Background: Chronic intravenous immunoglobulin (IVIg) is used to treat refractory myasthenia gravis (MG). This subgroup analysis evaluated response to eculizumab in patients receiving chronic IVIg before entry to REGAIN, a phase 3, randomized, double-blind, placebo-controlled study of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalized MG. Methods: IVIg was only permitted during REGAIN as rescue therapy; previously treated patients underwent a 4-week washout before randomization. Patients included in this analysis had received chronic IVIg ≥4 times in 1 year, with ≥1 dose within 6 months before REGAIN entry. Exacerbations and MG status changes were assessed. Results: Eighteen patients were evaluated; four experienced exacerbations (eculizumab-treated, 1/9; placebo-treated, 3/9). Clinically relevant improvements were larger with eculizumab than placebo, respectively (mean change, standard deviation [SD]: MG Activities of Daily Living score [MG-ADL], -5.3 [4.0] vs -2.1 [2.8]; Quantitative MG score [QMG], -4.1 [6.1] vs -1.3 [3.5]). More patients receiving eculizumab (7/9) had clinically meaningful responses (MG-ADL ≥3 and/or QMG ≥5 points) than those receiving placebo (3/9). Eculizumab safety was consistent with previous reports. Interim data from the open-label extension of REGAIN will be presented. Conclusions: In patients previously receiving chronic IVIg, eculizumab showed a trend toward meaningful clinical improvements and fewer exacerbations compared with placebo. (NCT01997229, NCT02301624).

Background: Patients with anti-acetylcholine receptor antibody-positive (AChR+) generalized myasthenia gravis (MG) unresponsive to conventional treatment experience greater disease burden than responsive patients. This is partly due to exacerbations, which may result in significant healthcare resource utilization. Eculizumab is well tolerated and gives clinically meaningful benefits in these patients. We evaluated the effect of long-term eculizumab treatment on exacerbations, hospitalizations and rescue therapy in the REGAIN study and its open-label extension. Methods: Exacerbations were defined as clinical worsening/deterioration, MG crises or rescue therapy usage; pre-study exacerbations/hospitalizations were defined from patient records. Event rates adjusted for patient-years were calculated for all patients in the pre-study year, patients receiving placebo during REGAIN, and patients receiving eculizumab during REGAIN and its open-label extension (median exposure, 27.5 months [range, 22 days–42.8 months]); rates were compared using a Poisson regression model. Results: Eculizumab treatment reduced exacerbations by 65% (p=0.0057), hospitalizations by 71% (p=0.0316) and rescue therapy use by 66% (p=0.0072) versus placebo. Eculizumab treatment reduced exacerbations by 74% and hospitalizations by 83% (both p<0.0001) versus the pre-study year. Conclusions: Long-term eculizumab treatment reduces disease burden and healthcare resource utilization, demonstrating continuing improvements in clinical endpoints that lead to additional meaningful outcomes for patients with AChR+ generalized MG. (NCT01997229, NCT02301624).

Documenting past changes in the East Antarctic surface mass balance is important to improve ice core chronologies and to constrain the ice-sheet contribution to global mean sea-level change. Here we reconstruct past changes in the ratio of surface mass balance (SMB ratio) between the EPICA Dome C (EDC) and Dome Fuji (DF) East Antarctica ice core sites, based on a precise volcanic synchronization of the two ice cores and on corrections for the vertical thinning of layers. During the past 216 000 a, this SMB ratio, denoted SMBEDC/SMBDF, varied between 0.7 and 1.1, being small during cold periods and large during warm periods. Our results therefore reveal larger amplitudes of changes in SMB at EDC compared with DF, consistent with previous results showing larger amplitudes of changes in water stable isotopes and estimated surface temperature at EDC compared with DF. Within the last glacial inception (Marine Isotope Stages, MIS-5c and MIS-5d), the SMB ratio deviates by up to 0.2 from what is expected based on differences in water stable isotope records. Moreover, the SMB ratio is constant throughout the late parts of the current and last interglacial periods, despite contrasting isotopic trends.

We report a recent result of the FUGIN project, a Galactic plane CO survey using the Nobeyama 45-m Telescope and the FOREST receiver. In the third galactic quadrant, 42 square degrees are observed and 4752 molecular clouds are detected. Among them, 12 clouds are located at R (distance from the Galactic center) > 16 kpc. Molecular clouds at R < 16 kpc trace the Local, Perseus, and Outer arms.

Extended abstract of a paper presented at Microscopy and Microanalysis 2012 in Phoenix, Arizona, USA, July 29 – August 2, 2012.

The current trends in stimulated Brillouin scattering and optical phase conjugation are overviewed. This report is formed by the selected papers presented in the “Fifth International Workshop on stimulated Brillouin scattering and phase conjugation 2010” in Japan. The nonlinear properties of phase conjugation based on stimulated Brillouin scattering and photo-refraction can compensate phase distortions in the high power laser systems, and they will also open up potentially novel laser technologies, e.g., phase stabilization, beam combination, pulse compression, ultrafast pulse shaping, and arbitrary waveform generation.

Epitaxial Fe-Te-Se thin films were deposited by pulsed laser deposition at 250 ~ 600 °C on SrTiO3 (100, STO), MgO (100), LaAlO3 (100, LAO) and CaF2 (100) single crystal substrates. Best superconducting film was grown on CaF2: T c onset = 20.0 K and T c 0 = 16.18 K with T dep = 300 °C, 45000 pulses, 3 Hz. The critical current density J c at 4.2 K was 0.41×106A/cm2 at 0 T and 0.23×106 A/cm2 at 9 T. Angular dependence of J c showed broad c-axis correlated peak when B ≥ 3 T.

Extended abstract of a paper presented at Microscopy and Microanalysis 2011 in Nashville, Tennessee, USA, August 7–August 11, 2011.

Maternal food restriction is known to cause developmental hypertension in offspring. We have previously shown that maternal high-protein diet can reverse fetal programming of hypertension and that branched-chain amino acid (BCAA) concentrations in maternal and fetal plasma were increased by maternal high-protein intake. Then, we hypothesized that isocaloric supplementation with BCAA to a maternal food restriction can reverse the adverse outcome. Pregnant rats were divided into four groups at 7.5 days postcoitum: normally nourished (NN) and 70% undernourished (UN) groups with and without BCAA supplementation (NN–standard diet (SD), NN–BCAA, UN–SD and UN–BCAA groups). Compared with pups in the NN groups, those in the UN–SD group had significantly increased systolic blood pressure (SBP) at 8 and 16 weeks of age (P < 0.05). However, the elevation of SBP was not observed in offspring in the UN–BCAA group. Offspring glomeruli number of the UN groups was significantly lower (P < 0.05) than that of the NN groups, independent of BCAA supplementation. Angiotensin II receptor type 2 (ATR2) mRNA and protein expression in the kidney was significantly augmented in the UN–BCAA group at 30 weeks of age. In conclusion, BCAA supplementation during maternal food restriction prevents developmental hypertension together with increased ATR2 expression in adult offspring kidney.

In order to realize the magnetic refrigeration system, it is necessary to develop a 100 W class refrigerator with COP > 7.5. This requires us to find new magnetic refrigerant materials, of which cooling capacity is 2.5 times higher than that of Gd. In this paper, first we discuss the cooling capacity of magnetic refrigerant materials to achieve COP = 7.5. Then, we compare the experimental results of MnAsSb, MnFe(PGe) and La(FeCoSi)13 compounds with the calculated cooling capacity. It is suggested that a composite layer material of MnFe(PGe) would show excellent cooling capacity in the temperature span of 20 K.

La(Fe0.88Si0.12)13 shows peculiar magnetic properties such as the first order paramagnetic-ferromagnetic transition and magnetic-field induced metamagnetic transition accompanied by the lattice expansion. The practical application using the magnetic transition temperature controlled by hydrogen absorption is expected in this compound. Here, the electronic structure of La(Fe0.88Si0.12)13 has been investigated by photoemission spectroscopy using synchrotron soft x-rays. The Fe 3s core-level photoemission spectra below and above the Curie temperature TC exhibit a satellite structure at ~ 4.3 eV higher binding energy than the main peak, which is attributed to the exchange splitting due to the local moment of Fe. The exchange splitting of the Fe 3s photoemission spectrum with the asymmetric line shape shows that the magnetization of La(Fe0.88Si0.12)13 is derived by the exchange split Fe 3d bands like the itinerant ferromagnetism in Fe metal, while the magnetic transition of La(Fe0.88Si0.12)13 is the first order. The valence band photoemission spectrum shows temperature dependence across the TC . The temperature dependence of the photoemission spectra is discussed based on the difference between the electronic structure in the ferromagnetic phase and that in the paramagnetic phase.