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Posterior urethral valves (PUV) are congenital anomalies characterized by the persistence of mucosal folds in the urethra, leading to various degrees of obstruction. They are the most common cause of lower urinary tract obstruction in fetuses and children, with a severe prognosis, as one-third of affected children develop end-stage renal disease before adulthood. The French National Diagnostic and Care Protocol (NDCP) aim to provide healthcare professionals with guidelines for the optimal diagnostic and therapeutic management of PUV from the fetal stage to adolescence. The guidelines emphasize early diagnosis through prenatal ultrasound and the importance of a multidisciplinary approach involving pediatric urologists, nephrologists, and other specialists. It outlines prenatal interventions such as vesico-amniotic shunting and postnatal surgical options like endoscopic valve ablation to alleviate obstruction and preserve renal function. Long-term follow-up is crucial for monitoring renal function, managing bladder dysfunction, and preventing complications such as urinary tract infections and chronic kidney disease. The guidelines also identify off-label pharmaceuticals and necessary specialty products not typically covered by insurance. By standardizing care pathways and promoting consistent, high-quality care, the guidelines aim to improve the prognosis and quality of life for children with PUV, setting a benchmark for managing this rare condition in pediatric urology.

Background Spinocerebellar ataxia 27B is the most common genetic late onset cerebellar ataxia (LOCA). However, it commonly overlaps with other genetic LOCA as with the cerebellar form of multiple system atrophy (MSA-C). Objectives To pinpoint which clinical signs and symptoms best discriminate between FGF14  + from FGF14  − patients at symptoms’ onset. Methods Twenty SCA27B (≥ 250 GAA repeat expansion) patients were retrospectively matched by gender and age at disease onset with 20 negative FGF14 (−) LOCA patients and with 20 MSA-C patients. Clinical features were ranked based on their contribution towards distinguishing between the groups (feature importance ranking). Results SCA27B patients had significantly higher rates of episodic symptoms, cerebellar oculomotor signs, dysdiadochokinesia, and alcohol intolerance than LOCA- FGF14  − ataxia patients. The lack of autonomic symptoms and MRI signs in SCA27B patients were the most discriminating features from MSA-C. An AUC of 0.87 was obtained if using the “top 3 clinical features model” (episodic symptoms, cerebellar oculomotor signs and dysdiadochokinesia) to distinguish SCAB27 from LCOA FGF14  − . Regarding MRI findings, no significant differences were found between SCA27B and FGF14  − patients, while a positive hot cross buns sign and the presence of brainstem atrophy were key distinguishing features between SCA27B from MSA-C patients ( p  < 0.005). Conclusion Our pilot case–control study contributes to the identification of early clinical symptoms to differentiate SCA27B to LOCA patients including FGF14 - and MSA-C ones. From a feature perspective, while clinical features are crucial, identifying surrogate biomarkers—such as ocular or gait parameters—could aid in the early diagnosis and follow-up of SCA27B patients.

To study the dynamics of an orogen-foreland system and more particularly the impact of surface processes on its development, we base our work on two main axes. In a fi rst part, using analogue tapered wedge models, we investigate the interplay between tectonics, erosion and sedimentation during the orogenic growth. The basis model mimics the evolution of a wedge section extending from the orogen to its foreland basin, with both the structural and the lithological heritages simulated. In a second part, the focus is on the recent tectonic and geomorphologic processes affecting the Swiss Prealpes klippen belt and surrounding structural units (Molasse Basin and Jura fold-and-thrust belt). Remote sensing analyses allow to evaluate the impact of tectonics on the drainage pattern and landscape morphology. In addition, local fi eld investigations in the Prealpes provide information on the nature and kinematic of brittle fractures and the paleostress fi elds.Insights from analogue modelingThe series of seventeen analogue model experiments is based on a section across the north-wes tern Alpine wedge and foreland basin. This type of analogue model obeys to the dynamics of a tapered wedge and the structures produced are determined by the wedge mechanics. The orogen grows by incremental step of shortening, while outside processes such as erosion and sedimentation may infl uence the criticality of the taper. This is the case in the last six experiments, where erosion and sedimentation are manually performed in varying rates. These experiments are studied in detail and allowed to investigate and quantify: sediment budget, dynamics and timing of development of the orogen and adjacent foreland basin, uplift paths of particles, etc.The experiments show that two main mechanisms control the orogenic growth: frontal accretion and underplating. In the foreland basin, frontal accretion leads to the development of a foreland thrust belt, while in the hinterland, simple underthrusting and subsequent underplating lead to the formation of an antiformal nappe stack. The foreland basin evolution is mainly towards the front of the orogenic wedge but it differs depending on the amounts of erosion/sedimentation. Its evolution and internal structuring are governed by the wedge mechanics, thought to be the main controlling mechanism in a feedback interaction with surface processes. The basin grows to a threshold width and remains more or less constant during the subsequent evolution. This is possible because some older formed fold-and-thrust units are being eroded out of the record in the trailing part of the basin, while new units grow in its frontal part. During the development of the foreland fold-and-thrust belt, we observe the erosion of entire thrust units of freshly sedimented materials. As these types of events are untraceable in the tectonic and sedimentary record, this process leads to underestimating total shortening obtained from balan cing remaining units only.Our analogue models show the importance of feedback mechanisms of erosion/deposition on the wedge mechanics and the punctuated thrust development, as well as the cyclic behavior of surface processes. The variations in rates of erosion and sedimentation strongly modify the extent, the morphology, and the structure of the constitutive units. The timing of develop ment and the material paths also differ depending on the rates of surface processes in the different models. It appears for instance that particles located in the converging lower plate or in the upper plate show very complex uplift paths related to the different tectonic stages.The correlation between models and Alpine tectonic cross-sections emphasises the role of erosion and sedimentation on the dynamics and development of the orogen and adjacent Molasse Basin. Along strike changes in the geometry of the orogen can be explained by differences in surface processes. In addition, the main stages in evolution of the foreland basin and hinterland observed on the analogue models can be correlated in time and space with the major tectonosedimentary events of the Alpine belt. These stages of evolution are for instance corroborated by the nature of pebbles in the Alpine domain which co incide with the Alpine orogenic evolution and timing. Finally, we suggest that fl exural basin evolution mechanisms are secondary in the case of the Swiss Alpine Molasse Basin and that the fi rst order basin evolution is go verned by the internal mechanics of a tapered wedge type model.Insights from morphostructural study and stress analysis in the PrealpesThe Prealpes klippen are constituted by a series of allochthonous tectonic klippen located along the nor thern front of the Swiss and French Alps. Their emplacement onto the Alpine foreland took place in the Early Oligocene (30 Ma). The fracture pattern in the klippen is complex since it is the fi nal result of fold-and-thrust activity, neotectonics and certainly recent tectonics (post-nappe emplacement). To investigate the nature and kinematics of observed fractures in the Prealpes, we performed several types of analyses.We fi rst used remote sensing to determine the orientations of analysed structures. A morphological study of the area located between the klippen and the Molasse Plateau suggests that the hydrographic network mainly follows both glacial and structural trends. We then analysed locally the fracture and lineament orientations in three study areas located in the Swiss Chablais and in the Western Prealpes Romandes. Among different deformational models applicable to fault systems, the Riedel shear system appears to explain best the variety of obtained fault directions

Background In pigs, genetic progress has led to an increase in perinatal mortality, mainly due to reduced piglet maturity. The end of gestation (90-110 days of gestation) plays a decisive role in the acquisition of fetal maturity. The endometrium is crucial for its acquisition as it provides nutrients to the fetus through the placenta. The aim of this study is to describe part of the metabolism of the endometrium in late pregnancy and in relation with neonatal survival. For this purpose, we performed untargeted metabolomic measurements by 1H nuclear magnetic resonance and gas chromatography coupled to a flame-ionization detector on 224 endometrial samples and compared the two days of the end of gestation (D90-D110, term at 114 days) and two maternal breeds with contrasted birth survival: Large White (LW, higher birth mortality) and Meishan (MS, lower birth mortality). Results Out of the 191 metabolites available in the ASICS package reference library, 46 metabolites and nine neutral lipids were quantified in the endometrial samples. Twenty-two metabolites showed a significant differential abundance in the endometrium between 90 and 110 days of gestation. These differences highlighted a decrease in the amount of glucogenic amino acids such as aspartate, glutamate, and glutamine at D110, indicating a depletion of energy resources in the endometrium. As a result of hypoxic catabolism to maintain energy levels, hypoxanthine and succinate accumulated, possibly contributing to the regulation of hypoxia, ROS and modulation of inflammation of the endometrium during late pregnancy. The study also documented ten endometrial metabolites with a significant differential abundance between LW and MS sows. Glutathione metabolism metabolites showed a decreased abundance in LW, which may contribute to increased oxidative stress. Furthermore, the accumulation of glutamine and phenylalanine may be a possible response to lower amino acid availability in LW, inducing more cellular autophagy and lower maternal immune tolerance in LW endometrium compared to MS. Conclusions For the first time, these data provide a metabolic status of the endometrium during late gestation and between two extreme breeds for piglet survival. They reinforce the role of succinate, glutamine, and phenylalanine in influencing piglet survival birth.

Several observations in the world of comparative immunology in plants, insects, fish and eventually mammals lead to the discovery of trained immunity in the early 2010's. The first demonstrations provided evidence that innate immune cells were capable of developing memory after a first encounter with some pathogens. Trained immunity in mammals was initially described in monocytes with the Bacille Calmette-Guerin vaccine (BCG) or prototypical agonists like β-glucans. This phenomenon relies on epigenetic and metabolic modifications leading to an enhanced secretion of inflammatory cytokines when the host encounters homologous or heterologous pathogens. The objective of our research was to investigate the trained immunity, well-described in mouse and human, in other species of veterinary importance. For this purpose, we adapted an model of trained innate immunity in dogs. Blood enriched monocytes were stimulated with β-glucans and we confirmed that it induced an increased production of pro-inflammatory and anti-microbial compounds in response to bacterial stimuli. These results constitute the first demonstration of trained immunity in dogs and confirm its signatures in other mammalian species, with an implication of cellular mechanisms similar to those described in mice and humans regarding cellular epigenetics and metabolic regulations.

This paper describes an experience to incorporate the realization of virtual routes about the sculptural heritage of a city in the classroom by developing a simulation of the urban environment using a video game engine. Video game engines not only allow the creation of video games but also the creation and navigation of in-teractive three-dimensional worlds. For this research, Roblox Studio has been used, a simple and intuitive program in which no previous programming skills are required. During the 2018/2019 academic year, a pilot experience was carried out with 53 secondary school students who were given the task of designing a virtual environment in which they had to include 3D models of the sculptural her-itage of the city of Santa Cruz de Tenerife. Before starting the experience, the par-ticipants answered a questionnaire to obtain a previous idea of the students' knowledge about the creation of video games. Once the activity was finished and in order to evaluate the result of the activity, the participants answered a final questionnaire. The students emphasized that after the activity they are more aware of the sculptural heritage of Santa Cruz and that they consider themselves capable of creating their own interactive worlds with Roblox.

Background Embryonic and fetal development is very susceptible to the availability of nutrients that can interfere with the setting of epigenomes, thus modifying the main metabolic pathways and impacting the health and phenotypes of the future individual. We have previously reported that a 38% reduction of the methyl donor methionine in the diet of 30 female ducks reduced the body weight of their 180 mule ducklings compared to that of 190 ducklings from 30 control females. The maternal methionine-restricted diet also altered plasmatic parameters in 30 of their ducklings when compared to that of 30 ducklings from the control group. Thus, their plasma glucose and triglyceride concentrations were higher while their free fatty acid level and alanine transaminase activity were decreased. Moreover, the hepatic transcript level of 16 genes involved in pathways related to energy metabolism was significantly different between the two groups of ducklings. In the present work, we continued studying the liver of these newly hatched ducklings to explore the impact of the maternal dietary methionine restriction on the hepatic transcript level of 70 genes mostly involved in one-carbon metabolism and epigenetic mechanisms. Results Among the 12 genes ( SHMT1 , GART , ATIC , FTCD , MSRA , CBS , CTH , AHCYL1 , HSBP1 , DNMT3 , HDAC9 and EZH2 ) identified as differentially expressed between the two maternal diet groups ( p -value < 0.05), 3 of them were involved in epigenetic mechanisms. Ten other studied genes ( MTR , GLRX , MTHFR , AHCY , ADK , PRDM2 , EEF1A1 , ESR1 , PLAGL1 , and WNT11 ) tended to be differently expressed (0.05 <  p -value < 0.10). Moreover, the maternal dietary methionine restriction altered the number and nature of correlations between expression levels of differential genes for one-carbon metabolism and epigenetic mechanisms, expression levels of differential genes for energy metabolism, and phenotypic traits of ducklings. Conclusion This avian model showed that the maternal dietary methionine restriction impacted both the mRNA abundance of 22 genes involved in one-carbon metabolism or epigenetic mechanisms and the mRNA abundance of 16 genes involved in energy metabolism in the liver of the newly hatched offspring, in line with the previously observed changes in their phenotypic traits.