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We compared the immunogenetic data from 2666 patients affected by HLA-related autoimmune diseases with those from 4389 ethnically matched controls (3157 cord blood donors CBD, 1232 adult bone marrow donors BMD), to verify the appropriateness of HLA typing requests received in the past decade. The frequency of HLA-B*27 phenotype was 10.50% in 724 ankylosing spondylitis, 16.80% in 125 uveitis (3.41% BMD, 4.24% CBD,P<0.0001); HLA-B*51 allele was 15.57% in 212 Behçet’s disease (12.91% BMD, 9.88% CBD,P<0.0001); the HLA-DRB1-rheumatoid arthritis (RA) shared epitope was 13.72% in 554 RA (10.85% BMD, 13.48% CBD,P=0.016); the carriers of almost one of HLA-DQB1 susceptibility alleles were 84.91% in 795 celiac disease (CD) and 59.37% in 256 insulin-dependent diabetes mellitus (IDDM) (46.06% in 875 CBD, 42.75% in 662 BMDP<0.0001). Overall, our results show that the HLA marker frequencies were higher in patients than controls, but lower than expected from the literature data (excluding CD and IDDM) and demonstrate that, in complex immunogenetic conditions, a substantial number of genetic analyses are redundant and inappropriate, burdening to the public health costs. For this reason, we suggest the Italian Scientific Society of Immunogenetics to establish guidelines to improve the appropriateness of typing requests.

We report the diagnostic workup of a 75-year old woman seen in the outpatient clinic and complaining of a very common symptom that is walking difficulty and falls. We stress the complexity of the clinical examination and of the pathophysiology of equilibrium and gait disorders. They are numerous aetiologies that are very often associated in elderly patients. The therapeutic approach is necessarily multidisciplinary, both medical and paramedical. In the particular case of the patient illustrated in this vignette, the diagnosis was myofibrillar myopathy, a rare and recently described muscle disease. This is the occasion to stress that for many rare disorders, it is the disease, but not the signs or symptoms, which is rare.

In the context of the ASAI (Astrochemical Surveys At IRAM) project, we carried out an unbiased spectral survey in the millimeter window towards the well known low-mass Class I source SVS13-A. The high sensitivity reached (3-12 mK) allowed us to detect at least 6 HDO broad (FWHM ~ 4-5 km/s) emission lines with upper level energies up to Eu = 837 K. A non-LTE LVG analysis implies the presence of very hot (150-260 K) and dense (> 3 10^7 cm-3) gas inside a small radius ($\sim$ 25 AU) around the star, supporting, for the first time, the occurrence of a hot corino around a Class I protostar. The temperature is higher than expected for water molecules are sublimated from the icy dust mantles (~ 100 K). Although we cannot exclude we are observig the effects of shocks and/or winds at such small scales, this could imply that the observed HDO emission is tracing the water abundance jump expected at temperatures ~ 220-250 K, when the activation barrier of the gas phase reactions leading to the formation of water can be overcome. We derive X(HDO) ~ 3 10-6, and a H2O deuteration > 1.5 10-2, suggesting that water deuteration does not decrease as the protostar evolves from the Class 0 to the Class I stage., MNRAS Letters

The receptor for advanced glycation end-products (RAGE) is involved in the onset and progression of several inflammatory diseases. The RAGE primary transcript undergoes numerous alternative splicing (AS) events, some of which are species-specific. Here, we characterize the mouse-specific mRAGE_v4 splice variant, which is conserved in rodents and absent in primates. mRAGE_v4 derives from exon 9 skipping and encodes a receptor (M-RAGE) that lacks 9 amino acids between the transmembrane and the immunoglobulin (Ig) domains. RNA-Seq data confirm that in mouse lung mRAGE_v4 is the most abundant RAGE mRNA isoform after mRAGE, which codes for full-length RAGE (FL-RAGE), while in heart all RAGE variants are almost undetectable. The proteins M-RAGE and FL-RAGE are roughly equally abundant in mouse lung. Contrary to FL-RAGE, M-RAGE is extremely resistant to shedding because it lacks the peptide motif recognized by both ADAM10 and MMP9, and does not contribute significantly to soluble cRAGE formation. Thus, a cassette exon in RAGE corresponds to a specific function of the RAGE protein–the ability to be shed. Given the differences in RAGE AS variants between rodents and humans, caution is due in the interpretation of results obtained in mouse models of RAGE-dependent human pathologies.

Italy is host to a growing population of second-generation immigrants, children of immigrants born and raised in Italy. The current citizenship regime privileges Italian descent and makes naturalization very difficult, even for those born on Italian soil. This relegates a large population of second-generation immigrants to a marginal position, excluded from political, educational and labor integration upon their eighteenth birthday because of their citizenship status. Using data from group interviews, this paper investigates Italians' willingness to grant citizenship to second-generation immigrants. Three major themes emerged: how Italians define citizenship, the idea of conditional citizenship, and the significance of race in imagining the future of Italian society. This paper seeks to understand the dynamics underlying the debate about citizenship and gauge support for reforms to the current citizenship regime.

The objective of this study was to evaluate whether growth factors (FGF-2, FGF-4 and FGF-6) used alone or in combination with TGFβ2 are able to increase the proliferation and induce the differentiation of human bone marrow mesenchymal stem cells (hMSCs) to chondrocytes, with a view to using them in cartilage tissue engineering. Cells cultured in monolayer, used to test the activity of the growth factors on cell proliferation, showed that a combination of FGFs with TGFβ2 increases cell proliferation compared to cells cultured in control medium or in the presence of growth factors alone. The chondrogenic potential, evaluated in three-dimensional (3D) cell aggregates, showed that FGF-2 and FGF-6, when used in combination with TGFβ2 increased the size and glycosaminoglycan content of the cell aggregates without increasing cell number. Extracellular matrix (ECM) also showed higher collagen type II immunoreactivity, which was particularly evident in an area similar to a germinative pole that was observed only in pellets cultured with FGF-2 and FGF-6 combined with TGFβ2, or in pellets cultured with FGF-2 alone. Moreover, the RT-PCR assay has highlighted an increased expression of collagen type II and Sox9, used as gene markers for chondrogenesis. We can conclude that combinations of FGF-2 or FGF-6 with TGFβ2 may provide a novel tool to induce the differentiation of adult human mesenchymal stem cells for applications in cartilage tissue engineering. Copyright © 2011 John Wiley & Sons, Ltd.

The HH 212 protostellar system, in Orion B, has been mapped thanks to ALMA-Band 7 Cycle 1 and Cycle 4 observations of dueterated water (HDO) and acetaldehyde (CH$_3$CHO) emission with an angular resolution down to $\sim$0.15 arcsec (60 au). Many emission lines due to 14 CH$_3$CHO and 1 HDO transitions at high excitation ($E_{\rm u}$ between 163 K and 335 K) have been imaged in the inner $\sim$ 70 au region. The local thermal equilibrium analysis of the CH$_3$CHO emission leads to a temperature of 78$\pm$14 K and a column density of 7.6$\pm$3.2 $\times$ 10$^{15}$ cm$^{-2}$, which, when $N_{\rm H_2}$ of 10$^{24}$ cm$^{-2}$ is assumed, leads to an abundance of $X_{\rm CH_3CHO}$ $\simeq$ 8 $\times$ 10$^{-9}$. The large velocity gradient analysis of the HDO emission also places severe constraints on the volume density, n$_{\rm H_2}$ $\geq$ 10$^8$ cm$^{-3}$. The line profiles are 5--7 km s$^{-1}$ wide, and CH$_3$CHO and HDO both show a $\pm$ 2 km s$^{-1}$ velocity gradient over a size of $\sim$ 70 au (blue-shifted emission towards the north-west and red-shifted emission towards the south-east) along the disc equatorial plane, in agreement with what was found so far using other molecular tracers. The kinematics of CH$_3$CHO and HDO are consistent with the occurrence of a centrifugal barrier, that is, the infalling envelope-rotating disc ring, which is chemically enriched through low-velocity accretion shocks. The emission radius is $\sim$ 60 au, in good agreement with what was found before for another interstellar complex organic molecule such as NH$_2$CHO. We support a vertical structure for the centrifugal barrier, suggesting the occurrence of two outflowing, expanding, and rotating rings above and below (of about 40-45 au) the optically thick equatorial disc plane. It is tempting to speculate that these rings could probe the basis of a wind launched from this region., Comment: Astronomy & Astrophysics, in press

La recherche présentée s'est déroulée en trois phases. Une première recherche rétrospective a permis de constituer une population de référence composée de 18 enfants prématurés et qui bénéficient des soins usuels du service de néonatologie. Cette étude rétrospective analyse la durée et la forme de la transition entre une alimentation complètement passive (par sonde) et une alimentation entièrement active au sein ou au biberon. La CPAP influence cette évolution. Le deuxième groupe d'enfants prématurés (N = 24) a bénéficié d'une mise en forme en enroulement lors du nourrissement et d'un programme qui règle les quantités offertes en actif et en passif. L'effet de la CPAP est évalué. Le troisième groupe (N = 25) a bénéficié d'une mise en forme en enroulement lors du nourrissement, du même programme de transition mais en plus une stimulation périorale et orale est appliquée. L'effet de la CPAP est évalué. Dans les trois groupes, les mêmes analyses ont été réalisées. On montre un raccourcissement significatif et une homogénéisation des temps de transition. La CPAP nasale, si elle est présente plus de sept jours, a un effet négatif significatif, mais les stimulations périorales compensent cet effet délétère. De façon indépendante de l'âge de gestation, la vitesse d'ingestion montre que 90 % de ce que l'enfant est capable de prendre activement est absorbé durant les premières dix minutes. Ce fait permet d'adapter la gestion des repas : durée de l'alimentation passive et durée de l'offre du biberon ou du sein.

The distribution of the maximum annual three day snow fall depth H72, used for avalanche hazard mapping according to the Swiss procedure (Sp), is investigated for a network of 124 stations in the Alpine part of Switzerland, using a data set dating back to 1931. Stationarity in time is investigated, showing in practice no significant trend for the considered period. Building on previous studies about climatology of Switzerland and using an iterative approach based on statistical tests for regional homogeneity and scaling of H72 with altitude, seven homogenous regions are identified. A regional approach based on the index value is then developed to estimate the T-years return period quantiles of H72 at each single site i, H72i(T). The index value is the single site sample average μH72i. The dimensionless values of H*72i=H72i / μH72i are grouped in one sample for each region and their frequency of occurrence is accommodated by a General Extreme Value, GEV, probability distribution, including Gumbel. The proposed distributions, valid in each site of the homogeneous regions, can be used to assess the T-years return period quantiles of H*72i. It is shown that the value of H72i(T) estimated with the regional approach is more accurate than that calculated by single site distribution fitting, particularly for high return periods. A sampling strategy based on accuracy is also suggested to estimate the single site index value, i.e. the sample average μH72i, critical for the evaluation of the distribution of H72i. The proposed regional approach is valuable because it gives more accurate snow depth input to dynamics models than the present procedure based on single site analysis, so decreasing uncertainty in hazard mapping procedure., Natural Hazards and Earth System Sciences, 8 (4), ISSN:1561-8633, ISSN:1684-9981

Severe deficiency of arylsulphatase A (ARSA) activity was detected in a boy with delayed psychomotor development, coarse face and liver enlargement when he was aged 2. The case does not fit into the description of the Metachromatic leukodystrophy (MLD) proposed by Hagberg (1963) mainly because he did not deteriorate. Twelve years of follow up did not show any signs of decerebration or further intellectual decline; only speech was, and still is, absent. The reduced ARSA activity in leukocytes and fibroblasts of the father of the propositus suggests that he is heterozygote and that the ARSA deficiency of the propositus is inherited. The ARSA activity of the mother overlaps with the lowest control. The assumption that the mother is a carrier of different mutations could explain the discrepancy between the clinical and biochemical findings of the patient. This is a further family in which mutations other than the typical one can be postulated to explain variable clinical or biochemical pictures of MLD.

The effect of a new three-step anodic spark deposition process, labeled TiSpark, including two consecutive treatments performed first in a P solution and second in Ca solution, followed by an additional alkali etching step, was investigated for the improvement of osseointegration of commercial grade 2 titanium, machined (Ti) or Al2O3 sandblasted (Ti-SA), cylindrical implants (12 mm in length and 4 mm in diameter) in cortical bone of 12 adult sheep. Histomorphometric and microhardness measurements were carried out at each experimental time (4, 8, and 12 weeks) to quantify the bone-to-implant contact around the implants as well as the newly bone hardness and bone maturation index. TiSpark treated surfaces were covered by a thick layer of crystalline anatase TiO2 and by a further Ca/P layer. Bone tissue extends and grows on the surface of the TiSpark treated implants without any fibrous tissue, enhancing the short-term osseointegration properties of implant. Bone mineralization rate was also influenced by the chemical composition of implants and sandblasted materials presented the lowest bone maturation rate at the interface. Data suggests that the TiSpark treatment produces a modification of the Ti surface, which presents good bioactivity and may be suitable for achieving a stable implant osseointegration. © 2007 Wiley Periodicals, Inc. J Biomed Mater Res, 2008

This is the first of three companion papers that summarize the theoretical and experimental work carried out to develop a prototype smart panel with 16 decentralized vibration control units for the reduction of sound radiation/transmission. The smart panel is made of a thin aluminium plate with 16 closely spaced accelerometer sensor and piezoceramic actuator transducer pairs connected by single-channel velocity feedback controllers (i.e., active damping units). In this paper a preliminary theoretical study is carried out to assess the behaviour of the smart panel when it is mounted on the top of a rectangular cavity with rigid walls. The smart panel is excited either by the acoustic field produced in the cavity by a monopole source or by a transverse point force. The simulations carried out have shown that for both the acoustic and the force sources, good reductions of the averaged kinetic energy or total sound power radiation can be achieved within a band 0– 2 kHz . The theoretical study is preceeded by a general review of the development of smart panels for the control of sound radiation/transmission. In particular, the various approaches developed for the design of sensors and actuators are analyzed with reference to the control of tonal disturbances using feed-forward controllers and the control of stationary random disturbances using feedback controllers.

This is the third of three companion papers that summarize the theoretical and experimental work carried out to develop a prototype smart panel with 16 decentralized vibration control units for the reduction of sound transmission/radiation. In this paper the control effectiveness of the smart panel has been assessed experimentally by measuring, in a anechoic chamber, the reduction of its total sound power radiation when it is mounted on a Perspex box with very thick and rigid side walls. The panel has been excited either by the acoustic field produced by a loudspeaker placed in the Perspex box or directly by a point force generated with a shaker. The vibration averaged over the panel surface has also been measured with a laser vibrometer. Laser measurements have also been taken at some specific frequencies, in such a way as to highlight the spatial vibration of the panel with the 16 decentralized control units. The various tests carried out have shown that the 16 control units can produce good reductions of the radiated sound power and averaged vibratory field over the panel surface, particularly when the panel is excited by the shaker. Indeed, when the panel is excited by the shaker, reductions of the sound radiation were measured in all the third octave bands in the frequency range 0–5 kHz and it was found that there were at least seven bands with reductions between 5 and 8 dB. Also, the averaged vibration field over the panel surface was found to be completely damped down in a frequency range between 0 and 1 kHz with reductions of about 10 dB in correspondence to all resonance frequencies.

Objectives: The aim of the present study was to evaluate the long-lasting efficacy of a combined surgical protocol, using immediate implant and subepithelial connective tissue graft for single-tooth replacement. The advantages of this single-center, longitudinal, randomized, blind examiner research were the following: preservation of both keratinized mucosa amount and bone tissue, optimal peri-implant marginal sealing, satisfactory aesthetic results, reduction in treatment time. Materials and methods: In the time period from 1990 to 1998, 116 patients were consecutively admitted for treatment with a total of 116 solid screw ITI-implants supporting single crowns. Ninety-six patients underwent the proposed combined treatment (test group), while 20 received only single immediate implants (control group). The observation time extended from 1 up to 9 years. Results: The 9-year cumulative survival rate was 100% for both test and control groups. Comparative statistical analysis of soft and hard tissue peri-implant parameters demonstrated better results in the test group than in the control during every single 3-year analysis and especially in the last observation interval. The test group also showed very good results in terms of aesthetic parameters, which estimated the keratinized mucosa width, the alignment of crown emergence profile and the patient's satisfaction. Conclusion: Single-tooth replacement by immediate solid screw ITI implants in association with connective tissue autograft was demonstrated to be a predictable procedure. Moreover, this treatment can be considered as a sure system to reach an excellent functional and harmonious aesthetic restoration.

Objective: This study was conducted to test the hypothesis that locomotion can improve cognitive development in a paediatric population with meningomyelocele (MMC) and hydrocephalus. Methods: Twenty-nine children with MMC and shunted hydrocephalus were studied. All had motor impairment, but after physiotherapy and training walking was possible in 23 of them (5 autonomously and 18 with an aid), while 6 had recourse to a wheelchair. In all 23 cases neuroimaging (TC scan and/or MRI) was performed to obtain data on the sequelae of perinatal lesions, alterations of the corpus callosum, and the presence or absence of cortical abnormalities and ventricular dilatation. All subjects underwent a neuropsychological assessment including the Wechsler Pre-school and Primary Scale for Infants (WIPPSI) and the Wechsler Intelligence Scale for Children Revisited (WISC-R). Statistical analysis was carried out with the ANOVA test. Results: Even though global I.Q. in these MMC children was within the normal range, the characteristic splitting between verbal I.Q. (VIQ) and performance I.Q. (PIQ) was observed, but the most interesting observation was a significant performance-related difference (P=0.044 and P=0.012) between ambulatory patients (both with and without aids) and those who were dependent on wheelchairs (PIQ: 83–85 vs 63).

We present analytical solutions for the relative permeabilities governing a Darcy scale description of three-phase flow of immiscible fluids. We consider flow taking place within a capillary tube with circular cross-section for two settings corresponding to (a) a water wet and (b) an oil wet configuration. Momentum transfer between the moving phases, which leads to the phenomenon of viscous coupling, is explicitly accounted by imposing continuity of velocity and shear stress at the fluid-fluid interfaces. The macroscopic model describing the system at the Darcy scale includes three-phase effective relative permeabilities, Kij,r, accounting for the flow rate of the ith-phase due to the presence of the jth-phase. These effective coefficients are function of phases saturation, fluids viscosity and wettability of the medium. Our results show that (i) the relative permeability Kii,r of the wetting phase is only a function of its own saturation while Kii,r of the non-wetting phases depend on the saturations of all the fluids; (ii) viscous coupling effects (elucidated by Kij,r with i ≠ j) can be relevant in water wet and oil wet systems.

Human cytomegalovirus (HCMV) is a ubiquitous beta human herpesvirus able to influence infected cell survival and proliferation and to modulate the host immune response. As there is accumulating evidence that HCMV is detected in primary intracranial astrocytic tumours, in this study we looked for the presence of HCMV in intracranial tumours and tried to correlate this eventual presence with the anti-HCMV systemic immunoreactivity and with the detection of HCMV in peripheral blood.In this study, we analysed 43 glioblastomas (GBM), 14 oligodendrogliomas (OL) and 20 meningiomas (MG) by immunofluorescence (IF) targeting HCMV immediate early antigen (IE1) and by nested PCR (nPCR) amplifying HCMV glycoprotein B (gB).Detection of IE1 by IF showed the presence of HCMV in 70% of GBM, 57% of OL and 85% of MG, in contrast to gB nPCR, which detected HCMV in only 50% of GBM, 38% of OL and 46% of MG. Unexpectedly, HCMV DNA and antigens were detected within GBM, OL and MG of patients that exhibit negative viral serology. More surprisingly, PCR on the peripheral blood did not detect HCMV in patients with a HCMV-positive tumour.Our results are in agreement with previous observations demonstrating HCMV in glial tumours and highlight the presence of HCMV in meningiomas. We also showed that anti-HCMV specific systemic immunoreactivity and detection of HCMV in peripheral blood are not predictive of HCMV presence in primary intracranial tumours.

Signals released by leukocytes contribute to tumor growth and influence the efficacy of antineoplastic treatments. The outcome of peritoneal carcinomatosis treatments is unsatisfactory, possibly because chemotherapy activates events that have in the long-term deleterious effects. In this study we offer evidence that 5-fluorouracile (5-FU), besides provoking apoptosis of MC38 colon carcinoma cells, induces a striking attraction of leukocytes both in an orthotopic model of colon carcinomatosis in vivo and in monocyte-migration assays in vitro. Leukocyte attraction depends on the presence of High Mobility Group Box 1 (HMGB1), an endogenous immune adjuvant and chemoattractant released by dying cells. Leukocyte recruitment is prevented in vivo and in vitro using blocking antibodies against HMGB1 and its competitive antagonist BoxA or by interfering with HMGB1 expression. Autophagy is required for leukocyte chemoattraction, since the latter abates upon pharmacological blockade of the autophagic flux while activation of autophagy per se, in the absence of death of colon carcinoma cells, is not sufficient to attract leukocytes. Our results identify autophagy induction and HMGB1 release in colon carcinoma cells as key events responsible for 5-FU elicited leukocyte attraction and define a novel rate-limiting target for combinatorial therapies.

The mathematical modeling of the NF-κB oscillations has attracted considerable attention in recent times, but there is a lack of simple models in the literature that can capture the main features of the dynamics of this important transcription factor. For this reason we propose a simple model that summarizes the key steps of the NF-κB pathway. We show that the resulting 5-dimensional dynamical system can reproduce different phenomena observed in experiments. Our model can display smooth and spiky oscillations in the amount of nuclear NF-κB and can reproduce the variety of dynamics observed when different stimulations such as TNF-α and LPS are used. Furthermore we show that the model can be easily extended to reproduce the expression of early, intermediate and late genes upon stimulation. As a final example we show that our simple model can mimic the different transcriptional outputs observed when cells are treated with two different drugs leading to nuclear localization of NF-κB: Leptomycin B and Cycloheximide.

We described an unusual clinical phenotype of three siblings, one male and two females aged 25, 24 and 16 years respectively, of first-degree cousins Moroccan parents; another brother and sister were not affected. At the age of three years for the young sister and six years for the two older, they presented with acute appearance of an inflammatory clinical phenotype characterized by joint stiffness, redness and pain, associated with rapidly evolution of proximal muscle weakness leading to difficulties in walking, arising from the floor and running. CK blood levels were normal. The diagnosis of juvenile polyarthritis was proposed and the joints inflammatory phenotype successfully treated with TNF α inhibitor. A muscle biopsy was performed in the oldest and the youngest at 3 and 7 years respectively from the onset of symptoms. The biopsy revealed a granulomatous myositis with CD68 and CD3 positive inflammatory infiltrate and muscular necrosis. Mycobacterium tuberculosis and non tuberculosis were excluded by PCR as were other sporadic causes of granulomatous myositis such as sarcoidosis, infection and neoplastic diseases. Thirteen to nineteen years after the symptoms, all three siblings show joint contractures especially in proximal limb segment as well as in the shoulders, hips and knees. Atrophy of the distal muscles of the lower limbs is clearly noticeable, without muscle weakness. Recently, the younger sister have been investigated by muscular MRI that showed an intramuscular edema of the thigh and twins muscles. Recently, mutations in different genes associated with Mendelian transmission of dysimmunity disease have been demonstrated, and it is likely that this until now unreported association belongs to this emerging group of diseases.

Abstract: The solution structure of the wild-type A box of rat HMG1 has been determined by heteronuclear three-dimensional NMR and molecular modelling. This structure adopts a "L" shape structure globaly similar to that described for a mutant A box and other HMG boxes. However the wild-type A box contains two Cys residues which could form intramolecular bridge in an oxidative environment. This should be into competition with a contribution of the thiol groups in the binding ofHMG proteins to cisplatin-modified DNA.

This study was performed to describe the impact of preventive options on the psychological condition of BRCA1/BRCA2 carriers. A sample of 52 cancer-affected (C-A) and 27 cancer-unaffected (C-UN) women were enrolled after gene test disclosure (T0). Psychological evaluations were performed at T0 and 15 months later (T1). The surgical options were more likely to be chosen in C-A women (62%), although a consistent proportion of C-UN women (30%) also opt for these preventive measures. At the baseline, both samples had average anxiety and depression scores below the cut-off value, restrained average cancer worry scores and a risk perception consistent with the risk percentage provided during genetic counselling. The longitudinal results indicated no clinically meaningful variations in the anxiety and depression scores in either of the two samples. Statistically significant reductions in cancer-risk perception emerged in women who chose surgery in both C-A and C-UN women. In BRCA1/BRCA2 mutation carriers, surveillance does not influence their initial psychological condition, whereas prophylactic surgery has a significant impact in reducing the perceived risk and worry about getting sick. C-A and C-UN women have to be considered as two separate populations of BRCA mutation carriers requiring personalized approaches to risk management.

Purpose This work aimed to study and compare the behavior of macrophages and human osteoclast-like cells cultured in contact with four commercial bone substitute materials used as graft for ridge augmentation techniques. Two materials are of natural origin and two are synthetic bone substitutes. Methods Macrophage activation and cytokine release were assessed using SEM analysis and a sandwich ELISA kit while the activity of human osteoclast-like cells was studied quantifying calcium released from the substrata studied together with SEM analysis of cell morphology and pit formation on the bone substitutes. Results Hydroxyapatite proved to be the greatest macrophage activator, while the bovine derived material turned out to be the only bone substitute that does not induce macrophages to release IFNγ. No material had direct induction of osteoclast precursor differentiation even if mature osteoclasts showed the highest activity on the human derived material followed by bone ceramic, while the bovine derived material was the substrate with the least osteoclast activity. Conclusions The four materials reacted differently with the cells tested and between them, the bovine derived bone, being the lowest macrophage and osteoclast activator may be considered a good bone substitute for clinical situations requiring greater time of permanence of the material at the site of implantation, while human derived bone will be more suitable for clinical applications that require lower time of permanence.

Quebec City - Quebec - Canada

Sodium cyanoborohydride in the presence of zinc iodide is found to reduce epimeric tertiary alcohols, whose carbinol group is a stereogenic center in a a stereoconvergent way

We set out to describe 17 patients with septo-optic dysplasia (SOD), focusing on the little-explored neurological, cognitive, and neuro-ophthalmological components. A further aim was to identify possible clinical correlations and phenotypic characteristics within the diagnostic spectrum.We collected clinical-instrumental data (from the history, general and neurological examination, developmental assessment, and neuro-ophthalmological, neuroradiological, neurophysiological, and endocrinological evaluations) on nine males and eight females (mean age 34.4mo, SD 31.6; range 4mo-9y 6mo) diagnosed with SOD who were referred to our Centre of Child Neuro-ophthalmology between 1999 and 2010.We observed a heterogeneous clinical spectrum characterized by nervous system, visual, and endocrine dysfunctions; optic nerve involvement was present in all 17 children, midline brain defects in 14, and cortical developmental malformations in seven. Developmental/cognitive delay and relational and communication difficulties were observed in eight and seven children, respectively, and reduced visual acuity and oculomotor dysfunction were observed in all. Pituitary hormone deficiencies were present in nine children.Nervous system involvement emerged as a key feature of SOD. As part of a holistic approach to the disease, particular attention should be paid to this aspect. The emergence of new clinical correlations and correlations between clinical features and three SOD subtypes opens the way for better clarification of this disease and, therefore, more targeted diagnosis, follow-up, and care of affected children.

Background Titanium and its alloy represent the most commonly used biomaterials worldwide designed for bone-contact under-load applications, which often require specific mechanical properties. In particular, a large number of different biomimetic surface treatments have been developed to speed up the osteointegration process, which facilitates a reduction in recovery time. Purpose The aim of this work is to investigate the physical-chemical, mechanical and bioactivity properties of an innovative biomimetic treatment on titanium performed using Anodic Spark Deposition (ASD) electrochemical treatment. Methods The proposed Asd treatment was obtained in an electrochemical solution containing silicon, calcium, phosphorous and sodium followed by an alkali etching. Surface morphology was characterized using SEM and laser profilometry. Chemical and structural composition was assessed by EDS, ICP/OES and XRD analysis. Vickers micro hardness and static contact angle measurements were performed to assess the surface mechanical properties and wettability. Results The proposed anodization treatment was capable of providing a chemical and morphologic modified titanium oxide layer, adherent and characterized by superhydrophilic properties. The microporous morphology was enriched by calcium, silicon, sodium and phosphorous. After incubation in Kokubo's simulated Body Fluid (sBF) the treatment showed very high mineralization potential compared to the reference surfaces, accounting for a deposited hydroxyapatite layer as thick as 12 μm after 14 days of SBF incubation. Conclusions on the basis of the results obtained in this study, we believe that the novel silicon-based ASD biomimetic treatment represents a promising treatment capable of enhancing the osteointegration of titanium for dental and orthopedic applications.

Two oscillatory episodes took place in the Argentinean power system at 22:20 and 22:50, June 7th, 2008. The first one started when a 40 MVAr capacitors bank was disconnected and the second one started when a 500 kV line was disconnected. Both oscillatory episodes produced great variation of active and reactive power in several 330 kV and 500 kV power system nodes. At first sight, these oscillations seemed inter-area electromechanical oscillations, but carried out studies suggest that this oscillatory behavior could be a consequence of interaction between Reactive Power Control of generators and the ac-dc Converter Control of high power loads.

The aim of the present study is to describe end-of-life preferences of advanced cancer patients willing to talk about death issues. Eighty-eight advanced cancer patients were interviewed through End of Life Preferences Interview (ELPI), a 23-item interview covering a wide range of end-of-life care issues. Most interviewed subjects were home care patients and their median survival after ELPI administration was 69 days. In total, 100% of responders expressed the will to receive some kind of information on the disease process and/or the treatments proposed. Approximately 77% declared to be willing to talk about what it is important at the end of life in case of worsening of their conditions and 31% prefer to be left alone in difficult moments. Approximately 67% choose home as the preferred place of death and 63% think it is preferable to die in a state of unconsciousness induced by drugs. About half of responders declare to believe in any kind of life after death and 40% consider very important to find any meaning at the end of life. ELPI can be a useful instrument to adapt the model of care to the specific needs and values of each patient.

On the assumption that EBV integration into the genome of human B lymphocyte might lead to the inactivation of a hypothetical gene determining the limited duplicative capacity and consequently participate to the cell immortalization, a search for the human-virus junction was done. This led to the identification of a site of integration in the central part of the heavy chain of the immunoglobulin region. The coincidence of the involvement of the site in lymphomatogenesis with the first complete characterization of an integration site led to the speculation that the heavy chain gene itself might be an important controller of cell duplication in the B lymphocyte.

Inflammatory processes in brain tissue have been described in human epilepsy of various aetiologies and in experimental models of seizures. This, together with the anticonvulsant properties of anti-inflammatory therapies both in clinical and in experimental settings, highlights the important role of brain inflammation in the aetiopathogenesis of seizures. Preclinical investigations in experimental models using pharmacological and genetic tools have identified a significant contribution of interleukin-1 (IL-1) type 1 receptor/Toll-like receptor (IL-1R/TLR) signalling to seizure activity. This signalling can be activated by ligands associated with infections (pathogen-associated molecular patterns) or by endogenous molecules, such as proinflammatory cytokines (e.g. IL-1beta) or danger signals [damage-associated molecular patterns, e.g. high-mobility group box 1 (HMGB1)]. IL-1beta and HMGB1 are synthesized and released by astrocytes and microglia in the rodent brain during seizures. Notably, a rapid release of HMGB1 from neurons appears to be triggered by proconvulsant drugs even before seizure occurrence and is involved in their precipitation of seizures. The activation of IL-1R/TLR signalling mediates rapid post-translational changes in N-methyl-d-aspartate-gated ion channels in neurons. A long-term decrease in seizure threshold has also been observed, possibly mediated by transcriptional activation of genes contributing to molecular and cellular plasticity. This emerging evidence identifies specific targets with potential anticonvulsant effects in drug-resistant forms of epilepsy.

In recent years, the interest in the research on energy production systems fed by biofuels has increased. Gaseous fuels obtained through biomass conversion processes such as gasification, pyrolysis and pyrogasification are generally defined as synthesis gas (syngas). The use of synthesis gas in small-size energy systems, such as those used for distributed micro-cogeneration, has not yet reached a level of technological maturity that could allow a large market diffusion. For this reason, further analyses (both experimental and numerical) have to be carried out to allow these technologies to achieve performance and reliability typical of established technologies based on traditional fuels. In this paper, a numerical analysis of a combustor of a 100-kW micro gas turbine fed by natural gas and biomass-derived synthesis gas is presented. The work has been developed in the framework of a collaboration between the Engineering Department of the University of Ferrara, the Istituto Motori - CNR (Napoli), and Turbec S.p A. of Corporeno di Cento (FE). The main features of the micro gas turbine Turbec T100, located at the Istituto Motori - CNR, are firstly described. A decompression and distribution system allows the feeding of the micro gas turbine with gaseous fuels characterized by different compositions. Moreover, a system of remote monitoring and control together with a data transfer system has been developed in order to set the operative parameters of the machine. The results of the tests performed under different operating conditions are then presented. Subsequently, the paper presents the numerical analysis of a model of the micro gas turbine combustor. The combustor model is validated against manufacturer performance data and experimental data with respect to steady state performance, i.e., average outlet temperature and emission levels. A sensitivity analysis on the model capability to simulate different operating conditions is then performed. The combustor model is used to simulate the combustion of a syngas, composed of different ratios of hydrogen, carbon monoxide, methane, carbon dioxide and water. The results in terms of flame displacement, temperature and emission distribution and values are analyzed and compared to the natural gas simulations. Finally, some simple modifications to the combustion chamber are proposed and simulated both with natural gas and syngas feeding.