Critical Current Studies on Deformed Nb-Ti Strands

The Nb-Ti hard conductors used in LHC dipole and quadrupole magnets are Rutherford cables composed of several tens of strands. During the cabling process, the strands are severely compacted especially at the thin edge of the cable. In order to assess, on the whole wire length, the deformation effect on the transport current of the wires, LHC-type Nb-Ti superconducting strands of various types were flattened by means of rollers. The critical current was then measured as a function of deformation and applied magnetic field at both 4.3 K and 1.9 K. The measurements were performed for both orientations (flat face perpendicular or parallel to magnetic field). The critical current density anisotropy of such deformed strands and the correlation with magnetization effects are discussed. This study permits to better understand and to quantify the critical current degradation of few percent observed in strands due to cabling. Comparisons with wires extracted from Rutherford cables are presented

Critical Current Density in Superconducting Nb-Ti Strands in the 100 mT to 11 T Applied Field Range

The knowledge of the critical current density in a wide temperature and applied magnetic field range is a crucial issue for the design of a superconducting magnet, especially for determining both current and temperature margins. The critical current density of LHC-type Nb-Ti strands of 0.82 and 0.48 mm diameter was measured by means of critical current and magnetization measurements at both 4.2 K and 1.9 K and for a broad magnetic field range (up to 11 T). For the magnetic field range common to both measurement methods, critical current density values as extracted from transport current and from magnetization data are compared and found fairly consistent. Our experimental data are compared to other sets from literature and to scaling laws as well

Finite Element Model to Study the Deformations of Nb3_{3}Sn Wires for the Next European Dipole (NED)

The Next European Dipole (NED) activity is aimed at the development of a large-aperture, high-field superconducting magnet relying on high-performances Nb$_{3}$Sn conductors. Part of the NED program is devoted to the mechanical study of a new generation of Nb$_{3}$Sn wires and to predict and describe their behavior under the severe loading conditions of the cabling process. The deformation resulting from the cabling process was simulated through mechanical analyses by Finite Elements (FE). The ensuing results of FE analyses are presented, allowing the wire behavior under simple uni-axial loads to be described. They are compared to cross section micrographs of deformed wires

Finite Element Model to Study the Deformations of Nb3Sn{\rm Nb}_{3}{\rm Sn} Wires for the Next European Dipole (NED)

The next European dipole (NED) activity is aimed at the development of a large-aperture, high-field superconducting magnet relying on high-performances Nb3Sn conductors. Part of the NED program is devoted to the mechanical study of a new generation of Nb3Sn wires and to predict and describe their behavior under the severe loading conditions of the cabling process. The deformation resulting from the cabling process was simulated through mechanical analyses by finite elements (FE). The ensuing results of FE analyses are presented, allowing the wire behavior under simple uni-axial loads to be described. They are compared to cross section micrographs of deformed wires

Status of the LHC Superconducting Cable Mass Production

Six contracts have been placed with industrial companies for the production of 1200 tons of the superconducting (SC) cables needed for the main dipoles and quadrupoles of the Large Hadron Collider (LHC). In addition, two contracts have been placed for the supply of 470 tons of NbTi and 26 tons of Nb sheets. The main characteristic of the specification is that it is functional. This means that the physical, mechanical and electrical properties of strands and cables are specified without defining the manufacturing processes. Facilities for the high precision measurements of the wire and cable properties have been implemented at CERN, such as strand and cable critical current, copper to superconductor ratio, interstrand resistance, magnetisation, RRR at 4.2 K and 1.9 K. The production has started showing that the highly demanding specifications can be fulfilled. This paper reviews the organisation of the contracts, the test facilities installed at CERN, the various types of measurements and the results of the main physical properties obtained on the first batches. The status of the deliveries is presented

Space-time evolution of electron cascades in diamond

Here we describe model calculations to follow the spatio-temporal evolution of secondary electron cascades in diamond. The band structure of the insulator has been explicitly incorporated into the calculations as it affects ionizations from the valence band. A Monte-Carlo model was constructed to describe the path of electrons following the impact of a single electron of energy E 250 eV. The results show the evolution of the secondary electron cascades in terms of the number of electrons liberated, the spatial distribution of these electrons, and the energy distribution among the electrons as a function of time. The predicted ionization rates (5-13 electrons in 100 fs) lie within the limits given by experiments and phenomenological models. Calculation of the local electron density and the corresponding Debye length shows that the latter is systematically larger than the radius of the electron cloud. This means that the electron gas generated does not represent a plasma in a single impact cascade triggered by an electron of E 250 eV energy. This is important as it justifies the independent-electron approximation used in the model. At 1 fs, the (average) spatial distribution of secondary electrons is anisotropic with the electron cloud elongated in the direction of the primary impact. The maximal radius of the cascade is about 50 A at this time. As the system cools, energy is distributed more equally, and the spatial distribution of the electron cloud becomes isotropic. At 90 fs maximal radius is about 150 A. The Monte-Carlo model described here could be adopted for the investigation of radiation damage in other insulators and has implications for planned experiments with intense femtosecond X-ray sources.Comment: 26 pages, latex, 13 figure

Genetic analysis of patients with Fuchs endothelial corneal dystrophy in India

<p>Abstract</p> <p>Background</p> <p>Mutations in <it>COL8A2 </it>gene which encodes the collagen alpha-2 (VIII) chain have been identified in both familial and sporadic cases of Fuchs endothelial corneal dystrophy (FECD). Heterozygous mutations in the <it>SLC4A11 </it>gene are also known to cause late-onset FECD. Therefore we screened for <it>COL8A2</it>, <it>SLC4A11 </it>gene variants in Indian FECD patients.</p> <p>Methods</p> <p>Eighty patients with clinically diagnosed FECD and 100 age matched normal individuals were recruited. Genomic DNA was isolated from peripheral blood leukocytes. Mutations in <it>COL8A2</it>, <it>SLC4A11 </it>coding regions were screened using bi-directional sequencing. Fischer's exact test or Pearson's chi squared test were used to predict the statistical association of genotypes with the phenotype.</p> <p>Results</p> <p>Screening of <it>COL8A2 </it>gene revealed 2 novel c.1610G>A, c.1643A>G and 3 reported variations c.112G>A, c.464G>A and c.1485G>A. In <it>SLC4A11 </it>gene, novel c.1659C>T, c.1974C>T and reported c.405G>A, c.481A>C and c.639G>A variants were identified. However all the variations in both the genes were also present in unaffected controls.</p> <p>Conclusions</p> <p>This is the first study analysing <it>COL8A2 </it>gene in Indian patients with FECD. No pathogenic mutations were identified in <it>COL8A2</it>. Merely silent changes, which showed statistically insignificant association with FECD, were identified in the screening of <it>SLC4A11 </it>gene. These results suggest that <it>COL8A2</it>, <it>SLC4A11 </it>genes may not be responsible for FECD in patients examined in this study.</p

Clinical and immunologic phenotype associated with activated phosphoinositide 3-kinase δ syndrome 2: A cohort study

Background Activated phosphoinositide 3-kinase δ syndrome (APDS) 2 (p110δ-activating mutations causing senescent T cells, lymphadenopathy, and immunodeficiency [PASLI]–R1), a recently described primary immunodeficiency, results from autosomal dominant mutations in PIK3R1, the gene encoding the regulatory subunit (p85α, p55α, and p50α) of class IA phosphoinositide 3-kinases. Objectives We sought to review the clinical, immunologic, and histopathologic phenotypes of APDS2 in a genetically defined international patient cohort. Methods The medical and biological records of 36 patients with genetically diagnosed APDS2 were collected and reviewed. Results Mutations within splice acceptor and donor sites of exon 11 of the PIK3R1 gene lead to APDS2. Recurrent upper respiratory tract infections (100%), pneumonitis (71%), and chronic lymphoproliferation (89%, including adenopathy [75%], splenomegaly [43%], and upper respiratory tract lymphoid hyperplasia [48%]) were the most common features. Growth retardation was frequently noticed (45%). Other complications were mild neurodevelopmental delay (31%); malignant diseases (28%), most of them being B-cell lymphomas; autoimmunity (17%); bronchiectasis (18%); and chronic diarrhea (24%). Decreased serum IgA and IgG levels (87%), increased IgM levels (58%), B-cell lymphopenia (88%) associated with an increased frequency of transitional B cells (93%), and decreased numbers of naive CD4 and naive CD8 cells but increased numbers of CD8 effector/memory T cells were predominant immunologic features. The majority of patients (89%) received immunoglobulin replacement; 3 patients were treated with rituximab, and 6 were treated with rapamycin initiated after diagnosis of APDS2. Five patients died from APDS2-related complications. Conclusion APDS2 is a combined immunodeficiency with a variable clinical phenotype. Complications are frequent, such as severe bacterial and viral infections, lymphoproliferation, and lymphoma similar to APDS1/PASLI-CD. Immunoglobulin replacement therapy, rapamycin, and, likely in the near future, selective phosphoinositide 3-kinase δ inhibitors are possible treatment options

Hematopoietic Cell Transplantation Cures Adenosine Deaminase 2 Deficiency: Report on 30 Patients.

Deficiency of adenosine deaminase 2 (DADA2) is an inherited inborn error of immunity, characterized by autoinflammation (recurrent fever), vasculopathy (livedo racemosa, polyarteritis nodosa, lacunar ischemic strokes, and intracranial hemorrhages), immunodeficiency, lymphoproliferation, immune cytopenias, and bone marrow failure (BMF). Tumor necrosis factor (TNF-α) blockade is the treatment of choice for the vasculopathy, but often fails to reverse refractory cytopenia. We aimed to study the outcome of hematopoietic cell transplantation (HCT) in patients with DADA2. We conducted a retrospective study on the outcome of HCT in patients with DADA2. The primary outcome was overall survival (OS). Thirty DADA2 patients from 12 countries received a total of 38 HCTs. The indications for HCT were BMF, immune cytopenia, malignancy, or immunodeficiency. Median age at HCT was 9 years (range: 2-28 years). The conditioning regimens for the final transplants were myeloablative (n = 20), reduced intensity (n = 8), or non-myeloablative (n = 2). Donors were HLA-matched related (n = 4), HLA-matched unrelated (n = 16), HLA-haploidentical (n = 2), or HLA-mismatched unrelated (n = 8). After a median follow-up of 2 years (range: 0.5-16 years), 2-year OS was 97%, and 2-year GvHD-free relapse-free survival was 73%. The hematological and immunological phenotypes resolved, and there were no new vascular events. Plasma ADA2 enzyme activity normalized in 16/17 patients tested. Six patients required more than one HCT. HCT was an effective treatment for DADA2, successfully reversing the refractory cytopenia, as well as the vasculopathy and immunodeficiency. HCT is a definitive cure for DADA2 with &gt; 95% survival