Lafora Disease Masquerading as Hepatic Dysfunction

Lafora disease is fatal intractable progressive myoclonic epilepsy. It is frequently characterized by epileptic seizures, difficulty walking, muscle spasms, and dementia in late childhood or adolescence. We chronicle here an unusual case of an asymptomatic young male soccer player who presented with elevated liver enzymes. Neurological examination was unremarkable. The diagnostic workup for hepatitis, infectious etiologies, autoimmune disorders, hemochromatosis, Wilson\u27s disease, alpha-1 antitrypsin deficiency, and other related diseases was inconclusive. He subsequently underwent an uneventful percutaneous liver biopsy. Based on the pathognomonic histopathological findings, Lafora disease was considered the likely etiology. The present study is a unique illustration of this rare disorder initially manifesting with abnormal liver enzymes. It underscores the importance of clinical suspicion of Lafora disease in cases with unexplained hepatic dysfunction. Prompt liver biopsy and genetic testing should be performed to antedate the onset of symptoms in these patients

Failure and strain gradient analyses in incremental forming using GTN model

Single point incremental forming (SPIF) process is gaining popularity for rapid manufacturing of complex 3D shapes. The present study is aimed at numerically predicting the failure using the Gurson-Tvergaard-Needleman (GTN) model and further clarifying the deformation mechanics in SPIF. Deformation mechanics and failure in SPIF process are examined numerically by simulating a straight groove test. Solid finite element (FE) models have been employed to investigate deformation modes. The GTN damage model has successfully predicted forming limits in the straight groove test. It is shown that failure, regardless of the incremental sheet forming (ISF) conditions, occurs when the damage parameter (d) approaches one. Furthermore, effective plastic strain, hydrostatic pressure, vertical tool force and through thickness strain gradient increase with an increase in step size. Finally, it is shown that an increase in hydrostatic pressure and increase in through thickness strain gradient enhance formability

Sustainable leadership and wellbeing of healthcare personnel: A sequential mediation model of procedural knowledge and compassion

IntroductionIn healthcare organizations, saving patients’ lives while maintaining the staff’s wellbeing, performance and competencies were challenging during the COVID-19 pandemic. Although the complexity of healthcare settings is widely recognized, the pandemic evidenced the necessity of attending to the employees’ wellbeing in such a sector. This research aims to examine the effect of sustainable leadership on wellbeing of healthcare personnel. Furthermore, we also evaluate whether procedural knowledge and compassion act as mediators in such a relationship.MethodsThe hypothesized model was tested in healthcare organizations in a South Asian country, and the data were collected during the pandemic crisis. A total of 366 health personnel (physicians and nurses) participated in this research. With Hayes’ PROCESS macro, we examined all the direct and indirect paths, including sequential mediation.ResultsThe findings confirm the impact of sustainable leadership on wellbeing and this relationship is also mediated by procedural knowledge and compassion.Discussion/conclusionSustainable leadership fosters wellbeing among healthcare workers via the sequential mediation of procedural knowledge and compassion. Study findings suggest that sustainable leaders can trigger procedural knowledge among employees which in turn crafts the state of compassion in them that leads to their wellbeing. Theoretical and practical implications are discussed in light of study findings

Challenges and practices identification in complex outsourcing relationships: A systematic literature review

Complex IT outsourcing relationships aptitude several benefits such as increased cost likelihood and lowered costs, higher scalability and flexibility upon demand. However, by virtue of its complexity, the complex outsourcing typically necessitates the interactions among various stakeholders from diverse regions and cultures, making it significantly more challenging to manage than traditional outsourcing. Furthermore, when compared to other types of outsourcing, complex outsourcing is extremely difficult because it necessitates a variety of control and coordination mechanisms for project management, which proportionally increases the risk of project failure. In order to overcome the failure of projects in complex outsourcing relationships, there is a need of robust systematic research to identify the key challenges and practices in this area. Therefore, this research implements systematic literature review as a research method and works as a pioneer attempt to accomplish the aforementioned objectives. Upon furnishing the SLR results, the authors identified 11 major challenges with 67 practices in hand from a total of 85 papers. Based on these findings, the authors intend to construct a comprehensive framework in the future by incorporating robust methodologies such as AHP and fuzzy logic, among others.Qatar National Library and Qatar University - grant No. IRCC- 2021-010

Diethyl (1-hydr­oxy-1-phenyl­ethyl)phospho­nate

The title compound, C12H19O4P, has a distorted tetra­hedral geometry around the P atom. The molecules form dimers with R 2 2(10) ring motifs due to inter­molecular O—H⋯O hydrogen bonds. The double-bonded O atom of the phospho­nate group behaves as an acceptor and the hydr­oxy group acts as a donor. Both of the ethyl groups are disordered with occupancies of 0.55:0.45 and 0.725:0.275

SCOL: Supervised Contrastive Ordinal Loss for Abdominal Aortic Calcification Scoring on Vertebral Fracture Assessment Scans

Abdominal Aortic Calcification (AAC) is a known marker of asymptomatic Atherosclerotic Cardiovascular Diseases (ASCVDs). AAC can be observed on Vertebral Fracture Assessment (VFA) scans acquired using Dual-Energy X-ray Absorptiometry (DXA) machines. Thus, the automatic quantification of AAC on VFA DXA scans may be used to screen for CVD risks, allowing early interventions. In this research, we formulate the quantification of AAC as an ordinal regression problem. We propose a novel Supervised Contrastive Ordinal Loss (SCOL) by incorporating a label-dependent distance metric with existing supervised contrastive loss to leverage the ordinal information inherent in discrete AAC regression labels. We develop a Dual-encoder Contrastive Ordinal Learning (DCOL) framework that learns the contrastive ordinal representation at global and local levels to improve the feature separability and class diversity in latent space among the AAC-24 genera. We evaluate the performance of the proposed framework using two clinical VFA DXA scan datasets and compare our work with state-of-the-art methods. Furthermore, for predicted AAC scores, we provide a clinical analysis to predict the future risk of a Major Acute Cardiovascular Event (MACE). Our results demonstrate that this learning enhances inter-class separability and strengthens intra-class consistency, which results in predicting the high-risk AAC classes with high sensitivity and high accuracy.Comment: Accepted in conference MICCAI 202

Plant Invasion and N₂O Emission in Forest Ecosystems

Nitrogen (N) is a key factor for any ecosystem and has been found limited for biomass production. More N in forest ecosystem and their efficient utilization will contribute to the maximization in their growth, competition, and reproduction. Invasive plants capture and utilize more N than native plants and accelerate N cycles through altering the structure and community of soil microbes and the litter decomposition rates, under microclimate conditions, resulting in an increase of N availability. All these factors are promoting the invasiveness of plants and cause further ecological and economic damage and decline in native biodiversity. Plant invasions affect soil microbial community, soil physiochemical properties, and litter decomposition rates, promoting N cycle and releasing more nitrous oxide (N2O) into the atmosphere, further facilitating global warming, causing changes in the geographic ranges of some invasive species. Also, a better understanding of the mechanism, affecting factors, impacts, and control of the invasive species will lead to proper forest management. Proper and effective management will ensure the control of invasive species which includes invasive plant inventory, early deduction and rapid response, management plan and implication, and government support

Co-designing Indus Water-Energy-Land Futures

The Indus River Basin covers an area of around 1 million square kilometers and connects four countries: Afghanistan, China, India, and Pakistan. More than 300 million people depend to some extent on the basin’s water, yet a growing population, increasing food and energy demands, climate change, and shifting monsoon patterns are exerting increasing pressure. Under these pressures, a “business as usual” (BAU) approach is no longer sustainable, and decision makers and wider stakeholders are calling for more integrated and inclusive development pathways that are in line with achieving the UN Sustainable Development Goals. Here, we propose an integrated nexus modeling framework co-designed with regional stakeholders from the four riparian countries of the Indus River Basin and discuss challenges and opportunities for developing transformation pathways for the basin’s future