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The article looks at a study regarding phenotypic variability in xeroderma pigmentosum group G and combining the skin alterations with neurological and systemic abnormalities of Cockayne syndrome. It mentions chest X-rays showed mildly enlarged heart but no electrical defect was observed at electrocardiography (ECG). It also mentions magnetic resonance imaging revealed severe white matter abnormalities and no signs of photosensitivity.

Purpose: An innovative strategy to improve the sensitivity of positron emission tomography (PET)- based treatment verification in ion beam radiotherapy is proposed. Methods: Low counting statistics PET images acquired during or shortly after the treatment (Measured PET) and a Monte Carlo estimate of the same PET images derived from the treatment plan (Expected PET) are considered as two frames of a 4D dataset. A 4D maximum likelihood reconstruction strategy was adapted to iteratively estimate the annihilation events distribution in a reference frame and the deformation motion fields that map it in the Expected PET and Measured PET frames. The outputs generated by the proposed strategy are as follows: (1) an estimate of the Measured PET with an image quality comparable to the Expected PET and (2) an estimate of the motion field mapping Expected PET to Measured PET. The details of the algorithm are presented and the strategy is preliminarily tested on analytically simulated datasets. Results: The algorithm demonstrates (1) robustness against noise, even in the worst conditions where 1.5?104 true coincidences and a random fraction of 73% are simulated; (2) a proper sensitivity to different kind and grade of mismatches ranging between 1 and 10 mm; (3) robustness against bias due to incorrect washout modeling in the Monte Carlo simulation up to 1/3 of the original signal amplitude; and (4) an ability to describe the mismatch even in presence of complex annihilation distributions such as those induced by two perpendicular superimposed ion fields. Conclusions: The promising results obtained in this work suggest the applicability of the method as a quantification tool for PET-based treatment verification in ion beam radiotherapy. An extensive assessment of the proposed strategy on real treatment verification data is planned. [ABSTRACT FROM AUTHOR]

Cold rolled SS is suitable for deep drawing operations, however the presence of resilient scales or oxides on the surface can damage the deep drawing punch/die sets and emphasise creep phenomena of the material. These oxides can be formed during the hot rolling process preceding the cold one, or during the bright annealing performed between the steps of thickness reduction of the coil. Then a cleaning of the SS surface before drawing is necessary to have a longer life of the tools. The removal of the surface oxide scale can be performed with electrolytic pickling using neutral electrolytes, but the process parameters can be varied depending on the amount of oxides present on the surface of the starting material. To optimise the process efficiency, then, an univocal scale of the degree of oxidation of the bright annealed SS was determined through microscopical analysis and potentiodynamic tests. [ABSTRACT FROM AUTHOR]

A series of waterborne coatings obtained from aqueous dispersions of fluorinated polyurethane ionomers were characterized by Differential Scanning Calorimetry (DSC), Contact Angle measurements (CA), and Electrochemical Impedance Spectroscopy (EIS) with evaluation of water uptake and barrier properties at prolonged exposure. All the considered materials showed two glass transition temperatures and the formation of highly hydrophobic surfaces with CA values against water well above 100°. On the other hand the compositive parameters of the polymers—presence and concentration of cationic as well as anionic groups, linear or crosslinked molecular structure—showed a significant effect on the electrochemical behaviour. In particular it resulted that less water absorption is shown by coatings with lower ionic character, while crosslinked anionomer structures show excellent long-term barrier properties. The research offers useful indication towards the design of a new class of hydrophobic barrier coatings from aqueous dispersions. [ABSTRACT FROM AUTHOR]

Epoxy resins, heavily loaded with fillers, are used as fairing compounds in coating systems to fill superficial imperfections and protect the hull of yachts and ships; they are complex composite materials whose final performance can be heavily affected by the conditions of application. In this paper the curing process of an innovative formulation was studied in function of the temperature and humidity, in order to evaluate how these environmental parameters influence the reaction evolution and gain further insight into the reaction mechanism. The study of the curing process was carried out by thermogravimetric analysis, differential calorimetric analysis and FT-IR spectroscopy. From the thermal analysis the obtained crosslink degree ranged between 92 and 99%, at 10 °C and 25 °C respectively. The samples cured at 25 °C reached the maximum curing degree in one week independently from the humidity. The samples cured at lower temperature (10 °C), even after two weeks, did not reach the complete crosslinking. On the other side, it was evident that the humidity did not influence the curing at 25 °C, while it had a slight effect at lower temperature (10 °C). Results from FTIR spectroscopy at 25 °C evidenced changes in the bands characterizing the two components of the resin during the curing reaction, in particular the disappearance of the oxirane ring band. [ABSTRACT FROM AUTHOR]

From the commercial point of view the aesthetics of large luxury yachts has the same importance of their technical performances and reliability. In this perspective the first impact that a new construction can exert on a potential owner takes place through the overall appearance of the hull and superstructures. The hull colour, in particular, is becoming more and more determinant to make a yacht more attractive. After a long, undisturbed supremacy of white and cream colours, now an increasing number of owners are opting for dark colours, like blue, green or black. For a perfect painting output these colours needs a perfectly smooth surface as they have the tendency to emphasize every small defect of the hull surface. This aspect can be further amplified by those shape defects affecting hull sides, such as bump and hollows, coming from welding plates each other and plates to frames. For this reason, in case of steel and aluminium vessels, the surface finishing must be achieved by a long and delicate process of filling and fairing. Notwithstanding very accurate preparation, hull surfaces can undergo undesired alteration because of long expositions to solar radiation. This phenomenon is amplified when the surface is painted with dark colours which have the tendency of absorbing much greater solar heat. As stated by a previous investigation carried out on superyachts painted with dark colours, the surface of hull sides after a long exposition to solar radiation can reach temperatures close to 70–80°. In order to evaluate possible distortions of hull sides caused by thermal loads, an investigation has been carried out on the influence of temperature on the mechanical behaviour of steel plates coated by epoxy filler. The study has been developed by FEM structural analyses on a portion of a superyacht hull in the region of aft sides where the flatness of shell makes more visible possible distortions. Both steel and aluminium material have been considered covered by filler layers of different kind and thickness. A preliminary experimental investigation has been performed on a number of steel and aluminium specimens in order to calibrate the numerical models. [ABSTRACT FROM AUTHOR]

The X-linked Emery–Dreifuss muscular dystrophy (X-EDMD) is a hereditary muscle disorder associated with cardiac involvement. Sinus node dysfunction and atrioventricular conduction defects, typical of X-EDMD, occur in both males and females and may result in sudden cardiac death unless treated by permanent pacing. The objective of the study was to determine the frequency and relevance of X-EDMD in heart conduction system disease in young individuals treated with a pacemaker implant. The medical history of 3450 paced individuals in the region of South Moravia, Czech republic, was reviewed. Thirty-five patients, 20 males and 15 females, with idiopathic heart conduction disease of onset before age 40 were identified and screened for X-EDMD. Within these 35 individuals, only one male was found to carry a mutation in X-EDMD gene. We conclude that the clinical relevance of X-EDMD in heart conduction system disease is very low. It should, however, be included into the diagnostic work-up of young male individuals with idiopathic cardiac conduction disturbances. [ABSTRACT FROM AUTHOR]