BACKGROUND AND METHODS: Growth failure is characteristic of untreated mucopolysaccharidosis type VI (MPS VI: Maroteaux-Lamy syndrome). Growth was studied in fifty-six MPS VI patients (5 to 29 years old) prior to and for up to 240 weeks of weekly infusions of recombinant human arylsulfatase B (rhASB) at 1 mg/kg during Phase 1/2, Phase 2, Phase 3 or Phase 3 Extension clinical trials. Height, weig...

In order to study the influence of wind shear on aerodynamic characteristics large turbines, taking 5MW turbine blade model published by NREL as research object, eddy simulation (LES) turbines was carried out using XFlow fluid software based Lattice-Boltzmann method (LBM). WALE turbulence used at 3, 11.2 and 25m/s speeds. The effect factors axial thrust torque is compared with data NREL. result...

* corresponding author. e-mail: [email protected] The interaction of Mo(VI) and tetrazolium violet (TV) has been examined. The ion-associate formed is extracted into 1,2-dichloroethane. The tetrazolium violet : molybdenum(VI) : 3,4,5-trihydroxybenzoic acid ratio (R) is 2 : 1 : 2. The optimum conditions have been established. Beer’s law is obeyed in the range of 0.2–5 μg ml–1 molybdenum(VI...

Understanding the lattice dynamics and low thermal conductivities of IV-VI, V2-VI3 and V materials is critical to the development of better thermoelectric and phase-change materials. Here we provide a link between chemical bonding and low thermal conductivity. Our first-principles calculations reveal that long-ranged interaction along the 〈100〉 direction of the rocksalt structure exist in lead ...

Introduction IV-VI compounds are regarded as average group V material. In crystalline group V elements and IV-VI compounds, the Peierls distortion occurs. Strong bonds with short bond length and relatively weak bonds with long bond length coexist in the crystal. Therefore anisotropic contraction of the local structure is expected under pressure [1]. In crystalline SnSe, atoms form two dimension...

Explorer. FUSE is operated for NASA by the Johns Hopkins University under NASA contract NAS5-3298. ABSTRACT A new ephemeris has been determined for the supersoft X-ray binary CAL 83 using MACHO photometry. With an improved orbital period of 1.047568 days, it is now possible to phase together photometric and spectroscopic data obtained over the past two decades with new far ultraviolet spectra t...