demonstrating biological activity in vivo. Our data provide proof of concept that i.c.v. delivery of AAV9.Syn.hCDKL5 to neonatal male Cdkl5 knockout mice reduces pathology and reduces aberrant behavior. Functional improvements were seen at doses of 3e11 to 5e11 vector genomes/g brain, which resulted in transfection of ≥50% of the neurons. Functional improvements were not seen at lower doses, suggesting a requirement for broad distribution for efficacy., Competing Interests: Declaration of interests G.V., J.N., J.G., J.S., P.L., M.P., S.D., L.C., Y.V., M.J.K., L.P., E.F., S.S., B.R., P.Y., C.S., J.P., K.S., S.J., J.L., A.M., E.M.W., Z.W., and M.W. are or were at the time the work employees of PTC Therapeutics. M.C.W. is the President and CEO of NeuroDigiTech and was paid by PTC Therapeutics for this work., (Copyright © 2024 The American Society of Gene and Cell Therapy. Published by Elsevier Inc. All rights reserved.)

Despite the unmet needs of patients living with cyclin-dependent kinase-like 5 (CDKL5) deficiency disorder (CDD) and the challenges facing a rare population with small patient numbers, now is a time of unprecedented opportunities to turn scientific breakthroughs into safe and effective treatments for families of CDD patients. New data collected for over a decade and an evolution in genetics technologies have resulted in transformational new treatments currently in development for CDD. This progress is in great part due to the patient advocacy efforts early on to drive development of stakeholder research tools necessary to de-risk industry entry into the CDD space, family participation in longitudinal natural history studies, and a robust caregiver-reported database. Cumulatively, these efforts offered new insights into CDD, specifically patterns in disease progression, helped identify the most burdensome symptoms to patients and caregivers, improved clinical trial design, and reduced financial barriers for therapeutic development for potential industry partners. This paper documents the growth of a small patient community through relationship building and collaboration. The International Foundation for CDKL5 Research is mindful of ongoing challenges namely the long research timelines, high development and production costs, and inequitable access to approved therapies. Therefore, sustaining strong early resources while recognizing opportunities that engagement, advocacy, and funding can accelerate progress remains at the heart of the agile foundation strategy., Competing Interests: The authors declare that there is no conflict of interest., (© The Author(s), 2024.)

Cyclin-dependent kinase-like 5 (CDKL5) deficiency disorder (CDD) is a neurodevelopmental disease caused by mutations in the X-linked CDKL5 gene and characterized by early-onset epilepsy, intellectual disability, and autistic features. To date, the etiological mechanisms underlying CDD are largely unknown and no effective therapies are available. The Cdkl5 knock-out (KO) mouse has been broadly employed in preclinical studies on CDD; Cdkl5-KO mice display neurobehavioral abnormalities recapitulating most CDD symptoms, including alterations in motor, sensory, cognitive, and social abilities. However, most available preclinical studies have been carried out on adult Cdkl5-KO mice, so little is known about the phenotypic characteristics of this model earlier during development. Furthermore, major autistic-relevant phenotypes, for example, social and communication deficits, have been poorly investigated and mostly in male mutants. Here, we assessed the autistic-relevant behavioral phenotypes of Cdkl5-KO mice during the first three post-natal weeks and in adulthood. Males and females were tested, the latter including both heterozygous and homozygous mutants. Cdkl5 mutant pups showed qualitative and quantitative alterations in ultrasonic communication, detected first at 2 weeks of age and confirmed later in adulthood. Increased levels of anxiety-like behaviors were observed in mutants at 3 weeks and in adulthood, when stereotypies, reduced social interaction and memory deficits were also observed. These behavioral effects of the mutation were evident in both sexes, being more marked and varied in homozygous than heterozygous females. These findings provide novel evidence for the autistic-relevant behavioral profile of the Cdkl5 mouse model, thus supporting its use in future preclinical studies investigating CDD pathology and autism spectrum disorders., (© 2024 The Author(s). Autism Research published by International Society for Autism Research and Wiley Periodicals LLC.)

A total of 1534 and 308 articles were published in the journal Current Drug Delivery (CDD), from 2004 and 2019 to 2021, respectively. In this commentary, their impacts were analyzed based on search data about citation times in the Web of Science. These publications were categorized from different standpoints and evaluated in terms of their citations, particularly in the year 2021. The thematic, contemporary, and local features of these articles, as well as the article types and publication formats, were interpreted. Results demonstrated that CDD should be loyal to the contents about drug delivery, particularly nano-drug delivery systems and nano-pharmaceutical technologies. Publications from the developing and developed countries and regions showed no remarkable differences; therefore, submissions are similarly welcomed. Research articles and review articles are the main stream of CDD. The ratio of review papers is about 30%, which is reasonable but should not be further extended. Moreover, open publications with an article processing charge always have a high impact than those with subscription., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

High-precision measurements of 184Os/188Os, 186Os/188Os, and 187Os/188Os ratios are significant in the fields of geochemistry and cosmochemistry. However, no high-precision measurement technique exists for simultaneously obtaining all three ratios using a static method for samples with an Os content of <1 ng or 186OsO 3 ‾ and 187OsO 3 ‾ ion-beam intensities of <150 mV. This greatly limits research on rare samples with small sample sizes or low Os contents, such as Lunar, Martian, or old Earth samples. This paper reports a static method, which could achieve the simultaneous measurement of 9 Faraday cups (FCs) with high-signal/noise ratio 1012 Ω amplifiers and 1013 Ω amplifiers and two compact discrete dynodes (CDDs). By analyzing two calibration solutions, a precision value of less than 3‰ (2RSD) could be achieved for 184Os/188Os ratios, even if the 184OsO 3 ‾ intensity was as low as 1000 cps. The precision values for the 186Os/188Os and 187Os/188Os ratios were similar and could be better than 0.066‰ (2RSD) when the intensities of 186OsO 3 ‾ and 187OsO 3 ‾ were greater than 30 mV, which can be obtained with conventional 1011 Ω amplifiers only at signals larger than 150 mV. Three geological reference materials were used in this study. The precision values of 184Os/188Os, 186Os/188Os, and 187Os/188Os ratios reached 2, 0.061, and 0.050‰ (2RSD), respectively, when the maximum Os amount was approximately 12 ng, and 87, 15, and 10‰ (2RSD), respectively, when the maximum Os amount was as low as approximately 66 pg. Additionally, our results show that changes in the volume of oxygen added during measurement can cause significant variations in the oxygen isotope composition. This static measurement method not only avoids the nonlinear signal change of the instrument during the analysis process but also realizes the accurate removal of the oxygen isobaric interference in the run, which could improve the precision of 184Os/188Os, 186Os/188Os, and 187Os/188Os ratios for small-size/low-signal samples. [Display omitted] • Combining Faraday cups and CDDs for Os analysis. • Simultaneous measurement for all Os isotopes and O isotopes [ABSTRACT FROM AUTHOR]

The clinical phenotype of Cyclin-Dependent Kinase-Like 5 (CDKL5) deficiency disorder (CDD) has been delineated but neuroimaging features have not been systematically analyzed. We studied brain magnetic resonance imaging (MRI) scans in a cohort of CDD patients and reviewed age at seizure onset, seizure semiology, head circumference. Thirty-five brain MRI from 22 unrelated patients were included. The median age at study entry was 13.4 years. In 14/22 patients (85.7%), MRI in the first year of life was unremarkable in all but two. In 11/22, we performed MRI after 24 months of age (range 2.5-23 years). In 8 out of 11 (72.7%), MRI showed supratentorial atrophy and in six cerebellar atrophy. Quantitative analysis detected volumetric reduction of the whole brain (-17.7%, P-value = 0.014), including both white matter (-25.7%, P-value = 0.005) and cortical gray matter (-9.1%, P-value = 0.098), with a reduction of surface area (-18.0%, P-value = 0.032), mainly involving the temporal regions, correlated with the head circumference (ρ = 0.79, P-value = 0.109). Both the qualitative structural assessment and the quantitative analysis detected brain volume reduction involving the gray and white matter. These neuroimaging findings may be related to either progressive changes due to CDD pathogenesis, or to the extreme severity of epilepsy, or both. Larger prospective studies are needed to clarify the bases for the structural changes we observed., (© The Author(s) 2023. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Cytidine 5'-monophosphate (5'-CMP), a key intermediate for the production of nucleotide derivatives, has been extensively used in food, agriculture, and medicine industries. Compared to RNA degradation and chemical synthesis, the biosynthesis of 5'-CMP has attracted wide attention due to its relatively low cost and eco-friendliness. In this study, we developed a cell-free regeneration of ATP based on polyphosphate kinase 2 (PPK2) to manufacture 5'-CMP from cytidine (CR). McPPK2 from Meiothermus cerbereus exhibited high specific activity (128.5 U/mg) and was used to accomplish ATP regeneration. McPPK2 and LhUCK (a uridine-cytidine kinase from Lactobacillus helveticus) were combined to convert CR to 5'-CMP. Further, the degradation of CR was inhibited by knocking out cdd from the Escherichia coli genome to enhance 5'-CMP production. Finally, the cell-free system based on ATP regeneration maximized the titer of 5'-CMP up to 143.5 mM. The wider applicability of this cell-free system was demonstrated in the synthesis of deoxycytidine 5'-monophosphate (5'-dCMP) from deoxycytidine (dCR) by incorporating McPPK2 and BsdCK (a deoxycytidine kinase from Bacillus subtilis). This study suggests that the cell-free regeneration of ATP based on PPK2 has the advantage of great flexibility for producing 5'-(d)CMP and other (deoxy)nucleotides. [ABSTRACT FROM AUTHOR]

Avenanthramides are secondary metabolites most prominently found in oat (Avena sativa.L). These are amides of cinnamoyl-anthranilic acids derivatives. Due to their fascinating biological potential, these compounds have attracted the curiosity of chemists. Herein, the theoretical background of the electronic transition in UV-Vis and ECD spectra of the (E&Z) isomers of N-(4′-Hydroxy-cinnamoyl)− 5- hydroxyanthranilic acid was studied. The physical mechanism of the electron transfer process during light excitation is more intuitively described with two-dimensional and three-dimensional visualization methods. The characteristics of the UV-Vis spectrum were extended to the electron-hole conceptualization and the charge transfer process was described with charge difference density (CDD) and transition density matrix (TDM). The interaction mechanism between light and molecule in ECD is quantitatively revealed by the transition electric dipole moment, transition magnetic dipole moment, and their tensor product. A well-defined UV –Vis spectrum was obtained for the E isomer whereas the ECD spectrum for the Z isomer. [Display omitted] • UV-Vis and ECD spectra of AVA (E&Z) isomers were analyzed. • 2-D visualization of TDM and the electric-magnetic interaction are carried out. • A well-defined UV –Vis spectrum was obtained for E isomer. • The high-intensity peaks in ECD was obtained for Z isomer. [ABSTRACT FROM AUTHOR]