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Background Recent standards of care mention chest radiography (CR) but not chest computed tomography (CT) in routine annual follow-up of children with cystic fibrosis (CF). To minimise radiation risk, CT or CR should only be performed if they impact clinical decision making. We investigated whether in addition to a wide range of commonly used clinical parameters, chest CT and/or CR in routine follow-up of CF patients influence clinical decisions. Methods 36 web based clinical vignettes (i.e. case simulations) were designed using clinical data from patients aged 8–18 years, randomly selected from two CF centres in The Netherlands. In a randomized cross-over design, clinicians assessed eight vignettes and suggested therapeutic/diagnostic management on two occasions, with a ten-week interval. Radiological information (CT or CR) was included at only one of the two assessments, in random order. Any differences in management could be attributed to information from CT or CR, and were compared by McNemar analysis. Results 44 European and Australian clinicians completed a total of 143 CT vignette pairs and 167 CR vignette pairs. CT was associated with a significant increase in antifungal treatment (Risk Ratio (RR) 2.8 (1.3–6.0, p = .02)), bronchoscopies (RR 1.6 (1.1–2.5, p = .04)), mycobacterial cultures (RR 1.3 (1.0–1.5, p = .02)), and ‘need for hospitalization’ (i.e. intravenous antibiotics and/or bronchoscopy) (RR 1.4 (1.0–1.9, p = .03)). CR led to a significant increase in inhaled antibiotics only (RR 1.3 (1.0–1.6, p = .04)). Conclusions CT but not CR, at routine biennial follow-up was associated with several changes in treatment and/or diagnostic testing, including the need for hospitalization.

An extensive wind tunnel test campaign devoted to the characterization of the aerodynamic and aeroacoustic behaviour of the new Space Launcher VEGA-C has been carried out in the trisonic wind tunnel available at the National Institute for Aerospace Research (INCAS) in Bucharest. The present paper summarizes the main results of the aeroacoustic investigation with a specific focus on the buffeting analysis. For this reason, the results presented herein are limited to the transonic conditions which are the most critical in terms of occurrence of flow instabilities. The scaled instrumented model of the VEGA-C launcher has been equipped with flush mounted Kulite sensors that provided the distribution of the wall pressure fluctuations. Pressure measurements at the wall of the wind tunnel have been carried out as well, with the scope of measuring reference signals to be used for cleaning the model pressure data from the background noise. Numerical simulations have been also performed to facilitate the physical interpretation of the experimental outcomes by qualitatively identifying the position of the shockwaves and tracking their evolution along the launcher for increasing Mach number. Data processing provides temporal statistics of the wall pressure signals as well as spectral quantities that give clear indications about the absence of buffeting.

Oxidative depolymerization of lignin obtained from pine and willow can be achieved in a novel system encompassing the ionic liquid (IL) 1-butylimidazolium hydrogensulfate coupled with a vanadium based polyoxometalate (POM) under oxygen rich conditions. Along with an array of phenols and functionalized aromatics, vanillin and syringaldehyde were the main products extracted from the IL. The overall yield of aldehyde products were shown to be higher on lignin samples obtained with shorter pretreatment times, with vanillin being the exclusive aldehyde product obtained from pine. In the presence of molecular oxygen, the highest yield of aldehyde products was obtained when 5 wt % of the POM relative to the IL was employed and constituted the major product in the extracted oils. This system succeeds in exploiting the ability of ILs to depolymerize lignin and the remarkable properties of the POM to oxidize the lignin fragments into useful platform chemicals.

Acidic anions of ionic liquids have been demonstrated as efficient catalysts for the cleavage of the β-O-4 ether linkage prevalent in the lignin superstructure. Through the use of lignin model compounds with varying functionality and by monitoring reaction kinetics, a full mechanistic investigation into the hydrolysis of the β-O-4 linkage in acidic ionic liquid solutions is reported. Hammett acidities are reported for different 1-butyl-3-methylimidazolium hydrogen sulfate [C4C1im][HSO4] ionic liquid systems with varying acid and water concentrations and were correlated to substrate reactivity. Results show that the rate of ether cleavage increases with an increase in acidity and the initial dehydration of the model compound is the rate-determining step of the reaction. The Eyring activation parameters of the reaction in hydrogen sulfate ionic liquids with a variety of cations are reported, indicating a consistent E1 dehydration mechanism. Hydrogen bonding in protic ionic liquids was shown to significantly influence anion–cation interactions, consequently altering the solvation of the protonated starting material and therefore the overall rate of reaction. Comparison of reaction rates in these ionic liquids with results within aqueous or aqueous/organic media indicate that the ionic liquids facilitate more rapid cleavage of the β-O-4 ether linkage even under less acidic conditions. All the reported results give a complete overview of both the mechanistic and solvation effects of acidic ionic liquids on lignin model compounds and provide scope for the appropriate selection and design of ionic liquids for lignin processing.

Riassunto Obiettivi Valutare le possibili associazioni tra inclusione canina e specifiche lesioni secondarie. Materiali e metodi E stato analizzato un campione di 74 pazienti con canino mascellare incluso, diagnosticato clinicamente e attraverso lo studio dei modelli, delle fotografie intraorali e dell’ortopantomografia pretrattamento, in stadio di maturazione CS2-CS4. E stata valutata radiograficamente la presenza delle seguenti lesioni: pericoronite e cisti follicolare, inclusione e riassorbimento radicolare di denti adiacenti al canino. Risultati La prevalenza delle lesioni associate all’inclusione del canino mascellare e stata la seguente: pericoronite 42,202%; cisti follicolare 22,018%; riassorbimento radicolare 33,027%; inclusione di denti contigui 1,835%. Conclusioni Una diagnosi precoce di inclusione canina e di fondamentale importanza per le possibili complicanze associate.

We have identified elements present in the ionic liquid–vacuum outer atomic surface of 23 ionic liquids using high sensitivity low-energy ion scattering (LEIS), a very surface sensitive technique. We show that the probability of cationic heteroatoms being present at the ionic liquid–vacuum outer atomic surface is very low; we detected imidazolium nitrogen for only one of the 18 imidazolium based ionic liquids investigated, no nitrogen for the two ammonium based ionic liquids and a very small amount of phosphorus for two of the three phosphonium-based ionic liquids. We determine that the anion is always present at the ionic liquid–vacuum outer atomic surface, even for very large cations containing dodecyl alkyl chains or longer; these chains dominate the ionic liquid–vacuum outer atomic surface, but are not sufficiently densely packed to completely cover the anions. We demonstrate the presence of strong hydrogen bond acceptor adsorption sites at the ionic liquid–vacuum outer atomic surface. We demonstrate that the amount of ion present at the ionic liquid–vacuum outer atomic surface can be tuned by varying the size of the other ion; larger cations (or anions) occupy more of the ionic liquid–vacuum outer atomic surface, leaving less room for anions (or cations). By identifying elements present at the ionic liquid–vacuum outer atomic surface, conclusions can be drawn on the orientations of anions nearest the vacuum. We show that for five different anions there is a most probable ion orientation, but other anion orientations also exist, demonstrating the presence of multiple anion orientations. The imidazolium cations nearest to the vacuum also show similar multi-orientation behaviour. This variety of atoms present and therefore ion orientations is expected to be central to controlling surface reactivity. In addition, our results can be used to quantitatively validate simulations of the ionic liquid–vacuum surface at a molecular level. Overall, our studies, in combination with literature data from different techniques and simulations, provide a clear picture of ionic liquid–vacuum outer atomic surfaces.

The results in this paper arise from an investigation into control strategies designed to trap a vortex in a cavity such that the flow remains attached with no large scale vortex shedding. Progress in this general area is required to advance the development of thick winged aircraft where, for example, such wings could be used to store more fuel. A discrete vortex computational dynamics model of the flow is used to develop simple but effective control strategies. In this paper, the use of constant suction or a PI controller to stabilize the flow, followed by oscillatory open loop suction to maintain stability with a significantly reduced effort is investigated. To support the simulation studies, results from wind tunnel experiments are given.

Willow biomass was subjected to different pretreatment conditions with triethylammonium hydrogen sulfate as solvent, and the produced lignin solutions were treated by oxidation either homogeneously using H2O2 as oxidant or by heterogeneous catalysis using TiO2. Lignin, residual lignin, oil, and the recovered ionic liquid (IL) were characterized in order to determine the effects of each treatment. Lignin was successfully extracted and depolymerized by oxidation and characterized by ATR-IR, HPSEC, and py-GCMS. The obtained oil was characterized by GCMS; it was composed mainly of acids derived from the sugar and lignin fractions, the TiO2 catalyzed oils being richer in phenolic derived compounds than sugar fractions. The final ionic liquid was characterized in order to determine its suitability to be reutilized.

Background: Currently no tools to predict risk of acute (AP) and recurrent pancreatitis (ARP) in children with cystic fibrosis (CF) are available. We assessed the prevalence of AP/ARP and tested the potential role of Pancreatic Insufficiency Prevalence (PIP) score in a cohort of children with CF. Methods: We identified two groups of children, on the basis of presence/absence of AP/ARP, who were compared for age at diagnosis, clinical features, genotypes and sweat chloride level. PIP score was calculated for each patient. Results: 10/167 (5.9%) experienced at least one episode of AP during follow up; 10/10 were pancreatic sufficient (PS). Patients with AP/ARP showed a PIP score ≤ 0.25 more frequently (6/10) than patients without AP/ARP. The odds ratio (95% CI) of developing pancreatitis was 4.54 (1.22-16.92) for patients with PIP < 0.25 when compared with those who have a PIP score > 0.25 (p 0.0151). PIP score was correlated with sweat chloride test (p < 0.01). Conclusion: PIP score, PS status and normal/borderline sweat chloride levels could be applied to predict pancreatitis development in children with CF. ARP could lead to pancreatic insufficiency. © 2014 European Cystic Fibrosis Society

Recent technological advancements have made the use of particle image velocimetry (PIV) more widespread for studying turbulent flows over a wide range of scales. Although PIV does not threaten to make obsolete more mature techniques, such as hot-wire anemometry (HWA), it is justifiably becoming an increasingly important tool for turbulence research. This paper assesses the ability of PIV to resolve all relevant scales in a classical turbulent flow, namely grid turbulence, via a comparison with theoretical predictions as well as HWA measurements. Particular attention is given to the statistical convergence of mean turbulent quantities and the spatial resolution of PIV. An analytical method is developed to quantify and correct for the effect of the finite spatial resolution of PIV measurements. While the present uncorrected PIV results largely underestimate the mean turbulent kinetic energy and energy dissipation rate, the corrected measurements agree to a close approximation with the HWA data. The transport equation for the second-order structure function in grid turbulence is used to establish the range of scales affected by the limited resolution. The results show that PIV, due to the geometry of its sensing domain, must meet slightly more stringent requirements in terms of resolution, compared with HWA, in order to provide reliable measurements in turbulence.

Resume Objectif Le but de ce travail preliminaire est d'etudier l'utilisation du systeme de soins dans le mois qui a precede une tentative de suicide (TS), pour les patients ayant consulte durant une annee les urgences de deux hopitaux de Liege, Belgique. Methodologie Nous avons etudie les liens existant entre le diagnostic (axe I, DSM IV), le type de la TS, et les contacts avec les professionnels de sante durant le mois precedant la TS de 549 patients. Resultats Les troubles de l'adaptation et depressifs suivis d'intoxications ethyliques sont les diagnostics les plus frequents. Dans le mois precedant la TS, 80 % des patients avaient eu un contact avec un professionnel de sante (medecin generaliste dans 50 % des cas). Discussion L'adequation de la prise en charge proposee est discutee en fonction du delai necessaire pour l'obtention du prochain rendez-vous, tout en soulignant l'importance de l'identification des patients a risque suicidaire.

In 35 patients with bilateral primary open-angle glaucoma (POAG), with asymmetrical evolution of the neuropticopathy between the two eyes, we compared within each patient, the response to ibopamine and the perimetric defect. In 88% of cases (31/35 patients) the eye with the most severe perimetric defect had high intraocular pressure or a larger IOP increase after ibopamine. This result was highly significant at the sign test (P

Objective: The c.3909C>G (N1303K) is the most frequent mutation after the c.1521_1523delCTT (F508del) in Lebanon. A CFTR gene screening was performed on 10 Lebanese and 5 French CF patients carrying c.3909C>G. Parental studies revealed that all individuals have an association of c.3909C>G (exon 24) with c.869+11C>T (intron 7) in cis, inducing a new complex allele (CA). Since both mutations are located in two distant regions, we studied their combined impact by two plasmid constructions. Method: Firstly, we studied the impact of c.3909C>G on alternative splicing (AS), localization and maturation of the CFTR protein. Secondly, to study the impact of c.869+11C>T on AS, an ex-vivo study will be conducted using a plasmid containing the exon 7 and its flanking introns. After transfection of different type of eukaryotic cells, RNA extraction and RT-PCR, cDNA will be sequenced to verify the possible AS. Results: We showed that c.3909C>G affects the localization and the process. The in-silico study is in favor for a possible role of c.869+11C>T in AS. In fact, the used algorithm human splicing Finder showed that c.869+11C>T might affect the AS since it is located near the donor site. Conclusion: The class II mutation, c.3909C>G, induces mild phenotype with few CFTR on membrane. We suggest that the CA c.[869+11C>T;3909C>G], will act like a class I mutation, explaining the severe phenotype in some c.3909C>G patients. So, the classification of a mutation is not sufficient in a clinical approach, as the possible presence of a CA may alter its the specific effect. Therefore, an individualized approach is required to perform proper diagnostic and treatment when available.

We previously reported that the combination of two safe proteostasis regulators, cysteamine and epigallocatechin gallate (EGCG), can be used to improve deficient expression of the cystic fibrosis transmembrane conductance regulator (CFTR) in patients homozygous for the CFTR Phe508del mutation. Here we provide the proof-of-concept that this combination treatment restored CFTR function and reduced lung inflammation (P

Objective: To assess the level of fecal elastase-1 (FE-1) in relation to CFTR gene mutations in patients with cystic fibrosis (CF). Materials and Methods: This study included 49 patients (23 boys) with CF, mean age 8.43±0.99 years. CF was established on the basis of a positive sweat test (Macroduct, Wescor USA) and the results of molecular genetic analysis. Genetic diagnosis was realized for 36 CFTR mutations in genetic laboratories from Germany and France. To detect pancreatic insufficiency (PI) was defined FE-1 (ScheBo Biotech, Germany). The values of FE-1 in the range 0–100mg/g, are characteristic for severe exocrine PI, 100–200mg/g for moderate exocrine PI, and >200mg/g for pancreatic sufficiency. Results: F508del mutation was revealed in 73.33% cases (40.0% − homozygotes), known genotype with other mutations was found in 18.26% cases. Low values of FE-1 (7.89±3.70mg/g) confirmed exocrine PI in 81.64% patients with CF. PI with FE-1 of 2.90±0.78mg/g was confirmed in 100% children with homozygous state of F508del mutation. F508del mutation in heterozygous state caused decreased levels of FE-1 (33.69±19.34mg/g). Only 5.88% patients with CF F508del heterozygous were pancreatic sufficient and other 11.7% cases were moderate PI. Non-F508del CFTR mutations determined FE-1 levels of 218.64±64.87mg/g, with PI in 57.89% patients. Conclusion: The F508del mutations in homozygous state were associated with severe pancreatic exocrine insufficiency in all patients. Heterozygous state of mutation F508del requires association with another mutation and in most cases led to pancreatic enzyme deficiency. Non-F508del CFTR mutations are not obligatory associated with PI.

Purpose To assess the clinical efficacy of ibopamine eye drops in severe hypotony secondary to chronic progressive uveitis. Methods Case report. A 47-year-old man with a 37-year history of diffuse uveitis and severe refractory hypotony was treated with topical 2% ibopamine (Trazyl®) six times a day. Intraocular pressure, visual acuity, visual field and side effects were recorded during 15 months of follow-up. Results IOP, visual acuity and visual field increased after four days of therapy and lasted for two months when the drug was suspended because of the onset of filamentous keratopathy. A new course of treatment with 2% ibopamine eye drops in a different solvent (BSS®) resulted in a stable increase in IOP, VA and visual field, with no side effects in a follow-up of 13 months. Conclusions Ibopamine 2% eye drops in BSS® solvent seem effective in the treatment of uveitis-related hypotony.

One hundred forty-five patients are receiving renal replacement therapy at our center: 127 are on hemodialysis, 18 on peritoneal dialysis. From 2000 through 2005 a total of 215 arteriovenous fistulas (57% distal, 31% proximal, 12% prosthesis) were created. After some time we felt the necessity to design a vascular access surveillance system because the uremic population was increasingly constituted by aged people, including many diabetics and people suffering from hypertension with generalized vascular disease. The system comprised objective examination of the access at every dialysis session; kinetic Kt/V every 3 months; and a recirculation test (GIT) every 6 months. When there were anomalies in one of the above test results, color Doppler was performed. Detection of major stenosis with altered velocity parameters was a clear indication for angiography, possibly followed by angioplasty (PTA). In the indicated period, 15 patients underwent this procedure, and PTA was performed in all of them, often with placement of an endovascular stent. The results showed a survival of 12 months in 85% of patients and 18 months in 71% of patients. On the whole these data are comparable with others in the medical literature. The incidence of restenosis can be considered acceptable: it occurred in 3 cases out of 15 and could be corrected by PTA. In conclusion, PTA plus stent implantation is a valid method allowing quick and reliable correction of stenosis; a vascular access surveillance protocol and color Doppler imaging allow patient selection for angiography. PTA is to be considered an intermediate phase in stenosis correction before suggesting a new vascular access intervention to the patient.

An experimental test campaign has been performed by means of Pressure Sensitive Paint Technique (PSP) and Particle Image Velocimetry (PIV) on a thin high cambered airfoil at low Reynolds number. These techniques have been selected for their characteristics of lowintrusivity and high spatial resolution. Details of the experimental set-up and results shall be reported. Experimental investigation has been intended to get detailed information about the pressure distribution and the flow field characteristics around and downstream a nonconventional airfoil. Pressure and velocity fields have been integrated to obtain the airfoil lift, drag and aerodynamic moment coefficients in the investigated range of angles of attack. Measurements have been performed in a low-scale open circuit Eiffel type wind tunnel on a 2D wing profile model. The test campaign has been characterized by two Reynolds numbers (200.000 and 300.000) and a model incidence ranging between - -5° and +15° (1° step).

This paper summarises the activities performed in order to investigate the formation, motion and persistence of trailing vortices released by large commercial aircraft. Two different experimental test campaigns were carried out in two different large towing tank facilities performing PIV measurements. The lesson learned by the first investigation and by the experience of the scientific community, the solution adopted in order to characterise the wake vortex by the initial formation phase down to wake collapsing in the far-wake region are illustrated. Together with the adopted technical solution, with the selected experimental facility and with the improved measurement system, a particular care is taken: on the data analysis description, on the importance of advanced PIV algorithms and on the selected postprocessing methods for evaluating the main magnitudes characterising the vortex structures.

The scope of this work is to evaluate the capability of PIV to measure small-scale properties of a turbulent flow. To this aim, due to the available theoretical and empirical results, homogeneous and isotropic turbulence was selected for the investigations.

The formation, motion and persistence of trailing vortices, shed by large commercial aircraft, is a relevant aspect to the safety and productivity of air traffic. An aircraft encountering a wake vortex formation on its path may experience severe control problem as uncontrollable rolling moment or loss of height. Air traffic control separation regulations determine the minimum distance between aircraft on the same path both in cruise condition, both approaching airports in high lift condition. The ICAO separation distance procedure foresees four nautical miles between two heavy (> 136 tons) aircraft. Reducing the trailing vortex hazardous allows increasing safety and capacity of the airports. During the designing phase of new commercial aircraft different philosophies can be adopted, as developing low vortex wing or developing active or passive systems generating counter rotating vortex or turbulence, leading the wake vortex collapse. In all cases an accurate wake vortex investigation is necessary. Nowadays aerodynamic wind tunnels provide accurate data of the wake vortex behavior up to near and extended near filed (8-10 wing spans) behind the aircraft model, using five hole probes arrays, Hot Wire probes, LDV or PIV techniques. A valuable facility is the catapult wind tunnel allowing investigation up to 80 wing spans (mid field) using smoke visualisation, PlV technique and LIDAR system. The mid/far field investigation is still obtained performing flight tests and measuring the released wake by ground station as LIDAR or ultrasound or density gradient technique. During the last two years in the framework of the European research project, named C-Wake, the wake vortex was also investigated in towing tank facilities, normally used for naval applications. These kind of facilities were used for wake vortex visualisation in the seventies but nowadays thanks to the availability of new measurement techniques, it is possible carrying out quantitative measurements. PlV measurements were performed at INSEAN large towing tank facility to investigate the wake vortex behavior up to 200-250 wing span behind the model corresponding to 6.2 - 7.5 nautical miles. I

This paper illustrates results of instantaneous flow field measurements which were performed by the PIV group of DLR Gottingen during test campaigns in three different wind tunnel facilities of the Dutch-German Wind Tunnel foundation (DNW). The test results described first were obtained in the low speed tunnel LST in order to obtain detailed flow field information about the wake of a new high speed propeller model. The second test was performed in the transonic wind tunnel TWG in the frame of a German helicopter research project. Here the flow field above a pitching airfoil equipped with a flap driven by an integrated piezoelectric actuator has been observed with high spatial resolution. During the third test, stereoscopic flow field measurements were performed in the wake of a rotorcraft model in order to characterize the blade tip vortices at different flight conditions. The test was conducted in the 6 x 8 m2 open jet test section of the large low speed facility LLF which required observation distances exceeding 9 meters between camera and light sheet.

Objectives: Oxidative stress biomarkers as reactive oxygen species are induced by the sustained activation of neutrophils and other CF-derived defects in the lung of CF patients. Observed defects include an impaired glutathione (GSH) metabolism. Its supplementation may counterbalance the oxidative stress. A randomized, single blind controlled trial of inhaled GSH versus placebo (NCT01450267) is underway in order to evaluate the effect of GSH in cohort of CF patients. We report preliminary data on tolerability to GSH in a pediatric subset of enrolled patients. Methods: 48 CF patients (F 23, age M±DS 13.53 yrs), in regular follow up at the Regional Pediatric CF Center of Naples, were enrolled for RCT. The main inclusion criteria were: CF diagnosis by sweat test and/or two CF causing mutations, age of patients >6 yrs, FEV1% >40% of the predicted value, negative culture for Burkholderia cepacia. Spirometry was performed before and 10 and 60 minutes after GSH inhalation test (10mg/kg, maximum dosage 600mg/dose) in order to assess tolerability. Conclusions: No patients showed a decrease in FEV1% >15% after GSH inhalation as defined in the study design. A statistically significant increase was observed for FEF25−75% after 10 and 60 minutes from inhalation (FEF25−75 M±DS: T0 71.64±33.35 VS T10 76.37±36.73; p< 0.02 and T0 71.64±33.35 VS T60: 80.26±35.25; p< 0.0001) and for FEV1% after 60 minutes from inhalation (FEV1 M±DS: T0 97.90±21.03 VS T60 100.01±19.42; p< 0.01). No side effects were reported. On the basis of these preliminary results we are currently evaluating the efficacy of inhaled GSH on pulmonary function and inflammatory markers within a 12 months therapy.

Background Neuroimaging studies showed impairment of the basal ganglia and thalamus in preclinical stages of Huntington9s disease (HD) characterised by progressive volume loss, microstructural impairment and microglial activation. Therefore, biomarkers sensitive to all of these changes are needed. Aims We assessed the multimodal MRI as biomarker of the disease vulnerability and progression. Methods 3D T1 weighted image MRI acquisition was performed on a 3T Siemens scanner. We evaluated the macrostructure, microstructure and iron content of five vulnerable subcortical structures (high risk: caudate, putamen and pallidus; and low risk: thalamus and accumbens) in comparison with medial temporal lobe structures (hippocampus and amygdala) relatively preserved in early stages of the disease. 17 presymptomatic, 14 symptomatic (stages I–II) and 31 age and gender matched control subjects were scanned using the same protocol. Results Pre-HD subjects had a selective decrease of volume and microstructural changes in high risk structures (caudate, putamen and pallidus) and increased iron accumulation in all three nuclei (but only statistically significant in the globus pallidus). HD patients had lower volume than pre-HD in all high risk nuclei, higher microstructural impairment in the striatum but not significant differences in iron content. The lesion extended to low risk subcortical nuclei (thalamus and accumbens). Furthermore, volume and microstructure correlated with the clinical measures of progression of the disease. Conclusions This study provides proof of in vivo selective striatal degeneration in presymptomatic stages of the disease. Different parameters of MRI acquisition provide complementary information that give clues to the understanding of the pathophysiology underlying HD and its progression.