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Background and Aims: Biliary atresia is a rare and devastating bile duct disease that occurs during the neonatal period. Timely identification and prompt surgical intervention is critical for improving the outcome. The aim of the study was to develop a new machine learning–based prediction model for the detection of biliary atresia. Methods: Neonates aged

Background & Aims: Bile salt export pump (BSEP) deficiency frequently necessitates liver transplantation in childhood. In contrast to two predicted protein truncating mutations (PPTMs), homozygous p.D482G or p.E297G mutations are associated with relatively mild phenotypes, responsive to surgical interruption of the enterohepatic circulation (siEHC). The phenotype of patients with a compound heterozygous genotype of one p.D482G or p.E297G mutation and one PPTM has remained unclear. We aimed to assess their genotype-phenotype relationship. Methods: From the NAPPED database, we selected patients with homozygous p.D482G or p.E297G mutations (BSEP1/1; n = 31), with one p.D482G or p.E297G, and one PPTM (BSEP1/3; n = 30), and with two PPTMs (BSEP3/3; n = 77). We compared clinical presentation, native liver survival (NLS), and the effect of siEHC on NLS. Results: The groups had a similar median age at presentation (0.7-1.3 years). Overall NLS at age 10 years was 21% in BSEP1/3 vs. 75% in BSEP1/1 and 23% in BSEP3/3 (p

A FAC (flow-accelerated corrosion) test was performed for a straight pipe composed of the SA335 Gr P22 and SA106 Gr B (SA106-SA335-SA106) types of steel with welds as a function of the flow rate in the range of 7–12 m/s at 150 °C and with DO

Conventional integral effect test facilities were constructed to enable the precise observation of thermal–hydraulic phenomena and reactor behaviors under postulated accident conditions to prove reactor safety. Although these facilities improved the understanding of thermal–hydraulic phenomena and reactor safety, applications of new technologies and their performance tests have been limited owing to the cost and large scale of the facilities. Various nuclear technologies converging 4th industrial revolution technologies such as artificial intelligence, drone, and 3D printing, are being developed to improve plant management strategies. Additionally, new conceptual passive safety systems are being developed to enhance reactor safety. A new integral effect test facility having a noticeable scaling ratio, i.e., the (UNIST reactor innovation loop (URI-LO), is designed and constructed to improve the technical quality of these technologies by performance and feasibility tests. In particular, the URI-LO, which is constructed using a transparent material, enables better visualization and provides physical insights on multidimensional phenomena inside the reactor system. The facility design based on three-level approach is qualitatively validated with preliminary analyses, and its functionality as a test facility is confirmed through a series of experiments. The design feature, design validation, functionality test, and future utilization of the URI-LO are introduced.

Summary: Background: Few studies have described the aetiologies of neonatal cholestasis, and the overall neonatal cholestasis-related mortality (NCM) rate is unclear. We investigated the aetiology and outcome of neonatal cholestasis in a tertiary hospital and developed an NCM prediction model for these patients. Methods: Patients aged 1.0 mg/dL were retrospectively screened. Diagnostic and laboratory data during the 8-week follow-up period after enrolment between 2005 and 2020 were extracted digitally, and medical charts were reviewed manually by clinicians. Logistic regression was used to derive a prediction model for the 1-year mortality outcome of neonatal cholestasis, and performance evaluation and external validation were conducted for the NCM prediction model. Findings: We enrolled 4028 neonates with DB of >1.0 mg/dL at least once. Prematurity and birth injury (35.4%), complex heart anomalies (18.6%), liver diseases (11.4%), and gastrointestinal anomalies (9.2%) were the most common aetiologies; 398 (9.9%) patients died before one year of age. The peak value of DB was positively correlated to the 1-year mortality rate. In the multivariate analysis, simple laboratory indices, including platelet, prothrombin time, aspartate aminotransferase, albumin, direct bilirubin, creatinine, and C-reactive protein, were independent predictors of 1-year mortality outcome of complete-case subjects. Using these laboratory indices, a logistic regression-based NCM prediction model was constructed. It showed acceptable performances on discrimination (area under the curve, 0.916), calibration (slope, 1.04) and Brier scoring (0.072). The external validation of the sample (n = 920) from two other centres also revealed similar performance profiles of the NCM model. Interpretation: Various aetiologies of neonatal cholestasis were identified in a tertiary hospital, resulting in unfavourable outcomes of a large proportion. The NCM prediction model may have the potential to help clinicians to be aware of high-risk neonatal cholestasis.

Thermal management of mobile electronics has been carried out because performance of the application processor has increased and power dissipation in miniaturized devices is proportional to its functionalities. There have been various studies on thermal analyses related to mobile electronics with the objectives of improving analysis methodologies and cooling strategies to guarantee device safety. Despite these efforts, failure to control thermal energy, especially in smartphones, has resulted in explosions, because thermal behaviors in the device under various operating conditions have not been sufficiently conducted. Therefore, several scenarios that caused the failure in thermal management of smartphone was analyzed to provide improved insight into thermal design deducing the parameters, that affect the thermal management of device. Overcurrent in battery due to malfunction of battery management system or immoderate addition of functionalities to the application processor are considered as reliable causes leading to the recent thermal runaways and explosions. From the analyses, it was also confirmed that the heat generation of the battery, which have not been considered importantly in previous literature, has significant effect on thermal management, and heat spreading could be suppressed according to arrangement of AP and battery. The heat pipe, which is utilized as a cooling device in mobile electronics, was also included in the thermal analyses. Although the heat pipes have been expected to improve the thermal management in mobile electronics, it showed limited heat transfer capacity due to its operating conditions and miniaturization. The demonstrated results of our analysis warn against vulnerabilities of smartphones in terms of safety in design. Keywords: Smartphone, Thermal analysis, Cooling, Lithium ion battery, Heat pipe

MPV17-related hepatocerebral mitochondrial DNA depletion syndrome (MDS) is a very rare condition, and only a few cases have been reported in East Asian countries. Here, we describe four Korean children affected by hepatocerebral MDS. The DGUOK, POLG1, and MPV17 genes were analyzed, and all patients had MPV17 mutations.

A combination of flow-accelerated corrosion (FAC) tests and corresponding computational fluid dynamics (CFD) tests were performed to determine the hydrodynamic parameters that could help predict the highly susceptible location to FAC in the elbow section. The accelerated FAC tests were performed on a specimen containing elbow sections fabricated using commercial 2-inch carbon steel pipe. The tests were conducted at flow rates of 9 m/s under the following conditions: water temperature of 150 °C, dissolved oxygen

Flow accelerated corrosion (FAC) is a type of pipe corrosion in which the pipe thickness decreases depending on the fluid flow conditions. In nuclear power plants, FAC mainly occurs in the carbon steel pipes of a secondary system. However, because the temperature of a secondary system pipe is over 150 °C, in situ monitoring using a conventional ultrasonic non-destructive testing method is difficult. In our previous study, we developed a waveguide ultrasonic thickness measurement system. In this study, we applied a waveguide ultrasonic thickness measurement system to monitor the thinning of the pipe according to the change in pH.The Korea Atomic Energy Research Institute installed FAC-proof facilities, enabling the monitoring of internal fluid flow conditions, which were fixed for ~1000 h to analyze the effect of the pH. The measurement system operated without failure for ~3000 h and the pipe thickness was found to be reduced by ~10% at pH 9 compared to that at pH 7. The thickness of the pipe was measured using a microscope after the experiment, and the reliability of the system was confirmed with less than 1% error. This technology is expected to also be applicable to the thickness-reduction monitoring of other high-temperature materials.

Cast austenitic stainless steel (CASS) is used for fabricating different components of the primary reactor coolant system of pressurized water reactors. However, the thermal embrittlement of CASS resulting from long-term operation causes structural safety problems. Ultrasonic testing for flaw detection has been used to assess the thermal embrittlement of CASS; however, the high scattering and attenuation of the ultrasonic wave propagating through CASS make it difficult to accurately quantify the flaw size. In this paper, we present a different approach for evaluating the thermal embrittlement of CASS by assessing changes in the material properties of CASS using a nonlinear ultrasonic technique, which is a potential nondestructive method. For the evaluation, we prepared CF8M specimens that were thermally aged under four different heating conditions. Nonlinear ultrasonic measurements were performed using a contact piezoelectric method to obtain the relative ultrasonic nonlinearity parameter, and a mini-sized tensile test was performed to investigate the correlation of the parameter with material properties. Experimental results showed that the ultrasonic nonlinearity parameter had a correlation with tensile properties such as the tensile strength and elongation. Consequently, we could confirm the applicability of the nonlinear ultrasonic technique to the evaluation of the thermal embrittlement of CASS. Keywords: Cast austenitic stainless steel, CF8M, Nonlinear ultrasonic technique, Tensile test

In order to monitor the pipe thinning caused by flow-accelerated corrosion (FAC) that occurs in coolant piping systems, a shear horizontal ultrasonic pitch-catch waveguide technique was developed for accurate pipe wall thickness monitoring. A clamping device for dry coupling contact between the end of the waveguide and pipe surface was designed and fabricated. A computer program for multi-channel on-line monitoring of the pipe thickness at high temperature was also developed. Both a four-channel buffer rod pulse-echo type and a shear horizontal ultrasonic waveguide type for high-temperature thickness monitoring system were successfully installed to the test section of the FAC proof test facility. The overall measurement error can be estimated as ± 10 μm during a cycle from room temperature to 200°C.