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Previous research has supported selecting development-matched targets rather than age-matched targets to teach play skills to children with autism spectrum disorder. However, few studies have been conducted, and replications and extensions of this research are needed. The current study replicated Pane et al. (2022) by comparing the acquisition of development-matched and age-matched play targets when teaching play skills to four children with autism. No contrived prompts or consequences were used to teach play skills in either condition. Extensions included identifying targets via a newer version of the Developmental Play Assessment, targeting different play categories, assessing additional imitation skills, and conducting a caregiver assessment to identify socially valid toys, play actions, and vocalizations based on each participant's common experiences and preferences as well as their caregiver's values and preferences. As in Pane et al., participants demonstrated a higher level of scripted play actions in the development-matched condition.

UNLABELLED: Biodegradable plastics are urgently needed to replace petroleum-derived polymeric materials and prevent their accumulation in the environment. To this end, we isolated and characterized a halophilic and alkaliphilic bacterium from the Great Salt Lake in Utah. The isolate was identified as a Halomonas species and designated CUBES01. Full-genome sequencing and genomic reconstruction revealed the unique genetic traits and metabolic capabilities of the strain, including the common polyhydroxyalkanoate (PHA) biosynthesis pathway. Fluorescence staining identified intracellular polyester granules that accumulated predominantly during the strains exponential growth, a feature rarely found among natural PHA producers. CUBES01 was found to metabolize a range of renewable carbon feedstocks, including glucosamine and acetyl-glucosamine, as well as sucrose, glucose, fructose, and further glycerol, propionate, and acetate. Depending on the substrate, the strain accumulated up to ~60% of its biomass (dry wt/wt) in poly(3-hydroxybutyrate), while reaching a doubling time of 1.7 h at 30°C and an optimum osmolarity of 1 M sodium chloride and a pH of 8.8. The physiological preferences of the strain may not only enable long-term aseptic cultivation but also facilitate the release of intracellular products through osmolysis. The development of a minimal medium also allowed the estimation of maximum polyhydroxybutyrate production rates, which were projected to exceed 5 g/h. Finally, also, the genetic tractability of the strain was assessed in conjugation experiments: two orthogonal plasmid vectors were stable in the heterologous host, thereby opening the possibility of genetic engineering through the introduction of foreign genes. IMPORTANCE: The urgent need for renewable replacements for synthetic materials may be addressed through microbial biotechnology. To simplify the large-scale implementation of such bio-processes, robust cell factories that can utilize sustainable and widely available feedstocks are pivotal. To this end, non-axenic growth-associated production could reduce operational costs and enhance biomass productivity, thereby improving commercial competitiveness. Another major cost factor is downstream processing, especially in the case of intracellular products, such as bio-polyesters. Simplified cell-lysis strategies could also further improve economic viability.

ABSTRACT: The advent of immunological therapies has revolutionized the treatment of solid and haematological cancers over the last decade. Licensed therapies which activate the immune system to target cancer cells can be broadly divided into two classes. The first class are antibodies that inhibit immune checkpoint signalling, known as immune checkpoint inhibitors (ICIs). The second class are cell‐based immune therapies including chimeric antigen receptor T lymphocyte (CAR‐T) cell therapies, natural killer (NK) cell therapies, and tumour infiltrating lymphocyte (TIL) therapies. The clinical efficacy of all these treatments generally outweighs the risks, but there is a high rate of immune‐related adverse events (irAEs), which are often unpredictable in timing with clinical sequalae ranging from mild (e.g. rash) to severe or even fatal (e.g. myocarditis, cytokine release syndrome) and reversible to permanent (e.g. endocrinopathies).The mechanisms underpinning irAE pathology vary across different irAE complications and syndromes, reflecting the broad clinical phenotypes observed and the variability of different individual immune responses, and are poorly understood overall. Immune‐related cardiovascular toxicities have emerged, and our understanding has evolved from focussing initially on rare but fatal ICI‐related myocarditis with cardiogenic shock to more common complications including less severe ICI‐related myocarditis, pericarditis, arrhythmias, including conduction system disease and heart block, non‐inflammatory heart failure, takotsubo syndrome and coronary artery disease. In this scientific statement on the cardiovascular toxicities of immune therapies for cancer, we summarize the pathophysiology, epidemiology, diagnosis, and management of ICI, CAR‐T, NK, and TIL therapies. We also highlight gaps in the literature and where future research should focus.

Oculomotor deficits are common in hereditary ataxia, but disproportionally neglected in clinical ataxia scales and as outcome measures for interventional trials. Quantitative assessment of oculomotor function has become increasingly available and thus applicable in multicenter trials and offers the opportunity to capture severity and progression of oculomotor impairment in a sensitive and reliable manner. In this consensus paper of the Ataxia Global Initiative Working Group On Digital Oculomotor Biomarkers, based on a systematic literature review, we propose harmonized methodology and measurement parameters for the quantitative assessment of oculomotor function in natural-history studies and clinical trials in hereditary ataxia. MEDLINE was searched for articles reporting on oculomotor/vestibular properties in ataxia patients and a study-tailored quality-assessment was performed. One-hundred-and-seventeen articles reporting on subjects with genetically confirmed (n=1134) or suspected hereditary ataxia (n=198), and degenerative ataxias with sporadic presentation (n=480) were included and subject to data extraction. Based on robust discrimination from controls, correlation with disease-severity, sensitivity to change, and feasibility in international multicenter settings as prerequisite for clinical trials, we prioritize a core-set of five eye-movement types: (i) pursuit eye movements, (ii) saccadic eye movements, (iii) fixation, (iv) eccentric gaze holding, and (v) rotational vestibulo-ocular reflex. We provide detailed guidelines for their acquisition, and recommendations on the quantitative parameters to extract. Limitations include low study quality, heterogeneity in patient populations, and lack of longitudinal studies. Standardization of quantitative oculomotor assessments will facilitate their implementation, interpretation, and validation in clinical trials, and ultimately advance our understanding of the evolution of oculomotor network dysfunction in hereditary ataxias.

Background: The Pediatric Eating Assessment Tool (PEDI-EAT-10) is a reliable and valid tool for rapid identification of dysphagia in patients aged 18 months to 18 years. Aims: To translate and adapt the PEDI-EAT-10 into the Italian language and evaluate its validity and reliability. Methods & Procedures: The translation and cross-cultural adaptation of the tool consisted of five stages: initial translation, synthesis of the translations, back translation, expert committee evaluation and test of the prefinal version. The internal consistency of the translated tool was analysed in a clinical group composed of 200 patients with special healthcare needs aged between 18 months and 18 years. They were consecutively enrolled at the Rare Disease Unit, Paediatrics Department, Fondazione Policlinico Agostino Gemelli-IRCCS, Rome. For test-retest reliability, 50 caregivers filled in the PEDI-EAT-10 questionnaire for a second time after a 2-week period. Construct validity was established by comparing data obtained from patients with data from healthy participants (n = 200). The study was approved by the local ethics committee. Outcomes & Results: Psychometric data obtained from patients (104 M; mean age = 8.08 ± 4.85 years; median age = 7 years) showed satisfactory internal consistency (Cronbach's a = 0.89) and test-retest reliability (Pearson r = 0.99; Spearman r = 0.96). A total of 30% of children were classified as having a high risk of penetration/aspiration. The Italian PEDI-EAT-10 mean total score of the clinical group was significantly different from that resulting from healthy participants. Conclusions & Implications: The PEDI-EAT-10 was successfully translated into Italian, validated and found to be a reliable one-page rapid screening tool to identify dysphagia in children and adolescents with special needs.

Characterizing bedside oculomotor deficits is a critical factor in defining the clinical presentation of hereditary ataxias. Quantitative assessments are increasingly available and have significant advantages, including comparability over time, reduced examiner dependency, and sensitivity to subtle changes. To delineate the potential of quantitative oculomotor assessments as digital-motor outcome measures for clinical trials in ataxia, we searched MEDLINE for articles reporting on quantitative eye movement recordings in genetically confirmed or suspected hereditary ataxias, asking which paradigms are most promising for capturing disease progression and treatment response. Eighty-nine manuscripts identified reported on 1541 patients, including spinocerebellar ataxias (SCA2, n = 421), SCA3 (n = 268), SCA6 (n = 117), other SCAs (n = 97), Friedreich ataxia (FRDA, n = 178), Niemann-Pick disease type C (NPC, n = 57), and ataxia-telangiectasia (n = 85) as largest cohorts. Whereas most studies reported discriminatory power of oculomotor assessments in diagnostics, few explored their value for monitoring genotype-specific disease progression (n = 2; SCA2) or treatment response (n = 8; SCA2, FRDA, NPC, ataxia-telangiectasia, episodic-ataxia 4). Oculomotor parameters correlated with disease severity measures including clinical scores (n = 18 studies (SARA: n = 9)), chronological measures (e.g., age, disease duration, time-to-symptom onset; n = 17), genetic stratification (n = 9), and imaging measures of atrophy (n = 5). Recurrent correlations across many ataxias (SCA2/3/17, FRDA, NPC) suggest saccadic eye movements as potentially generic quantitative oculomotor outcome. Recommendation of other paradigms was limited by the scarcity of cross-validating correlations, except saccadic intrusions (FRDA), pursuit eye movements (SCA17), and quantitative head-impulse testing (SCA3/6). This work aids in understanding the current knowledge of quantitative oculomotor parameters in hereditary ataxias, and identifies gaps for validation as potential trial outcome measures in specific ataxia genotypes.

BACKGROUND: Dilated cardiomyopathy (DCM) is a major complication of, and leading cause of mortality in Duchenne muscular dystrophy (DMD). Its severity, age at onset, and rate of progression display wide variability, whose molecular bases have been scarcely elucidated. Potential DCM-modifying factors include glucocorticoid (GC) and cardiological treatments, DMD mutation type and location, and variants in other genes. METHODS AND RESULTS: We retrospectively collected 3138 echocardiographic measurements of left ventricular ejection fraction (EF), shortening fraction (SF), and end-diastolic volume (EDV) from 819 DMD participants, 541 from an Italian multicentric cohort and 278 from the Cooperative International Neuromuscular Group Duchenne Natural History Study (CINRG-DNHS). Using generalized estimating equation (GEE) models, we estimated the yearly rate of decrease of EF (-0.80%) and SF (-0.41%), while EDV increase was not significantly associated with age. Utilizing a multivariate generalized estimating equation (GEE) model we observed that mutations preserving the expression of the C-terminal Dp71 isoform of dystrophin were correlated with decreased EDV (-11.01 mL/m2, p = 0.03) while for dp116 were correlated with decreased EF (-4.14%, p =

Anaelisis buetir soael daepaet diguenaekaen uentuek membaentue meningkaetkaen kueaelitaes bute ir soael melaeluei revisi aetaeue membueane g soale yaeng tidaek efektif, dane daepaet diguenaekaen sebaegaei informaesi diaegnostik paedae siswae, aepaekahe merekae sude ahe memaehame i materie yane g telahe diaejaerkaen. Paedae kaeryae ilmiaeh ini, peneliti aekaen melaekuke aen aenaelisis buetir soael uelaengaen haeriaen mengguenaekane model Rasche yaeng melibatkanee sate upe aeraemeter unte uke menguke uer tingkate kesulite ane soale pade ae materie lingkaerane kelase V SDN 1 Syaemtaliraee Baeyue . Tuejueaen penelitiaen ini aedaelaeh une tuek mengetaehuie baegaeimaenae kueale itaes buetir soael daen kemame puanee siswae sekolahe dase ar,e khuse use nyae SDN 1 Syamte aliraee Baeyue Penelitiane ini merupe aekaen penelitiane evaelueaetif dengaen pendekaetaen deskriptif kueaentitaetif. Haesil penelitiaen menuenjuekkaen baehwae semueae soael yaeng diuejikaen paedae pesertae tes aedale aeh vaelid dane reliabile itas.e Hasile Tingkate kesuke aranee daeri 10 soale yane g diujie ade aelahe 4 soale tergolong muedaeh, 4 soael tergolong sedane g daen 2 soael tergolong saengaet suekaer

Checking the identity of motorcycle owners when leaving the college area is a mandatory activity for security officers to ensure that vehicles entering and exiting the college are the same driver. The conventional checking process often causes the impact of vehicle queues when the volume of vehicles increases. Therefore, an intelligent system is needed to detect multi-plate vehicles automatically. One approach in the world of image detection of an object is the use of the YOLO (You Only Look Once) algorithm. This algorithm predicts bounding boxes and possible classes in a single frame. This research divides objects into 3 classes, namely vehicles, driver's faces, and vehicle plates. The dataset used was 74 varied images consisting of 50 training data, 12 validation data and 12 testing data. The image was trained using 300 epochs and a batch size of 8 and resulted in an F1 score calculation for detecting objects reaching 92%.

Abstract Background Facioscapulohumeral dystrophy (FSHD) is a myopathy characterized by the loss of repressive epigenetic features affecting the D4Z4 locus (4q35). The assessment of DNA methylation at two regions (DUX4-PAS and DR1) of D4Z4 locus proved to be an effective method to detect epigenetic signatures compatible with FSHD. The present study aims at validating the employment of this method into clinical practice and improving the protocol by refining the classification thresholds of 4qA/4qA patients. To this purpose, 218 subjects with clinical suspicion of FSHD collected in 2022–2023 were analyzed. Each participant underwent in parallel the traditional FSHD molecular testing (D4Z4 sizing) and the proposed methylation assay. The results provided by both analyses were compared to evaluate the concordance and calculate the performance metrics of the methylation test. Results Among the 218 subjects, the 4q variant type distribution was 54% 4qA/4qA, 43% 4qA/4qB and 3% 4qB/4qB. The methylation analysis was performed only on carriers of at least one 4qA allele. After refining the classification threshold, the test reached the following performance metrics: sensitivity = 0.90, specificity = 1.00 and accuracy = 0.93. These results confirmed the effectiveness of the methylation assay in identifying patients with genetic signature compatible with FSHD1 and FSHD2 based on their DUX4-PAS and DR1 profile, respectively. The methylation data were also evaluated with respect to the clinical information. Conclusions The study confirmed the ability of the method to accurately identify methylation profiles compatible with FSHD genetic signatures considering the 4q genotype. Moreover, the test allows the detection of hypomethylated profiles in asymptomatic patients, suggesting its potential application in identifying preclinical conditions in patients with positive family history and FSHD genetic signatures. Furthermore, the present work emphasizes the importance of interpreting methylation profiles considering the patients’ clinical data.

The industrial revolution 4.0 which is occurring rapidly has resulted in changes in various fields, IoT is a technology that is currently widely used to automate processes in everyday life. In this research, IoT was used to monitor the development of goat livestock in Dermaji village. The problem with IoT development is that when you want to display data or results obtained, IoT does not work well if the information displayed is less interactive and difficult for end users to understand, so a solution needs to be created to display the results obtained by developing website and mobile-based applications. The aim of this research is to integrate an IoT platform with Android mobile-based websites and applications by implementing a RESTful web service API. Web and mobile application development uses the RAD method which consists of several stages, namely the design stage of system requirements, system design and implementation into a programming language. In integrating IoT with websites and mobile, RESTful web service technology is applied. The result of this research is an IoT platform that is integrated with website and Android mobile based applications.

Objectives: This study aimed to map the incidence of acute respiratory infections (ARIs) among under-5 children in Indonesia, address the triple burden of malnutrition, and analyze the impact of malnutrition on ARIs, taking into account the environmental and wealth disparities in Indonesia. Methods: This study utilized an ecological design, analyzing aggregate data from the Indonesia Nutrition Survey, 2022. It encompassed 33 provinces and 486 districts/cities, involving a total of 334 878 children under 5 years of age. Partial least squares structural equation modeling was employed to investigate the relationships among wealth, environment, malnutrition (stunting, wasting, and underweight), and ARIs. Results: The proportion of ARI cases in Indonesia was generally concentrated in central Sumatra, the western and eastern parts of Java, and eastern Papua. In contrast, the northern part of Sumatra, central Kalimantan, central Sulawesi, and central Papua had a higher proportion of malnutrition cases compared to other regions. Negative associations were found between malnutrition and ARIs (path coefficient =-0.072; p

Abstract Background To counteract soil degradation, it is important to convert conventional agricultural practices to environmentally sustainable management practices. To this end, the application of biostimulants could be considered a good strategy. Compost, produced by the composting of biodegradable organic compounds, is a source of natural biostimulants, such as humic acids, which are naturally occurring organic compounds that arise from the decomposition and transformation of organic residues, and compost tea, a compost-derived liquid formulated produced by compost water-phase extraction. This study aimed to determine the molecular responses of the roots of tomato plants (cv. Crovarese) grown under hydroponic conditions and subjected to biostimulation with humic substances (HSs) and filtered sterile compost tea (SCT). Results The 13C CPMAS NMR of humic acids (HA) and SCT revealed strong O-alkyl-C signals, indicating a high content of polysaccharides.Thermochemolysis identified over 100 molecules, predominantly from lignin, fatty acids, and biopolymers. RNA-Seq analysis of tomato roots treated with HA or SCT revealed differentially expressed genes (DEGs) with distinct patterns of transcriptional reprogramming. Notably, HA treatment affected carbohydrate metabolism and secondary metabolism, particularly phenylpropanoids and flavonoids, while SCT had a broader impact on hormone and redox metabolism. Both biostimulants induced significant gene expression changes within 24 h, including a reduction in cell wall degradation activity and an increase in the expression of hemicellulose synthesis genes, suggesting that the treatments prompted proactive cell wall development. Conclusions The results demonstrate that HS and SCT can mitigate stress by activating specific molecular mechanisms and modifying root metabolic pathways, particularly those involved in cell wall synthesis. However, gene regulation in response to these treatments is complex and influenced by various factors. These findings highlight the biostimulatory effects of HS and SCT, suggesting their potential application in crop biofertilization and the development of innovative breeding strategies to maximize the benefits of humic substances for crops. Further research is needed to fully elucidate these mechanisms across various contexts and plant species.

The Sorrento Peninsula is characterized by a significant occurrence of examples of vernacular architecture, which exhibit distinctive morphological and constructive features. These have been the subject of numerous studies. However, some buildings have undergone a process of transformation over time, the details of which have yet to be investigated. Architectures that initially held a rural character were enlarged and ennobled, thereby becoming what could be termed as “villas of delights”. However, these clearly manifest their origin based on the permanence of some vernacular features. This paper focuses on the analysis of a case study, Villa Murat, which is exemplary in illustrating this process. This thorough interdisciplinary research combines historical investigation, based mainly on archival documents, with a direct examination of the Villa. This has enabled the retracing of the building’s evolution and of the events that occurred in it. An integrated survey, which employed photogrammetry and laser scanning, enabled the assessment of the current state of conservation. The ultimate objective of this research is to propose conservative interventions which, in conjunction with the suggested new intended use, could ensure the preservation of the Villa.

The digital divide in Indonesia is rather big. This study aims to investigate the demographic and socioeconomic determinants affecting the quantity of uses of the Internet in Indonesia. The data used are based on the National Socioeconomic Survey in 2021. The object of the analysis is population aged 15 years and above possessing at least one digital gadget. The dependent variable is the purpose of the Internet use. The independent variables included gender, age, number of household members, marital status, education, employment status, place of dwelling, island of residence, and number of types of digital gadgets owned. A binary logistic regression model was employed in the analysis. The results of the study indicate that a higher quantity of uses of the Internet is associated with a female sex, younger age, smaller number of household members, unmarried status, higher education, dwelling in urban areas, living in Java Island, and owning more than one type of digital gadgets. These findings suggest the need to improve access to the Internet among men, older people, ever-married individuals, rural dwellers, and Outer Java inhabitants, to promote family planning, to improve education and accessibility of more types of digital gadgets in order to bridge the digital divide and to boost the global competitiveness index 4.0 in Indonesia.

The main factor that can determine the pyrolysis reaction is reactor type. However, the operational system of the pyrolysis reactor has a problem due to the low energy supply and low thermal conductivity of plastic. In addition, the particle agglomeration phenomenon occurs due to the presence of a thin layer due to the natural adhesive effect. This research made the development of the reactor design by the addition of a reactor section. The addition of a screw scrolls as a pressure drop system and fin parts on the condenser becomes the novelty. The aims of adding some sections are to reduce the particle agglomeration phenomenon and increase the quantity of syngas-pyrolysis oil from the system. The methodology is divided into design, manufacture, and pyrolysis experiments. This system can produce more pyrolysis oil compared to systems that use catalysts. The oil yield produced reached 88% at a target temperature of 450°C.

Dalam dunia komputasi awan yang semakin berkembang, ancaman serangan Distributed Denial of Service (DDoS) menjadi isu yang sangat krusial. Penelitian ini bertujuan untuk mengembangkan dan mengimplementasikan model prediksi serangan DDoS menggunakan algoritma Random Forest dan analisis perilaku jaringan. Dataset CICIDS2017 digunakan sebagai sumber data utama untuk melatih dan menguji model prediksi yang dikembangkan. Pemilihan algoritma Random Forest didasarkan pada kemampuannya yang tinggi dalam menangani data besar dan kompleks serta kemampuannya dalam mengenali pola anomali yang sering menjadi indikasi serangan siber. Hasil pengujian menunjukkan bahwa model ini mencapai akurasi yang signifikan dengan precision sebesar 97,8%, recall sebesar 98,2%, dan F1-score sebesar 98,0%. Analisis perilaku jaringan yang diterapkan, melibatkan fitur-fitur dinamis seperti waktu antar paket (Inter-Arrival Time/IAT), ukuran rata-rata segmen, dan jumlah paket per detik, yang terbukti efektif dalam meningkatkan kemampuan deteksi model. Implementasi model dalam lingkungan komputasi awan menunjukkan bahwa metode ini dapat diintegrasikan dengan sistem deteksi intrusi (Intrusion Detection Systems/IDS) yang sudah ada untuk memberikan lapisan perlindungan tambahan terhadap serangan DDoS. Berdasarkan hasil yang diperoleh, penelitian ini merekomendasikan penggunaan kombinasi algoritma Random Forest dan analisis perilaku jaringan sebagai solusi yang efektif untuk mendeteksi serangan DDoS dalam lingkungan komputasi awan. Penelitian lanjutan disarankan untuk mengembangkan dan menguji model dengan dataset yang lebih beragam serta mengoptimalkan algoritma untuk meningkatkan performa deteksi. Kata kunci: Random Forest, DDoS, Cloud Computing

Abstract We report the long-term results of a randomized trial (GITMO, AML-R2), comparing 1:1 the combination of busulfan and cyclophosphamide (BuCy2, n = 125) and the combination of busulfan and fludarabine (BuFlu, n = 127) as conditioning regimen in acute myeloid leukemia patients (median age 51 years, range 40–65) undergoing allogeneic hematopoietic stem cell transplantation. With a median follow-up of 6 years, significantly better non-relapse mortality (NRM) was confirmed in BuFlu recipients, which is sustained up to 4 years after transplant (10% vs. 20%, p = 0.0388). This difference was higher in patients older than 51 years (11% in BuFlu vs. 27% in BuCy2, p = 0.0262). The cumulative incidence of relapse, which was the first cause of death in the entire study population, did not differ between the two randomized arms. Similarly, the leukemia-free survival (LFS) and overall survival (OS) were not different in the two cohorts, even when stratifying patients per median age. Graft-and relapse-free survival (GRFS) in BuFlu arm vs. the BuCy2 arm was 25% vs. 20% at 4 years and 20% vs. 17% at 10 years. Hence, the benefit gained by NRM reduction is not offsets by an increased relapse. Leukemia relapse remains a major concern, urging the development of new therapeutic approaches.

Abstract Objective The dentato‐thalamo‐cortical tract (DTT) is the main cerebellar efferent pathway. Degeneration of the DTT is a core feature of Friedreich ataxia (FRDA). However, it remains unclear whether DTT disruption is spatially specific, with some segments being more impacted than others. This study aimed to investigate microstructural integrity along the DTT in FRDA using a profilometry diffusion MRI (dMRI) approach. Methods MRI data from 45 individuals with FRDA (mean age: 33.2 ± 13.2, Male/Female: 26/19) and 37 healthy controls (mean age: 36.5 ± 12.7, Male/Female:18/19) were included in this cross‐sectional multicenter study. A profilometry analysis was performed on dMRI data by first using tractography to define the DTT as the white matter pathway connecting the dentate nucleus to the contralateral motor cortex. The tract was then divided into 100 segments, and dMRI metrics of microstructural integrity (fractional anisotropy, mean diffusivity and radial diffusivity) at each segment were compared between groups. The process was replicated on the arcuate fasciculus for comparison. Results Across all diffusion metrics, the region of the DTT connecting the dentate nucleus and thalamus was more impacted in FRDA than downstream cerebral sections from the thalamus to the cortex. The arcuate fasciculus was minimally impacted. Interpretation Our study further expands the current knowledge about brain involvement in FRDA, showing that microstructural abnormalities within the DTT are weighted to early segments of the tract (i.e., the superior cerebellar peduncle). These findings are consistent with the hypothesis of DTT undergoing anterograde degeneration arising from the dentate nuclei and progressing to the primary motor cortex.

Summary: Background: The advent of disease-modifying treatments (DMT) has changed natural history in 5q Spinal muscular atrophy (SMA). The aim of this study was to report survival and functional aspects in all the Italian type I children born since 2016. Methods: The study included all symptomatic children with type I SMA born since January 1st, 2016, when DMTs became available in Italy. All the Italian SMA referral centers provided data on survival and motor, respiratory, and nutritional status. To compare survival rate pre and post DMTs approval, we also included similar data from SMA I patients born between January 1st, 2010, and December 31st, 2015. A two-proportion z-test was conducted to compare the two cohorts. The significance level was set at p 16 h/day ventilation or tracheostomy with continuous invasive ventilation. 130 of the 199 survivors (65%) achieved independent sitting, and 175 (87.9%) did not require tube feeding. Interpretation: Our study provides a picture of the ‘new natural history’ of type I SMA, confirming the impact of the new therapies on the progression of type I with longer survival r and has better motor, respiratory and nutritional. Funding: This research was partially funded by grants from the Italian Ministry of Health.

IntroductionPiwi proteins and the associated Piwi-interacting RNAs (piRNAs) coordinate a surveillance system that protects the animal genome from DNA damage induced by transposable element (TE) mobilization. While the pathway has been described in detail in the fruit fly Drosophila melanogaster, much less is known in more basal insects. Rhodnius prolixus is an hemipteran insect and one of the major vectors of Chagas disease. Rhodnius acquired specific classes of horizontally transferred transposons (HTTs) by feeding on bats, opossums and squirrel monkeys, thus providing the opportunity to investigate the piRNA-base response against HTTs in this species.MethodsSmallRNA-Seq reads mapping to HTTs and resident transposable elements were quantified and checked for piRNA features like 1U a 10A biases, ping-pong and phasing signatures. Uniquely mapped piRNAs were used to identify piRNA clusters in Rhodnius’ genome. RNA-Seq data was used to quantify transposon and Rp-PIWI genes expression levels and were validated by qRT-PCR.ResultsBy analyzing the temporal dynamics of piRNA cluster expression and piRNA production during critical stages of Rhodnius development, we show that peak levels of ∼28 nt long piRNAs correlate with reduced HTT and resident TE expression primarily during embryogenesis. Strikingly, while resident TEs piRNAs seem to engage in a typical ping-pong amplification mechanism, sense and antisense HTT piRNAs instead overlap by ∼20 nt or do not display ping-pong signatures.DiscussionOur data shed light on the biogenesis and functions of the piRNAs in Rhodnius prolixus and reveal that piRNAs, but not the siRNA pathway, responded to HTTs that were recently transferred from vertebrate tetrapods to a hematophagous insect of medical relevance.

This research explores the development of a real-time measurement system for light intensity and voltage in solar panels. It utilizes the ATmega328 microcontroller with an INA219 sensor for voltage measurement, a BH1750 sensor for light intensity measurement, and a logger module. The objective of this research is to assist and reduce losses in the use of solar panels. The research method is a simplified version of the 4D model, reduced to 3D: define, design, and develop. This system is designed to accurately collect data directly from solar panels, with a recorded data error rate of 23%. The gathered data is stored via an SD card on the logger module and converted into CSV text format. Test results indicate that this system can measure accurately and store data in a readily accessible and processable format. ATmega328, as the system's core, allows accurate real-time monitoring of solar panel conditions while storing data in CSV format, facilitating further analysis. Therefore, this system can be a valuable tool for monitoring and enhancing the energy efficiency produced by solar panels.

Purpose: Maximal speed is an important physical-fitness attribute for female Australian footballers. The effects of sprint training, maximal strength, and technical training have been reported in laboratory studies. However, no study has determined the combined effect and relative contribution of these training modalities on maximal speed adaptation in situ. Therefore, the aim of this study was to determine the training factors affecting maximal speed adaptation during a preseason in subelite female Australian Rules footballers. Methods: Maximal speed during field training, predicted 1-repetition maximum (1RM) for box squat and hip thrust, and sprint biomechanics were assessed during early and late preseason (∼9 wk apart) in 15 female subelite Australian Rules Football players (age 20 [3] y). On-field training volume and intensity (total distance, high-speed running, very-high-speed running, and maximal speed) were determined using a Global Positioning System. A multivariate regression model was used to determine the factors associated with changes in maximal speed across the preseason. Results: The preseason training program had a small effect on maximal speed and large to very large effects on strength and sprint biomechanics. The multivariate regression with the greatest fit (P <.001, R2 =.939) included change in estimated 1RM box squat (β = −0.63), total distance per week (β = −0.55), and change in hip projection (β = 0.16) as factors. Multivariate regression of biomechanical factors (P =.044, R2 =.717) and maximal strength factors (P =.003, R2 =.676) were also significant. Conclusion: The development of maximal speed across a preseason is dependent on (1) total distance per week, (2) maximal strength adaptation, and (3) sprint technique adaptation in female subelite Australian rules football players. [ABSTRACT FROM AUTHOR]