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IN 2021, BAPETEN, AS THE REGULATORY BODY, IS ESTABLISHING A BAPETEN REGULATION REGARDING THE REACTOR COOLANT SYSTEM AND RELATED SYSTEMS, WHICH CURRENTLY ARE NOT YET AVAILABLE. Therefore, it is crucial to establish the BAPETEN Regulation. Based on the reasons, before setting the BAPETEN Regulation, it is necessary to conduct a study that is expected to provide a more comprehensive description and provide recommendations on what things need to be regulated in the BAPETEN Regulation, especially for gas-cooled reactors. The method used in this study is a literature study from various relevant references. The result of this study is that it is essential to require a capacity of the ultimate heat sink, including the spent nuclear fuel storage pool and a minimum period of the ability of the top heat sink in the accident analysis if the decay heat in the storage pool and the residual heat in the reactor core fail simultaneously. On the other hand, it is also necessary to require a margin of uncertainty to evaluate a situation and take corrective action. Likewise, independent and redundant access to the ultimate heat sink is needed to increase reliability. As for gas-cooled reactors, it is required to adapt the terms used. In addition, it is necessary to determine the appropriate definition because some of the terms used in water-cooled reactors have the same terms as gas-cooled reactors but have different functions. Keywords: Regulatory assessment, coolant system, related systems, gas-cooled reactors

Abstract Introduction: Platelet function disorders (PFDs) are a group of heterogenous bleeding disorders with varying bleeding phenotype. Intraoperative and post-operative bleeding are serious complications among patients with PFDs undergoing surgery. There are very few studies in literature that have specifically investigated surgery associated bleeding complications in PFDs. The aim of this study was to utilize a large national dataset to describe surgeries performed in patients with PFD, characterize the bleeding associated with these surgical procedures and outline the therapeutic approaches adopted. Methods: In this retrospective study, the ATHNdataset was queried for demographic data, PFD diagnosis, surgeries among patients with PFD, intraoperative and post-operative bleeding episodes and treatment. Descriptive statistics were used. The ATHNdataset captures information from patients with bleeding and clotting disorders from over 140 federally funded hemophilia and thrombosis treatment centers (HTCs) in the US. Patients authorize inclusion of their demographic and clinical information in this de-identified Health Insurance Portability and Accountability Act (HIPAA)-compliant data set. Results: From January 2010 to March 2020, the ATHNdataset captured 2767 patients with PFDs, of which 1769 (63.93%) were female and 998 (36.1%) were male, with 1393 patients between 0-18 years (50%) and 1374 (50%) adults >18 years. PFDs identified include 32 patients with Bernard Soulier syndrome (1.16%), 131 patients with Glanzmann thrombasthenia (4.7%), 4 patients with Gray platelet syndrome (0.14%), 29 patients with Hermansky Pudlak syndrome (1%), 1548 patients with storage pool deficiency (55.9%), and 1023 patients diagnosed as PFD (36.9%). A total of 3252 procedures were reported between 2010 and 2020; 1271 patients (46%) patients with at least one documented procedure. Figure 1 shows common procedures among patients with PFDs. Surgery-associated bleeding episodes (includes intraoperative and post-operative bleeds) were reported with 69 procedures (2.1%), which included intraoperative bleeds reported for 18 procedures (0.5%) and post-operative bleeds reported for 51 procedures (1.6%). Of the 60 procedures in patients with Glanzmann thrombasthenia, surgery-associated bleeding episodes were reported after 9 dental procedures (41%), 1 circumcision (25%) and 11 other surgeries/procedures (18.3%). Of the 6 procedures in patients with Bernard Soulier syndrome, no intraoperative or post-operative bleeding episodes were reported. Of 1688 procedures in patients with storage pool deficiency, surgery-associated bleeding episodes were reported after 26 dental procedures (1.5%) and 62 other surgeries/procedures (3.67%). No intraoperative or post-operative mortality was reported among these patients. Of 1272 patients who underwent at least 1 procedure, 646 patients (50.7%) received some form of treatment before/during/after a procedure. Among these 646 patients, 2794 exposure days of hemostasis medications were used before/during/after procedures. Among these, 49% were prior to the procedure, 0.7 % during the procedure and 49.5% after the procedure. Treatments used are shown in figure 2. Conclusion: Our study shows that patients with PFDs have a substantial risk of bleeding associated with surgery. Identifying the risk of bleeding by type and providing appropriate pre-surgical prophylaxis can decrease rates of surgery-associated bleeding in PFDs. Figure 1 Figure 1. Disclosures Sidonio: Sanofi, Takeda, Octapharma, Bayer, Biomain, Grifols, Kedrion, Genentech. Catalyst, Guardian Therapeutics, Novo Nordisk, Hema Biologics, Uniqure.: Consultancy, Honoraria. Ahuja: Genentech: Membership on an entity's Board of Directors or advisory committees; Takeda: Other: DSMB member ; XaTek, Inc: Patents & Royalties; Sanofi: Membership on an entity's Board of Directors or advisory committees.

Abstract Introduction/Objective Microdeletion of a region of the short arm of chromosome 19 results in a very rare syndrome called 19p13.3 deletion syndrome, which manifest itself in developmental delay as well as structural abnormalities such as facial dysmorphism and macrocephaly. Methods/Case Report We present a case of 14-month-old patient, born at term and was large for her gestational age. She had dysmorphic facial features including posterior cleft palate for which, she required placement of G-tube. Post-delivery, she experienced respiratory distress as well as hypoglycemic episodes. Over the period of time, her mother also noticed occasional bleeding through her gums with teething. Genetic workup was performed, which revealed 2.4 Mb of microdeletion at chromosome 19 region p13.3, including deletion of PIAS4, MAP2K2, GNA11, TBXA2R, RAX2 genes. TBXA2R mutation is associated with bleeding disorder due to a defect in platelet aggregation. The mutation in TBXA2R can lead to platelet type 13 bleeding disorder. For this purpose, a platelet aggregation study was performed to evaluate platelet function disorders. However, the result of the platelet aggregation study was inconclusive as it showed decrease responses to all agonists including arachidonic acid, epinephrine, ADP, collagen and ristocetin. Further work-up by electron microscopy (EM) of platelets (PL) revealed a significant decrease of delta granules (DG) (0.89 DG/PL, normal 4-6 DG/PL), consistent with delta granule storage pool deficiency (δ-SPD). Other abnormalities observed by EM included occasional gray platelets, platelets with immature and/or decreased numbers of α-granules, and rare giant α-granules. Results (if a Case Study enter NA) NA Conclusion To the best of our knowledge, no other case of 19p13.3 microdeletion syndrome with δ-SPD and associated abnormalities in α-granules has previously been described in the literature. Although it is unclear if there is any relationship between δ-SPD and 19p13.3 deletion syndrome, further investigation is warranted.

Many issues associated with managing centralized database include data isolation, redundancy, inconsistency, and atomicity of updates, among others; however, distributed database implementation over high-performance compute nodes maximizes information value across the networks. Also, analysis of bigdata generated/consumed over the mobile Internet, Internet of Things (IoT) and cloud computations necessitates low-latency reads and updates over cloud clusters. Conventionally, services in distributed systems demand optimized transactions. This paper examines transaction generation over distributed storage pool using suggested reference architectures of fragmentation using hybrid semi-join operations to offer mobility transparency as an additional ingredient of integrity transparency offer of DDBMS. Distributed storage pool is simulated using configured WLAN to activate multiple file transfers concurrently, engaging mobile nodes and large file sizes. Major functionality desired in the storage pool is improvised by storage virtualization whereby a global schema query optimizer effects transaction management to characterized latency-driven throughputs achieved by joint optimization of network and storage virtualization. Measurements and evaluations gave the best overall performance of low-latency reads and updates using the provisioned mobile-transmission control protocol (M-TCP). Appreciable improvement in service delivery is offered using distributed storage pool (DSP) facilitated with hybridized RAID construction and copy mechanisms. Improved response-time and speed-up transmissions evidently showed low-latency read and update transactions, depicting improved service delivery. Evaluating the DDBMS model simulated in the DSP architecture, all complexity (overheads) associated with conventional shared systems were minimized.

Introduction male circumcision is a common procedure, generally performed during the newborn period. Few reports have described circumcision in patients with bleeding disorders. Aim to determine bleeding rate after circumcision in neonatal male subjects who were diagnosed later in life with delta-storage pool disease (SPD). Methods we retrospectively reviewed the medical records of male subjects (<18 years of age) who were diagnosed with SPD later in life and were circumcised at birth without hemostatic prophylaxis due to lack of family history at that time from 2000-2020. Intraoperative/postoperative bleeding and bleeding severity were the main outcomes evaluated. Results 153 male subjects were included. Circumcision was performed at a median age of 2 days (range, 1 day-4 months). The main indication for circumcision was parental request. Median severity of granule deficiency was 2.76 dense granules/platelet (range, 1.12-3.82 DG/Plt). None of the subjects had intraoperative bleeding. Three subjects (2%) had postoperative bleeding and only one (0.65%) required ER intervention to stop bleeding. Conclusion the overall incidence of bleeding in our subjects with SPD who were undiagnosed and untreated at circumcision, is comparable to that reported for patients without a bleeding disorder.

AbstractMagma ascent, storage, and discharge in the trans-crustal magmatic system are keys to long-term volcanic output and short-term eruption dynamics. How a distinct magma batch transports from a deep reservoir(s) to a pre-eruptive storage pool with eruptible magma remains elusive. Here we show that repetitive very-long-period signals (VLPs) beneath the Aso volcano are preceded by a short-lived (~50–100 s), synchronous deformation event ~3 km apart from the VLP source. Source mechanism of a major volumetric component (~50–440 m3 per event) and a minor low-angle normal-fault component, together with petrological evidence, suggests episodic transport of discrete magma batches from an over-pressured chamber roof to a pre-eruptive storage pool near the brittle-ductile transition regime. Magma ascent velocity, decompression rate, and cumulative magma output deduced from recurrent deformation events before recent 2014 and 2016 eruptions reconcile retrospective observations of the eruption style, tephra fallouts, and plume heights, promising real-time evaluation of upcoming eruptions.

The misleading information spreads into the society, the people move on the basis of it. This mechanism, what I call “rumor disaster” is examined in this note. Authorities, specialists, intellectuals, and so on, oftentimes, might take part in this process. In this note, the spent fuel storage pool of Tokyo Electric Power Fukushima Daiichi N.P.P. Unit 4 is the case used for discussion because there are relatively abundant materials and the resulting impact to the society is very big.