Cutis Laxa syndrome: a case report

Cutis laxa (CL) is a heterogeneous group of inherited and acquired connective tissue disorders characterized by a loose skin and variable systemic involvement (inguinal hernia, cardiopulmonary disease, and emphysema). Autosomal dominant, autosomal recessive and x-linked recessive patterns have been described in the inherited forms. Acquired forms of this disease have been associated with a previous inflammatory skin disorder (urticaria...). The characteristic symptomatological pattern is resulting from paucity of elastic fibers. We report an 18 months old baby boy with a congenital cutis laxa. He was admitted in pediatric unit for respiratory disorders. The diagnosis of CL syndrome is based on clinical assessment of typical skin features and the associated extracutaneous finding

Left Ventricle Diverticulum with Partial Cantrell's Syndrome

Cantrell syndrome is a very rare congenital disease associating five features: a midline, upper abdominal wall disorder, lower sternal abnormality, anterior diaphragmatic defect, diaphragmatic pericardial abnormality, and congenital abnormalities of the heart. In this paper, we report a case of partial Cantrell's syndrome with left ventricular diverticulum, triatrial situs solitus, ventricular septal defect, dextrorotation of the heart, an anterior pericardial diaphragmatic defect, and a midline supraumbilical abdominal wall defect with umbilical hernia. The 5-month-old patient underwent a successful cardiac surgical procedure. A PTFE membrane was placed on the apex of the heart to facilitate reopening of the patient’s chest. Postoperative course was uneventful. The patient was discharged with good clinical condition and with a normal cardiac function

Transport of Water and Gases through EVA/PVC blend films – Permeation and DSC investigations.

The transport of water vapor and gases (oxygen and carbon dioxide) through poly(ethylene-co-vinyl acetate) (EVA) films of different VA content, poly(vinylchloride) (PVC) and EVA/PVC blend films, was analysed from permeation measurements. A plasticization effect of water on the material was observed for EVA films with more than 19% wt. of VA content and for the EVA/PVC blends, while for gas permeation practically all the experimental curves are characterized by a constant diffusion coefficient, whatever the VA content of the copolymer used. The increase in water absorption with the VA content leads to a steady increase in the water permeability of the EVA copolymers. By mixing the glassy PVC polymer with the EVA copolymer (in a rubbery state) reduced water and gas permeability is observed, resulting mainly from the decrease of the diffusivity due to the low segment mobility of the dense PVC material able to create hydrogen bonds between the hydrogen atoms and the Cl-substituted carbon of PVC with VA carbonyls. Compared to EVA copolymers, the EVA/PVC blends with equivalent VA contents are better in terms of selectivity

Synthesis and coordination chemistry of 2-(di-2-pyridylamino)pyrimidine; structural aspects of spin crossover in an Fe(II) complex

This paper was accepted on February 26 20122-(Di-2-pyridylamino)pyrimidine (L), a potentially ditopic tetradentate ligand, was synthesized from commercially available di-2-pyridylamine and 2-chloropyrimidine. Despite being capable of bridging two metal atoms with bidentate chelation of both metal centres, L prefers to chelate or bridge through the more basic pyridyl donors of the di-2-pyridylamine moiety. Mononuclear trans-[Fe(NCS)2(L)2] and [Cu(L)2(H2O)](BF4)2•H2O complexes, and a discrete [Ag2(L)4](PF6)2 metallomacrocycle were isolated and structurally characterized by X-ray crystallography. A mononuclear palladium complex [PdCl2(L)]•(solvate), where solvate = ½H2O or CH2Cl2, was also readily obtained in 71% yield. One example of the ligand acting as a bis(bidentate) bridging ligand was observed in a dinuclear [(PdCl2)2(L)]•¾H2O complex that was obtained only in very low yield (ca. 3%) from the reaction that produced [PdCl2(L)]•½H2O. trans-[Fe(NCS)2(L)2] undergoes a temperature dependent HS-LS (HS = high spin; LS = low spin) crossover at ca. 205 K that was 2 observed by X-ray crystallography and magnetic measurements and attempts were made to understand the structural basis of this process. Despite efforts to isolate examples of L bridging two iron(II) centres, only the mononuclear trans-[Fe(NCS)2(L)2] species could be obtained.Rachel S. Crees, Boujemma Moubaraki, Keith S. Murray, and Christopher J. Sumb

Interpreting Reactor Antineutrino Anomalies with STEREO data

Anomalies in past neutrino measurements have led to the discovery that these particles have non-zero mass and oscillate between their three flavors when they propagate. In the 2010's, similar anomalies observed in the antineutrino spectra emitted by nuclear reactors have triggered the hypothesis of the existence of a supplementary neutrino state that would be sterile i.e. not interacting via the weak interaction. The STEREO experiment was designed to study this scientific case that would potentially extend the Standard Model of Particle Physics. Here we present a complete study based on our full set of data with significantly improved sensitivity. Installed at the ILL (Institut Laue Langevin) research reactor, STEREO has accurately measured the antineutrino energy spectrum associated to the fission of 235U. This measurement confirms the anomalies whereas, thanks to the segmentation of the STEREO detector and its very short mean distance to the core (10~m), the same data reject the hypothesis of a light sterile neutrino. Such a direct measurement of the antineutrino energy spectrum suggests instead that biases in the nuclear experimental data used for the predictions are at the origin of the anomalies. Our result supports the neutrino content of the Standard Model and establishes a new reference for the 235U antineutrino energy spectrum. We anticipate that this result will allow to progress towards finer tests of the fundamental properties of neutrinos but also to benchmark models and nuclear data of interest for reactor physics and for observations of astrophysical or geo-neutrinos.Comment: 21 pages, 13 figure

Measurement of the cross-section and charge asymmetry of WW bosons produced in proton-proton collisions at s=8\sqrt{s}=8 TeV with the ATLAS detector

This paper presents measurements of the $W^+ \rightarrow \mu^+\nu$ and $W^- \rightarrow \mu^-\nu$ cross-sections and the associated charge asymmetry as a function of the absolute pseudorapidity of the decay muon. The data were collected in proton--proton collisions at a centre-of-mass energy of 8 TeV with the ATLAS experiment at the LHC and correspond to a total integrated luminosity of 20.2~\mbox{fb^{-1}}. The precision of the cross-section measurements varies between 0.8% to 1.5% as a function of the pseudorapidity, excluding the 1.9% uncertainty on the integrated luminosity. The charge asymmetry is measured with an uncertainty between 0.002 and 0.003. The results are compared with predictions based on next-to-next-to-leading-order calculations with various parton distribution functions and have the sensitivity to discriminate between them.Comment: 38 pages in total, author list starting page 22, 5 figures, 4 tables, submitted to EPJC. All figures including auxiliary figures are available at https://atlas.web.cern.ch/Atlas/GROUPS/PHYSICS/PAPERS/STDM-2017-13

Search for chargino-neutralino production with mass splittings near the electroweak scale in three-lepton final states in √s=13 TeV pp collisions with the ATLAS detector

A search for supersymmetry through the pair production of electroweakinos with mass splittings near the electroweak scale and decaying via on-shell W and Z bosons is presented for a three-lepton final state. The analyzed proton-proton collision data taken at a center-of-mass energy of √s=13  TeV were collected between 2015 and 2018 by the ATLAS experiment at the Large Hadron Collider, corresponding to an integrated luminosity of 139  fb−1. A search, emulating the recursive jigsaw reconstruction technique with easily reproducible laboratory-frame variables, is performed. The two excesses observed in the 2015–2016 data recursive jigsaw analysis in the low-mass three-lepton phase space are reproduced. Results with the full data set are in agreement with the Standard Model expectations. They are interpreted to set exclusion limits at the 95% confidence level on simplified models of chargino-neutralino pair production for masses up to 345 GeV

Search for direct stau production in events with two hadronic tau-leptons in root s=13 TeV pp collisions with the ATLAS detector

A search for the direct production of the supersymmetric partners ofτ-leptons (staus) in final stateswith two hadronically decayingτ-leptons is presented. The analysis uses a dataset of pp collisions corresponding to an integrated luminosity of139fb−1, recorded with the ATLAS detector at the LargeHadron Collider at a center-of-mass energy of 13 TeV. No significant deviation from the expected StandardModel background is observed. Limits are derived in scenarios of direct production of stau pairs with eachstau decaying into the stable lightest neutralino and oneτ-lepton in simplified models where the two staumass eigenstates are degenerate. Stau masses from 120 GeV to 390 GeV are excluded at 95% confidencelevel for a massless lightest neutralino