Experimental Thermal–Hydraulic Testing of a Mock-Up of the Fuel-Breeder Pin Concept for the EU-DEMO HCPB Breeding Blanket

The fusion program in the Karlsruhe Institute of Technology (KIT) leads the R&D of the DEMO helium-cooled pebble bed (HCPB) breeding blanket within the work package breeding blanket (WPBB) of the Eurofusion Consortium in the European Union (EU). A new design of the HCPB breeder zone, with a layout inspired by a nuclear reactor fuel rod arrangement, was developed recently and called the fuel-breeder pin concept. In addition, a mock-up (MU) of this fuel-breeder pin was designed and manufactured at KIT in order to test and validate its thermal–hydraulic performance. This paper reports on the results of the first experimental campaign dedicated to the fuel-breeder pin MU testing that was performed in the Helium Loop Karlsruhe (HELOKA) facility. The paper presents: (i) the integration of the fuel-breeder pin MU into the HELOKA loop including considerations of the experimental set-up, (ii) an overview of the plan for the experimental campaigns, and (iii) a discussion of the experimental results with a focus on aspects relevant for the validation of the thermal–hydraulic design of the HCPB breeder zone

Experimental thermal-hydraulic testing of a prototypical mock-up of the fuel-breeder pin concept for the EU-DEMO HCPB breeding blanket

The fusion program in the Karlsruhe Institute of Technology (KIT) leads the R&D of the DEMO helium-cooled pebble bed (HCPB) breeding blanket within the work package breeding blanket (WPBB) of the Eurofusion Consortium in the European Union (EU). A new design of the HCPB breeder zone, with a layout inspired by a nuclear reactor fuel rod arrangement, was developed recently and called the fuel-breeder pin concept. In addition, a mock-up (MU) of this fuel-breeder pin was designed and manufactured at KIT in order to test and validate its thermal–hydraulic performance. This paper reports on the results of the first experimental campaign dedicated to the fuel-breeder pin MU testing that was performed in the Helium Loop Karlsruhe (HELOKA) facility. The paper presents: (i) the integration of the fuel-breeder pin MU into the HELOKA loop including considerations of the experimental set-up, (ii) an overview of the plan for the experimental campaigns, and (iii) a discussion of the experimental results with a focus on aspects relevant for the validation of the thermal–hydraulic design of the HCPB breeder zone

Engineering Design of the European DEMO HCPB Breeding Blanket Breeder Zone Mockup

The Helium Cooled Pebble Bed (HCPB) breeding blanket is one of the two driver-blanket candidates for the European fusion demonstration power plant (EU DEMO) within the framework of the EUROfusion Consortium. As the EU DEMO program is going, testing of mockups becomes increasingly important. In this article, the engineering design of a first-ever breeder zone mockup of the EU DEMO HCPB breeding blanket is reported. The mockup will be tested in the high-pressure, high temperature, helium facility (HELOKA) at Karlsruhe Institute of Technology. This mockup will act as a test rig to validate heat transfer correlations, CFD software, and thermal hydraulics systems codes. As pressure equipment, the mockup shall conform to the latest European Union Pressure Equipment Directive 2014/68/EU. The design description, rationale and test matrix, and corresponding analyses are discussed and presented

Therapy-refractory Panton Valentine Leukocidin-positive community-acquired methicillin-sensitive Staphylococcus aureus sepsis with progressive metastatic soft tissue infection: a case report

We report a case of fulminant multiple organ failure including the Acute Respiratory Distress Syndrome (ARDS), haemodynamic, and renal failure due to community-acquired methicillin-sensitive Panton Valentine Leukocidin (PVL) positive spa-type 284 (ST121) Staphylococcus aureus septic shock. The patient's first clinical symptom was necrotizing pneumonia. Despite organism-sensitive triple antibiotic therapy with linezolid, imipenem and clindamycin from the first day of treatment, progressive abscess formation in multiple skeletal muscles was observed. As a result, repeated surgical interventions became necessary. Due to progressive soft tissue infection, the anti-microbial therapy was changed to a combination of clindamycin and daptomycin. Continued surgical and antimicrobial therapy finally led to a stabilisation of the patients' condition. The clinical course of our patient underlines the existence of a "PVL-syndrome" which is independent of in vitro Staphylococcus aureus susceptibility. The PVL-syndrome should not only be considered in patients with soft tissue or bone infection, but also in patients with pneumonia. Such a condition, which may easily be mistaken for uncomplicated pneumonia, should be treated early, aggressively and over a long period of time in order to avoid relapsing infection

Mutation Analysis of NR5A1 Encoding Steroidogenic Factor 1 in 77 Patients with 46, XY Disorders of Sex Development (DSD) Including Hypospadias

BACKGROUND: Mutations of the NR5A1 gene encoding steroidogenic factor-1 have been reported in association with a wide spectrum of 46,XY DSD (Disorder of Sex Development) phenotypes including severe forms of hypospadias. METHODOLOGY/PRINCIPAL FINDINGS: We evaluated the frequency of NR5A1 gene mutations in a large series of patients presenting with 46,XY DSD and hypospadias. Based on their clinical presentation 77 patients were classified either as complete or partial gonadal dysgenesis (uterus seen at genitography and/or surgery, n = 11), ambiguous external genitalia without uterus (n = 33) or hypospadias (n = 33). We identified heterozygous NR5A1 mutations in 4 cases of ambiguous external genitalia without uterus (12.1%; p.Trp279Arg, pArg39Pro, c.390delG, c140_141insCACG) and a de novo missense mutation in one case with distal hypospadias (3%; p.Arg313Cys). Mutant proteins showed reduced transactivation activity and mutants p.Arg39Pro and p.Arg313Cys did not synergize with the GATA4 cofactor to stimulate reporter gene activity, although they retained their ability to physically interact with the GATA4 protein. CONCLUSIONS/SIGNIFICANCE: Mutations in NR5A1 were observed in 5/77 (6.5%) cases of 46,XY DSD including hypospadias. Excluding the cases of 46,XY gonadal dysgenesis the incidence of NR5A1 mutations was 5/66 (7.6%). An individual with isolated distal hypopadias carried a de novo heterozygous missense mutation, thus extending the range of phenotypes associated with NR5A1 mutations and suggesting that this group of patients should be screened for NR5A1 mutations