JGC (Japan)

Primary hyperoxaluria (PH) is a rare autosomal recessive disorder characterized by an abnormal increase in urinary oxalate excretion. Primary hyperoxaluria type 1 (PH1), the most common PH subtype, is caused by a deficiency of the liver-specific enzyme alanine:glyoxylate aminotransferase, which is localized to peroxisomes. In Japan, the status of PH1 has not been studied in two decades, and few longitudinal studies of PH1 patients have been conducted worldwide. This study aims to analyze the present situation regarding PH1 in Japan. We conducted a literature review of PH1 cases reported in Japan between 2003 and 2023. Twenty patients were diagnosed with PH1 during the study period, including 7 males, 12 females, and 1 patient with an unspecified sex. The median ages at symptom onset and diagnosis were 4.21 years (range: 0.17–47) and 5.5 years (range: 0.17–43), respectively. Ten patients (50%) were diagnosed before age 18, four (44%) of whom had already progressed to end-stage renal disease (ESRD). Among the six patients diagnosed after 18 years, all had ESRD or progressed to ESRD soon after diagnosis. The overall survival (OS) rate was 81% at 5, 10 and 20 years post-diagnosis. The mean observation period was 5.62 years (range: 0–32.6). Few longitudinal studies on PH1 patients have been conducted in Japan or worldwide. Over the past 20 years, the median ages at both symptom onset and diagnosis have significantly decreased, and the OS rate has increased. Early diagnosis and intervention may contribute to better outcomes for PH1 patients in Japan.Supplementary InformationThe online version contains supplementary material available at 10.1007/s00240-025-01931-w.

An integrated plant comprising a VOYGR TM plant and a solid oxide electrolysis cell facility was modeled using process simulation to determine the advantages and disadvantages of such an integrated plant.Three different process configurations for supplying heat to the solid oxide electrolysis cell facility from various sources in the VOYGR TM plant, that is, extraction steam from the steam turbine, main steam from the steam generator, and electric power from the generator, were studied and compared.The results indicated that the two steam supply cases had higher total system efficiencies than the all-electric case had.After comparing not only the total system efficiencies but also the plant engineering and operability of the two steam cases and identifying their relative advantages and disadvantages, this study concluded that the most preferrable configuration could not be determined intrinsically since it depended on the particular requirements of the project being considered.Furthermore, the study found that if the total system efficiency of the solid oxide electrolysis cell exceeded some threshold value, then the hydrogen production efficiency of an integrated plant could significantly exceed that of a VOYGR TM plant operating alone and the carbon-free hydrogen production of such an integrated plant could surpass the system efficiency of a conventional nuclear power plant.

AimWith the rapid increase of aging societies worldwide, ensuring road safety among older adults has become a critical public health concern. This study aimed to assess driving risks among community‐dwelling older adults with mild cognitive impairment (MCI).MethodsA total of 387 participants aged 65 years and older were recruited from Akita Prefecture, Japan. Physical functions, such as walking speed and grip strength, cognitive functions, and depressive symptoms were evaluated alongside driving behaviors. Participants were classified into MCI‐positive and MCI‐negative groups based on cognitive assessments.ResultsThe results demonstrated significant differences in driving risk, physical performance, and cognitive abilities between the two groups. MCI‐positive individuals exhibited higher driving risks and poorer functional performance. Receiver operating characteristic (ROC) curve analysis identified a cutoff score of 5.5 on the Driving Risk Checklist‐25 (DRCL‐25), with a sensitivity of 45.5% and specificity of 89.7% for identifying MCI.ConclusionsThese findings underscore the importance of integrating physical and cognitive assessments to enhance the accuracy of driving risk evaluations. The DRCL‐25 shows promise as a detection tool for MCI, facilitating the development of interventions aimed at maintaining mobility and improving road safety among older drivers. Future research should focus on refining the checklist and evaluating its applicability across diverse populations and settings. Geriatr Gerontol Int 2025; 25: 1194–1199.

Abstract Where sizes of run pipe and branch are largely different, branch outlet fittings set on run pipes are usually selected to compose branch joints. CRA (corrosion resistant alloy) solid piping materials are often selected when process fluids are corrosive, e.g., produced water of oil and gas crude. To optimize cost of process plant construction, CRA clad or weld overlay on CS (carbon steel) / low alloy steel parent metal (hereafter CRA clad material) may be applied to process piping. However, manufacturing process of piping materials constrains the minimum diameter of CRA clad piping materials such as NPS 6 (DN 150), therefore, CRA solid materials are used to lesser diameters than the minimum. CRA clad materials also constrain the minimum size of fabricated branch design because branch holes on CRA clad piping material must also be protected by CRA weld overlay. And accessibility of welding torch/arc and visibility of molten pool during weld overlay work on small bore holes must be considered in terms of weldability and weld overlay quality. To make up for these shortcomings, other production method for holes on CRA clad run piping materials, such as insert sleeve made of CRA materials welded with groove joint on the holes, were introduced. Mockup coupons of the sleeve design branch and conventional CRA weld overlay design were prepared with branch outlet fitting, and weldability and corrosion resistance were studied by the comparison using both mockup coupons. This paper discusses and evaluates the weldability, corrosion resistance and materials selection of both branch designs and potentials that can eliminate the piping design constraints with their comparison.

Abstract This study presents the replication analysis of the shaking test for floating seismic isolation system (FSIS) conducted using the world largest shaking table “E-Defense” in Japan. The FSIS technology can provide prominent seismic isolation effects not only for the horizontal direction but also for the vertical direction. One of the unique features in FSIS is the air cavity rooms equipped with the bottom side of the floating structure. These air cavity rooms play a role of suspensions reducing resonant frequency of the floating structures in the vertical motions so that the seismic isolation effect can be obtained for the vertical direction. Although the seismic isolation effects were substantiated by the shaking tests, there were some limitations in the shaking test. In this study, the dynamic analyses have been performed to comprehend the dynamic behaviors of the floating structures in the shaking test by replicating the same situation as the shaking test, and to evaluate the actual seismic isolation performance by eliminating factors disturbing the seismic isolation effects in the shaking test. Through the replication analyses, main dynamic modes of the FSIS test facilities could be represented. Also, the simulation results revealed that the bulging effect of the pool vessel augments the response of the floating structure so that the seismic isolation effects were reduced. This effect was underpinned by conducting the sensitivity analyses on the condition that the pool vessel was changed to be stiffer. This fact will contribute to the development of the seismic analysis methodology applicable to the actual design of FSIS paired nuclear power plants.

A compact planar phased array equipped with an innovative low-cost dielectric lens, which significantly improves performance in terms of gain, beam direction, and sidelobe levels, has been designed and realized. The proposed system, suitable for future millimeter-wave communications, operates from 27.5 to 29.5 GHz, offers high gain, and is capable of beam scanning up to <tex xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink">$\pm 60^{\circ}$</tex>.

Current measures to prevent viral infections use disinfectants that are highly irritating to the skin and cause health problems such as alcohol-induced hypersensitivity. Therefore, a demand for safer disinfectants exists. ε-Poly-L-lysine (polylysine, EPL) has been used as a food preservative for many years because of its safety and antibacterial properties. In recent years, we developed an antiviral agent consisting only of food additives containing EPL. Studies on feline calicivirus, a norovirus substitute, have shown that serine proteases decompose the protein structure of the outer shell of this virus. The combination of serine proteases and EPL further enhanced the virucidal effects of serine proteases like subtilisin. Investigations of the antiviral effects of this combination revealed its inhibitory effects on enveloped viruses, such as the influenza A virus, but not on non-enveloped viruses. To confirm the damage to viruses, whose infectivity was inhibited by the combined treatment with serine proteases and EPL, morphological changes in virus particles after treatment were observed using transmission electron microscopy. Following the treatment with serine proteases or EPL, the outer shells of the examined viruses were damaged. As the outer shell contributes to the infection of host cells, this morphological alteration might be responsible for the reduced infectivity of these viruses after the combination treatment with serine proteases and EPL.

Representing molecules as graphs is a natural approach for capturing their structural information, with atoms depicted as nodes and bonds as edges. Although graph-based similarity calculation approaches, such as the graph edit distance, have been proposed for calculating molecular similarity, these approaches are nondeterministic polynomial (NP)-hard and thus computationally infeasible for routine use, unlike fingerprint-based methods. To address this limitation, we developed GESim, an ultrafast graph-based method for calculating molecular similarity on the basis of von Neumann graph entropy. GESim enables molecular similarity calculations by considering entire molecular graphs, and evaluations using two benchmarks for molecular similarity suggest that GESim has the ability to differentiate between highly similar molecules, even in cases where other methods fail to effectively distinguish their similarity. GESim is provided as an open-source package on GitHub at https://github.com/LazyShion/GESim.

Abstract Back shielding by inert gas is required to not only single-sided butt joints but also fillet joints of piping in accordance with specification or standard such as API RP582 [1] where chromium content of base metal is high, e.g., greater than 2.5 %, and wall thickness is thin, e.g., 1/4” (6.5 mm) or lesser, for preventing oxidation in atmospheric environment by welding heat input. However, the volume of back shielding space on inside piping by fillet welding, such as joints between run pipe and piping support or reinforcing pad, is sometimes too large to purge oxygen out with economical rationality due to limitation of place of back shielding isolations, especially during piping erection period at construction site. Recently, non-backing gas practices with gas metal arc welding in short circuit mode (GMAW-S) [2] and gas tungsten arc welding (GTAW) [3] have been developed and implemented to single-sided butt joints of stainless steel (SS) process piping in oil and gas industries with successful results [4]. Therefore, oxidation of the fluid-contacting side can be considered as nonmaterial from a corrosion resistance viewpoint regardless of welding method and back shielding. This paper presents study results of corrosion resistance of fillet welds on SS piping in comparison to back shielding conditions and introduces the possibility of omission of back shielding for fillet welds.