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Hereditary Hemorrhagic Telangiectasia (HHT), also known as Rendu-Osler syndrome, is a genetic vascular disorder affecting 1 in 5000–8000 individuals worldwide. This rare disease is characterized by various vascular defects including epistaxis, blood vessel dilations (telangiectasia) and arteriovenous malformations (AVM) in several organs. About 90% of the cases are associated with heterozygous mutations of ACVRL1 or ENG genes, that respectively encode a bone morphogenetic protein receptor (activin receptor-like kinase 1, ALK1) and a co-receptor named endoglin. Less frequent mutations found in the remaining 10% of patients also affect the gene SMAD4 which is part of the transcriptional complex directly activated by this pathway. Presently, the therapeutic treatments for HHT are intended to reduce the symptoms of the disease. However, recent progress has been made using drugs that target VEGF (vascular endothelial growth factor) and the angiogenic pathway with the use of bevacizumab (anti-VEGF antibody). Furthermore, several exciting high-throughput screenings and preclinical studies have identified new molecular targets directly related to the signaling pathways affected in the disease. These include FKBP12, PI3-kinase and angiopoietin-2. This review aims at reporting these recent developments that should soon allow a better care of HHT patients.

يتلمس مؤلفو کتاب \"الاقتصاد السياسي للتعليم في العالم العربي\" إجابات في الاقتصادات السياسية ... التي تشكل بنية الحكم الوطني في المنطقة، وقد أظهروا بوضوح، من خلال ما قدموه من دراسات عن شمال إفريقيا ومنطقة الخليج، إضافة إلى وجهات نظر مقارنة تجارب دول شرق آسيا وأمريكا اللاتينية، أن الجهود المبذولة لتحسين التعليم، وتعزيز التنمية الاقتصادية تبعا لذلك وتوسيع قاعدة المواطنة التي تشكل عمدة استقرار وفعالية أنظمة حكم، ستفشل إلى الحد الذي تصبح معه النخب الحاكمة غير قادرة على زيادة قوتها السياسية والاقتصادية على حساب الصالح العام، يتكون الكتاب من أحد عشر بحثا توزعتها أقسام ثلاثة، بالإضافة إلى قسم تمهيدي مكون من دراستين هما بمثابة المدخلين للإشكاليات التي تناولها الكتاب، فالأولى تتمثل في البحث الأول المشترك بين هشام العلوي وروبرت سبرينغبورغ حيث عرضا بشكل عام لإشكاليات التعليم في العالم العربي من وجهة نظر الاقتصاد السياسي، واضعين القارئ في سياق الكتاب وهويته البحثية، وتتمثل الثانية في بحث إسحاق ديوان الذي تناول بدراسة رصدية إحصائية وتحليلية العلاقة بين إشكاليتي التعليم والديمقراطية في العالم العربي.

Autonomous driving is a complex task that requires high-level hierarchical reasoning. Various solutions based on hand-crafted rules, multi-modal systems, or end-to-end learning have been proposed over time but are not quite ready to deliver the accuracy and safety necessary for real-world urban autonomous driving. Those methods require expensive hardware for data collection or environmental perception and are sensitive to distribution shifts, making large-scale adoption impractical. We present an approach that solely uses monocular camera inputs to generate valuable data without any supervision. Our main contributions involve a mechanism that can provide steering data annotations starting from unlabeled data alongside a different pipeline that generates path labels in a completely self-supervised manner. Thus, our method represents a natural step towards leveraging the large amounts of available online data ensuring the complexity and the diversity required to learn a robust autonomous driving policy.

Tris and Four are now fugitives on the run, hunted by Jeanine, the leader of the power-hungry Erudite elite. Racing against time, they must find out what Tris's family sacrificed their lives to protect, and why the Erudite leaders will do anything to stop them. Haunted by her past choices but desperate to protect the ones she loves, Tris, with Four at her side, faces one impossible challenge after another as they unlock the truth about the past and ultimately the future of their world.

Background and Objectives: Orthognathic surgery is used to restore a correct anatomical and functional relationship between the jaws, with postoperative nasal septal deviation (NSD) being a common complication of Le Fort I osteotomy (LF-IO). The aim of this study was to evaluate the occurrence of NSD after LF-IO and to identify possible risk factors. Materials and Methods: Pre- and postoperative cone beam computed tomography (CBCT) scans from 2018 to 2023 of 102 patients after LF-IO were analyzed. After categorizing the preoperative NSDs according to the Mladina classification, the next step was to measure the angle of deviation and classify the severity grades. Pre- and postoperative NSDs were compared using a paired Wilcoxon signed-rank test and postoperative changes in NSD were correlated with surgery-relevant characteristics by calculating Spearman’s correlation coefficients. Results: Postoperatively, an increase in NSD was observed in 62 cases and 35 patients showed a decrease. In both cases with an increase and a decrease in NSD, the preoperatively measured deviations showed a highly significant difference compared to postoperative NSDs (both p < 0.001). Age correlated significantly with increases in deviation (r = 0.28, p = 0.014, CI: −1.0–−0.068) and anterior maxillary displacement showed a significant correlation with a decrease in NSD (r = 0.296, p = 0.042, CI: 0.006–1.0). Gender, cranial and caudal movements of the maxilla had no influence on the results of the NSDs. Conclusions: LF-IO has an influence on NSD and can both intensify and attenuate it. In addition, the risk of an increase in nasal deviation after this surgical procedure rises with the patient’s age and decreases with anterior displacement of the maxilla.

In the first part of the paper, the authors present the characteristics of the robots for all types of terrain. In the second part, two categories of robots are proposed: a robot with hybrid locomotion system and a modular robot. For the last category, if different modules are combined, a family of modular robots adaptable to different types of terrain can be obtained. The solutions proposed by the authors allow the study of the mobility and adaptability of robots to different types of terrain.

The contemporary landscape of substance use disorders, particularly opioid use disorder, presents a complex and pressing public health concern, marked by escalating rates of mortality and morbidity worldwide. Although extensive research has been conducted on the efficacy of interventions for opioid use disorder, a notable research gap exists concerning the perspectives of individuals in long-term recovery (defined as exceeding 2 years). Specifically, how these individuals view pharmacological and non-pharmacological treatment approaches remains largely unexplored in the existing literature. Grounded in the biopsychosocial model, this study investigates patients’ perceptions of medication-assisted treatment and abstinence-based therapies. Purposive sampling was utilized, and participants included ten individuals who were interviewed using semi-structured interviews. The findings reveal a consensus among participants advocating for short-term use of medication, emphasizing its role in managing withdrawal symptoms while expressing concerns about potential dependency. Additionally, the study highlights the transformative impact of peer support programs, particularly Alcoholics Anonymous, in fostering personal growth and accountability. Overall, the research aims to inform positive social change through treatment protocols and public health policies that integrate the lived experiences of those in sustained recovery, ultimately contributing to a more compassionate and effective approach to addiction treatment.

Die aktive Strömungsbeeinflussung kann in vielen technischen Bereichen zur Optimierung von aerodynamischen Eigenschaften bzw. zur Kompensation von Störungen eingesetzt werden. Allerdings ist die technische Realisierung der aktiven Strömungsbeeinflussung in vielen Fällen sehr herausfordernd. Dies gilt insbesondere bei der Umsetzung in einem geschlossenen Regelkreis. Zwei dieser Herausforderungen, auf die in dieser Arbeit eingegangen wird, sind die Beschreibung der Strömung in einem recheneffizienten Modell sowie der Umgang mit mechanischen Beschränkungen der Aktuation. Die beschriebenen Methoden werden im Rahmen dieser Arbeit genutzt, um ausgewählte strömungsmechanische Herausforderungen beim Betrieb einer Gasturbine mit Gleichraumverbrennung zu adressieren.Im ersten Teil der Arbeit wird eine Methode zur Zustandsraummodellierung auf Basis sogenannter Physics-Informed Neural Networks vorgestellt. Dieser Ansatz erlaubt es, physikalische Informationen aus einer Systembeschreibung mit partiellen Differentialgleichungen in der Anpassung eines Zustandsraummodells zu berücksichtigen. Damit können auch für Systemzustände, die nicht explizit in der Anpassung des Modells berücksichtigt wurden, zuverlässige Ergebnisse erzielt werden. Das vorgestellte Modell kann allerdings im Gegensatz zu einer numerischen Lösung der partiellen Differentialgleichungen in Echtzeit ausgewertet werden und ermöglicht so, die Anwendung in einem modellgestützten Mess- oder Regelungsverfahren. Im Rahmen dieser Arbeit wird die Modellierungsmethode jeweils für ein räumlich eindimensionales System auf Basis der Burgers-Gleichung bzw. den Euler-Gleichungen getestet. Es wird sowohl die Verwendung in einer Zustandsschätzung mit einem erweiterten Kalman-Filter untersucht als auch der Einsatz in einer modellprädiktiven Regelung.Der zweite Teil der Arbeit befasst sich mit der Regelung von zyklischen Prozessen, in denen die Aktuation beschränkt ist. Im Speziellen wird auf die Beschränkung auf einen ganzzahligen Wertebereich eingegangen. Dieser Fall ist in der aktiven Strömungsbeeinflussung vor allem bei der Verwendung von schaltenden Bauteilen mit einer beschränkten Aktuationsfrequenz relevant. Es wird ein Syntheseverfahren für eine normoptimale iterativ lernende Regelung (ILR) mit einem ganzzahlig beschränkten Eingang vorgestellt. Dabei stellt das Syntheseverfahren sicher, dass die relevanten Eigenschaften der reellwertigen normoptimalen ILR erhalten bleiben. Zusätzlich wird der Sonderfall einer binären ILR untersucht und ein Verfahren vorgestellt, mit dem unter bestimmten Umständen der Rechenaufwand der Regelung deutlich reduziert werden kann. Die Methode wird an einem akustischen Versuchsaufbau getestet, der ein Plenum hinter den Verbrennunsgrohren eine Gasturbine mit Gleichraumverbrennung nachbildet.Zusätzlich wird als Alternative zur normoptimalen ILR ein modellfreier Ansatz auf Basis einer Extremwertregelung (EWR) vorgestellt. Auch in diesem Verfahren können ganzzahlige Eingangsbeschränkungen für zyklische Prozesse berücksichtigt werden. Diese zyklische EWR wird an einem experimentellen Aufbau, einer sogenannten stoßfreien Explosionsverbrennung (SEC), getestet. Ziel für die SEC ist dabei, den Druckanstieg zu maximieren und damit möglichst gut eine Gleichraumverbrennung anzunähern.

The world is undergoing dramatic changes in the way society uses and governs forest resources. Global trade to serve urban populations has emerged as the dominant driver of deforestation. The multinational corporations working to serve global markets are now the primary actors linked to large scale deforestation. Such corporatization is often associated with intensification; in the timber and pulp sectors harvesting of natural forests has given way to a heavy reliance on fast-growing planted forests. While these changes have been a frequent source of concern among groups seeking to slow deforestation, these evolving threats may also provide new opportunities for conservation through supply chain governance. The shifting dynamics affecting global forests have led some observers to wonder whether an end to net global deforestation might be within reach. Forest transitions - shifts from extended periods of deforestation to forest expansion - in several developing countries have reinforced this optimism. To explore the impacts of emerging forest trends, this dissertation focuses on the dramatic changes that have taken place in Chile's forests over the past 30 years. Using a combination of remote sensing, land change science and econometric methods, my co-authors and I seek to answer a variety of questions. What are the extent, patterns and socio-ecological quality of Chile's forest transition? Will an increasing reliance on timber plantations lead to increased competition for forested lands, or ease harvesting pressure on natural forests? Has supply chain governance in Chile's timber sector reduced natural forest clearing? How have Chile's afforestation subsidies affected biodiversity and carbon sequestration? In answering each question, we seek to balance a detailed description of Chile's experience with the generation of insights to inform our understanding of the future of the world's forests.