STING-associated vasculopathy with onset in infancy (SAVI) presenting with skin lesions.

STING-associated vasculopathy with onset in infancy (SAVI) is caused by pathogenic gain-of-function variants in the gene TMEM173 (also named stimulator of interferon genes, STING1). This report details the case of an 11-year-old girl with SAVI who presented with skin-limited symptoms and discusses the phenotype-genotype correlations of the TMEM173 variant present in our patient. Treatment of SAVI focuses on preventing the development or progression of organ damage by reducing systemic inflammation. We summarize the available treatments for this syndrome.

Site percolation threshold of composite square lattices and its agroecology applications.

We analyze the percolation threshold of square lattices comprising a combination of sites with regular and extended neighborhoods. We found that the percolation threshold of these composed systems smoothly decreases with the fraction of sites with extended neighbors. This behavior can be well-fitted by a Tsallis q-Exponential function. We found a relation between the fitting parameters and the differences in the gyration radius among neighborhoods. We also compared the percolation threshold with the critical susceptibility of nearest and next-to-nearest neighbor monoculture plantations vulnerable to the spread of phytopathogen. Notably, the critical susceptibility in monoculture plantations can be described as a linear combination of two composite systems. These results allow the refinement of mathematical models of phytopathogen propagation in agroecology. In turn, this improvement facilitates the implementation of more efficient computational simulations of agricultural epidemiology that are instrumental in testing and formulating control strategies.

Adiabatic lapse rate of real gases.

We derive a formula for the dry adiabatic lapse rate of atmospheres composed of real gases. We restrict our study to those described by a family of two-parameter cubic equations of state and the recent Guevara-Rodríguez noncubic equation. Since our formula depends on the adiabatic curves, we compute them all at once, considering molecules that can move, rotate, and vibrate, for any equation of state. To illustrate our results, we estimate the lapse rate of the troposphere of Titan, obtaining a better approximation to the observed data in some instances, when compared to the estimation provided by the virial expansion up to the third order.

Percolation-intercropping strategies to prevent dissemination of phytopathogens on plantations.

Phytophthora is one of the most aggressive and worldwide extended phytopathogens that attack plants and trees. Its effects produce tremendous economical losses in agronomy and forestry since no effective fungicide exists. We propose to combine percolation theory with an intercropping sowing configuration as a non-chemical strategy to minimize the dissemination of the pathogen. In this work, we model a plantation as a square lattice where two types of plants are arranged in alternating columns or diagonals, and Phytophthora zoospores are allowed to propagate to the nearest and next-to-nearest neighboring plants. We determine the percolation threshold for each intercropping configuration as a function of the plant's susceptibilities and the number of inoculated cells at the beginning of the propagation process. The results are presented as phase diagrams where crop densities that prevent the formation of a spanning cluster of susceptible or diseased plants are indicated. The main result is the existence of susceptibility value combinations for which no spanning cluster is formed even if every cell in the plantation is sowed. This finding can be useful in choosing a configuration and density of plants that minimize damages caused by Phytophthora. We illustrate the application of the phase diagrams with the susceptibilities of three plants with a high commercial value.

Analysis of uncontrolled phosphorus precipitation in anaerobic digesters under thermophilic and mesophilic conditions.

This study compares the operation of mesophilic and thermophilic anaerobic digestion of sewage sludge and their effects in uncontrolled phosphorus precipitation. The research has been carried out using a pilot plant consisting of two digesters of 1.6 m3 working volume, treating the mixed sludge of Alzira WWTP (Valencia, Spain). The digesters were operated in parallel, at different conditions: mesophilic (38 ± 2.0°C) and thermophilic (55 ± 2.5°C) temperatures and organic loading rates (OLR) ranging from 1.1 to 1.7 kg volatile solids (VS) m-3 d-1 and different hydraulic retention times (HRT) 20, 15 and 12 days. Uncontrolled precipitation was evaluated through P, Mg and Ca mass balances in both digesters. The results revealed that up to 82% of the available P and 81% of the available Mg precipitated in the mesophilic digester at HRT = 20 days which suggests the possible formation of struvite in both digesters. At lower HRT (HRT = 12 days) Mg and Ca precipitation was negligible and P fixation has been attributed to the possible formation of iron phosphates or adsorption processes on solid surfaces.

Adiabatic lapse rate of nonideal gases: The role of molecular interactions and vibrations.

We report a formula for the dry adiabatic lapse rate that depends on the compressibility factor and the adiabatic curves. Then, to take into account the nonideal behavior of the gases, we consider molecules that can move, rotate, and vibrate and the information of molecular interactions through the virial coefficients. We deduce the compressibility factor in its virial expansion form and the adiabatic curves within the virial expansion up to any order. With this information and to illustrate the mentioned formula, we write the lapse rate for the ideal gas, and the virial expansion up to the second and third coefficient cases. To figure out the role of the virial coefficients and vibrations, under different atmospheric conditions, we calculate the lapse rate for Earth, Mars, Venus, Titan, and the exoplanet Gl 581d. Furthermore, for each one we consider three models in the virial expansion: van der Waals, square-well, and hard-sphere. Also, when possible, we compare our results to the experimental data. Finally, we remark that for Venus and Titan, which are under extreme conditions of pressure or temperature, our calculations are in good agreement with the observed values, in some instances.

Site-bond percolation solution to preventing the propagation of Phytophthora zoospores on plantations.

We propose a strategy based on the site-bond percolation to minimize the propagation of Phytophthora zoospores on plantations, consisting in introducing physical barriers between neighboring plants. Two clustering processes are distinguished: (i) one of cells with the presence of the pathogen, detected on soil analysis, and (ii) that of diseased plants, revealed from a visual inspection of the plantation. The former is well described by the standard site-bond percolation. In the latter, the percolation threshold is fitted by a Tsallis distribution when no barriers are introduced. We provide, for both cases, the formulas for the minimal barrier density to prevent the emergence of the spanning cluster. Though this work is focused on a specific pathogen, the model presented here can also be applied to prevent the spreading of other pathogens that disseminate, by other means, from one plant to the neighboring ones. Finally, the application of this strategy to three types of commercially important Mexican chili plants is also shown.

Dataset of volatile compounds identified, quantified and GDA generated of the maturation process of silver tequila in new French oak barrels.

This article contains data obtained by following the evolution of minor volatile compounds throughout 32 weeks of 100% Agave Silver tequila maturation in new French oak barrels under real cellar conditions. Barrels were made with the same cooperage methods in four French regions. Tequila samples were obtained every 2 weeks; volatile compounds were extracted and analyzed by GC-MS. Volatile compounds were identified and relatively quantified in % of Area. Obtained data are presented in three datasets: Identified compounds, quantification according to barrel origin, and quantification according to maturation time. General Discriminant Analysis of the quantification data sets are also shown. Interpretation of the data and discussion can be found in "Evolution of volatile compounds during the maturation process of Silver tequila in new French oak barrels" Martín-del-Campo, López-Ramírez and Estarrón-Espinosa [1].

Area covered by disks in small-bounded continuum percolating systems: An application to the string percolation model.

In string percolation model, the study of colliding systems at high energies is based on a continuum percolation theory in two dimensions, where the number of strings distributed in the surface of interest is strongly determined by the size and energy of the colliding particles. It is also expected that the surface where the disks are lying be finite, defining a system without periodic boundary conditions. In this work, we report modifications to the fraction of the area covered by disks in continuum percolating systems due to a finite number of disks and bounded by different geometries: circle, ellipse, triangle, square, and pentagon, which correspond to the first Fourier modes of the shape fluctuation of the initial state after the particle collision. We find that the deviation of the fraction of area covered by disks from its corresponding value in the thermodynamic limit satisfies a universal behavior, where the free parameters depend on the density profile, number of disks, and shape of the boundary. Consequently, it is also found that the color suppression factor of the string percolation model is modified by a damping function related to the small-bounded effects. Corrections to the temperature and the speed of sound defined in string systems are also shown for small and elliptically bounded systems.

Stochastic curvature of enclosed semiflexible polymers.

The conformational states of a semiflexible polymer enclosed in a compact domain of typical size a are studied as stochastic realizations of paths defined by the Frenet equations under the assumption that stochastic "curvature" satisfies a white noise fluctuation theorem. This approach allows us to derive the Hermans-Ullman equation, where we exploit a multipolar decomposition that allows us to show that the positional probability density function is well described by a telegrapher's equation whenever 2a/ℓ_{p}>1, where ℓ_{p} is the persistence length. We also develop a Monte Carlo algorithm for use in computer simulations in order to study the conformational states in a compact domain. In addition, the case of a semiflexible polymer enclosed in a square domain of side a is presented as an explicit example of the formulated theory and algorithm. In this case, we show the existence of a polymer shape transition similar to the one found by Spakowitz and Wang [Phys. Rev. Lett. 91, 166102 (2003)PRLTAO0031-900710.1103/PhysRevLett.91.166102] where in this case the critical persistence length is ℓ_{p}^{*}≃a/8 such that the mean-square end-to-end distance exhibits an oscillating behavior for values ℓ_{p}>ℓ_{p}^{*}, whereas for ℓ_{p}<ℓ_{p}^{*} it behaves monotonically increasing.

Behaviour of emerging contaminants in sewage sludge after anaerobic digestion.

Nowadays, there is an increasing concern over the presence of contaminants in the aquatic environment, where they can be introduced from wastewater after their incomplete removal in the treatment plants. In this work, degradation of selected emerging pollutants in the aqueous and solid phases of sewage sludge has been investigated after anaerobic digestion using two different digesters: mesophilic and thermophilic. Initially, sludge samples were screened by ultra-high-performance liquid chromatography coupled to quadrupole time-of-flight mass spectrometry (UHPLC-QTOF MS) for identification of emerging contaminants in the samples. In a second step, a target quantitative method based on LC coupled to tandem MS was applied for selected pollutants identified in the previous screening. The behaviour of the compounds under anaerobic conditions was studied estimating the degradation efficiency and distribution of compounds between both sludge phases. Irbesartan and benzoylecgonine seemed to be notably degraded in both phases of the sludge. Venlafaxine showed a significant concentration decrease in the aqueous phase in parallel to an increase in the solid phase. The majority of the compounds showed an increase of their concentrations in both phases after the digestion. Concentrations in the solid phase were commonly higher than in the aqueous for most contaminants, indicating that they were preferentially adsorbed onto the solid particles.

Analytical methodologies based on LC-MS/MS for monitoring selected emerging compounds in liquid and solid phases of the sewage sludge.

In this work, two analytical methodologies based on liquid chromatography coupled to tandem mass spectrometry (LC-MS/MS) were developed for quantification of emerging pollutants identified in sewage sludge after a previous wide-scope screening. The target list included 13 emerging contaminants (EC): thiabendazole, acesulfame, fenofibric acid, valsartan, irbesartan, salicylic acid, diclofenac, carbamazepine, 4-aminoantipyrine (4-AA), 4-acetyl aminoantipyrine (4-AAA), 4-formyl aminoantipyrine (4-FAA), venlafaxine and benzoylecgonine. The aqueous and solid phases of the sewage sludge were analyzed making use of Solid-Phase Extraction (SPE) and UltraSonic Extraction (USE) for sample treatment, respectively. The methods were validated at three concentration levels: 0.2, 2 and 20 µg L(-1) for the aqueous phase, and 50, 500 and 2000 µg kg(-1) for the solid phase of the sludge. In general, the method was satisfactorily validated, showing good recoveries (70-120%) and precision (RSD < 20%). Regarding the limit of quantification (LOQ), it was below 0.1 µg L(-1) in the aqueous phase and below 50 µg kg(-1) in the solid phase for the majority of the analytes. The method applicability was tested by analysis of samples from a wider study on degradation of emerging pollutants in sewage sludge under anaerobic digestion. The key benefits of these methodologies are: • SPE and USE are appropriate sample procedures to extract selected emerging contaminants from the aqueous phase of the sewage sludge and the solid residue. • LC-MS/MS is highly suitable for determining emerging contaminants in both sludge phases. • Up to our knowledge, the main metabolites of dipyrone had not been studied before in sewage sludge.

Treatment of a submerged anaerobic membrane bioreactor (SAnMBR) effluent by an activated sludge system: the role of sulphide and thiosulphate in the process.

This work studies the use of a well-known and spread activated sludge system (UCT configuration) to treat the effluent of a submerged anaerobic membrane bioreactor (SAnMBR) treating domestic wastewater. Ammonia, phosphate, dissolved methane and sulphide concentrations in the SAnMBR effluent were around 55 mg NH4-N L(-1), 7 mg PO4-P L(-1), 30 mg non-methane biodegradable COD L(-1), and 105 mg S(2-) L(-1) respectively. The results showed a nitrification inhibition caused by the presence of sulphur compounds at any of the solids retention time (SRT) studied (15, 20 and 25 days). This inhibition could be overcome increasing the hydraulic retention time (HRT) from 13 to 26 h. Among the sulphur compounds, sulphide was identified as the substance which caused the nitrification inhibition. When the nitrification was well established, removal rates of nitrogen and phosphorus of 56% and 45% were reached respectively. The sulphide present in the influent was completely oxidised to sulphate, contributing this oxidation to the denitrification process. Moreover, the presence of methanotrophic bacteria, detected by FISH technique, could also contribute to the denitrification.

Carbohydrate-functionalized nanovaccines preserve HIV-1 antigen stability and activate antigen presenting cells.

The functionalization of polymeric nanoparticles with ligands that target specific receptors on immune cells offers the opportunity to tailor adjuvant properties by conferring pathogen mimicking attributes to the particles. Polyanhydride nanoparticles are promising vaccine adjuvants with desirable characteristics such as immunomodulation, sustained antigen release, activation of antigen presenting cells (APCs), and stabilization of protein antigens. These capabilities can be exploited to design nanovaccines against viral pathogens, such as HIV-1, due to the important role of dendritic cells (DCs) and macrophages in viral spread. In this work, an optimized process was developed for carbohydrate functionalization of HIV-1 antigen-loaded polyanhydride nanoparticles. The carbohydrate-functionalized nanoparticles preserved antigenic properties upon release and also enabled sustained antigen release kinetics. Particle internalization was observed to be chemistry-dependent with positively charged nanoparticles being taken up more efficiently by DCs. Up-regulation of the activation makers CD40 and CD206 was demonstrated with carboxymethyl-α-d-mannopyranosyl-(1,2)-d-mannopyranoside functionalized nanoparticles. The secretion of the cytokines IL-6 and TNF-α was shown to be chemistry-dependent upon stimulation with carbohydrate-functionalized nanoparticles. These results offer important new insights upon the interactions between carbohydrate-functionalized nanoparticles and APCs and provide foundational information for the rational design of targeted nanovaccines against HIV-1.

Diagnóstico radiológico del encondroma protuberans humeral / Radiological diagnosis of enchondroma protuberans of the humerus

El encondroma protuberans (EP) es un tumor exofítico de estirpe cartilaginosa, poco frecuente, que se describe como una lesión ósea intramedular que protruye por uno de los lados del hueso afecto a través de un defecto cortical, extendiéndose a las partes blandas adyacentes. Sus peculiares características de imagen hacen que los diagnósticos diferenciales principales sean el osteocondroma, el condrosarcoma y los tumores condrales periósticos. En este artículo describimos los hallazgos radiológicos del EP en el húmero en dos pacientes y revisamos la bibliografía, resaltando las características de imagen que pueden permitir un diagnóstico definitivo. Identificar la conexión entre los dos componentes de la lesión a través de un defecto cortical es un hallazgo clave en el diagnóstico. Con las técnicas de imagen disponibles, especialmente la resonancia magnética (RM), podemos establecer el diagnóstico de este tipo de lesiones, permitiendo la elección del tratamiento adecuado y disminuyendo la posibilidad de recidivas locales o malignización (AU)

Enchondroma protuberans (EP) is an uncommon exophytic cartilaginous tumor described as an intramedullary osseous lesion that protrudes through a cortical defect in one of the sides of the affected bone and expands to the adjacent soft tissues. Due to its peculiar features on imaging studies, the main differential diagnosis is with osteochondroma, chondrosarcoma, and periosteal chondral tumors. In this article, we describe the imaging findings in two patients with EP in the humerus. We review the literature and discuss the imaging features that can enable a definitive diagnosis. The identification of the connection between the two components of the lesion through a cortical defect is a key finding for the diagnosis. Available imaging techniques, especially MRI, make it possible to establish the diagnosis in this type of lesions and thus to choose the appropriate treatment, reducing the chance of local recurrence and malignant transformation (AU)

[Radiological diagnosis of enchondroma protuberans of the humerus]. / Diagnóstico radiológico del encondroma protuberans humeral.

Enchondroma protuberans (EP) is an uncommon exophytic cartilaginous tumor described as an intramedullary osseous lesion that protrudes through a cortical defect in one of the sides of the affected bone and expands to the adjacent soft tissues. Due to its peculiar features on imaging studies, the main differential diagnosis is with osteochondroma, chondrosarcoma, and periosteal chondral tumors. In this article, we describe the imaging findings in two patients with EP in the humerus. We review the literature and discuss the imaging features that can enable a definitive diagnosis. The identification of the connection between the two components of the lesion through a cortical defect is a key finding for the diagnosis. Available imaging techniques, especially MRI, make it possible to establish the diagnosis in this type of lesions and thus to choose the appropriate treatment, reducing the chance of local recurrence and malignant transformation.

A high statistics measurement of the Lambda(+)(c) lifetime.

A high statistics measurement of the Lambda(+)(c) lifetime from the Fermilab fixed-target FOCUS photoproduction experiment is presented. We describe the analysis technique with particular attention to the determination of the systematic uncertainty. The measured value of 204.6 +/- 3.4 (stat) +/- 2.5 (syst) fs from 8034 +/- 122 Lambda(+)(c)-->pK(-)pi(+) decays represents a significant improvement over the present world average.

Search for CP violation in the decays D+--> K(S)pi+ and D+-->K(S)K+.

A high-statistics sample of photoproduced charm from the FOCUS experiment has been used to search for direct CP violation in the decay rates for D+-->K(S)pi+ and D+-->K(S)K+. We have measured the following asymmetry parameters relative to D+-->K-pi+pi+: A(CP)(K(S)pi+) = (-1.6+/-1.5+/-0.9)%, A(CP)(K(S)K+) = (+6.9+/-6.0+/-1.5)%, and A(CP)(K(S)K+) = (+7.1+/-6.1+/-1.2)% relative to D+-->K(S)pi+. We have also measured the relative branching ratios and found Gamma(D+-->K(0)pi+)/Gamma(D+-->K-pi+pi+) = (30.60+/-0.46+/-0.32)%, Gamma(D+-->K(0)K+)/Gamma(D+-->K-pi+pi+) = (6.04+/-0.35+/-0.30)%, and Gamma(D+-->K(0)K+)/Gamma(D+-->K(0)pi+) = (19.96+/-1.19+/-0.96)%.

Measurement of the branching ratios of D(+) and D(+)(s) hadronic decays to four-body final states containing a K(S).

We have studied hadronic four-body decays of D(+) and D(+)(s) mesons with a K(S) in the final state using data recorded during the 1996-1997 fixed-target run of the Fermilab high energy photoproduction experiment FOCUS. We report a new branching ratio measurement of gamma(D(+)-->K(S)K-pi(+)pi(+))/gamma(D(+)-->K(S)pi(+)pi(+)pi(-)) = 0.0768+/-0.0041+/-0.0032. We make the first observation of three new decay modes with branching ratios gamma(D(+)-->K(S)K+pi(+)pi(-))/gamma(D(+)-->K(S)pi(+)pi(+)pi(-)) = 0.0562+/-0.0039+/-0.0040, gamma(D(+)-->K(S)K+K-pi(+))/gamma(D(+)-->K(S)pi(+)pi(+)pi(-)) = 0.0077+/-0.0015+/-0.0009, and gamma(D(+)(s)-->K(S)K+pi(+)pi(-))/gamma(D(+)(s)-->K(S)K-pi(+)pi(+)) = 0.586+/-0.052+/-0.043, where in each case the first error is statistical and the second error is systematic.

Study of the decay D0 --> K+pi-.

Using a large sample of photoproduced charm mesons from the FOCUS experiment at Fermilab (FNAL-E831), we observe the decay D0-->K+pi- with a signal yield of 149+/-31 events compared to a similarly cut sample consisting of 36 760+/-195 D0-->K-pi+ events. We use the observed ratio of D0-->K+pi- to D0-->K-pi+ (0.404+/-0.085+/-0.025)% to obtain a relationship between the D0 mixing and doubly Cabibbo suppressed decay parameters.