Space shuttle: Basic supersonic force data for a Grumman delta wing orbiter configuration ROS-NB1

Supersonic force data for scale model of space shuttle delta wing orbite

Silicon vacancy containing two hydrogen atoms studied with electron paramagnetic resonance and infrared absorption spectroscopy, Phys

Float-zone and Czochralski-grown silicon crystals have been implanted with protons or deuterons. Electronparamagnetic-resonance measurements performed during illumination with light at 1064 nm reveal a signal, labeled DK5, in addition to the well-known signal from VO*-the excited spin-triplet state of the oxygenvacancy defect. The DK5 signal originates from a spin-triplet state of a vacancy-type defect with monoclinic-I ͑near-orthorhombic-I͒ symmetry. In contrast to the VO* signal, DK5 has about the same intensity in the spectra recorded on oxygen-lean and oxygen-rich samples, which indicates that the DK5 defect is not oxygen related. However, the close resemblance between the D tensors of DK5 and VO* strongly suggests that the electron-spin distributions are similar in the two defects. Moreover, anisotropic hyperfine splittings from two proton spins are partially resolved in the DK5 signal. The signal is assigned to VH 2 * , the excited spin-triplet state of the silicon vacancy containing two hydrogen atoms, which is the simplest defect consistent with the observed properties. The isochronal annealing behavior of DK5 coincides with that of two infrared-absorption lines at 2063 and 2077 cm Ϫ1 , which, like DK5, are observable only during illumination. These lines are assigned to Si-H stretch modes of VH 2 *

Plasma prostaglandin F2α and plasma 13,14-dihydro-15-keto-prostaglandin F2α levels in women during induction of labor with i. v. infusion of prostaglandin F2α in relation to uterine contractions

Peer Reviewe

Biallelic inherited SCN8A variants, a rare cause of SCN8A‐related developmental and epileptic encephalopathy

ObjectiveMonoallelic de novo gain‐of‐function variants in the voltage‐gated sodium channel SCN8A are one of the recurrent causes of severe developmental and epileptic encephalopathy (DEE). In addition, a small number of de novo or inherited monoallelic loss‐of‐function variants have been found in patients with intellectual disability, autism spectrum disorder, or movement disorders. Inherited monoallelic variants causing either gain or loss‐of‐function are also associated with less severe conditions such as benign familial infantile seizures and isolated movement disorders. In all three categories, the affected individuals are heterozygous for a SCN8A variant in combination with a wild‐type allele. In the present study, we describe two unusual families with severely affected individuals who inherited biallelic variants of SCN8A.MethodsWe identified two families with biallelic SCN8A variants by diagnostic gene panel sequencing. Functional analysis of the variants was performed using voltage clamp recordings from transfected ND7/23 cells.ResultsWe identified three probands from two unrelated families with DEE due to biallelic SCN8A variants. Each parent of an affected individual carried a single heterozygous SCN8A variant and exhibited mild cognitive impairment without seizures. In both families, functional analysis demonstrated segregation of one allele with complete loss‐of‐function, and one allele with altered biophysical properties consistent with partial loss‐of‐function.SignificanceThese studies demonstrate that SCN8A DEE may, in rare cases, result from inheritance of two variants, both of which exhibit reduced channel activity. In these families, heterozygosity for the dominant variants results in less severe disease than biallelic inheritance of two variant alleles. The clinical consequences of variants with partial and complete loss of SCN8A function are variable and likely to be influenced by genetic background.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/153117/1/epi16371_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/153117/2/epi16371.pd

Treatment challenges in and outside a specialist network setting: Pancreatic neuroendocrine tumours

Pancreatic Neuroendocrine Neoplasms comprise a group of rare tumours with special biology, an often indolent behaviour and particular diagnostic and therapeutic requirements. The specialized biochemical tests and radiological investigations, the complexity of surgical options and the variety of medical treatments that require individual tailoring, mandate a multidisciplinary approach that can be optimally achieved through an organized network. The present study describes currents concepts in the management of these tumours as well as an insight into the challenges of delivering the pathway in and outside a Network

Treatment challenges in and outside a network setting: Head and neck cancers

Head and neck cancer (HNC) is a rare disease that can affect different sites and is characterized by variable incidence and 5-year survival rates across Europe. Multiple factors need to be considered when choosing the most appropriate treatment for HNC patients, such as age, comorbidities, social issues, and especially whether to prefer surgery or radiation-based protocols. Given the complexity of this scenario, the creation of a highly specialized multidisciplinary team is recommended to guarantee the best oncological outcome and prevent or adequately treat any adverse effect. Data from literature suggest that the multidisciplinary team-based approach is beneficial for HNC patients and lead to improved survival rates. This result is likely due to improved diagnostic and staging accuracy, a more efficacious therapeutic approach and enhanced communication across disciplines. Despite the benefit of MTD, it must be noted that this approach requires considerable time, effort and financial resources and is usually more frequent in highly organized and high-volume centers. Literature data on clinical research suggest that patients treated in high-accrual centers report better treatment outcomes compared to patients treated in low-volume centers, where a lower radiotherapy-compliance and worst overall survival have been reported. There is general agreement that treatment of rare cancers such as HNC should be concentrated in high volume, specialized and multidisciplinary centers. In order to achieve this goal, the creation of international collaboration network is fundamental. The European Reference Networks for example aim to create an international virtual advisory board, whose objectives are the exchange of expertise, training, clinical collaboration and the reduction of disparities and enhancement of rationalize migration across Europe. The purpose of our work is to review all aspects and challenges in and outside this network setting planned for the management of HNC patients

Testicular germ-cell tumours and penile squamous cell carcinoma: Appropriate management makes the difference

Germ-cell tumours (GCT) of the testis and penile squamous cell carcinoma (PeSCC) are a rare and a very rare uro-genital cancers, respectively. Both tumours are well defined entities in terms of management, where specific recommendations - in the form of continuously up-to-dated guide lines-are provided. Impact of these tumour is relevant. Testicular GCT affects young, healthy men at the beginning of their adult life. PeSCC affects older men, but a proportion of these patients are young and the personal consequences of the disease may be devastating. Deviation from recommended management may be a reason of a significant prognostic worsening, as proper treatment favourably impacts on these tumours, dramatically on GCT and significantly on PeSCC. RARECAREnet data may permit to analyse how survivals may vary according to geographical areas, histology and age, leading to assume that non-homogeneous health-care resources may impact the cure and definitive outcomes. In support of this hypothesis, some epidemiologic datasets and clinical findings would indicate that survival may improve when appropriate treatments are delivered, linked to a different accessibility to the best health institutions, as a consequence of geographical, cultural and economic barriers. Finally, strong clues based on epidemiological and clinical data support the hypothesis that treatment delivered at reference centres or under the aegis of a qualified multi-institutional network is associated with a better prognosis of patients with these malignancies. The ERN EURACAN represents the best current European effort to answer this clinical need

Understanding the everyday designer in organisations

This paper builds upon the existing concept of an everyday designer as a non-expert designer who carries out design activities using available resources in a given environment. It does so by examining the design activities undertaken by non-expert, informal, designers in organisations who make use of the formal and informal technology already in use in organisations while designing to direct, influence, change or transform the practices of people in the organisation. These people represent a cohort of designers who are given little attention in the literature on information systems, despite their central role in the formation of practice and enactment of technology in organisations. The paper describes the experiences of 18 everyday designers in an academic setting using three concepts: everyday designer in an organisation, empathy through design and experiencing an awareness gap. These concepts were constructed through the analysis of in-depth interviews with the participants. The paper concludes with a call for tool support for everyday designers in organisations to enable them to better understand the audience for whom they are designing and the role technology plays in the organisation

The state of the Martian climate

60°N was +2.0°C, relative to the 1981–2010 average value (Fig. 5.1). This marks a new high for the record. The average annual surface air temperature (SAT) anomaly for 2016 for land stations north of starting in 1900, and is a significant increase over the previous highest value of +1.2°C, which was observed in 2007, 2011, and 2015. Average global annual temperatures also showed record values in 2015 and 2016. Currently, the Arctic is warming at more than twice the rate of lower latitudes