Case Report: Analysis of Preserved Umbilical Cord Clarified X-Linked Anhidrotic Ectodermal Dysplasia With Immunodeficiency in Deceased, Undiagnosed Uncles

Family history is one key in diagnosing inborn errors of immunity (IEI); however, disease status is difficult to determine in deceased relatives. X-linked anhidrotic ectodermal dysplasia with immunodeficiency is one of the hyper IgM syndromes that is caused by a hypomorphic variant in the nuclear factor kappa beta essential modulator. We identified a novel IKBKG variant in a 7-month-old boy with pneumococcal rib osteomyelitis and later found that his mother has incontinentia pigmenti. Genetic analysis of preserved umbilical cords revealed the same variant in two of his deceased maternal uncles. Analysis of preserved umbilical cord tissue from deceased relatives can provide important information for diagnosing IEI in their descendants.

Analysis of Magnetohydrodynamic Perturbations in the Radial-field Solar Wind from Parker Solar Probe Observations

We report analysis of sub-Alfvénic magnetohydrodynamic (MHD) perturbations in the low-β radial-field solar wind employing the Parker Solar Probe spacecraft data from 2018 October 31 to November 12. We calculate wavevectors using the singular value decomposition method and separate MHD perturbations into three eigenmodes (Alfvén, fast, and slow modes) to explore the properties of sub-Alfvénic perturbations and the role of compressible perturbations in solar wind heating. The MHD perturbations show a high degree of Alfvénicity in the radial-field solar wind, with the energy fraction of Alfvén modes dominating (∼45%–83%) over those of fast modes (∼16%–43%) and slow modes (∼1%–19%). We present a detailed analysis of a representative event on 2018 November 10. Observations show that fast modes dominate magnetic compressibility, whereas slow modes dominate density compressibility. The energy damping rate of compressible modes is comparable to the heating rate, suggesting the collisionless damping of compressible modes could be significant for solar wind heating. These results are valuable for further studies of the imbalanced turbulence near the Sun and possible heating effects of compressible modes at MHD scales in low-β plasma.

Performance evaluation of various deployment scenarios of the 3-replicated Cassandra NoSQL cluster on AWS

A concept of distributed replicated NoSQL data storages Cassandra-like, HBase, MongoDB has been proposed to effectively manage Big Data set whose volume, velocity and variability are difficult to deal with by using the traditional Relational Database Management Systems. Tradeoffs between consistency, availability, partition tolerance and latency is intrinsic to such systems. Although relations between these properties have been previously identified by the well-known CAP and PACELC theorems in qualitative terms, it is still necessary to quantify how different consistency settings, deployment patterns and other properties affect system performance.This experience report analysis performance of the Cassandra NoSQL database cluster and studies the tradeoff between data consistency guaranties and performance in distributed data storages. The primary focus is on investigating the quantitative interplay between Cassandra response time, throughput and its consistency settings considering different single- and multi-region deployment scenarios. The study uses the YCSB benchmarking framework and reports the results of the read and write performance tests of the three-replicated Cassandra cluster deployed in the Amazon AWS. In this paper, we also put forward a notation which can be used to formally describe distributed deployment of Cassandra cluster and its nodes relative to each other and to a client application. We present quantitative results showing how different consistency settings and deployment patterns affect Cassandra performance under different workloads. In particular, our experiments show that strong consistency costs up to 22 % of performance in case of the centralized Cassandra cluster deployment and can cause a 600 % increase in the read/write requests if Cassandra replicas and its clients are globally distributed across different AWS Regions.

Tribological study of a polymer bushing wear with a steel axle for an automatic car tensioner

This paper aims to show through a factorial experimental design significant factors on the polymeric bushing wear PA66, including their amplitude, frequency and spring height, when interacting with a steel axle SAE1018 (from Society of Automotive Engineers - SAE) in a tension belt applied. It was verified that factors as amplitude and frequency are significant and it was possible to propose a mathematical model to predict this wear behaviour for the proposed test. Moreover, an adhesion wear behaviour was verified, whereof a polyamide film was observed on the axle transferred by the bushing. Through the microscopy report analysis, it was possible to see the transfer film topology characterisation for a better comprehension of the adhered polyamide film from the bushing to the axle surface.

Upright walking has driven unique vascular specialization of the hominin ilium

Background A novel physis in hominins modulates broadening and shortening of the ilium. We report analysis of a vascular canal system whose origin may be associated with this physis and which appears to be also unique to hominins. Its presence is potentially identifiable in the fossil record by its association with a highly enlarged foramen that is consistently present in modern humans and hominin fossils. Methods We measured the diameter of this foramen in humans, fossil hominins, and African great apes and corrected for body size. Results The mean relative human foramen diameter is significantly greater than those of either Pan or Gorilla. Moreover, eight of the nine values of the Cohen’s d for these differences in ratios are highly significant and support the ordering of magnitudes: Pan < Gorilla < Homo. The relative foramen diameter of A.L. 288-1 is above the 75th percentile of all other hominoids and at the high end of humans. The foramen is also present in ARA-VP-6/500. Conclusions We posit that the presence and significant enlargement of this foramen in fossils can reasonably serve as an indicator that its anterior inferior iliac spine emerged via the unique hominin physis. The foramen can therefore serve as an indicator of hominin iliac ontogenetic specialization for bipedality in fossil taxa.

APSA Graduate Placement Report: Analysis of Political Science Placements for 2018-2020

The Graduate Placement Report details findings on political science placements for the 2018-2019 and 2019-2020 academic years, preceding and during the first year of the COVID-19 pandemic. More candidates, specifically candidates from top National Research Council (NRC)-ranked institutions found first placements in contingent academic positions, still indicating an alteration to the most desirable placement path, with a post-doc or research position immediately after receipt of a PhD. Having a PhD, and full funding are strong determinants of placement. Men and non-URMs continue to take full-time post-doc positions as their first placements. There were more URMs and women in tenure-track positions in 2019-2020 than in 2018-2019. There was an overall increase in the number of candidates who did not find placement during the 2019-2020 academic year, which can most likely be attributed to impacts of the ongoing COVID-19 pandemic, and a lower than usual response rate.

Circ-FURIN Expression Enhances Osteoblast Differentiation In Dental Pulp Stem Cells Via SOX11 Signaling Pathway Sponging miR-125

Background: Many studies have found that circRNA plays a part in osteoblast differentiation. However, its mechanism remains unknown. Methods: High-throughput sequencing was used to identifield the different expression of circRNA during osteogenic dental pulp stem cells (DPSCs) differentiation. Luciferase report analysis and RT-qPCR were used to clarify the expression and regulation relationship among circ-FURIN, miR-125 and SOX11. The heterotopic bone formation experiment was further used to confirm the osteoblast differentiation of DPSC with different expression of circ-FURIN, miR-125 and SOX11. Results: Study indicated that circ-FURIN expression remarkably increased during osteoblast differentiation, yet circ-FURIN knockdown suppressed it. Bioinformatics and luciferase results discovered that miR-125 is the downstream target of circ-FURIN. Furthermore, circ-FURIN upregulation decreased miR-125 expression. MiR-125 upregulation restored the promotion effect of circ-FURIN on osteogenic DPSC differentiation. Luciferase report analysis verified that SOX11 is miR-125 downstream target. miR-125 overexpression suppressed osteogenic DPSC differentiation through targeting SOX11. SOX11 overexpression restored miR-125 inhibitory effect on osteogenic DPSC differentiation. In vivo experiments with heterotopic bone model suggested that circ-FURIN overexpression has crucial function to enhance heterotopic bone formation. Conclusions: In summary, circ-FURIN enhances osteoblast DPSC differentiation via the SOX11 signaling pathway by sponging miR-125. These findings suggest a novel therapeutic target for osteoporosis treatment.

Severe methanol poisoning treated with a novel hemodialysis system: a case report, analysis, and review

In May and June 2020, an outbreak of methanol poisoning arose in the southwest United States linked to ingestion of contaminated hand sanitizer imported during the coronavirus disease 2019 pandemic, ultimately resulting in over a dozen hospitalizations and at least four deaths in New Mexico and Arizona. In this report, we describe one of these cases in which profound methanol intoxication was successfully treated with the Tablo® Hemodialysis System, the first reported case of toxic alcohol poisoning treated with this novel device. We carry out a formal regression analysis of the serial methanol levels obtained in this case to conservatively estimate that intermittent hemodialysis with Tablo achieved a clearance of methanol of 239 mL/min (95% confidence interval, 173–305 mL/min), a clearance that is well within the previously published standard of care. We conclude by reviewing both the treatment of toxic alcohol poisoning and the determinants of small molecule clearance with hemodialysis, emphasizing the importance of optimizing the dialytic treatment of intoxications with extended treatment times and the use of high-efficiency dialyzers.