Effective calcineurin inhibitor treatment in adult-onset steroid-resistant nephrotic syndrome with a novel splice donor site variant of TRPC6: a case report.

Transient receptor potential canonical 6 (TRPC6) variants, which were initially detected in adult-onset familial focal segmental glomerulosclerosis (FSGS), were also identified in pediatric-onset one. Here, we present a patient with adult-onset steroid-resistant nephrotic syndrome (SRNS) who harbored a likely pathogenic TRPC6 variant and partially responded to calcineurin inhibitors (CNIs). A 44-year-old woman with stable rheumatoid arthritis, systemic lupus erythematosus, and Sjögren's syndrome was presented with nephrotic syndrome.

Chirality in Singlet Fission: Controlling Singlet Fission in Aqueous Nanoparticles of Tetracenedicarboxylic Acid Ion Pairs.

The singlet fission characteristics of aqueous nanoparticles, self-assembled from ion pairs of tetracene dicarboxylic acid and various amines with or without chirality, are thoroughly investigated. The structure of the ammonium molecule, the counterion, is found to play a decisive role in determining the molecular orientation of the ion pairs and its regularity, spectroscopic properties, the strength of the intermolecular coupling between the tetracene chromophores, and the consequent singlet fission process. Using chiral amines has led to the formation of crystalline nanosheets and efficient singlet fission with a triplet quantum yield as high as 133% ±20% and a rate constant of 6.

Determination of the point resolution of high-resolution transmission electron microscope using the through-focus technique.

From the viewpoint of evaluating the instrumental performance of high-resolution electron microscopy (HREM), the Scherzer condition was investigated using information theory. As a result, the optimum defocus amount Δf can be expressed based on [Formula: see text] , and the formula [Formula: see text] is obtained. Furthermore, a procedure for measuring point resolution using the through-focus technique is developed, and a new method for determining the spherical aberration coefficient using the variance of Δf is introduced in the procedure.

Renal sinus fat is associated with intrarenal hemodynamic abnormalities independent of visceral fat in patients with chronic kidney disease.

Objective: Obesity is a risk factor of chronic kidney disease (CKD), contributing to the rising incidence of cardiometabolic diseases. Renal sinus fat (RSF) is an ectopic fat depot located at the renal cavity that could impair renal function and hemodynamic through compression of renal structures. The major purpose of this study was to explore the relationship between RSF accumulation and renal dysfunction in CKD patients.

Combined effect of interstitial-substitutional elements on dislocation dynamics in nitrogen-added austenitic stainless steels.

Combined addition of interstitial-substitutional elements has been acknowledged to contribute to the increase in the strengths of steels. For further improvements in mechanical properties, their atomic-scale interaction mechanisms with dislocations are required to be examined. In this study, both high-resolution transmission electron microscopy and atom-probe tomography were used to correlate interstitial-substitutional elements with dislocation characteristics in austenitic stainless steels.

Association Between Renal Sinus Fat and Cardiometabolic and Renin-Angiotensin System Parameters in Primary Aldosteronism.

Context: Renal sinus fat (RSF) accumulation is associated with cardiometabolic diseases, such as obesity, diabetes, hypertension, and chronic kidney disease. However, clinical implications of RSF in primary aldosteronism (PA) remain unclear.

Objective: We aimed to investigate relationships between RSF volume and key cardiometabolic and renin-angiotensin system (RAS) parameters in PA patients and clarify the differences in these relationships between unilateral and bilateral subtypes.

Adipocyte NMNAT1 expression is essential for nuclear NAD biosynthesis but dispensable for regulating thermogenesis and whole-body energy metabolism.

Nicotinamide adenine dinucleotide (NAD) functions as an essential cofactor regulating a variety of biological processes. The purpose of the present study was to determine the role of nuclear NAD biosynthesis, mediated by nicotinamide mononucleotide adenylyltransferase 1 (NMNAT1), in thermogenesis and whole-body energy metabolism. We first evaluated the relationship between NMNAT1 expression and thermogenic activity in brown adipose tissue (BAT), a key organ for non-shivering thermogenesis.

Three-dimensional electron tomography and recent expansion of its applications in materials science.

Electron tomography (ET) is a powerful tool for elucidating the properties and functionalities of materials. The innovative development of aberration-corrected electron microscopy in the early 21st century and the remarkable progress in the development of detectors, equipment and devices related to ET have resulted in substantial improvements in resolution. However, not only advances in hardware but also remarkable developments in reconstruction algorithms and related three-dimensional (3D) analysis methods have contributed to the resolution improvements.

Development of Alveolar Hemorrhage After Pfizer-BioNTech COVID-19 mRNA Vaccination in a Patient With Renal-Limited Anti-neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Case Report.

Since the coronavirus disease 2019 (COVID-19) pandemic continues and a new variant of the virus has emerged, the COVID-19 vaccination campaign has progressed. Rare but severe adverse outcomes of COVID-19 vaccination such as anaphylaxis and myocarditis have begun to be noticed. Of note, several cases of new-onset antineutrophil cytoplasmic antibody-associated vasculitis (AAV) after COVID-19 mRNA vaccination have been reported.

Assessment of coastal anthropo-ecological system dynamics in response to a tsunami catastrophe of an unprecedented magnitude encountered in Japan.

On 11 March 2011, a catastrophic earthquake and subsequent tsunami hit the Pacific coast of northern Japan, devastating many of the towns, villages and coastal ecosystems located along the shoreline. To assess the impacts of the disaster, we investigated temporal dynamics of fish and epibenthic megafaunal community structure in relation to changes in a range of physical, biological and anthropogenic variables between 2007 and 2018 in Onagawa Bay. Commercially important fish such as greenlings, Japanese anchovy, flatfishes, rockfishes were consistently abundant in both larval and adult fish assemblages.

Active Extraction of Experience of Adverse Drug Reactions in Children.

Objective: Safety information regarding the use of medication, over-the-counter drugs, and supplements for Japanese children is scarce. The aim of this study was to clarify adverse drug reaction (ADR) experiences in children and consider the method to collect ADRs efficiently.

Methods: We conducted a questionnaire survey regarding the ADR experiences of 20,412 children who were attending a preschool or kindergarten in the cities of Warabi and Toda, Saitama Prefecture, in May 2013.

Novel nonlinear reconstruction method with grey-level quantisation units for electron tomography.

We report a new computed tomography reconstruction method, named quantisation units reconstruction technique (QURT), applicable to electron and other fields of tomography. Conventional electron tomography methods such as filtered back projection, weighted back projection, simultaneous iterative reconstructed technique, etc. suffer from the 'missing wedge' problem due to the limited tilt-angle range.

Plan-view characterization of intergranular precipitates on grain boundaries by combination of FIB lift out method and TEM analyses: A case study in austenitic stainless steel.

A new characterization method is proposed to study intergranular precipitates of polycrystalline material in the planar manner. A dual beam focused ion beam (FIB) - scanning electron microscopy (SEM) was applied to fabricate thin FIB lamella with a grain boundary parallel to the lamella to investigate for transmission electron microscopy (TEM). Distributions, microstructures and compositions of intergranular precipitates of austenitic stainless steel were then examined by TEM, scanning transmission electron microscopy (STEM), and energy dispersive X-ray spectroscopy (EDS).

Short-term effectiveness of biologics in patients with moderate-to-severe plaque psoriasis: A systematic review and network meta-analysis.

Background: Complete lesion clearance is important to patients with psoriasis.

Objective: To conduct a network meta-analysis of randomized controlled trials of biologic agents available for psoriasis in Japan, using mixed-treatment comparisons.

Methods: MEDLINE and EMBASE were searched to identify randomized clinical trials (placebo-controlled or head-to-head) of infliximab, adalimumab, ustekinumab, secukinumab, ixekizumab, brodalumab, risankizumab or guselkumab in adult patients with moderate-to-severe plaque psoriasis published in English between 01 January 2000 and 31 August 2019.

Analysis of environmental and microbiological changes in Onagawa Bay immediately after the tsunami of the Great East Japan Earthquake based on sediment cores.

We investigated two sediment cores to understand whether a tsunami in Onagawa Bay, Japan caused environmental changes. The value of δC ranged from -21.9‰ to -24.

Nanoscopic Porous Iridium/Iridium Dioxide Superstructures (15 nm): Synthesis and Thermal Conversion by In Situ Transmission Electron Microscopy.

Porous particle superstructures of about 15 nm diameter, consisting of ultrasmall nanoparticles of iridium and iridium dioxide, are prepared through the reduction of sodium hexachloridoiridate(+IV) with sodium citrate/sodium borohydride in water. The water-dispersible porous particles contain about 20 wt % poly(N-vinylpyrrolidone) (PVP), which was added for colloidal stabilization. High-resolution transmission electron microscopy confirms the presence of both iridium and iridium dioxide primary particles (1-2 nm) in each porous superstructure.

Structural and compositional study of precipitates in under-aged Cu-added Al-Mg-Si alloy.

Atomic scale characterization of fine precipitates in an under-aged Cu added Al-Mg-Si alloy was carried out by combination of atomically-resolved annular dark-field scanning transmission electron microscopy and energy dispersive X-ray spectroscopy. Two types of precipitates were observed in the alloy. In the case of ordered β" precipitates, β" was proposed as MgAlSi (x ≈ 1) with solute Cu atoms replacing Al site of β" precipitate.

Scalable faceted voids with luminescent enhanced edges in WS monolayers.

A scalable approach is needed in the formation of atomically flat edges with specific terminations to enhance local properties for optoelectronic, nanophotonic and energy applications. We demonstrate point defect clustering-driven faceted void formations with luminescent enhanced edges in WS2 monolayers during large-scale CVD growth and controlled annealing. With the aid of aberration-corrected scanning transmission electron microscopy (AC-STEM) high angle annular dark field (HAADF) imaging, we probed atomic terminations of S and W to explain observed luminescence enhancement in alternate edges.

Hybridizing semiconductor nanocrystals with metal-organic frameworks for visible and near-infrared photon upconversion.

Hybrid materials consisting of semiconductor nanocrystals and metal-organic frameworks (MOFs) were prepared for the first time to achieve photon upconversion based on triplet-triplet annihilation (TTA-UC) in the solid-state, which allowed TTA-UC with large anti-Stokes shifts in the visible and near-infrared regions.

Photoresponsive Nanosheets of Polyoxometalates Formed by Controlled Self-Assembly Pathways.

Anionic Keggin polyoxometalates (POMs) and ether linkage-enriched ammonium ions spontaneously self-assemble into rectangular ultrathin nanosheets in aqueous media. The structural flexibility of the cation is essential to form oriented nanosheets; as demonstrated by single-crystal X-ray diffraction measurements. The difference in initial conditions exerts significant influence on selecting for self-assembly pathways in the energy landscape.