La maladie de Fanconi: à propos d’une nouvelle observation

La maladie de Fanconi ou l'anémie de Fanconi (AF) est une maladie  génétique rare à transmission autosomique récessive. Elle est marquée parune hétérogénéité phénotypique. Certains symptômes et notamment la triade classique faite d'une petite taille, d'un syndrome malformatif varié et parfois discret et d'une insuffisance médullaire d'apparition précoce, doivent faire évoquer le diagnostic. Nous rapportons le cas d'un enfant âgé de sept ans, suivi et traité pour une luxation congénitale des hanches, qui présentait une pancytopénie avec à l'examen clinique on note un faciès dysmorphique triangulaire, une duplication du pouce droit, une surélévation de l'épaule gauche et un retard staturo-pondéral

β-decay half-lives and β-delayed neutron emission probabilities for several isotopes of Au, Hg, Tl, Pb, and Bi, beyond N = 126

Background: There have been measurements on roughly 230 nuclei that are β-delayed neutron emitters. They range from 8 He up to 150La. Apart from 210Tl, with a branching ratio of only 0.007%, no other neutron emitter has been measured beyond A = 150. Therefore, new data are needed, particularly in the region of heavy nuclei around N = 126, in order to guide theoretical models and help understand the formation of the third r-process peak at A ∼ 195. Purpose: To measure both β-decay half-lives and neutron branching ratios of several neutron-rich Au, Hg, Tl, Pb, and Bi isotopes beyond N = 126. Method: Ions of interest were produced by fragmentation of a 238U beam, selected and identified via the GSI-FRS fragment separator. A stack of segmented silicon detectors (SIMBA) was used to measure ion implants and β decays. An array of 30 3 He tubes embedded in a polyethylene matrix (BELEN) was used to detect neutrons with high efficiency and selectivity. A self-triggered digital system is employed to acquire data and to enable time correlations. The latter were analyzed with an analytical model and results for the half-lives and neutron-branching ratios were derived by using the binned maximum-likelihood method. Results: Twenty new β-decay half-lives are reported for 204−206Au, 208–211Hg, 211–216Tl, 215–218Pb, and 218–220Bi, nine of them for the first time. Neutron emission probabilities are reported for 210,211Hg and 211–216Tl. Conclusions: The new β-decay half-lives are in good agreement with previous measurements on nuclei in this region. The measured neutron emission probabilities are comparable to or smaller than values predicted by global models such as relativistic Hartree Bogoliubov plus the relativistic quasi-particle random phase approximation (RHB + RQRPA).Spanish Ministerio de Economía y Competitividad-FPA2011- 28770-C03-03, FPA2008-04972-C03-3, AIC-D2011-0705, FPA2011-24553, FPA2008-6419, FPA2010-17142, FPA2014-52823-C2-1-P, FPA2014- 52823-C2-2-P, and CPAN CSD-2007-00042 (Ingenio2010)Program Severo Ochoa-SEV-2014-0398German Helmholtz Association (Young Investigators)-VH-NG 627 (LISA-Lifetime Spectroscopy for Astrophysics)Nuclear Astrophysics Virtual Institute-VH-VI-417German Bundesministerium für Bildung und Forschung-06MT7178 / 05P12WOFNFSpanish Nuclear Security Council (CSN)-Catedra ArgosUK Science & Technology Facilities Council (STFC)-ST/F012012/

Grande tache pigmentée pileuse révélant une forme familiale de la maladie de Von Recklinghausen

La neurofibromatose de type 1 (NF1) ou maladie de Von Recklinghausen appartient au groupe de maladies appelées phacomatoses. C'est une affection autosomique dominante relativement rare. La NF1 est caractérisée par une extrême variabilité clinique que l'on retrouve également au sein d'une même famille. Le tableau clinique de la NF1 associe, le plus souvent, de multiples taches café au lait, des lentigines axillaires ou inguinales, des neurofibromes cutanés et des nodules de Lisch. Les difficultés d'apprentissage sont fréquentes et peuvent être graves dans certaines formes cliniques. Il est important de détecter précocement les neurofibromes plexiformes, les gliomes intracérébraux, les tumeurs des gaines nerveuses, les anomalies vasculaires et les dysplasies osseuses. L'évolution est imprévisible ce qui rend le pronostic incertain par une éventuelle survenue dedégénérescence malignes. Nous rapportons ici l'observation d'une grande tache cutanée pigmentée pilleuse de découverte fortuite qui nous a révélé deux cas familiaux de neurofibromatose 1 d'expression différente

Elastomer-based visuotactile sensor for normality of robotic manufacturing systems

Modern aircrafts require the assembly of thousands of components with high accuracy and reliability. The normality of drilled holes is a critical geometrical tolerance that is required to be achieved in order to realize an efficient assembly process. Failure to achieve the required tolerance leads to structures prone to fatigue problems and assembly errors. Elastomer-based tactile sensors have been used to support robots in acquiring useful physical interaction information with the environments. However, current tactile sensors have not yet been developed to support robotic machining in achieving the tight tolerances of aerospace structures. In this paper, a novel elastomer-based tactile sensor was developed for cobot machining. Three commercial silicon-based elastomer materials were characterised using mechanical testing in order to select a material with the best deformability. A Finite element model was developed to simulate the deformation of the tactile sensor upon interacting with surfaces with different normalities. Additive manufacturing was employed to fabricate the tactile sensor mould, which was chemically etched to improve the surface quality. The tactile sensor was obtained by directly casting and curing the optimum elastomer material onto the additively manufactured mould. A machine learning approach was used to train the simulated and experimental data obtained from the sensor. The capability of the developed vision tactile sensor was evaluated using real-world experiments with various inclination angles, and achieved a mean perpendicularity tolerance of 0.34°. The developed sensor opens a new perspective on low-cost precision cobot machining

Chest wall reconstruction still has place in Today’s modern practice:" a tertiary center experience"

Background: The key factor following chest wall resection is the preservation of the stability and integrity of the chest wall to support the respiration and protect the underlying organs. The present study aims to evaluate the use of the available grafts and prosthetic materials at our center in chest wall reconstruction with adherence to the proper surgical techniques, good perioperative and postoperative care to obtain good results. Methods: This is a retrospective single center study that concludes all patients underwent chest wall reconstruction for a variety of defects resulting from resection of tumors, trauma due to primarily firearms or motor car accidents, resection of radio necrotic tissues, infection and dehiscence of median sternotomy wounds after cardiac surgery.  Results: Study population consisted of 30 patients between January 2015and may 2018, among them were 20 male (70%) and 10 female patients (30%), with a median age of 43 ± 16.3 years, resection and reconstruction was performed in 23 cases (15 neoplastic,5 infective and  3 firearm cases) while reconstruction alone was performed in 7 (traumatic flail chest)  cases. Eighteen patients, underwent rib resection with an average 4.18 ± 2.2 ribs (range 2-6). Associated lung resection was performed in 5 patients (27.8 %): diaphragmatic resection was done in 2 cases in addition total sternal resection was performed in 5 cases. Most of the patients (96.7%) had primary healing of their wounds. there was one death (3.3%) in the early postoperative period. The average length of hospital stay for all patients was 8.7 days (range: 5–15). Respiratory complications occurred in three cases in the form of atelectasis and pneumonia at the ipsilateral side of reconstruction. Three cases suffered wound seroma which successfully managed by daily dressing and antibiotic coverage. Conclusions: according to our study and the analysis of similar studies, adequate perioperative preparation of patient undergoing chest wall resection and reconstruction with adherence to effective surgical techniques allowed us to use the available materials at our center for chest wall reconstruction with good and effective results without adding burden in terms of cost on the patient

A novel vision-based multi-functional sensor for normality and position measurements in precise robotic manufacturing

Cobots play an essential role in the fourth industrial revolution and the automation of complex manufacturing processes. However, cobots still face challenges in achieving high precision, which obstructs their usage in precise applications such as the aerospace industry. Nonetheless, advances in perception systems unlock new cobot manufacturing capabilities. This paper presents a novel multi-functional sensor that combines visual and tactile feedback using a single optical sensor, featuring a moving gate mechanism. This work also marks the first integration of Vision-Based Tactile Sensing (VBTS) into a robotic machining end-effector. The sensor provides vision-based tactile perception capabilities for precise normality control and exteroceptive perception for robot localization and positioning. Its performance is experimentally demonstrated in a precise robotic deburring application, where the sensor achieves the high-precision requirements of the aerospace industry with a mean normality error of 0.13° and a mean positioning error of 0.2 mm. These results open a new paradigm for using vision-based sensing for precise robotic manufacturing, which surpasses conventional approaches in terms of precision, weight, size, and cost-effectiveness

RAD: Reinforcement Authentication DYMO Protocol for MANET

Mobile ad hoc network (MANET) does not have fixed infrastructure or centralized server which manages the connections between the nodes. Rather, the nodes in MANET move randomly. Thus, it is risky to exchange data between nodes because there is a high possibility of having malicious node in the path. In this paper, we will describe a new authentication technique using message digest 5 (MD5), hashing for dynamic MANET on demand protocol (DYMO) based on reinforcement learning. In addition, we will describe an encryption technique that can be used without the need for a third party to distribute a secret key. After implementing the suggested model, results showed a remarkable enhancement in securing the path by increasing the packet delivery ratio and average throughput. On the other hand, there was an increase in end to end delay due to time spent in cryptographic operations

Removal of imidacloprid from polluted water using adsorption and membrane separation technologies

In this work, the stability of imidacloprid in fresh water and sludge was studied. The results revealed that the pesticide is unstable in both media. In freshwater, it underwent hydrolysis whereas a degradation to several metabolites has been observed in sludge. The rate constants for the hydrolysis and degradation at 25°C were 0.0.0067 and 0.0.0099 d–1, respectively. Monitoring the degradation of imidacloprid in sludge by high-pressure liquid chromatography-mass spectrometry (HPLC-MS) revealed that five metabolites have emerged during the study. These metabolites include imidacloprid urea, imidacloprid-guanidine, 6-hydroxynicotinic acid, an olefin, and 5-hydroxy, 1-(6-chloro-3-pyridylmethyl)-2-(nitroimino)-imidazolidin-5-ol. The efficiency of Al-Quds University Wastewater Treatment Plant towards the removal of imidacloprid indicates that the ultrafiltration-hollow fiber unit was insufficient, whereas the ultrafiltration-spiral wound, activated carbon, and reverse osmosis units were efficient for complete removal of the pesticide. Adsorption experiments of imidacloprid using either activated charcoal or micelle-clay complex were found to fit Langmuir isotherms better than Freundlich isotherm. The data demonstrate a higher Langmuir Qmax value for the activated charcoal (126.6 mg g–1) when compared to the micelle-clay complex (11.76 mg g–1). Filtration column experiments, conducted with mixed micelle-clay complex and sand (using a ratio of 1/50 by mass) at a flow rate of 2 mL min–1 and influent concentration of 50 mg L–1, revealed that a sufficient removal of imidacloprid was achieved in the first fraction of 100 mL elution. These findings indicate that the adsorption technology using the micelle-clay complex provides efficient removal of imidacloprid in continuous flow mode