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Subjects with Megalencephaly-Capillary Malformation-Polymicrogyria syndrome (MCAP) can present with a Chiari Malformation Type 1 and resulting alterations in cerebrospinal fluid (CSF) dynamics, which may require surgical treatment. The aim of this paper is to describe the features of children with MCAP who underwent surgical decompression for CM1, and to explore the PIK3CA variant allele frequency (VAF) identified in cerebellar parenchyma and other adjacent structures.This study reviewed two cases of children with CM1 and MCAP who underwent surgical decompression treatment. These two cases were part of a national cohort of 12 MCAP patients who had CM1, due to their surgical eligibility. Tissue samples were obtained from the cerebellar tonsils and adjacent anatomical structures during the surgical procedures. Samples were then subsequently analyzed for PIK3CA postzygotic variants.In both cases, alterations in CSF dynamics, specifically hydrocephalus and syringomyelia, were observed and required surgical treatment. PIK3CA targeted sequencing determined the VAF of the postzygotic variant in both cerebellar and adjacent bone/connective tissues.The recognition of a CM1 comorbidity in MCAP patients is of paramount importance when considering personalized treatment options, especially because these patients are at higher risk of developing complications during surgical decompression surgery. The variable PIK3CA VAF identified in the different analyzed tissues might help explain the heterogeneous nature and severity of anomalies observed in the volume of the posterior fossa structures in MCAP patients and associated CSF and venous disorders.

Sinonasal intestinal-type adenocarcinomas (ITACs) are rare and aggressive tumors, closely related to professional exposure to wood dusts or leather. Here we explored the role of non-coding RNAs controlling MUC2 in liquid biopsies and tumors from ITAC patients with the aim of identifying biomarkers and molecular mechanisms to improve early diagnosis, prognosis, and therapeutic approaches for this rare cancer.MiR-34c-3p, lncRNA AF147447 and MUC2 were measured in tumors and normal mucosa, in nasal washings (NW) from the affected and non-affected nostril and in plasma from 17 ITAC patients. The Apparent Diffusion Coefficient (ADC) was also evaluated by Magnetic Resonance Imaging.MiR-34c was higher in ITACs compared to the corresponding normal mucosa (p = 0.021). Differentiated tumors exhibited higher miR-34c levels (p = 0.025) and lower ADC values (p0.001) compared to mucinous ones and these parameters were also inversely correlated (r = 0.87; p = 0.001). High MUC2 tumor expression was associated with orbital extension (p = 0.010). Low miR-34c levels in NW were associated with orbital (p = 0.009) and intracranial (p = 0.031) extension and with advanced TNM stage (p = 0.054). Functional analysis identified Wnt, Focal adhesion, MAPK and inflammatory signalings among the pathways most enriched in mir-34c targets.Our results suggest measuring miR-34c in NW as a biomarker for early diagnosis and monitoring of ITAC patients and for the surveillance of wood and leather exposed workers. Further research on the involvement of miR-34c regulated pathways in ITAC tumorigenesis may also allow the development of new therapeutic approaches for this rare cancer.

Background: Severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2)-associated coronavirus disease-2019 (COVID-19) is spreading worldwide challenging healthcare resources. To provide the best possible care for patients, efficient prognosis is needed. Methods: We performed a multicentre cohort study to develop and validate a risk score for severe respiratory failure (SRF) among hospitalized patients diagnosed with COVID-19. Study population included all adult patients with RT-PCR on nasopharyngeal swab positive for SARS-CoV-2, hospitalized from February 22 to April 3 2020, at 11 hospitals from four Italian Regions. Exclusion criteria were early ( 30bpm, or respiratory distress. Findings: Of the 1265 included patients, 1044 patients were analyzed, (581 derivation, 463 validation cohort). Mean age was 65.7±15 years, 661 (63·3%) were male. SRF rates were 29% and 38% in derivation and validation cohort, respectively. At multivariate analysis, risk factors for SRF in the derivation cohort were age ≥70 years, obesity, body temperature ≥38°C, RR ≥22bpm, lymphocytes ≤900/mm3, creatinine ≥ 1 mg/dl, C-reactive protein ≥10mg/dl, and lactate dehydrogenase ≥350IU/L at hospitalization. Area under receiver-operator curve (AUROC) was 0·90 (95%CI 0.86-0.93). Assigning points to each variable an individual risk score (PREDICO score) was obtained. At risk score of >3, sensitivity, specificity, positive and negative predictive values were 71·6% (64-78), 89·1% (85-92), 72·9% (65-79), and 88·4% (85-91), respectively; positive and negative likelihood ratios were 6·56 (95% CI 4·9-8·77) and 0·31 (95%CI 0·25-0·40), respectively. PREDICO score showed similar prognostic ability in the validation cohort: AUROC 0·80 (0·74-0·88). Interpretation: Using eight variables, easy to obtain at hospitalization, patients with COVID-19 could be classified according to their probability of developing SRF during hospitalization. PREDICO score can be useful to allocate resources and prioritize treatments during COVID-19 pandemic. Trial Registration: NCT04316949. Funding Statement: None. Declaration of Interests: Authors state no conflict of interest related to the content of the present study. Ethics Approval Statement: The study was approved by the Ethic Committee of the promoting center (Comitato Etico Indipendente di Area Vasta Emilia Centro, n. 283/2020/Oss/AOUBo).

The paper analyzes data about recharge of electric cars in Rome during 2013 as a part of a national research project (P.R.I.M.E.). The electric vehicles were recharged through the public Enel Distribuzione recharging infrastructure. For each recharge, the initial and final time were registered together with the electricity absorbed from the grid. The total number of recharges was about 7700. The first step of the investigation is the statistical analysis of the distribution of recharges in daily time slots in order to analyze the recharge behavior of Italian drivers. For each day and for each time slot, literature data from the Italian national grid operator (Terna) were used to retrieve the energy mix used to produce electricity in that day and in that time slot. In the third step, electricity generation mixes were used to obtain emission factors for greenhouse (CO2) and pollutant emissions (CO, NOx, HC and particulate). Using information about the electric consumption of vehicles registered in Rome, the emission factors in g/km were obtained and compared with the limits set by European legislation for conventional (gasoline and diesel).

Stress adaptation is essential for neuronal health. While the fundamental role of mitochondria in neuronal development has been demonstrated, it is still not clear how adult neurons respond to alterations in mitochondrial function and how neurons sense, signal, and respond to dysfunction of mitochondria and their interacting organelles. Here, we show that neuron-specific, inducible in vivo ablation of the mitochondrial fission protein Drp1 causes ER stress, resulting in activation of the integrated stress response to culminate in neuronal expression of the cytokine Fgf21. Neuron-derived Fgf21 induction occurs also in murine models of tauopathy and prion disease, highlighting the potential of this cytokine as an early biomarker for latent neurodegenerative conditions.

The present investigation describes the results of a research project (P.R.I.M.E.) aimed at testing the performance and the environmental impact of an electric city car in Italian cities. The vehicle considered in the project is the Daimler AG Smart ForTwo Electric Drive. A Smart ED vehicle was tested at the University of Salento for six months over different driving conditions (routes, traffic, use of auxiliaries). A data acquisition system has been designed on purpose and assembled on board to provide information about driving cycle and energy flows. The system was also used to evaluate the losses of energy during recharges due to the battery cooling system. The experimental tests were used to identify the average, minimum and maximum consumption of electricity in the Smart ED in Lecce according to driving conditions and in particular according to the usage of auxiliaries. The measured data of electric consumption have been used to quantify the emissions of CO2 and pollution of the vehicle using information about the Italian electricity production mix of each recharging event and the emissions factors of the Italian power plants with an innovative and comprehensive methodology.

During apoptosis mitochondria undergo cristae remodeling and fragmentation, but how the latter relates to outer membrane permeabilization and downstream caspase activation is unclear. Here we show that the mitochondrial fission protein Dynamin Related Protein (Drp) 1 participates in cytochrome c release by selected intrinsic death stimuli. While Bax, Bak double deficient (DKO) and Apaf1(-/-) mouse embryonic fibroblasts (MEFs) were less susceptible to apoptosis by Bcl-2 family member BID, H(2)O(2), staurosporine and thapsigargin, Drp1(-/-) MEFs were protected only from BID and H(2)O(2). Resistance to cell death of Drp1(-/-) and DKO MEFs correlated with blunted cytochrome c release, whereas mitochondrial fragmentation occurred in all cell lines in response to all tested stimuli, indicating that other mechanisms accounted for the reduced cytochrome c release. Indeed, cristae remodeling was reduced in Drp1(-/-) cells, potentially explaining their resistance to apoptosis. Our results indicate that caspase-independent mitochondrial fission and Drp1-dependent cristae remodeling amplify apoptosis. This article is part of a Special Issue entitled 'EBEC 2016: 19th European Bioenergetics Conference, Riva del Garda, Italy, July 2-6, 2016', edited by Prof. Paolo Bernardi.

Background: Genetic and environmental components may contribute to acne causation. Objective: We sought to assess the impact of family history, personal habits, dietary factors, and menstrual history on a new diagnosis of moderate to severe acne. Methods: We conducted a case-control study in dermatologic outpatient clinics in Italy. Cases (205) were consecutive those receiving a new diagnosis of moderate to severe acne. Control subjects (358) were people with no or mild acne, coming for a dermatologic consultation other than for acne. Results: Moderate to severe acne was strongly associated with a family history of acne in first-degree relatives (odds ratio 3.41, 95% confidence interval 2.31-5.05). The risk was reduced in people with lower body mass index with a more pronounced effect in male compared with female individuals. No association with smoking emerged. The risk increased with increased milk consumption (odds ratio 1.78, 95% confidence interval 1.22-2.59) in those consuming more than 3 portions per week. The association was more marked for skim than for whole milk. Consumption of fish was associated with a protective effect (odds ratio 0.68, 95% confidence interval 0.47-0.99). No association emerged between menstrual variables and acne risk. Limitations: Some degree of overmatching may arise from choosing dermatologic control subjects and from inclusion of mild acne in the control group. Conclusions: Family history, body mass index, and diet may influence the risk of moderate to severe acne. The influence of environmental and dietetic factors in acne should be further explored.

Myxoid liposarcoma exhibits a peculiar clinical behavior, with a tendency to spread to serosal membranes, distant soft tissues, and bones, even in the absence of lung metastases. Therapy-related hematological neoplasms are well-known side effects of cytotoxic chemotherapy. We describe an exceptional case of metastatic myxoid liposarcoma of the spine associated with therapy-related refractory anemia with excess of blasts in a 37-year-old woman who underwent multi-agent chemotherapy for a myxoid liposarcoma of the left thigh. Microscopic examination of the bone marrow biopsy revealed dysplastic features, with abnormal localization of immature precursors and micromegakaryocytes, and islands of undifferentiated oval small/medium-size cells, suggestive of acute myeloid leukemia arising in the setting of a myelodysplastic syndrome. Immunohistochemistry was not discriminant. Cytogenetic analyses of bone marrow aspirate disclosed the presence of 2 different rearrangements, subsequently confirmed by fluorescent in situ hybridization and was crucial in making the correct diagnosis.

Dielectric behaviour of flux grown substituted crystals bearing composition SrGaxInyFe12−(x+y)O19 (x = 5, 7, 9; y = 0.8, 1.3, 1.0) is reported. Variation of dielectric constant (ɛ/), dielectric loss (tan δ) and ac conductivity (σac) with temperature in the range 30–500 °C under the frequency of the applied ac field in the range 1–104 kHz for all the varied compositions are analyzed and explained. Swift heavy ion irradiation effects on these parameters are investigated. On irradiation the crystals show changes in the values of dielectric constant (ɛ/), dielectric loss (tan δ) and ac conductivity (σac). The frequency variation of ɛ/ and tan δ is explained by Koop's phenomenological theory and hopping frequency of electrons between Fe2+ and Fe3+ on octahedral sites for both irradiated and unirradiated crystals.

The case we describe is, to our knowledge, the first primary epithelioid leiomyosarcoma of the endothoracic fascia reported in the literature. The endothoracic fascia is localized between the costal parietal pleura and the inner face of the thoracic wall and contains connective, adipose, smooth muscular tissues, lymphoid aggregates and vessels. Malignant tumors of the endothoracic fascia are difficult to identify because they quickly infiltrate the lung, the chest wall and the mediastinum. When discovered, these tumors are classified as primary soft tissue sarcomas of these sites. In the present case, early diagnosis of an asymptomatic lesion allowed us to establish its origin in the endothoracic fascia. The CT characteristics suggested a non-aggressive lesion, with clearly defined profiles and without signs of infiltration of the adjacent lung, fat and cartilages. Histological differential diagnosis and role of radiology in the management of the case are discussed. As radiological techniques spread and improve, we expect the diagnosis of endothoracic fascia primary tumors could become more frequent.

An in vitro antibiotic susceptibility assay for Staphylococcus aureus biofilms developed on 96-well polystyrene tissue culture plates was performed to elucidate the activity of the 27 residues cathelicidin peptide BMAP-28, quinupristin/dalfopristin (Q/D), linezolid, and vancomycin. Efficacy studies were performed in a rat model of staphylococcal CVC infection. Silastic catheters were implanted into the superior cava. Twenty-four hours after implantation the catheters were filled with BMAP-28. Thirty minutes later rats were challenged via the CVC with 1.0 × 10 6 CFU of S. aureus strain Smith diffuse. Administration of antibiotics into the CVC at a concentration equal to the MBC observed using adherent cells, or at a much higher concentration (1024 μg/mL) began 24 h later. The inhibition activities of all antibiotics against adherent bacteria were at least two-four-fold lower that against freely growing cells. When antibiotics were used in BMAP-28 pre-treated wells, they showed higher activities. The in vivo studies showed that when CVCs were pre-treated with BMAP-28 or with a high dose of antibiotics, biofilm bacterial load was reduced from 10 7 to 10 3 CFU/mL and bacteremia reduced from 10 3 to 10 1 CFU/mL. When CVCs were treated with both BMAP-28 and antibiotics, biofilm bacterial load was further decreased to 10 1 CFU/mL and bacteremia was not detected. These results suggest that CVC pre-treated with BMAP-28 represents an attractive choice for the treatment of device-related infections caused by staphylococci.

Zero-field μ SR experiments were performed on a series of polycrystalline samples of AA 3 ′ Mn 4 O 12 double perovskites ( A = Na + , Ca 2 + , La 3 + ; A ′ = Cu 2 + , Mn 3 + ) synthesized under high pressure. Spontaneous precession frequencies, of comparable magnitude to those previously reported in LaMnO 3 , were observed in all the family members except CaMn 7 O 12 . For all the samples, μ SR yielded determinations of the magnetic transition temperatures and of the order parameter in agreement with other techniques, but also revealed a peculiar phase separation in CaMn 7 O 12 , absent in the other compounds.

The 50 MeV Li 3+ ion irradiation induced modification on mechanical characteristics of flux grown strontium hexaferrite crystals of the type SrGa x In y Fe 12−( x + y ) O 19 (where x =0, 5, 7, 9 and y =0, 0.8, 1.3, 1.0) have been studied. Mechanical characteristics including Vicker's microhardness, density, fracture mechanics, crack propagation, brittleness index, yield strength of the crystals are assessed. Variation of microhardness with load is explained by using Hays and Kendall's law with the concept of Newtonian resultant pressure. The decrease in Vickers microhardness values of irradiated crystals is explained because of amorphization in the material. The cracks developed are classified into palmqvist and median types. Variation of load independent H v and density with Ga–In concentration are discussed. The average values of fracture toughness ( K c ), brittleness index ( B i ) and yield strength ( σ y ) are also determined.

We have carried out a comparative study of the low-temperature dependence of the magnetic penetration depth λ in (1) superconducting NbN films grown on R -plane sapphire and MgO substrates and (2) Bi2212 single crystals with different doping levels ranging from under- to over-doping. The NbN films display textured or epitaxial microstructure depending on growth conditions. The thermally activated dependence of λ ( T ) characteristic of conventional s-wave superconductors was found only in the epitaxial or thin textured NbN films. All thick textured films exhibit below 4 K a linear dependence Δλ ∼ T characteristic of a gapless superconducting state. This is quantitatively explained by a model of granularity-induced thermal phase fluctuations of the order parameter. As for Bi2212, we have found in all crystals a linear low-temperature dependence Δλ ab ∼ T in the ab -plane. The slope Δλ ab / ΔT displays a pronounced minimum ∼8 A/K at optimum doping. This value is in good agreement with the d-wave model proposed for cuprates. However, this model fails to account for the large slope values >40 A/K observed at both under- and over-doping. These values are quantitatively explained by the same model of phase fluctuations adopted for the textured NbN films, in spite of the difference between these two superconducting systems.

Neutron scattering has been used to study the magnetic correlations and the spin dynamics in the ferromagnetic lanthanum-manganite La0.75Ca0.25MnO3 above and below the Curie temperature. This material is known to exhibit colossal magnetoresistence behaviour, and its origin is strictly correlated to electron-lattice coupling. Quasielastic neutron scattering measurements show the presence of a diffusive central peak in the low-Q region (up to about 0.5 A−1) that persists above TC. The correlation length deduced from this contribution is about 8 A, the diffusion coefficient derived from the Q-dependence of the quasielastic broadening agrees with that expected for electron diffusion ‘assisted’ by lattice distortions.

Samples of Cu 1− x Re x Ba 2 YCu 2 O w , with nominal x =0, 0.05, 0.1 and 0.15, and 6.91⩽ w ⩽7.17 were prepared and characterized. The aim was to evaluate the possibility of improving the transport and magnetic properties of CuBa 2 YCu 2 O w (YBCO) by substituting Cu in the reservoir blocks (Cu1) with a high-valent element such as Re. X-ray powder diffraction and energy dispersive spectroscopy (EDS) studies indicated that Re substitutes for Cu1 up to x ≈0.1, beyond which it segregates, mostly by forming a cubic phase with a ≈8.275 A. Such an impurity was identified as YBa 2 ReO 6 on the basis of the powder x-ray and neutron diffraction data and EDS microanalysis. The crystal structure of Cu 1− x Re x Ba 2 YCu 2 O w was refined by the Rietveld analysis based on powder neutron diffraction data. It is orthorhombic (Pmmm space group), with a , b and c (≈3.84, 3.88 and 11.70 A) depending on x and w . The Re cations were found to occupy the (0 0 0) position and to be surrounded by 6 oxygen atoms at the vertices of an octahedron (2O1, 2O4 and 2O5). Such units do not significantly perturb the YBCO structure. No ordering between Cu and Re was observed by electron diffraction. The resistive and magnetic measurements indicated that the optimum oxygen doping of the Re-doped YBCO occurs at w >7. The corresponding Cu average valence is ≈2.27±1%. T c of the optimally doped samples is not very sensitive to the Re content and remains as high as ≈91 K when x =0.1. The residual resistivity extrapolated at T =0 K scales linearly with x , according to the Mathiessen’s rule. SQUID measurements for the x =0.05 and x =0.1 samples indicated that H irr and j c are lower than those of the undoped phase at any temperature.

We present a study performed on several LaMnO3+x/2 compositions. Detailed analysis around x=0.09 detects phase separation between a higher transition, Tc, and a lower one TN, with coexisting fluctuating and magnetically ordered clusters (the Neel point, TN, being the onset of an inhomogeneous non-collinear antiferromagnetic order detected by neutrons). The ordered clusters give rise to an extremely fast, static Kubo–Toyabe muon depolarization, while mixed static and dynamic local fields are detected inside the fluctuating clusters. Below TN precession frequency lines are resolved, despite the huge broadening introduced by localized Mn4+ ordered moments. A phase diagram is obtained and briefly discussed in the context of electronic phase separation.

The application of external pressure to cuprate superconductors induces increases in T c . The same cannot be stated for the chemical pressure, applied through substituting large cations with smaller ones, for example, Sr for Ba. Both types of pressure induce T c increases in only one system among the cuprates, namely La 2− x (Ba 1− y Sr y ) x CuO 4 . For this system, T c increases either for increasing y or for increasing the external pressure. By studying the crystal structure and the superconducting properties of several samples in the system Y(Ba 1− x Sr x ) 2 Cu 3 O w as a function of x and w , we show that in this system the substitution of Sr for Ba reduces the unit cell volume as does the external pressure. However, it also reduces the strain of the Ba/Sr layers that seem to be needed for optimizing the charge transfer between the chain and planar Cu cations. The strain of the Ba layer seems to scale well with T c , at least in the YBCO system.

Millimeter-size crystals of AlSr2YCu2O7 were synthesized by the flux method in alumina crucibles. Different mixtures of SrO:CuO (ranging from 25:75 to 12:88 mol ratio) were used as flux. No aluminum was added to the reagents. Depending on the flux composition and cooling rate, crystals different in morphology and size were obtained. The crystals appear to be tetragonal, space group P4/mmm. However, electron diffraction photographs, in agreement with Ramirez-Castellanos et al. (1), reveal that the symmetry is othorhombic with a cell a=2at, b=4at, and c=2ct (the subscript t refers to the YBCO tetragonal cell). The structural refinement was based on X-ray data collected with a KCCD Nonius diffractometer equipped with a graphite monochromator and AgKα radiation. One hundred and ninety-seven independent reflections with respect to the space symmetry quoted above, with I>3σ, were used. The arrangement is that of a YBCO structure in which the chain Cu cations have been replaced by Al. This substitution induces the movement of the oxygen atoms in the basal layer in such a way as to form, together with the apical oxygen atoms, corner-sharing zig-zag chains of oxygen tetrahedra centered around the Al cations and running parallel to the a-axis either tetragonally or orthorhombically. The b- and c-axes are quadrupled and doubled, respectively, because the zig-zag chains are out of phase perpendicularly to these directions. The additional distortion results in the shift of Al to xx0 (x≈0.056) from 000, O1 to xxz (x≈0.073) from 00z, and O3 to (0.5, y, 0) (y≈0.116) from (0.5, 0, 0).

The partial molar enthalpy of incorporation of oxygen (Δ ox H ) and enthalpy of formation (Δ f H ) have been measured via high-temperature reaction calorimetry for YBa 2 Cu 3− x M x O y (where M=Ni, Zn) in the composition range 0.0 x ox H and Δ f H values were nearly independent of dopant for both Ni and Zn, with enthalpies of oxidation in the range −86 to −110 kJ/(mol 1 2 O 2 ) and enthalpies of formation in the range −110 to −118 kJ/mol. The independence of thermodynamic behavior from superconducting properties is consistent with an electronic mechanism for T c suppression in Zn-doped YBCO superconductors. Oxygen contents, lattice parameters, and T c measurements are also reported.

We report zero-field μ SR experiments on two Ba 1 - x La x VS 3 samples ( x = 0 and 0.1 ). Muon spin rotation confirmed a magnetic ground state in both compounds, but also revealed for the stoichiometric material an ordering temperature much lower than any previously reported one. Along with the observed stabilization of magnetism in the electron-doped compound, this suggests that magnetism in the nominally undoped BaVS 3 is essentially an extrinsic property, possibly related to slight deviations from the stoichiometry, and that the perfectly pure material is non-magnetic.

In order to explain why Tc of all Hg-12(n-1)n compounds increases with pressure and decreases with chemical substitutions, the variation of the Hg-1223 structure as a function of external pressure is compared to that induced in the same structure by total substitution of Ba with Sr and partial substitution of Hg with Re. The introduction of Sr in the structure seems to induce all the structural changes needed to simulate the structure stable under pressure except the thickness of the superconducting blocks, which decreases with pressure and increases by Sr substitution. The Re substitution does not seem to play an important role except for the stability of the structure and seems to contribute to produce overdoped samples.

We describe a chemical method for determining the manganese concentration and average oxidation state in mixed-valence compounds, and in particular in complex La Mn perovskites. The method is based on two independent iodometric titrations, with amperometric dead-stop end-point detection. The oxygen stoichiometry and the molecular weight of the compounds can also be evaluated if secondary phases or multi-valent elements different from manganese are not present. The method was applied to the analysis of La Mn perovskites with the same cation stoichiometry but synthesized under different conditions. Significant variations of the Mn average valence were measured, which corresponded to different physical and structural properties of the materials.

A study of the magnetic properties of La 2 Cu 1 − x Zn x O 4 + δ (0 ≤ x ≤ 0.13; δ = 0.02) by means of magnetization measurements ( M vs T , H ) is reported. The Neel temperature of the 3D antiferromagnetic order (AF) is found to decrease with Zn substitution much more rapidly than in samples without excess oxygen. The effect of Zn substitution on the metamagnetic M vs. H transition is discussed, analyzing the temperature dependence of both the critical field H c and the weak ferromagnetic moment M s for different compositions x .