Recent progress in quantum Monte Carlo with modern nucleon-nucleon interactions have enabled the successful description of properties of light nuclei and neutron- rich matter. Of particular interest ...is the nuclear symmetry energy, the energy cost of creating an isospin asymmetry, and its connection to the structure of neutron stars. Combining these advances with recent observations of neutron star masses and radii gives insight into the equation of state of neutron-rich matter near and above the saturation density. In particular, neutron star radius measurements constrain the derivative of the symmetry energy.
A multinational outbreak of nosocomial fusarium meningitis occurred among immunocompetent patients who had undergone surgery with epidural anesthesia in Mexico. The pathogen involved had a high ...predilection for the brain stem and vertebrobasilar arterial system and was associated with high mortality from vessel injury. Effective treatment options remain limited; in vitro susceptibility testing of the organism suggested that it is resistant to all currently approved antifungal medications in the United States. To highlight the severe complications associated with fusarium infection acquired in this manner, we report data, clinical courses, and outcomes from 13 patients in the outbreak who presented with symptoms after a median delay of 39 days.
Summary
Hereditary angioedema with C1 inhibitor deficiency (C1‐INH‐HAE) is a rare autosomal dominant disease caused by mutations in the C1 inhibitor gene SERPING1. Phenotype and clinical features of ...the disease are extremely heterogeneous, varying even within the same family. Compared to HAE cohorts in other countries, the genetic background of the Swiss HAE patients has not yet been elucidated. In the present study we investigated the mutational spectrum of the SERPING1 gene in 19 patients of nine unrelated Swiss families. The families comprise a total of 111 HAE‐affected subjects which corresponds to approximately 70% of all HAE‐affected patients living in Switzerland. Three of the identified mutations are newly described. Members of family A with a nucleotide duplication as genetic background seem to have a more intense disease manifestation with a higher attack frequency compared to the other families. Newly designed genetic screening tests allow a fast and cost‐efficient testing for HAE in other family members.
Identified mutations in the SERPING1 gene
Minimal Cooling of Neutron Stars: A New Paradigm Page, Dany; Lattimer, James M; Prakash, Madappa ...
The Astrophysical journal. Supplement series,
12/2004, Letnik:
155, Številka:
2
Journal Article
Summary
The Swiss National Registry for Primary Immunodeficiency Disorders (PID) was established in 2008, constituting a nationwide network of paediatric and adult departments involved in the care of ...patients with PID at university medical centres, affiliated teaching hospitals and medical institutions. The registry collects anonymized clinical and genetic information on PID patients and is set up within the framework of the European database for PID, run by the European Society of Immunodeficiency Diseases. To date, a total of 348 patients are registered in Switzerland, indicating an estimated minimal prevalence of 4·2 patients per 100 000 inhabitants. Distribution of different PID categories, age and gender are similar to the European cohort of currently 19 091 registered patients: ‘predominantly antibody disorders’ are the most common diseases observed (n = 217/348, 62%), followed by ‘phagocytic disorders’ (n = 31/348, 9%). As expected, ‘predominantly antibody disorders’ are more prevalent in adults than in children (78 versus 31%). Within this category, ‘common variable immunodeficiency disorder’ (CVID) is the most prevalent PID (n = 98/217, 45%), followed by ‘other hypogammaglobulinaemias’ (i.e. a group of non‐classified hypogammaglobulinaemias) (n = 54/217, 25%). Among ‘phagocytic disorders’, ‘chronic granulomatous disease’ is the most prevalent PID (n = 27/31, 87%). The diagnostic delay between onset of symptoms and diagnosis is high, with a median of 6 years for CVID and more than 3 years for ‘other hypogammaglobulinaemias’.
In the ipsocentric formulation of the response of a molecular charge cloud to an external magnetic field, each point is taken as its own gauge origin. Orbital contributions to induced current density ...can then be defined in both localized and delocalized pictures of electronic structure, and the mapping of these contributions exposes a physical criterion for the localizability of orbitals. It is found that intrinsically localize d orbitals support local, closed-loop circulations, whereas attempts to impose localization on intrinsically de localize d orbitals yield unphysical, open-ended flowlines. This distinction is illustrated by current density maps for benzene, naphthalene, anthracene, tetracene, coronene, pyracylene, and borazine computed at the ab initio, coupled Hartree−Fock, CTOCD-DZ (continuous transformation of origin-diamagnetic zero) level. The computed maps recover the chemical intuition of a polycyclic aromatic hydrocarbon as comprising an intrinsically delocalized π-system atop a localized σ-valence system. When maximal localization is enforced, the local forms of π orbitals in the hydrocarbons all display open flowlines that build up into complex global patterns such as the counter-rotating ring currents in circulenes and the leaky perimeter circulations of the linear acenes. In contrast, the π system of borazine displays islands of closed circulation and is well represented by localized orbitals.
The objectives of this tutorial are as follows: 1) to help students and researchers develop a basic understanding of how pulsed-power systems are used to create high-energy-density (HED) matter; 2) ...to develop a basic understanding of a new, compact, and efficient pulsed-power technology called linear transformer drivers (LTDs); 3) to understand why LTDs are an attractive technology for driving HED physics (HEDP) experiments; 4) to contrast LTDs with the more traditional Marx-generator/pulse-forming-line approach to driving HEDP experiments; and 5) to briefly review the history of LTD technology as well as some of the LTD-driven HEDP research presently underway at universities and research laboratories across the globe. This invited tutorial is part of the Mini-Course on Charged Particle Beams and High-Powered Pulsed Sources, held in conjunction with the 44th International Conference on Plasma Science in May of 2017.
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