Requirements of the authorized examinator physician (AME) for class II: interpretative doubts and suggestions

The purpose of this document is to clarify the requirements that a doctor must have for carrying out the Authorized Examiner (AME) activity for class II workers. We have consulted the normative sources that indicate the requisite that the authorized physicians must possess and subsequently we have compared the norms, the European Union regulations and the national ones issued by the National Civil Aviation Body (ENAC), as well as the guidelines indicated by the EASA. Finally, Authors give suggestions that can be given to ENAC regarding the issue of the authorization as AME examiner for class II workers

Evaluating the contribution of the gene TARDBP in Italian patients with amyotrophic lateral sclerosis

Background and objectives: Genetic variants in the gene TARDBP, encoding TDP-43 protein, are associated with amyotrophic lateral sclerosis (ALS) in familial (fALS) and sporadic (sALS) cases. Objectives of this study were to assess the contribution of TARDBP in a large cohort of Italian ALS patients, to determine the TARDBP-associated clinical features and to look for genotype-phenotype correlation and penetrance of the mutations.Methods: A total of 1992 Italian ALS patients (193 fALS and 1799 sALS) were enrolled in this study. Sanger sequencing of TARDBP gene was performed in patients and, when available, in patients' relatives.Results: In total, 13 different rare variants were identified in 43 index cases (10 fALS and 33 sALS) with a cumulative mutational frequency of 2.2% (5.2% of fALS, 1.8% of sALS). The most prevalent variant was the p.A382T followed by the p.G294V. Cognitive impairment was detected in almost 30% of patients. While some variants, including the p.G294V and the p.G376D, were associated with restricted phenotypes, the p.A382T showed a marked clinical heterogeneity regarding age of onset, survival and association with cognitive impairment. Investigations in parents, when possible, showed that the variants were inherited from healthy carriers and never occurred de novo.Conclusions: In our cohort, TARDBP variants have a relevant frequency in Italian ALS patients and they are significantly associated with cognitive impairment. Clinical presentation is heterogeneous. Consistent genotype-phenotype correlations are limited to some mutations. A marked phenotypic variability characterizes the p.A382T variant, suggesting a multifactorial/oligogenic pathogenic mechanism

Histone H3.3 beyond cancer: Germline mutations in Histone 3 Family 3A and 3B cause a previously unidentified neurodegenerative disorder in 46 patients

Although somatic mutations in Histone 3.3 (H3.3) are well-studied drivers of oncogenesis, the role of germline mutations remains unreported. We analyze 46 patients bearing de novo germline mutations in histone 3 family 3A (H3F3A) or H3F3B with progressive neurologic dysfunction and congenital anomalies without malignancies. Molecular modeling of all 37 variants demonstrated clear disruptions in interactions with DNA, other histones, and histone chaperone proteins. Patient histone posttranslational modifications (PTMs) analysis revealed notably aberrant local PTM patterns distinct from the somatic lysine mutations that cause global PTM dysregulation. RNA sequencing on patient cells demonstrated up-regulated gene expression related to mitosis and cell division, and cellular assays confirmed an increased proliferative capacity. A zebrafish model showed craniofacial anomalies and a defect in Foxd3-derived glia. These data suggest that the mechanism of germline mutations are distinct from cancer-associated somatic histone mutations but may converge on control of cell proliferation

S hear performance of timber-GFRP double-lap adhesive joints for civil engineering applications

Adhesive bonding technology make it possible to join different materials and to assemble lightweight joints with high mechanical performance. These advantages over conventional technologies have led to a growing interest in these joints in timber construction. This study reports the results of an experimental campaign on double-lap adhesive joints between timber and GFRP adherends assembled with two different commercial structural epoxy adhesives. The aim of the present study is to evaluate the applicability of the bonding technique in the field of civil engineering and in the manufacture of window frames with adhesive joints (patent application No. 1020000023128, inventor Prof. Munafò). The results obtained after curing under laboratory conditions are compared with those obtained after exposure to high temperatures (40 °C) and relative humidity (100% RH). The results show that the effect of artificial ageing varies depending on the adhesive and generally leads to a reduction in the ultimate strengths and stiffness for both adhesives. The best mechanical performance in terms of ultimate load and stiffness is obtained by the epoxy adhesive EPX1 in each configuration tested, with values for ultimate load and displacements sufficient to ensure the mechanical performance in the range of applications studied. The epoxy adhesive EPX2 showed a significant decay of mechanical performance after artificial ageing and exhibited lower ultimate displacements and loads

Effect of temperature and relative humidity on the shear performance of double-lap adhesive joints between steel and glass adherends

The use of large glass areas is a hallmark of modern architecture, and glass is also used as a structural element in many applications. Recent developments in the field of structural adhesives have led to the development of bonding solutions between steel and glass adherends that meet the structural and aesthetic requirements for high performance buildings. Indeed, the use of adhesive bonding technology allows for uniform stress distribution, enabling ductile hybrid joints to be achieved by combining materials with different mechanical properties. However, the main weakness of this assembly system is the influence of high values of temperature and humidity values on the mechanical behaviour of bonded joints. This paper reports the experimental results obtained on double-lap adhesive joints between steel and float glass adherends. In particular, the results obtained after curing under laboratory conditions are compared with those obtained after exposure to high temperature (40 °C) and relative humidity (100% RH). The performances obtained with two two-component epoxy adhesives (EPX1, EPX2), one polyurethane adhesive (PU) and one silicone adhesive (SIL) are compared and discussed. The results of the experimental campaigns show that the epoxy adhesives achieve the best mechanical performance in relation to the studied application, while the SIL adhesive exhibits the worst mechanical performance in each case

Ignition of spray flame with multiple spark

The movies have been taken at 4000 fps. The spark is placed at a distance z from the nozzle and at 5mm from the enclosure. The ignition unit delivers at 100Hz a spark lasting 8ms, followed by 2ms of no activity. The front and side views are shown. The main flow is from top to botton and the swirling flow from right to left (evident in the front view movies). The flame ignites a long time after the spark sequence has begun. The work is described in: T. Marchione, S.F. Ahmed, E. Mastorakos, "Ignition of turbulent swirling n-heptane spray flames using single and multiple sparks", Combustion and Flame 156 (2009) 166–180

Effect of nylon fabric reinforcement on the mechanical performance of adhesive joints made between glass and GFRP

The use of reinforcements in adhesive joints makes the stress distribution more uniform, improving their mechanical performance and adhesion. The present paper aims to verify the effectiveness and efficiency of the insertion of nylon 6 fabric in the adhesive layer, to study their applicability and functionality in building components. The increase in stiffness achieved by applying nylon 6 fabric in the adhesive layer between glass and GFRP pultruded profiles and steel laminates applied to GFRP beams is investigated. Three different epoxy adhesives and one epoxy resin are used and compared. Three different types of tests are carried out in order to study the different properties of the reinforcement system: tensile tests on GFRP/GFRP single-lap adhesive joints, with and without nylon fabric reinforcement; tensile tests on double-lap adhesive joints between float glass and pultruded GFRP profiles reinforced with nylon fabric according to four configurations (in the middle plane of the adhesive layer, on the glass surfaces, on the GFRP surfaces, on both GFRP and glass configurations) to verify the influence of its position; three-point bending tests on long GFRP tubular profiles reinforced with steel plates and nylon fabric in different configurations, to study resistance to bending loads. The results from the experimental campaign show the effectiveness of the reinforcement system using nylon fabric 6. In general, both a reduction in ultimate strength and an increase in joint stiffness compared to unreinforced configurations are observed