Sirolimus Ointment for Facial Angiofibromas in Individuals with Tuberous Sclerosis Complex.

BACKGROUND: Facial angiofibromas affect most patients with tuberous sclerosis complex. They tend to progress, can cause recurrent bleeding and facial disfigurement, and have significant psychological effects. We reviewed the effectiveness and safety of topical sirolimus ointment 0.1%. We also assessed the effect of treatment on quality of life. METHODS: We report our experience in using sirolimus ointment in 14 patients with TSC (9 children and 5 adults). The impact of sirolimus ointment was monitored with digital photography, dermatological review using a validated Facial Angiofibroma Severity Index (FASI), and quality of life assessments using the questionnaires PedsQL for children and SF36 for adults. RESULTS: The FASI scores were improved in 12/14 cases after six months' treatment, and improvement was more likely in children (median FASI scores of improvement after treatment were 3 points for children and 1 for adults). Proxy-reported PedsQL scores for the total psychosocial domain improved significantly in the children in the cohort with treatment. CONCLUSIONS: Sirolimus ointment 0.1% administered once a day was effective in treating facial angiofibromas. It appears to be safe and well tolerated and to have a positive impact on patients' quality of life. It appeared to be most beneficial when started in childhood

Distinct magnetotransport and orbital fingerprints of chiral bobbers

While chiral magnetic skyrmions have been attracting significant attention in the past years, recently, a new type of a chiral particle emerging in thin films $-$ a chiral bobber $-$ has been theoretically predicted and experimentally observed. Here, based on theoretical arguments, we provide a clear pathway to utilizing chiral bobbers for the purposes of future spintronics by uncovering that these novel chiral states possess inherent transport fingerprints that allow for their unambiguous electrical detection in systems comprising several types of chiral states. We reveal that unique transport and orbital characteristics of bobbers root in the non-trivial magnetization distribution in the vicinity of the Bloch points, and demonstrate that tuning the details of the Bloch point topology can be used to drastically alter the emergent response properties of chiral bobbers to external fields, which bears great potential for engineering chiral dynamics and cognitive computing.Comment: Supplementary available upon reques

Evaluation of Kelvin probe force microscopy for imaging grain boundaries in chalcopyrite thin films

In view of the outstanding performance of polycrystalline thin film solar cells on the basis of Cu In,Ga Se2, the electrical activity at grain boundaries currently receives considerable attention. Recently, Kelvin probe force microscopy KPFM has been applied to characterize of the properties of individual grain boundaries, observing a drop in the surface potential in many cases. We present finite element simulations of the electrostatic forces to assess the experimental resolution of KPFM. Depending on the tip sample distance, the observed drop in the work function amounts to only a fraction of the real surface potential drop. The simulations are considered for different grain boundary models and consequences for the quantitative evaluation of experimental results are discusse

Unveiling the corona of the Milky Way via ram-pressure stripping of dwarf satellites

<p>The spatial segregation between dwarf spheroidal (dSph) and dwarf irregular galaxies in the Local Group has long been regarded as evidence of an interaction with their host galaxies. In this paper, we assume that ram-pressure stripping is the dominant mechanism that removed gas from the dSphs and we use this to derive a lower bound on the density of the corona of the Milky Way at large distances (R similar to 50-90 kpc) from the Galactic Centre. At the same time, we derive an upper bound by demanding that the interstellar medium of the dSphs is in pressure equilibrium with the hot corona. We consider two dwarfs (Sextans and Carina) with well-determined orbits and star formation histories. Our approach introduces several novel features: (i) we use the measured star formation histories of the dwarfs to derive the time at which they last lost their gas and (via a modified version of the Kennicutt-Schmidt relation) their internal gas density at that time; (ii) we use a large suite of 2D hydrodynamical simulations to model the gas stripping; and (iii) we include supernova feedback tied to the gas content. Despite having very different orbits and star formation histories, we find results for the two dSphs that are in excellent agreement with one another. We derive an average particle density of the corona of the Milky Way at R = 50-90 kpc in the range n(cor) = 1.3-3.6 x 10(-4) cm(-3). Including additional constraints from X-ray emission limits and pulsar dispersion measurements (that strengthen our upper bound), we derive Galactic coronal density profiles. Extrapolating these to large radii, we estimate the fraction of baryons (missing baryons) that can exist within the virial radius of the Milky Way. For an isothermal corona (T-cor = 1.8 x 10(6) K), this is small - just 10-20 per cent of the expected missing baryon fraction, assuming a virial mass of 1-2 x 10(12) M-circle dot. Only a hot (T-cor = 3 x 10(6) K) and adiabatic corona can contain all of the Galaxy's missing baryons. Models for the Milky Way must explain why its corona is in a hot adiabatic thermal state; or why a large fraction of its baryons lie beyond the virial radius.</p>

Crystal structure of polymeric carbon nitride and the determination of its process-temperature-induced modifications

Based on the arrangement of two-dimensional 'melon', we construct a unit cell for polymeric carbon nitride (PCN) synthesized via thermal polycondensation, whose theoretical diffraction powder pattern includes all major features measured in x-ray diffraction. With the help of this unit cell, we describe the process-temperature-induced crystallographic changes in PCN that occur within a temperature interval between 510 and 610 °C. We also discuss further potential modifications of the unit cell for PCN. It is found that both triazine- and heptazine-based g-C3N4 can only account for minor phases within the investigated synthesis products

Gaseous argon time projection chamber with electroluminescence enhanced optical readout

Systematic uncertainties in accelerator oscillation neutrino experiments arise mostly from nuclear models describing neutrino-nucleus interactions. To mitigate these uncertainties, we can study neutrino-nuclei interactions with detectors possessing enhanced hadron detection capabilities at energies below the nuclear Fermi level. Gaseous detectors not only lower the particle detection threshold but also enable the investigation of nuclear effects on various nuclei by allowing for changes in the gas composition. This approach provides valuable insights into the modelling of neutrino-nucleus interactions and significantly reduces associated uncertainties. Here, we discuss the design and first operation of a gaseous argon time projection chamber optically read. The detector operates at atmospheric pressure and features a single stage of electron amplification based on a thick GEM. Here, photons are produced with wavelengths in the vacuum ultraviolet regime. In an optical detector the primary constraint is the light yield. This study explores the possibility of increasing the light yield by applying a low electric field downstream of the ThGEM. In this region, called the electroluminescence gap, electrons propagate and excite the argon atoms, leading to the subsequent emission of photons. This process occurs without any further electron amplification, and it is demonstrated that the total light yield increases up to three times by applying moderate electric fields of the order of 3~kV/cm. Finally, an indirect method is discussed for determining the photon yield per charge gain of a ThGEM, giving a value of 18.3 photons detected per secondary electron

Scaling of the distribution of fluctuations of financial market indices

We study the distribution of fluctuations over a time scale $\Delta t$ (i.e., the returns) of the S&P 500 index by analyzing three distinct databases. Database (i) contains approximately 1 million records sampled at 1 min intervals for the 13-year period 1984-1996, database (ii) contains 8686 daily records for the 35-year period 1962-1996, and database (iii) contains 852 monthly records for the 71-year period 1926-1996. We compute the probability distributions of returns over a time scale $\Delta t$, where $\Delta t$ varies approximately over a factor of 10^4 - from 1 min up to more than 1 month. We find that the distributions for $\Delta t \leq$ 4 days (1560 mins) are consistent with a power-law asymptotic behavior, characterized by an exponent $\alpha \approx 3$, well outside the stable L\'evy regime $0 < \alpha < 2$. To test the robustness of the S&P result, we perform a parallel analysis on two other financial market indices. Database (iv) contains 3560 daily records of the NIKKEI index for the 14-year period 1984-97, and database (v) contains 4649 daily records of the Hang-Seng index for the 18-year period 1980-97. We find estimates of $\alpha$ consistent with those describing the distribution of S&P 500 daily-returns. One possible reason for the scaling of these distributions is the long persistence of the autocorrelation function of the volatility. For time scales longer than $(\Delta t)_{\times} \approx 4$ days, our results are consistent with slow convergence to Gaussian behavior.Comment: 12 pages in multicol LaTeX format with 27 postscript figures (Submitted to PRE May 20, 1999). See http://polymer.bu.edu/~amaral/Professional.html for more of our work on this are