Mucopolysaccharidosis IVA: Diagnosis, Treatment, and Management.

Mucopolysaccharidosis type IVA (MPS IVA, or Morquio syndrome type A) is an inherited metabolic lysosomal disease caused by the deficiency of the N-acetylglucosamine-6-sulfate sulfatase enzyme. The deficiency of this enzyme accumulates the specific glycosaminoglycans (GAG), keratan sulfate, and chondroitin-6-sulfate mainly in bone, cartilage, and its extracellular matrix. GAG accumulation in these lesions leads to unique skeletal dysplasia in MPS IVA patients. Clinical, radiographic, and biochemical tests are needed to complete the diagnosis of MPS IVA since some clinical characteristics in MPS IVA are overlapped with other disorders. Early and accurate diagnosis is vital to optimizing patient management, which provides a better quality of life and prolonged life-time in MPS IVA patients. Currently, enzyme replacement therapy (ERT) and hematopoietic stem cell transplantation (HSCT) are available for patients with MPS IVA. However, ERT and HSCT do not have enough impact on bone and cartilage lesions in patients with MPS IVA. Penetrating the deficient enzyme into an avascular lesion remains an unmet challenge, and several innovative therapies are under development in a preclinical study. In this review article, we comprehensively describe the current diagnosis, treatment, and management for MPS IVA. We also illustrate developing future therapies focused on the improvement of skeletal dysplasia in MPS IVA

Comparison of Chemical and Mechanical Surface Treatments on Metallic Precision Spheres for Using as Optical Reference Artifacts

[EN] The improvement of industrial manufacturing processes requires measurement procedures and part inspection tasks to be faster and faster while remaining effective. In this sense, the capabilities of noncontact measuring systems are of great help, not only because of the great amount of data they provide but also for the ease of the integration of these systems as well as their automation, minimising the impact on the industry. This work presents a comparative study on the influence of two surface treatments performed on low-cost, high-precision metallic spheres on the suitability of these spheres to be used as artefacts for the calibration of optical sensors, specifically laser triangulation sensors. The first surface treatment is sandblasting (a mechanical process), whose effect has been studied and presented in previous work. The second treatment focused on in this paper is acid etching (a chemical process). The comparison has been performed by evaluating the same metrological characteristics on two identical groups of spheres of similar type (diameter and accuracy), each of which was subjected to a different treatment. It was necessary to obtain the reference values of the metrological parameters with high accuracy, which involved measuring the spheres with a coordinate measuring machine (CMM) by contact probing. Likewise, spheres were scanned by a laser triangulation sensor mounted on the same CMM. The results derived from both the contact and laser measurements and before and after treating the surfaces were used to compare four parameters: point density, sphere diameter, sphere form deviation, and standard deviation of the best-fit sphere to the corresponding point cloud. This research has revealed that acid etching produces better optical qualities on the surfaces than the mirror-like original ones, thus enhancing the laser sensor capturing ability. However, such chemical etching has affected the metrological characteristics of the spheres to a greater extent than that produced by sandblasting. This difference is due to the variability of the chemical etching, caused by the high aggressiveness of the acid, which makes the process very sensitive to the time of exposure to the acid and the orientations of the spheres in the bath.SIUniversity Institute of Industrial Technology of Asturias, IUTA, through the research project ref. SV-21-GIJON-1-06

Computing “Small” 1–Homological Models for Commutative Differential Graded Algebras

We use homological perturbation machinery specific for the algebra category [13] to give an algorithm for computing the differential structure of a small 1– homological model for commutative differential graded algebras (briefly, CDGAs). The complexity of the procedure is studied and a computer package in Mathematica is described for determining such models.Ministerio de Educación y Ciencia PB98–1621–C02–02Junta de Andalucía FQM–014

A resilience-based comparative study between optical burst switching and optical circuit switching technologies

Personal use of this material is permitted. Permission from IEEE must be obtained for all other uses, in any current or future media, including reprinting/republishing this material for advertising or promotional purposes, creating new collective works, for resale or redistribution to servers or lists, or reuse of any copyrighted component of this work in other works. J. A. Hernández, J. Aracil, V. López, J. F. Palacios, and O. G. de Dios, "A resilience-based comparative study between optical burst switching and optical circuit switching technologies", in International Conference on Transparent Optical Networks, 2006, p. 231-234Internet operators and ISP providers have traditionally designed network resources following an over-planning policy, on attempts to maintain a desired grade of service and network availability, regardless of network failures. This work presents a comparative study of two resilience mechanisms in the design of optical networks either based on optical burst switching (OBS) or optical circuit switching (OCS): the M:N protection scheme with dedicated backup channels. It is further analysed and discussed the benefits and disadvantages of such mechanism based on an analytical mode

Discriminant analysis of the specialty of elite cyclist

The different demands of competition coupled with the morphological and physiological characteristics of cyclists have led to the appearance of cycling specialities. The aims of this study were to determine the differences in the anthropometric and physiological features in road cyclists with different specialities, and to develop a multivariate model to classify these specialities and predict which speciality may be appropriate to a given cyclist. Twenty male, elite amateur cyclists were classified by their trainers as either flat terrain riders, hill climbers, or all-terrain riders. Anthropometric and cardiorespiratory studies were then undertaken. The results were analysed by MANOVA and two discriminant tests. Most differences between the speciality groups were of an anthropometric nature. The only cardiorespiratory variable that differed significantly (p < 0.05) was maximum oxygen consumption with respect to body weight (VO2max/kg). The first discriminant test classified 100% of the cyclists within their true speciality; the second, which took into account only anthropometric variables, correctly classified 75%. The first discriminant model allows the likely speciality of still non-elite cyclists to be predicted from a small number of variables, and may therefore help in their specific training

Indexed left atrial size predicts all-cause and cardiovascular mortality in patients undergoing aortic valve surgery

[Abstract] OBJECTIVES: The enlargement of the left atrium has been identified as a marker of chronically increased left ventricular filling pressure and left ventricular diastolic dysfunction. This study aims to evaluate the association of indexed left atrial diameter with stroke, cardiovascular mortality, the combined event, and all-cause mortality in patients who underwent aortic valve surgery. METHODS: Indexed left atrial diameter was measured in 2011 adult patients (mean age, 70.9 ± 10.8 years; 58.7% were men) who underwent aortic valve surgery between January 2008 and March 2016. RESULTS: On the basis of the criteria of the American Society of Echocardiography, indexed left atrial diameter was normal in 64% of patients, mildly enlarged in 12.4% of patients, moderately enlarged in 9.2% of patients, and severely enlarged in 14.3% of patients. Over a mean follow-up period of 3.2 ± 2.1 years, there were 334 deaths and 97 strokes. Cardiovascular mortality survival at 5 years among patients with normal, mild, moderate, and severe left atrial enlargement was 91.6%, 86.8%, 77.9%, and 77.4%, respectively (P < .001). After covariable adjustment, Cox regression analysis showed indexed left atrial diameter as an independent predictor of all-cause mortality (hazard ratio per 1-cm/m2 increment, 1.545; 95% confidence interval, 1.252-1.906, P < .001), cardiovascular death (hazard ratio per 1-cm/m2 increment, 1.971; 95% confidence interval, 1.541-2.520; P < .001), and the combined event (hazard ratio per 1-cm/m2 increment, 1.673; 95% confidence interval, 1.321-2.119; P < .001). CONCLUSIONS: Indexed left atrial diameter is a strong predictor of long-term outcomes in patients with aortic valve diseases who undergo surgery

Nanostructured Ti thin films by magnetron sputtering at oblique angles

The growth of Ti thin films by the magnetron sputtering technique at oblique angles and at room temperature is analysed from both experimental and theoretical points of view. Unlike other materials deposited in similar conditions, the nanostructure development of the Ti layers exhibits an anomalous behaviour when varying both the angle of incidence of the deposition flux and the deposition pressure. At low pressures, a sharp transition from compact to isolated, vertically aligned, nanocolumns is obtained when the angle of incidence surpasses a critical threshold. Remarkably, this transition also occurs when solely increasing the deposition pressure under certain conditions. By the characterization of the Ti layers, the realization of fundamental experiments and the use of a simple growth model, we demonstrate that surface mobilization processes associated to a highly directed momentum distribution and the relatively high kinetic energy of sputtered atoms are responsible for this behaviourJunta de Andalucía P12-FQM- 2265Ministerio de Ciencia e Innovación CSD2008- 00023, MAT2013-42900-P, MAT2013-40852-R, MAT2014-59772-C2-1, MAT2011- 2908

Kinetic Energy-induced Growth Regimes of Nanocolumnar Ti Thin Films Deposited by Evaporation and Magnetron Sputtering

We experimentally analyze different growth regimes of Ti thin films associated to the existence of kinetic energy-induced relaxation mechanisms in the material's network when operating at oblique geometries. For this purpose, we have deposited different films by evaporation and magnetron sputtering under similar geometrical arrangements and at low temperatures. With the help of a well-established growth model we have found three different growth regimes: (i) low energy deposition, exemplified by the evaporation technique, carried out by species with typical energies in the thermal range, where the morphology and density of the film can be explained by solely considering surface shadowing processes, (ii) magnetron sputtering under weak plasma conditions, where the film growth is mediated by surface shadowing mechanisms and kinetic-energy-induced relaxation processes, and (iii) magnetron sputtering under intense plasma conditions, where the film growth is highly influenced by the plasma, and whose morphology is defined by nanocolumns with similar tilt than evaporated films, but with much higher density. The existence of these three regimes explains the variety of morphologies of nanocolumnar Ti thin films grown at oblique angles under similar conditions in the literature.EU-FEDER and MINECO-AEI 201560E055EU-FEDER and MINECO-AEI MAT2014-59772-C2-1-PEU-FEDER and MINECO-AEI MAT2016-79866-REU-FEDER and MINECO-AEI MAT2015-69035-REDCUniversity of Seville V and VI PPIT-U

Theoretical and experimental characterization of TiO2 thin films deposited at oblique angles

The microstructural features of amorphous TiO2 thin films grown by the electron beam physical vapour deposition technique at oblique angles have been experimentally and theoretically studied. The microstructural features of the deposited films were characterized by considering both, the column tilt angle and the increase of the column thickness with height. A Monte Carlo model of the film growth has been developed that takes into account surface shadowing, short-range interaction between the deposition species and the film surface, as well as the angular broadening of the deposition flux when arriving at the substrate. The good match between simulations and experimental results indicates the importance of these factors in the growth and microstructural development of thin films deposited at oblique angles.Ministerio de Innovación, MAT 2007-65764, CONSOLIDER INGENIO 2010-CSD2008-00023, PIE 200960I132Junta de Andalucía TEP2275, TEP5283, P07-FQM-03298, FQM-690

Laser line scanner aptitude for the measurement of Selective Laser Melting parts

[EN] When looking for any metrological verification of parts manufactured by metal laser printing with optical equipment, it is necessary to ensure the traceability of the measurements that can be obtained. The difficulty of this process lies in the fact that these measurements are obtained on point clouds captured from surfaces with high form errors and poor surface finishes, even when this type of surface usually undergoes processes to improve the surface finish, such as sandblasting. This research focuses precisely on the analysis of the metrological suitability of a laser line scanner (laser triangulation sensor) on parts manufactured by Selective Laser Melting (SLM). The study starts from the design of a test part specifically oriented to the printing process with SLM metal powder bed. This test part was printed in 17-4PH stainless steel and then sandblasted. The test part was measured in a Coordinate Measuring Machine (CMM), obtaining reference GD&T values. The measurement was carried out under pre-sandblasting ("as built") and post-sandblasting conditions, thus providing interesting information about the erosion rate of this post process. A state-of-the-art laser sensor was employed for the metrological comparison, mounted on the same available CMM that was used for contact measurements. In this research three analyses were carried out: the quality of 3D metal printed parts with respect to CAD model, the effect of the sandblasting post-process, and the accuracy of the measurements obtained with the laser line sensor. In addition, this work conducts an in-depth study about the influence of point cloud treatment and filtering procedures, by comparing the filtering methods applied by different reverse engineering software packages. The study leads to the conclusion that filters based on the standard deviation of the point cloud are the best candidates in order to obtain laser measurements closer to the contact measurements.SIAuthors thank to the financial support provided by the Junta de Castilla y León (project LE027P17-FEDER funds) and also to two student grants awarded by the University Institute of Industrial Technology of Asturias (IUTA, ref. SV-19-GIJON-1-14) and by the Young researcher mobility program of SIF (Manufacturing Engineering Spanish Society)