Centrality Dependence Of The Pseudorapidity Density Distribution For Charged Particles In Pb-pb Collisions At √snn=2.76tev

7264/Mai61062

Review of clinical presentation and diagnosis of mucopolysaccharidosis IVA

Mucopolysaccharidosis type IVA (MPS IVA) was described in 1929 by Luis Morquio from Uruguay and James Brailsford from England, and was later found as an autosomal recessive lysosomal storage disease. MPS IVA is caused by mutations in the gene encoding the enzyme, N-acetylgalactosamine-6-sulfate sulfatase (GALNS). Reduced GALNS activity results in impaired catabolism of two glycosaminoglycans (GAGs), chondroitin-6-sulfate (C6S) and keratan sulfate (KS). Clinical presentations of MPS IVA reflect a spectrum of progression from a severe ”classical” phenotype to a mild “attenuated” phenotype. More than 180 different mutations have been identified in the GALNS gene, which likely explains the phenotypic heterogeneity of the disorder. Accumulation of C6S and KS manifests predominantly as short stature and skeletal dysplasia (dysostosis multiplex), including atlantoaxial instability and cervical cord compression. However, abnormalities in the visual, auditory, cardiovascular, and respiratory systems can also affect individuals with MPS IVA. Diagnosis is typically based on clinical examination, skeletal radiographs, urinary GAG, and enzymatic activity of GALNS in blood cells or fibroblasts. Deficiency of GALNS activity is a common assessment for the laboratory diagnosis of MPS IVA; however, with recently increased availability, gene sequencing for MPS IVA is often used to confirm enzyme results. As multiple clinical presentations are observed, diagnosis of MPS IVA may require multi-system considerations. This review provides a history of defining MPS IVA and how the understanding of the disease manifestations has changed over time. A summary of the accumulated knowledge is presented, including information from the International Morquio Registry. The classical phenotype is contrasted with attenuated cases, which are now being recognized and diagnosed more frequently. Laboratory based diagnoses of MPS IVA are also discussed

Constraining the Neutron Star Mass–Radius Relation and Dense Matter Equation of State with NICERNICER. I. The Millisecond Pulsar X-Ray Data Set

International audienceWe present the set of deep Neutron Star Interior Composition Explorer (NICER) X-ray timing observations of the nearby rotation-powered millisecond pulsars PSRs J0437−4715, J0030+0451, J1231−1411, and J2124−3358, selected as targets for constraining the mass–radius relation of neutron stars and the dense matter equation of state (EoS) via modeling of their pulsed thermal X-ray emission. We describe the instrument, observations, and data processing/reduction procedures, as well as the series of investigations conducted to ensure that the properties of the data sets are suitable for parameter estimation analyses to produce reliable constraints on the neutron star mass–radius relation and the dense matter EoS. We find that the long-term timing and flux behavior and the Fourier-domain properties of the event data do not exhibit any anomalies that could adversely affect the intended measurements. From phase-selected spectroscopy, we find that emission from the individual pulse peaks is well described by a single-temperature hydrogen atmosphere spectrum, with the exception of PSR J0437−4715, for which multiple temperatures are required

Performance Of The Alice Experiment At The Cern Lhc

292

Production of Sigma (1385)(+/-) and Xi (1530)(0) in proton-proton collisions at root s=7 TeV

The production of the strange and double-strange baryon resonances (Sigma (1385)(+/-), Xi (1530)(0)) has been measured at mid-rapidity (vertical bar y vertical bar < 0.5) in proton-proton collisions at root s = 7 TeV with the ALICE detector at the LHC. Transverse momentum spectra for inelastic collisions are compared to QCD-inspired models, which in general underpredict the data. A search for the phi (1860) pentaquark, decaying in the Xi pi channel, has been carried out but no evidence is seen

Measurement Of The Cross Section For Electromagnetic Dissociation With Neutron Emission In Pb-pb Collisions At √snn=2.76 Tev

1092

Measurement of visible cross sections in proton-lead collisions at root s(NN)=5.02 TeV in van der Meer scans with the ALICE detector

In 2013, the Large Hadron Collider provided proton-lead and lead-proton collisions at the center-of-mass energy per nucleon pair root s(NN) = 5.02 TeV. Van der Meer scans were performed for both configurations of colliding beams, and the cross section was measured for two reference processes, based on particle detection by the T0 and V0 detectors, with pseudo-rapidity coverage 4.6 < eta < 4.9, -3.3 < eta < -3.0 and 2.8 < eta < 5.1, -3.7 < eta < -1.7, respectively. Given the asymmetric detector acceptance, the cross section was measured separately for the two configurations. The measured visible cross sections are used to calculate the integrated luminosity of the proton-lead and lead-proton data samples, and to indirectly measure the cross section for a third, configuration-independent, reference process, based on neutron detection by the Zero Degree Calorimeters