Two patients with history of STEC-HUS, posttransplant recurrence and complement gene mutations

Hemolytic uremic syndrome (HUS) is a disease of microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. About 90% of cases are secondary to infections by Escherichia coli strains producing Shiga-like toxins (STEC-HUS), while 10% are associated with mutations in genes encoding proteins of complement system (aHUS). We describe two patients with a clinical history of STEC-HUS, who developed end-stage renal disease (ESRD) soon after disease onset. They received a kidney transplant but lost the graft for HUS recurrence, a complication more commonly observed in aHUS. Before planning a second renal transplantation, the two patients underwent genetic screening for aHUS-associated mutations that revealed the presence of a heterozygous CFI mutation in patient #1 and a heterozygous MCP mutation in patient #2, and also in her mother who donated the kidney. This finding argues that the two cases originally diagnosed as STEC-HUS had indeed aHUS triggered by STEC infection on a genetic background of impaired complement regulation. Complement gene sequencing should be performed before kidney transplantation in patients who developed ESRD following STEC-HUS since they may be undiagnosed cases of aHUS, at risk of posttransplant recurrence. Furthermore, genetic analysis of donors is mandatory before living-related transplantation to exclude carriers of HUS-predisposing mutations. Two patients with a clinical history of D+ hemolytic uremic syndrome associated with Shiga-toxin-producing 0157:H7 E. coli and recurrence in the kidney graft carry heterozygous mutations in the genes encoding complement factor I (patient 1) and membrane cofactor protein (patient 2). © Copyright 2013 The American Society of Transplantation and the American Society of Transplant Surgeons

Neuroimaging Evidence of Major Morpho-Anatomical and Functional Abnormalities in the BTBR T+TF/J Mouse Model of Autism

BTBR T+tf/J (BTBR) mice display prominent behavioural deficits analogous to the defining symptoms of autism, a feature that has prompted a widespread use of the model in preclinical autism research. Because neuro-behavioural traits are described with respect to reference populations, multiple investigators have examined and described the behaviour of BTBR mice against that exhibited by C57BL/6J (B6), a mouse line characterised by high sociability and low self-grooming. In an attempt to probe the translational relevance of this comparison for autism research, we used Magnetic Resonance Imaging (MRI) to map in both strain multiple morpho-anatomical and functional neuroimaging readouts that have been extensively used in patient populations. Diffusion tensor tractography confirmed previous reports of callosal agenesis and lack of hippocampal commissure in BTBR mice, and revealed a concomitant rostro-caudal reorganisation of major cortical white matter bundles. Intact inter-hemispheric tracts were found in the anterior commissure, ventro-medial thalamus, and in a strain-specific white matter formation located above the third ventricle. BTBR also exhibited decreased fronto-cortical, occipital and thalamic gray matter volume and widespread reductions in cortical thickness with respect to control B6 mice. Foci of increased gray matter volume and thickness were observed in the medial prefrontal and insular cortex. Mapping of resting-state brain activity using cerebral blood volume weighted fMRI revealed reduced cortico-thalamic function together with foci of increased activity in the hypothalamus and dorsal hippocampus of BTBR mice. Collectively, our results show pronounced functional and structural abnormalities in the brain of BTBR mice with respect to control B6 mice. The large and widespread white and gray matter abnormalities observed do not appear to be representative of the neuroanatomical alterations typically observed in autistic patients. The presence of reduced fronto-cortical metabolism is of potential translational relevance, as this feature recapitulates previously-reported clinical observations

Analytical modeling and characterization of ring beam profiles for high-power lasers used in industrial manufacturing

Active fibre lasers are widely used in the industry for different manufacturing applications ranging from cutting, to welding and additive manufacturing. The recent introduction of the multiple-core fibre lasers allows these sources to flexibly change the Power Density Distribution (PDD) from conventional Gaussian profiles towards ring shapes. While the advantages of the novel beam shapes over the conventional ones are still being explored, the need for modeling tools to define the PDD shapes becomes more evident. This work studies the analytical modeling of Gaussian to ring profiles with the aim to move towards standardized parameters referable to the manufacturing processes. The proposed models combine Gaussian and annular components to define the novel beam shapes. Among the different models assessed, the Torus and Multi-Gaussian approaches exhibited the best fitting quality thus enabling the definition of descriptive metrics of the PDD. The modeling framework developed was validated on an industrial Laser Powder Bed Fusion (LPBF) system with a double-core light source. The beam shape variation along the propagation axis was assessed to analyze the effect of defocusing using the developed beam parameters. Eventually, the best performing model was furtherly validated with a bead on plate experiment to explain how the model coefficients can be jointly exploited to predict the material response using a Gaussian or a ring beam profile

In-source dynamic beam shaping of Al alloys processed via LPBF: Effect of novel beam profiles on surface roughness and microstructure

Next-generation fiber laser sources can dynamically combine Gaussian beams with ring distributions within the source. The irradiance manipulation may add flexibility to the Laser Powder Bed Fusion process, for instance to control microstructure or surface roughness. However, their use is limited by a lack of studies revealing how beam shaping can be exploited in a reproducible manner. Accordingly, this work shows the use of novel beam profiles provided by a multi-core fiber laser source in the LPBF of AlSi7Mg0.6. Irradiance profiles were characterized, then large experiments were conducted to investigate their effect on surface roughness and microstructure of produced parts

Tailoring the microstructure of Fe-2.9wt.%Si alloy in laser powder bed fusion using in-source beam shaping

Tuning the irradiance profile of the laser beam opens up new possibilities in terms of controlling the thermal field the material is subjected to during laser powder bed fusion (PBF-LB/M). This control can be exploited to manipulate the material's microstructure. In this work, a contemporary high power fibre laser with in-source beam shaping capability was used to test different irradiance profiles ranging from Gaussian to ring during the PBF-LB/M of Fe-2.9wt.%Si alloy. This soft magnetic alloy is used in electrical machines, and its magnetic properties are known to be correlated to the microstructure. Initially, the experimental work assessed the influence of peak and ring irradiance levels on densification and grain morphology. In a second experimental run, the influence of the beam profile on the conventionally employed energy density was verified. The results demonstrated that the use of combined Gaussian-ring profiles offers a method for manipulating grain size and shape from a columnar towards an equiaxed shape, without requiring a change in the scan strategy

Low-temperature tensile properties of meta-stable β titanium Ti-5Al-5V-5Mo-3Cr alloy fabricated by pulsed laser powder bed fusion

Ti-5Al-5V-5Mo-3Cr (Ti-5553) alloy is increasingly used in structural airplane components, such as landing gear and fuselage, thanks to its high strength, good ductility, and low density. These components are consistently subjected to sub-zero temperatures during the flight. In this study, pulsed laser powder bed fusion (L-PBF) parameters were firstly optimized to fabricate highly dense (>99%) parts, and their effect on microstructure and microhardness was analyzed. Finally, tensile properties were evaluated under sub-zero temperature conditions and compared to the standard room ones to validate the feasibility of using Ti5553 under low-temperature environments

The influence of gravimetric moisture content on studded shoe–surface interactions in soccer

It is desirable for the studs of a soccer shoe to penetrate the sport surface and provide the player with sufficient traction when accelerating. Mechanical tests are often used to measure the traction of shoe–surface combinations. Mechanical testing offers a repeatable measure of shoe–surface traction, eliminating the inherent uncertainties that exist when human participant testing is employed, and are hence used to directly compare the performance of shoe–surface combinations. However, the influence specific surface characteristics has on traction is often overlooked. Examining the influence of surface characteristics on mechanical test results improves the understanding of the traction mechanisms at the shoe–surface interface. This allows footwear developers to make informed decisions on the design of studded outsoles. The aim of this paper is to understand the effect gravimetric moisture content has on the tribological mechanisms at play during stud–surface interaction. This study investigates the relationships between: the gravimetric moisture content of a natural sand-based soccer surface; surface stiffness measured via a bespoke impact test device; and surface traction measured via a bespoke mechanical test device. Regression analysis revealed that surface stiffness decreases linearly with increased gravimetric moisture content (p = 0.04). Traction was found to initially increase and then decrease with gravimetric moisture content. It was observed that: a surface of low moisture content provides low stud penetration and therefore reduced traction; a surface of high moisture content provides high stud penetration but also reduced traction due to a lubricating effect; and surfaces with moisture content in between the two extremes provide increased traction. In this study a standard commercially available stud was used and other studs may provide slightly different results. The results provide insight into the traction mechanisms at the stud–surface interface which are described in the paper. The variation between traction measurements shows the influence gravimetric moisture content will have on player performance. This highlights the requirement to understand surface conditions prior to making comparative shoe–surface traction studies and the importance of using a studded outsole that is appropriate to the surface condition during play

The effect of in-source spatial beam shaping on the laser welding of e-mobility metals and alloys

Electromobility applications require several welded connections using demanding materials often in dissimilar combinations. Copper, aluminium, or steel alloys are laser welded for energy storage and traction related components. On the one hand, high power fiber laser sources provide in-source beam shaping solutions able to modify the irradiance profile towards ring-shaped beams. On the other hand, research focused on the effect of the beam shapes on the melting mechanisms and process quality is still in progress. This work studies the effect of different beam profiles on AISI301LN, AA6082 and pure Cu with a 5 kW fiber laser. Linear trends of power over penetration depth as a function of speed confirms the validity of employing the lumped heat capacity model for ring-shaped beams. Moreover, the specific melting fluence is observed to exhibit an exponential decaying trend with the proportion of power allocated in the fiber core, irrespective of tested material

Electromagnetic shielding properties of LPBF produced Fe2.9wt.%Si alloy

Ferromagnetic materials are used in various applications such as rotating electrical machines, wind turbines, electromagnetic shielding, transformers, and electromagnets. Compared to hard magnetic materials, their hysteresis cycles are featured by low values of coercive magnetic field and high permeability. The application of additive manufacturing to ferromagnetic materials is gaining more and more attraction. Indeed, thanks to a wider geometrical freedom, new topological optimized shapes for stator/rotor shapes can be addressed to enhance electric machines performances. However, the properties of the laser powder bed fusion (LPBF) processed alloy compared to conventionally produced counterpart must be still addressed. Accordingly, this paper presents for the first time the use of the LPBF for the manufacturing of Fe2.9wt.%Si electromagnetic shields. The process parameter selection material microstructure and the magnetic shielding factor are characterized

Characterization of LPBF Produced Fe2.9wt.%Si for Electromagnetic Actuator

This study aims to produce Fe2.9wt.%Si ferromagnetic material via laser powder bed fusion (L-PBF) for the realization of electromagnetic actuators (EMA). This study is necessary as there are no documents in scientific literature regarding the manufacturing of Iron-Silicon plungers using the L-PBF additive manufacturing (AM) technique. The microstructure, and magnetic properties were characterized using various techniques. The results indicate that the samples produced via L-PBF process exhibit good magnetic properties (μ = 748, H C= 87.7 [A/m] ) especially after annealing treatment at 1200° C for 1h (μ = 3224, H C= 69.1 [A/m]), making it a promising material for use in electromagnetic actuators