An Unusual Triad in Pediatric Neurology:A Case Report on Cerebral Palsy, Epilepsy, and Duchenne Muscular Dystrophy

We present a case of an unusual triad in pediatric neurology: a currently 12-year-old boy with cerebral palsy and epilepsy who was later also diagnosed with Duchenne muscular dystrophy. We describe the clinical path that resulted in this exceptional diagnosis. This case report illustrates how different neurological disorders may overshadow each other. In addition, it demonstrates that every child with cerebral palsy and either an atypical clinical course or with inexplicable laboratory values-as well as every infant boy born to a theoretical Duchenne muscular dystrophy carrier-should be subjected to additional investigations.</p

Dystrophin Distribution and Expression in Human and Experimental Temporal Lobe Epilepsy

Objective: Dystrophin is part of a protein complex that connects the cytoskeleton to the extracellular matrix. In addition to its role in muscle tissue, it functions as an anchoring protein within the central nervous system such as in hippocampus and cerebellum. Its presence in the latter regions is illustrated by the cognitive problems seen in Duchenne Muscular Dystrophy (DMD). Since epilepsy is also supposed to constitute a comorbidity of DMD, it is hypothesized that dystrophin plays a role in neuronal excitability. Here, we aimed to study brain dystrophin distribution and expression in both, human and experimental temporal lobe epilepsy (TLE). Method: Regional and cellular dystrophin distribution was evaluated in both human and rat hippocampi and in rat cerebellar tissue by immunofluorescent colocalization with neuronal (NeuN and calbindin) and glial (GFAP) markers. In addition, hippocampal dystrophin levels were estimated by Western blot analysis in biopsies from TLE patients, post-mortem controls, amygdala kindled (AK)-, and control rats. Results: Dystrophin was expressed in all hippocampal pyramidal subfields and in the molecular-, Purkinje-, and granular cell layer of the cerebellum. In these regions it colocalized with GFAP, suggesting expression in astrocytes such as Bergmann glia (BG) and velate protoplasmic astrocytes. In rat hippocampus and cerebellum there were neither differences in dystrophin positive cell types, nor in the regional dystrophin distribution between AK and control animals. Quantitatively, hippocampal full-length dystrophin (Dp427) levels were about 60% higher in human TLE patients than in post-mortem controls (p < 0.05), whereas the level of the shorter Dp71 isoform did not differ. In contrast, AK animals showed similar dystrophin levels as controls. Conclusion: Dystrophin is ubiquitously expressed by astrocytes in the human and rat hippocampus and in the rat cerebellum. Hippocampal full-length dystrophin (Dp427) levels are upregulated in human TLE, but not in AK rats, possibly indicating a compensatory mechanism in the chronic epileptic human brain

Earth Through the Eyes of NAPA-1: Commissioning Results and the Next Steps in CubeSat Earth Observation

Disruptive CubeSat technology has brought scientific missions within reach that were previously only achievable through larger spacecraft. Satellite Earth Observation is now the new frontier for governments, private industry, and academia. With the recent launch of the Napa-1 satellite the Royal Thai Airforce (RTAF) has joined the ranks by having its first ever Earth Observation CubeSat in space. Its design, launch, early operations (LEOPS), and commissioning have been carried out by ISISpace, supporting the market’s need for imagery from space. Napa-1, meaning firmament in Thai, is a 6U CubeSat with the Gecko Imager from SCS Space as its primary payload, capable of taking RGB snapshot images with a 39-meter ground sampling distance (GSD) from 500 km altitude. In addition, the TriScape camera from Simera Sense flies onboard as an in-orbit technology demonstrator and is capable of delivering high-quality images with a GSD of 5 meter in the RGB bands. With well over 200 images taken by the primary payload this paper will look back on this exciting first period of Napa-1’s operational life and proudly present the very first images taken by the satellite and the lessons learnt throughout this turnkey mission. With that many images taken and that much data generated, the implemented onboard- and on ground data handling systems have been put to the test. ISISpace has made use of KUBOS’ Major Tom for command and control and having integrated a low-level processing tool, also for image data preview and delivery. Insight is provided into the systems and tools in place for image target planning, image acquisition, satellite command and control, and data delivery to the customer. How is it ensured targets are successfully captured? How is the usefulness of the image data efficiently validated? Subsequently, how is knowledge transfer to the customer accomplished to ensure successful routine operations? ISISpace will share the valuable lessons learnt from the mission planning, data handling, operations, and training points of view and show relevant in-orbit data on, for example, attitude behavior and temperature. In parallel, ISISpace has taken the next step in CubeSat Earth observation by using Napa-1 as a baseline while accommodating larger data streams and leveraging a higher level of automation. Together with the companies Simera Sense and Pinkmatter Solutions, multispectral images with automated on ground data processing (L0 up to L3) are to be delivered by the follow-up mission, Napa-2, to be launched in the summer of 2021. Details on this mission, including a further outlook at how CubeSat imagery and on ground processing will be shaped in the next few years will be provided

Financialisation at a Watershed in the USA

In the period following the Great Recession of 2007-9 the financialisation of the US economy reached a watershed characterised by stagnant financial profits, falling proportions of financial sector and mortgage debt, and rising proportion of public debt. The main macroeconomic indicators of financialisation in the USA show structural breaks that can be dated around the period of the Great Recession. The reliance of households on the formal financial system appears to have weakened for the first time since the early 1980s. The financial sector has lacked the dynamism of the previous three decades becoming more reliant on government. The state has increased its own indebtedness and supported large financial institutions via unconventional monetary policy measures. At the same time, state intervention has tightened the regulatory framework for big banks. The future path of financialisation in the USA will depend heavily on government policy with regard to state debt and financial regulation, although the scope for boosting financialisation is narrow

Consumer credit in comparative perspective

We review the literature in sociology and related fields on the fast global growth of consumer credit and debt and the possible explanations for this expansion. We describe the ways people interact with the strongly segmented consumer credit system around the world—more specifically, the way they access credit and the way they are held accountable for their debt. We then report on research on two areas in which consumer credit is consequential: its effects on social relations and on physical and mental health. Throughout the article, we point out national variations and discuss explanations for these differences. We conclude with a brief discussion of the future tasks and challenges of comparative research on consumer credit.Accepted manuscrip

Effect of intraperitoneal chemotherapy concentration on morbidity and survival

Contains fulltext : 218098.pdf (publisher's version ) (Open Access)BACKGROUND: Selected patients with colorectal peritoneal metastases are treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). The concentration of intraperitoneal chemotherapy reflects the administered dose and perfusate volume. The aim of this study was to calculate intraperitoneal chemotherapy concentration during HIPEC and see whether this was related to clinical outcomes. METHODS: An observational multicentre study included consecutive patients with colorectal peritoneal metastases who were treated with CRS-HIPEC between 2010 and 2018 at three Dutch centres. Data were retrieved from prospectively developed databases. Chemotherapy dose and total circulating volumes of carrier solution were used to calculate chemotherapy concentrations. Postoperative complications, disease-free and overall survival were correlated with intraoperative chemotherapy concentrations. Univariable and multivariable logistic regression, Cox regression and survival analyses were performed. RESULTS: Of 320 patients, 220 received intraperitoneal mitomycin C (MMC) and 100 received oxaliplatin. Median perfusate volume for HIPEC was 5.0 (range 0.7-10.0) litres. Median intraperitoneal chemotherapy concentration was 13.3 (range 7.0-76.0) mg/l for MMC and 156.0 (91.9-377.6) mg/l in patients treated with oxaliplatin. Grade III or higher complications occurred in 75 patients (23.4 per cent). Median overall survival was 36.9 (i.q.r. 19.5-62.9) months. Intraperitoneal chemotherapy concentrations were not associated with postoperative complications or survival. CONCLUSION: CRS-HIPEC was performed with a wide variation in intraperitoneal chemotherapy concentrations that were not associated with complications or survival

Nuclear recoil response of liquid xenon and its impact on solar 8B neutrino and dark matter searches

Knowledge of the ionization and scintillation responses of liquid xenon (LXe) to nuclear recoils is crucial for LXe-based dark matter experiments. Current calibrations carry large uncertainties in the low-energy region below $\sim3$ keV$_nr$ where signals from dark matter particles of $<$10 GeV/c$^2$ masses are expected. The coherent elastic neutrino-nucleus scattering (CE$\nu$NS) by solar $^8$B neutrinos also results in a continuum of nuclear recoil events below 3.0 keV$_{nr}$ (99\% of events), which further complicates low-mass dark matter searches in LXe experiments. In this paper, we describe a method to quantify the uncertainties of low-energy LXe responses using published calibration data, followed by case studies to evaluate the impact of yield uncertainties on ${^8}$B searches and low-mass dark matter sensitivity in a typical ton-scale LXe experiment. We conclude that naively omitting yield uncertainties leads to overly optimistic limits by factor $\sim2$ for a 6 GeV/c$^2$ WIMP mass. Future nuclear recoil light yield calibrations could allow experiments to recover this sensitivity and also improve the accuracy of solar ${^8}$B flux measurements

The decline and rise of neighbourhoods: the importance of neighbourhood governance

There is a substantial literature on the explanation of neighbourhood change. Most of this literature concentrates on identifying factors and developments behind processes of decline. This paper reviews the literature, focusing on the identification of patterns of neighbourhood change, and argues that the concept of neighbourhood governance is a missing link in attempts to explain these patterns. Including neighbourhood governance in the explanations of neighbourhood change and decline will produce better explanatory models and, finally, a better view about what is actually steering neighbourhood change