Nephrotic Syndrome in Children: From Bench to Treatment

Idiopathic nephrotic syndrome (INS) is the most frequent form of NS in children. INS is defined by the association of the clinical features of NS with renal biopsy findings of minimal changes, focal segmental glomerulosclerosis (FSGS), or mesangial proliferation (MP) on light microscopy and effacement of foot processes on electron microscopy. Actually the podocyte has become the favourite candidate for constituting the main part of the glomerular filtration barrier. Most cases are steroid sensitive (SSINS). Fifty percents of the latter recur frequently and necessitate a prevention of relapses by nonsteroid drugs. On the contrary to SSINS, steroid resistant nephrotic syndrome (SRINS) leads often to end-stage renal failure. Thirty to forty percents of the latter are associated with mutations of genes coding for podocyte proteins. The rest is due to one or several different circulating factors. New strategies are in development to antagonize the effect of the latter

Neutron to proton ratios of quasiprojectile and midrapidity emission in the 64^{64}Zn + 64^{64}Zn reaction at 45 MeV/nucleon

Simultaneous measurement of both neutrons and charged particles emitted in the reaction $^{64}$Zn + $^{64}$Zn at 45 MeV/nucleon allows comparison of the neutron to proton ratio at midrapidity with that at projectile rapidity. The evolution of N/Z in both rapidity regimes with increasing centrality is examined. For the completely re-constructed midrapidity material one finds that the neutron-to-proton ratio is above that of the overall $^{64}$Zn + $^{64}$Zn system. In contrast, the re-constructed ratio for the quasiprojectile is below that of the overall system. This difference provides the most complete evidence to date of neutron enrichment of midrapidity nuclear matter at the expense of the quasiprojectile

Multiple-beam pulse shaping and preamplification

Glass fusion laser systems typically use a master oscillator-power amplifier (MOPA) architecture, where control of the optical pulse temporal and spatial parameters is accomplished mainly in the master oscillator and low power optics. The pulses from this low power ``front end`` are amplified in the power amplifier, which modifies the pulse shape temporally and spatially. Nonlinear frequency conversion crystals following the amplifier further change the pulse before it reaches the target. To effectively control the optical pulse on target for different types of experiments, and compensate for nonlinearity in the preceding optics, the front end system must be versatile enough to easily control many pulse parameters over a large range. The front end pulse generation system described in this article represents a new approach to this problem. The proposed National Ignition Facility (NIF) has 192 beamlines, each of which requires an input pulse of up to 12 Joules in around 4 ns equivalent square pulse length. Considerations of laser architecture for supplying each of these beamlines from a central oscillator system were crucial in the design of the front end. Previous lasers have used bulk optics to split a single oscillator signal and report beams to multiple amplifier chains. A key idea in the current design is to replace bulk optic transport with fibers, eliminating large opto-mechanical subsystems. Another important concept is convenient pulse forming using low voltage integrated optic modulators. The integrated optic and fiber optic concepts resulted in the current pulse generation designs for NEF. An important advantage is that each of the beamlines can have an independently controlled temporal pulse shape, which provides for precise balance of instantaneous power on target

Fragment Isospin as a Probe of Heavy-Ion Collisions

Isotope ratios of fragments produced at mid-rapidity in peripheral and central collisions of 114Cd ions with 92Mo and 98Mo target nuclei at E/A = 50 MeV are compared. Neutron-rich isotopes are preferentially produced in central collisions as compared to peripheral collisions. The influence of the size (A), density, N/Z, E*/A, and Eflow/A of the emitting source on the measured isotope ratios was explored by comparison with a statistical model (SMM). The mid-rapidity region associated with peripheral collisions does not appear to be neutron-enriched relative to central collisions.Comment: 12 pages including figure

Fragment Production in Non-central Collisions of Intermediate Energy Heavy Ions

The defining characteristics of fragment emission resulting from the non-central collision of 114Cd ions with 92Mo target nuclei at E/A = 50 MeV are presented. Charge correlations and average relative velocities for mid-velocity fragment emission exhibit significant differences when compared to standard statistical decay. These differences associated with similar velocity dissipation are indicative of the influence of the entrance channel dynamics on the fragment production process

Construction and commissioning of a technological prototype of a high-granularity semi-digital hadronic calorimeter

A large prototype of 1.3m3 was designed and built as a demonstrator of the semi-digital hadronic calorimeter (SDHCAL) concept proposed for the future ILC experiments. The prototype is a sampling hadronic calorimeter of 48 units. Each unit is built of an active layer made of 1m2 Glass Resistive Plate Chamber(GRPC) detector placed inside a cassette whose walls are made of stainless steel. The cassette contains also the electronics used to read out the GRPC detector. The lateral granularity of the active layer is provided by the electronics pick-up pads of 1cm2 each. The cassettes are inserted into a self-supporting mechanical structure built also of stainless steel plates which, with the cassettes walls, play the role of the absorber. The prototype was designed to be very compact and important efforts were made to minimize the number of services cables to optimize the efficiency of the Particle Flow Algorithm techniques to be used in the future ILC experiments. The different components of the SDHCAL prototype were studied individually and strict criteria were applied for the final selection of these components. Basic calibration procedures were performed after the prototype assembling. The prototype is the first of a series of new-generation detectors equipped with a power-pulsing mode intended to reduce the power consumption of this highly granular detector. A dedicated acquisition system was developed to deal with the output of more than 440000 electronics channels in both trigger and triggerless modes. After its completion in 2011, the prototype was commissioned using cosmic rays and particles beams at CERN.Comment: 49 pages, 41 figure

The porin and the permeating antibiotic: A selective diffusion barrier in gram-negative bacteria

Gram-negative bacteria are responsible for a large proportion of antibiotic resistant bacterial diseases. These bacteria have a complex cell envelope that comprises an outer membrane and an inner membrane that delimit the periplasm. The outer membrane contains various protein channels, called porins, which are involved in the influx of various compounds, including several classes of antibiotics. Bacterial adaptation to reduce influx through porins is an increasing problem worldwide that contributes, together with efflux systems, to the emergence and dissemination of antibiotic resistance. An exciting challenge is to decipher the genetic and molecular basis of membrane impermeability as a bacterial resistance mechanism. This Review outlines the bacterial response towards antibiotic stress on altered membrane permeability and discusses recent advances in molecular approaches that are improving our knowledge of the physico-chemical parameters that govern the translocation of antibiotics through porin channel