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This article presents long-term data on the efficacy of gene replacement therapy (GRT) for spinal muscular atrophy (SMA) using onasemnogene abeparvovec (OA). The study includes patients who were older, heavier, or more severely affected than those in previous clinical trials, as well as a large cohort of presymptomatic SMA patients identified through newborn screening. The results show that younger patients treated with OA had better motor function outcomes compared to those treated at a later age. The study provides valuable evidence for future treatment decisions, particularly for patients with limited trial data available. [Extracted from the article]

This article, published in the journal Neuropediatrics, discusses a case of ocular myasthenia, a common presentation of myasthenia gravis (MG) in children. The case presented challenges in diagnosis and treatment, requiring specialized laboratory tests and high doses of pyridostigmine. The patient initially had right-sided ptosis and diplopia, which worsened and showed rapid alteration. Despite negative ACh-receptor antibodies and normal CSF, MRI, and electrophysiology results, the patient was successfully treated with pyridostigmine. The article highlights the importance of live cell-based assays in confirming the diagnosis of ocular MG when other tests are negative, and suggests that higher doses of pyridostigmine may be necessary for successful treatment. [Extracted from the article]

Fe/MgO/Fe single-crystalline magnetotunneling structures were epitaxially grown on GaAs(001). An independent magnetization switching in the Fe electrodes was achieved by pinning the magnetization of the Fe-top electrode by antiferromagnetic coupling across a Cr spacer to another Fe film. Such Fe/Cr/Fe/MgO/Fe/GaAs(001) structures exhibit in-plane anisotropy, which ensures a discrete switching behavior of magnetization simplifying the shape of the hysteresis loop. The field dependence of magnetization was measured along the [110] and [100] crystallographic directions of GaAs(001) substrate and modeled by minimizing the total magnetic energy of the system. A peculiar magneto-optical behavior (similar to a reversed hysteresis loop) is explained by magneto-optical phase angles, which are determined mainly by the optical properties of the semiconducting GaAs substrate. [ABSTRACT FROM AUTHOR]

We have grown Fe films on an epitaxial Pd monolayer on Cu(001) single crystals at room temperature, both Fe and Pd by pulsed laser deposition. The presence of the Pd interlayer influences growth, structure, and magnetism of the Fe films. Up to the thickness of 1.6 ML the Fe films show an out-of-plane easy axis of magnetization which changes to in-plane above. A linear dependence of the longitudinal Kerr rotation on the Fe thickness is obtained despite existing structural transformations. A strong increase of the Curie temperature TC is observed for the Fe films on Pd/Cu(001) compared to the Fe films grown directly on Cu(001). A correlation of the magnetic properties with possible structural changes and the role of Pd monolayer is discussed. [ABSTRACT FROM AUTHOR]

Thermal deposition (TD) and pulsed laser deposition (PLD) techniques were used to prepare GaAs/Fe(001) crystalline ultrathin films. The magnetic environment of Fe at the Fe/GaAs(001) interface was investigated by means of conversion electron Mössbauer spectroscopy using an 57Fe probe layer having the equivalent thickness of two atomic layers. The magnetic anisotropies were measured using ferromagnetic resonance. PLD samples were affected by an appreciably higher degree of interface alloying compared with the TD films. The interface in-plane fourfold (cubic) anisotropy is significantly increased and the in-plane interface uniaxial anisotropy is significantly decreased in the PLD samples compared to those prepared using TD. The effective perpendicular demagnetizing field in the PLD films is significantly decreased compared to the TD samples due to an unexpected increase in the interface uniaxial perpendicular anisotropy at the Fe/Au(001) interface. [ABSTRACT FROM AUTHOR]

The magnetization states of 20-nm-thick rectangular Co thin-film elements are studied with micromagnetic modeling and x-ray magnetic circular dichroism photoemission electron microscopy. The energies of ten domain configurations obtained in the modeling are compared with the frequency of occurrence of the corresponding virgin domain structures as a function of aspect ratio from 1:1 to 1:3 and of width from 200 to 600 nm. The results show that the abundance of the virgin states is largely determined by the magnetic energy densities of the elements. [ABSTRACT FROM AUTHOR]

We show that for some ultrathin multilayer structures the proportionality of the longitudinal Kerr ellipticity signal to the magnetic film thickness does not take place. Instead, thinner magnetic layers give even twice larger contributions than the thicker ones located a few monolayers deeper. This effect is demonstrated both experimentally and theoretically on ultrathin Fe/Cr/Fe sandwich systems prepared on GaAs(001) surfaces. Such a behavior is explained by a depth variation of phase relations between the optically reflected wave and the wave generated by the magneto-optical interaction. This mechanism becomes very effective close to the phase where the magneto-optical ellipticity cancels. [ABSTRACT FROM AUTHOR]

We have performed magnetic domain imaging with spatial, temporal, and layer resolution using x-ray photoelectron emission microscopy. The element selectivity of x-ray magnetic circular dichroism allows the magnetization dynamics of the different magnetic layers in spin-valve-like FeNi/Cu/Co trilayers to be studied separately, using the time structure of synchrotron radiation. The unique possibilities of this technique have been used to study the influence of the intrinsic magnetic properties of the different layers on the magnetization dynamics and the interlayer magnetic coupling. © 2004 American Institute of Physics. [ABSTRACT FROM AUTHOR]

We demonstrate, that spin-polarized electron energy loss spectroscopy is a unique tool to determine spin-wave dispersion curves of ultrathin films and surfaces over the whole Brillouin zone. For ultrathin Co films on Cu(001) the excitation of a spin wave appears as a well defined peak in the loss spectrum. 3 ML Fe films on 1 ML Co/Cu(001) films show spin-wave excitation peaks as well though much broader compared to the Co case. The spin-wave energy at the surface zone boundary of about 140 meV for the Fe film is nearly a factor of 2 smaller than that of the Co films. © 2004 American Institute of Physics. [ABSTRACT FROM AUTHOR]

Fe/Cr/Fe trilayers were epitaxially grown on atomically flat GaAs(001). For the thickness of Cr spacer layer corresponding to antiferromagnetic coupling, “reversed” minor hysteresis loops were measured with longitudinal magneto-optical Kerr effect (MOKE), i.e., a negative “magnetization” signal was detected when the thicker bottom Fe layer was saturated along the applied field. This behavior is interpreted by depth variations of the MOKE sensitivity. Magnetization reversal shows that both antiferromagnetic switching and spin–flop transition fields depend on the ratio of both Fe film thicknesses. The shape of the MOKE loops becomes more complex with further deposition of MgO and Fe layers on the top of the Fe/Cr/F/GaAs(001) stack. Superconducting quantum interference device measurements confirm the interpretation of the MOKE loops and demonstrate homogeneity and sharpness of the interfaces in the structures. © 2004 American Institute of Physics. [ABSTRACT FROM AUTHOR]

Presents information on a study which demonstrated high-resolution imaging of domains and domain walls in the scanning electron microscope. Imaging of surface domains and domain walls; Discussion of stoner excitations in itinerant ferromagnets; Examination of spin-polarized photoemission from thin films.

Studies the diffusion of Fe, Co and Ni from a dot deposited onto a W(110) substrate. Analysis of spread directions for all three metals to <100>; Influence of step directions on the substrate surface; Inducing of spreading anisotropy by the cap layer; Formation of a structure during the spreading process.

Tetragonal distortion in Fe0.5Co0.5 alloy films grown epitaxially on Rh(001) substrates results in an easy magnetization axis perpendicular to the film plane up to the thickness of 17 ML. The distortion is supported by a Rh-overlayer; thus the strong perpendicular anisotropy can be kept when another Fe0.5Co0.5 film is grown on top of the Rh/Fe0.5Co0.5/Rh(001) structure. Depending on the thickness of the Rh spacer, the top and bottom Fe0.5Co0.5 films are either ferro- or antiferromagnetically coupled. The net magnetization of the antiferromagnetically coupled Fe0.5Co0.5/Rh/Fe0.5Co0.5 bilayer switches at the field which depends on the difference between magnetizations of both the Fe0.5Co0.5 layers. The final covering of the Fe0.5Co0.5/Rh/Fe0.5Co0.5/Rh(001) structure with Rh increases the switching field. The effect is explained by a locally enhanced magnetization in the Rh/Fe1-xCox interfaces. [ABSTRACT FROM AUTHOR]

In tetragonally distorted Fe0.5Co0.5 alloy films grown epitaxially on Pd(001), Ir(001), and Rh(001) substrates the crystal field locates the electronic states near the Fermi level (EF) with one being below EF and the other above EF with an energy separation smaller than in bulk nondistorted material. This results in a strong uniaxial anisotropy and an easy magnetization axis perpendicular to the film plane up to the thickness up to which the films remain tetragonally distorted. The strongest perpendicular anisotropy is achieved when the Fe0.5Co0.5 films are grown on Rh(001) (c/a=1.24); it systematically decreases for Ir(001) (c/a=1.18) and Pd(001) (c/a=1.13) substrates. The phenomenon can be understood as a result of an increasing uniaxial anisotropy with an increasing c/a ratio up to the maximum at c/a=1.24 for which the maximum uniaxial magnetic anisotropy has been theoretically predicted. [ABSTRACT FROM AUTHOR]

Copyright of Monatsschrift Kinderheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Der Nervenarzt is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract: The particle size of polymer colloids is a key characteristic. It directly affects Raman measurements due to light scattering by particles. This publication exploits the extent to which these spectral changes can be correlated to the particle sizes by using a spectral hard model and data‐driven model. To this end, spectra of aqueous polystyrene nanoparticles are decomposed into pure component spectral models by means of indirect hard modeling (IHM). Each pure component model is characterized by multiple peak‐shaped Voigt profiles. By fitting the model to spectra of polymer colloids, spectral changes due to light scattering by particles are incorporated in the model parameters. The resulting parameter values are used as input for data‐driven partial least squares (PLS) regression to extract particle sizes. The method is applied to Raman spectra of polystyrene nanoparticles of 23–60 nm diameter measured repeatedly at concentrations 0.17–1 wt%. The hybrid model predicts the particle size with R2 = 0.99. In contrast, purely data‐driven PLS regression of the spectral intensities with 3 latent PLS variables results in R2 from 0.78 to 0.83. Resulting PLS scores portray a clear differentiability of Raman scattering and light scattering by particles within IHM model parameters. PLS regression coefficients further allow identification of individual peak parameters that correlate with particle size, which substantiates previous findings on correlation between Raman signal and scattering by polymer colloids. Combining IHM and data‐driven PLS regression demonstrates more accurate prediction of particle size for extended usage of Raman spectroscopy as comprehensive process analytical technology to determine sample concentrations and particle data. [ABSTRACT FROM AUTHOR]

Fe/MgO/Fe/Cr/Fe tunneling structures were epitaxially grown directly on GaAs(001) without buffer layer. Interdiffusion in the Fe/GaAs interface was investigated using conversion electron Mössbauer spectroscopy. An independent magnetization switching in the Fe electrodes was achieved by pinning the magnetization of the Fe-top electrode by antiferromagnetic coupling across a Cr spacer to another Fe film. For transport measurements, optical lithography and ion etching were applied to reduce the junction area. A tunneling magnetoresistance of 10%–12% at room temperature was found. [ABSTRACT FROM AUTHOR]

The magnetic domain patterns of epitaxial single-crystalline Co/FeMn bilayers and Co/Cu/FeMn trilayers were investigated by magnetic circular dichroism domain imaging using photoelectron emission microscopy. The as-grown domain size increases continuously with increasing Cu layer thickness, which is attributed to the decrease of the interlayer exchange coupling between ferromagnetic Co and antiferromagnetic FeMn layers. Domain images of the Co layer acquired after applying different external magnetic fields show a decrease in coercivity with increasing Cu layer thickness, confirming the reduction of magnetic coupling energy with increasing Cu thickness. © 2004 American Institute of Physics. [ABSTRACT FROM AUTHOR]

We have studied the magnetic states of self-organized Fe islands on W(001) by means of spin-polarized scanning tunneling spectroscopy (Sp-STS). Single-domain and vortex states in the nanoscale islands have been observed. The high-resolution magnetic images enable to experimentally determine the boundary between the single-domain and vortex states. The single-domain state was always found below a thickness of 6 nm and a diameter of 120 nm in Fe islands. The boundary directly observed with Sp-STS is consistent with theoretical predictions. © 2004 American Institute of Physics. [ABSTRACT FROM AUTHOR]

Magnetization as a function of temperature calculated with Monte Carlo techniques is compared to experimental results of Fe stripes grown on vicinal Cu(111) surfaces. The stripes are step decorations grown with molecular beam epitaxy (MBE), are 1-2 monolayers thick, and display perpendicular magnetization. The atomic parameters are determined from fully relativistic electronic structure calculations. The moments are found to be 2.57&micron;[sub B], with some variation due to film thickness, and uniaxial anisotropy of 40 &micron;Ry/atom for Fe atoms facing vacuum. The Heisenberg model extended to include crystalline anisotropy as well as dipole-dipole interactions is considered for two different values of the exchange constant: J=20 and 2 meV. Under a large applied field (4000 G), the calculated saturation magnetization falls slowly from 507 emu/cm[sup 3] with an increase in temperature until it falls rapidly around 600 K, after which a more modest falloff with an increase in temperature is observed. For larger d the rapid change occurs for higher temperatures. The importance of disorder in the height and width of the stripes is investigated by generating stripe geometries with a model that incorporates nucleation and growth of Fe particles at step edges under the constraint of constant deposition from MBE. The primary effect of disorder in the stripes is to reduce the saturated magnetization at lower temperatures. [ABSTRACT FROM AUTHOR]

The electron transport and magnetoresistance (MR) were investigated in high quality crystalline epitaxial Fe(001) and Au(001) films and exchange coupled Au/Fe/Au/Fe/GaAs(001) trilayer structures. Fits to the experimental data were based on the semiclassical Boltzmann equation, which incorporates the electronic properties obtained from first-principles local density functional calculations. The fits require a surprisingly high asymmetry for the spin dependent electron lifetimes in Fe, τ[sup ↓]/τ[sup ↑]=10 at room temperature. Despite the large atomic terraces at the Au/vacuum and Fe/GaAs interfaces the scattering at the outer interfaces was found to be diffuse. The origin of MR in Au/Fe/Au/Fe/GaAs(001) structures is due to electron channeling in the Au spacer layer. The measured MR is consistent with the diffusivity parameters s[sup ↑]=0.55, s[sup ↓]=0.77 at the metal-metal interfaces. © 2001 American Institute of Physics. [ABSTRACT FROM AUTHOR]

The giant magnetoresistance of crystalline Fe/Cu/Fe(001) epitaxial structures characterized by scanning tunneling microscopy are presented. Fe/Cu/Fe(001) trilayers capped with Au are grown directly on GaAs(001) using a new procedure for producing pure Fe layers with As-free Fe surfaces on GaAs(001). The temperature dependence of the magnetoconductance and sheet resistance measured from 4 to 300 K is modeled by the Boltzmann equation assuming that the mean free paths in the crystalline epitaxial layers are equal to those in bulk materials. The results of the simple model suggest that the coefficient of the specular scattering at the Fe/GaAs interface is R=0.45, while the scattering at the outer Au interface is diffuse. Spin asymmetry scattering at the metallic interfaces is ΔT=|T[sup ↑]-T[sup ↓]|=0.34, T[sup avg]=(T[sup ↑]+T[sup ↓])/2=0.79. The sheet resistance was best modeled using a low temperature mean free path of 25 nm in the Fe layer. © 2000 American Institute of Physics. [ABSTRACT FROM AUTHOR]

Photoelectron emission microscopy is combined with soft x-ray magnetic circular dichroism (XMCD) absorption spectroscopy to obtain local element-resolved quantitative magnetic properties with microscopic resolution. This is applied to study 0–14 ML Fe wedges with a slope of 0.055 ML/μm on 6 ML Co/Cu(001). Local XMCD spectra at the Fe L[sub 2,3] edge confirm the presence of three magnetically different thickness regions of Fe with effective spin moments of 2.5μ[sub B] (0–4.5 ML), 0.7μ[sub B] (4.5–11 ML), and 1.8μ[sub B] (>11 ML). The value of 0.7μ[sub B] in the second phase is consistent with an fcc Fe phase containing nonferromagnetic layers underneath a ferromagnetic surface. © 2000 American Institute of Physics. [ABSTRACT FROM AUTHOR]

The correlation between mechanical stress and magnetic anisotropy of Ni and Fe films on Cu(001) is investigated. The magnetoelastic coupling and the film stress during the growth are measured in situ with a highly sensitive optical bending beam technique. For Ni a dramatically reduced magnetoelastic coupling of B[sub 1]=3.5 MJ/m[sup 3] is found for films thinner than 10 ML, roughly one third of the bulk value of 9.4 MJ/m3. This change is explained by a strain correction to the magnetoelastic coupling. The influence of the interfaces does not significantly contribute to the magnetoelastic coupling. A very small magnetoelastic coupling of 0.4 MJ/m3 for Fe films in the range from 12 ML to 25 nm is attributed mainly to the crystallographic orientation of the bcc–Fe. © 2000 American Institute of Physics. [ABSTRACT FROM AUTHOR]

We present static measurements of the domain structure and dynamic results on domain wall motion and local susceptibility obtained by spin-polarized scanning tunneling microscopy. The topography and the magnetic structure of the sample are recorded simultaneously with down to 10 nm resolution. With this technique, domain wall movement on Co(0001) is studied in situ. In some cases, the magnetization of the sample is locally influenced by the stray field of the tip. Measuring higher harmonics in the tunneling current allows one to quantify this influence and measure magnetic susceptibilities on similar scales. © 2000 American Institute of Physics. [ABSTRACT FROM AUTHOR]

Localized electrochemical deposition of small Co clusters on Au (111) has been achieved by using the tip of a scanning tunneling microscope (STM) as an electrochemical nanoelectrode. In contrast to most of the reported techniques to create nanostructures, this approach avoids irreversible modifications of both substrate and deposit during the preparation process. A special polarization routine of the STM tip allows a purely electrochemical growth of Co clusters with diameters as small as 15 nm. © 2000 American Institute of Physics. [ABSTRACT FROM AUTHOR]

The quality of chemical imaging, especially multisensor hyperspectral imaging, strongly depends on sample preparation techniques and instrumental infrastructure but also on the choice of an appropriate imaging substrate. To optimize the combined imaging of Raman microspectroscopy, scanning-electron microscopy and energy-dispersive X-ray spectroscopy, a novel substrate was developed based on sputtering of highly purified aluminium onto classical microscope slides. The novel aluminium substrate overcomes several disadvantages of classical substrates like impurities of the substrate material and contamination of the surface as well as surface roughness and homogeneity. Therefore, it provides excellent conditions for various hyperspectral imaging techniques and enables high-quality multisensor hyperspectral chemical imaging at submicron lateral resolutions. [ABSTRACT FROM AUTHOR]

Presents information on the study conducted which assessed the electronic and magnetic properties of normally immiscible FeCu(100) alloy films. Measurements of the magnetic circular dichroism in soft x-ray absorption of these fcc FeCu alloy films; Behavior of the magnetic moments of the Fe films; Experimental procedures used in the study; Results of the study.

Presents information on a study which investigated magnetic circular dichroism in valence band photoemission (MCDAD) from 15 monolayer thick face-centered cubic nickel (Ni) films on copper. Illustration of a series of intensity spectra for different photon energies; Discussion on the relativistic band structure of Ni along the change axis; Demonstration of MCDAD.

Deals with a study which performed magnetic dichroism measurements of the valence bands of films of fcc Co/Cu (001) using angle-resolved ultraviolet photoemission in low-symmetry, off-normal emission geometries and linearly polarized light. Evidence of both spin-dependent surface transmission and magnetic dichroism in the angular distribution of photoelectrons.

An improved spin-polarized electron energy loss spectroscopy apparatus with high momentum resolution was developed during the last few years. It is equipped with a multichannel energy analyzer and multichannel spin imaging detector. This technique allows to see features on the energy loss spectra, angular dependence of asymmetry and intensity and pronounced sensitivity on the primary energy. In this work, we studied dipolar scattering resonances, spin-dependent electron-hole excitations and their momentum dependence and collective magnetic excitation, i.e. high energy magnons. Copyright © 2016 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Background: Pontocerebellar hypoplasia type 6 (PCH6) is a mitochondrial disease caused by mutations in the RARS2 gene. RARS2 encodes mitochondrial arginyl transfer RNA synthetase, an enzyme involved in mitochondrial protein translation. A total of 27 patients from 14 families have been reported so far. Characteristic clinical features comprise neonatal lactic acidosis, severe encephalopathy, intractable seizures, feeding problems and profound developmental delay. Most patients show typical neuroradiologic abnormalities including cerebellar hypoplasia and progressive pontocerebellar atrophy. Methods: We describe the clinical, biochemical and molecular features of 2 siblings with a novel homozygous mutation in RARS2. Both patients presented neonatally with lactic acidosis. While the older sibling had severe neurological symptoms with microcephaly, seizures and developmental delay, the younger patient was still neurologically asymptomatic at the age of 2 months. Results: MRI studies in both children lacked pontocerebellar involvement. The expression of the OXPHOS complex proteins was decreased in both patients, whereas oxygen consumption was increased. Conclusions: Characteristic neuroradiological abnormalities of PCH6 such as vermis and cerebellar hypoplasia and progressive pontocerebellar atrophy may be missing in patients with RARS2 mutations. RARS2 testing should therefore also be performed in patients without pontocerebellar hypoplasia but otherwise typical clinical symptoms. [ABSTRACT FROM AUTHOR]

We describe a new "complete" spin-polarized electron energy loss spectrometer comprising a spin-polarized primary electron source, an imaging electron analyzer, and a spin analyzer of the "spin-polarizing mirror" type. Unlike previous instruments, we have a high momentum resolution of less than 0.04 Å-1, at an energy resolution of 90-130 meV. Unlike all previous studies which reported rather broad featureless data in both energy and angle dependence, we find richly structured spectra depending sensitively on small changes of the primary energy, the kinetic energy after scattering, and of the angle of incidence. The key factor is the momentum resolution. [ABSTRACT FROM AUTHOR]

We have designed and constructed a spin polarized low energy electron diffraction system working in the reflected electron pulse counting mode. This system is capable of measuring asymmetries due to spin-orbit and exchange interactions. Photoemission from a strained GaAs/GaAsP super lattice is used as the source of spin polarized electrons. Spin-orbit asymmetry is evaluated for Ir(100) single crystal at various energies. Subsequently, exchange asymmetry has been evaluated on 40 monolayer Fe deposited on Ir(100). This instrument proves to be useful in understanding structure and magnetism at surfaces. [ABSTRACT FROM AUTHOR]

We present clinical features and genetic results of 1206 index patients and 124 affected relatives who were referred for genetic testing of Charcot--Marie--Tooth (CMT) neuropathy at the laboratory in Aachen between 2001 and 2012. Genetic detection rates were 56% in demyelinating CMT (71% of autosomal dominant (AD) CMT1/CMTX), and 17% in axonal CMT (24% of AD CMT2/CMTX). Three genetic defects (PMP22 duplication/deletion, GJB1/Cx32 or MPZ/P0 mutation) were responsible for 89.3% of demyelinating CMT index patients in whom a genetic diagnosis was achieved, and the diagnostic yield of the three main genetic defects in axonal CMT (GJB1/Cx32, MFN2, MPZ/P0 mutations) was 84.2%. De novo mutations were detected in 1.3% of PMP22 duplication, 25% of MPZ/P0, and none in GJB1/Cx32. Motor nerve conduction velocity was uniformly <38m/s in median or ulnar nerves in PMP22 duplication, >40m/s in MFN2, and more variable in GJB1/Cx32, MPZ/P0 mutations. Patients with CMT2A showed a broad clinical severity regardless of the type or position of the MFN2 mutation. Out of 75 patients, 8 patients (11%) with PMP22 deletions were categorized as CMT1 or CMT2. Diagnostic algorithms are still useful for cost-efficient mutation detection and for the interpretation of large-scale genetic data made available by next generation sequencing strategies. [ABSTRACT FROM AUTHOR]

Copyright of Monatsschrift Kinderheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The article presents the case of a 16-year-old male patient with bilateral optic neuropathy caused by a mutation in the ND6-gene. Brief information about Leber's hereditary optic neuropathy (LHON) is presented. Also discussed are the results of the laboratory examinations conducted on the patient. Noted also is the administration of idebenone as beginning therapy for the patient, which resulted to continuous improvement in vision.

Background Successful antiviral treatment of decompensated hepatitis B with HBV polymerase inhibitors is associated with improvement of liver function. To what extent liver function also improves in cirrhotic patients with chronic hepatitis C receiving novel interferon-free (IFN-free) therapies is unknown. Aim To study liver function in cirrhotic HCV patients receiving IFN-free therapies. Methods We here studied 80 consecutive patients with advanced HCV associated liver cirrhosis including 34 patients (43%) with Child B/C cirrhosis and 42 patients (53%) with platelet counts of <90.000/μL receiving different combinations of direct acting antivirals without interferon [sofosbuvir/ribavirin ( n = 56), sofosbuvir/simeprevir ± ribavirin ( n = 15) and sofosbuvir/daclatasvir ± ribavirin ( n = 9)]. The majority of patients was infected with HCV genotype 1 ( n = 50); HCV genotypes 2, 3 and 4 were present in 4, 24 and 2 patients, respectively. Results Liver function parameters including albumin, bilirubin, cholinesterase and prothrombin time all improved in the majority of patients during antiviral therapy irrespectively of the underlying HCV genotype, however, with different kinetics. MELD scores improved until post-treatment week 12 in 44% of the patients but worsened in 15%. A sustained virological response was achieved in 63% of the patients. HCV RNA relapse led to moderate ALT increases in 15/23 patients but was not associated with hepatic decompensations. Conclusion This real-world single centre study showed that interferon-free treatment of hepatitis C patients with advanced liver cirrhosis restores liver function, and may thereby reduce the need for liver transplantations. [ABSTRACT FROM AUTHOR]

An in situ stress measurement setup using an optical 2-beam curvature technique is described which is compatible with the stringent growth conditions of pulsed laser deposition (PLD) of perovskite oxides, which involves high substrate temperatures of 1000 K and oxygen partial pressures of up to 1 × 10-4 millibars. The stress measurements are complemented by medium energy electron diffraction (MEED), Auger electron spectroscopy, and additional growth rate monitoring by a quartz microbalance. A shielded filament is used to allow for simultaneous stress and MEED measurements at high substrate temperatures. A computer-controlled mirror scans an excimer laser beam over a stationary PLD target. This avoids mechanical noise originating from rotating PLD targets, and the setup does not suffer from limited lifetime issues of ultra high vacuum (UHV) rotary feedthroughs. [ABSTRACT FROM AUTHOR]

Here, we present the first systematic study on the temperature dependence of the extension of the superconducting proximity effect in a 1-2 atomic layer thin metallic film, surrounding a superconducting Pb island. Scanning tunneling microscopy/spectroscopy (STM/STS) measurements reveal the spatial variation of the local density of state on the film from 0.38 up to 1.8 K. In this temperature range the superconductivity of the island is almost unaffected and shows a constant gap of a 1.20 ± 0.03 meV. Using a superconducting Nb-tip a constant value of the proximity length of 17 ± 3 nm at 0.38 and 1.8 K is found. In contrast, experiments with a normal conductive W-tip indicate an apparent decrease of the proximity length with increasing temperature. This result is ascribed to the thermal broadening of the occupation of states of the tip, and it does not reflect an intrinsic temperature dependence of the proximity length. Our tunneling spectroscopy experiments shed fresh light on the fundamental issue of the temperature dependence of the proximity effect for atomic monolayers, where the intrinsic temperature dependence of the proximity effect is comparably weak. [ABSTRACT FROM AUTHOR]

HCV RNA levels correlate with the long-term outcome of hepatitis C in liver transplant recipients. Nucleic acid testing ( NAT) is usually used to confirm HCV reinfection and to examine viral loads after liver transplantation. HCV core antigen ( HCVcoreAg) testing could be an alternative to NAT with some potential advantages including very low intra- and interassay variabilities and lower costs. The performance of HCVcoreAg testing in organ transplant recipients is unknown. We prospectively studied 1011 sera for HCV RNA and HCVcoreAg in a routine real-world setting including 222 samples obtained from patients after liver or kidney transplantation. HCV RNA and HCVcoreAg test results showed a consistency of 98% with a very good correlation in transplanted patients ( r > 0.85). The correlation between HCV RNA and HCVcoreAg was higher in sera with high viral loads and in samples from patients with low biochemical disease. Patients treated with tacrolimus showed a better correlation between both parameters than individuals receiving cyclosporine A. HCV RNA/ HCVcoreAg ratios did not differ between transplanted and nontransplanted patients, and HCV RNA and HCVcoreAg kinetics were almost identical during the first days after liver transplantation. HCVcoreAg testing can be used to monitor HCV viral loads in patients after organ transplantation. However, the assay is not recommended to monitor antiviral therapies. [ABSTRACT FROM AUTHOR]

Background Antimicrobial stewardship programmes aim to optimise use of antibiotics and are now mandatory in all Australian hospitals. Aim We aimed to identify barriers to and enablers of appropriate antimicrobial prescribing among hospital doctors. Methods Two paper-based and one web-based surveys were administered at three Australian university teaching hospitals from March 2010 to May 2011. The 18-item questionnaire recorded doctors' level of experience, their knowledge regarding the use of common antimicrobials and their attitudes regarding antimicrobial prescribing. Local survey modifications allowed inclusion of specific questions on: infections in intensive care unit patients, clinical microbiology and use of local guidelines. Results The respondents ( n = 272) were comprised of 96 (35%) registrars, 67 (25%) residents, 57 (21%) interns and 47 (17%) consultant hospital doctors. Forty-one per cent were working in a medical specialty. Identified barriers included: gaps in antimicrobial prescribing knowledge (especially among interns), a lack of awareness about which antimicrobials were restricted and a reliance on senior colleagues to make antimicrobial prescribing decisions. Enablers of optimal prescribing included: an acknowledgement of the need for assistance in prescribing and reported readiness to consult national prescribing guidelines. These results were used to help guide and prioritise interventions to improve prescribing practices. Conclusion A transferable knowledge and attitudes survey tool can be used to highlight barriers and facilitators to optimal hospital antimicrobial prescribing in order to inform tailored antimicrobial stewardship interventions. [ABSTRACT FROM AUTHOR]

We report on the design of an ion source for the production of single and double charged Helium ions with kinetic energies in the range from 300 eV down to 5 eV. The construction is based on a commercial sputter ion gun equipped with a Wien-filter for mass/charge separation. Retardation of the ions from the ionizer potential (2 keV) takes place completely within the lens system of the sputter gun, without modification of original parts. For 15 eV He+ ions, the design allows for beam currents up to 30 nA, limited by the space charge repulsion in the beam. For He2+ operation, we obtain a beam current of 320 pA at 30 eV, and 46 pA at 5 eV beam energy, respectively. In addition, operating parameters can be optimized for a significant contribution of metastable He*+ (2s) ions. [ABSTRACT FROM AUTHOR]