An unusual coalition of medullary nephrocalcinosis with a novel genotypic variant of Alport syndrome type-1

Alport’s syndrome is a type of inherited disorder of the basement membrane characterized by a spectrum of phenotypes ranging from progressive renal injury to varied extrarenal manifestations comprising auditory and ocular abnormalities. Here in, we present a 3-year-old child born out of nonconsanguineous marriage who presented with fever, intermittent microscopic haematuria, and recurrent gross haematuria, proteinuria with normal auditory brainstem response and ocular slit lamp examination findings. Renal biopsy yielded normal light microscopy and immunofluorescence study whereas minimal changes in the glomerular basement membrane (GBM) collagen were detected on electron microscopy, suggesting possibilities of Alport’s syndrome. Ultrasonographic renal imaging yielded the presence of bilateral medullary nephrocalcinosis. Angiotensin converting enzyme inhibitors along with angiotensin receptor blockers were used to curb the disease progression. A final clinical exome sequencing corroborated the phenotype with a diagnosis of Alport’s syndrome type-1 linked to a novel pathogenic variant c.1892dup (p.Gly632ArgfsTer2) showing hemizygous single base pair insertion/duplication in COL4A5 gene. To the best of our knowledge, this unusual association of Alport’s syndrome with medullary nephrocalcinosis has not been reported worldwide in any previous medical literature making this report a primi one

Digital Twin for Power Plants, Energy Savings and other Complex Engineering Systems

Digital Twin (DT) is a digital representation of a machine, service, or production system that consists of models, information, and data used to characterize properties, conditions, and behavior of the system. Renewable energy integration will make future power plants more complex with addition of varieties of Power-to-X technologies, Electrolysis to green hydrogen, onsite storage and transport of hydrogen, and use of pure or blended hydrogen, etc. These future power plants need robust DT architecture to achieve high Reliability, Availability and Maintainability at lower cost. In this research work, a comprehensive and robust DT architecture for power plants is proposed that also can be implemented in other similar complex capital-intensive large engineering systems. The novelty and advantages of the proposed DT is asserted by reviewing the state-of-the-art of DT in energy industries and its potential to transform these industries. Then the proposed DT architecture and its five components are explained and discussed. More specifically, the main contributions of the present work include: 1. Overview of DT key research and development for energy savings applications to consider important findings, research gaps and the needed future development for the proposed DT for power plants. 2. Overview of DT key research for power plants including applications, frameworks and architectures to consider important findings and to confirm the novelty and robustness of the proposed DT. 3. Proposing and demonstrating new robust DT architecture for power plants and other similar complex capital-intensive large engineering systems

Systems Design, Fabrication, and Testing of a High-Speed Miniature Motor for Cryogenic Cooler

The long-term storage of liquid hydrogen for space missions is of considerable interest to NASA. To this end, the Reverse Turbo-Brayton Cryocooler (RTBC) is considerably lighter than conventional designs and a potentially viable and attractive solution for NASA's long-term Zero-Boil-off (ZBO) hydrogen storage system for future space missions. We present the systems design, fabrication, and performance evaluation of the Permanent Magnet Synchronous Motor (PMSM) powering a cryocooler capable of removing 20 W of heat at 18 K with a COP of 0.005 and driven by two 2-kW permanent magnet synchronous motors operating at 200 000 rpm and at room temperature and 77 K. Structural, thermal, and rotordynamic aspects of system design are considered

Biocontrol Potential of Forest Tree Endophytes

Peer reviewe

An Experimental Investigation Of Liquid Jet Impingement And Single-Phase Spray Cooling Using Polyalphaolefin

Experiments on triangular and rectangular array jet impingement and single-phase spray cooling have been performed to determine the effect of both cooling techniques on heat transfer coefficient ( h ) and the coolant mass flux required for a given cooling load. Experiments were performed with circular orifices and nozzles for different H / D values from 1.5 to 26 and Reynolds number range of 219 to 837, which is quite lower than the ranges employed in widely used correlations. The coolant used was polyalphaolefin. The experiments simulated the boundary condition produced at the surface of the stator of a high power low-density generator or motor. For the custom fabricated orifices, commercial nozzles, and conditions used in this study, both cooling configurations showed enhancement of heat transfer coefficient as H / D increases to a certain limit after which it starts to decrease. The heat transfer coefficient always increases with Reynolds number. In keeping with previous studies, single-phase spray cooling technique can provide the same heat transfer coefficient as jets at a slightly lower mass flux, but with much higher-pressure head. Special Nu d correlations that account for the range of parameters and coolant studied in this work are derived

Comparison Between Evm And Rsm Turbulence Models In Predicting Flow And Heat Transfer In Rib-Roughened Channels

A 3D analysis of two-equation eddy viscosity (EVMs) and Reynolds stress (RSM) turbulence models and their application to solve flow and heat transfer in rotating rib-roughened internal cooling channels is the main focus of this study. The flow in these channels is affected by ribs, rotation, buoyancy, bends and boundary conditions. The EVMs considered are the standard k-ε model of Launder and Spalding [1], the renormalization group k-ε model of Yakhot and Orszag [2], the realizable k-ε model of Shih et al. [3], the standard k-ω model of Wilcox [4] and the shear-stress transport (SST) k-ω model of Menter [5]. The viscosity-affected near-wall region is resolved by enhanced near-wall treatment using combined two-layer model with enhanced wall functions. The results for both stationary and rotating channels showed the advantages of Reynolds stress model (RSM), Gibson and Launder [6], Launder [7] and Launder et al. [8] in predicting the flow field and heat transfer compared to two-equation EVMs that need corrections to account for streamline curvature, buoyancy and rotation. © 2006 Taylor & Francis

Effect Of Coriolis And Centrifugal Forces On Turbulence And Heat Transfer At High Rotation And Buoyancy Numbers In A Rib-Roughened Internal Cooling Channel

Prediction of three-dimensional flow field and heat transfer in a two pass rib-roughened square internal cooling channel of turbine blades with rounded staggered ribs rotating at high rotation and density ratios is the main focus of this study. Rotation, buoyancy, ribs, and geometry affect the flow within these channels. The full two-pass channel with bend and with rounded staggered ribs with fillets (e/Dh =0.1 and P/e=10) as tested by Wagner et. al [1992] is investigated. RSM is used in this study and enhanced wall treatment approach to resolve the near wall viscosity-affected region. RSM model was validated against available experimental data (which are primarily at low rotation and buoyancy numbers). The model was then used for cases with high rotational numbers (0.24, 0.475, 0.74 and 1) and high-density ratios (0.13, 0.23, and 0.3). Particular attention is given to how secondary flow, Reynolds stresses, turbulence intensity, and heat transfer are affected by coriolis and buoyancy/centrifugal forces caused by high levels of rotation and density ratios. A linear correlation for 4-sideaverage Nusselt number as a function of rotation number is derived

Effect Of Coriolis And Centrifugal Forces On Turbulence And Transport At High Rotation And Density Ratios In A Rib-Roughened Channel

Prediction of three-dimensional flow field and heat transfer in a two pass rib-roughened square internal cooling channel of turbine blades with rounded staggered ribs rotating at high rotation and density ratios is the main focus of this study. Rotation, buoyancy, ribs, and geometry affect the flow within these channels. The full two-pass channel with bend and with rounded staggered ribs with fillets (e / Dh = 0.1 and P / e = 10) as tested by Wagner et al. [J.H. Wagner, B.V. Johnson, R.A. Graziani, F.C. Yeh, Heat transfer in rotating serpentine passages with trips normal to the flow, ASME J. Turbomach. 114 (1992) 847-857] is investigated. Reynolds stress model (RSM) turbulence model is used for this study. To resolve the near wall viscosity-affected region, enhanced wall treatment approach is employed. RSM model was validated against available experimental data (which are primarily at low rotation and buoyancy numbers). The model was then used for cases with higher rotational numbers (0.24, 0.475, 0.74 and 1) and higher density ratios (0.13, 0.23, and 0.3). Particular attention is given to how secondary flow, Reynolds stresses, turbulence intensity, and heat transfer are affected by Coriolis and buoyancy/centrifugal forces, caused by high levels of rotation and density ratios. A linear correlation for 4-side-average Nusselt number as a function of rotation number is derived. © 2007 Elsevier Masson SAS. All rights reserved

Fluid Flow And Heat Transfer In Rotating Curved Duct At High Rotation And Density Rations

Prediction of flow field and heat transfer of high rotation numbers and density ratio flow in a square internal cooling channels of turbine blades with U-turn as tested by Wagner et. al (1991) is the main focus of this study. Rotation, buoyancy and strong curvature affect the flow within these channels. Due to the fact that RSM turbulence model can respond to the effects of rotation, streamline curvature and anisotropy without the need for explicit modeling, it is employed for this study as it showed improved prediction compared to isotropic twoequation models. The near wall region was modeled using enhanced wall treatment approach. RSM was validated against available experimental data (which are primarily at low rotation and buoyancy numbers). The model was then used for cases with high rotation numbers (as much as 1.29) and high-density ratios (up to 0.4). Particular attention is given to how secondary flow, velocity and temperature profiles, turbulence intensity and Nusselt number area affected by coriolis and buoyancy/centrifugal forces caused by high levels of rotation and buoyancy in the immediate vicinity of the bend. The results showed that 4-side-average Nu, similar to low Ro cases, increases linearly by increasing rotation number and, unlike low Ro cases, decreases slightly by increasing density ratio