Recent materials compatibility studies in refractory metal-alkali metal systems for space power applications.

Science.gov (United States)

Harrison, R. W.; Hoffman, E. E.; Davies, R. L.

1972-01-01

Advanced Rankine and other proposed space power systems utilize refractory metals in contact with both single-phase and two-phase alkali metals at elevated temperatures. A number of recent compatibility experiments are described which emphasize the excellent compatibility of refractory metals with the alkali metals, lithium, sodium, and potassium, under a variety of environmental conditions. The alkali metal compatibilities of tantalum-, columbium-, molybdenum-, and tungsten-base alloys are discussed.

Severe neonatal epileptic encephalopathy and KCNQ2 mutation: neuropathological substrate?

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Charlotte eDalen Meurs-Van Der Schoor

2014-12-01

Full Text Available Background:Neonatal convulsions are clinical manifestations in a heterogeneous group of disorders with different etiology and outcome. They are attributed to several genetic causes. Methods:We describe a patient with intractable neonatal seizures who died from respiratory compromise during a status epilepticus. Results:This case report provides EEG, MRI, genetic analysis and neuropathological data. Genetic analysis revealed a de novo heterozygous missense mutation in the KCNQ2 gene, which encodes a subunit of a voltage-gated potassium channel. KCNQ2 gene mutation is associated with intractable neonatal seizures. EEG, MRI data as well as mutation analysis have been described in other KCNQ2 cases. Postmortem neuropathologic investigation revealed mild malformation of cortical development with increased heterotopic neurons in the deep white matter compared to an age-matched control subject. The new finding of this study is the combination of a KCNQ2 mutation and the cortical abnormalities. Conclusions:KCNQ2 mutations should be considered in neonates with refractory epilepsy of unknown cause. The mild cortical malformation is an important new finding, though it remains unknown whether these cortical abnormalities are due to the KCNQ2 mutation or are secondary to the refractory seizures.

Potential refractory alloy requirements for space nuclear power applications

International Nuclear Information System (INIS)

Cooper, R.H. Jr.

1984-01-01

In reviewing design requirements for refractory alloys for space nuclear applications, several key points are identified. First, the successful utilization of refractory alloys is considered an enabling requirement for the successful deployment of high efficiency, lightweight, and small space nuclear systems. Second, the recapture of refractory alloy nuclear technology developed in the 1960s and early 1970s appears to be a pacing activity in the successful utilization of refractory alloys. Third, the successful application of refractory alloys for space nuclear applications will present a significant challenge to both the materials and the systems design communities

Spectrum of mutations in RARS-T patients includes TET2 and ASXL1 mutations

OpenAIRE

Szpurka, Hadrian; Jankowska, Anna M.; Makishima, Hideki; Bodo, Juraj; Bejanyan, Nelli; Hsi, Eric D.; Sekeres, Mikkael A.; Maciejewski, Jaroslaw P.

2010-01-01

While a majority of patients with refractory anemia with ring sideroblasts and thrombocytosis harbor JAK2V617F and rarely MPLW515L, JAK2/MPL-negative cases constitute a diagnostic problem. 23 RARS-T cases were investigated applying immunohistochemical phospho-STAT5, sequencing and SNP-A-based karyotyping. Based on the association of TET2/ASXL1 mutations with MDS/MPN we studied molecular pattern of these genes. Two patients harbored ASXL1 and another 2 TET2 mutations. Phospho-STAT5 activation ...

SINTERED REFRACTORY TUNGSTEN ALLOYS. Gesinterte hochschmelzende wolframlegierungen

Energy Technology Data Exchange (ETDEWEB)

Kieffer, R.; Sedlatschek, K.; Braun, H.

1971-12-15

Dependence of the melting point of the refractory metals on their positions in the periodic system - alloys of tungsten with other refractory metals - sintering of the alloys - processing of the alloys - technological properties.

Spectrum of mutations in RARS-T patients includes TET2 and ASXL1 mutations.

Science.gov (United States)

Szpurka, Hadrian; Jankowska, Anna M; Makishima, Hideki; Bodo, Juraj; Bejanyan, Nelli; Hsi, Eric D; Sekeres, Mikkael A; Maciejewski, Jaroslaw P

2010-08-01

While a majority of patients with refractory anemia with ring sideroblasts and thrombocytosis harbor JAK2V617F and rarely MPLW515L, JAK2/MPL-negative cases constitute a diagnostic problem. 23 RARS-T cases were investigated applying immunohistochemical phospho-STAT5, sequencing and SNP-A-based karyotyping. Based on the association of TET2/ASXL1 mutations with MDS/MPN we studied molecular pattern of these genes. Two patients harbored ASXL1 and another 2 TET2 mutations. Phospho-STAT5 activation was present in one mutated TET2 and ASXL1 case. JAK2V617F/MPLW515L mutations were absent in TET2/ASXL1 mutants, indicating that similar clinical phenotype can be produced by various MPN-associated mutations and that additional unifying lesions may be present in RARS-T. Copyright (c) 2010 Elsevier Ltd. All rights reserved.

EGFR Mutation Status in Uighur Lung Adenocarcinoma Patients

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Li SHAN

2013-02-01

Full Text Available Background and objective Epidermal growth factor receptor (EGFR, a transmembrane protein, is a member of the tyrosine kinase family. Gefitinib, an EGFR tyrosine-kinase inhibitors, has shown a high response rate in the treatment of lung cancer in patients with EGFR mutation. However, significant differences in EGFR mutations exist among different ethnic groups. The aim of this study is to investigate the prevalence of EGFR mutations in Uighur lung adenocarcinoma patients by using a rapid and sensitive detection method and to analyze EGFR mutation differences compared with Han lung adenocarcinoma patients. Methods We examined lung adenocarcinoma tissues from 138 patients, including 68 Uighur lung adenocarcinoma patients and 70 Han lung adenocarcinoma patients, for EGFR mutations in exons 18, 19, 20, and 21 by using the amplification refractory mutation system (ARMS PCR method. The mutation differences between Uighur and Han lung adenocarcinoma were compared by using the chi-square test method. Results EGFR mutations were detected in 43 (31.2% of the 138 lung adenocarcinoma patients. EGFR mutations were detected in 11 (16.2% of the 68 Uighur lung adenocarcinoma patients and in 32 (45.7% of the 70 Han lung adenocarcinoma patients. Significant differences were observed in the EGFR mutations between Uighur lung adenocarcinoma patients and Han lung adenocarcinoma patients (P<0.001. Conclusion Our results indicate that the EGFR mutation in Uighur lung adenocarcinoma patients (16.2% is significantly lower than that in Han lung adenocarcinoma patients (45.7%.

Response to rituximab in a refractory case of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus

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Niaz Faraz

2010-01-01

Full Text Available Thrombotic thrombocytopenic purpura (TTP is a serious disorder with a significant morbidity and mortality. Majority of cases of TTP are idiopathic, but some cases may be secon-dary to connective tissue diseases. TTP has been rarely associated with systemic lupus erythe-matosus (SLE and may be refractory to treatment with plasma exchange, requiring immuno-suppressive therapy. We describe a patient with TTP and SLE who was refractory to plasma exchange and corticosteroids but responded to anti-CD20 antibody rituximab with continued re-mission after eight months of follow-up. Rituximab appears to be an effective treatment in re-fractory cases of TTP associated with SLE.

Slagging gasifier refractories. A new pathway to longer refractory life

Energy Technology Data Exchange (ETDEWEB)

Schnake, Mark [Harbinson-Walker Refractories Company, Mexico, MO (United States)

2013-07-01

Solid fuel slagging gasification to convert coal or petroleum coke feedstocks into syngas has rapidly evolved over the last 25 years. The gasifier is a high temperature, high pressure reaction chamber. Operating temperatures are between 1250 and 1575 C. Pressures will be between 20.4 and 68 atm. Syngas has been typically used for chemical feedstocks, fuel for power plants, or for steam and hydrogen generation in other industrial applications. Ash which comes from the solid fuel during gasification has many impurities. It melts during the gasifier reactor operation forming a liquid that penetrates the refractory lining. Given time, the refractory will wear away from thermal spalling, structural spalling, or overheating of the refractory. In some cases, all three wear mechanisms are seen in the same gasifier lining. Industry users have identified refractory life as one major limiting factor in worldwide use of this technology. Users have stated if the refractory liner can increase on-line availability of the gasifier operation, more industry acceptance of this technology is possible. Harbison-Walker Refractories Company will review destructive factors affecting lining life and discuss new refractory materials that have dramatically increased gasifier lining life and reliability. New refractory materials will be presented and supported by field trial results and post mortem analysis.

Blocking DNA Repair in Advanced BRCA-Mutated Cancer

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In this trial, patients with relapsed or refractory advanced cancer and confirmed BRCA mutations who have not previously been treated with a PARP inhibitor will be given BMN 673 by mouth once a day in 28-day cycles.

High alumina refractories

International Nuclear Information System (INIS)

Simao, L.C.; Lopes, A.B.; Galvao Filho, N.B.; Souza, R.B. de

1989-01-01

High alumina refractories with 92 to 96.5% Al 2 O 3 were produced using brown and white fused as aggregate. Those refractories present only alumina-α and mullite as crystalline mineralogical phase. Other physical and chemical characteristics are similar to the ones found in refractories produced in Brazil, Japan and U.S.A. The most important physical and chemical tests used for the characterization of the raw materials and refractories, complemented by those realized at high temperatures, plus X-ray Difractometry and optical microscopy are presented, besides the refractory formulation and main parameters of production [pt

Recurrent mutations in the CDKL5 gene: genotype-phenotype relationships.

Science.gov (United States)

Bahi-Buisson, Nadia; Villeneuve, Nathalie; Caietta, Emilie; Jacquette, Aurélia; Maurey, Helene; Matthijs, Gert; Van Esch, Hilde; Delahaye, Andrée; Moncla, Anne; Milh, Mathieu; Zufferey, Flore; Diebold, Bertrand; Bienvenu, Thierry

2012-07-01

Mutations in the cyclin-dependent kinase-like 5 gene (CDKL5) have been described in epileptic encephalopathies in females with infantile spasms with features that overlap with Rett syndrome. With more than 80 reported patients, the phenotype of CDKL5-related encephalopathy is well-defined. The main features consist of seizures starting before 6 months of age, severe intellectual disability with absent speech and hand stereotypies and deceleration of head growth, which resembles Rett syndrome. However, some clinical discrepancies suggested the influence of genetics and/or environmental factors. No genotype-phenotype correlation has been defined and thus there is a need to examine individual mutations. In this study, we analyzed eight recurrent CDKL5 mutations to test whether the clinical phenotype of patients with the same mutation is similar and whether patients with specific CDKL5 mutations have a milder phenotype than those with other CDKL5 mutations. Patients bearing missense mutations in the ATP binding site such as the p.Ala40Val mutation typically walked unaided, had normocephaly, better hand use ability, and less frequent refractory epilepsy when compared to girls with other CDKL5 mutations. In contrast, patients with mutations in the kinase domain (such as p.Arg59X, p.Arg134X, p.Arg178Trp/Pro/Gln, or c.145 + 2T > C) and frameshift mutations in the C-terminal region (such as c.2635_2636delCT) had a more severe phenotype with infantile spasms, refractory epileptic encephalopathy, absolute microcephaly, and inability to walk. It is important for clinicians to have this information when such patients are diagnosed. Copyright © 2012 Wiley Periodicals, Inc.

Development and inter-laboratory validation of unlabeled probe melting curve analysis for detection of JAK2 V617F mutation in polycythemia vera.

Science.gov (United States)

Wu, Zhiyuan; Yuan, Hong; Zhang, Xinju; Liu, Weiwei; Xu, Jinhua; Zhang, Wei; Guan, Ming

2011-01-01

JAK2 V617F, a somatic point mutation that leads to constitutive JAK2 phosphorylation and kinase activation, has been incorporated into the WHO classification and diagnostic criteria of myeloid neoplasms. Although various approaches such as restriction fragment length polymorphism, amplification refractory mutation system and real-time PCR have been developed for its detection, a generic rapid closed-tube method, which can be utilized on routine genetic testing instruments with stability and cost-efficiency, has not been described. Asymmetric PCR for detection of JAK2 V617F with a 3'-blocked unlabeled probe, saturate dye and subsequent melting curve analysis was performed on a Rotor-Gene® Q real-time cycler to establish the methodology. We compared this method to the existing amplification refractory mutation systems and direct sequencing. Hereafter, the broad applicability of this unlabeled probe melting method was also validated on three diverse real-time systems (Roche LightCycler® 480, Applied Biosystems ABI® 7500 and Eppendorf Mastercycler® ep realplex) in two different laboratories. The unlabeled probe melting analysis could genotype JAK2 V617F mutation explicitly with a 3% mutation load detecting sensitivity. At level of 5% mutation load, the intra- and inter-assay CVs of probe-DNA heteroduplex (mutation/wild type) covered 3.14%/3.55% and 1.72%/1.29% respectively. The method could equally discriminate mutant from wild type samples on the other three real-time instruments. With a high detecting sensitivity, unlabeled probe melting curve analysis is more applicable to disclose JAK2 V617F mutation than conventional methodologies. Verified with the favorable inter- and intra-assay reproducibility, unlabeled probe melting analysis provided a generic mutation detecting alternative for real-time instruments.

Development and inter-laboratory validation of unlabeled probe melting curve analysis for detection of JAK2 V617F mutation in polycythemia vera.

Directory of Open Access Journals (Sweden)

Zhiyuan Wu

Full Text Available BACKGROUND: JAK2 V617F, a somatic point mutation that leads to constitutive JAK2 phosphorylation and kinase activation, has been incorporated into the WHO classification and diagnostic criteria of myeloid neoplasms. Although various approaches such as restriction fragment length polymorphism, amplification refractory mutation system and real-time PCR have been developed for its detection, a generic rapid closed-tube method, which can be utilized on routine genetic testing instruments with stability and cost-efficiency, has not been described. METHODOLOGY/PRINCIPAL FINDINGS: Asymmetric PCR for detection of JAK2 V617F with a 3'-blocked unlabeled probe, saturate dye and subsequent melting curve analysis was performed on a Rotor-Gene® Q real-time cycler to establish the methodology. We compared this method to the existing amplification refractory mutation systems and direct sequencing. Hereafter, the broad applicability of this unlabeled probe melting method was also validated on three diverse real-time systems (Roche LightCycler® 480, Applied Biosystems ABI® 7500 and Eppendorf Mastercycler® ep realplex in two different laboratories. The unlabeled probe melting analysis could genotype JAK2 V617F mutation explicitly with a 3% mutation load detecting sensitivity. At level of 5% mutation load, the intra- and inter-assay CVs of probe-DNA heteroduplex (mutation/wild type covered 3.14%/3.55% and 1.72%/1.29% respectively. The method could equally discriminate mutant from wild type samples on the other three real-time instruments. CONCLUSIONS: With a high detecting sensitivity, unlabeled probe melting curve analysis is more applicable to disclose JAK2 V617F mutation than conventional methodologies. Verified with the favorable inter- and intra-assay reproducibility, unlabeled probe melting analysis provided a generic mutation detecting alternative for real-time instruments.

Merits of using andalusite-based refractories compared to bauxite-based refractories

OpenAIRE

Nyoka, M.; Brazier, D.; Courtney, T.; Parry, R.A.

2013-01-01

Historically bauxite-based refractories have been used in applications where andalusite-based refractories could work. Bauxite-based refractories were chosen over andalusite-based refractories mainly because of the availability of low-cost Chinese bauxite and also because many furnaces were designed by international companies that cannot easily access high-quality products. Currently, the availability of low-cost bauxite is under threat as a result of high export duties and tariffs as well as...

Clinical features and course of refractory anemia with ring sideroblasts associated with marked thrombocytosis

Science.gov (United States)

Broseus, Julien; Florensa, Lourdes; Zipperer, Esther; Schnittger, Susanne; Malcovati, Luca; Richebourg, Steven; Lippert, Eric; Cermak, Jaroslav; Evans, Jyoti; Mounier, Morgane; Raya, José Maria; Bailly, François; Gattermann, Norbert; Haferlach, Torsten; Garand, Richard; Allou, Kaoutar; Besses, Carlos; Germing, Ulrich; Haferlach, Claudia; Travaglino, Erica; Luno, Elisa; Pinan, Maria Angeles; Arenillas, Leonor; Rozman, Maria; Perez Sirvent, Maria Luz; Favre, Bernardine; Guy, Julien; Alonso, Esther; Ahwij, Nuhri; Jerez, Andrés; Hermouet, Sylvie; Maynadié, Marc; Cazzola, Mario; Girodon, François

2012-01-01

Background Refractory anemia with ring sideroblasts associated with marked thrombocytosis was proposed as a provisional entity in the 2001 World Health Organization classification of myeloid neoplasms and also in the 2008 version, but its existence as a single entity is contested. We wish to define the clinical features of this rare myelodysplastic/myeloproliferative neoplasm and to compare its clinical outcome with that of refractory anemia with ring sideroblasts and essential thrombocythemia. Design and Methods We conducted a collaborative retrospective study across Europe. Our database included 200 patients diagnosed with refractory anemia with ring sideroblasts and marked thrombocytosis. For each of these patients, each patient diagnosed with refractory anemia with ring sideroblasts was matched for age and sex. At the same time, a cohort of 454 patients with essential thrombocythemia was used to compare outcomes of the two diseases. Results In patients with refractory anemia with ring sideroblasts and marked thrombocytosis, depending on the Janus Kinase 2 V617F mutational status (positive or negative) or platelet threshold (over or below 600×109/L), no difference in survival was noted. However, these patients had shorter overall survival and leukemia-free survival with a lower risk of thrombotic complications than did patients with essential thrombocythemia (P<0.001) but better survival (P<0.001) and a higher risk of thrombosis (P=0.039) than patients with refractory anemia with ring sideroblasts. Conclusions The clinical course of refractory anemia with ring sideroblasts and marked thrombocytosis is better than that of refractory anemia with ring sideroblasts and worse than that of essential thrombocythemia. The higher risk of thrombotic events in this disorder suggests that anti-platelet therapy might be considered in this subset of patients. From a clinical point of view, it appears to be important to consider refractory anemia with ring sideroblasts and

The Association of Factor V Leiden Mutation with Recurrent Pregnancy Loss

International Nuclear Information System (INIS)

Kashif, M.; Saeed, A.

2015-01-01

Objective: To determine the association of factor V Leiden mutation with recurrent pregnancy loss. Methods: The case-control study was conducted at the Department of Haematology, Armed Forces Institute of Pathology, Rawalpindi, Pakistan, from January to June 2012, and comprised women of 18 to 45 years of age who had a history of recurrent pregnancy loss, and controls with no history of pregnancy loss. All the subjects belonged to Punjabi ethnic group. Three ml blood was taken from cases and controls and deoxyribonucleic acid was extracted. In order to identify Factor V Leiden mutation, polymerase chain reaction method was utilised combined with the amplification refractory mutation system. Data was analysed using SPSS 17. Results: Of the 112 subjects, 56(50 percent) were in each of the two groups. The presence of factor V Leiden mutation among the cases was 3(5.4 percent) while it was absent among the controls. The mutation was significantly associated with recurrent pregnancy loss (p=0.017).Recurrent pregnancy loss was higher in cases than controls (p=0.001). Conclusion: Factor V Leiden mutation was significantly associated with recurrent pregnancy loss. It should be considered one of the causes of recurrent pregnancy loss. (author)

Development of a cost-efficient novel method for rapid, concurrent genotyping of five common single nucleotide polymorphisms of the brain derived neurotrophic factor (BDNF) gene by tetra-primer amplification refractory mutation system.

Science.gov (United States)

Wang, Cathy K; Xu, Michael S; Ross, Colin J; Lo, Ryan; Procyshyn, Ric M; Vila-Rodriguez, Fidel; White, Randall F; Honer, William G; Barr, Alasdair M

2015-09-01

Brain derived neurotrophic factor (BDNF) is a molecular trophic factor that plays a key role in neuronal survival and plasticity. Single nucleotide polymorphisms (SNPs) of the BDNF gene have been associated with specific phenotypic traits in a large number of neuropsychiatric disorders and the response to psychotherapeutic medications in patient populations. Nevertheless, due to study differences and occasionally contrasting findings, substantial further research is required to understand in better detail the association between specific BDNF SNPs and these psychiatric disorders. While considerable progress has been made recently in developing advanced genotyping platforms of SNPs, many high-throughput probe- or array-based detection methods currently available are limited by high costs, slow processing times or access to advanced instrumentation. The polymerase chain reaction (PCR)-based, tetra-primer amplification refractory mutation system (T-ARMS) method is a potential alternative technique for detecting SNP genotypes efficiently, quickly, easily, and cheaply. As a tool in psychopathology research, T-ARMS was shown to be capable of detecting five common SNPs in the BDNF gene (rs6265, rs988748, rs11030104, 11757G/C and rs7103411), which are all SNPs with previously demonstrated clinical relevance to schizophrenia and depression. The present technique therefore represents a suitable protocol for many research laboratories to study the genetic correlates of BDNF in psychiatric disorders. Copyright Copyright © 2015 John Wiley & Sons, Ltd. Copyright © 2015 John Wiley & Sons, Ltd.

Refractory Materials for Flame Deflector Protection System Corrosion Control: Coatings Systems Literature Survey

Science.gov (United States)

Calle, Luz M.; Hintze, Paul E.; Parlier, Christopher R.; Sampson, Jeffrey W.; Coffman, Brekke E.; Coffman, Brekke E.; Curran, Jerome P.; Kolody, Mark R.; Whitten, Mary; Perisich, Steven;

Refractory vasculitis

NARCIS (Netherlands)

Rutgers, Bram; Kallenberg, Cees G. M.

Refractory vasculitis occurs in 4-5% of patients with anti-neutrophil cytoplasmic antibody associated vasculitis (AAV). Differences between therapies used for refractory disease are mostly reflected in the percentages of complete and partial remissions, but also in the number of serious side

A new Leukemia Prognostic Scoring System for refractory/relapsed adult acute myelogeneous leukaemia patients: a GOELAMS study.

Science.gov (United States)

Chevallier, P; Labopin, M; Turlure, P; Prebet, T; Pigneux, A; Hunault, M; Filanovsky, K; Cornillet-Lefebvre, P; Luquet, I; Lode, L; Richebourg, S; Blanchet, O; Gachard, N; Vey, N; Ifrah, N; Milpied, N; Harousseau, J-L; Bene, M-C; Mohty, M; Delaunay, J

2011-06-01

A simplified prognostic score is presented based on the multivariate analysis of 138 refractory/relapsed acute myeloid leukaemia (AML) patients (median age 55 years, range: 19-70) receiving a combination of intensive chemotherapy+Gemtuzumab as salvage regimen. Overall, 2-year event-free survival (EFS) and overall survival (OS) were 29±4% and 36±4%, respectively. Disease status (relapse Leukemia Prognostic Scoring System was then validated on an independent cohort of 111 refractory/relapsed AML patients. This new simplified prognostic score, using three clinical and biological parameters routinely applied, allow to discriminate around two third of the patients who should benefit from a salvage intensive regimen in the setting of refractory/relapsed AML patients. The other one third of the patients should receive investigational therapy.

Trans-jugular intrahepatic porto-systemic shunt placement for refractory ascites: a 'real-world' UK health economic evaluation.

Science.gov (United States)

Parker, Matthew J; Guha, Neil; Stedman, Brian; Hacking, Nigel; Wright, Mark

2013-07-01

To assess the benefit of trans-jugular intrahepatic porto-systemic shunt (TIPS) placement for refractory ascites. A retrospective observational study of all patients undergoing TIPS for refractory ascites in our hospital between 2003 and 2012. Secondary care. Cirrhotic patients with refractory ascites. We examined direct real-world (National Health Service) health related costs in the year before and after the TIPS procedure took place. Data were collected relating to the need for reintervention and hepatic encephalopathy. Data were available for 24 patients who underwent TIPS for refractory ascites (86% of eligible patients). TIPS was technically successful in all cases. Mean number of bed days in the year prior to TIPS was 30.3 and 14.3 in the year following (p=0.005). No patient had ascites at the end of the year after the TIPS with less requirement for paracentesis over the course of the year (p<0.001). Mean reduction in cost was £2759 per patient. TIPS was especially cost-effective in patients requiring between 6 and 12 drains per year with a mean saving of £9204 per patient. TIPS is both a clinically effective and economically advantageous therapeutic option for selected patients with refractory ascites.

Dramatic effect of levetiracetam in early-onset epileptic encephalopathy due to STXBP1 mutation.

Science.gov (United States)

Dilena, Robertino; Striano, Pasquale; Traverso, Monica; Viri, Maurizio; Cristofori, Gloria; Tadini, Laura; Barbieri, Sergio; Romeo, Antonino; Zara, Federico

2016-01-01

Syntaxin Binding Protein 1 (STXBP1) mutations determine a central neurotransmission dysfunction through impairment of the synaptic vesicle release, thus causing a spectrum of phenotypes varying from syndromic and non-syndromic epilepsy to intellectual disability of variable degree. Among the antiepileptic drugs, levetiracetam has a unique mechanism of action binding SV2A, a glycoprotein of the synaptic vesicle release machinery. We report a 1-month-old boy manifesting an epileptic encephalopathy with clonic seizures refractory to phenobarbital, pyridoxine and phenytoin that presented a dramatic response to levetiracetam with full epilepsy control and EEG normalization. Genetic analysis identified a novel de novo heterozygous mutation (c.[922A>T]p.[Lys308(∗)]) in the STXBP1 gene that severely affects the protein. The observation of a dramatic efficacy of levetiracetam in a case of STXBP1 epileptic encephalopathy refractory to other antiepileptic drugs and considerations regarding the specific mechanism of action of levetiracetam modulating the same system affected by STXBP1 mutations support the hypothesis that this drug may be able to reverse specifically the disease epileptogenic abnormalities. Further clinical observations and laboratory studies are needed to confirm this hypothesis and eventually lead to consider levetiracetam as the first choice treatment of patients with suspected or confirmed STXBP1-related epilepsies. Copyright © 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Refractory alloy technology for space nuclear power applications

International Nuclear Information System (INIS)

Cooper, R.H. Jr.; Hoffman, E.E.

1984-01-01

Purpose of this symposium is twofold: (1) to review and document the status of refractory alloy technology for structural and fuel-cladding applications in space nuclear power systems, and (2) to identify and document the refractory alloy research and development needs for the SP-100 Program in both the short and the long term. In this symposium, an effort was made to recapture the space reactor refractory alloy technology that was cut off in midstream around 1973 when the national space nuclear reactor program began in the early 1960s, was terminated. The six technical areas covered in the program are compatibility, processing and production, welding and component fabrication, mechanical and physical properties, effects of irradiation, and machinability. The refractory alloys considered are niobium, molybdenum, tantalum, and tungsten. Thirteen of the 14 pages have been abstracted separately. The remaining paper summarizes key needs for further R and D on refractory alloys

Pharmacotherapy for Refractory and Super-Refractory Status Epilepticus in Adults.

Science.gov (United States)

Holtkamp, Martin

2018-03-01

Patients with prolonged seizures that do not respond to intravenous benzodiazepines and a second-line anticonvulsant suffer from refractory status epilepticus and those with seizures that do not respond to continuous intravenous anesthetic anticonvulsants suffer from super-refractory status epilepticus. Both conditions are associated with significant morbidity and mortality. A strict pharmacological treatment regimen is urgently required, but the level of evidence for the available drugs is very low. Refractory complex focal status epilepticus generally does not require anesthetics, but all intravenous non-anesthetizing anticonvulsants may be used. Most descriptive data are available for levetiracetam, phenytoin and valproate. Refractory generalized convulsive status epilepticus is a life-threatening emergency, and long-term clinical consequences are eminent. Administration of intravenous anesthetics is mandatory, and drugs acting at the inhibitory gamma-aminobutyric acid (GABA) A receptor such as midazolam, propofol and thiopental/pentobarbital are recommended without preference for one of those. One in five patients with anesthetic treatment does not respond and has super-refractory status epilepticus. With sustained seizure activity, excitatory N-methyl-d-aspartate (NMDA) receptors are increasingly expressed post-synaptically. Ketamine is an antagonist at this receptor and may prove efficient in some patients at later stages. Neurosteroids such as allopregnanolone increase sensitivity at GABA A receptors; a Phase 1/2 trial demonstrated safety and tolerability, but randomized controlled data failed to demonstrate efficacy. Adjunct ketogenic diet may contribute to termination of difficult-to-treat status epilepticus. Randomized controlled trials are needed to increase evidence for treatment of refractory and super-refractory status epilepticus, but there are multiple obstacles for realization. Hitherto, prospective multicenter registries for pharmacological

The three stages of epilepsy in patients with CDKL5 mutations.

Science.gov (United States)

Bahi-Buisson, Nadia; Kaminska, Anna; Boddaert, Nathalie; Rio, Marlène; Afenjar, Alexandra; Gérard, Marion; Giuliano, Fabienne; Motte, Jacques; Héron, Delphine; Morel, Marie Ange N'guyen; Plouin, Perrine; Richelme, Christian; des Portes, Vincent; Dulac, Olivier; Philippe, Christophe; Chiron, Catherine; Nabbout, Rima; Bienvenu, Thierry

2008-06-01

Mutations in the X-linked cyclin-dependent kinase-like 5 (CDKL5) gene are responsible for a severe encephalopathy with early epilepsy. So far, the electroclinical phenotype remains largely unknown and no clear genotype-phenotype correlations have been established. To characterize the epilepsy associated with CDKL5 mutations and to look for a relationship between the genotype and the course of epilepsy. We retrospectively analyzed the electroclinical phenotypes of 12 patients aged from 2.5 to 19 years diagnosed with pathogenic CDKL5 mutations and one patient with a novel intronic sequence variation of uncertain pathogenicity and examined whether the severity of the epilepsy was linked to the type and location of mutations. The epilepsy course reveals three successive stages: (Stage I) early epilepsy (onset 1-10 weeks) with normal interictal electroencephalogram (EEG) (10/13) despite frequent convulsive seizures; (Stage II) epileptic encephalopathy with infantile spasms (8/8) and hypsarrhythmia (8/8). At the age of evaluation, seven patients were seizure free and six had developed refractory epilepsy (stage III) with tonic seizures and myoclonia (5/6). Interestingly, the patients carrying a CDKL5 mutations causing a truncation of the catalytic domain tended to develop a more frequent refractory epilepsy than patients with mutations located downstream (4/6, 66.6% versus 1/6, 16%) although, these trends are not yet significant. Our data contribute to a better definition of the epileptic phenotype in CDKL5 mutations, and might give some clues to a potential relationship between the phenotype and the genotype in these patients.

Refractory metal alloys and composites for space power systems

International Nuclear Information System (INIS)

Stephens, J.R.; Petrasek, D.W.; Titran, R.H.

1994-01-01

Space power requirements for future NASA and other United States missions will range from a few kilowatts to megawatts of electricity. Maximum efficiency is a key goal of any power system in order to minimize weight and size so that the space shuttle may be used a minimum number of times to put the power supply into orbit. Nuclear power has been identified as the primary power source to meet these high levels of electrical demand. One method to achieve maximum efficiency is to operate the power supply, energy conversion system, and related components at relatively high temperatures. NASA Lewis Research Center has undertaken a research program on advanced technology of refractory metal alloys and composites that will provide base line information for space power systems in the 1900's and the 21st century. Basic research on the tensile and creep properties of fibers, matrices, and composites will be discussed

RAPID DETECTION OF -THALASSEMIA MUTATIONS IN THAILAND USING MULTIPLEX ARMS

Directory of Open Access Journals (Sweden)

D. Shimbhu

2017-11-01

Full Text Available The number of mutations underlining b-thalassemia generate a wide variety of different clinical phenotypes. An understanding of the genotype is important for medical personnel in order to provide proper counseling to patients and their families. Characterization of these mutations should aid the planning of a prenatal diagnosis program for bthalassemia. The heterogeneity of the mutations makes it difficult and time consuming to identify the mutation in some individuals. We developed a single-tube multiplex amplification refractory mutation system (multiplex ARMS to identify common ethnic- specific b-thalassemia mutations. Confirmation of multiplex ARMS results was carried out using direct sequencing. Three thousand three hundred twenty two people from Phitsanulok province were screened for the b-thalassemia trait by quantitation of HbA2 with microcolumn chromatography and the genotypes of mutations were characterized using multiplex ARMS and direct sequencing. We found that the deletion at codons 41/42 (-TCTT was the most frequent (48%, codon 17 (A®T (30%, -28 (A®G (6% and IVS-I-1(G®T (6% were the second and third in frequency respectively. A -87 (C®A mutation (4%, IVS II-654 (C®T (2%, codons 71/72 (+A (2% and codon 35 (C®A mutations (2% were also found. These techniques were found to be a valuable tool for analysis of b-thalassemia mutations because they are accurate, simple, and speedy in operation. The application for the diagnosis of severe thalassemia in high-risk pregnancies is promising.

Refractory alloy technology for space nuclear power applications

Energy Technology Data Exchange (ETDEWEB)

Cooper, R.H. Jr.; Hoffman, E.E. (eds.)

1984-01-01

Purpose of this symposium is twofold: (1) to review and document the status of refractory alloy technology for structural and fuel-cladding applications in space nuclear power systems, and (2) to identify and document the refractory alloy research and development needs for the SP-100 Program in both the short and the long term. In this symposium, an effort was made to recapture the space reactor refractory alloy technology that was cut off in midstream around 1973 when the national space nuclear reactor program began in the early 1960s, was terminated. The six technical areas covered in the program are compatibility, processing and production, welding and component fabrication, mechanical and physical properties, effects of irradiation, and machinability. The refractory alloys considered are niobium, molybdenum, tantalum, and tungsten. Thirteen of the 14 pages have been abstracted separately. The remaining paper summarizes key needs for further R and D on refractory alloys. (DLC)

Autoradiographic study of corrosion of refractories

International Nuclear Information System (INIS)

Lisenenkova, S.B.; Kucheryavyi, M.N.; Bursteva, T.A.

1988-01-01

A comparative study was made of the character of the interaction between a container-glass melt consisting of sodium calcium silicate and refractories in various furnace sections using an autoradiographic method. Static tests were conducted on specimens of the following refractories: chrome-aluminum-zircon, Bakor 41, corundum, a high alumina refractory, and a refractory based on tin dioxide. The specimens were activated by calcium 45. Autoradiography and photomicrography indicated that an intrinsic feature of all refractories was that calcium from the melt penetrated the refractories along the weak link; for fused-cast refractories, the glass phase; and for sintered refractories, through the binder and cracks

Response to parenteral iron therapy distinguish unexplained refractory iron deficiency anemia from iron-refractory iron deficiency anemia.

Science.gov (United States)

Akin, M; Sarbay, H; Guler, S; Balci, Y I; Polat, A

2016-04-01

We evaluated that response to parenteral iron therapy could be helpful in distinguishing the types of iron deficiency anemia. This study analyzed responses to IV iron sucrose therapy of 15 children with unexplained refractory iron deficiency anemia (URIDA). We compared the results at diagnosis, 6 weeks and 6 months after the therapy. Results were compared with responses of 11 patients' results with iron-refractory iron deficiency anemia (IRIDA) from our previous study. Six weeks after the start of treatment, ferritin, MCV, MCH and Hb values were in normal range in 10 patients. The increase in Hb, MCH, MCV, and ferritin values ranged 2.6-3.5 g/dL, 1.7-4.2 pg, 2-9 fL, and 13-25 ng/mL, respectively. In five patients, Hb, MCH, and MCV mean (range) values [11.2 g/dL (11-12.2), 24.5 pg (24-25.6), and 67 fL (65-70)] were nearly normal but ferritin mean (range) values [9.8 ng/mL (8-11)] were below normal. Six weeks after the start of treatment, Hb, MCH, MCV and ferritin values of patients with IRIDA were increased. The increase in Hb, MCH, MCV, and ferritin values ranged 0.8-2.7 g/dL, 1.7-4.2 pg, 2-9 fL, and 13-25 ng/mL, respectively. IRIDA is only partially responsive to parenteral iron supplementation. In conclusion, this study demonstrated that the response to intravenous iron therapy for the URIDA cases improved blood parameters more effectively than hereditary IRIDA. Response to parenteral iron therapy would be helpful to distinguish unexplained refractory IDA from hereditary IRIDA for clinicians who do not have access to hepcidin or TMPRS6 mutation analysis. © 2016 John Wiley & Sons Ltd.

Refractory Research Group - U.S. DOE, Albany Research Center [Institution Profile

Energy Technology Data Exchange (ETDEWEB)

Bennett, James P.

2004-09-01

The refractory research group at the Albany Research Center (ARC) has a long history of conducting materials research within the U.S. Bureau of Mines, and more recently, within the U.S. Dept. of Energy. When under the U.S. Bureau of Mines, research was driven by national needs to develop substitute materials and to conserve raw materials. This mission was accomplished by improving refractory material properties and/or by recycling refractories using critical and strategic materials. Currently, as a U.S. Dept of Energy Fossil Energy field site, research is driven primarily by the need to assist DOE in meeting its vision to develop economically and environmentally viable technologies for the production of electricity from fossil fuels. Research at ARC impacts this vision by: • Providing information on the performance characteristics of materials being specified for the current generation of power systems; • Developing cost-effective, high performance materials for inclusion in the next generation of fossil power systems; and • Solving environmental emission and waste problems related to fossil energy systems. A brief history of past refractory research within the U.S. Bureau of Mines, the current refractory research at ARC, and the equipment and capabilities used to conduct refractory research at ARC will be discussed.

Trans-jugular intrahepatic porto-systemic shunt placement for refractory ascites: a ‘real-world’ UK health economic evaluation

Science.gov (United States)

Parker, Matthew J; Guha, Neil; Stedman, Brian; Hacking, Nigel; Wright, Mark

2013-01-01

Objective To assess the benefit of trans-jugular intrahepatic porto-systemic shunt (TIPS) placement for refractory ascites. Design A retrospective observational study of all patients undergoing TIPS for refractory ascites in our hospital between 2003 and 2012. Setting Secondary care. Patients Cirrhotic patients with refractory ascites. Main outcome measures We examined direct real-world (National Health Service) health related costs in the year before and after the TIPS procedure took place. Data were collected relating to the need for reintervention and hepatic encephalopathy. Results Data were available for 24 patients who underwent TIPS for refractory ascites (86% of eligible patients). TIPS was technically successful in all cases. Mean number of bed days in the year prior to TIPS was 30.3 and 14.3 in the year following (p=0.005). No patient had ascites at the end of the year after the TIPS with less requirement for paracentesis over the course of the year (p<0.001). Mean reduction in cost was £2759 per patient. TIPS was especially cost-effective in patients requiring between 6 and 12 drains per year with a mean saving of £9204 per patient. Conclusions TIPS is both a clinically effective and economically advantageous therapeutic option for selected patients with refractory ascites. PMID:28839725

Stochastic histories of refractory interstellar dust

International Nuclear Information System (INIS)

Liffman, K.; Chayton, D.D.

1988-01-01

The authors calculate histories for refractory dust particles in the interstellar medium. The double purposes are to learn something of the properties of interstellar dust as a system and to evaluate with specific assumptions the cosmic chemical memory interpretation of a specific class of isotopic anomalies. They assemble the profile of a particle population from a large number of stochastic, or Monte Carlo, histories of single particles, which are necessarily taken to be independent with this approach. They specify probabilities for each of the events that may befall a given particle and unfold its history by a sequence of random numbers. They assume that refractory particles are created only by thermal condensation within stellar material during its ejection from stars, and that these refractory particles can be destroyed only by being sputtered to a size too small for stability or by being incorporated into the formation of new stars. In order to record chemical detail, the authors take each new refractory particle to consist of a superrefractory core plus a more massive refractory mantle. They demonstrate that these superrefractory cores have effective lifetimes much longer than the turnover time of dust mass against sputtering. As examples of cosmic chemical memory they evaluate the 16 O-richness of interstellar aluminum and mechanisms for the 48 Ca/ 50 Ti correlation. Several related consequences of this approach are discussed

In vivo somatic mutation systems in the mouse

International Nuclear Information System (INIS)

Russell, L.B.

1979-01-01

In an effort to meet the need for a fast and cheap in vivo prescreen for inherited mammalian point mutations, a somatic forward-mutation method, originally developed in an x-ray experiment, has more recently been tested in work with chemical mutagens. The method makes use of coat-color mutations because the gene product is usually locally expressed, mosaics can be detected with minimal effort, and opportunities for making comparison with induction of germinal point mutations are greatest.--Following treatment of embryos that are heterozygous at specific coat-color loci, various induced genetic changes can result in expression of the recessive (RS) in clones derived from mutant melanocyte precursor cells. However, other events, such as decrease in the number of precursor cells, or disturbed differentiation, can also result in spots, which with careful classification can usually be distinguished from RS's on the basis of their location and color. When this is done, the relative RS frequencies for a series of compounds at least roughly parallel the relative spermatogonial mutation rates. The fact that easily measurable (though low) RS rates are obtained with compounds that have yielded negative results in spermatogonial tests is not surprising in view of the fact that RS's can be caused by several mechanisms besides point mutation.--In spite of the parallelism observed in one laboratory, the usefulness of the in vivo somatic mutation method as a prescreen could come to be doubted because of major discrepancies between results of similar experiments at different laboratories. However, It appears probable that at least some of these discrepancies are due to failure to discriminate between spots that probably resulted from melanocyte insufficiency and spots that resulted from expression of the recessive.--Reverse somatic mutation systems can potentially avoid some of the pitfalls of forward mutation systems. Such system are still in developmental stages

NOvel Refractory Materials for High Alkali, High Temperature Environments

Energy Technology Data Exchange (ETDEWEB)

Hemrick, J.G.; Griffin, R. (MINTEQ International, Inc.)

2011-08-30

Refractory materials can be limited in their application by many factors including chemical reactions between the service environment and the refractory material, mechanical degradation of the refractory material by the service environment, temperature limitations on the use of a particular refractory material, and the inability to install or repair the refractory material in a cost effective manner or while the vessel was in service. The objective of this project was to address the need for new innovative refractory compositions by developing a family of novel MgO-Al2O3 spinel or other similar magnesia/alumina containing unshaped refractory composition (castables, gunnables, shotcretes, etc) utilizing new aggregate materials, bond systems, protective coatings, and phase formation techniques (in-situ phase formation, altered conversion temperatures, accelerated reactions, etc). This family of refractory compositions would then be tailored for use in high-temperature, highalkaline industrial environments like those found in the aluminum, chemical, forest products, glass, and steel industries. A research team was formed to carry out the proposed work led by Oak Ridge National Laboratory (ORNL) and was comprised of the academic institution Missouri University of Science and Technology (MS&T), and the industrial company MINTEQ International, Inc. (MINTEQ), along with representatives from the aluminum, chemical, glass, and forest products industries. The two goals of this project were to produce novel refractory compositions which will allow for improved energy efficiency and to develop new refractory application techniques which would improve the speed of installation. Also methods of hot installation were sought which would allow for hot repairs and on-line maintenance leading to reduced process downtimes and eliminating the need to cool and reheat process vessels.

Development of electro fused aggregates for use in refractories for the burning zone of cement kilns

International Nuclear Information System (INIS)

Ferreira, Luis Leonardo Horne Curimbaba

2006-01-01

Electro fused aggregates are largely used in refractory production due to the better performance reached when they are employed. In this work electro fused aggregates were designed for application in refractories for the burning zone of cement kilns. Initially reaction evaluation was conducted aiming the identification of the most prone refractory systems when single refractory phases react with Portland cement phases at high temperatures. In the next step, raw materials of the best refractory systems were electro fused to generate different aggregate compositions. The electro fused aggregates properties were evaluated and the classified ones were used to produce refractory bricks for the burning zone of cement kilns. General characteristics of these bricks were measured and compared with a standard magnesia-spinel refractory. Aggregates of the system Mg O - TiO 2 - Ca O, more specifically aggregates belonged to the compatibility triangle Mg O - Mg 2 TiO 4 - CaTiO 3 , showed suitable characteristics for development of refractories for the burning zone cement kilns. (author)

Refractory alloy component fabrication

International Nuclear Information System (INIS)

Young, W.R.

1984-01-01

Purpose of this report is to describe joining procedures, primarily welding techniques, which were developed to construct reliable refractory alloy components and systems for advanced space power systems. Two systems, the Nb-1Zr Brayton Cycle Heat Receiver and the T-111 Alloy Potassium Boiler Development Program, are used to illustrate typical systems and components. Particular emphasis is given to specific problems which were eliminated during the development efforts. Finally, some thoughts on application of more recent joining technology are presented. 78 figures

21 CFR 864.7280 - Factor V Leiden DNA mutation detection systems.

Science.gov (United States)

2010-04-01

... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Factor V Leiden DNA mutation detection systems....7280 Factor V Leiden DNA mutation detection systems. (a) Identification. Factor V Leiden deoxyribonucleic acid (DNA) mutation detection systems are devices that consist of different reagents and...

Refractory metal based superalloys

International Nuclear Information System (INIS)

Alonso, Paula R.; Vicente, Eduardo E.; Rubiolo, Gerardo H.

1999-01-01

Refractory metals are looked as promising materials for primary circuits in fission reactors and even as fusion reactor components. Indeed, superalloys could be developed which take advantage of their high temperature properties together with the benefits of a two- phase (intermetallic compound-refractory metal matrix) coherent structure. In 1993, researchers of the Office National d'Etudes et de Recherches Aerospatiales of France reported the observation of such a coherent structure in the Ta-Ti-Zr-Al-Nb-Mo system although the exact composition is not reported. The intermetallic compound would be Ti 2 AlMo based. However, the formation of this compound and its possible coexistence with a disordered bcc phase in the ternary system Ti-Al-Mo is a controversial subject in the related literature. In this work we develop a technique to obtain homogeneous alloys samples with 50 Ti-25 Al-25 Mo composition. The resulting specimens were characterized by optical and electronic metallography (SEM), microprobe composition measurements (EPMA) and X-ray diffraction (XRD) analyses. The results show the evidence for a bcc (A2→B2) ordering reaction in the Ti-Al-Mo system in the 50 Ti-25 Al-25 Mo composition. (author)

Dramatic Response with Single-Agent Ibrutinib in Multiply Relapsed Marginal Zone Lymphoma with MYD88L265P Mutation

Directory of Open Access Journals (Sweden)

Ryan C. Lynch

2017-09-01

Full Text Available The B-cell receptor signaling pathway is important in the lymphomagenesis of many lymphomas, including marginal zone lymphoma (MZL. Herein we describe a case of extranodal MZL refractory to multiple lines of therapy. The presence of an IgM paraprotein prompted further evaluation, and the patient was found to have an MYD88L265P mutation. Treatment with ibrutinib led to a dramatic response with prompt resolution of symptoms and significant improvement in measurable sites of disease. The excellent response to ibrutinib in our patient with MYD88L265P-mutated refractory MZL supports a biological rationale for its use.

A Nonsense Mutation in FAM161A Is a Recurrent Founder Allele in Dutch and Belgian Individuals With Autosomal Recessive Retinitis Pigmentosa

NARCIS (Netherlands)

Van Schil, Kristof; Klevering, B. Jeroen; Leroy, Bart P.; Pott, Jan Willem R.; Bandah-Rozenfeld, Dikla; Zonneveld-Vrieling, Marijke N.; Sharon, Dror; den Hollander, Anneke I.; Cremers, Frans P. M.; De Baere, Elfride; Collin, Rob W. J.; van den Born, L. Ingeborgh

PURPOSE. To identify mutations in FAM161A underlying autosomal recessive retinitis pigmentosa (arRP) in the Dutch and Belgian populations and to investigate whether common FAM161A-associated phenotypic features could be identified. METHODS. Homozygosity mapping, amplification-refractory mutation

Directed light fabrication of refractory metals and alloys

International Nuclear Information System (INIS)

Fonseca, J.C.; Lewis, G.K.; Dickerson, P.G.; Nemec, R.B.

1999-01-01

This report covers deposition of refractory pure metals and alloys using the Directed Light Fabrication (DLF) process and represents progress in depositing these materials through September 1998. In extending the DLF process technology to refractory metals for producing fully dense, structurally sound deposits, several problems have become evident. (1) Control of porosity in DLF-deposited refractory metal is difficult because of gases, apparently present in commercially purchased refractory metal powder starting materials. (2) The radiant heat from the molten pool during deposition melts the DLF powder feed nozzle. (3) The high reflectivity of molten refractory metals, at the Nd-YAG laser wavelength (1.06microm), produces damaging back reflections to the optical train and fiber optic delivery system that can terminate DLF processing. (4) The current limits on the maximum available laser power to prevent back reflection damage limit the parameter range available for densification of refractory metals. The work to date concentrated on niobium, W-25Re, and spherodized tungsten. Niobium samples, made from hydride-dehydride powder, had minimal gas porosity and the deposition parameters were optimized; however, test plates were not made at this time. W-25Re samples, containing sodium and potassium from a precipitation process, were made and porosity was a problem for all samples although minimized with some process parameters. Deposits made from potassium reduced tungsten that was plasma spherodized were made with minimized porosity. Results of this work indicate that further gas analysis of starting powders and de-gassing of starting powders and/or gas removal during deposition of refractory metals is required

Refractoriness in human atria

DEFF Research Database (Denmark)

Skibsbye, Lasse; Jespersen, Thomas; Christ, Torsten

2016-01-01

BACKGROUND: Refractoriness of cardiac cells limits maximum frequency of electrical activity and protects the heart from tonic contractions. Short refractory periods support major arrhythmogenic substrates and augmentation of refractoriness is therefore seen as a main mechanism of antiarrhythmic...... drugs. Cardiomyocyte excitability depends on availability of sodium channels, which involves both time- and voltage-dependent recovery from inactivation. This study therefore aims to characterise how sodium channel inactivation affects refractoriness in human atria. METHODS AND RESULTS: Steady......-state activation and inactivation parameters of sodium channels measured in vitro in isolated human atrial cardiomyocytes were used to parameterise a mathematical human atrial cell model. Action potential data were acquired from human atrial trabeculae of patients in either sinus rhythm or chronic atrial...

Refractory of Furnaces to Reduce Environmental Impact

International Nuclear Information System (INIS)

Hanzawa, Shigeru

2011-01-01

The energy load of furnaces used in the manufacturing process of ceramics is quite large. Most of the environmental impact of ceramics manufacturing is due to the CO 2 produced from this high energy load. To improve this situation, R and D has focused on furnace systems and techniques of control in order to reduce energy load. Since furnaces are comprised of refractory, consideration of their mechanical and thermal characteristics is important. Herein are described several refractory types which were chosen through comparison of the characteristics which contribute to heat capacity reduction, heat insulating reinforcement and high emissivity, thereby improving thermal radiation heat transfer efficiency to the ceramic articles. One selected refractory material which will reduce the environmental impact of a furnace, chosen considering low heat capacity and high emissivity characteristics, is SiC. In this study, thermal radiation heat transfer efficiency improvement and its effect on ceramic articles in the furnace and oxidation behaviour were investigated at 1700K. A high density SiC refractory, built into the furnace at construction, has relatively high oxidation durability and has the ability to reduce environmental impact-CO 2 by 10 percent by decreasing the furnace's energy load. However, new oxidation prevention techniques for SiC will be necessary for long-term use in industrial furnaces, because passive to active oxidation transition behaviour of commercial SiC refractory is coming to close ideal.

Refractory of Furnaces to Reduce Environmental Impact

Science.gov (United States)

Hanzawa, Shigeru

2011-10-01

The energy load of furnaces used in the manufacturing process of ceramics is quite large. Most of the environmental impact of ceramics manufacturing is due to the CO2 produced from this high energy load. To improve this situation, R&D has focused on furnace systems and techniques of control in order to reduce energy load. Since furnaces are comprised of refractory, consideration of their mechanical and thermal characteristics is important. Herein are described several refractory types which were chosen through comparison of the characteristics which contribute to heat capacity reduction, heat insulating reinforcement and high emissivity, thereby improving thermal radiation heat transfer efficiency to the ceramic articles. One selected refractory material which will reduce the environmental impact of a furnace, chosen considering low heat capacity and high emissivity characteristics, is SiC. In this study, thermal radiation heat transfer efficiency improvement and its effect on ceramic articles in the furnace and oxidation behaviour were investigated at 1700K. A high density SiC refractory, built into the furnace at construction, has relatively high oxidation durability and has the ability to reduce environmental impact-CO2 by 10 percent by decreasing the furnace's energy load. However, new oxidation prevention techniques for SiC will be necessary for long-term use in industrial furnaces, because passive to active oxidation transition behaviour of commercial SiC refractory is coming to close ideal.

Improved cooler design of electric arc furnace refractory in mining industry using thermal analysis modeling and simulation

International Nuclear Information System (INIS)

Istadi, I.; Bindar, Y.

2014-01-01

Production of steel and nickel using the electric arc furnace should be focused on the intensification of energy. Improvement of energy efficiency of the most consuming facilities was achieved by improving the use of alternative energy minimization such as reducing the heat lost of hot gases, minimizing the heat radiated through refractory linings of metallurgical furnaces, and cooling the highly thermally stressed components. The refractory of electric arc furnace should be modified to achieve the best cooling system of the furnace. In this physical modeling and simulation works, four modification scenarios of wall refractory designs were simulated, i.e. refractory with basic design, refractory with deep plate coolers, refractory with extra plate coolers, and refractory with wall falling film coolers. Finally, the use of deep plate cooler and the existing waffle cooler system was considered to be the best design of efficient electric arc furnace operationally. - Highlights: • Electric arc furnace design should be focused on the intensification of energy. • Refractory of electric arc furnace were modified to achieve the best cooling system. • Four modification scenarios of the wall refractory designs were simulated. • Use of deep plate cooler and existing waffle cooler system is the best cooling

Low frequency of c-MPL gene mutations in Iranian patients with Philadelphia-negative myeloproliferative disorders.

Science.gov (United States)

Ghotaslou, A; Nadali, F; Chahardouli, B; Alizad Ghandforosh, N; Rostami, S H; Alimoghaddam, K; Ghavamzadeh, A

2015-01-01

Myeloproliferative disorders are a group of diseases characterized by increased proliferation of myeloid lineage. In addition to JAK2V617F mutation, several mutations in the c-MPL gene have been reported in patients with philadelphia-negative chronic myeloproliferative disorders that could be important in the pathogenesis of diseases. The aim of the present study was to investigate the frequency of c-MPL and JAK2V617F mutations in Iranian patients with Philadelphia-negativemyeloproliferative disorders. Peripheral blood samples were collected from 60 patients with Philadelphia-negative MPD) Subgroups ET and PMF) and 25 healthy subjects as control group. The mutation status of c-MPL and Jak2V617F were investigated by using Amplification-refractory mutation system (ARMS) and Allele-Specific PCR (AS-PCR), respectively. The results were confirmed by sequencing. Among 60 patients, 34 (56.6%) and 1(1.7%) had Jak2V617F and c-MPL mutation, respectively. Patients with Jak2V617F mutation had higher WBC counts and hemoglobin concentration than those without the mutation (p= 0.005, p=0.003). In addition, for all healthy subjects in control group, mutations were negative. The present study revealed that the c-MPL mutations unlike the Jak2V617F mutations are rare in Iranian patients with Ph-negative MPNs and the low mutation rate should be considered in the design of screening strategies of MPD patients.

The association of JAK2V617F mutation and leukocytosis with thrombotic events in essential thrombocythemia.

Science.gov (United States)

Hsiao, Hui-Hua; Yang, Ming-Yu; Liu, Yi-Chang; Lee, Ching-Ping; Yang, Wen-Chi; Liu, Ta-Chih; Chang, Chao-Sung; Lin, Sheng-Fung

2007-11-01

The Janus kinase 2 mutation, JAK2 (V617F), and megakaryocytic mutations, MPL (W515L/K), have been identified and correlated with a subtype of essential thrombocythemia (ET) patients. We investigated the frequency of mutations in ET patients and analyzed the relationship with their clinical features. Fifty-three ET patients were enrolled in the study. The amplification refractory mutation system was applied for the mutation survey of the JAK2V617F, while the polymerase chain reaction with sequencing was used for the mutation survey of MPLW515L/K. Thirty-five (66%) patients harboring the JAK2 (V617F) mutation, including 3 homozygous and 32 heterozygous changes, but no MPLW515L/K mutation, were found. During follow-up, 17 (32.1%) patients suffered from documented thrombotic events, with 15 having JAK2V617F mutations. Statistical analysis showed that patients with the JAK2 mutation had significantly higher leukocytes, hemoglobin level, and thrombotic event (p = 0.043, p = 0.001, and p = 0.029, respectively). Thrombotic events were also significantly correlated with leukocytosis and older age. The JAK2V617F mutation was noted in a certain population of ET patients and correlated with leukocytosis, high hemoglobin level, and thrombosis. Therefore, detection of the JAK2V617F mutation can affect not only the diagnosis, but also the management of ET patients.

Plasma spraying of refractory metals and refractory hard materials. State of the art

International Nuclear Information System (INIS)

Eschnauer, H.; Lugscheider, E.; Jaeger, D.

1989-01-01

Suitable spraying processes for manufacturing refractory metals, refractory hard materials as well as spray materials with refractory components are the VPS- and IPS-spraying techniques. The advantages of these special spraying process variations are described. The reactive spraying materials are systematically organized. The characteristical properties used in purpose of improving the substrate surfaces are explained. Finally some examples of the latest results of research concerning plasma spraying of reactive materials are shown. 16 refs., 10 figs. (Author)

Surgical disconnection of patent paraumbilical vein in refractory hepatic encephalopathy.

Science.gov (United States)

Ishikawa, Yoshinori; Yoshida, Hiroshi; Mamada, Yasuhiro; Taniai, Nobuhiko; Bando, Koichi; Mizuguchi, Yoshiaki; Kakinuma, Daisuke; Kanda, Tomohiro; Akimaru, Koho; Tajiri, Takashi

2008-06-01

Refractory hepatic encephalopathy (HE) frequently develops in patients with cirrhosis and portal-systemic shunt. Recently, patients with refractory HE associated with portal-systemic shunt have been treated with interventional radiology. We describe a promising new treatment for portal-systemic shunt, ligation of the patent paraumbilical vein (PUV) after partial splenic embolization, in patients with refractory HE. Four patients with cirrhosis (3 women and 1 man; mean age, 56 years) and refractory HE due to a patent PUV were studied. Patency of the PUV had recurred in 1 patient after primary occlusion by interventional radiological procedures. The Child-Pugh class was B in 2 patients and C in 2. Before the present treatment, all patients had been hospitalized at least 3 times because of recurrent HE. Partial splenic embolization was performed in all patients to decrease portal venous pressure before surgery. Surgical ligation of the patent PUV was performed under epidural anesthesia. The patent PUV was carefully skeletonized and doubly ligated. Esophageal varices were evaluated with upper gastrointestinal endoscopy before and after surgery. The mean follow-up duration was 15.8 months. After ligation, there were no clinically significant complications. Esophageal varices were unchanged. The serum ammonia level was higher before surgery (162.3 +/- 56.4 mug/dL, mean +/- SD) than after surgery (41.8 +/- 20.2 mug/dL; p=0.0299). No patient had symptoms of HE. Ligation of the patent PUV is an effective treatment for patients with refractory HE.

Directed light fabrication of refractory metals and alloys

International Nuclear Information System (INIS)

Fonseca, J.C.; Lewis, G.K.; Dickerson, P.G.; Nemec, R.B.

1999-01-01

This report covers work performed under Order No. FA0000020 AN Contract DE-AC12-76SN00052 for deposition of refractory pure metals and alloys using the Directed Light Fabrication (DLF) process and represents the progress in depositing these materials through September 1998. In extending the DLF process technology to refractory metals for producing fully dense, structurally sound deposits, several problems have become evident. 1. Control of porosity in DLF-deposited refractory metal is difficult because of gases, apparently present in commercially purchased refractory metal powder starting materials. 2. The radiant heat from the molten pool during deposition melts the DLF powder feed nozzle. 3. The high reflectivity of molten refractory metals, at the Nd-YAG laser wavelength (1.06microm), produces damaging back reflections to the optical train and fiber optic delivery system that can terminate DLF processing. 4. The current limits on the maximum available laser power to prevent back reflection damage limit the parameter range available for densification of refractory metals. The work to date concentrated on niobium, W-25Re, and spherodized tungsten. Niobium samples, made from hydride-dehydride powder, had minimal gas porosity and the deposition parameters were optimized; however, test plates were not made at this time. W-25Re samples, containing sodium and potassium from a precipitation process, were made and porosity was a problem for all samples although minimized with some process parameters. Deposits made from potassium reduced tungsten that was plasma spherodized were made with minimized porosity. Results of this work indicate that further gas analysis of starting powders and de-gassing of starting powders and/or gas removal during deposition of refractory metals is required

High rate of mutation K103N causing resistance to nevirapine in Indian children with acquired immunodeficiency syndrome

Directory of Open Access Journals (Sweden)

Sehgal S

2008-01-01

Full Text Available In north India the number of paediatric cases with acquired immunodeficiency syndrome (AIDS is on the rise. Most drug combinations used for treatment of AIDS incorporate nevirapine, resistance to which develops very fast if given singly or because of unplanned interruptions. This paper investigates presence of mutations at codon 103 and codon 215 of the HIV pol gene causing resistance to nevirapine and zidovudine (AZT respectively in 25 children with AIDS. Mutations T215Y and K103N were detected by a nested cum amplification refractory mutation system polymerase chain reaction (ARMS PCR and the results were confirmed by direct sequencing in five randomly selected cases. Nineteen patients had received nevirapine containing regimen and six were drug naive. Mutation K103N was observed in 56% (14/25 of the children while mutation T215Y was found in none. Two of the six drug naοve children also showed K103N mutation. Thus, Indian children drug naοve or treated with nevirapine containing regimens show a high rate of mutation conferring resistance to nevirapine which calls for a judicious use of nevirapine both in antenatal and postnatal setting.

Theoretical and practical aspects about corrosion of refractories used in steel metallurgy: part 3: characterization of commercial refractories

International Nuclear Information System (INIS)

Braganca, S.R.

2012-01-01

In this study, it was reviewed the main aspects found in the literature about refractories corrosion, evaluating the feasibility of certain tests and relating them with experimental results. The physical properties and microstructure of commercial refractories were analyzed, considering the differences between them and the quality implications and probable life of the refractory. Thus, it was studied the various types of refractories used as lining on steel ladle. Magnesia-carbon and doloma-carbon refractories were analyzed, highlighting the differences between them. The examined refractory showed characteristics favoring high resistance to corrosion process, presenting a series of properties to be selected in accordance with industry practice. (author)

Experimental constraints on heating and cooling rates of refractory inclusions in the early solar system

International Nuclear Information System (INIS)

Boynton, W.V.

1987-01-01

The refractory inclusions in carbonaceous chondrites were the subject of considerable interest since their discovery. These inclusions contain minerals that are predicted to be some of the earliest condensates from the solar nebula, and contain a plethora of isotopic anomalies of unknown origin. Of particular interest are those coarse-grained inclusions that contain refractory metal particles (Fe, Ni, Pt, Ru, Os Ir). Experimental studies of these inclusions in terrestrial laboratories are, however, complicated because the dense particles tend to settle out of a molten or partially molten silicate material. Heating experiments in the Space Station technology and microgravity in order to observe the effects of metal nuggets (which may act as heterogeneous nucleation sites) on nucleation rates in silicate systems and to measure simultaneously the relative volatilization rate of siderophile and lithophile species. Neither experiment is possible in the terrestrial environment

The importance of assessing medication exposure to the definition of refractory disease in systemic lupus erythematosus.

Science.gov (United States)

Arnaud, Laurent; Zahr, Noël; Costedoat-Chalumeau, Nathalie; Amoura, Zahir

2011-09-01

Treatment of patients with Systemic Lupus Erythematosus (SLE) who have active disease refractory to current therapeutic strategies continues to be a real challenge. Here, we propose that the classic definition of refractory SLE patients - failure to achieve adequate response to the standard of care - should be further refined to incorporate the dimension of adequate drug exposure. Inter-individual pharmacokinetic variability may induce insufficient exposure to many drugs used in SLE, leading to both apparent inefficacy of treatments and inappropriate therapeutic escalation. Among others, we have shown that individual assessment of exposure to mycophenolic acid, the active metabolite of mycophenolate mofetil (MMF) could be used to determine whether a given patient received adequate doses of MMF. We have also shown that measuring blood concentrations of hydroxychloroquine could be used as an efficient way to assess observance, which is a critical issue since a significant proportion of refractory SLE patients is likely to have poor observance as the primary source of treatment failure. Finally, we have underlined the importance of assessing drug interactions as SLE patients often require, in addition to immunosuppressants, several other drugs to prevent or treat associated conditions, which may result in decreased exposure to immunosuppressants. Considering these data, we believe that refractory SLE patients should not only be defined as the failure to achieve adequate therapeutic response to the standard of care, but should also incorporate the dimension of inadequate pharmacokinetic exposure and include drug blood level, interaction and observance monitoring. Copyright © 2011. Published by Elsevier B.V.

Development of diagnosis and repair system for steelmaking refractories; Seikoyo taikabutsu no shindan hoshu gijutsu no kakuritsu

Energy Technology Data Exchange (ETDEWEB)

Aso, S.; Harada, S.; Tsutsui, Y. [Nippon Steel Corp., Tokyo (Japan)

1996-06-01

The latest measurement equipment and repair method were introduced into the work area of all refractories in the process of torpedo cars, hot metal preliminary treatment, hot metal pans, secondary refining, and cast pans so as to improve the cost of the newest advanced steelmaking refractories. As a result, the refractory cost could be reduced by 10%. This paper introduces the improvement in RH refractories and refractories for hot metal preliminary treatment. As for basic items, in addition to the visual observation, the thickness of refractories was quantified using equipment (laser profile meter, thermotracer, and NS sensor) for the furnace stop in the minimum remaining length. An ITV was also installed in the facilities where the visual check is difficult to carry out. Repair is mainly done by spraying. A thermal spraying repair method with high durability was used for the repair. A method that relieves a thermal shock was used for preheating. Moreover, measures that make the temperature gradient in the thickness direction uniform by using newly developed microwave drying equipment were taken for drying of unburned refractories. 4 refs., 13 figs., 2 tabs.

Geographical distribution of β-globin gene mutations in Syria.

Science.gov (United States)

Murad, Hossam; Moasses, Faten; Dabboul, Amir; Mukhalalaty, Yasser; Bakoor, Ahmad Omar; Al-Achkar, Walid; Jarjour, Rami A

2018-04-11

Objectives β-Thalassemia disease is caused by mutations in the β-globin gene. This is considered as one of the common genetic disorders in Syria. The aim of this study was to identify the geographical distribution of the β-thalassemia mutations in Syria. Methods β-Globin gene mutations were characterized in 636 affected patients and 94 unrelated carriers using the amplification refractory mutations system-polymerase chain reaction technique and DNA sequencing. Results The study has revealed the presence of 38 β-globin gene mutations responsible for β-thalassemia in Syria. Important differences in regional distribution were observed. IVS-I.110 [G > A] (22.2%), IVS-I.1 [G > A] (17.8%), Cd 39 [C > T] (8.2%), IVS-II.1 [G > A] (7.6%), IVS-I.6 [T > C] (7.1%), Cd 8 [-AA] (6%), Cd 5 [-CT] (5.6%) and IVS-I.5 [G > C] (4.1%) were the eight predominant mutations found in our study. The coastal region had higher relative frequencies (37.9 and 22%) than other regions. A clear drift in the distribution of the third common Cd 39 [C > T] mutation in the northeast region (34.8%) to the northwest region (2.5%) was noted, while the IVS-I.5 [G > C] mutation has the highest prevalence in north regions. The IVS-I.6 [T > C] mutation had a distinct frequency in the middle region. Ten mutations -86 [C > G], -31 [A > G], -29 [A > G], 5'UTR; +22 [G > A], CAP + 1 [A > C], Codon 5/6 [-TG], IVS-I (-3) or codon 29 [C > T], IVS-I.2 [T > A], IVS-I.128 [T > G] and IVS-II.705 [T > G] were found in Syria for the first time. Conclusions These data will significantly facilitate the population screening, genetic counseling and prenatal diagnosis in Syrian population.

21 CFR 866.5900 - Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation detection system.

Science.gov (United States)

2010-04-01

... regulator (CFTR) gene mutation detection system. 866.5900 Section 866.5900 Food and Drugs FOOD AND DRUG...) gene mutation detection system. (a) Identification. The CFTR gene mutation detection system is a device... Guidance Document: CFTR Gene Mutation Detection System.” See § 866.1(e) for the availability of this...

Mutations in SYNGAP1 Cause Intellectual Disability, Autism, and a Specific Form of Epilepsy by Inducing Haploinsufficiency

DEFF Research Database (Denmark)

Berryer, Martin H; Hamdan, Fadi F; Klitten, Laura L

2013-01-01

De novo mutations in SYNGAP1, which codes for a RAS/RAP GTP-activating protein, cause nonsyndromic intellectual disability (NSID). All disease-causing point mutations identified until now in SYNGAP1 are truncating, raising the possibility of an association between this type of mutations and NSID...... also showed ataxia, autism, and a specific form of generalized epilepsy that can be refractory to treatment. All of these mutations occurred de novo, except c.283dupC, which was inherited from a father who is a mosaic. Biolistic transfection of wild-type SYNGAP1 in pyramidal cells from cortical...

Evaluation of the reusing of MGO-C refractory brick in refractory mixes

International Nuclear Information System (INIS)

Silva, R.D.S. da; Braganca, S.R.

2012-01-01

The residue from the use of MgO-C refractories in electric arc furnace presents, mostly, magnesium oxide in its composition and some slag contamination and impurities from the process of electrofusion scrap iron/steel. In this study, it was studied the characteristics of this residue and reused through its introduction into a commercial refractory mix, employed as material of coating and repair. This refractory mix was tested by thermogravimetric analysis, compressive strength, evaluation of plasticity and porosity as well as aspects of its installation, such as adhesion in situ. Results showed that there potential for reuse with the introduction of the waste in commercial mix was 30%, with little loss of compressive strength and plasticity. (author)

Refractory Coated/Lined Low Density Structures, Phase II

Data.gov (United States)

National Aeronautics and Space Administration — This project addresses the development of refractory coated or lined low density structures applicable for advanced future propulsion system technologies. The...

Nanostructured refractory thin films for solar applications

Science.gov (United States)

Ollier, E.; Dunoyer, N.; Dellea, O.; Szambolics, H.

2014-08-01

Selective solar absorbers are key elements of all solar thermal systems. Solar thermal panels and Concentrated Solar Power (CSP) systems aim respectively at producing heat and electricity. In both cases, a surface receives the solar radiation and is designed to have the highest optical absorption (lowest optical reflectivity) of the solar radiation in the visible wavelength range where the solar intensity is the highest. It also has a low emissivity in the infrared (IR) range in order to avoid radiative thermal losses. Current solutions in the state of the art usually consist in deposited interferential thin films or in cermets [1]. Structured surfaces have been proposed and have been simulated because they are supposed to be more efficient when the solar radiation is not normal to the receiving surface and because they could potentially be fabricated with refractory materials able to sustain high operating temperatures. This work presents a new method to fabricate micro/nanostructured surfaces on molybdenum (refractory metal with a melting temperature of 2623°C). This method now allows obtaining a refractory selective surface with an excellent optical selectivity and a very high absorption in the visible range. This high absorption performance was obtained by achieving a double structuration at micro and nano scales thanks to an innovative process flow.

Ketogenic Diet in Super-Refractory Status Epilepticus

OpenAIRE

Smith, Garnett; Press, Craig A.

2017-01-01

Researchers from the Children’s National Health System in Washington, D.C. studied the feasibility, rate of complications, and effect on seizures of initiating the Ketogenic Diet (KD) in pediatric patients with Super-Refractory Status Epilepticus (SRSE).

Compatibility of refractory materials with boiling sodium

International Nuclear Information System (INIS)

Meacham, S.A.

1976-01-01

The program employed to determine the compatibility of commercially available refractories with boiling sodium is described. The effects of impurities contained within the refractory material, and their relations with the refractory's physical stability are discussed. Also, since consideration of refractories for use as an insulating material within Liquid Metal Fast Breeder Reactor Plants (LMFBR's) is currently under investigation; recommendations, based upon this program, are presented

REFRACTORY THROMBOCYTOPENIA AND NEUTROPENIA: A DIAGNOSTIC CHALLENGE

OpenAIRE

Emmanuel Gyan; François Dreyfus; Pierre Fenaux

2015-01-01

Background. The 2008 WHO classification identified refractory cytopenia with unilineage dysplasia (RCUD) as a composite entity encompassing refractory anemia, refractory thrombocytopenia (RT), and refractory neutropenia (RN), characterized by 10% or more dysplastic cells in the bone marrow respective lineage. The diagnosis of RT and RN is complicated by several factors.  Diagnosing RT first requires exclusion of familial thrombocytopenia, chronic auto-immune thrombocytopenia, concomitant medi...

Refractory bin for burning

Energy Technology Data Exchange (ETDEWEB)

McPherson, D.L.; McPherson, T.L.

1989-12-26

This patent describes a refractory bin. It has a generally rectangular horizontal cross sectional configuration. It has wall structures each comprising an upper and a lower pair of elongated horizontal vertically spaced generally parallel support beams each having a vertical flange defining a support edge along its upper surface, a first generally rectangular refractory panel arranged with its lower edge at the bottom of the bin and with its outer surface in flat face contacting relation with the vertical flanges of the lower pair of support beams, a plurality of brackets each having a horizontal part and a vertical part and being secured to the outer surface of the first refractory panel.

Talimogene Laherparepvec and Nivolumab in Treating Patients With Refractory Lymphomas or Advanced or Refractory Non-melanoma Skin Cancers

Science.gov (United States)

2018-05-21

Adenoid Cystic Carcinoma; Adnexal Carcinoma; Apocrine Carcinoma; Eccrine Porocarcinoma; Extraocular Cutaneous Sebaceous Carcinoma; Hidradenocarcinoma; Keratoacanthoma; Malignant Sweat Gland Neoplasm; Merkel Cell Carcinoma; Microcystic Adnexal Carcinoma; NK-Cell Lymphoma, Unclassifiable; Non-Melanomatous Lesion; Paget Disease; Papillary Adenocarcinoma; Primary Cutaneous Mucinous Carcinoma; Refractory Anaplastic Large Cell Lymphoma; Refractory Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma; Refractory Mycosis Fungoides; Refractory Primary Cutaneous T-Cell Non-Hodgkin Lymphoma; Refractory T-Cell Non-Hodgkin Lymphoma; Sezary Syndrome; Signet Ring Cell Carcinoma; Skin Basal Cell Carcinoma; Skin Basosquamous Cell Carcinoma; Skin Squamous Cell Carcinoma; Spiradenocarcinoma; Squamous Cell Carcinoma of Unknown Primary Origin; Stage III Skin Cancer; Stage IV Skin Cancer; Sweat Gland Carcinoma; Trichilemmocarcinoma; Vulvar Squamous Cell Carcinoma

A structural systems biology approach for quantifying the systemic consequences of missense mutations in proteins.

Directory of Open Access Journals (Sweden)

Tammy M K Cheng

Full Text Available Gauging the systemic effects of non-synonymous single nucleotide polymorphisms (nsSNPs is an important topic in the pursuit of personalized medicine. However, it is a non-trivial task to understand how a change at the protein structure level eventually affects a cell's behavior. This is because complex information at both the protein and pathway level has to be integrated. Given that the idea of integrating both protein and pathway dynamics to estimate the systemic impact of missense mutations in proteins remains predominantly unexplored, we investigate the practicality of such an approach by formulating mathematical models and comparing them with experimental data to study missense mutations. We present two case studies: (1 interpreting systemic perturbation for mutations within the cell cycle control mechanisms (G2 to mitosis transition for yeast; (2 phenotypic classification of neuron-related human diseases associated with mutations within the mitogen-activated protein kinase (MAPK pathway. We show that the application of simplified mathematical models is feasible for understanding the effects of small sequence changes on cellular behavior. Furthermore, we show that the systemic impact of missense mutations can be effectively quantified as a combination of protein stability change and pathway perturbation.

Perspectives on environmental protection of refractory metals

International Nuclear Information System (INIS)

Perking, R.A.

1992-01-01

Alloys of refractory metals which combine high strength to weight ratios with useful low-temperature toughness and ductility have been designed to meet the requirements for the next generation of high-temperature aerospace structural materials with one exception: long term resistance to oxidation. It is considered unlikely that refractory metal alloys can be modified to possess useful resistance to oxidation as bulk materials or that coating can be designed to provide high reliability fail-safe protection for the structural alloys developed to date. Recent developments indicate that bulk alloys of W and Mo can be designed to possess short term resistance to oxidation, providing a base material which can be coated to extend useful life with fail-safe protection in the event of random coating failures. Current research on silicide coatings indicates that significant improvements in coating reliability and performance also are feasible. The technical basis for these conclusions is presented in this paper and the direction of future work that could lead to environmentally stable refractory metal alloy/coating systems is discussed

Emergency Surgery for Refractory Status Epilepticus.

Science.gov (United States)

Botre, Abhijeet; Udani, Vrajesh; Desai, Neelu; Jagadish, Spoorthy; Sankhe, Milind

2017-08-15

Management of refractory status epilepticus in children is extremely challenging. Two children with medically refractory status epilepticus, both of whom had lesional pathology on MRI and concordant data on EEG and PET scan. Emergency hemispherotomy performed in both patients. A complete, sustained seizure freedom obtained postoperatively. Emergency surgery is a treatment option in selected cases of drug refractory status epilepticus with lesional pathology and concordant data.

Updates in Refractory Status Epilepticus

Science.gov (United States)

Mahulikar, Advait; Suchdev, Kushak; Shah, Aashit

2018-01-01

Refractory status epilepticus is defined as persistent seizures despite appropriate use of two intravenous medications, one of which is a benzodiazepine. It can be seen in up to 40% of cases of status epilepticus with an acute symptomatic etiology as the most likely cause. New-onset refractory status epilepticus (NORSE) is a recently coined term for refractory status epilepticus where no apparent cause is found after initial testing. A large proportion of NORSE cases are eventually found to have an autoimmune etiology needing immunomodulatory treatment. Management of refractory status epilepticus involves treatment of an underlying etiology in addition to intravenous anesthetics and antiepileptic drugs. Alternative treatment options including diet therapies, electroconvulsive therapy, and surgical resection in case of a focal lesion should be considered. Short-term and long-term outcomes tend to be poor with significant morbidity and mortality with only one-third of patients reaching baseline neurological status. PMID:29854452

Detection of MPLW515L/K mutations and determination of allele frequencies with a single-tube PCR assay.

Science.gov (United States)

Takei, Hiraku; Morishita, Soji; Araki, Marito; Edahiro, Yoko; Sunami, Yoshitaka; Hironaka, Yumi; Noda, Naohiro; Sekiguchi, Yuji; Tsuneda, Satoshi; Ohsaka, Akimichi; Komatsu, Norio

2014-01-01

A gain-of-function mutation in the myeloproliferative leukemia virus (MPL) gene, which encodes the thrombopoietin receptor, has been identified in patients with essential thrombocythemia and primary myelofibrosis, subgroups of classic myeloproliferative neoplasms (MPNs). The presence of MPL gene mutations is a critical diagnostic criterion for these diseases. Here, we developed a rapid, simple, and cost-effective method of detecting two major MPL mutations, MPLW515L/K, in a single PCR assay; we termed this method DARMS (dual amplification refractory mutation system)-PCR. DARMS-PCR is designed to produce three different PCR products corresponding to MPLW515L, MPLW515K, and all MPL alleles. The amplicons are later detected and quantified using a capillary sequencer to determine the relative frequencies of the mutant and wild-type alleles. Applying DARMS-PCR to human specimens, we successfully identified MPL mutations in MPN patients, with the exception of patients bearing mutant allele frequencies below the detection limit (5%) of this method. The MPL mutant allele frequencies determined using DARMS-PCR correlated strongly with the values determined using deep sequencing. Thus, we demonstrated the potential of DARMS-PCR to detect MPL mutations and determine the allele frequencies in a timely and cost-effective manner.

Detection of MPLW515L/K mutations and determination of allele frequencies with a single-tube PCR assay.

Directory of Open Access Journals (Sweden)

Hiraku Takei

Full Text Available A gain-of-function mutation in the myeloproliferative leukemia virus (MPL gene, which encodes the thrombopoietin receptor, has been identified in patients with essential thrombocythemia and primary myelofibrosis, subgroups of classic myeloproliferative neoplasms (MPNs. The presence of MPL gene mutations is a critical diagnostic criterion for these diseases. Here, we developed a rapid, simple, and cost-effective method of detecting two major MPL mutations, MPLW515L/K, in a single PCR assay; we termed this method DARMS (dual amplification refractory mutation system-PCR. DARMS-PCR is designed to produce three different PCR products corresponding to MPLW515L, MPLW515K, and all MPL alleles. The amplicons are later detected and quantified using a capillary sequencer to determine the relative frequencies of the mutant and wild-type alleles. Applying DARMS-PCR to human specimens, we successfully identified MPL mutations in MPN patients, with the exception of patients bearing mutant allele frequencies below the detection limit (5% of this method. The MPL mutant allele frequencies determined using DARMS-PCR correlated strongly with the values determined using deep sequencing. Thus, we demonstrated the potential of DARMS-PCR to detect MPL mutations and determine the allele frequencies in a timely and cost-effective manner.

PCR-based methods for the detection of L1014 kdr mutation in Anopheles culicifacies sensu lato

Science.gov (United States)

Singh, Om P; Bali, Prerna; Hemingway, Janet; Subbarao, Sarala K; Dash, Aditya P; Adak, Tridibes

2009-01-01

Background Anopheles culicifacies s.l., a major malaria vector in India, has developed widespread resistance to DDT and is becoming resistant to pyrethroids–the only insecticide class recommended for the impregnation of bed nets. Knock-down resistance due to a point mutation in the voltage gated sodium channel at L1014 residue (kdr) is a common mechanism of resistance to DDT and pyrethroids. The selection of this resistance may pose a serious threat to the success of the pyrethroid-impregnated bed net programme. This study reports the presence of kdr mutation (L1014F) in a field population of An. culicifacies s.l. and three new PCR-based methods for kdr genotyping. Methods The IIS4-IIS5 linker to IIS6 segments of the para type voltage gated sodium channel gene of DDT and pyrethroid resistant An. culicifacies s.l. population from the Surat district of India was sequenced. This revealed the presence of an A-to-T substitution at position 1014 leading to a leucine-phenylalanine mutation (L1014F) in a few individuals. Three molecular methods viz. Allele Specific PCR (AS-PCR), an Amplification Refractory Mutation System (ARMS) and Primer Introduced Restriction Analysis-PCR (PIRA-PCR) were developed and tested for kdr genotyping. The specificity of the three assays was validated following DNA sequencing of the samples genotyped. Results The genotyping of this An. culicifacies s.l. population by the three PCR based assays provided consistent result and were in agreement with DNA sequencing result. A low frequency of the kdr allele mostly in heterozygous condition was observed in the resistant population. Frequencies of the different genotypes were in Hardy-Weinberg equilibrium. Conclusion The Leu-Phe mutation, which generates the kdr phenotype in many insects, was detected in a pyrethroid and DDT resistant An. culicifacies s.l. population. Three PCR-based methods were developed for kdr genotyping. All the three assays were specific. The ARMS method was refractory to non

PCR-based methods for the detection of L1014 kdr mutation in Anopheles culicifacies sensu lato

Directory of Open Access Journals (Sweden)

Dash Aditya P

2009-07-01

Full Text Available Abstract Background Anopheles culicifacies s.l., a major malaria vector in India, has developed widespread resistance to DDT and is becoming resistant to pyrethroids–the only insecticide class recommended for the impregnation of bed nets. Knock-down resistance due to a point mutation in the voltage gated sodium channel at L1014 residue (kdr is a common mechanism of resistance to DDT and pyrethroids. The selection of this resistance may pose a serious threat to the success of the pyrethroid-impregnated bed net programme. This study reports the presence of kdr mutation (L1014F in a field population of An. culicifacies s.l. and three new PCR-based methods for kdr genotyping. Methods The IIS4-IIS5 linker to IIS6 segments of the para type voltage gated sodium channel gene of DDT and pyrethroid resistant An. culicifacies s.l. population from the Surat district of India was sequenced. This revealed the presence of an A-to-T substitution at position 1014 leading to a leucine-phenylalanine mutation (L1014F in a few individuals. Three molecular methods viz. Allele Specific PCR (AS-PCR, an Amplification Refractory Mutation System (ARMS and Primer Introduced Restriction Analysis-PCR (PIRA-PCR were developed and tested for kdr genotyping. The specificity of the three assays was validated following DNA sequencing of the samples genotyped. Results The genotyping of this An. culicifacies s.l. population by the three PCR based assays provided consistent result and were in agreement with DNA sequencing result. A low frequency of the kdr allele mostly in heterozygous condition was observed in the resistant population. Frequencies of the different genotypes were in Hardy-Weinberg equilibrium. Conclusion The Leu-Phe mutation, which generates the kdr phenotype in many insects, was detected in a pyrethroid and DDT resistant An. culicifacies s.l. population. Three PCR-based methods were developed for kdr genotyping. All the three assays were specific. The ARMS method

ESHAP chemotherapy is efficient in refractory/relapsed primary central nervous system lymphoma: report of four cases

Directory of Open Access Journals (Sweden)

Ungur R

2015-10-01

Full Text Available Rodica Ungur,1,2 Adrian Tempescul,3 Christian Berthou,3 Cristina Bagacean,1,2 Doinel Radeanu,1 Adriana Muresan,1 Mihnea Zdrenghea1,21Department of Hematology, Iuliu Hatieganu University of Medicine and Pharmacy Cluj, 2Department of Hematology, Ion Chiricuta Oncology Institute, Cluj-Napoca, Romania; 3Department of Clinical Hematology, Institute of Cancerology and Hematology, Brest Teaching Hospital, Brest, FranceAbstract: Primary central nervous system non-Hodgkin’s lymphoma is a rare presentation, almost always of diffuse large B-cell type. Although there is no consensus regarding therapy for this condition, induction regimens are based on high-dose methotrexate and consolidation whole-brain radiotherapy, or, more preferred recently, blood–brain barrier penetrating drugs such as etoposide, cytarabine, and alkylating agents like temozolomide, ifosfamide, and lomustine. We present here four cases of relapsed/refractory primary central nervous system lymphoma treated with ESHAP (etoposide, solumedrol, high-dose cytarabine, and platinum chemotherapy to complete remission, with the eligible patients proceeding to autologous transplantation. We want to draw attention to this interesting, relatively well tolerated, underused therapeutic option, in a setting where treatment options are scarce and evidence-based recommendations are lacking.Keywords: cerebral lymphoma, PCNSL, refractory, relapsed, platinum

Tocilizumab for refractory organising pneumonia associated with Sjögren's disease.

Science.gov (United States)

Justet, Aurelien; Ottaviani, Sebastien; Dieudé, Philippe; Taillé, Camille

2015-05-14

Lung involvement in primary Sjögren syndrome occurs in approximately 10-20% of patients. Tocilizumab, an anti-interleukin-6 receptor antibody, has demonstrated efficacy and safety in small series of systemic sclerosis, and systemic lupus erythematosus, but its effect on interstitial lung manifestations of connective tissue diseases is not well known. We report the use of tocilizumab in a refractory organising pneumonia associated with Sjögren's disease. Our observation suggests that tocilizumab could be an alternative therapeutic in refractory organising pneumonia. 2015 BMJ Publishing Group Ltd.

The Geogenomic Mutational Atlas of Pathogens (GoMAP web system.

Directory of Open Access Journals (Sweden)

David P Sargeant

Full Text Available We present a new approach for pathogen surveillance we call Geogenomics. Geogenomics examines the geographic distribution of the genomes of pathogens, with a particular emphasis on those mutations that give rise to drug resistance. We engineered a new web system called Geogenomic Mutational Atlas of Pathogens (GoMAP that enables investigation of the global distribution of individual drug resistance mutations. As a test case we examined mutations associated with HIV resistance to FDA-approved antiretroviral drugs. GoMAP-HIV makes use of existing public drug resistance and HIV protein sequence data to examine the distribution of 872 drug resistance mutations in ∼ 502,000 sequences for many countries in the world. We also implemented a broadened classification scheme for HIV drug resistance mutations. Several patterns for geographic distributions of resistance mutations were identified by visual mining using this web tool. GoMAP-HIV is an open access web application available at http://www.bio-toolkit.com/GoMap/project/

Clinical and microbiological features of refractory periodontitis subjects.

Science.gov (United States)

Colombo, A P; Haffajee, A D; Dewhirst, F E; Paster, B J; Smith, C M; Cugini, M A; Socransky, S S

1998-02-01

The purpose of this investigation was to compare the clinical parameters and the site prevalence and levels of 40 subgingival species in successfully treated and refractory periodontitis subjects. 94 subjects received scaling and root planing and if needed, periodontal surgery and systemically administered tetracycline. 28 refractory subjects showed mean full mouth attachment loss and/or > 3 sites showing attachment loss > 2.5 mm within 1 year post-therapy. 66 successfully treated subjects showed mean attachment level gain and no sites with attachment loss > 2.5 mm. Baseline subgingival plaque samples were taken from the mesial aspect of each tooth and the presence and levels of 40 subgingival taxa were determined using whole genomic DNA probes and checkerboard DNA-DNA hybridization. The mean levels and % of sites colonized by each species (prevalence) was computed for each subject and differences between groups sought using the Mann-Whitney test. Most of the 40 species tested, including Actinobacillus actinomycetemcomitans, Porphyromonas gingivalis, Treponema denticola and Bacteroides forsythus, were equally or less prevalent in the refractory group. Prevotella nigrescens was significantly more prevalent in successfully treated subjects, while refractory subjects harbored a larger proportion of Streptococcus species, particularly Streptococcus constellatus. The odds of a subject being refractory was 8.6 (p or = 3.5% of the total DNA probe count. Since few microbiological differences existed between treatment outcome groups using DNA probes to known species, the predominant cultivable microbiota of 33 subgingival samples from 14 refractory subjects was examined. 85% of the 1649 isolates were identified using probes to 69 recognized subgingival species. The remaining unidentified strains were classified by analyzing 16S rRNA gene sequences. Many sequenced isolates were of taxa not considered a common part of the oral microbiota such as Acinetobacter baumanni

Refractory status epilepticus

Directory of Open Access Journals (Sweden)

Sanjay P Singh

2014-01-01

Full Text Available Refractory status epilepticus is a potentially life-threatening medical emergency. It requires early diagnosis and treatment. There is a lack of consensus upon its semantic definition of whether it is status epilepticus that continues despite treatment with benzodiazepine and one antiepileptic medication (AED, i.e., Lorazepam + phenytoin. Others regard refractory status epilepticus as failure of benzodiazepine and 2 antiepileptic medications, i.e., Lorazepam + phenytoin + phenobarb. Up to 30% patients in SE fail to respond to two antiepileptic drugs (AEDs and 15% continue to have seizure activity despite use of three drugs. Mechanisms that have made the treatment even more challenging are GABA-R that is internalized during status epilepticus and upregulation of multidrug transporter proteins. All patients of refractory status epilepticus require continuous EEG monitoring. There are three main agents used in the treatment of RSE. These include pentobarbital or thiopental, midazolam and propofol. RSE was shown to result in mortality in 35% cases, 39.13% of patients were left with severe neurological deficits, while another 13% had mild neurological deficits.

Refractory status epilepticus

Science.gov (United States)

Singh, Sanjay P; Agarwal, Shubhi; Faulkner, M

2014-01-01

Refractory status epilepticus is a potentially life-threatening medical emergency. It requires early diagnosis and treatment. There is a lack of consensus upon its semantic definition of whether it is status epilepticus that continues despite treatment with benzodiazepine and one antiepileptic medication (AED), i.e., Lorazepam + phenytoin. Others regard refractory status epilepticus as failure of benzodiazepine and 2 antiepileptic medications, i.e., Lorazepam + phenytoin + phenobarb. Up to 30% patients in SE fail to respond to two antiepileptic drugs (AEDs) and 15% continue to have seizure activity despite use of three drugs. Mechanisms that have made the treatment even more challenging are GABA-R that is internalized during status epilepticus and upregulation of multidrug transporter proteins. All patients of refractory status epilepticus require continuous EEG monitoring. There are three main agents used in the treatment of RSE. These include pentobarbital or thiopental, midazolam and propofol. RSE was shown to result in mortality in 35% cases, 39.13% of patients were left with severe neurological deficits, while another 13% had mild neurological deficits. PMID:24791086

Development of refractory concrete for extreme conditions

International Nuclear Information System (INIS)

Pundiene, I; Antonovich, V; Stonys, R; Demidova-Buiziniene, I

2011-01-01

Comparative analysis is provided for the properties of medium-cement refractory concrete with microsilica based on mullite filler in relation to different type of deflocculant. The effect of different deflocculants on refractory concrete structure formation, hydration, rheology, strength and heat resistance is discussed. Corrosion resistance test, determined that samples with hybrid deflocculant showed better resistance for slag penetration than samples with only the sodium tripolyphosphate or polycarboxylate ether deflocculant. Moreover, a composition of hybrid deflocculant let to control the rate of the hydration process and to get features of refractory refractory concrete.

High-Density Infrared Surface Treatments of Refractories

Energy Technology Data Exchange (ETDEWEB)

Tiegs, T.N.

2005-03-31

Refractory materials play a crucial role in all energy-intensive industries and are truly a crosscutting technology for the Industries of the Future (IOF). One of the major mechanisms for the degradation of refractories and a general decrease in their performance has been the penetration and corrosion by molten metals or glass. Methods and materials that would reduce the penetration, wetting, and corrosive chemistry would significantly improve refractory performance and also maintain the quality of the processed liquid, be it metal or glass. This report presents the results of an R&D project aimed at investigating the use of high-density infrared (HDI) heating to surface treat refractories to improve their performance. The project was a joint effort between Oak Ridge National Laboratory (ORNL) and the University of Missouri-Rolla (UMR). HDI is capable of heating the near-surface region of materials to very high temperatures where sintering, diffusion, and melting can occur. The intended benefits of HDI processing of refractories were to (1) reduce surface porosity (by essentially sealing the surface to prevent liquid penetration), (2) allow surface chemistry changes to be performed by bonding an adherent coating onto the underlying refractory (in order to inhibit wetting and/or improve corrosion resistance), and (3) produce noncontact refractories with high-emissivity surface coatings.

Genetics Home Reference: iron-refractory iron deficiency anemia

Science.gov (United States)

... refractory iron deficiency anemia Iron-refractory iron deficiency anemia Printable PDF Open All Close All Enable Javascript ... expand/collapse boxes. Description Iron-refractory iron deficiency anemia is one of many types of anemia , which ...

NOVEL REFRACTORY MATERIALS FOR HIGH ALKALI, HIGH TEMPERATURE ENVIRONMENTS

Energy Technology Data Exchange (ETDEWEB)

Hemrick, James Gordon [ORNL; Smith, Jeffrey D [ORNL; O' Hara, Kelley [University of Missouri, Rolla; Rodrigues-Schroer, Angela [Minteq International, Inc.; Colavito, [Minteq International, Inc.

2012-08-01

A project was led by Oak Ridge National Laboratory (ORNL) in collaboration with a research team comprised of the academic institution Missouri University of Science and Technology (MS&T), and the industrial company MINTEQ International, Inc. (MINTEQ), along with representatives from the aluminum, chemical, glass, and forest products industries. The project was to address the need for new innovative refractory compositions by developing a family of novel MgO-Al 2O3, MgAl2O4, or other similar spinel structured or alumina-based unshaped refractory compositions (castables, gunnables, shotcretes, etc.) utilizing new aggregate materials, bond systems, protective coatings, and phase formation techniques (in-situ phase formation, altered conversion temperatures, accelerated reactions, etc). This family of refractory compositions would then be tailored for use in high-temperature, high-alkaline industrial environments like those found in the aluminum, chemical, forest products, glass, and steel industries. Both practical refractory development experience and computer modeling techniques were used to aid in the design of this new family of materials. The newly developed materials were expected to offer alternative material choices for high-temperature, high-alkali environments that were capable of operating at higher temperatures (goal of increasing operating temperature by 100-200oC depending on process) or for longer periods of time (goal of twice the life span of current materials or next process determined service increment). This would lead to less process down time, greater energy efficiency for associated manufacturing processes (more heat kept in process), and materials that could be installed/repaired in a more efficient manner. The overall project goal was a 5% improvement in energy efficiency (brought about through a 20% improvement in thermal efficiency) resulting in a savings of 3.7 TBtu/yr (7.2 billion ft3 natural gas) by the year 2030. Additionally, new

Refractory hypertension: definition, prevalence, and patient characteristics.

Science.gov (United States)

Acelajado, Maria Czarina; Pisoni, Roberto; Dudenbostel, Tanja; Dell'Italia, Louis J; Cartmill, Falynn; Zhang, Bin; Cofield, Stacey S; Oparil, Suzanne; Calhoun, David A

2012-01-01

Among patients with resistant hypertension (RHTN), there are those whose blood pressure (BP) remains uncontrolled in spite of maximal medical therapy. This retrospective analysis aims to characterize these patients with refractory hypertension. Refractory hypertension was defined as BP that remained uncontrolled after ≥3 visits to a hypertension clinic within a minimum 6-month follow-up period. Of the 304 patients referred for RHTN, 29 (9.5%) remained refractory to treatment. Patients with refractory hypertension and those with controlled RHTN had similar aldosterone levels and plasma renin activity (PRA). Patients with refractory hypertension had higher baseline BP (175±23/97±15 mm Hg vs 158±25/89±15 mm Hg; P=.001/.005) and heart rate, and higher rates of prior stroke and congestive heart failure. During follow-up, the BP of patients with refractory hypertension remained uncontrolled (168.4±14.8/93.8±17.7 mm Hg) in spite of use of an average of 6 antihypertensive medications, while those of patients with controlled RHTN decreased to 129.3±11.2/77.6±10.8 mm Hg. Spironolactone reduced the BP by 12.9±17.8/6.6±13.7 mm Hg in patients with refractory hypertension and by 24.1±16.7/9.2±12.0 mm Hg in patients with controlled RHTN. In patients with RHTN, approximately 10% remain refractory to treatment. Similar aldosterone and PRA levels and a diminished response to spironolactone suggest that aldosterone excess does not explain the treatment failure. © 2011 Wiley Periodicals, Inc.

Punctate white matter lesions in full-term infants with neonatal seizures associated with SLC13A5 mutations

NARCIS (Netherlands)

Weeke, Lauren C; Brilstra, Eva; Braun, Kees P; Zonneveld-Huijssoon, Evelien; Salomons, Gajja S; Koeleman, BPC; van Gassen, Koen L; van Straaten, Henrica L; Craiu, Dana; de Vries, Linda S

INTRODUCTION: Early-onset epileptic encephalopathy caused by biallelic SLC13A5 mutations is characterized by seizure onset in the first days of life, refractory epilepsy and developmental delay. Little detailed information about the brain MRI features is available in these patients. METHODS:

[Frequency of the most common mutations of the CFTR gene in peruvian patients with cystic fibrosis using the ARMS-PCR technique].

Science.gov (United States)

Aquino, Ruth; Protzel, Ana; Rivera, Juan; Abarca, Hugo; Dueñas, Milagros; Nestarez, Cecilia; Purizaga, Nestor; Diringer, Benoit

2017-01-01

To determine the frequency of the ten most common mutations of the CFTR gene reported in Latin Americausing amplification-refractory mutation system-polymerase chain reaction (ARMS-PCR) in patients with cystic fibrosis (CF) in two referral hospitals in Peru during the year 2014. The frequency of the ten most common mutations of the CFTR gene was assessed in patients of the Hospital Nacional Edgardo Rebagliati Martins and the Instituto Nacional de Salud del Niño, both located in Lima, Peru. Blood samples were collected from 36 patients with CF, and the ARMS-PCR technique was used to determine the presence of these mutations. The study group included 73.5% of patients with a known diagnosis of CF in the country when the study was carried out. ARMS-PCR allowed three of the mutations to be identified in a combined 30.6% of the alleles from patients with CF, and 64.9% of the mutated alleles were not identified. The mutations found were p.Phe508del (22,2%), p.Gly542* (6,9%), and p.Arg1162* (1,4%). There is significant variability in both the frequency and type of mutations present in our study population and in what has been reported in other Latin American countries. It is necessary to perform studies that use complete sequencing technology for the CFTR gene to identify other mutations present in our population.

Mutation rules and the evolution of sparseness and modularity in biological systems.

Directory of Open Access Journals (Sweden)

Tamar Friedlander

Full Text Available Biological systems exhibit two structural features on many levels of organization: sparseness, in which only a small fraction of possible interactions between components actually occur; and modularity--the near decomposability of the system into modules with distinct functionality. Recent work suggests that modularity can evolve in a variety of circumstances, including goals that vary in time such that they share the same subgoals (modularly varying goals, or when connections are costly. Here, we studied the origin of modularity and sparseness focusing on the nature of the mutation process, rather than on connection cost or variations in the goal. We use simulations of evolution with different mutation rules. We found that commonly used sum-rule mutations, in which interactions are mutated by adding random numbers, do not lead to modularity or sparseness except for in special situations. In contrast, product-rule mutations in which interactions are mutated by multiplying by random numbers--a better model for the effects of biological mutations--led to sparseness naturally. When the goals of evolution are modular, in the sense that specific groups of inputs affect specific groups of outputs, product-rule mutations also lead to modular structure; sum-rule mutations do not. Product-rule mutations generate sparseness and modularity because they tend to reduce interactions, and to keep small interaction terms small.

Mechanical properties of refractory concretes for boilers; Propriedades mecanicas de concretos refratarios para caldeiras

Energy Technology Data Exchange (ETDEWEB)

Angioletto, E.; Pelisser, F.; Peterson, M.; Angioletto, E.; Rocha, M.R.; Arnt, A.B.C. [Universidade do Estado de Santa Catarina (UDESC), Criciuma, SC (Brazil)], E-mail: ean@unesc.net; Coelho, R. [Tractebel Energia, Florianpolis, SC (Brazil)

2010-07-01

Refractory concretes are used in extremely physically demanding conditions. An important example is concretes used as sealing systems in boilers, where their dimensional stability at high temperatures is fundamental to durability and has a strong impact on maintenance costs and system idleness. In this work, refractory concretes with different compositions were characterized and then tested by dilatometry, for compressive strength and in assays involving adherence between tensile concrete/metal inserts, simulating the fixing system in boilers. Analysis of the results showed that refractory concretes do not present retraction due to air during drying, eliminating the possibility of cracking. While casting the plate/prototype with metal inserts, satisfactory concrete/metal adherence was verified and surface cracking occurred that did not influence the tensile bond strength between the insert and the concrete. (author)

Quantum dots immunofluorescence histochemical detection of EGFR gene mutations in the non-small cell lung cancers using mutation-specific antibodies

Directory of Open Access Journals (Sweden)

Qu YG

2014-12-01

Full Text Available Yan-Gang Qu,1 Qian Zhang,2 Qi Pan,3 Xian-Da Zhao,4 Yan-Hua Huang,2 Fu-Chun Chen,3 Hong-Lei Chen41Department of Pathology, The Central Hospital of Enshi Autonomous Prefecture, Enshi, 2Department of Molecular Pathology, Wuhan Nano Tumor Diagnosis Engineering Research Center, Wuhan, Hubei, People’s Republic of China; 3Department of Thoracosurgery, Traditional Chinese Medical Hospital of Wenling, Wenling, Zhejiang, People’s Republic of China; 4Department of Pathology, School of Basic Medical Science, Wuhan University, Wuhan, Hubei, People’s Republic of ChinaBackground: Epidermal growth factor receptor (EGFR mutation status plays an important role in therapeutic decision making for non-small cell lung cancer (NSCLC patients. Since EGFR mutation-specific antibodies (E746-A750del and L858R have been developed, EGFR mutation detection by immunohistochemistry (IHC is a suitable screening test. On this basis, we want to establish a new screening test, quantum dots immunofluorescence histochemistry (QDs-IHC, to assess EGFR gene mutation in NSCLC tissues, and we compared it to traditional IHC and amplification refractory mutation system (ARMS.Materials and methods: EGFR gene mutations were detected by QDs-IHC, IHC, and ADx-ARMS in 65 cases of NSCLC composed of 55 formalin-fixed, paraffin-embedded specimens and ten pleural effusion cell blocks, including 13 squamous cell carcinomas, two adenosquamous carcinomas, and 50 adenocarcinomas.Results: Positive rates of EGFR gene mutations detected by QDs-IHC, IHC, and ADx-ARMS were 40.0%, 36.9%, and 46.2%, respectively, in 65 cases of NSCLC patients. The sensitivity of QDs-IHC when detecting EGFR mutations, as compared to ADx-ARMS, was 86.7% (26/30; the specificity for both antibodies was 100.0% (26/26. IHC sensitivity was 80.0% (24/30 and the specificity was 92.31% (24/26. When detecting EGFR mutations, QDs-IHC and ADx-ARMS had perfect consistency (κ=0.882; P<0.01. Excellent agreement was observed

Refractories for Industrial Processing. Opportunities for Improved Energy Efficiency

Energy Technology Data Exchange (ETDEWEB)

Hemrick, James G. [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States); Hayden, H. Wayne [Metals Manufacture Process and Controls Technology, Inc., Oak Ridge, TN (United States); Angelini, Peter [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States); Moore, Robert E. [R.E. Moore Associates, Maricopa, AZ (United States); Headrick, William L. [R.E. Moore Associates, Maricopa, AZ (United States)

2005-01-01

Refractories are a class of materials of critical importance to manufacturing industries with high-temperature unit processes. This study describes industrial refractory applications and identifies refractory performance barriers to energy efficiency for processing. The report provides recommendations for R&D pathways leading to improved refractories for energy-efficient manufacturing and processing.

Punctate white matter lesions in full-term infants with neonatal seizures associated with SLC13A5 mutations

NARCIS (Netherlands)

Weeke, Lauren C.; Brilstra, Eva; Braun, Kees P.; Zonneveld-Huijssoon, Evelien; Salomons, Gajja S.; Koeleman, Bobby P; van Gassen, Koen L. I.; van Straaten, Henrica L.; Craiu, Dana; de Vries, Linda S.

Introduction: Early-onset epileptic encephalopathy caused by biallelic SLC13A5 mutations is characterized by seizure onset in the first days of life, refractory epilepsy and develop mental delay. Little detailed information about the brain MRI features is available in these patients. Methods:

Energy conservation in industrial furnaces with vertical radiation roofs of reinforced refractory concrete

Energy Technology Data Exchange (ETDEWEB)

Grafe, E

1981-01-01

The paper discusses static systems for furnaces of reinforced refractory concrete, the temperature field over the finned-plate cross section, the calculation of the reinforced refractory concrete, experimental application in a flat open-hearth pusher furnace, a pack heating furnace, and a sinker furnace. There are cantilever beam plates, frames, and drop ceiling elements particularly suited for efficient use of high-performance burners.

Efficient fractal-based mutation in evolutionary algorithms from iterated function systems

Science.gov (United States)

Salcedo-Sanz, S.; Aybar-Ruíz, A.; Camacho-Gómez, C.; Pereira, E.

2018-03-01

In this paper we present a new mutation procedure for Evolutionary Programming (EP) approaches, based on Iterated Function Systems (IFSs). The new mutation procedure proposed consists of considering a set of IFS which are able to generate fractal structures in a two-dimensional phase space, and use them to modify a current individual of the EP algorithm, instead of using random numbers from different probability density functions. We test this new proposal in a set of benchmark functions for continuous optimization problems. In this case, we compare the proposed mutation against classical Evolutionary Programming approaches, with mutations based on Gaussian, Cauchy and chaotic maps. We also include a discussion on the IFS-based mutation in a real application of Tuned Mass Dumper (TMD) location and optimization for vibration cancellation in buildings. In both practical cases, the proposed EP with the IFS-based mutation obtained extremely competitive results compared to alternative classical mutation operators.

Tungsten and refractory metals 3, proceedings

International Nuclear Information System (INIS)

Bose, A.; Dowding, R.J.

1996-01-01

The Third International Conference on Tungsten and Refractory Metals was held in Greater Washington DC at the McLean Hilton, McLean Virginia, on November 15--16, 1995. This meeting was the third in a series of conferences held in the Washington DC area. The first meeting was in 1992 and was entitled ''International Conference on Tungsten and Tungsten Alloys.'' In 1994, the scope of the meeting was expanded to include other refractory metals such as molybdenum, iridium, rhenium, tantalum and niobium. The tremendous success of that meeting was the primary motivation for this Conference. The broader scope (the inclusion of other refractory metals and alloys) of the Conference was kept intact for this meeting. In fact, it was felt that the developments in the technology of these materials required a common forum for the interchange of current research information. The papers presented in this meeting examined the rapid advancements in the technology of refractory metals, with special emphasis on the processing, structure, and properties. Among the properties there was emphasis on both quasi-static and dynamic rates. Another topic that received considerable interest was the area of refractory carbides and tungsten-copper composites. One day of concurrent session was necessary to accommodate all of the presentations

Evaluation of olivine refractories for TES

Science.gov (United States)

Gay, B. M.; Cochrane, R. L.; Palmour, H., III; Paisley, M. J.

1982-02-01

The principal objectives of this program are to (1) experimentally determine the degree of improvement in thermal and mechanical performance that can be obtained with an olivine thermal storage brick made of domestic materials using advanced processing techniques compared with state-of-the-art as represented by commercial European bricks, (2) conduct an assessment of existing German ceramic process technology and determine its adaptability to domestic raw materials and manufacturing practices, and (3) investigate, on a limited basis, method for further improvement of domestic-olivine brick. To date, accomplishments include (1) installation of improved, computer-based instrumentation, (2) the use of this system to determine performance characteristics of a set of heat storage refractories under cyclic use conditions, (3) acquisition of the services of a knowledgeable European consultant, (4) continued lab-scale process/property optimization studies, and (5) comparative testing of olivine-based and magnesite-based heat storage refractories in the calorimetric test facility at Purdue University.

Refractories for steel-works

International Nuclear Information System (INIS)

Villanova, R.A.; Galant, C.L.; Haas, C.; Rosenbaum, V.

The routine procedures utilized for quality control of refractory materials used by PIRATINI's steel-works, are presented ' under an objetive and practical maner. The attention of the paper is concentrated upon the following' refractory types with higher consume: silicon-aluminous; aluminous; basic magnesia; basic chrom-magnesia. All steps of utilization are described, including specification, supplies programation, storage; sampling; physical tests, and also aplication procedures. Results from routine analysis during a six month period, by ' means of X-Ray Quantometry, using the fusion pearls procedure, are presented compared with Atomic Absorption [pt

Review of alkali metal and refractory alloy compatibility for Rankine cycle applications

International Nuclear Information System (INIS)

DiStefano, J.R.

1989-01-01

The principal corrosion mechanisms in refractory metal-alkali systems are dissolution, mass transfer, and impurity reactions. In general, niobium, tantalum, molybdenum, and tungsten have low solubilities in the alkali metals, even to very high temperatures, and static corrosion studies have verified that the systems are basically compatible. Loop studies with niobium and tantalum based alloys do not indicate any serious problems due to temperature gradient mass transfer. Above 1000 K, dissimilar metal mass transfer is noted between the refractory metals and iron or nickel based alloys. The most serious corrosion problems encountered are related to impurity reactions associated with oxygen

Development of AZS refractories for the glass industry

International Nuclear Information System (INIS)

Guzman, A.M.; Rodriguez, P.

2004-01-01

Refractory materials can support high temperatures, thermal strength and the contact with aggressive environments, for this reason they are widely used in the cement, glass and steel industry. Commercial AZS (alumina-zirconia-silica) refractories are a good alternative in refractory materials for the glass industry' because they can support the aggressive conditions during liquid processing of glass. However, another problem encountered in glass industry is contamination by refractory' material that fall into the molten glass, which can produce a series of defects in the final product. This research was conducted to develop new formulations of AZS refractories with different amounts of ZrO 2 with the purpose of improving the characteristics, properties and the work conditions in the glass melting furnaces and, at the same time, lower the costs this type of refractories. The results obtained indicate that the composition with low content of ZrO 2 can provide better properties than the commercial product, with some modifications in the particle size distribution. Copyright (2004) AD-TECH - International Foundation for the Advancement of Technology Ltd

Direct brazing of ceramics, graphite, and refractory metals

International Nuclear Information System (INIS)

Canonico, D.A.; Cole, N.C.; Slaughter, G.M.

1976-03-01

ORNL has been instrumental in the development of brazing filler metals for joining ceramics, graphite, and refractory metals for application at temperatures above 1000 0 C. The philosophy and techniques employed in the development of these alloys are presented. A number of compositions are discussed that have been satisfactorily used to braze ceramics, graphite, and refractory metals without a prior surface treatment. One alloy, Ti--25 percent Cr--21 percent V, has wet and flowed on aluminum oxide and graphite. Further, it has been utilized in making brazes between different combinations of the three subject materials. The excellent flowability of this alloy and alloys from the Ti--Zr--Ge system is evidenced by the presence of filler metal in the minute pores of the graphite and ceramics

Oral tranexamic acid lightens refractory melasma.

Science.gov (United States)

Tan, Aaron Wei Min; Sen, Priya; Chua, Sze Hon; Goh, Boon Kee

2017-08-01

Melasma is a common acquired hyperpigmentary disorder, particularly among Asians and Hispanics, but its exact pathomechanism is poorly understood. Tranexamic acid has been found to lighten melasma by interfering with the interaction of melanocytes and keratinocytes by inhibiting the plasminogen/plasmin system. The aim was to evaluate the therapeutic effects of oral tranexamic acid in the treatment of melasma refractory to topical skin-lightening agents. This retrospective study analyses patients with melasma recruited from a tertiary dermatological centre in Singapore between 1 August 2009 and 31 March 2011. The patients chosen had refractory melasma treated with oral tranexamic acid 250 mg twice daily in addition to pre-existing combination topical therapy. Objective assessment using the physician's global assessment and melasma area and severity index (MASI) scores were performed based on a post-hoc analysis of photographic records by three independent physicians. A paired t-test was used to evaluate the changes in the MASI scores pre-therapy and post-treatment. Statistical significance was defined as P tranexamic acid for a mean period of 3.7 ± 0.33 months, in addition to combination topical therapy. Their mean age was 47.2 ± 1.61 years. The mean MASI scores after tranexamic acid treatment (2.7 ± 1.6) were significantly lower (P tranexamic acid can serve as a safe and useful adjunct in the treatment of refractory melasma. © 2016 The Australasian College of Dermatologists.

Presence of c.3956delC mutation in familial adenomatous polyposis patients from Brazil.

Science.gov (United States)

Moreira-Nunes, Caroline Aquino; Alcântara, Diego di Felipe Ávila; Lima-Júnior, Sérgio Figueiredo; Cavalléro, Sandro Roberto de Araújo; Rey, Juan Antonio; Pinto, Giovanny Rebouças; de Assumpção, Paulo Pimentel; Burbano, Rommel Rodriguez

2015-08-21

To characterize APC gene mutations and correlate them with patient phenotypes in individuals diagnosed with familial adenomatous polyposis (FAP) in northern Brazil. A total of 15 individuals diagnosed with FAP from 5 different families from the north of Brazil were analyzed in this study. In addition to patients with histopathological diagnosis of FAP, family members who had not developed the disease were also tested in order to identify mutations and for possible genetic counseling. All analyzed patients or their guardians signed a consent form approved by the Research Ethics Committee of the João de Barros Barreto University Hospital (Belem, Brazil). DNA extracted from the peripheral blood of a member of each of the affected families was subjected to direct sequencing. The proband of each family was sequenced to identify germline mutations using the Ion Torrent platform. To validate the detected mutations, Sanger sequencing was also performed. The samples from all patients were also tested for the identification of mutations by real-time quantitative polymerase chain reaction using the amplification refractory mutation system. Through interviews with relatives and a search of medical records, it was possible to construct genograms for three of the five families included in the study. All 15 patients from the five families with FAP exhibited mutations in the APC gene, and all mutations were detected in exon 15 of the APC gene. In addition to the patients with a histological diagnosis of FAP, family members without disease symptoms showed the mutation in the APC gene. In the present study, we detected two of the three most frequent germline mutations in the literature: the mutation at codon 1309 and the mutation at codon 1061. The presence of c.3956delC mutation was found in all families from this study, and suggests that this mutation was introduced in the population of the State of Pará through ancestor immigration (i.e., a de novo mutation that arose in one

Refractory migraine in a headache clinic population

Directory of Open Access Journals (Sweden)

Fernandez-Torron Roberto

2011-08-01

Full Text Available Abstract Background Many migraineurs who seek care in headache clinics are refractory to treatment, despite advances in headache therapies. Epidemiology is poorly characterized, because diagnostic criteria for refractory migraine were not available until recently. We aimed to determine the frequency of refractory migraine in patients attended in the Headache Unit in a tertiary care center, according to recently proposed criteria. Methods The study population consisted of a consecutive sample of 370 patients (60.8% females with a mean age of 43 years (range 14-86 evaluated for the first time in our headache unit over a one-year period (between October 2008 and October 2009. We recorded information on clinical features, previous treatments, Migraine Disability Assessment Score (MIDAS, and final diagnosis. Results Overall migraine and tension-type headache were found in 46.4% and 20.5% of patients, respectively. Refractory migraine was found in 5.1% of patients. In refractory migraineurs, the mean MIDAS score was 96, and 36.8% were medication-overusers. Conclusions Refractory migraine is a relatively common and very disabling condition between the patients attended in a headache unit. The proposed operational criteria may be useful in identifying those patients who require care in headache units, the selection of candidates for combinations of prophylactic drugs or invasive treatments such as neurostimulation, but also to facilitate clinical studies in this patient group.

ELABORATION PROCESS OF REFRACTORY MATERIALS BY REACTIVE PROJECTION OF OXIDE, PEROXIDE AND METALS

OpenAIRE

Pilatti , M.; Cransveld , J.; Raymond , G.; Plumat , E.; Duvigneaud , P.

1986-01-01

A device has been set up for spraying with accuracy a compound having a controlled grain size of refractory oxide, peroxide and metals. The refractory material is obtained by melting and recristallisation on a preheated substrate. Composition in the CaO.Al2O3 system and in the CaO-Al2O3-ZrO2 system have been synthesized. They have been analysed by X-ray diffraction and SEM. Dense and well cristallized materials have been deposited. This process can be successfully applied for the repair of a ...

DiMeX: A Text Mining System for Mutation-Disease Association Extraction.

Science.gov (United States)

Mahmood, A S M Ashique; Wu, Tsung-Jung; Mazumder, Raja; Vijay-Shanker, K

2016-01-01

The number of published articles describing associations between mutations and diseases is increasing at a fast pace. There is a pressing need to gather such mutation-disease associations into public knowledge bases, but manual curation slows down the growth of such databases. We have addressed this problem by developing a text-mining system (DiMeX) to extract mutation to disease associations from publication abstracts. DiMeX consists of a series of natural language processing modules that preprocess input text and apply syntactic and semantic patterns to extract mutation-disease associations. DiMeX achieves high precision and recall with F-scores of 0.88, 0.91 and 0.89 when evaluated on three different datasets for mutation-disease associations. DiMeX includes a separate component that extracts mutation mentions in text and associates them with genes. This component has been also evaluated on different datasets and shown to achieve state-of-the-art performance. The results indicate that our system outperforms the existing mutation-disease association tools, addressing the low precision problems suffered by most approaches. DiMeX was applied on a large set of abstracts from Medline to extract mutation-disease associations, as well as other relevant information including patient/cohort size and population data. The results are stored in a database that can be queried and downloaded at http://biotm.cis.udel.edu/dimex/. We conclude that this high-throughput text-mining approach has the potential to significantly assist researchers and curators to enrich mutation databases.

Response properties of the refractory auditory nerve fiber.

Science.gov (United States)

Miller, C A; Abbas, P J; Robinson, B K

2001-09-01

The refractory characteristics of auditory nerve fibers limit their ability to accurately encode temporal information. Therefore, they are relevant to the design of cochlear prostheses. It is also possible that the refractory property could be exploited by prosthetic devices to improve information transfer, as refractoriness may enhance the nerve's stochastic properties. Furthermore, refractory data are needed for the development of accurate computational models of auditory nerve fibers. We applied a two-pulse forward-masking paradigm to a feline model of the human auditory nerve to assess refractory properties of single fibers. Each fiber was driven to refractoriness by a single (masker) current pulse delivered intracochlearly. Properties of firing efficiency, latency, jitter, spike amplitude, and relative spread (a measure of dynamic range and stochasticity) were examined by exciting fibers with a second (probe) pulse and systematically varying the masker-probe interval (MPI). Responses to monophasic cathodic current pulses were analyzed. We estimated the mean absolute refractory period to be about 330 micros and the mean recovery time constant to be about 410 micros. A significant proportion of fibers (13 of 34) responded to the probe pulse with MPIs as short as 500 micros. Spike amplitude decreased with decreasing MPI, a finding relevant to the development of computational nerve-fiber models, interpretation of gross evoked potentials, and models of more central neural processing. A small mean decrement in spike jitter was noted at small MPI values. Some trends (such as spike latency-vs-MPI) varied across fibers, suggesting that sites of excitation varied across fibers. Relative spread was found to increase with decreasing MPI values, providing direct evidence that stochastic properties of fibers are altered under conditions of refractoriness.

Two Siblings With a CDKL5 Mutation: Genotype and Phenotype Evaluation.

Science.gov (United States)

Hagebeuk, Eveline E O; Marcelis, Carlo L; Alders, Mariëlle; Kaspers, Ageeth; de Weerd, Al W

2015-10-01

This is the second report of a family with a recurrence of a CDKL5 mutation (c. 283-3_290del) in 2 sisters. Both parents tested negative for the mutation in all tissues, but germline mosaicism is likely. Clinically CDKL5 patients resemble those with Rett syndrome, caused by a MECP2 mutation, who experience a regression, after an initial normal development. Even though both siblings showed a typical CDKL5 phenotype, their presentation is different. From birth, the oldest daughter had a severe developmental delay, feeding problems, and hypotonia and experienced daily refractory seizures. The youngest daughter appeared to be normal until age 3 months. At that age seizures started, deterioration and regression became evident, and an epileptic encephalopathy developed. This report of familial recurrence, with suspected germline mosaicism in a healthy parent, has important consequences for genetic counseling. Although it is not possible to predict an exact recurrence risk, it is likely to be increased. © The Author(s) 2015.

An investigation into mineral processing of north Semnan refractory earth

International Nuclear Information System (INIS)

Aslani, S.; Samin-Bani-Hashemi, H.R.; Taghi-Zadeh, O.

2002-01-01

This paper is dealing with refractory earth of North Semnan. Having an area of 2000 square kilometers, Semnan province is mainly formed by sedimentary rocks with a verity of refractory earth, red earth and kaolin containing heavy minerals. The refractory earth of this area contains a considerable rate of aluminum oxide in shape of dia spore minerals, behemoth and gybsite along with heavy minerals of iron and titanium. To improve the quality of refractory earth, in order to be used in related industries, these minerals have to be separated. To assess the quality of refractory earth of North Semnan as the raw materials of refractory industries, their genesis and mineralogy properties have been precisely studied. Based on the rate of aluminium oxide of the refractory earth of North Semnan mines, a suitable mineral deposit has been selected for more investigation. Using XRD and X RF methods along with electronic and photo microscopes, the refractory earth and heavy minerals of them have been assessed. The elementary laboratory experiments of fragmentation and magnetic separation have been performed. It has been proved that the iron minerals can be separated and, therefore, the quality of the refractory earth can be improved. The separation of titanium minerals has to be investigated with other methods

Development of a New Ferrous Aluminosilicate Refractory Material for Investment Casting of Aluminum Alloys

Science.gov (United States)

Yuan, Chen; Jones, Sam; Blackburn, Stuart

2012-12-01

Investment casting is a time-consuming, labour intensive process, which produces complex, high value-added components for a variety of specialised industries. Current environmental and economic pressures have resulted in a need for the industry to improve current casting quality, reduce manufacturing costs and explore new markets for the process. Alumino-silicate based refractories are commonly used as both filler and stucco materials for ceramic shell production. A new ceramic material, norite, is now being produced based on ferrous aluminosilicate chemistry, having many potential advantages when used for the production of shell molds for casting aluminum alloy. This paper details the results of a direct comparison made between the properties of a ceramic shell system produced with norite refractories and a typical standard refractory shell system commonly used in casting industry. A range of mechanical and physical properties of the systems was measured, and a full-scale industrial casting trial was also carried out. The unique properties of the norite shell system make it a promising alternative for casting aluminum based alloys in the investment foundry.

Performance of refractory alloy-clad fuel pins

International Nuclear Information System (INIS)

Dutt, D.S.; Cox, C.M.; Millhollen, M.K.

1984-12-01

This paper discusses objectives and basic design of two fuel-cladding tests being conducted in support of SP-100 technology development. Two of the current space nuclear power concepts use conventional pin type designs, where a coolant removes the heat from the core and transports it to an out-of-core energy conversion system. An extensive irradiation testing program was conducted in the 1950's and 1960's to develop fuel pins for space nuclear reactors. The program emphasized refractory metal clad uranium nitride (UN), uranium carbide (UC), uranium oxide (UO 2 ), and metal matrix fuels (UCZr and BeO-UO 2 ). Based on this earlier work, studies presented here show that UN and UO 2 fuels in conjunction with several refractory metal cladding materials demonstrated high potential for meeting space reactor requirements and that UC could serve as an alternative but higher risk fuel

Characterisation and confirmation of rare beta-thalassaemia mutations in the Malay, Chinese and Indian ethnic groups in Malaysia.

Science.gov (United States)

Tan, Jin Ai Mary Anne; Chin, Pui See; Wong, Yean Ching; Tan, Kim Lian; Chan, Lee Lee; George, Elizabeth

2006-10-01

In Malaysia, about 4.5% of the Malay and Chinese populations are heterozygous carriers of beta-thalassaemia. The initial identification of rare beta-globin gene mutations by genomic sequencing will allow the development of simpler and cost-effective PCR-based techniques to complement the existing amplification refractory mutation system (ARMS) and gap-PCR used for the identification of beta-thalassaemia mutations. DNA from 173 beta-thalassaemia carriers and five beta-thalassaemia major patients from the Malay, Chinese and Indian ethnic groups were first analysed by ARMS and gap-PCR. Ninety-five per cent (174/183) of the 183 beta-globin genes studied were characterised using these two techiques. The remaining nine uncharacterised beta-globin genes (4.9%) were analysed using genomic sequencing of a 904 bp amplified PCR product consisting of the promoter region, exon 1, intervening sequence (IVS) 1, exon 2 and the 5' IVS2 regions of the beta-globin gene. The rare beta-globin mutations detected in the Chinese patients were CD27/28 (+C) and CD43 (GAG-TAG), and -88 (C-T) in an Indian patient. Beta-globin mutations at CD16 (-C), IVS1-1 (G-A), IVS2-1 (G-A), -86 (C-G) and Haemoglobin South Florida (CD1, GTG-ATG) were confirmed in the Malay patients. The seven rare beta-globin mutations and a rare haemoglobin variant confirmed in this study have been described in other populations but have not been previously described in Malaysian beta-thalassemia patients.

Multicenter Retrospective Analysis of the Effectiveness and Safety of Rituximab in Korean Patients with Refractory Systemic Lupus Erythematosus

Directory of Open Access Journals (Sweden)

So-Young Bang

2012-01-01

Full Text Available Objective. Although two recent randomized placebo-controlled trials of rituximab (RTX failed to demonstrate efficacy in systemic lupus erythematosus (SLE, clinicians continue to use off-label RTX for cases refractory to current treatments. We evaluated the effectiveness and safety of rituximab for patients with refractory SLE in Korea. Methods. We retrospectively analyzed multicenter patients treated with RTX in Korea. Results. 39 SLE patients treated with RTX were included in the following manner: lupus nephritis 43.6%, hematologic 33.3%, arthritis 7.8%, myositis 7.8%, and others 7.7%. All patients had responded poorly to at least one conventional immunosuppressive agent (mean 2.5 ± 1.1, cyclophosphamide 43.6%, mycophenolate mofetil 48.7%, and other drugs before RTX. Clinical improvements (complete or partial remission occurred in patients with renal disease, hematologic disease, arthritis, myositis, and other manifestations at 6 months after RTX. The SLEDAI score was significantly decreased from 10.8±7.1 at baseline to 6.7±4.0 at 6 months, 6.2±4.1 at 12 months, and 5.5±3.6 at 24 months after RTX (P<0.05. Among 28 clinical responders, 4 patients experienced a relapse of disease at 25±4 months. Infections were noted in 3 patients (7.7%. Conclusion. RTX could be an effective and relatively safe therapeutic option in patients with severe refractory SLE until novel B-cell depletion therapy is available.

Temperature and Thermal Stress Analysis of Refractory Products

Directory of Open Access Journals (Sweden)

Shaoyang Shi

2013-05-01

Full Text Available Firstly current status of temperature and thermal stress research of refractory product at home and aboard are analyzed. Finite element model of two classical refractory products is building by using APDL language. Distribution law of temperature and thermal stress of two typical refractory products-ladles and tundish are analyzed and their structures are optimized. Stress of optimal structure is dropped obviously, and operation life is increased effectively.

Extracorporeal membrane oxygenation for refractory cardiac arrest

Directory of Open Access Journals (Sweden)

Steven A Conrad

2017-01-01

Full Text Available Extracorporeal cardiopulmonary resuscitation (ECPR is the use of rapid deployment venoarterial (VA extracorporeal membrane oxygenation to support systemic circulation and vital organ perfusion in patients in refractory cardiac arrest not responding to conventional cardiopulmonary resuscitation (CPR. Although prospective controlled studies are lacking, observational studies suggest improved outcomes compared with conventional CPR when ECPR is instituted within 30-60 min following cardiac arrest. Adult and pediatric patients with witnessed in-hospital and out-of-hospital cardiac arrest and good quality CPR, failure of at least 15 min of conventional resuscitation, and a potentially reversible cause for arrest are candidates. Percutaneous cannulation where feasible is rapid and can be performed by nonsurgeons (emergency physicians, intensivists, cardiologists, and interventional radiologists. Modern extracorporeal systems are easy to prime and manage and are technically easy to manage with proper training and experience. ECPR can be deployed in the emergency department for out-of-hospital arrest or in various inpatient units for in-hospital arrest. ECPR should be considered for patients with refractory cardiac arrest in hospitals with an existing extracorporeal life support program, able to provide rapid deployment of support, and with resources to provide postresuscitation evaluation and management.

REFRACTORY FOR BLACK LIQUOR GASIFIERS

Energy Technology Data Exchange (ETDEWEB)

William L. Headrick Jr.; Alireza Rezaie

2003-12-01

surfaces in-situ; are functionally-graded to give the best combination of thermal, mechanical, and physical properties and chemical stability; or are relatively inexpensive, reliable repair materials. Material development will be divided into 2 tasks: Task 1, Development and property determinations of improved and existing refractory systems for black liquor containment. Refractory systems of interest include magnesia aluminate and baria aluminate spinels for binder materials, both dry and hydratable, and materials with high alumina contents, 85-95 wt%, aluminum oxide, 5.0-15.0 wt%, and BaO, SrO, CaO, ZrO and SiC. Task 2, Finite element analysis of heat flow and thermal stress/strain in the refractory lining and steel shell of existing and proposed vessel designs. Stress and strain due to thermal and chemical expansion has been observed to be detrimental to the lifespan of existing black liquor gasifiers. The thermal and chemical strain as well as corrosion rates must be accounted for in order to predict the lifetime of the gasifier containment materials.

Refractory for Black Liquor Gasifiers

Energy Technology Data Exchange (ETDEWEB)

William L. Headrick Jr; Alireza Rezaie

2003-12-01

surfaces in-situ; are functionally-graded to give the best combination of thermal, mechanical, and physical properties and chemical stability; or are relatively inexpensive, reliable repair materials. Material development will be divided into 2 tasks: Task 1, Development and property determinations of improved and existing refractory systems for black liquor containment. Refractory systems of interest include magnesia aluminate and baria aluminate spinels for binder materials, both dry and hydratable, and materials with high alumina contents, 85-95 wt%, aluminum oxide, 5.0-15.0 wt%, and BaO, SrO, CaO, ZrO and SiC. Task 2, Finite element analysis of heat flow and thermal stress/strain in the refractory lining and steel shell of existing and proposed vessel designs. Stress and strain due to thermal and chemical expansion has been observed to be detrimental to the lifespan of existing black liquor gasifiers. The thermal and chemical strain as well as corrosion rates must be accounted for in order to predict the lifetime of the gasifier containment materials.

Refractory for Black Liquor Gasifiers

Energy Technology Data Exchange (ETDEWEB)

William L. Headrick Jr; Alireza Rezaie; Xiaoting Liang; Musa Karakus; Jun Wei

2005-12-01

-situ; and were functionally-graded to give the best combination of thermal, mechanical and physical properties and chemical stability; and are relatively inexpensive, reliable repair materials. Material development was divided into 2 tasks: Task 1 was development and property determinations of improved and existing refractory systems for black liquor containment. Refractory systems of interest include magnesium aluminate and barium aluminate for binder materials, both dry and hydratable, and materials with high alumina contents, 85-95 wt%, aluminum oxide, 5.0-15.0 wt%, and BaO, SrO, CaO, ZrO2 and SiC. Task 2 was finite element analysis of heat flow and thermal stress/strain in the refractory lining and steel shell of existing and proposed vessel designs. Stress and strain due to thermal and chemical expansion has been observed to be detrimental to the lifespan of existing black liquor gasifiers. The thermal and chemical strain as well as corrosion rates must be accounted for in order to predict the lifetime of the gasifier containment materials.

Refractory for Black Liquor Gasifiers

Energy Technology Data Exchange (ETDEWEB)

William L. Headrick Jr; Musa Karakus; Xiaoting Liang

2005-10-01

-situ; and were functionally-graded to give the best combination of thermal, mechanical, and physical properties and chemical stability; or are relatively inexpensive, reliable repair materials. Material development were divided into 2 tasks: Task 1, Development and property determinations of improved and existing refractory systems for black liquor containment. Refractory systems of interest include magnesium aluminate and barium aluminate for binder materials, both dry and hydratable, and materials with high alumina contents, 85-95 wt%, aluminum oxide, 5.0-15.0 wt%, and BaO, SrO, CaO, ZrO{sub 2} and SiC. Task 2, Finite element analysis of heat flow and thermal stress/strain in the refractory lining and steel shell of existing and proposed vessel designs. Stress and strain due to thermal and chemical expansion has been observed to be detrimental to the lifespan of existing black liquor gasifiers. The thermal and chemical strain as well as corrosion rates must be accounted for in order to predict the lifetime of the gasifier containment materials.

Refractory for Black Liquor Gasifiers

Energy Technology Data Exchange (ETDEWEB)

William L. Headrick; Musa Karakus; Alireza Rezaie

2004-03-30

surfaces in-situ; are functionally-graded to give the best combination of thermal, mechanical, and physical properties and chemical stability; or are relatively inexpensive, reliable repair materials. Material development will be divided into 2 tasks: Task 1, Development and property determinations of improved and existing refractory systems for black liquor containment. Refractory systems of interest include magnesium aluminate and barium aluminate for binder materials, both dry and hydratable, and materials with high alumina contents, 85-95 wt%, aluminum oxide, 5.0-15.0 wt%, and BaO, SrO, CaO, ZrO2 and SiC. Task 2, Finite element analysis of heat flow and thermal stress/strain in the refractory lining and steel shell of existing and proposed vessel designs. Stress and strain due to thermal and chemical expansion has been observed to be detrimental to the lifespan of existing black liquor gasifiers. The thermal and chemical strain as well as corrosion rates must be accounted for in order to predict the lifetime of the gasifier containment materials.

REFRACTORY FOR BLACK LIQUOR GASIFIERS

Energy Technology Data Exchange (ETDEWEB)

William L. Headrick Jr; Musa Karakus; Xiaoting Liang; Alireza Rezaie

2004-07-01

surfaces in-situ; are functionally-graded to give the best combination of thermal, mechanical, and physical properties and chemical stability; or are relatively inexpensive, reliable repair materials. Material development will be divided into 2 tasks: Task 1, Development and property determinations of improved and existing refractory systems for black liquor containment. Refractory systems of interest include magnesium aluminate and barium aluminate for binder materials, both dry and hydratable, and materials with high alumina contents, 85-95 wt%, aluminum oxide, 5.0-15.0 wt%, and BaO, SrO, CaO, ZrO{sub 2} and SiC. Task 2, Finite element analysis of heat flow and thermal stress/strain in the refractory lining and steel shell of existing and proposed vessel designs. Stress and strain due to thermal and chemical expansion has been observed to be detrimental to the lifespan of existing black liquor gasifiers. The thermal and chemical strain as well as corrosion rates must be accounted for in order to predict the lifetime of the gasifier containment materials.

REFRACTORY FOR BLACK LIQUOR GASIFIERS

Energy Technology Data Exchange (ETDEWEB)

William L. Headrick Jr; Musa Karakus; Xiaoting Liang; Alireza Rezaie

2004-10-01

surfaces in-situ; are functionally-graded to give the best combination of thermal, mechanical, and physical properties and chemical stability; or are relatively inexpensive, reliable repair materials. Material development will be divided into 2 tasks: Task 1, Development and property determinations of improved and existing refractory systems for black liquor containment. Refractory systems of interest include magnesium aluminate and barium aluminate for binder materials, both dry and hydratable, and materials with high alumina contents, 85-95 wt%, aluminum oxide, 5.0-15.0 wt%, and BaO, SrO, CaO, ZrO{sub 2} and SiC. Task 2, Finite element analysis of heat flow and thermal stress/strain in the refractory lining and steel shell of existing and proposed vessel designs. Stress and strain due to thermal and chemical expansion has been observed to be detrimental to the lifespan of existing black liquor gasifiers. The thermal and chemical strain as well as corrosion rates must be accounted for in order to predict the lifetime of the gasifier containment materials.

REFRACTORY FOR BLACK LIQUOR GASIFIERS

Energy Technology Data Exchange (ETDEWEB)

William L. Headrick Jr; Musa Karakus; Xiaoting Liang; Jun Wei

2005-01-01

surfaces in-situ; are functionally-graded to give the best combination of thermal, mechanical, and physical properties and chemical stability; or are relatively inexpensive, reliable repair materials. Material development will be divided into 2 tasks: Task 1, Development and property determinations of improved and existing refractory systems for black liquor containment. Refractory systems of interest include magnesium aluminate and barium aluminate for binder materials, both dry and hydratable, and materials with high alumina contents, 85-95 wt%, aluminum oxide, 5.0-15.0 wt%, and BaO, SrO, CaO, ZrO{sub 2} and SiC. Task 2, Finite element analysis of heat flow and thermal stress/strain in the refractory lining and steel shell of existing and proposed vessel designs. Stress and strain due to thermal and chemical expansion has been observed to be detrimental to the lifespan of existing black liquor gasifiers. The thermal and chemical strain as well as corrosion rates must be accounted for in order to predict the lifetime of the gasifier containment materials.

REFRACTORY FOR BLACK LIQUOR GASIFIERS

Energy Technology Data Exchange (ETDEWEB)

William L. Headrick Jr; Musa Karakus; Xiaoting Liang; Jun Wei

2005-04-01

surfaces in-situ; are functionally-graded to give the best combination of thermal, mechanical, and physical properties and chemical stability; or are relatively inexpensive, reliable repair materials. Material development will be divided into 2 tasks: Task 1, Development and property determinations of improved and existing refractory systems for black liquor containment. Refractory systems of interest include magnesium aluminate and barium aluminate for binder materials, both dry and hydratable, and materials with high alumina contents, 85-95 wt%, aluminum oxide, 5.0-15.0 wt%, and BaO, SrO, CaO, ZrO{sub 2} and SiC. Task 2, Finite element analysis of heat flow and thermal stress/strain in the refractory lining and steel shell of existing and proposed vessel designs. Stress and strain due to thermal and chemical expansion has been observed to be detrimental to the lifespan of existing black liquor gasifiers. The thermal and chemical strain as well as corrosion rates must be accounted for in order to predict the lifetime of the gasifier containment materials.

REFRACTORY FOR BLACK LIQUOR GASIFIERS

Energy Technology Data Exchange (ETDEWEB)

William L. Headrick Jr; Musa Karakus; Xiaoting Liang

2005-07-01

surfaces in-situ; are functionally-graded to give the best combination of thermal, mechanical, and physical properties and chemical stability; or are relatively inexpensive, reliable repair materials. Material development will be divided into 2 tasks: Task 1, Development and property determinations of improved and existing refractory systems for black liquor containment. Refractory systems of interest include magnesium aluminate and barium aluminate for binder materials, both dry and hydratable, and materials with high alumina contents, 85-95 wt%, aluminum oxide, 5.0-15.0 wt%, and BaO, SrO, CaO, ZrO{sub 2} and SiC. Task 2, Finite element analysis of heat flow and thermal stress/strain in the refractory lining and steel shell of existing and proposed vessel designs. Stress and strain due to thermal and chemical expansion has been observed to be detrimental to the lifespan of existing black liquor gasifiers. The thermal and chemical strain as well as corrosion rates must be accounted for in order to predict the lifetime of the gasifier containment materials.

REFRACTORY FOR BLACK LIQUOR GASIFIERS

Energy Technology Data Exchange (ETDEWEB)

William L. Headrick Jr.; Alireza Rezaie

2004-04-01

surfaces in-situ; are functionally-graded to give the best combination of thermal, mechanical, and physical properties and chemical stability; or are relatively inexpensive, reliable repair materials. Material development will be divided into 2 tasks: Task 1, Development and property determinations of improved and existing refractory systems for black liquor containment. Refractory systems of interest include magnesium aluminate and barium aluminate spinels for binder materials, both dry and hydratable, and materials with high alumina contents, 85-95 wt%, aluminum oxide, 5.0-15.0 wt%, and BaO, SrO, CaO, ZrO{sub 2} and SiC. Task 2, Finite element analysis of heat flow and thermal stress/strain in the refractory lining and steel shell of existing and proposed vessel designs. Stress and strain due to thermal and chemical expansion has been observed to be detrimental to the lifespan of existing black liquor gasifiers. The thermal and chemical strain as well as corrosion rates must be accounted for in order to predict the lifetime of the gasifier containment materials.

Comparative evaluation of the locally manufactured low-alumina fireclay refractories

International Nuclear Information System (INIS)

Mohammad, D.; Ahmed, W.; Khan, M.I.

2007-01-01

Properties of domestically produced low-alumina refractories commonly known as medium duty fireclay refractories were studied and a comparative evaluation was performed. Refractory properties such as density, porosity, cold strength, reheat change and deformation at high-temperature were determined. Significant differences were discovered between these products sold as medium duty fireclay refractories. One brand marketed as medium duty refractory was found to be too inferior to be designated as medium duty firebrick. Absence of any standards and lack of awareness on the part of users of the various test methods appear to be the cause of this situation. (author)

Evaluation of technological properties of alumina refractory systems-zirconia and zirconia-silica-alumina

International Nuclear Information System (INIS)

Marinho, A.R.O.; Carvalho, T.U.S.; Fagury Neto, E.; Rabelo, A.A.

2014-01-01

Alumina-zirconia refractories are noted for being products of excellent cost-effective, however, zirconia may limit its use due to decreasing resistance to thermal shock. This study aims to evaluate these refractories with the addition of microsilica, which can greatly improve their properties. Were used the following starting materials: calcined alumina, zirconia (stabilized and monoclinic) in amounts of 2%, 4% and 6% by weight, plus microsilica (5%w.). The powders were milled together with binder and lubricant for conformation bodies by uniaxial pressing. The samples were dried, calcined and sintered at 1400 °C/2h were characterized using the methods of Archimedes, and scanning electron microscopy (SEM), chemical analysis using energy dispersive X-ray (EDS), and mechanical flexural strength tests at room temperature. Formulations with the presence of microsilica showed satisfactory results and optimized properties. (author)

Machining refractory alloys: an overview

International Nuclear Information System (INIS)

Christopher, J.D.

1984-01-01

Nontraditional machining is a generic term for those material removal processes that differ drastically from the historic operations such as turning, milling, drilling, tapping, and grinding. The use of primary energy modes other than mechanical, such as thermal, electrical, and chemical, sets these operations apart and reinforces their nontraditional label. Several of these newer processes have been very successful in machining close tolerance parts from refractory materials. This paper provides a general overview of both traditional and nontraditional aspects of machining refractory materials. 11 figures, 7 tables

Toll-like receptor 2 expression in refractory periapical lesions.

Science.gov (United States)

Desai, S V; Love, R M; Rich, A M; Seymour, G J

2011-10-01

To investigate the expression of TLR2 in refractory periapical lesions. Refractory periapical lesion biopsies were histopathologically and clinically categorized into asymptomatic periapical granuloma (n=10), symptomatic periapical granuloma (n=10) or periapical cyst (n=10) and prepared for immunohistochemical staining using antibodies to TLR2, CD3 and CD19 or staining with methyl green pyronin. Sections were viewed under light microscopy and the presence or absence of the target cells was correlated with the histopathological and clinical data. Additionally, TLR2 expression was quantified by counting TLR(+) cells. Various mononuclear inflammatory cells in the bacteria-induced periapical lesions were reactive to TLR2 antibody, with many showing morphological similarities to lymphocytes and plasma cells. Lymphocytes were the most numerous cells in the inflammatory infiltrate. In refractory periapical granuloma, CD3(+) T cells were more numerous, whereas in periapical cysts, CD19(+) B cells were more numerous. There was a statistically significant (Pperiapical granuloma than asymptomatic periapical granuloma or periapical cyst. The presence of TLR-expressing cells in periapical granulomas and cysts provides further evidence that periapical cysts are likely to be sustained by the immune system via reaction to bacterial antigens. © 2011 International Endodontic Journal.

KIT D816V mutation-positive cell fractions in lesional skin biopsies from adults with systemic mastocytosis

DEFF Research Database (Denmark)

Kielsgaard Kristensen, Thomas; Broesby-Olsen, Sigurd; Vestergaard, Hanne

2013-01-01

Most adults with systemic mastocytosis (SM) carry the somatic KIT D816V mutation, but the occurrence of the mutation in lesional skin remains to be characterized.......Most adults with systemic mastocytosis (SM) carry the somatic KIT D816V mutation, but the occurrence of the mutation in lesional skin remains to be characterized....

Wetting of refractory metals with copper base alloys

International Nuclear Information System (INIS)

Anikeev, E.F.; Kostikov, V.I.; Chepelenko, V.N.; Batov, V.M.

1978-01-01

The effect is studied of phosphorus upon the wetting of molybdenum, niobium and tantalum by an alloy of the system copper-silver (10%) as a function of contact time and phosphorus concentration. Experiments have been conducted in vacuum of 5x10 -4 mm Hg at 900 deg C. It is established that the introduction of phosphorus into a copper-silver alloy improves the wetting of molybdenum, niobium and tantalum. Formation of intermetallic compounds on the alloy-refractory metal interface can be avoided by adjusting the time of contact of the solder with molybdenum, niobium and tantalum. As a solder with 2.9% phosphorus spreads well over copper, it is suggested to use said solder for brazing copper and the investigated refractory metals in items intended for service at temperatures of up to 600 deg C

Production of lightweight refractory material by hydrothermal process

International Nuclear Information System (INIS)

Sulejmani, Ramiz B.

2002-01-01

Many different processes of production of lightweight refractories are well known over the World. Traditional production of lightweight refractories is by addition of combustibles or by a special frothing process. This work is concerned with hydrothermal of lightweight refractories from rice husk ash. The rice husk ash, used in present investigations were from Kocani region, R. Macedonia. The chemical analysis of the rice husk ash shows that it contains 91,8 - 93,7% SiO 2 and some alkaline and alkaline earth oxides. Microscopic and X - ray diffraction examinations of the rice husk ash have shown that it is composed of cristobalite, tridimite and amorphous silica. The composition of the mixture for lightweight refractory brick production is 93,4% rice husk ash and 6,6% Ca(OH) 2 . The mixtures were well mixed, moistened and pressed at 5 - 10 MPa. The hydrothermal reactions between calcium hydroxide and rice husk ash over the temperature range 80 - 160 o C were investigated. The period of autoclave treatment was from 2 to 72 h. After the hydrothermal treatment of the samples, the mineralogical composition, bulk density, density, cold crushing strength, porosity, refractoriness and thermal expansion were examined. Analysing the properties of the obtained samples it can be concluded that from rice husk ash and calcium hydroxide under hydrothermal condition it is possible to obtain lightweight acid refractory material with high quality.(Author)

Engineering data bases for refractory alloys

International Nuclear Information System (INIS)

Cooper, R.H. Jr.; Harms, W.O.

1985-01-01

Refractory alloys based on niobium, molybdenum, tantalum, and tungsten are required for the multi-100kW(e) space nuclear reactor power concepts that have been assessed in the SP-100 Program because of the extremely high temperatures involved. A review is presented of the technology efforts on the candidate refractory alloys in the areas of availability/fabricability, mechanical properties, irradiation effects, and compatibility. Of the niobium-base alloys, only Nb-1Zr has a data base that is sufficiently comprehensive for the high level of confidence required in the reference-alloy selection process for the reactor concept to be tested in the Ground Engineering System (GES) Phase of the SP-100 Program. Based on relatively short-term tests, the alloy PWC-11 (Nb-1Zr-0.1C) appears to have significantly greater creep strength than Nb-1Zr; however, concerns as to whether this precipitation-hardened alloy will remain thermally stable during seven years of full-power reactor operation need to be resolved. Additional information on the reference GES alloy will be needed for the detailed engineering design of a space power system and the fabrication of prototypical GES test components. Expedient development and demonstration of an adequate total manufacturing capability will be required if a high risk of significant schedule slippages and cost overruns is to be avoided. 4 refs., 1 fig., 3 tabs

EFFICACY OF RECURRENT RITUXIMAB TREATMENT IN PATIENT WITH SEVERE REFRACTORY SYSTEMIC JUVENILE RHEUMATOID ARTHRITIS

Directory of Open Access Journals (Sweden)

E.I. Alexeeva

2011-01-01

Full Text Available The article contains clinical case description of a severe systemic juvenile rheumatoid arthritis, that was refractory to classic immunosuppressant therapy. The disease was characterized by such extraarticular manifestations as fever, lymphadenopathy,  hepatosplenomegaly, polyserositis, generalized joint involvement and high activity in lab tests. As a result of severe clinical course of the disease, patients develop bilateral aseptic bone necrosis in coxofemoral joints and coxarthrosis. Against the background of glucocorticosteroid treatment the patient has developed hormone-dependency and hormone resistance. Inclusion into the treatment of anti-CD20 monoclonal antibodies (rituximab has stopped systemic manifestations of the disease, inflammation in the joints, normalized lab activity rates. The positive therapeutic effect allowed to perform surgery due to bilateral coxarthrosis. These results show that rituximab is highly effective in children with systemic juvenile rheumatoid arthritis, that is resistant to classic immunosupressants and glucocorticoides. Key words: children, systemic juvenile rheumatoid arthritis, rituximab, recurrent treatment, prosthetics, hip joint. (Voprosy sovremennoi pediatrii — Current Pediatrics. — 2011; 10 (5: 157–163.

Spinning of refractory metals

International Nuclear Information System (INIS)

Chang Wenkua; Zheng Han

1989-01-01

The effects of spinning process parameters including max. pass percentage reduction, spinning temperature, feed rate, lubricant and annealing technology on the quality of shaped components are summarized and discussed in the present paper. The above mentioned parameters are adopted in the process of spinning of barrel-shaped and specially shaped components of refractory metals and their alloys W, Mo, Nb, Zr, TZM molybdenum alloy, C-103, C-752 niobium alloy etc. The cause of leading to usual defects of spun products of refractory metals such as lamellar as 'scaling', crack, swelling, wrinkle, etc. have been analysed and the ways to eliminate the defects have been put forward. 8 figs., 5 tabs. (Author)

Migration of TRU-containing slag into candidate refractories for waste management

International Nuclear Information System (INIS)

Seymour, W.C.

1978-11-01

Results of initial tests on transuranic migration into commercial refractories are reported. Five castable refractories and a plastic-bonded refractory were tested using the cup test method. Initial results indicate that castable materials will not survive conditions expected in the slagging pyrolysis unit; plastic refractories appear to have better performance. The major mechanism of migration into the tested refractories is pore penetration. Al 2 O 3 and Al 2 O 3 -Cr 2 O 3 phases appear resistant to chemical diffusion of transuranics. 2 figures, 16 tables

A retrospective study on the management of patients with rituximab refractory follicular lymphoma.

Science.gov (United States)

Solal-Céligny, Philippe; Leconte, Pierre; Bardet, Aurélie; Hernandez, Juana; Troussard, Xavier

2018-01-01

Given that there are currently no clear recommendations regarding therapeutic options for rituximab refractory/relapsed follicular lymphoma patients, this study aimed to describe the real-life management of patients with refractory follicular lymphoma after systemic rituximab-containing regimens (rFL), and rFL patient characteristics. In this retrospective, national, multicentre study, descriptive analyses were mainly performed according to rituximab-containing regimen at rFL diagnosis [rituximab monotherapy (R-MONO), rituximab + chemotherapy (R-COMBO), and ongoing rituximab maintenance (R-MAINTAIN)]. The 459 analysed patients experienced rituximab-refractoriness between October 2013 and September 2015: R-MONO: 58 (13%), R-COMBO: 197 (43%), R-MAINTAIN: 204 (44%). Post-refractoriness strategies were heterogeneous: idelalisib ± rituximab (22%), without anti-lymphoma treatment (21%), rituximab-chemotherapy (21%) and stem cell transplantation (18%). Rituximab was continued in combination in 41% of cases. Chosen strategies varied according to patient age (without anti-lymphoma treatment: 28% of patients if ≥65 years vs. 12% if management and for the design of clinical trials in these patients. © 2017 John Wiley & Sons Ltd.

Development of alumino-silicate refractories in Ghana

International Nuclear Information System (INIS)

Kisiedu, A. K.; Tetteh, D.M.B.; Obiri, H. A.; Brenya, E. F.; Ayensu, A.

2008-01-01

Alumino-silicate (bauxite), andalusite, kaolin and clay were investigated for suitability in production of alumina, mullite and fireclay brick refractories. The raw materials were characterized by X-ray diffraction, differential thermal and silicate analyses. The x-ray diffraction analysis of alumina and mullite refractories fired at 1450 0 C, and fireclay bricks fired at 1350 0 C, indicated presence of corundum and alpha-alumina crystals. The values of thermal (fired) shrinkage, crushing, strength, porosity, water absorption and bulk density determined were 31.1%, 2.3 x 10 3 kg/m 3 , 4.86 x 10 6 N/m 2 and 13.2 % for mullite; 30.2%, 2.4 x 10 3 kg/m 3 , 3.20 x 10 6 N/m 2 and W = 12.8 % for alumina; and 25.2 %, 2.1 x 10 3 kg/m 3 , 2.61 x 10 6 N/m 2 and W = 11.8% for fireclay, respectively. Bauxite, andalusite and special kaolin were identified as potential raw materials for developing alumina and mullite refractories for construction of high temperature kilns and furnaces operating at 1350 0 C. The clay and kaolin minerals could be used to produce fireclay refractories for construction of incinerators operating at maximum temperatures of about 1000 0 C. The performance of the refractories was demonstrated by producing bricks to construct kilns and incinerators for the ceramic industry and hospitals. (au)

Evolution and Adaptation in Pseudomonas aeruginosa Biofilms Driven by Mismatch Repair System-Deficient Mutators

DEFF Research Database (Denmark)

Luján, Adela M.; Maciá, María D.; Yang, Liang

2011-01-01

, which are rarely eradicated despite intensive antibiotic therapy. Current knowledge indicates that three major adaptive strategies, biofilm development, phenotypic diversification, and mutator phenotypes [driven by a defective mismatch repair system (MRS)], play important roles in P. aeruginosa chronic...... infections, but the relationship between these strategies is still poorly understood. We have used the flow-cell biofilm model system to investigate the impact of the mutS associated mutator phenotype on development, dynamics, diversification and adaptation of P. aeruginosa biofilms. Through competition...... diversification, evidenced by biofilm architecture features and by a wider range and proportion of morphotypic colony variants, respectively. Additionally, morphotypic variants generated in mutator biofilms showed increased competitiveness, providing further evidence for mutator-driven adaptive evolution...

Deletion mutations of bacteriophage

International Nuclear Information System (INIS)

Ryo, Yeikou

1975-01-01

Resolution of mutation mechanism with structural changes of DNA was discussed through the studies using bacteriophage lambda. One of deletion mutations inductions of phage lambda is the irradiation of ultraviolet ray. It is not clear if the inductions are caused by errors in reparation of ultraviolet-induced damage or by the activation of int gene. Because the effective site of int gene lies within the regions unnecessary for existing, it is considered that int gene is connected to deletion mutations induction. A certain system using prophage complementarity enables to detect deletion mutations at essential hereditary sites and to solve the relations of deletion mutations with other recombination system, DNA reproduction and repairment system. Duplication and multiplication of hereditary elements were discussed. If lambda deletion mutations of the system, which can control recombination, reproduction and repairment of added DNA, are constructed, mutations mechanism with great changes of DNA structure can be solved by phage lambda. (Ichikawa, K.)

Refractory disease in antineutrophil cytoplasmic antibodies associated vasculitis

NARCIS (Netherlands)

Rutgers, Abraham; Kallenberg, Cornelis

Purpose of review Induction treatment of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) is not always successful and nonresponding patients are considered refractory. Recent findings Refractory disease should be subdefined to the treatment that was received.

Diagnosis and management of refractory celiac disease: a systematic review.

Science.gov (United States)

Labidi, Asma; Serghini, Meriem; Karoui, Sami; Boubaker, Jalel; Filali, Azza

2013-01-01

Refractory celiac disease is defined by persisting malabsorptive symptoms in spite of a strict gluten free diet for at least 6 to 12 months. Alternatives to gluten free diet seem to be still controversial. To describe the clinical and epidemiologic aspects of refractory celiac disease, and to identify therapeutic options in this condition. Systematic review and critical analysis of observational studies, clinical trials and case reports that focused on diagnosis and management of refractory celiac disease. Refractory celiac disease can be classified as type 1 or type 2 according to the phenotype of intraepithelial lymphocytes. Great complications such as enteropathy-associated T-cell lymphoma may occur in a subgroup of these patients mainly in refractory celiac disease type 2. Curative therapies are still lacking. Refractory celiac disease remains a diagnosis of exclusion. Its prognosis remains still dismal by the absence yet of curative therapies. However, some new treatments seem to hold promise during few cohort-studies.

Modern materials based on refractory compounds

International Nuclear Information System (INIS)

Kosolapova, T.Ya.

1979-01-01

Discussed are the existing methods for synthesizing powders of binary refractory compounds and high-productivity techniques which hold promise as regards the manufacture of highly disperse and pure powders. Plasmochemical synthesis is shown to be an effective method for obtaining practically all carbides, nitrides and borides. A description is given of three main methods for obtaining single crystals of refractory compounds (TiN, TiC, ZrC, ZrB 2 , NbC) fairly perfect in structure and composition. These processes include deposition from vapour-gas phase, melting in arc plasma and crystallization from solutions in metallic melts. The advantages have been shown of the self-propagating high-temperature synthesis of refractory compounds, ensuring the manufacture of products, close in composition to stoichiometric ones simultaneously with forming of items. Mechanical, thermal, abrasive, and resistive characteristics of the above materials are presented

A parylene-based dual channel microelectrophoresis system for rapid mutation detection via heteroduplex analysis

NARCIS (Netherlands)

Sukas, S.; Erson, Ayse Elif; Sert, Cuneyt; Kulah, Haluk

2008-01-01

A new dual channel micro-electrophoresis system for rapid mutation detection based on heteroduplex analysis was designed and implemented. Mutation detection was successfully achieved in a total separation length of 250 μm in less than 3 min for a 590 bp DNA sample harboring a 3 bp mutation causing

Optimization of the Technological Synthesis of Refractory Compounds

Science.gov (United States)

Gaidar, S. M.; Karelina, M. Yu.; Prikhod'ko, V. M.; Volkov, A. A.

2017-12-01

The results of experimental studies, which are related to the regulation of the fractional composition of refractory compounds by roll milling in using controlled roll opening and unbalanced peripheral speeds of rollers, are reported. The content of prepared fine, middle, and coarse fractions is within 50-80%; in this case, the milling time of synthesis products is less than the time of ball milling by an order of magnitude. The application of roll milling for refining the products of self-propagating high-temperature synthesis can be most efficient in using together with heat-generating reactor to solve the main problem of self-propagating synthesis (SHS), which is a problem for recent several decades (the problem is the creation of intense automated production of refractory compounds in using continuous manufacturing cycle within a energotechnological system with the recovery of a great quantity of heat released during SHS).

A Semantic Web-based System for Mining Genetic Mutations in Cancer Clinical Trials.

Science.gov (United States)

Priya, Sambhawa; Jiang, Guoqian; Dasari, Surendra; Zimmermann, Michael T; Wang, Chen; Heflin, Jeff; Chute, Christopher G

2015-01-01

Textual eligibility criteria in clinical trial protocols contain important information about potential clinically relevant pharmacogenomic events. Manual curation for harvesting this evidence is intractable as it is error prone and time consuming. In this paper, we develop and evaluate a Semantic Web-based system that captures and manages mutation evidences and related contextual information from cancer clinical trials. The system has 2 main components: an NLP-based annotator and a Semantic Web ontology-based annotation manager. We evaluated the performance of the annotator in terms of precision and recall. We demonstrated the usefulness of the system by conducting case studies in retrieving relevant clinical trials using a collection of mutations identified from TCGA Leukemia patients and Atlas of Genetics and Cytogenetics in Oncology and Haematology. In conclusion, our system using Semantic Web technologies provides an effective framework for extraction, annotation, standardization and management of genetic mutations in cancer clinical trials.

Combined mepacrine-hydroxychloroquine treatment in patients with systemic lupus erythematosus and refractory cutaneous and articular activity.

Science.gov (United States)

Ugarte, A; Porta, S; Ríos, R; Martinez-Zapico, A; Ortego-Centeno, N; Agesta, N; Ruiz-Irastorza, G

2018-01-01

Aim The aim of this study was to evaluate the clinical response to combined therapy with hydroxychloroquine and mepacrine in patients with systemic lupus erythematosus and refractory joint and/or skin disease. Methods Mepacrine was added to 46 systemic lupus erythematosus patients unresponsive to treatment with the following drug combinations: hydroxychloroquine + prednisone + immunosuppressive drugs ( n = 24), hydroxychloroquine + prednisone ( n = 16), hydroxychloroquine + prednisone + retinoids ( n = 2), hydroxychloroquine alone ( n = 1), hydroxychloroquine + one immunosuppressive drug ( n = 1), hydroxychloroquine + prednisone + one immunosuppressive drug + belimumab ( n = 1) or hydroxychloroquine + prednisone + belimumab ( n = 1). The outcome variable was the clinical response, either complete or partial, based on clinical judgement. The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) and the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score were additionally used. Results A total of 91% patients showed complete/partial response, with similar rates among those with joint or skin disease. In patients with cutaneous activity, a statistically significant decrease in the CLASI was seen. There also was a statistically significant decrease in the SLEDAI. The mean daily dose of prednisone decreased from 5.8 to 3.4 mg/d ( p = 0.001). Prednisone could be discontinued in 20% of patients. No serious adverse events were seen. Smoking was the only predictor of complete response. Conclusion In the setting of refractory skin and/or joint disease, the addition of mepacrine to previous therapy including hydroxychloroquine was safe and effective in reducing disease activity and decreasing prednisone doses. The fact that smokers responded better opens the door to further studying the combination of mepacrine-hydroxychloroquine as a first-line therapy in such

Refractory versus resistant hypertension: Novel distinctive phenotypes

Science.gov (United States)

Dudenbostel, Tanja; Siddiqui, Mohammed; Gharpure, Nitin; Calhoun, David A.

2017-01-01

Resistant hypertension (RHTN) is relatively common with an estimated prevalence of 10-20% of treated hypertensive patients. It is defined as blood pressure (BP) >140/90 mmHg treated with ≥3 antihypertensive medications, including a diuretic, if tolerated. Refractory hypertension is a novel phenotype of severe antihypertensive treatment failure. The proposed definition for refractory hypertension, i.e. BP >140/90 mmHg with use of ≥5 different antihypertensive medications, including a diuretic and a mineralocorticoid receptor antagonist (MRA) has been applied inconsistently. In comparison to RHTN, refractory hypertension seems to be less prevalent than RHTN. This review focuses on current knowledge about this novel phenotype compared with RHTN including definition, prevalence, mechanisms, characteristics and comorbidities, including cardiovascular risk. In patients with RHTN excess fluid retention is thought to be a common mechanism for the development of RHTN. Recently, evidence has emerged suggesting that refractory hypertension may be more of neurogenic etiology due to increased sympathetic activity as opposed to excess fluid retention. Treatment recommendations for RHTN are generally based on use and intensification of diuretic therapy, especially with the combination of a long-acting thiazide-like diuretic and an MRA. Based on findings from available studies, such an approach does not seem to be a successful strategy to control BP in patients with refractory hypertension and effective sympathetic inhibition in such patients, either with medications and/or device based approaches may be needed. PMID:29034321

Forming Refractory Insulation On Copper Wire

Science.gov (United States)

Setlock, J.; Roberts, G.

1995-01-01

Alternative insulating process forms flexible coat of uncured refractory insulating material on copper wire. Coated wire formed into coil or other complex shape. Wire-coating apparatus forms "green" coat on copper wire. After wire coiled, heating converts "green" coat to refractory electrical insulator. When cured to final brittle form, insulating material withstands temperatures above melting temperature of wire. Process used to make coils for motors, solenoids, and other electrical devices to be operated at high temperatures.

High-dose chemotherapy and auto-SCT for relapsed and refractory Hodgkin's lymphoma patients refractory to first-line salvage chemotherapy but responsive to second-line salvage chemotherapy.

Science.gov (United States)

Rauf, Muhammad Shahzad; Maghfoor, Irfan; Elhassan, Tusneem Ahmed M; Akhtar, Saad

2015-01-01

Relapsed or primary refractory Hodgkin's lymphoma (HL) patients refractory to first-line salvage chemotherapy (first salvage) and unable to undergo high-dose chemotherapy (HDC) and autologous stem cell transplant (auto-SCT) have very poor outcome. Some patients are offered second-line salvage chemotherapy (second salvage), if they are responsive and may receive HDC auto-SCT. We identified 31 patients (18 males, 13 females) from 1996-2012 who received second salvage prior to auto-SCT. Median age at auto-SCT is 22 years. Patients were grouped as (1) relapsed-refractory (Rel:Ref): patients with prior complete response (CR) and on relapse found refractory to first salvage and received second salvage and (2) refractory-refractory (Ref:Ref): patients refractory to both primary treatment and first salvage and received second salvage. Median follow-up is 63 months (18-170). Disease status after second salvage prior to HDC was CR 16 %, partial response (PR) 71 % and stable disease 13 %. After HDC auto-SCT, CR:PR: progressive disease was observed in 18 (58 %): four (12 %): nine (29 %) patients, respectively. Five-year overall survival (OS) for whole group is 57 % (Rel:Ref vs. Ref:Ref, 73 % vs. 48 %, p = 0.48). Progression-free survival (PFS) for whole group is 52 % (Rel:Ref vs. Ref:Ref, 73 % vs. 40 % respectively, p = 0.11). Second-line salvage is a valid approach with no long-term side effects for those HL patients who do not respond to first-line salvage chemotherapy and they can be candidate of HDC and stem cell transplant with a high ORR, the PFS and OS in relapse-refractory and refractory-refractory group of patients.

Gene mutation, quantitative mutagenesis, and mutagen screening in mammalian cells: study with the CHO/HGPRT system

International Nuclear Information System (INIS)

Hsie, A.W.

1980-01-01

We have employed CHO cells to develop and define a set of stringent conditions for studying mutation induction to TG resistance. Several lines of evidence support the CHO/HGPRT system as a specific-locus mutational assay. The system permits quantification of mutation at the HGPRT locus induced by various physical and chemical mutagens. The quantitative nature of the system provides a basis for the study of structure-function relationships of various classes of chemical mutagens. The intra- and interlaboratory reproducibility of this system suggests its potential for screening environmental agents for mutagenic activity

EGFR Mutations in Surgically Resected Fresh Specimens from 697 Consecutive Chinese Patients with Non-Small Cell Lung Cancer and Their Relationships with Clinical Features

Directory of Open Access Journals (Sweden)

Yuanyang Lai

2013-12-01

Full Text Available We aimed to reveal the true status of epidermal growth factor receptor (EGFR mutations in Chinese patients with non-small cell lung cancer (NSCLC after lung resections. EGFR mutations of surgically resected fresh tumor samples from 697 Chinese NSCLC patients were analyzed by Amplification Refractory Mutation System (ARMS. Correlations between EGFR mutation hotspots and clinical features were also explored. Of the 697 NSCLC patients, 235 (33.7% patients had tyrosine kinase inhibitor (TKIs sensitive EGFR mutations in 41 (14.5% of the 282 squamous carcinomas, 155 (52.9% of the 293 adenocarcinomas, 34 (39.5% of the 86 adenosquamous carcinomas, one (9.1% of the 11 large-cell carcinomas, 2 (11.1% of the 18 sarcomatoid carcinomas, and 2 (28.6% of the 7 mucoepidermoid carcinomas. TKIs sensitive EGFR mutations were more frequently found in female patients (p < 0.001, non-smokers (p = 0.047 and adenocarcinomas (p < 0.001. The rates of exon 19 deletion mutation (19-del, exon 21 L858R point mutation (L858R, exon 21 L861Q point mutation (L861Q, exon 18 G719X point mutations (G719X, including G719C, G719S, G719A were 43.4%, 48.1%, 1.7% and 6.8%, respectively. Exon 20 T790M point mutation (T790M was detected in 3 squamous carcinomas and 3 adenocarcinomas and exon 20 insertion mutation (20-ins was detected in 2 patients with adenocarcinoma. Our results show the rates of EGFR mutations are higher in all types of NSCLC in Chinese patients. 19-del and L858R are two of the more frequent mutations. EGFR mutation detection should be performed as a routine postoperative examination in Chinese NSCLC patients.

Refractory myasthenia gravis - clinical profile, comorbidities and response to rituximab.

Science.gov (United States)

Sudulagunta, Sreenivasa Rao; Sepehrar, Mona; Sodalagunta, Mahesh Babu; Settikere Nataraju, Aravinda; Bangalore Raja, Shiva Kumar; Sathyanarayana, Deepak; Gummadi, Siddharth; Burra, Hemanth Kumar

2016-01-01

Introduction: Myasthenia gravis (MG) is an antibody mediated autoimmune neuromuscular disorder characterized by fatigable muscle weakness. A proportion of myasthenia gravis patients are classified as refractory due to non responsiveness to conventional treatment. This retrospective study was done to evaluate clinical profile, epidemiological, laboratory, and features of patients with MG and mode of management using rituximab and complications. Methods: Data of myasthenia gravis patients admitted or presented to outpatient department (previous medical records) with MG between January 2008 and January 2016 were included. A total of 512 patients fulfilled the clinical and diagnostic criteria of myasthenia gravis of which 76 patients met the diagnostic certainty for refractory myasthenia gravis and were evaluated. Results: Out of 76 refractory MG patients, 53 (69.73%) patients fulfilled all the three defined criteria. The median age of onset of the refractory MG group was 36 years with a range of 27-53 years. In our study 25 patients (32.89%) belonged to the age group of 21-30 years. Anti-MuSK antibodies were positive in 8 non-refractory MG patients (2.06%) and 36 refractory MG patients (47.36%). Mean HbA 1C was found to be 8.6±2.33. The dose of administered prednisone decreased by a mean of 59.7% ( p =3.3x10 -8 ) to 94.6% ( p =2.2x10 -14 ) after the third cycle of rituximab treatment. Conclusion: The refractory MG patients are most commonly female with an early age of onset, anti-MuSK antibodies, and thymomas. Refractory MG patients have higher prevalence and poor control (HbA 1C >8%) of diabetes mellitus and dyslipidemia probably due to increased steroid usage. Rituximab is very efficient in treatment of refractory MG with adverse effects being low.

RESULTS OF OUTPATIENT PROGRAM ON EFFECTIVE THERAPY OF REFRACTORY ARTERIAL HYPERTENSION

Directory of Open Access Journals (Sweden)

M. M. Batyushin

2015-12-01

Full Text Available Aim. To increase in efficacy of antihypertensive therapy in patients with refractory arterial hypertension (HT.Material and methods. Patients with refractory HT were revealed during first month of program. The causes of refractory HT were analyzed. Combined antihypertensive therapy was prescribed to reach target level of blood pressure (BP. This therapy lasted 24 weeks and included angiotensin converting enzyme (ACE inhibitor, thiazid diuretic (indapamide and dihydropyridine calcium antagonist (nifedipine XL.Results. 200 patients with refractory HT were revealed. True refractory HT took place in 59,9% of patients and pseudo refractory HT – in 40,1% of patients. Lack of diuretics or combined antihypertensive therapy were the main reason of insufficient BP control. Proposed 3-drugs therapy resulted in reduction of systolic BP from 190 to 132 Hg mm and diastolic BP from 104 to 81 Hg mm. Target level of BP was reached in 94% patients. There were no side effects which demanded to stop therapy.Conclusion. High incidence of pseudorefractory HT (40,1% is revealed. Significant prevalence of renal disturbances especially chronic interstitial inflammatory could be responsible for refractory HT development. Use of 3-drugs therapy (ACE inhibitor, indapamide and nifedipine XL provides effective control of BP in refractory and pseudorefractory HT.

RESULTS OF OUTPATIENT PROGRAM ON EFFECTIVE THERAPY OF REFRACTORY ARTERIAL HYPERTENSION

Directory of Open Access Journals (Sweden)

M. M. Batyushin

2007-01-01

Full Text Available Aim. To increase in efficacy of antihypertensive therapy in patients with refractory arterial hypertension (HT.Material and methods. Patients with refractory HT were revealed during first month of program. The causes of refractory HT were analyzed. Combined antihypertensive therapy was prescribed to reach target level of blood pressure (BP. This therapy lasted 24 weeks and included angiotensin converting enzyme (ACE inhibitor, thiazid diuretic (indapamide and dihydropyridine calcium antagonist (nifedipine XL.Results. 200 patients with refractory HT were revealed. True refractory HT took place in 59,9% of patients and pseudo refractory HT – in 40,1% of patients. Lack of diuretics or combined antihypertensive therapy were the main reason of insufficient BP control. Proposed 3-drugs therapy resulted in reduction of systolic BP from 190 to 132 Hg mm and diastolic BP from 104 to 81 Hg mm. Target level of BP was reached in 94% patients. There were no side effects which demanded to stop therapy.Conclusion. High incidence of pseudorefractory HT (40,1% is revealed. Significant prevalence of renal disturbances especially chronic interstitial inflammatory could be responsible for refractory HT development. Use of 3-drugs therapy (ACE inhibitor, indapamide and nifedipine XL provides effective control of BP in refractory and pseudorefractory HT.

Heterozygous M1V variant of ELA-2 gene mutation associated with G-CSF refractory severe congenital neutropenia.

Science.gov (United States)

Setty, Bhuvana A; Yeager, Nicholas D; Bajwa, Rajinder P

2011-09-01

Severe congenital neutropenia is an autosomal recessive disorder characterized by maturation arrest at the promyelocyte/myelocyte phase in the bone marrow, absolute neutrophil count ELA-2 have been described. We report the case of a premature male infant with congenital neutropenia, associated with multiple infections, refractory to treatment with granulocyte colony stimulating factor who subsequently underwent matched sibling donor stem-cell transplant. He was found to be heterozygous for the M1V variant of the ELA-2 gene that we postulate to be causative for his severe neutropenia Copyright © 2011 Wiley-Liss, Inc.

Corrosion assessment of refractory materials for high temperature waste vitrification

International Nuclear Information System (INIS)

Marra, J.C.; Congdon, J.W.; Kielpinski, A.L.

1995-01-01

A variety of vitrification technologies are being evaluated to immobilize radioactive and hazardous wastes following years of nuclear materials production throughout the Department of Energy (DOE) complex. The compositions and physical forms of these wastes are diverse ranging from inorganic sludges to organic liquids to heterogeneous debris. Melt and off-gas products can be very corrosive at the high temperatures required to melt many of these waste streams. Ensuring material durability is required to develop viable treatment processes. Corrosion testing of materials in some of the anticipated severe environments is an important aspect of the materials identification and selection process. Corrosion coupon tests on typical materials used in Joule heated melters were completed using glass compositions with high salt contents. The presence of chloride in the melts caused the most severe attack. In the metal alloys, oxidation was the predominant corrosion mechanism, while in the tested refractory material enhanced dissolution of the refractory into the glass was observed. Corrosion testing of numerous different refractory materials was performed in a plasma vitrification system using a surrogate heterogeneous debris waste. Extensive corrosion was observed in all tested materials

Refractories in the Al2O3-ZrO2-SiO2 system

International Nuclear Information System (INIS)

Banerjee, S.P.; Bhadra, A.K.; Sircar, N.R.

1978-01-01

The effect of addition of ZrO 2 in different proportions in the refractories of the Al 2 O 3 -SiO 2 system was studied. The investigation was confined to two broad ranges of compositions incorporating zirconia (15-30 percent and 80-85 percent) in the Al 2 O 3 -ZrO 2 -SiO 2 system. The overall attainment of properties is dependent upon the mode of fabrication and firing, and bears a relationship with the phase assemblages and the relative proportion thereof. Of the different characteristics, the trend of dissociation of zircon has been found to be specially significant vis-a-vis the temperature of firing and thermal shock resistance. Reassociation of the dissociated products has been ascribed to bring forth improved resistance to thermal spalling. The different products developed during this investigation are considered to be very promising which find useful applications in view of the properties attained by them. (auth.)

Bone marrow immunophenotyping by flow cytometry in refractory cytopenia of childhood

NARCIS (Netherlands)

A.M. Aalbers (Anna Maartje); M.M. van den Heuvel-Eibrink (Marry); I. Baumann (Irith); M.N. Dworzak (Michael); H. Hasle (Henrik); F. Locatelli (Franco); B. de Moerloose (Barbara); M. Schmugge; E. Mejstříková (Ester); M. Nováková (Michaela); M. Zecca (Marco); C.M. Zwaan (Christian Michel); J.G. te Marvelde (Jeroen); A.W. Langerak (Anton); J.J.M. van Dongen (Jacques); R. Pieters (Rob); C.M. Niemeyer (Charlotte); V.H.J. van der Velden (Vincent)

2015-01-01

textabstractRefractory cytopenia of childhood is the most common type of childhood myelodysplastic syndrome. Because the majority of children with refractory cytopenia have a normal karyotype and a hypocellular bone marrow, differentiating refractory cytopenia from the immune-mediated bone marrow

Granisetron transdermal system improves refractory nausea and vomiting in gastroparesis.

Science.gov (United States)

Simmons, Kellie; Parkman, Henry P

2014-06-01

Symptoms of gastroparesis include nausea and vomiting, which can markedly diminish quality of life. Nausea and vomiting can also make treatment with oral antiemetics problematic. Our aim was to determine whether treatment-resistant nausea and vomiting in patients with gastroparesis improve after granisetron transdermal patch (GTP) therapy. In an open-label pilot study, patients with gastroparesis and symptoms of nausea and vomiting refractory to conventional treatment were treated with GTP. After 2 weeks, patients were asked to assess their therapeutic response using the Clinical Patient Grading Assessment Scale (CPGAS; +7 = completely better; 0 = no change; -7 = very considerably worse). Responders were defined as CPGAS score >0, non-responders as ≤0. Patients (n = 36) were treated with GTP. Of these 36 patients, one patient discontinued treatment due to the GTP not adhering to the skin. Of the remaining 35 patients, 18 improved, 15 remained the same, and two worsened. The average CPGAS score was +1.8 ± 0.4 (SEM) (P < 0.05 vs 0). Of the 18 patients with improvement, the average CPGAS score was +3.7 ± 0.3 (SEM), corresponding to "somewhat" to "moderately better" improvement in nausea/vomiting. Side effects occurred in nine patients: four developed constipation, three patients had skin rash, and two reported headaches. GTP was moderately effective in reducing refractory symptoms of nausea and/or vomiting from gastroparesis in 50% of patients. Mild side effects were reported by 25% of patients. GTP may be an effective treatment for nausea and vomiting in gastroparesis, and further study is warranted.

Fluid Force-Induced Detachment Criteria for Nonmetallic Inclusions Adhered to a Refractory/Molten Steel Interface

Science.gov (United States)

Dieguez Salgado, Uxia; Weiß, Christian; Michelic, Susanne K.; Bernhard, Christian

2018-05-01

Since nonmetallic inclusions (NMIs) in steel cannot be completely avoided, a greater understanding of their development and evolution during the steelmaking process is required. In particular, this includes the adhesion of microinclusions to the refractory/steel interface in the flow control system between the tundish and the mold. This phenomenon, commonly referred to as clogging, causes losses in productivity and product quality. Inclusions transported from the bulk melt to the boundary layer may adhere to the refractory/steel interface due to formation of a fluid cavity. A detailed model was derived for the detachment of NMIs adhering to a nozzle wall and is based on the local hydrodynamic conditions combined with the specific interfacial properties in the system consisting of the inclusions, the refractories, and the steel. The model is evaluated for three different application-oriented cases. This study has been focused on providing a better understanding of fluid flow in the near-wall region in order to reduce clogging during steelmaking.

A new experimental system for study on adaptive mutations

Institute of Scientific and Technical Information of China (English)

Lü; Zhong; (

2001-01-01

［1］Luria, S. E., Delbrück, M., Mutation of bacteria from virus sensitivity to virus resistance, Genetics, 1943, 28: 491.［2］Lederberg, J., Lederberg, E. M., Replica plating and indirect selection of bacteria mutants, J. Bacteriol., 1952, 63: 399.［3］Carins, J., Overbaugh, J., Miller, S., The origin of mutants, Nature, 1988, 355: 142.［4］Foster, P. L., Adaptive mutation: the uses of adversity, Annu. Rev. Microbiol., 1993, 47: 467.［5］Hall, B. G., Adaptive mutagenesis: a process that generates almost exclusively beneficient mutations, Genetica, 1998, 102/103: 109.［6］Kasak, L., Horak, R., Kivisaar, M., Promotor-creating mutations in Psuedmonas putida: A model system for the study of mutation in starving bacteria, PNAS, 1997, 94: 3134.［7］Steele, D. F., Jinks-Robertson, An examination of adaptive reversion in Saccharomyces cerevisiae, Genetics, 1992, 132: 9.［8］Davis, R. W., Botstein, Roth, J. R., Advanced Bacterial Genetics--A Manual for Genetic Engineering, New York: Cold Spring Harbor Laboratory Press, 1980, 13.［9］Miller, J. H., Experiments in Molecular Genetics, New York: Cold Spring Harbor Laboratory Press, 1972, 352.［10］ Lea, D. E., Coulson, C. A., The distribution of the numbers of mutants in bacterial populations, J. Genetics, 1949, 49: 264.［11］ Hughes, K. T., Roth, J. R., Transitory cis complementation: a method for provided transposition function to defective transposons, Genetics, 1988, 119: 9.［12］ Sanderson, K. E., Roth J., Linkage map of Salmonella typhimurium, Microbiol. Rev., 1988, 52: 485.［13］ He, B., Shiau, A., Choi, K. Y., Genes of the E. coli pur region are negatively controlled by a repressor-operator interaction, J. Bacteriol., 1990, 172: 4555.［14］ Liu, B., Huang, Y., Wang, A. Q., Regulation of purine biosynthetic genes expression in Salmonella typhimurium (IV)--Oc mutation site of purG and its function analysis, Science in China, Ser. C, 1997, 40(3): 238.［15］ Tang, H., Qin, J. C., Wang, A. Q

Colectomy for refractory constipation

DEFF Research Database (Denmark)

Raahave, Dennis; Loud, Franck Bjørn; Christensen, Elsebeth

2010-01-01

OBJECTIVE: This study evaluated the type of colectomy, postoperative complications, functional results, and satisfaction in patients with constipation refractory to conservative therapy. Further, colonic transit time (CTT), faecal load (coprostasis), and colon length (redundancies) were compared ...

Statistical experimental design for refractory coatings

International Nuclear Information System (INIS)

McKinnon, J.A.; Standard, O.C.

2000-01-01

The production of refractory coatings on metal casting moulds is critically dependent on the development of suitable rheological characteristics, such as viscosity and thixotropy, in the initial coating slurry. In this paper, the basic concepts of mixture design and analysis are applied to the formulation of a refractory coating, with illustration by a worked example. Experimental data of coating viscosity versus composition are fitted to a statistical model to obtain a reliable method of predicting the optimal formulation of the coating. Copyright (2000) The Australian Ceramic Society

Typical corrosion of alumina refractory in aluminum reflow oven

International Nuclear Information System (INIS)

Baldo, Jaoa B.

2014-01-01

The refractory linings of furnaces for secondary melting of aluminum, are exposed to intense attack by the molten metal. This occurs, because molten aluminum has a strong reducing power over the refractory oxide components, particularly Fe 2 O 3 , SiO 2 and TiO 2 . In this work, based on X-ray diffraction and scanning electron microscopy, it is presented a post mortem study of the mechanisms that lead to a premature wear of a 80% Al2O3 chemically bonded refractory bricks, used in the metal line of an aluminum re-melting furnace. The SEM analysis demonstrated that the oxides SiO 2 and TiO 2 contained in the refractory were reduced and transformed into their metallic elements causing an intense structural spalling. (author)

Neonatal Marfan Syndrome: Report of a Case with an Inherited Splicing Mutation outside the Neonatal Domain.

Science.gov (United States)

Le Gloan, Laurianne; Hauet, Quentin; David, Albert; Hanna, Nadine; Arfeuille, Chloé; Arnaud, Pauline; Boileau, Catherine; Romefort, Bénédicte; Benbrik, Nadir; Gournay, Véronique; Joram, Nicolas; Baron, Olivier; Isidor, Bertrand

2016-02-01

We report a child and her mother affected by Marfan syndrome. The child presented with a phenotype of neonatal Marfan syndrome, revealed by acute and refractory heart failure, finally leading to death within the first 4 months of life. Her mother had a common clinical presentation. Genetic analysis revealed an inherited FBN1 mutation. This intronic mutation (c.6163+3_6163+6del), undescribed to date, leads to exon 49 skipping, corresponding to in-frame deletion of 42 amino acids (p.Ile2014_Asp2055del). FBN1 next-generation sequencing did not show any argument for mosaicism. Association in the same family of severe neonatal and classical Marfan syndrome illustrates the intrafamilial phenotype variability.

Beta-blockers in cirrhosis and refractory ascites

DEFF Research Database (Denmark)

Kimer, Nina; Feineis, Martin; Møller, Søren

2015-01-01

OBJECTIVE: It is currently discussed if beta-blockers exert harmful effects and increase mortality in patients with cirrhosis and refractory ascites. In this study, we provide an overview of the available literature in this field in combination with a retrospective analysis of 61 patients...... trials (9 trials on propranolol, 1 case-control study and 4 retrospective analyses) were identified. One trial suggested an increased mortality in patients treated with beta-blockers and refractory ascites. The results of the remaining trials were inconclusive. No increase in mortality among beta-blocker......-treated patients was found in the present retrospective analysis. CONCLUSIONS: Treatment with beta-blockers may increase mortality in patients with cirrhosis and refractory ascites. However, the current evidence is sparse and high-quality studies are warranted to clarify the matter....

Identification of a Non-Gatekeeper Hot Spot for Drug-Resistant Mutations in mTOR Kinase.

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Wu, Tzung-Ju; Wang, Xiaowen; Zhang, Yanjie; Meng, Linghua; Kerrigan, John E; Burley, Stephen K; Zheng, X F Steven

2015-04-21

Protein kinases are therapeutic targets for human cancer. However, "gatekeeper" mutations in tyrosine kinases cause acquired clinical resistance, limiting long-term treatment benefits. mTOR is a key cancer driver and drug target. Numerous small-molecule mTOR kinase inhibitors have been developed, with some already in human clinical trials. Given our clinical experience with targeted therapeutics, acquired drug resistance in mTOR is thought likely, but not yet documented. Herein, we describe identification of a hot spot (L2185) for drug-resistant mutations, which is distinct from the gatekeeper site, and a chemical scaffold refractory to drug-resistant mutations. We also provide new insights into mTOR kinase structure and function. The hot spot mutations are potentially useful as surrogate biomarkers for acquired drug resistance in ongoing clinical trials and future treatments and for the design of the next generation of mTOR-targeted drugs. Our study provides a foundation for further research into mTOR kinase function and targeting. Copyright © 2015 The Authors. Published by Elsevier Inc. All rights reserved.

Halo Formation During Solidification of Refractory Metal Aluminide Ternary Systems

Science.gov (United States)

D'Souza, N.; Feitosa, L. M.; West, G. D.; Dong, H. B.

2018-02-01

The evolution of eutectic morphologies following primary solidification has been studied in the refractory metal aluminide (Ta-Al-Fe, Nb-Al-Co, and Nb-Al-Fe) ternary systems. The undercooling accompanying solid growth, as related to the extended solute solubility in the primary and secondary phases can be used to account for the evolution of phase morphologies during ternary eutectic solidification. For small undercooling, the conditions of interfacial equilibrium remain valid, while in the case of significant undercooling when nucleation constraints occur, there is a departure from equilibrium leading to unexpected phases. In Ta-Al-Fe, an extended solubility of Fe in σ was observed, which was consistent with the formation of a halo of μ phase on primary σ. In Nb-Al-Co, a halo of C14 is formed on primary CoAl, but very limited vice versa. However, in the absence of a solidus projection it was not possible to definitively determine the extended solute solubility in the primary phase. In Nb-Al-Fe when nucleation constraints arise, the inability to initiate coupled growth of NbAl3 + C14 leads to the occurrence of a two-phase halo of C14 + Nb2Al, indicating a large undercooling and departure from equilibrium.

Fire and explosion hazards of refractory compound powders

International Nuclear Information System (INIS)

Krivtsov, V.A.; Kostina, E.S.

1978-01-01

Data on fire and explosion hazards of refractory compound powders (HfC, ZrC,LaB 6 , ZrN, etc.) are presented. It is shown that refractory compounds can be fire- and exposion hazardous in various degrees. Qualitative and quantitative estimations of one of fire-hazard characteristics - smoldering temperature - are presented

Monitoring and advisory system for refractory materials fireing production in VSŽ Košice

Directory of Open Access Journals (Sweden)

Kostúr Karol

1996-03-01

Full Text Available The tunnel furnace produces refractory building materials. Various types of building materials are fired in the temperature interval 1450-1700 •C. The tunnel furnace is approximately 160 m long and consists of 53 moduls, each about length 3 m. Usually three zones of the tunnel furnace are considering: warming, firing and colding. The furnace works in upstream regime. The fired material moves againts the flow of cold air and combustion products. The fuel is the earth gas. The paper is devoted to pointing out some opportunities for the use of classical IBM PC compatible computers for the design of small on-line real-time systems. PC’ s data acquisition card provides high transfer rate for data transfer and primary processing of measured values of technological processes in a tunnel furnace.

Regional and ethnic distribution of beta thalassemia mutations and effect of consanguinity in patients referred for prenatal diagnosis

International Nuclear Information System (INIS)

Hafeez, M.

2007-01-01

To determine the regional and ethnic distribution of beta thalassemia mutation and the effect of consanguinity in patients referred for prenatal diagnosis of beta b-thalassemia and to target the high risk population for screening. A total of 499 couples were referred to Gentec Lab., Lahore, from all over Pakistan for prenatal diagnosis of b-thalassemia. After counseling, chorionic villus sampling was done between 10-16 weeks of gestation. DNA analysis was done by Amplification Refractory Mutation System (ARMS) for type of mutation in the Armed Forces Institute of Pathology, Rawalpindi. Ethnicity, race and consanguineous relationship of parents was determined.b-thalassemia was prevalent in Punjabis (60.7%) followed by Saraikees (25.5%). Castewise it was most frequent in Rajputs followed by Jatts, Arain, Sheikhs and Pathans. 56.7% of the couples were first cousins and 19.8% were relatives. The commonest mutations were Frameshift 8-9 (Fr8-9) 33.5%, Intervening Sequence 1-5 (IVS 1-5) 17.2%, Fr4142 - 8%, IVS 1-1 - 5.2%, Deletion 619 (Del 619) 4.2% and Codon 5 (Cd 5) - 4.2%. In samples sent for analysis, 53.1% turned out to be carriers (trait), 25.3% were diseased (thalassemia major) and 21.6% were normal. P-value of all results was less than 0.001. In this series, the highest frequency was found in Punjabi Rajputs. The commonest mutation was Fr 8-9. Most parents were first cousins. Premarital thalassemia carrier testing can effectively reduce the disease. (author)

Immunomodulatory therapy in refractory/recurrent ovarian cancer.

Science.gov (United States)

Chen, Chao-Yu; Lai, Chyong-Huey; Yang, Lan-Yan; Tang, Yun-Hsin; Chou, Hung-Hsueh; Chang, Chee-Jen; Lin, Cheng-Tao

2015-04-01

To investigate the efficacy and toxicity of immunomodulatory therapy (IMT) alone or as an add-on to palliative/salvage chemotherapy in patients with refractory/recurrent epithelial ovarian cancer (EOC). We retrospectively analyzed the efficacy and toxicity of IMT in 15 patients with refractory/recurrent EOC who had previously received multiple chemotherapy regimens. The median age of the patients was 56 years (range, 41-75 years). Three patients were platinum-sensitive, two were platinum-resistant, and the remaining 10 patients were refractory to platinum-based front-line chemotherapy. IMT consisted of picibanil (OK-432) on Day 1, interleukin-2 and/or interferon-α on Day 2 administered by subcutaneous injection (every week or 2-weekly). Five patients never received metronomic oral cyclophosphamide. After IMT, three patients achieved partial remission (PR, lasting for 11 months, ≥ 12 months, and 16 months), and six patients had stable disease (SD). The disease stabilizing rate (PR+SD) was 60% (3/3 in platinum-sensitive and 6/12 in platinum-resistant/refractory patients). The absolute lymphocyte count (ALC) at 1 month after IMT was significantly higher in the PR+SD group (median 1242.0/μL) than in the progression group (median 325.0/μL) (p = 0.012). No ≥ Grade 3 toxicities were observed. The median post-IMT survival time was 12 months (range, 2-39 months). IMT alone or add-on to palliative/salvage chemotherapy for refractory/recurrent EOC achieves a substantial disease stabilizing rate without severe toxicity, which might be a potential option in selected patients. The ALC 1 month after IMT could be an early indicator to disease stabilization. Copyright © 2015. Published by Elsevier B.V.

Key clinical features to identify girls with CDKL5 mutations.

Science.gov (United States)

Bahi-Buisson, Nadia; Nectoux, Juliette; Rosas-Vargas, Haydeé; Milh, Mathieu; Boddaert, Nathalie; Girard, Benoit; Cances, Claude; Ville, Dorothée; Afenjar, Alexandra; Rio, Marlène; Héron, Delphine; N'guyen Morel, Marie Ange; Arzimanoglou, Alexis; Philippe, Christophe; Jonveaux, Philippe; Chelly, Jamel; Bienvenu, Thierry

2008-10-01

Mutations in the human X-linked cyclin-dependent kinase-like 5 (CDKL5) gene have been shown to cause infantile spasms as well as Rett syndrome (RTT)-like phenotype. To date, less than 25 different mutations have been reported. So far, there are still little data on the key clinical diagnosis criteria and on the natural history of CDKL5-associated encephalopathy. We screened the entire coding region of CDKL5 for mutations in 183 females with encephalopathy with early seizures by denaturing high liquid performance chromatography and direct sequencing, and we identified in 20 unrelated girls, 18 different mutations including 7 novel mutations. These mutations were identified in eight patients with encephalopathy with RTT-like features, five with infantile spasms and seven with encephalopathy with refractory epilepsy. Early epilepsy with normal interictal EEG and severe hypotonia are the key clinical features in identifying patients likely to have CDKL5 mutations. Our study also indicates that these patients clearly exhibit some RTT features such as deceleration of head growth, stereotypies and hand apraxia and that these RTT features become more evident in older and ambulatory patients. However, some RTT signs are clearly absent such as the so called RTT disease profile (period of nearly normal development followed by regression with loss of acquired fine finger skill in early childhood and characteristic intensive eye communication) and the characteristic evolution of the RTT electroencephalogram. Interestingly, in addition to the overall stereotypical symptomatology (age of onset and evolution of the disease) resulting from CDKL5 mutations, atypical forms of CDKL5-related conditions have also been observed. Our data suggest that phenotypic heterogeneity does not correlate with the nature or the position of the mutations or with the pattern of X-chromosome inactivation, but most probably with the functional transcriptional and/or translational consequences of CDKL5

Clinical implications of somatic mutations in aplastic anemia and myelodysplastic syndrome in genomic age.

Science.gov (United States)

Maciejewski, Jaroslaw P; Balasubramanian, Suresh K

2017-12-08

Recent technological advances in genomics have led to the discovery of new somatic mutations and have brought deeper insights into clonal diversity. This discovery has changed not only the understanding of disease mechanisms but also the diagnostics and clinical management of bone marrow failure. The clinical applications of genomics include enhancement of current prognostic schemas, prediction of sensitivity or refractoriness to treatments, and conceptualization and selective application of targeted therapies. However, beyond these traditional clinical aspects, complex hierarchical clonal architecture has been uncovered and linked to the current concepts of leukemogenesis and stem cell biology. Detection of clonal mutations, otherwise typical of myelodysplastic syndrome, in the course of aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria has led to new pathogenic concepts in these conditions and created a new link between AA and its clonal complications, such as post-AA and paroxysmal nocturnal hemoglobinuria. Distinctions among founder vs subclonal mutations, types of clonal evolution (linear or branching), and biological features of individual mutations (sweeping, persistent, or vanishing) will allow for better predictions of the biologic impact they impart in individual cases. As clonal markers, mutations can be used for monitoring clonal dynamics of the stem cell compartment during physiologic aging, disease processes, and leukemic evolution. © 2016 by The American Society of Hematology. All rights reserved.

[Therapy-resistant and therapy-refractory arterial hypertension].

Science.gov (United States)

Wallbach, M; Koziolek, M J

2018-05-02

Therapy-resistant and therapy-refractory arterial hypertension differ in prevalence, pathogenesis, prognosis and therapy. In both cases, a structured approach is required, with the exclusion of pseudoresistance and, subsequently, secondary hypertension. Resistant hypertension has been reported to be more responsive to intensified diuretic therapy, whereas refractory hypertension is presumed to require sympathoinhibitory therapy. Once the general measures and the drug-based step-up therapy have been exhausted, interventional procedures are available.

Bulk Vitrification Castable Refractory Block Protection Study

Energy Technology Data Exchange (ETDEWEB)

Hrma, Pavel R.; Bagaasen, Larry M.; Beck, Andrew E.; Brouns, Thomas M.; Caldwell, Dustin D.; Elliott, Michael L.; Matyas, Josef; Minister, Kevin BC; Schweiger, Michael J.; Strachan, Denis M.; Tinsley, Bronnie P.; Hollenberg, Glenn W.

2005-05-01

Bulk vitrification (BV) was selected for a pilot-scale test and demonstration facility for supplemental treatment to accelerate the cleanup of low-activity waste (LAW) at the Hanford U.S. DOE Site. During engineering-scale (ES) tests, a small fraction of radioactive Tc (and Re, its nonradioactive surrogate) were transferred out of the LAW glass feed and molten LAW glass, and deposited on the surface and within the pores of the castable refractory block (CRB). Laboratory experiments were undertaken to understand the mechanisms of the transport Tc/Re into the CRB during vitrification and to evaluate various means of CRB protection against the deposition of leachable Tc/Re. The tests used Re as a chemical surrogate for Tc. The tests with the baseline CRB showed that the molten LAW penetrates into CRB pores before it converts to glass, leaving deposits of sulfates and chlorides when the nitrate components decompose. Na2O from the LAW reacts with the CRB to create a durable glass phase that may contain Tc/Re. Limited data from a single CRB sample taken from an ES experiment indicate that, while a fraction of Tc/Re is present in the CRB in a readily leachable form, most of the Tc/Re deposited in the refractory is retained in the form of a durable glass phase. In addition, the molten salts from the LAW, mainly sulfates, chlorides, and nitrates, begin to evaporate from BV feeds at temperatures below 800 C and condense on solid surfaces at temperatures below 530 C. Three approaches aimed at reducing or preventing the deposition of soluble Tc/Re within the CRB were proposed: metal lining, sealing the CRB surface with a glaze, and lining the CRB with ceramic tiles. Metal liners were deemed unsuitable because evaluations showed that they can cause unacceptable distortions of the electric field in the BV system. Sodium silicate and a low-alkali borosilicate glaze were selected for testing. The glazes slowed down molten salt condensate penetration, but did little to reduce the

Refractory myasthenia gravis – clinical profile, comorbidities and response to rituximab

Science.gov (United States)

Sudulagunta, Sreenivasa Rao; Sepehrar, Mona; Sodalagunta, Mahesh Babu; Settikere Nataraju, Aravinda; Bangalore Raja, Shiva Kumar; Sathyanarayana, Deepak; Gummadi, Siddharth; Burra, Hemanth Kumar

2016-01-01

Introduction: Myasthenia gravis (MG) is an antibody mediated autoimmune neuromuscular disorder characterized by fatigable muscle weakness. A proportion of myasthenia gravis patients are classified as refractory due to non responsiveness to conventional treatment. This retrospective study was done to evaluate clinical profile, epidemiological, laboratory, and features of patients with MG and mode of management using rituximab and complications. Methods: Data of myasthenia gravis patients admitted or presented to outpatient department (previous medical records) with MG between January 2008 and January 2016 were included. A total of 512 patients fulfilled the clinical and diagnostic criteria of myasthenia gravis of which 76 patients met the diagnostic certainty for refractory myasthenia gravis and were evaluated. Results: Out of 76 refractory MG patients, 53 (69.73%) patients fulfilled all the three defined criteria. The median age of onset of the refractory MG group was 36 years with a range of 27–53 years. In our study 25 patients (32.89%) belonged to the age group of 21–30 years. Anti-MuSK antibodies were positive in 8 non-refractory MG patients (2.06%) and 36 refractory MG patients (47.36%). Mean HbA1C was found to be 8.6±2.33. The dose of administered prednisone decreased by a mean of 59.7% (p=3.3x10–8) to 94.6% (p=2.2x10–14) after the third cycle of rituximab treatment. Conclusion: The refractory MG patients are most commonly female with an early age of onset, anti-MuSK antibodies, and thymomas. Refractory MG patients have higher prevalence and poor control (HbA1C >8%) of diabetes mellitus and dyslipidemia probably due to increased steroid usage. Rituximab is very efficient in treatment of refractory MG with adverse effects being low. PMID:27790079

IMPROVED CORROSION RESISTANCE OF ALUMINA REFRACTORIES

Energy Technology Data Exchange (ETDEWEB)

John P. Hurley; Patty L. Kleven

2001-09-30

The initial objective of this project was to do a literature search to define the problems of refractory selection in the metals and glass industries. The problems fall into three categories: Economic--What do the major problems cost the industries financially? Operational--How do the major problems affect production efficiency and impact the environment? and Scientific--What are the chemical and physical mechanisms that cause the problems to occur? This report presents a summary of these problems. It was used to determine the areas in which the EERC can provide the most assistance through bench-scale and laboratory testing. The final objective of this project was to design and build a bench-scale high-temperature controlled atmosphere dynamic corrosion application furnace (CADCAF). The furnace will be used to evaluate refractory test samples in the presence of flowing corrodents for extended periods, to temperatures of 1600 C under controlled atmospheres. Corrodents will include molten slag, steel, and glass. This test should prove useful for the glass and steel industries when faced with the decision of choosing the best refractory for flowing corrodent conditions.

Molecular Epidemiology of EGFR Mutations in Asian Patients with Advanced Non-Small-Cell Lung Cancer of Adenocarcinoma Histology – Mainland China Subset Analysis of the PIONEER study

Science.gov (United States)

Shi, Yuankai; Li, Junling; Zhang, Shucai; Wang, Mengzhao; Yang, Shujun; Li, Ning; Wu, Gang; Liu, Wei; Liao, Guoqing; Cai, Kaican; Chen, Liang’an; Zheng, Meizhen; Yu, Ping; Wang, Xiuwen; Liu, Yunpeng; Guo, Qisen; Nie, Ligong; Liu, Jiwei; Han, Xiaohong

2015-01-01

Epidermal growth factor receptor (EGFR) mutations are the strongest response predictors to EGFR tyrosine kinase inhibitors (TKI) therapy, but knowledge of the EGFR mutation frequency on lung adenocarcinoma is still limited to retrospective studies. The PIONEER study (NCT01185314) is a prospective molecular epidemiology study in Asian patients with newly diagnosed advanced lung adenocarcinoma, aiming to prospectively analyze EGFR mutation status in IIIB/IV treatment-naïve lung adenocarcinomas in Asia. We report the mainland China subset results. Eligible patients (≥20 yrs old, IIIB/IV adenocarcinoma and treatment-naïve) were registered in 17 hospitals in mainland China. EGFR was tested for mutations by amplification refractory mutation system using biopsy samples. Demographic and clinical characteristics were collected for subgroup analyses. A total of 747 patients were registered. Successful EGFR mutation analysis was performed in 741, with an overall mutation rate of 50.2%. The EGFR active mutation rate is 48.0% (with 1.3% of combined active and resistance mutations). Tobacco use (>30 pack-year vs. 0–10 pack-year, OR 0.27, 95%CI: 0.17–0.42) and regional lymph nodes involvement (N3 vs. N0, OR 0.47, 95%CI: 0.29–0.76) were independent predictors of EGFR mutation in multivariate analysis. However, even in regular smokers, the EGFR mutation frequency was 35.3%. The EGFR mutation frequency was similar between diverse biopsy sites and techniques. The overall EGFR mutation frequency of the mainland China subset was 50.2%, independently associated with the intensity of tobacco use and regional lymph nodes involvement. The relatively high frequency of EGFR mutations in the mainland China subset suggest that any effort to obtain tissue sample for EGFR mutation testing should be encouraged. PMID:26599344

Refractory castables Al2O3-MgO advanced boron-containing

International Nuclear Information System (INIS)

Morbioli, G.G.; Braulio, M.A.L.; Pandolfelli, V.C.

2011-01-01

Alumina-magnesia refractory castables present high corrosion resistance mainly due to the in situ spinel (MgAl 2 O 4 ) formation. For the steel industry, the earlier its generation the best would be the castable's working performance. Taking this aspect into account, alternatives to replace silica fume, which is the most traditional liquid former inducer in this system, were evaluated in this study, by adding boron-containing compounds. Faster spinel and calcium hexaaluminate (CA 6 ) formation was attained through a transient liquid, ensuring remarkable castable's creep resistance and hot modulus of rupture. In this context, a novel route to design in situ spinel forming castable is presented, with great potential to increase the performance of this class of refractory. (author)

Thermodynamics of carbon deoxidation and aluminium deoxidation of refractory metals

International Nuclear Information System (INIS)

Garg, S.P.; Sundaram, C.V.

1975-01-01

Carbon has been used for removing the last traces of oxygen from many refractory metals by evaporation of CO at high temperature and vacuum. Similar purification can also be achieved employing aluminium deoxidation by evaporation of Al 2 Osub(g). In the present paper a theoretical thermodynamic approach has been attempted to evaluate the deoxidation tendencies of refractory metals of groups IV to VI by these two processes. Expressions have been theoretically derived, relating the concentration product of carbon and oxygen in the metal (which is a measure of the carbon deoxidation tendency of the metal) with temperature and pressure, for various M-C-O systems. Similarly the relative vapour pressure values of Alsub(2)Osub(g),Alsub(g)andMO(g) (suboxide of the metal) over various M-Al-O systems have been calculated as a function of aluminium and oxygen contents of the metal. From these analyses, it has been shown that a substantial amount of oxygen can be removed from M-Al-O alloys by aluminium deoxidation. The estimated values are compared with the reported values based on experiment. (author)

Unique TTC repeat base pair loss mutation in cases of pure neural leprosy: A survival strategy of Mycobacterium leprae?

Directory of Open Access Journals (Sweden)

Abhishek De

2015-01-01

Full Text Available Background: Genomic reduction helps obligate intracellular microbes to survive difficult host niches. Adaptation of Mycobacterium leprae in cases of pure neural leprosy (PNL in the intracellular niche of peripheral nerves can be associated with some gene loss. Recently, a stable but variable number of tandem repefzats (TTC have been reported in strains of M. leprae. FolP and rpoB genes are the two common mutation sites which deal with the susceptibility of the bacteria to drugs. Aim: We attempted to find if genomic reduction of M. leprae in context of these TTC repeats or mutations in folP1 and rpoB can be the reason for the restriction of M. leprae in the nerves in PNL. Materials and Methods: DNA extracts taken from fine needle aspiration of affected nerves of 24 PNL cases were studied for tandem repeats with 21TTC primer in multiplex-PCR. Mutations were also studied by PCR Amplification of SRDR (Sulphone Resistance Determining Region of the folP1 and multiple primer PCR amplification refractory mutation system (MARS of the rpoB. Results: Of the 24 PNL, only 1 patient showed mutation in the rpoB gene and none in the folp1 gene. Studying the mutation in TTC region of the M. leprae gene we found that all the cases have a loss of a few bases in the sequence. Conclusion: We can conclude that there is consistent loss in the bases in the TTC region in all cases of pure neural Hansen and we postulate that it may be an adaptive response of the bacteria to survive host niche resulting in its restriction to peripheral nerves.

Defining refractory migraine: results of the RHSIS Survey of American Headache Society members.

Science.gov (United States)

Schulman, Elliott A; Peterlin, B Lee; Lake, Alvin E; Lipton, Richard B; Hanlon, Alexandra; Siegel, Sherry; Levin, Morris; Goadsby, Peter J; Markley, Herbert G

2009-04-01

To gauge consensus regarding a proposed definition for refractory migraine proposed by Refractory Headache Special Interest Section, and where its use would be most appropriate. Headache experts have long recognized that a subgroup of headache sufferers remains refractory to treatment. Although different groups have proposed criteria to define refractory migraine, the definition remains controversial. The Refractory Headache Special Interest Section of the American Headache Society developed a definition through a consensus process, assisted by a literature review and initial membership survey. A 12-item questionnaire was distributed at the American Headache Society meeting in 2007 during a platform session and at the Refractory Headache Special Interest Section symposium. The same questionnaire was subsequently sent to all American Headache Society members via e-mail. A total of 151 responses from AHS members form the basis of this report. The survey instrument was designed using Survey Monkey. Frequencies and percentages of the survey were used to describe survey responses. American Headache Society members agreed that a definition for refractory migraine is needed (91%) that it should be added to the International Classification of Headache Disorders-2 (86%), and that refractory forms of non-migraine headache disorders should be defined (87%). Responders believed a refractory migraine definition would be of greatest value in selecting patients for clinical drug trials. The current refractory migraine definition requires a diagnosis of migraine, interference with function or quality of life despite modification of lifestyle factors, and adequate trials of acute and preventive medicines with established efficacy. The proposed criteria for the refractory migraine definition require failing 2 preventive medications to meet the threshold for failure. Although 42% of respondents agreed with the working definition of refractory migraine, 43% favored increasing the

The Role of Refractory Dissolved Organic Matter in Ocean Carbon Sequestration

DEFF Research Database (Denmark)

Jørgensen, Linda

The ocean assimilates a large amount of atmospheric CO2 and is potentially a buffer for climate change. A fraction of the assimilated CO2 is incorporated into algal biomass and further converted into refractory dissolved organic matter (DOM). Carbon bound in refractory DOM has the potential...... studies the prokaryotic production and degradation of oceanic refractory DOM and discusses the reasons for the persistent nature of this large DOM fraction. The first two papers investigate the microbial carbon pump, i.e. prokaryotic transfor-mation of organic carbon into refractory DOM. The results show...... DOM compounds in the ocean are rare—possibly too rare to sustain viable uptake and assimilation. Hence, the dilute concentration of individual compounds is a possible explanation for the apparent refractory nature of most DOM in the ocean. Understanding the mechanisms that control the quality...

Intraepithelial lymphocytes in refractory celiac disease : lost in transition

NARCIS (Netherlands)

Schmitz, Frederike

2014-01-01

Refractory coeliac disease type II (RCDII) is a severe complication of coeliac disease. Whereas celiac disease can successfully be treated by the strict avoidance of gluten, refractory celiac patients show no remission despite a gluten-free diet. The pathology of RCDII is only partially understood,

Allogeneic Transplant in ELANE and MEFV Mutation Positive Severe Cyclic Neutropenia: Review of Prognostic Factors for Secondary Severe Events

Directory of Open Access Journals (Sweden)

Onyemaechi N. Okolo

2017-01-01

Full Text Available Objective and Importance. Cyclic neutropenia (CyN is a rare autosomal dominant inherited disorder due to the mutation ELANE primarily affecting bone marrow stem cells and is characterized by recurrent neutropenia every 2 to 4 weeks. Symptoms vary from benign to severe, including death. Postulations on the cause of wide spectrum in symptom presentation include the possibility of other genetic mutations, such as MEFV. Recommended treatment for CyN is G-CSF to keep ANC higher to minimize risk of infection. Case. A 25-year-old male diagnosed with CyN, on G-CSF but worsening quality of life. Pretransplant investigations revealed ELANE mutation positive severe CyN along with familial Mediterranean fever (MEFV mutation. Intervention. Bone marrow transplantation as treatment for dual mutation (ELANE and MEFV mutation positive severe CyN. Conclusion. BMT may be considered as an alternative treatment for severe CyN in patients who are refractory to G-CSF. It is postulated that in our patient the combined mutations (CyN and MEFV may have contributed to the severity of this individual’s symptoms. We suggest CyN patients who present with severe symptoms have evaluation with ELANE mutation testing, Periodic Fever Syndromes Panel, and routine marrow assessment with FISH, conventional cytogenetics, and morphological evaluation for MDS/AML.

Clinical Features of Refractory Ascites in Outpatients

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Wanda Regina Caly

Full Text Available OBJECTIVES: To present the clinical features and outcomes of outpatients who suffer from refractory ascites. METHODS: This prospective observational study consecutively enrolled patients with cirrhotic ascites who submitted to a clinical evaluation, a sodium restriction diet, biochemical blood tests, 24 hour urine tests and an ascitic fluid analysis. All patients received a multidisciplinary evaluation and diuretic treatment. Patients who did not respond to the diuretic treatment were controlled by therapeutic serial paracentesis, and a transjugular intrahepatic portosystemic shunt was indicated for patients who required therapeutic serial paracentesis up to twice a month. RESULTS: The most common etiology of cirrhosis in both groups was alcoholism [49 refractory (R and 11 non-refractory ascites (NR]. The majority of patients in the refractory group had Child-Pugh class B cirrhosis (p=0.034. The nutritional assessment showed protein-energy malnutrition in 81.6% of the patients in the R group and 35.5% of the patients in the NR group, while hepatic encephalopathy, hernia, spontaneous bacterial peritonitis, upper digestive hemorrhage and type 2 hepatorenal syndrome were present in 51%, 44.9%, 38.8%, 38.8% and 26.5% of the patients in the R group and 9.1%, 18.2%, 0%, 0% and 0% of the patients in the NR group, respectively (p=0.016, p=0.173, p=0.012, p=0.012, and p=0.100, respectively. Mortality occurred in 28.6% of the patients in the R group and in 9.1% of the patients in the NR group (p=0.262. CONCLUSION: Patients with refractory ascites were malnourished, suffered from hernias, had a high prevalence of complications and had a high postoperative death frequency, which was mostly due to infectious processes.

Resistant and Refractory Hypertension: Antihypertensive Treatment Resistance vs Treatment Failure

Science.gov (United States)

Siddiqui, Mohammed; Dudenbostel, Tanja; Calhoun, David A.

2017-01-01

Resistant or difficult to treat hypertension is defined as high blood pressure that remains uncontrolled with 3 or more different antihypertensive medications, including a diuretic. Recent definitions also include controlled blood pressure with use of 4 or more medications as also being resistant to treatment. Recently, refractory hypertension, an extreme phenotype of antihypertensive treatment failure has been defined as hypertension uncontrolled with use of 5 or more antihypertensive agents, including a long-acting thiazide diuretic and a mineralocorticoid receptor antagonist. Patients with resistant vs refractory hypertension share similar characteristics and comorbidities, including obesity, African American race, female sex, diabetes, coronary heart disease, chronic kidney disease, and obstructive sleep apnea. Patients with refractory vs resistant hypertension tend to be younger and are more likely to have been diagnosed with congestive heart failure. Refractory hypertension might also differ from resistant hypertension in terms of underlying cause. Preliminary evidence suggests that refractory hypertension is more likely to be neurogenic in etiology (ie, heightened sympathetic tone), vs a volume-dependent hypertension that is more characteristic of resistant hypertension in general. PMID:26514749

Performance assessment of refractory samples in the Los Alamos Controlled Air Incinerator

International Nuclear Information System (INIS)

Hutchins, D.A.; Borduin, L.C.; Koenig, R.A.; Vavruska, J.S.; Warner, C.L.

1986-01-01

A refractory evaluation project was initiated in 1979 to study the performance of six selected refractory materials within the Los Alamos Controlled Air Incinerator (CAI). Determining refractory resistance to thermal shock, chemical attack, and plutonium uptake was of particular interest. The experimental refractories were subjected to a variety of waste materials, including transuranic (TRU) contaminated wastes, highly chlorinated compounds and alkaline metal salts of perchlorate, chlorate, nitrate and oxylate, over the six year period of this study. Results of this study to date indicate that the use of high alumina, and possibly specialty plastic refractories, is advisable for the lining of incinerators used for the thermal destruction of diverse chemical compounds. 12 refs., 4 tabs

White-Coat Effect Is Uncommon in Patients With Refractory Hypertension.

Science.gov (United States)

Siddiqui, Mohammed; Judd, Eric K; Oparil, Suzanne; Calhoun, David A

2017-09-01

Refractory hypertension is a recently described phenotype of antihypertensive treatment failure defined as uncontrolled blood pressure (BP) despite the use of ≥5 different antihypertensive agents, including chlorthalidone and spironolactone. Recent studies indicate that refractory hypertension is uncommon, with a prevalence of ≈5% to 10% of patients referred to a hypertension specialty clinic for uncontrolled hypertension. The prevalence of white-coat effect, that is, uncontrolled automated office BP ≥135/85 mm Hg and controlled out-of-office BP hypertensive patients overall is ≈30% to 40%. The prevalence of white-coat effect among patients with refractory hypertension has not been previously reported. In this prospective evaluation, consecutive patients referred to the University of Alabama at Birmingham Hypertension Clinic for uncontrolled hypertension were enrolled. Refractory hypertension was defined as uncontrolled automated office BP ≥135/85 mm Hg with the use of ≥5 antihypertensive agents, including chlorthalidone and spironolactone. Automated office BP measurements were based on 6 serial readings, done automatically with the use of a BpTRU device unobserved in the clinic. Out-of-office BP measurements were done by 24-hour ambulatory BP monitor. Thirty-four patients were diagnosed with refractory hypertension, of whom 31 had adequate ambulatory BP monitor readings. White-coat effect was present in only 2 patients, or 6.5% of the 31 patients with refractory hypertension, suggesting that white-coat effect is largely absent in patients with refractory hypertension. These findings suggest that white-coat effect is not a common cause of apparent lack of BP control in patients failing maximal antihypertensive treatment. © 2017 American Heart Association, Inc.

Distributed Fiber Optic Sensor for On-Line Monitoring of Coal Gasifier Refractory Health

Energy Technology Data Exchange (ETDEWEB)

Wang, Anbo [Center for Photonics Technology, Blacksburgh, VA (United States); Yu, Zhihao [Center for Photonics Technology, Blacksburgh, VA (United States)

2015-11-30

This report summarizes technical progress on the program “Distributed Fiber Optic Sensor for On-Line Monitoring of Coal Gasifier Refractory Health,” funded by the National Energy Technology Laboratory of the U.S. Department of Energy, and performed by the Center for Photonics Technology of the Bradley Department of Electrical and Computer Engineering at Virginia Tech. The scope of work entails analyses of traveling grating generation technologies in an optical fiber, as well as the interrogation of the gratings to infer a distributed temperature along the fiber, for the purpose of developing a real-time refractory health condition monitoring technology for coal gasifiers. During the project period, which is from 2011-2015, three different sensing principles were studied, including four-wave mixing (FWM), coherent optical time-domain reflectometer (C-OTDR) and Brillouin optical time-domain analysis (BOTDA). By comparing the three methods, the BOTDA was selected for further development into a complete bench-top sensing system for the proposed high-temperature sensing application. Based on the input from Eastman Chemical, the industrial collaborator on this project, a cylindrical furnace was designed and constructed to simulate typical gasifier refractory temperature conditions in the laboratory, and verify the sensor’s capability to fully monitor refractory conditions on the back-side at temperatures up to 1000°C. In the later stages of the project, the sensing system was tested in the simulated environment for its sensing performance and high-temperature survivability. Through theoretical analyses and experimental research on the different factors affecting the sensor performance, a sensor field deployment strategy was proposed for possible future sensor field implementations.

Friction measurements of steel on refractory bricks

International Nuclear Information System (INIS)

Eiselstein, L.E.

1981-08-01

During startup or shutdown of a pool-type LMFBR, substantial shear stresses may arise between the base of the steel reactor vessel and the refractory brick support base. The magnitude of these stresses, which result from differences in thermal expansion, can be estimated if the friction coefficient is known. This report describes experiments to determine friction coefficients between 2 1/4 Cr-1Mo steel and several refractory materials and to examine effects to contact pressure, temperature, sliding velocity, lubricants, and surface condition

Characterization of refractory brick based on local raw material from Lampung Province - Indonesia

Science.gov (United States)

Amin, Muhammad; Suryana, Yayat I.; Isnugroho, Kusno; Aji, Bramantyo B.; Birawidha, David C.; Hendronursito, Yusup

2018-04-01

Refractories are non-metallic inorganic materials that are difficult to melt at high temperatures and used in high-temperature casting industries. Refractories are classified into their constituent mineral feed stocks, refractories having typical plot properties commonly called fire bricks. In the manufacture of refractory bricks that exist in the market during the use of mangrove materials derived from abroad that is from China. In this research the refractory brick materials used are quartz sand, feldspart, kaolin, bentonite, and ball clay. All materials come from local Lampung Province - Indonesia. The experiment, there are 7 kinds of experimental composition, made of plot shape with size 230 mm, 65 mm in thickness, 114 mm height mould using manual press machine with 10 tons power and burning at 1400°C for 5 hours. Refractory brick product is done by physical test in the form of porosity, specific gravity, compressive strength and XRF and SEM characteristics. The result of XRF characteristic of refractory brick composition of 1 to 5 compared to the refractory brick type SK 34 in the market and the result of composition 1 is a composition close to refractory brick composition type SK 34 namely SiO2 is 54.21 %, Al2O3 is 25.38 % and test Physical of Bulk density is 2.25 g/cm3, porosity is 18.98 % and compressive strength is 325 kg/cm2.

Refractory benign esophageal strictures

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Gopal Goyal

2016-01-01

Full Text Available Refractory benign esophageal stricture (RBES is a frequently encountered problem worldwide. These strictures arise from various causes such as corrosive injury, radiation therapy, peptic origin, ablative therapy, and after surgery. Most strictures can be treated successfully with endoscopic dilatation using bougies or balloons, with only a few complications. Those patients who fail after serial dilatation with bougies or balloons will come to the category of refractory strictures. Dilatation combined with intralesional steroid injections can be considered for peptic strictures, whereas incisional therapy has been demonstrated to be effective for short anastomotic strictures. When these therapeutic options do not resolve the stenosis, stent placement should be considered. Self-bougienage can be proposed to a selected group of patients with a proximal stenosis. Most of the patients of RBES respond to above-mentioned treatment and occasional patient may require surgery as the final treatment option. This review aims to provide a comprehensive approach toward endoscopic management of RBESs based on current literature and personal experience.

Illness identity in young adults with refractory epilepsy.

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Luyckx, Koen; Oris, Leen; Raymaekers, Koen; Rassart, Jessica; Moons, Philip; Verdyck, Ludo; Mijnster, Teus; Mark, Ruth E

2018-03-01

Refractory epilepsy is an intrusive condition with important implications for daily functioning in emerging and young adulthood. The present study examined the degree to which refractory epilepsy is integrated in one's identity, and examined how such a sense of illness identity was related to health-related quality of life (HRQOL). A total of 121 18- to 40-year-old patients with refractory epilepsy (56.2% women) completed self-report questionnaires assessing the four illness identity states of acceptance, enrichment, engulfment, and rejection (Illness Identity Questionnaire (IIQ)); HRQOL (Quality of Life in Epilepsy Inventory - 31); and seizure frequency and severity (Liverpool Seizure Severity Scale (LSSS)). Illness identity scores were compared with a sample of 191 patients with a nonneurological chronic disease (congenital heart disease). Hierarchical regression analyses were conducted to assess the predictive value of illness identity for HRQOL when simultaneously controlling for demographic and clinical features. Patients with refractory epilepsy scored higher on rejection and engulfment and lower on acceptance when compared with patients with congenital heart disease. Further, seizure severity and number of medication side-effects were positively related to engulfment and negatively to acceptance. Finally, when simultaneously controlling for various demographic and clinical variables, illness identity significantly predicted HRQOL (with engulfment being the strongest and most consistent predictor). The extent to which patients with refractory epilepsy succeed in integrating their illness into their identity may have important implications for HRQOL. Clinicians should be especially attentive for signs that patients feel engulfed by their epilepsy. Copyright © 2018 Elsevier Inc. All rights reserved.

Ketogenic diet treatment for pediatric super-refractory status epilepticus.

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Appavu, Brian; Vanatta, Lisa; Condie, John; Kerrigan, John F; Jarrar, Randa

2016-10-01

We aimed to study whether ketogenic diet (KD) therapy leads to resolution of super-refractory status epilepticus in pediatric patients without significant harm. A retrospective review was performed at Phoenix Children's Hospital on patients with super-refractory status epilepticus undergoing ketogenic diet therapy from 2011 to 2015. Ten children with super-refractory status epilepticus, ages 2-16 years, were identified. 4/10 patients had immune mediated encephalitis, including Rasmussen encephalitis, anti-N-methyl-d-aspartate receptor encephalitis, and post-infectious mycoplasma encephalitis. Other etiologies included Lennox Gastaut Syndrome, non-ketotic hyperglycinemia, PCDH19 and GABRG2 genetic epilepsy, New Onset Refractory Status Epilepticus, and Febrile Infection-Related Epilepsy Syndrome. 4/10 patients' EEG features suggested focal with status epilepticus, and 6/10 suggested generalized with status epilepticus. Median hospital length was 61days and median ICU length was 27days. The median number of antiepileptic medications prior to diet initiation was 3.0 drugs, and the median after ketogenic diet treatment was 3.5 drugs. Median duration of status epilepticus prior to KD was 18days. 9/10 patients had resolution of super-refractory status epilepticus in a median of 7days after diet initiation. 8/9 patients were weaned off anesthesia within 15days of diet initiation, and within 1day of achieving ketonuria. 1/10 patients experienced side effects on the diet requiring supplementation. Most patients achieved resolution of status epilepticus on KD therapy, suggesting it could be an effective therapy that can be utilized early in the treatment of children with super refractory status epilepticus. Copyright © 2016. Published by Elsevier Ltd.

Development of electro fused aggregates for use in refractories for the burning zone of cement kilns; Desenvolvimento de agregados eletrofundidos para utilizacao em refratarios para a zona de queima de fornos de cimento

Energy Technology Data Exchange (ETDEWEB)

Ferreira, Luis Leonardo Horne Curimbaba

2006-07-01

Electro fused aggregates are largely used in refractory production due to the better performance reached when they are employed. In this work electro fused aggregates were designed for application in refractories for the burning zone of cement kilns. Initially reaction evaluation was conducted aiming the identification of the most prone refractory systems when single refractory phases react with Portland cement phases at high temperatures. In the next step, raw materials of the best refractory systems were electro fused to generate different aggregate compositions. The electro fused aggregates properties were evaluated and the classified ones were used to produce refractory bricks for the burning zone of cement kilns. General characteristics of these bricks were measured and compared with a standard magnesia-spinel refractory. Aggregates of the system Mg O - TiO{sub 2} - Ca O, more specifically aggregates belonged to the compatibility triangle Mg O - Mg{sub 2}TiO{sub 4} - CaTiO{sub 3}, showed suitable characteristics for development of refractories for the burning zone cement kilns. (author)

Effects of Temperature, Oxygen Partial Pressure, and Materials Selection on Slag Infiltration into Porous Refractories for Entrained-Flow Gasifiers

Science.gov (United States)

Kaneko, Tetsuya Kenneth

The penetration rate of molten mineral contents (slag) from spent carbonaceous feedstock into porous ceramic-oxide refractory linings is a critical parameter in determining the lifecycle of integrated gasification combined cycle energy production plants. Refractory linings that withstand longer operation without interruption are desirable because they can mitigate consumable and maintenance costs. Although refractory degradation has been extensively studied for many other high-temperature industrial processes, this work focuses on the mechanisms that are unique to entrained-flow gasification systems. The use of unique feedstock mixtures, temperatures from 1450 °C to 1600 °C, and oxygen partial pressures from 10-7 atm to 10-9 atm pose engineering challenges in designing an optimal refractory material. Experimentation, characterization, and modeling show that gasifier slag infiltration into porous refractory is determined by interactions between the slag and the refractory that either form a physical barrier that impedes fluid flow or induce an increased fluid viscosity that decelerates the velocity of the fluid body. The viscosity of the slag is modified by the thermal profile of the refractory along the penetration direction as well as reactions between the slag and refractory that alter the chemistry, and thereby the thermo-physical properties of the fluid. Infiltration experiments reveal that the temperature gradient inherently present along the refractory lining limits penetration. A refractory in near-isothermal conditions demonstrates deeper slag penetration as compared to one that experiences a steeper thermal profile. The decrease in the local temperatures of the slag as it travels deeper into the refractory increases the viscosity of the fluid, which in turn slows the infiltration velocity of fluid body into the pores of the refractory microstructure. With feedstock mixtures that exhibit high iron-oxide concentrations, a transition-metal-oxide, the oxygen

A novel FKRP-related muscular dystrophy founder mutation in South African Afrikaner patients with a phenotype suggestive of a dystrophinopathy

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M M Mudau

2017-01-01

Full Text Available Background. Fukutin-related protein (FKRP muscular dystrophy is an autosomal recessive disorder caused by mutations in the FKRP gene. The condition is often misdiagnosed as a dystrophinopathy. A previously unreported mutation, c.1100T>C in exon 4 of FKRP, had been identified in homozygous form in two white South African (SA Afrikaner patients clinically diagnosed with a dystrophinopathy. Objectives. To investigate whether the c.1100T>C mutation and the common European FKRP mutation c.826C>A are present in other patients of Afrikaner origin with suspected dystrophinopathy, and whether a founder haplotype exists. Methods. The c.1100T>C mutation was initially tested for using an amplification refractory mutation system technique in 45 white SA Afrikaner patients who had tested negative using multiplex ligation probe amplification screening for exonic deletions/duplications in the dystrophin gene. Sequencing analysis was used to confirm the c.1100T>C mutation and screen for the c.826C>A mutation. Two cohorts (each numbering 100 of Afrikaans and other white controls were screened for the c.1100T>C and c.826C>A mutations, respectively. Results. Of the 45 patients, 8 patients (17.8% were homozygous for c.1100T>C, 2 (4.4% were compound heterozygotes for c.1100T>C and c.826C>A, and 1 (2.2% was heterozygous for c.1100T>C with a second unidentified mutation. The c.1100T>C mutation was found in 1/100 controls, but no heterozygotes for the c.826C>A mutation were identified. Linked marker analysis for c.1100T>C showed a common haplotype, suggesting a probable founder mutation in the SA Afrikaner population. Conclusion. FKRP mutations may be relatively common in Afrikaners, and screening should be considered in patients who have a suggestive phenotype and test negative for a dystrophinopathy. This test will be useful for offering diagnostic, carrier and prenatal testing for affected individuals and their families. As FKRP muscular dystrophy is autosomal

Historic, clinical, and prognostic features of epileptic encephalopathies caused by CDKL5 mutations.

Science.gov (United States)

Moseley, Brian D; Dhamija, Radhika; Wirrell, Elaine C; Nickels, Katherine C

2012-02-01

Mutations within the X-linked cyclin-dependent kinase-like 5 (CDKL5) gene are important causes of early-onset epileptic encephalopathies. We sought to determine the historic, clinical, and prognostic features of epilepsy secondary to CDKL5 mutations. We performed retrospective chart reviews of children at our institution with epilepsy and CDKL5 mutations. Six children were identified. One manifested a deletion in exons 10-15 of the CDKL5 gene, another manifested a single base-pair duplication in exon 3, and the rest manifested base-pair exchanges. The mean age of seizure onset was 1.8 months (range, 1-3 months). Although the majority (4/6, 67%) presented with partial-onset seizures, all children developed infantile spasms. All children demonstrated developmental delay and visual impairment. Although such mutations are X-linked, two children were boys. They did not present with more severe phenotypes than their female counterparts. Despite trials of antiepileptic drugs (mean, 5; range, 3-7), steroids/adrenocorticotropic hormone (4/6; 67%), and the ketogenic diet (6/6; 100%), all children manifested refractory seizures at last follow-up. Although no treatment eliminated seizures, topiramate, vigabatrin, and the ketogenic diet were most helpful at reducing seizure frequency. Copyright © 2012 Elsevier Inc. All rights reserved.

Compatibility of refractory alloys with space reactor system coolants and working fluids

International Nuclear Information System (INIS)

DeVan, J.H.; DiStefano, J.R.; Hoffman, E.E.

1984-01-01

The bulk of this report deals with compatibility studies in liquid lithium and boiling potassium. Substantial information is also presented concerning the reactivity of niobium and tantalum alloys with residual gases in high and ultrahigh vacuum atmospheres. The remaining information, which is much less extensive, covers the compatibility behavior of molybdenum and tungsten alloys in alkali metals and a qualitative assessment of the use of refractory metals for containing helium in a closed Brayton cycle. 22 references, 29 figures, 14 tables

Evolution and adaptation in Pseudomonas aeruginosa biofilms driven by mismatch repair system-deficient mutators.

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Adela M Luján

Full Text Available Pseudomonas aeruginosa is an important opportunistic pathogen causing chronic airway infections, especially in cystic fibrosis (CF patients. The majority of the CF patients acquire P. aeruginosa during early childhood, and most of them develop chronic infections resulting in severe lung disease, which are rarely eradicated despite intensive antibiotic therapy. Current knowledge indicates that three major adaptive strategies, biofilm development, phenotypic diversification, and mutator phenotypes [driven by a defective mismatch repair system (MRS], play important roles in P. aeruginosa chronic infections, but the relationship between these strategies is still poorly understood. We have used the flow-cell biofilm model system to investigate the impact of the mutS associated mutator phenotype on development, dynamics, diversification and adaptation of P. aeruginosa biofilms. Through competition experiments we demonstrate for the first time that P. aeruginosa MRS-deficient mutators had enhanced adaptability over wild-type strains when grown in structured biofilms but not as planktonic cells. This advantage was associated with enhanced micro-colony development and increased rates of phenotypic diversification, evidenced by biofilm architecture features and by a wider range and proportion of morphotypic colony variants, respectively. Additionally, morphotypic variants generated in mutator biofilms showed increased competitiveness, providing further evidence for mutator-driven adaptive evolution in the biofilm mode of growth. This work helps to understand the basis for the specific high proportion and role of mutators in chronic infections, where P. aeruginosa develops in biofilm communities.

Load-carrying capabilities of refractory alloys for space reactor power applications

International Nuclear Information System (INIS)

Horak, J.A.

1985-01-01

To achieve sufficient thermodynamic efficiency, space nuclear power systems must operate above 1000 0 C. A quantitative evaluation of the existing mechanical properties data for the refractory alloys relevant to space nuclear power systems design lifetimes up to seven years at temperatures up to 1400 0 C is being conducted. The most important properties for space nuclear power systems are long-term high-temperature (>1000 0 C) creep strength and ductility, low-temperature ( 0 C) fracture toughness [including ductile-to-brittle transition temperature, (DBTT)], and ductility at high strain rates; of special concern are the above properties for weldments of refractory alloys, composition, applied stress, test temperature, test environment (e.g., vacuum, lithium), and thermomechanical treatment (TMT) history. Currently being evaluated are, in order of ascending mp, selected alloys of niobium (e.g., Nb-1% Zr, Nb-1% Zr-0.1% C), molybdenum (e.g., Mo-13% Re), tantalum (e.g., ASTAR-811C), and tungsten (e.g., CVD W and W-25% Re). Creep properties of these alloys have been correlated via Larson-Miller, Manson-Hafered, and other empirical parameters; creep equations have been developed from these correlations. 12 figs., 8 tabs

Central Diabetes Insipidus in Refractory Antineutrophil Cytoplasmic Antibody-associated Vasculitis.

Science.gov (United States)

Ohashi, Keiji; Morishita, Michiko; Watanabe, Haruki; Sada, Ken-Ei; Katsuyama, Takayuki; Miyawaki, Yoshia; Katsuyama, Eri; Narazaki, Mariko; Tatebe, Noriko; Watanabe, Katsue; Kawabata, Tomoko; Wada, Jun

2017-11-01

We herein describe two cases of refractory antineutrophil cytoplasmic antibody-associated vasculitis (AAV) complicated with diabetes insipidus (DI) possibly related to hypertrophic pachymeningitis (HP). One patient had microscopic polyangiitis and HP, which were refractory to cyclophosphamide, azathioprine, rituximab, mycophenolate mofetil (MMF), and mizoribine. Remission was finally achieved with the use of etanercept, but DI occurred 5 years later. The other patient had granulomatosis with polyangiitis, which that was refractory to cyclophosphamide, methotrexate, MMF, and rituximab. DI subsequently developed, but was successfully treated with etanercept. Dura mater hypertrophy was macroscopically observed in the latter case.

A Review on Recent Advances on Magnesia-Doloma Refractories by Nano-Technology.

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Salman Ghasemi-Kahrizsangi

2017-07-01

Full Text Available Due to exhibiting an excellent chemical resistance against basic environments at high temperature, good thermal shock resistance, thermodynamic stability in the presence of carbon, and a suitable abrasion resistance, MgO-CaO refractories are widely used in argon-oxygen decarburization furnaces in the metallurgy industry and cement rotary kilns. Furthermore, MgO-CaO refractories are beneficial to removing inclusions from molten steels; thus, they have been considered to be one of the effective refractory types for processing clean steel products. Also, MgO-CaO materials have become one of the attractive steelmaking refractories because of their low cost and high ore reserves.  However, in spite of these primary advantages, the application of MgO-CaO refractories has not been popular due to its tendency to hydration when exposed to the atmosphere.  In world most of MgO-CaO bricks producers used of organic components such as tar, pitch, and peck for produce MgO-CaO refractories. But during the application of these bricks in steel and cement and industrials, they released CO and CO2 gases to air and pollute the atmosphere. For this reason, recently some researcher investigate the effect of additive nanoparticles on MgO-CaO refractories performance. They reported the used of Nano-additive have acceptable results and additive nanoparticles can replace by aforementioned environment contaminating organic compounds. In this study, we reviewed all effort that done for improving the hydration resistance of MgO-CaO refractories by application of Nano-additives with an emphasis on the beneficial the use of additives nanoparticle for reduction of environmental pollution by various industries which used of MgO-CaO refractory bricks.

Carbothermic reduction of refractory metals

International Nuclear Information System (INIS)

Anderson, R.N.; Parlee, N.A.D.

1976-01-01

The reduction of stable refractory metal oxides by carbon is generally unacceptable since the product is usually contaminated with carbides. The carbide formation may be avoided by selecting a solvent metal to dissolve the reactive metal as it is produced and reduce its chemical activity below that required for carbide formation. This approach has been successfully applied to the oxides of Si, Zr, Ti, Al, Mg, and U. In the case where a volatile suboxide, a carbonyl reaction, or a volatile metal occur, the use of the solvent metal appears satisfactory to limit the loss of material at low pressures. In several solute--solvent systems, vacuum evaporation is used to strip the solvent metal from the alloy to give the pure metal

Brentuximab Vedotin Treatment for Primary Refractory Hodgkin Lymphoma

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Hung-Bo Wu

2013-01-01

Full Text Available Up to 40% of patients with advanced Hodgkin lymphoma (HL become refractory or relapsed after current standard chemotherapy, among which primary refractory HL confers a particularly poor outcome. With intensive salvage chemotherapy and autologous stem cell transplantation, the long-term remission rate for these patients was only 30%, but more selective treatments with higher therapeutic index are needed. We report the experience of using a new anti-CD30 immunotoxin, brentuximab vedotin, in salvage treatment of a 30-year-old woman with primary refractory Hodgkin lymphoma. The patient presented with SVC syndrome due to the bulky mediastinal tumor and was confirmed to have classical Hodgkin lymphoma, nodular sclerosis type, stage IIIA. The tumor responded to induction chemotherapy transiently, but local progression was noted during subsequent cycles of treatment. Salvage radiotherapy to the mediastinal tumor, obtained no remission but was followed by rapid in-field progression and then lung metastasis. She declined stem cell transplantation and received salvage brentuximab vedotin (BV therapy, which induced dramatic shrinkage of tumor without significant side effects. Serial followup of PET/CT imaging confirmed a rapid and continuous complete remission for 12 months. Although durability of the remission needs further observation, this case illustrates the excellent efficacy of brentuximab vedotin in primary refractory Hodgkin lymphoma.

Association of a Novel Nonsense Mutation in KIAA1279 with Goldberg-Shprintzen Syndrome.

Science.gov (United States)

Salehpour, Shadab; Hashemi-Gorji, Feyzollah; Soltani, Ziba; Ghafouri-Fard, Soudeh; Miryounesi, Mohammad

2017-01-01

Goldberg-Shprintzen syndrome (OMIM 609460) (GOSHS) is an autosomal recessive multiple congenital anomaly syndrome distinguished by intellectual disability, microcephaly, and dysmorphic facial characteristics. Most affected individuals also have Hirschsprung disease and/or gyral abnormalities of the brain. This syndrome has been associated with KIAA1279 gene mutations at 10q22.1. Here we report a 16 yr old male patient referred to Center for Comprehensive Genetic Services, Tehran, Iran in 2015 with cardinal features of GOSHS in addition to refractory seizures. Whole exome sequencing in the patient revealed a novel nonsense (stop gain) homozygous mutation in KIAA1279 gene (KIAA1279: NM_015634:exon6:c.C976T:p.Q326X). Considering the wide range of phenotypic variations in GOSHS, relying on phenotypic characteristics for discrimination of GOSH from similar syndromes may lead to misdiagnosis. Consequently, molecular diagnostic tools would help in accurate diagnosis of such overlapping phenotypes.

Repair-resistant mutation in Neurospora

International Nuclear Information System (INIS)

Stadler, D.; Macleod, H.; Loo, M.

1987-01-01

Chronic UV treatment produces severalfold fewer mutations in Neurospora conidia than does the same total dose of acute UV. Experiments were designed to determine the conditions required for chronic UV mutagenesis. Measurement of the coincidence frequency for two independent mutations revealed the existence of a subset of cells which are mutable by chronic UV. Analysis of forward mutation at the mtr locus showed that the genetic alterations produced by chronic UV were virtually all point mutants, even though the assay system could detect alterations or deletions extending into neighboring genes. A significant fraction of the mutants produced by acute UV were multigenic deletions. The size of the dose-rate effect (acute UV mutation frequency divided by chronic UV mutation frequency) was compared for several different mutation assay systems. Forward mutations (recessive lethals and mtr) gave values ranging from four to nine. For events which were restricted to specific molecular sites (specific reversions and nonsense suppressor mutations), there was a wider range of dose-rate ratios. This suggests that chronic UV mutation may be restricted to certain molecular sequences or configurations

Testing of the melter lid refractory for the West Valley Demonstration Project (WVDP)

International Nuclear Information System (INIS)

Gupta, A.; Jain, V.; Mahoney, J.L.; Holman, T.M.

1991-01-01

Monofrax H and Mulfrax 202 refractory were tested for potential application as the melter lid refractory for the WVDP. Resistance to spalling and corrosion by the slurry and offgas salts were primary criteria for selection. Test specimens were subjected to thermal cycling between 450 and 1,100C for five weeks. Visual examination indicated some corrosion but no spalling. SEM/EDS analysis was performed to determine the glass/refractory interface corrosion mechanism. The refractory selection basis will be discussed

Sporadic periventricular nodular heterotopia: Classification, phenotype and correlation with Filamin A mutations.

Science.gov (United States)

Liu, Wenyu; Yan, Bo; An, Dongmei; Xiao, Jiahe; Hu, Fayun; Zhou, Dong

2017-07-01

The purpose of this study was to better delineate the clinical spectrum of periventricular nodular heterotopia (PNH) in a large patient population after long term follow up. Specifically, this study aimed to relate PNH subtypes to clinical or epileptic outcomes, epileptic discharges and underlying Filamin A (FLNA) mutations by analyzing anatomical features. The study included 100 patients with radiologically confirmed nodular heterotopia. Patients' FLNA gene sequences and medical records were analyzed. Two-sided Chi-square test and Fisher's exact t-test were used to assess associations between the distribution of PNHs and specific clinical features. Based on imaging data, patients were subdivided into three groups: (a) classical (bilateral frontal and body, n=41 patients), (b) bilateral asymmetrical or posterior (n=16) and (c) unilateral heterotopia (n=43). Most patients with classical heterotopia were females (P=0.033) and were likely to have arachnoid cysts (P=0.025) and cardiac abnormalities (P=0.041), but were mostly seizure-free. Additionally, hippocampal abnormalities (P=0.022), neurological deficits (P=0.028) and cerebellar abnormalities (P=0.005) were more common in patients with bilateral asymmetrical heterotopia. Patients with unilateral heterotopia were prone to develop refractory epilepsy (P=0.041). FLNA mutations were identified in 8 patients. Each group's distinctive genetic mutations, epileptic discharge patterns and overall clinical outcomes confirm that the proposed classification system is reliable. These findings could not only be an indicator of a more severe morphological and clinical phenotype, but could also have clinical implications with respect to the epilepsy management and optimization of therapeutic options. Copyright © 2017 Elsevier B.V. All rights reserved.

Intravitreal dexamethasone implants for the treatment of refractory scleritis combined with uveitis in adult-onset Still's disease: a case report.

Science.gov (United States)

Ahn, Seong Joon; Hwang, Sun Jin; Lee, Byung Ro

2016-11-08

Adult-onset Still's disease is a systemic inflammatory disease which presents with uveitis and scleritis in the eye. Intravitreal dexamethasone implants are used for the treatment of refractory uveitis. A 19-year-old woman diagnosed to have adult-onset Still's disease for fevers, joint pain, and a salmon-colored bumpy rash presented with scleritis and uveitis in the left eye. Topical and systemic steroids with oral methotrexate failed to control the inflammation. We performed intravitreal injections of dexamethasone implants for side effects of steroid and refractory ocular inflammation. The therapy resulted in improvements in the patient's uveitis with reductions in scleral vessel engorgement and redness. There was no recurrence of uveitis or scleritis during 4 months following treatment. Intravitreal injections of dexamethasone implants may result in clinical improvements of refractory scleritis combined with uveitis.

A novel fully automated molecular diagnostic system (AMDS for colorectal cancer mutation detection.

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Shiro Kitano

Full Text Available BACKGROUND: KRAS, BRAF and PIK3CA mutations are frequently observed in colorectal cancer (CRC. In particular, KRAS mutations are strong predictors for clinical outcomes of EGFR-targeted treatments such as cetuximab and panitumumab in metastatic colorectal cancer (mCRC. For mutation analysis, the current methods are time-consuming, and not readily available to all oncologists and pathologists. We have developed a novel, simple, sensitive and fully automated molecular diagnostic system (AMDS for point of care testing (POCT. Here we report the results of a comparison study between AMDS and direct sequencing (DS in the detection of KRAS, BRAF and PI3KCA somatic mutations. METHODOLOGY/PRINCIPAL FINDING: DNA was extracted from a slice of either frozen (n = 89 or formalin-fixed and paraffin-embedded (FFPE CRC tissue (n = 70, and then used for mutation analysis by AMDS and DS. All mutations (n = 41 among frozen and 27 among FFPE samples detected by DS were also successfully (100% detected by the AMDS. However, 8 frozen and 6 FFPE samples detected as wild-type in the DS analysis were shown as mutants in the AMDS analysis. By cloning-sequencing assays, these discordant samples were confirmed as true mutants. One sample had simultaneous "hot spot" mutations of KRAS and PIK3CA, and cloning assay comfirmed that E542K and E545K were not on the same allele. Genotyping call rates for DS were 100.0% (89/89 and 74.3% (52/70 in frozen and FFPE samples, respectively, for the first attempt; whereas that of AMDS was 100.0% for both sample sets. For automated DNA extraction and mutation detection by AMDS, frozen tissues (n = 41 were successfully detected all mutations within 70 minutes. CONCLUSIONS/SIGNIFICANCE: AMDS has superior sensitivity and accuracy over DS, and is much easier to execute than conventional labor intensive manual mutation analysis. AMDS has great potential for POCT equipment for mutation analysis.

Effectiveness of botulinum toxin A in treatment of refractory erythromelalgia

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Kuan-Hsiang Lin

2013-05-01

Full Text Available Erythromelalgia is characterized by intense burning pain, erythema, and heat in affected areas after precipitating factors such as warm temperature or stress. It is refractory to treatment in some situations. We describe a woman with adenosquamous cell carcinoma of the lung and medically refractory erythromelalgia. The symptoms of erythromelalgia presented as refractory to any medical treatment. Due to the unresponsive nature of her condition, botulinum toxin type A (onabotulinumtoxin A was injected over both of her cheeks, periodically for six cycles. Her symptoms responded dramatically to subcutaneous and intradermal injection of botulinum toxin type A. Repetitive injection demonstrated consistent and reproducible responses, and the efficacy was maintained for approximately 1 month. No adverse effects or complications were noted. Botulinum toxin type A might be safe and effective as an alternative treatment for refractory erythromelalgia, but further large-scale studies are required.

Theoretical and practical aspects about corrosion of refractories used in steel metallurgy: part 3: characterization of commercial refractories; Aspectos teoricos e praticos sobre a corrosao de refratarios utilizados na metalurgia: caracterizacao de refratarios comerciais

Energy Technology Data Exchange (ETDEWEB)

Braganca, S.R., E-mail: saulorb@ufrgs.br [Universidade Federal do Rio Grande Sul (DEMAT/UFRGS), RS (Brazil)

2012-07-01

In this study, it was reviewed the main aspects found in the literature about refractories corrosion, evaluating the feasibility of certain tests and relating them with experimental results. The physical properties and microstructure of commercial refractories were analyzed, considering the differences between them and the quality implications and probable life of the refractory. Thus, it was studied the various types of refractories used as lining on steel ladle. Magnesia-carbon and doloma-carbon refractories were analyzed, highlighting the differences between them. The examined refractory showed characteristics favoring high resistance to corrosion process, presenting a series of properties to be selected in accordance with industry practice. (author)

Interventional embolization for refractory hyperthyroidism

International Nuclear Information System (INIS)

Jiang Guomin; Zhang Yunping; Chen Yaxian; Zhao Jinwei; Yang Weihuai; Chen Jinluo

2001-01-01

Objective: To evaluate the clinical effects of the thyroid artery embolization for refractory hyperthyroidism. Methods: Super selective catheterization and interventional embolization of thyroid superior and inferior arteries were performed with PVA microspheres and Gelfoam particles. Changes of function and size of thyroid were investigated after embolization. Results: The procedures were succeeded in all 21 patients. Followed up by 3-12 months, serum level of thyroid hormones dropped significantly (median FT 3 from 17.1 pmol/L to 7.44 pmol/L, median FT 4 from 51.1 pmol/L to 23.3 pmol/L, P< 0.01). The size of thyroid glands decreased remarkably. Symptoms of hyperthyroidism were controlled in 4 patients and were effectively controlled through low dose antithyroid medication in 17 patients. No serious complications occurred. Conclusions: Thyroid artery embolization is a new safe and effective method for refractory hyperthyroidism

Efficacy of infliximab in refractory posterior uveitis in Behcet's disease patients

OpenAIRE

Ayman K. El Garf; Amira A. Shahin; Sherif A. Shawky; Mohammed A. Azim; Dina A. Effat; Sherry K. Abdelrahman

2018-01-01

Aim of the work: Ocular manifestations are the main cause of morbidity in Behcet's disease (BD). Infliximab (IFX), a chimeric monoclonal antibody directed against tumor necrosis factor-alpha, may be efficient in refractory uveitis due to BD. The aim of this study was to assess the efficacy and safety of IFX in the treatment of patients with BD-associated refractory posterior uveitis (PU). Patient and Methods: Twenty patients with refractory Behcet's PU received IFX therapy as intravenous infu...

Ketogenic diet in 3 cases of childhood refractory status epilepticus

DEFF Research Database (Denmark)

Sort, Rune; Born, Alfred P; Pedersen, Karen N.

2013-01-01

Refractory status epilepticus (RSE) in children is associated with a significant risk of death or neurological morbidity. Recently attention has been drawn to the ketogenic diet (KD) as an acute treatment, as it has shown promise in controlling seizures in otherwise refractory status epilepticus...

Esophageal Stent for Refractory Variceal Bleeding: A Systemic Review and Meta-Analysis

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Xiao-Dong Shao

2016-01-01

Full Text Available Background. Preliminary studies suggest that covered self-expandable metal stents may be helpful in controlling esophageal variceal bleeding. Aims. To evaluate the effectiveness and safety of esophageal stent in refractory variceal bleeding in a systematic review and meta-analysis. Methods. A comprehensive literature search was conducted on PubMed, EMBASE, and Cochrane Library covering the period from January 1970 to December 2015. Data were selected and abstracted from eligible studies and were pooled using a random-effects model. Heterogeneity was assessed using I2 test. Results. Five studies involving 80 patients were included in the analysis. The age of patients ranged from 18 to 91 years. The mean duration of follow-up was 46.8 d (range, 30–60 d. The success rate of stent deployment was 96.7% (95% CI: 91.6%–99.5% and complete response to esophageal stenting was in 93.9% (95% CI: 82.2%–99.6%. The incidence of rebleeding was 13.2% (95% CI: 1.8%–32.8% and the overall mortality was 34.5% (95% CI: 24.8%–44.8%. Most of patients (87.4% died from hepatic or multiple organ failure, and only 12.6% of patients died from uncontrolled bleeding. There was no stent-related complication reported and the incidence of stent migration was 21.6% (95% CI: 4.7%–46.1%. Conclusion. Esophageal stent may be considered in patients with variceal bleeding refractory to conventional therapy.

Super Refractory Status Epilepticus

African Journals Online (AJOL)

user

et al did retrospective cohort study from 1 January st. 1994 to 31 March 1998 at Presbyterian Medical. Centre in Columbia, to determine the frequency, risk factors and impact on the outcome of RSE. They found out that 69% of seizures recurred after. Key Words: Super refractory status epilepticus, Zambia. Medical Journal of ...

Process technology - rare and refractory metals

International Nuclear Information System (INIS)

Gupta, C.K.; Bose, D.K.

1989-01-01

India has fairly rich resreves of rare and refractory metals. Abundant sources of ilmenite, rutile, zircon and rare earths are found in the placer deposits of the southern and eastern coasts of the country. Columbite-tantalite occur in mica and the mining belts of Bihar and cassiterite deposits are found in Bastar (Madhya Pradesh). Vanadium as a minor associate occurs in bauxites and in the vast deposits of titaniferrous magnetites. Over the years, research and development and pilot plant works in many research organisations in India have built up a sound technological base in the country for process metallurgy of many refractory and rare earth metals starting from their indigenous sources. The present paper provides a comprehensive view of the developments that have taken place till now on the processing of various refractory and rare earth metals with particular reference to the extensive work carried out at the Department of Atomic Energy. The coverage includes mineral benification separation of individual elements, preparation of pure intermediates, techniques of reduction to metal and final purification. The paper also reviews some of the recent developments that have been taken place in these fields and the potential application of these metals in the foreseeable future. (author). 22 refs., 18 fi g., 7 tabs

Reuse of ultrafine mineral wool production waste in the manufacture of refractory concrete.

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Stonys, R; Kuznetsov, D; Krasnikovs, A; Škamat, J; Baltakys, K; Antonovič, V; Černašėjus, O

2016-07-01

The paper deals with the mineral wool production waste (cupola dust - CD), presents CD characterization and aims to reuse CD in production of refractory concrete with calcium aluminate cement. The study of CD covers its chemical, phase and thermal analyses along with the morphological study and determination of particles size distribution. Zeta-potential, electrical conductivity and pH values of CD suspension are presented in the paper as well. Commercial microsilica additive in refractory concrete has been replaced with cupola dust. Compositions of refractory concrete have been prepared by incorporating 1%, 2% and 3% of CD. The bulk density, ultrasonic wave velocity, cold crushing strength and thermal shock resistance of the created refractory concrete have been determined. Based on experimental results, it has been found that cupola dust may be used for the production of refractory concrete. The environmental impact related to the CD reuse in refractory concrete production has been evaluated as well. Copyright © 2016 Elsevier Ltd. All rights reserved.

New system for vacuum deposition of refractory materials using an atmospheric-pressure inductively coupled plasma

International Nuclear Information System (INIS)

Merkle, B.D.; Kniseley, R.N.; Schmidt, F.A.

1987-01-01

We have successfully developed a technique utilizing an atmospheric-pressure inductively coupled plasma combined with a low-pressure deposition chamber for deposition of thin films. The equipment and method of operation are discussed. Refractory powders (Nb and Y 2 O 3 ) were injected into the plasma and deposited as Nb and substoichiometric yttrium oxide, YO/sub 1.49/, onto Fe and Cu substrates. The substoichiometric yttrium oxide deposit adhered well to the Fe and Cu substrates, while the Nb deposit adhered well to the Fe only. The Nb deposit on the Cu substrate flaked and peeled probably because of stresses induced from the thermal expansion mismatch between the Nb and Cu. Further studies will be undertaken to better understand the processes occurring in this type of plasma-coating system in order to optimize the instrumental parameters for particular coating applications

When myasthenia gravis is deemed refractory: clinical signposts and treatment strategies

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Mantegazza, Renato; Antozzi, Carlo

2018-01-01

The prognosis for patients with myasthenia gravis (MG) has improved significantly over the past half century, including substantial reductions in mortality and morbidity. However, approximately 10% of patients fails to respond adequately to current therapies and are considered treatment refractory, or treatment intolerant, and up to 80% have disease that fails to achieve complete stable remission. Although patients with autoantibodies to muscle-specific tyrosine kinase (anti-MuSK positive) are more likely to become treatment refractory than those with autoantibodies to the acetylcholine receptor (anti-AChR positive), each of these serotypes is substantially represented in the refractory MG population. Other risk factors for becoming treatment refractory include history of thymoma or thymectomy and female sex. A modified treatment algorithm for MG is proposed: patients who have disease that fails to respond to the stepwise approach to therapy, are treatment intolerant, or who require chronic rescue measures despite ongoing therapy, should be considered treatment refractory and emerging therapies should be considered. Three emerging monoclonal antibody-based therapies are discussed: the anti-B-cell agent rituximab; the terminal complement activation inhibitor eculizumab; and belimumab, which targets B-cell activating factor. Increased understanding of molecular pathophysiology and accurate antibody subtyping in MG should lead to the use of new therapeutic agents and successful management of treatment-refractory patients. PMID:29403543

Refractory organic substances in the environment

National Research Council Canada - National Science Library

Frimmel, F. H

2002-01-01

... and its Quality Control in Fractions of Refractory Organic Substances and the Corresponding Original Water Samples 39 Introduction 39 Description of Analytical Methods 41 Sample Treatment 41 Fl...

Evaluation of Alternative Refractory Materials for the Main Flame Deflectors at KSC Launch Complexes

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Calle, Luz Marina; Trejo, David; Rutkowsky, Justin

2006-01-01

The deterioration of the refractory materials used to protect the KSC launch complex steel base structures from the high temperatures during launches results in frequent and costly repairs and safety hazards. KSC-SPEC-P-0012, Specification for Refractory Concrete, is ineffective in qualifying refractory materials. This study of the specification and of alternative refractory materials recommends a complete revision of the specification and further investigation of materials that were found to withstand the environment of the Solid Rocket Booster main flame deflector better than the refractory materials in current use in terms of compressive strength, tensile strength, modulus of rupture, shrinkage, and abrasion.

Gene Mutation Profiles in Primary Diffuse Large B Cell Lymphoma of Central Nervous System: Next Generation Sequencing Analyses

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Todorovic Balint, Milena; Jelicic, Jelena; Mihaljevic, Biljana; Kostic, Jelena; Stanic, Bojana; Balint, Bela; Pejanovic, Nadja; Lucic, Bojana; Tosic, Natasa; Marjanovic, Irena; Stojiljkovic, Maja; Karan-Djurasevic, Teodora; Perisic, Ognjen; Rakocevic, Goran; Popovic, Milos; Raicevic, Sava; Bila, Jelena; Antic, Darko; Andjelic, Bosko; Pavlovic, Sonja

2016-01-01

The existence of a potential primary central nervous system lymphoma-specific genomic signature that differs from the systemic form of diffuse large B cell lymphoma (DLBCL) has been suggested, but is still controversial. We investigated 19 patients with primary DLBCL of central nervous system (DLBCL CNS) using the TruSeq Amplicon Cancer Panel (TSACP) for 48 cancer-related genes. Next generation sequencing (NGS) analyses have revealed that over 80% of potentially protein-changing mutations were located in eight genes (CTNNB1, PIK3CA, PTEN, ATM, KRAS, PTPN11, TP53 and JAK3), pointing to the potential role of these genes in lymphomagenesis. TP53 was the only gene harboring mutations in all 19 patients. In addition, the presence of mutated TP53 and ATM genes correlated with a higher total number of mutations in other analyzed genes. Furthermore, the presence of mutated ATM correlated with poorer event-free survival (EFS) (p = 0.036). The presence of the mutated SMO gene correlated with earlier disease relapse (p = 0.023), inferior event-free survival (p = 0.011) and overall survival (OS) (p = 0.017), while mutations in the PTEN gene were associated with inferior OS (p = 0.048). Our findings suggest that the TP53 and ATM genes could be involved in the molecular pathophysiology of primary DLBCL CNS, whereas mutations in the PTEN and SMO genes could affect survival regardless of the initial treatment approach. PMID:27164089

Comparative study of different methodologies to detect the JAK2 V617F mutation in chronic BCR-ABL1 negative myeloproliferative neoplasms

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Alline Didone

2016-04-01

Full Text Available Objectives: A mutation in the JAK2 gene, V617F, has been identified in several BCR-ABL1 negative myeloproliferative neoplasms (MPN: polycythemia vera (PV, essential thrombocythemia (ET, and primary myelofibrosis (PMF. Defining the presence or absence of this mutation is an essential part of clinical diagnostic algorithms and patient management. Here, we aimed to evaluate the performance of three PCR-based assays: Amplification Refractory Mutation System (ARMS, High-Resolution Melting analysis (HRM, and Sanger direct sequencing, and compare their results with those obtained by a PCR restriction fragment polymorphism assay (PCR-RFLP. Design and methods: We used blood samples from 136 patients (PV=20; PMF=20; ET=28, and other MPN suspected cases=68. Results: Comparable results were observed among the four assays in patients with PV, PMF, and MPN suspected cases. In patients with a diagnosis of ET, the JAK2 V617F mutation was detected in 67.8% of them by the PCR-ARMS and PCR-HRM assay and in 64% of them by the conventional Sanger sequence approach. The PCR-ARMS and PCR-HRM assays were 100% concordant. With these tests, only one of the 20 patients with ET and one of the three patients with clinically suspected MPN gave different results compared with those obtained by the PCR-RFLP. Conclusions: Our results have demonstrated that the PCR-ARMS and PCR-HRM assays could detect the JAK2 V617F mutation effectively in MPN patients, but PCR-HRM assays are rapid and the most cost-effective procedures. Keywords: Myeloproliferative, JAK2 V617F, Mutation, Wild type, Screening

Mutation analysis of β-thalassemia in East-Western Indian population: a recent molecular approach

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Shah, Parth S; Shah, Nidhi D; Ray, Hari Shankar P; Khatri, Nikunj B; Vaghasia, Ketan K; Raval, Rutvik J; Shah, Sandip C; Rao, Mandava V

2017-01-01

Background β-Thalassemia is the most prevalent genetic disorder in India. Its traits and coinheritance vary from mild to severe conditions, resulting in thalassemia minor, intermediate, and major, depending upon many factors. Purpose The objective of this study was to identify the incidence of β-thalassemia traits, their coinheritance, and mutations, as well as to support the patients already diagnosed with β-thalassemia in East-Western Indian population for better management. Patients and methods Seventy-five referral cases for β-thalassemia were analyzed for various β-thalassemia traits, heterozygosity, and homozygosity conditions. Blood phenotypic parameters using cell counter and capillary electrophoresis were investigated. Analyses of eight common mutations of thalassemia in India were carried out using polymerase chain reaction-amplification refractory mutation system, end point polymerase chain reaction, and DNA sequencing methods. Results Of these (75) referral cases from East-Western Indian region, 68 were positive for β-thalassemia (90.67%). The majority of case types were of β-thalassemia minor (49, 65.33%), followed by HbE traits (6, 8.0%) and β-thalassemia major, including heterozygous and homozygous (5, 6.66%; 4, 5.33%) types and then HbE homozygous (2, 2.66%), as well as one each of the HbE/β-thalassemia and HbD/β-thalassemia (1, 1.34%) combination. Mutation analysis also revealed that the highest frequency of mutation was c.92+5G>C (41, 60.29%) followed by deletion 619bp (9, 13.23%) and c.79G>A (8, 11.76%) in our study group. Five cases (nos. 24, 27, 33, 58, and 71) exhibited coinheritance between β0/β+ (2), β0/β D (1), and c.124_127delTTCT/β+ or β0(2) affecting the Rajasthani and Gujarati populations in our study of the Western region of India. Conclusion We strongly recommend these Western populations for genetic screening before adopting reproductive technologies and interracial marital relations. PMID:28546763

Molecular Profiling of Refractory Adrenocortical Cancers and Predictive Biomarkers to Therapy

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Sherri Z. Millis

2015-01-01

Full Text Available Purpose Current first-line chemotherapy for patients with metastatic adrenocortical cancer (ACC includes doxorubicin, etoposide, cisplatin, and mitotane with a reported response rate of only 23.2%. New therapeutic leads for patients with refractory tumors are needed; there is no standard second-line treatment. Methods Samples from 135 ACC tumors were analyzed by immunohistochemistry, in situ hybridization (FISH or CISH, and/or gene sequencing at a single commercial reference laboratory (Caris Life Sciences to identify markers associated with drug sensitivity and resistance. Results Overexpression of proteins related to demonstrated chemotherapy sensitivity or resistance included topoisomerase 1, progesterone receptor, and topoisomerase 2-alpha in 46%, 63%, and 42% of cases, respectively. Loss of excision repair cross-complementary group 1 (ERCC1, phosophatase and tensin homolog, O(6-methylguanine-methyltransferase, and ribonucleotide reductase M1 (RRM1 was identified in 56%, 59%, 71%, and 58% of cases, respectively. Other aberrations included overexpression of programmed death-ligand 1 or programmed cell death protein 1 tumor-infiltrating lymphocytes in >40% of cases. In all, 35% of cases had a mutation in the canonical Wnt signaling pathway (either CTNNB1 or APC and 48% had a mutation in TP53. No other genomic alterations were identified. Conclusion Biomarker alterations in ACC may be used to direct therapies, including recommendations for and potential resistance of some patients to traditional chemotherapies, which may explain the low response rate in the unselected population. Limited outcomes data support the use of mitotane and platinum therapies for patients with low levels of the proteins RRM1 and ERCC1.

Therapeutic burst-suppression coma in pediatric febrile refractory status epilepticus.

Science.gov (United States)

Lin, Jainn-Jim; Chou, Cheng-Che; Lan, Shih-Yun; Hsiao, Hsiang-Ju; Wang, Yu; Chan, Oi-Wa; Hsia, Shao-Hsuan; Wang, Huei-Shyong; Lin, Kuang-Lin

2017-09-01

Evidence for the beneficial effect of therapeutic burst-suppression coma in pediatric patients with febrile refractory status epilepticus is limited, and the clinical outcomes of this treatment strategy are largely unknown. Therefore, the aim of this study was to explore the outcomes of therapeutic burst-suppression coma in a series of children with febrile refractory status epilepticus. We retrospectively reviewed consecutive pediatric patients with febrile refractory status epilepticus admitted to our pediatric intensive care unit between January 2000 and December 2013. The clinical characteristics were analyzed. Thirty-five patients (23 boys; age range: 1-18years) were enrolled, of whom 28 (80%) developed super-refractory status epilepticus. All of the patients received the continuous administration of intravenous antiepileptic drugs for febrile refractory status epilepticus, and 26 (74.3%) achieved therapeutic burst-suppression coma. All of the patients received mechanical ventilatory support, and 26 (74.3%) received inotropic agents. Eight (22.9%) patients died within 1month. The neurologically functional outcomes at 6months were good in six (27.3%) of the 22 survivors, of whom two returned to clinical baseline. The patients with therapeutic burst-suppression coma were significantly associated with hemodynamic support than the patients with electrographic seizures control (p=0.03), and had a trend of higher 1-month mortality rate, worse 6months outcomes, and a longer duration of hospitalization. Our results suggest that therapeutic burst-suppression coma to treat febrile refractory status epilepticus may lead to an increased risk of hemodynamic instability and a trend of worse outcomes. Copyright © 2017 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Zirconium determination in refractories (gravimetric method)

International Nuclear Information System (INIS)

Capiotto, N.; Narahashi, Y.; Perish, P.G.; Souza, J.R. de

1991-01-01

A gravimetric method for zirconium determination in refractories is described. X-ray fluorescence analysis is also employed in this experiment and considerations about interfering elements are presented. (M.V.M.)

Development of various welding techniques for refractory and reactive metals and alloys

International Nuclear Information System (INIS)

Tonpe, Sunil; Saibaba, N.

2016-01-01

Nuclear Fuel Complex (NFC), Hyderabad, India with its excellent manufacturing facilities, produces nuclear fuel and structural components for nuclear reactors. NFC has taken up the challenging job of production of various critical components made out of refractory and reactive metals and alloys for nuclear and aerospace applications as an indigenization import substitute program. Refractory metals are prime candidates for many high temperature aerospace components because of refractory metal's high melting points and inherent creep resistance. The use of refractory metals is often limited because of their poor room temperature properties, inadequate oxidation resistance at elevated temperatures, difficulties associated with joining or welding etc. These advanced materials demand stringent requirement with respect to chemistry, dimensional tolerances, mechanical and metallurgical properties. This paper discusses in detail various welding techniques adopted in NFC for refractory and reactive metals and alloys such as Nb, Zr, Ti, Ta, Zircaloy, Titanium-half alloy etc. to manufacture various components and assemblies required for nuclear and aerospace applications

Applications of radioisotopes for studying refractory wear-out in Bhilai Steel Plant

International Nuclear Information System (INIS)

Dubey, R.S.; Bose, U.P.; Shipstone, A.J.

1979-01-01

In Bhilai Steel Plant, investigations were carried out to study the refractory wear-out of (i) hearth bottom of blast furnaces, (ii) roof of open hearth furnaces, and (iii) hot metal mixer lining, by using radioisotope tracer techniques with a view to evaluate the life of the refractory lining at various locations and to help in planning its timely hot and cold repairs. The life of the refractory lining has the effective bearing on the overall production and hence on the economy of the plant. The two radiometric methods employed for studying the erosion of the refractory lining, by using isotope inserted bricks at various positions without damaging the lining are (i) based on recording the penetration of gamma rays emitting from the radioactive isotopes inserted at definite points of the brick lining and, (ii) by detecting the radioactivity of the pig iron or steel arising due to washing away of the respective radioactive isotopes previously inserted in the lining. In hot mixers also radioisotope sources were placed in the critical location of refractory lining and the washing out of radioisotope due to refractory brick wear out was detected by radiogauging at site. It has been found that radiotracer technique with periodic radiogauging is very useful method for tracing the radioisotope source if more than one refractory brick with isotope is placed, as in the case of open hearth furnaces. The results of radioanalysis revealed that radioactivity coming alongwith hot metal steel has been far below the permissible limit of concentration i.e. 20 micro-curie per ton of metal. Further, during dismantling of the residual refractory lining of open hearth furnaces or hot metal mixers, bricks containing radioisotopes have been successfully retrieved for safe disposal. (auth.)

Presence of two alternative kdr-like mutations, L1014F and L1014S, and a novel mutation, V1010L, in the voltage gated Na+ channel of Anopheles culicifacies from Orissa, India

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Bhatt Rajendra M

2010-05-01

Full Text Available Abstract Background Knockdown resistance in insects resulting from mutation(s in the voltage gated Na+ channel (VGSC is one of the mechanisms of resistance against DDT and pyrethroids. Recently a point mutation leading to Leu-to-Phe substitution in the VGSC at residue 1014, a most common kdr mutation in insects, was reported in Anopheles culicifacies-a major malaria vector in the Indian subcontinent. This study reports the presence of two additional amino acid substitutions in the VGSC of an An. culicifacies population from Malkangiri district of Orissa, India. Methods Anopheles culicifacies sensu lato (s.l. samples, collected from a population of Malkangiri district of Orissa (India, were sequenced for part of the second transmembrane segment of VGSC and analyzed for the presence of non-synonymous mutations. A new primer introduced restriction analysis-PCR (PIRA-PCR was developed for the detection of the new mutation L1014S. The An. culicifacies population was genotyped for the presence of L1014F substitution by an amplification refractory mutation system (ARMS and for L1014S substitutions by using a new PIRA-PCR developed in this study. The results were validated through DNA sequencing. Results DNA sequencing of An. culicifacies individuals collected from district Malkangiri revealed the presence of three amino acid substitutions in the IIS6 transmembrane segments of VGSC, each one resulting from a single point mutation. Two alternative point mutations, 3042A>T transversion or 3041T>C transition, were found at residue L1014 leading to Leu (TTA-to-Phe (TTT or -Ser (TCA changes, respectively. A third and novel substitution, Val (GTG-to-Leu (TTG or CTG, was identified at residue V1010 resulting from either of the two transversions–3028G>T or 3028G>C. The L1014S substitution co-existed with V1010L in all the samples analyzed irrespective of the type of point mutation associated with the latter. The PIRA-PCR strategy developed for the

Prediction of BRAF mutation status of craniopharyngioma using magnetic resonance imaging features.

Science.gov (United States)

Yue, Qi; Yu, Yang; Shi, Zhifeng; Wang, Yongfei; Zhu, Wei; Du, Zunguo; Yao, Zhenwei; Chen, Liang; Mao, Ying

2017-10-06

OBJECTIVE Treatment with a BRAF mutation inhibitor might shrink otherwise refractory craniopharyngiomas and is a promising preoperative treatment to facilitate tumor resection. The aim of this study was to investigate the noninvasive diagnosis of BRAF-mutated craniopharyngiomas based on MRI characteristics. METHODS Fifty-two patients with pathologically diagnosed craniopharyngioma were included in this study. Polymerase chain reaction was performed on tumor tissue specimens to detect BRAF and CTNNB1 mutations. MRI manifestations-including tumor location, size, shape, and composition; signal intensity of cysts; enhancement pattern; pituitary stalk morphology; and encasement of the internal carotid artery-were analyzed by 2 neuroradiologists blinded to patient identity and clinical characteristics, including BRAF mutation status. Results were compared between the BRAF-mutated and wild-type (WT) groups. Characteristics that were significantly more prevalent (p < 0.05) in the BRAF-mutated craniopharyngiomas were defined as diagnostic features. The minimum number of diagnostic features needed to make a diagnosis was determined by analyzing the receiver operating characteristic (ROC) curve. RESULTS Eight of the 52 patients had BRAF-mutated craniopharyngiomas, and the remaining 44 had BRAF WT tumors. The clinical characteristics did not differ significantly between the 2 groups. Interobserver agreement for MRI data analysis was relatively reliable, with values of Cohen κ ranging from 0.65 to 0.97 (p < 0.001). A comparison of findings in the 2 patient groups showed that BRAF-mutated craniopharyngiomas tended to be suprasellar (p < 0.001), spherical (p = 0.005), predominantly solid (p = 0.003), and homogeneously enhancing (p < 0.001), and that patients with these tumors tended to have a thickened pituitary stalk (p = 0.014). When at least 3 of these 5 features were present, a tumor might be identified as BRAF mutated with a sensitivity of 1.00 and a specificity of 0

Fragmentation process of vitrified ceramic waste (VCW) aiming its incorporation in silico-aluminous refractory concrete for production of refractory bricks

International Nuclear Information System (INIS)

Gomes, L.B.; Brandalise, R.N.; Santos, V. dos; Bergmann, C.P.

2012-01-01

Ceramic industry generates large amounts of waste, usually disposed in landfills. Reuse could minimize their generation and provides sustainable solutions. However, the energy cost of grinding these waste becomes a hindrance to their reuse. This work aims to obtain particle sizes of vitrified ceramic waste (VCW) using a fast, efficient and low cost fragmentation process as well as its use in refractory concrete. The results shows a wide range of particle size of VCW, which can be used as a promising source of raw material for production of refractory concrete. (author)

MUTATIONS IN THE ARX GENE: CLINICAL, ELECTROENCEPHALOGRAPHIC AND NEUROIMAGING FEATURES IN 3 PATIENTS

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I. V. Ivanova

2017-01-01

Full Text Available The Aristaless-related homeobox (ARX gene is a member of the paired-type homeodomain transcription factor family with critical roles in embryonic development, particularly in the developing brain. Mutations in ARX gene demonstrate striking intra- and interfamilial pleiotropy together with genetic heterogeneity and lead to a broad spectrum of diseases. They give rise to 4 key phenotypic features: a different types of brain malformation, abnormal genitalia, epilepsy and intellectual disability. Authors present 3 clinical cases: a girl with duplication on the short arm of X-chromosome (Xp11.22-p22.33, which include genes ARX and CDKL5; a girl and a boy with a missense mutation in ARX gene that have not been previously described (chrX:25031522C>A, causes the substitution of an amino acid in the 197 protein position (p.Gly197Val, NM_139058.2. All patients suffer from severe epilepsy, that is refractory to antiepileptic drugs, and all of them have different degrees of psychomotor delay. The patients with missense mutation also have movement disorders: stereotypic movements in the girl and choreo athetosis and dystonia in the boy. Electroencephalographic abnormalities have been identified in all patients, and there were not significant abnormalities on magnetic resonance imaging in all cases. The described cases broaden the clinical spectrum of mutations in ARX gene.

Depression in patients with refractory temporal lobe epilepsy

Directory of Open Access Journals (Sweden)

Eleonora Borges Gonçalves

2011-10-01

Full Text Available OBJECTIVE: To evaluate the comorbidity of depressive disorders in patients with refractory temporal lobe epilepsy (TLE. METHOD: We evaluated 25 consecutive patients with refractory TLE (16 women and 9 men, using semi-structured psychiatric interviews, according to the International Classification of Diseases (ICD-10, and the Beck Depression Inventory. RESULTS: Seventeen of 25 patients (68% had depressive disorder: 6 with dysthymia, three with major depressive episodes and 8 with recurrent depressive disorders. Two (8% were diagnosed with mixed anxiety and depression. Only 5 of 17 patients (29.4% were previously diagnosed with depressive disorder and received prior antidepressant treatment. Duration of epilepsy was significantly higher in patients with depressive disorder (p=0.016, but there was no relationship between depression and seizure frequency. CONCLUSION: This study confirmed that depressive disorders are common and underdiagnosed in patients with TLE refractory to AEDs. Patients with longer duration of epilepsy are at higher risk of having depression.

Alkali metal-refractory metal biphase electrode for AMTEC

Science.gov (United States)

Williams, Roger M. (Inventor); Bankston, Clyde P. (Inventor); Cole, Terry (Inventor); Khanna, Satish K. (Inventor); Jeffries-Nakamura, Barbara (Inventor); Wheeler, Bob L. (Inventor)

1989-01-01

An electrode having increased output with slower degradation is formed of a film applied to a beta-alumina solid electrolyte (BASE). The film comprises a refractory first metal M.sup.1 such as a platinum group metal, suitably platinum or rhodium, capable of forming a liquid or a strong surface adsorption phase with sodium at the operating temperature of an alkali metal thermoelectric converter (AMTEC) and a second refractory metal insoluble in sodium or the NaM.sup.1 liquid phase such as a Group IVB, VB or VIB metal, suitably tungsten, molybdenum, tantalum or niobium. The liquid phase or surface film provides fast transport through the electrode while the insoluble refractory metal provides a structural matrix for the electrode during operation. A trilayer structure that is stable and not subject to deadhesion comprises a first, thin layer of tungsten, an intermediate co-deposited layer of tungsten-platinum and a thin surface layer of platinum.

Estimation of sensing characteristics for refractory nitrides based gain assisted core-shell plasmonic nanoparticles

Science.gov (United States)

Shishodia, Manmohan Singh; Pathania, Pankaj

2018-04-01

Refractory transition metal nitrides such as zirconium nitride (ZrN), hafnium nitride (HfN) and titanium nitride (TiN) have emerged as viable alternatives to coinage metals based plasmonic materials, e.g., gold (Au) and silver (Ag). The present work assesses the suitability of gain assisted ZrN-, HfN- and TiN-based conventional core-shell nanoparticles (CCSNPs) and multilayered core-shell nanoparticles (MCSNPs) for refractive index sensing. We report that the optical gain incorporation in the dielectric layer leads to multifold enhancement of the scattering efficiency (Qsca), substantial reduction of the spectral full width at half maximum, and a higher figure of merit (FOM). In comparison with CCSNPs, the MCSNP system exhibits superior sensing characteristics such as higher FOM, ˜ 45% reduction in the critical optical gain, response shift towards the biological window, and higher degree of tunability. Inherent biocompatibility, growth compatibility, chemical stability and flexible spectral tuning of refractory nitrides augmented by superior sensing properties in the present work may pave the way for refractory nitrides based low cost sensing.

Endurance exercise rescues progeroid aging and induces systemic mitochondrial rejuvenation in mtDNA mutator mice

Science.gov (United States)

Safdar, Adeel; Bourgeois, Jacqueline M.; Ogborn, Daniel I.; Little, Jonathan P.; Hettinga, Bart P.; Akhtar, Mahmood; Thompson, James E.; Melov, Simon; Mocellin, Nicholas J.; Kujoth, Gregory C.; Prolla, Tomas A.; Tarnopolsky, Mark A.

2011-01-01

A causal role for mitochondrial DNA (mtDNA) mutagenesis in mammalian aging is supported by recent studies demonstrating that the mtDNA mutator mouse, harboring a defect in the proofreading-exonuclease activity of mitochondrial polymerase gamma, exhibits accelerated aging phenotypes characteristic of human aging, systemic mitochondrial dysfunction, multisystem pathology, and reduced lifespan. Epidemiologic studies in humans have demonstrated that endurance training reduces the risk of chronic diseases and extends life expectancy. Whether endurance exercise can attenuate the cumulative systemic decline observed in aging remains elusive. Here we show that 5 mo of endurance exercise induced systemic mitochondrial biogenesis, prevented mtDNA depletion and mutations, increased mitochondrial oxidative capacity and respiratory chain assembly, restored mitochondrial morphology, and blunted pathological levels of apoptosis in multiple tissues of mtDNA mutator mice. These adaptations conferred complete phenotypic protection, reduced multisystem pathology, and prevented premature mortality in these mice. The systemic mitochondrial rejuvenation through endurance exercise promises to be an effective therapeutic approach to mitigating mitochondrial dysfunction in aging and related comorbidities. PMID:21368114

Plasma deposition of refractories

International Nuclear Information System (INIS)

Kudinov, V.V.; Ivanov, V.M.

1981-01-01

The problems of deposition, testing and application of plasma coating of refractory metals and oxides are considered. The process fundamentals, various manufacturing procedures and equipment for their realization are described in detail. Coating materials are given (Al, Mg, Al 2 O 3 , ZrO 2 , MgAlO 4 ) which are used in reactor engineering and their designated purposes are shown [ru

The management of metastatic radioiodine-refractory differentiated thyroid cancer requires an integrated approach including both directed and systemic therapies.

Science.gov (United States)

Cooray, Shamil D; Topliss, Duncan J

2017-01-01

A 58-year-old man with metastatic radioiodine-refractory differentiated thyroid cancer (DTC) presented with left thigh and right flank numbness. He had known progressive and widespread bony metastases, for which he received palliative radiotherapy, and multiple bilateral asymptomatic pulmonary metastases. CT scan and MRI of the spine revealed metastases at right T10-L1 vertebrae with extension into the central canal and epidural disease at T10 and T11 causing cord displacement and canal stenosis but retention of spinal cord signal. Spinal surgery was followed by palliative radiotherapy resulting in symptom resolution. Two months later, sorafenib received approval for use in Australia and was commenced and up-titrated with symptomatic management of mild adverse effects. Follow-up CT scan three months after commencement of sorafenib revealed regression of pulmonary metastases but no evident change in most bone metastases except for an advancing lesion eroding into the right acetabulum. The patient underwent a right total hip replacement, intra-lesional curettage and cementing. After six months of sorafenib therapy, CT scanning showed enlarging liver lesions with marked elevation of serum thyroglobulin. Lenvatinib was commenced and sorafenib was ceased. He now has stable disease with a falling thyroglobulin more than 5 years after metastatic radioiodine-refractory DTC was diagnosed. In DTC, 5% of distant metastases become radioiodine-refractory, resulting in a median overall survival of 2.5-3.5 years. Tyrosine kinase inhibitor (TKI) therapy has recently been demonstrated to increase progression-free survival in these patients but poses some unique management issues and is best used as part of an integrated approach with directed therapy. Directed therapies may have greater potential to control localised disease and related symptoms when compared to systemic therapies.Consider TKI therapy in progressive disease where benefits outweigh risks.Active surveillance and

Barrier Coatings for Refractory Metals and Superalloys

Energy Technology Data Exchange (ETDEWEB)

SM Sabol; BT Randall; JD Edington; CJ Larkin; BJ Close

2006-02-23

In the closed working fluid loop of the proposed Prometheus space nuclear power plant (SNPP), there is the potential for reaction of core and plant structural materials with gas phase impurities and gas phase transport of interstitial elements between superalloy and refractory metal alloy components during service. Primary concerns are surface oxidation, interstitial embrittlement of refractory metals and decarburization of superalloys. In parallel with kinetic investigations, this letter evaluates the ability of potential coatings to prevent or impede communication between reactor and plant components. Key coating requirements are identified and current technology coating materials are reviewed relative to these requirements. Candidate coatings are identified for future evaluation based on current knowledge of design parameters and anticipated environment. Coatings were identified for superalloys and refractory metals to provide diffusion barriers to interstitial transport and act as reactive barriers to potential oxidation. Due to their high stability at low oxygen potential, alumina formers are most promising for oxidation protection given the anticipated coolant gas chemistry. A sublayer of iridium is recommended to provide inherent diffusion resistance to interstitials. Based on specific base metal selection, a thin film substrate--coating interdiffusion barrier layer may be necessary to meet mission life.

Barrier Coatings for Refractory Metals and Superalloys

International Nuclear Information System (INIS)

SM Sabol; BT Randall; JD Edington; CJ Larkin; BJ Close

2006-01-01

In the closed working fluid loop of the proposed Prometheus space nuclear power plant (SNPP), there is the potential for reaction of core and plant structural materials with gas phase impurities and gas phase transport of interstitial elements between superalloy and refractory metal alloy components during service. Primary concerns are surface oxidation, interstitial embrittlement of refractory metals and decarburization of superalloys. In parallel with kinetic investigations, this letter evaluates the ability of potential coatings to prevent or impede communication between reactor and plant components. Key coating requirements are identified and current technology coating materials are reviewed relative to these requirements. Candidate coatings are identified for future evaluation based on current knowledge of design parameters and anticipated environment. Coatings were identified for superalloys and refractory metals to provide diffusion barriers to interstitial transport and act as reactive barriers to potential oxidation. Due to their high stability at low oxygen potential, alumina formers are most promising for oxidation protection given the anticipated coolant gas chemistry. A sublayer of iridium is recommended to provide inherent diffusion resistance to interstitials. Based on specific base metal selection, a thin film substrate--coating interdiffusion barrier layer may be necessary to meet mission life

Prognostic Factors for Refractory Status Epilepticus

Directory of Open Access Journals (Sweden)

J. Gordon Millichap

2013-03-01

Full Text Available Researchers at the Mayo Clinic, Rochester, MN studied the outcome and identified prognostic factors for refractory status epilepticus (RSE in 54 adult patients, median age 52 years [range 18-93].

First Ti-XANES analyses of refractory inclusions from Murchison

International Nuclear Information System (INIS)

Simon, S.B.; Sutton, S.R.; Grossman, L.

2009-01-01

Ti valence in refractory phases is an important recorder of redox conditions in the early solar nebula. We report the valence of Ti in pyroxene, spinel and hibonite in spinel-hibonite and spinel-pyroxene inclusions and in a coarse hibonite grain. A system of solar composition is so reducing that Ti 3+ and Ti 4+ can coexist, making the valence of Ti a valuable indicator of f O2 conditions during formation of nebular materials. The Ti 3+ /Ti 4+ ratios observed in the Ti-rich phases fassaite and rhoenite in coarse-grained refractory inclusions from CV3 chondrites have been shown to be quantitatively consistent with formation in a gas of solar composition (log f O2 = IW-6.8), but these are the only objects in chondrites for which this is the case. Here, we report the valence of Ti in various phases in refractory inclusions from the Murchison CM2 chondrite. The second-highest temperature, major-element-bearing phase predicted to condense from a gas of solar composition, hibonite (ideally CaAl 12 O 19 ), can contain significant amounts of Ti, but the hibonite structure can have oxygen vacancies, so calculation of Ti valence from stoichiometry of electron probe analyses is not recommended for hibonite. To date, the only reported measurement of Ti valence in meteoritic hibonite was done by electron spin resonance, on coarse crystals from a Murchison hibonite-perovskite-melilite inclusion. Spinel and most of the pyroxene in CM inclusions contain too little Ti for derivation of Ti 3+ /Ti 4+ ratios from electron probe analyses. X-ray absorption near edge spectroscopy (XANES), however, allows determination of Ti valence in relatively Ti-poor phases. In the present work, we apply synchrotron microXANES to a large hibonite grain from Murchison and to spinel-hibonite (sp-hib) and spinel-pyroxene (sp-pyx) inclusions from Murchison, refractory materials whose Ti 3+ /Ti 4+ ratios have not been previously measured. Analysis of these samples allows comparison of Ti valence of (1

Refractory disease in autoimmune diseases

NARCIS (Netherlands)

Vasconcelos, Carlos; Kallenberg, Cees; Shoenfeld, Yehuda

Refractory disease (RD) definition has different meanings but it is dynamic, according to knowledge and the availability of new drugs. It should be differentiated from severe disease and damage definitions and it must take into account duration of adequate therapy and compliance of the patient. It

CDKL5 and ARX mutations are not responsible for early onset severe myoclonic epilepsy in infancy.

Science.gov (United States)

Nabbout, Rima; Depienne, Christel; Chipaux, Mathilde; Girard, Benoit; Souville, Isabelle; Trouillard, Oriane; Dulac, Olivier; Chelly, Jamel; Afenjar, Alexandra; Héron, Delphine; Leguern, Eric; Beldjord, Cherif; Bienvenu, Thierry; Bahi-Buisson, Nadia

2009-11-01

Severe myoclonic epilepsy of infancy (SMEI) or Dravet syndrome (DS) is a distinctive epilepsy syndrome often associated with de novo mutations in the SCN1A gene. However, 25-30% patients with SMEI/DS are negative for SCN1A mutation screening, suggesting that other molecular mechanisms may account for these disorders. Given the overlapping and heterogeneous clinical features of CDKL5- and ARX-related epilepsies and SMEI/DS, we postulated that CDKL5 mutations in females and ARX mutations gene in males may be associated with early onset seizures forms of SMEI/DS. Twenty-eight patients with early onset SMEI/DS before 6 months negative for SCN1A mutational screening were selected and screened for mutations in the ARX gene in males (n=14) or the CDKL5 gene in females (n=14). No mutations in either gene were found except one intronic variation of uncertain pathogenicity in the CDKL5 gene. All patients started seizures at mean age of 3.48 months. Thirteen patients had familial history of epilepsy or febrile seizures. Patients evolved toward refractory epilepsy with generalized tonic clonic seizures (18/28) and myoclonia (23/28) and severe neurological impairment with autistic features (13/28), ataxia (14/28) and spasticity (5/28). No patient ever exhibited infantile spasms, dystonia, or Rett-like features. Our results illustrate that mutation screening of ARX and CDKL5 is not effective in patients selected on the basis of clinical signs associated to early onset SMEI/DS. In addition, they might reflect that other phenotypic features associated with CDKL5 mutations (Rett-like features, infantile spasm) or ARX mutations (dystonia, spasticity) are more distinctive. 2009 Elsevier B.V. All rights reserved.

Chemical analysis of refractories by plasma spectrometry

International Nuclear Information System (INIS)

Coutinho, C.A.

1990-01-01

X-ray spectrometry has been, since the last two or three decades, the traditional procedure for the chemical analysis of refractories, due to its high degree of accuracy and speed to produce analytical results. An interesting alternative to X-ray fluorescence is provided by the Inductively Coupled Plasma Spectrometry technique, for those laboratories where wet chemistry facilities are already available or process control is not required at high speed, or investiment costs have to be low. This paper presents results obtained by plasma spectroscopy for the analysis of silico - aluminous refractories, showing calibration curves, precion and detection limits. Considerations and comparisons with X-ray fluorescence are also made. (author) [pt

Improvements in or relating to refractory materials

International Nuclear Information System (INIS)

Peckett, J.W.A.

1980-01-01

A process is described for the production of a refractory material which includes heating an intermediate material containing carbon to cause a thermally induced reaction involving carbon in the intermediate material, wherein the intermediate material has been produced by heating a shaped gel precipitated gel, and the carbon in the intermediate material for participating in the thermally induced reaction has been produced from a gelling agent, or a derivative thereof, incorporated in the gel during gel precipitation. As examples, the refractory material may comprise uranium/plutonium oxide, or uranium/plutonium carbide, or thorium/uranium carbide, or tungsten carbide, or tungsten carbide/cobalt metal. (author)

Zirconium determination in refractories (gravimetric method)

International Nuclear Information System (INIS)

Capiotto, N.; Narahashi, Y.; Perish, C.G.; Souza, J.R.

1991-01-01

The zirconium determination in refractories is described, consisting in two separation methods for eliminating the interferences. The formatted product is calcined at 1100 0 C and determined gravimetrically as Zr P z 07. (author)

Genetics of refractoriness to Plasmodium falciparum in the mosquito Anopheles stephensi

NARCIS (Netherlands)

Feldmann, A.M.; Gemert, Geert-Jan van; Vegte-Bolmer, Marga G. van de; Jansen, Ritsert C.

1998-01-01

We previously selected a line of the malaria vector mosquito Anopheles stephensi refractory (resistant) to the human malaria parasite Plasmodium falciparum, using in vitro infections with P. falciparum gametocytes. This report presents data on the genetic background of refractoriness. The results of

REFRACTORY HYPERTENSION: EVIDENCE OF HEIGHTENED SYMPATHETIC ACTIVITY AS A CAUSE OF ANTIHYPERTENSIVE TREATMENT FAILURE

Science.gov (United States)

Dudenbostel, Tanja; Acelajado, Maria C.; Pisoni, Roberto; Li, Peng; Oparil, Suzanne; Calhoun, David A.

2015-01-01

Refractory hypertension is an extreme phenotype of treatment failure defined as uncontrolled blood pressure (BP) in spite of ≥5 classes of antihypertensive agents, including chlorthalidone and a mineralocorticoid receptor antagonist. A prospective evaluation of possible mechanisms of refractory hypertension has not been done. The goal of this study was to test for evidence of heightened sympathetic tone as indicated by 24-hr urinary (U-) normetanephrine levels, clinic and ambulatory heart rate (HR), HR variability (HRV), arterial stiffness as indexed by pulse wave velocity (PWV), and systemic vascular resistance (SVR) compared to patients with controlled resistant hypertension. Forty-four consecutive patients, 15 with refractory and 29 with controlled resistant hypertension, were evaluated prospectively. Refractory hypertensive patients were younger (48±13.3 vs. 56.5±14.1 years, p=0.038) and more likely female (80.0 vs 51.9 %, p=0.047) compared to patients with controlled resistant hypertension. They also had higher U-normetanephrine levels (464.4±250.2 vs. 309.8±147.6 μg/24h, p=0.03), higher clinic HR (77.8±7.7 vs. 68.8±7.6 bpm, p=0.001) and 24-hr ambulatory HR (77.8±7.7 vs 68.8±7.6, p=0.0018), higher PWV (11.8±2.2 vs. 9.4±1.5 m/s, p=0.009), reduced HRV (4.48 vs. 6.11, p=0.03), and higher SVR (3795±1753 vs. 2382±349 dyne·sec·cm5·m2, p=0.008). These findings are consistent with heightened sympathetic tone being a major contributor to antihypertensive treatment failure and highlight the need for effective sympatholytic therapies in patients with refractory hypertension. PMID:25987662

Productivity Costs in Patients with Refractory Chronic Rhinosinusitis

Science.gov (United States)

Rudmik, Luke; Smith, Timothy L.; Schlosser, Rodney J.; Hwang, Peter H.; Mace, Jess C.; Soler, Zachary M.

2014-01-01

Objective Disease-specific reductions in patient productivity can lead to substantial economic losses to society. The purpose of this study was to: 1) define the annual productivity cost for a patient with refractory chronic rhinosinusitis (CRS) and 2) evaluate the relationship between degree of productivity cost and CRS-specific characteristics. Study Design Prospective, multi-institutional, observational cohort study. Methods The human capital approach was used to define productivity costs. Annual absenteeism, presenteeism, and lost leisure time was quantified to define annual lost productive time (LPT). LPT was monetized using the annual daily wage rates obtained from the 2012 US National Census and the 2013 US Department of Labor statistics. Results A total of 55 patients with refractory CRS were enrolled. The mean work days lost related to absenteeism and presenteeism was 24.6 and 38.8 days per year, respectively. A total of 21.2 household days were lost per year related to daily sinus care requirements. The overall annual productivity cost was $10,077.07 per patient with refractory CRS. Productivity costs increased with worsening disease-specific QoL (r=0.440; p=0.001). Conclusion Results from this study have demonstrated that the annual productivity cost associated with refractory CRS is $10,077.07 per patient. This substantial cost to society provides a strong incentive to optimize current treatment protocols and continue evaluating novel clinical interventions to reduce this cost. PMID:24619604

Productivity costs in patients with refractory chronic rhinosinusitis.

Science.gov (United States)

Rudmik, Luke; Smith, Timothy L; Schlosser, Rodney J; Hwang, Peter H; Mace, Jess C; Soler, Zachary M

2014-09-01

Disease-specific reductions in patient productivity can lead to substantial economic losses to society. The purpose of this study was to: 1) define the annual productivity cost for a patient with refractory chronic rhinosinusitis (CRS) and 2) evaluate the relationship between degree of productivity cost and CRS-specific characteristics. Prospective, multi-institutional, observational cohort study. The human capital approach was used to define productivity costs. Annual absenteeism, presenteeism, and lost leisure time was quantified to define annual lost productive time (LPT). LPT was monetized using the annual daily wage rates obtained from the 2012 U.S. National Census and the 2013 U.S. Department of Labor statistics. A total of 55 patients with refractory CRS were enrolled. The mean work days lost related to absenteeism and presenteeism were 24.6 and 38.8 days per year, respectively. A total of 21.2 household days were lost per year related to daily sinus care requirements. The overall annual productivity cost was $10,077.07 per patient with refractory CRS. Productivity costs increased with worsening disease-specific QoL (r = 0.440; p = 0.001). Results from this study have demonstrated that the annual productivity cost associated with refractory CRS is $10,077.07 per patient. This substantial cost to society provides a strong incentive to optimize current treatment protocols and continue evaluating novel clinical interventions to reduce this cost. © 2014 The American Laryngological, Rhinological and Otological Society, Inc.

Felbamate in an adult population with severe refractory epilepsy.

LENUS (Irish Health Repository)

Kearney, H

2011-04-05

Felbamate (FBM) is efficacious in treating patients with refractory epilepsy but was withdrawn due to cases of aplastic anaemia, hepatic failure and five reported deaths. FBM is currently used in specialist centres and is only being used in one Irish centre to date. This papers aim is to review the efficacy and safety experience of FBM in Irish adult patients with refractory epilepsy. A retrospective chart review was done on patients\\' medical records. Patients were subdivided into responders and non responders based on change in seizure frequency and side effects were recorded for all. Of the 13 patients on FBM nine patients responded to FBM, four patients did not. FBM is a safe and efficacious alternative in an Irish adult population with refractory epilepsy. However close monitoring is still required given the potential fatal side effects that are possible with this anticonvulsant.

MICROWAVE MEASUREMENT OF REFRACTORY MATERIALS AT HIGH-TEMPERATURE

International Nuclear Information System (INIS)

Kharkovsky, S.; Zoughi, R.; Smith, J.; Davis, B.; Limmer, R.

2009-01-01

Knowledge of the electrical behavior of refractory materials may enable the development and optimization of microwave nondestructive techniques to detect and evaluate changes in their physical properties while the materials are in service. This paper presents the results of a limited and preliminary investigation in which two refractory materials (dense chrome and dense zircon) were subjected to increasing temperature in a furnace and in which a frequency-modulated continuous-wave radar operating in the frequency range of 8-18 GHz radar was used to evaluate their attenuation properties.

Microwave Measurement of Refractory Materials at High-Temperature

Science.gov (United States)

Kharkovsky, S.; Zoughi, R.; Smith, J.; Davis, B.; Limmer, R.

2009-03-01

Knowledge of the electrical behavior of refractory materials may enable the development and optimization of microwave nondestructive techniques to detect and evaluate changes in their physical properties while the materials are in service. This paper presents the results of a limited and preliminary investigation in which two refractory materials (dense chrome and dense zircon) were subjected to increasing temperature in a furnace and in which a frequency-modulated continuous-wave radar operating in the frequency range of 8-18 GHz radar was used to evaluate their attenuation properties.

Mutation Rates of STR Systems in Danes

DEFF Research Database (Denmark)

Andersen, Kim Emil; Bøttcher, Susanne Gammelgaard; Christensen, Susanne

Danish paternity cases in the period 1999 to 2005 were investigated regarding mutation rates in STR loci. STR-typing was performed by the Applied Biosystems AmplfStr Profiler Plus kit in the period 1999 to early 2005, hereafter named the PP9, and by Applied Biosystems AmplfStr Identifier kit for ...... and kits. Sex and STR locus specific mutation rates were estimated with 95% confidence limits by the method of Clopper and Pearson (1934)....

Ten patients with refractory status epilepticus in an intensive care department

NARCIS (Netherlands)

ter Maaten, JC; van Schijndel, RJM; Heimans, JJ; Schreuder, WO

1998-01-01

Status epilepticus (SE) is a serious disease, associated with a high morbidity and mortality, particularly if refractory to initial therapy. We describe the clinical manifestations and outcome in ten cases with refractory SE admitted to our medical intensive care unit. Three of these selected group

Refractory absence epilepsy associated with GLUT-1 deficiency syndrome.

LENUS (Irish Health Repository)

Byrne, Susan

2011-05-01

GLUT-1 deficiency syndrome (GLUT-1 DS) is a disorder of cerebral glucose transport associated with early infantile epilepsy and microcephaly. We report two boys who presented with refractory absence epilepsy associated with hypoglycorrhachia, both of whom have genetically confirmed GLUT-1 DS. We propose that these children serve to expand the phenotype of GLUT-1 DS and suggest that this condition should be considered as a cause of refractory absence seizures in childhood.

Treatment of Refractory Idiopathic Supraorbital Neuralgia Using Percutaneous Pulsed Radiofrequency.

Science.gov (United States)

Luo, Fang; Lu, Jingjing; Ji, Nan

2018-02-26

No ideal therapeutic method currently exists for refractory idiopathic supraorbital neuralgia patients who do not respond to conservative therapy, including medications and nerve blocks. Pulsed radiofrequency is a neuromodulation technique that does not produce sequelae of nerve damage after treatment. However, the efficacy of percutaneous pulsed radiofrequency for the treatment of refractory idiopathic supraorbital neuralgia is still not clear. The purpose of our study was to evaluate the efficacy and safety of pulsed radiofrequency treatment of the supraorbital nerve for refractory supraorbital neuralgia patients. We prospectively investigated the long-term effects of ultrasound-guided percutaneous pulsed radiofrequency in the treatment of 22 refractory idiopathic supraorbital neuralgia patients. A reduction in the verbal pain numeric rating scale score of more than 50% was used as the standard of effectiveness. The effectiveness rates at different time points within 2 years were calculated. After a single pulsed radiofrequency treatment, the effectiveness rate at 1 and 3 months was 77%, and the rates at 6 months, 1 year, and 2 years were 73%, 64%, and 50%, respectively. Except for a small portion of patients (23%) who experienced mild upper eyelid ecchymosis that gradually disappeared after approximately 2 weeks, no obvious complications were observed. In conclusion, the results of our study demonstrate that for patients with refractory idiopathic supraorbital neuralgia, percutaneous pulsed radiofrequency may be an effective and safe treatment choice. © 2018 World Institute of Pain.

Bulk Vitrification Performance Enhancement: Refractory Lining Protection Against Molten Salt Penetration

Energy Technology Data Exchange (ETDEWEB)

Hrma, Pavel R.; Bagaasen, Larry M.; Schweiger, Michael J.; Evans, Michael B.; Smith, Benjamin T.; Arrigoni, Benjamin M.; Kim, Dong-Sang; Rodriguez, Carmen P.; Yokuda, Satoru T.; Matyas, Josef; Buchmiller, William C.; Gallegos, Autumn B.; Fluegel, Alexander

2007-08-06

Bulk vitrification (BV) is a process that heats a feed material that consists of glass-forming solids and dried low-activity waste (LAW) in a disposable refractory-lined metal box using electrical power supplied through carbon electrodes. The feed is heated to the point that the LAW decomposes and combines with the solids to generate a vitreous waste form. This study supports the BV design and operations by exploring various methods aimed at reducing the quantities of soluble Tc in the castable refractory block portion of the refractory lining, which limits the effectiveness of the final waste form.

Application of Single Strand Conformational Polymorphism (PCR-SSCP) in Identification of Some Beta-Globin Gene Mutations in A Group of Egyptian Beta-Thalassemia Patients and Carriers

International Nuclear Information System (INIS)

Somaya, E.T.; Soliman, M.D

2010-01-01

The present study investigated whether the single-strand conformational polymorphism (SSCP) method could be employed to identify (rather than simply detect) four of the most common beta-globin gene mutations in the Egyptian population: IVS-I-110, IVS-I-6, the IVS-I-1, and Codon 39. Using DNA from 90 beta-thalassemia patients and carriers, by PCR the appropriate 238-bp region of the human beta-globin gene was amplified, the reaction products (Single-stranded DNA) were analyzed by none denaturing polyacrylamide gel electrophoresis, and the bands visualized by silver staining. Single-stranded DNA (ssDNA) fragments showed reproducible pattern of bands that were characteristic of the mutations present. With the use of control samples containing six of the 10 possible combinations of the four beta-globin gene mutations under study, we were able to predict the mutations present in 23 out of 90 (26.4%) of the patients studied. These predictions were confirmed independently by the amplification refractory mutation system (ARMS) method. It is concluded that this non-radioactive PCR-SSCP method can be used to reliably identify mutations in beta-thalassemia patients, provided that suitable controls are available. However, usefulness of this method for determining the genotype of beta-thalassaemic individuals is obviously limited by the great number of controls required. Moreover, the ability to detect mutations by SSCP is in general lower compared to other methods, ARMS, DGGE or DHPLC, which are reported to detect 49.5% to 73% of the mutations present. The SSCP method is nevertheless much easier to employ than other methods and is especially successful for beta-thalassemia carriers. This method would thus be particularly useful for an initial screening of target groups (prenatal diagnosis)

Usefulness of high-dose intravenous human immunoglobulins treatment for refractory recurrent pericarditis.

Science.gov (United States)

Moretti, Michele; Buiatti, Alessandra; Merlo, Marco; Massa, Laura; Fabris, Enrico; Pinamonti, Bruno; Sinagra, Gianfranco

2013-11-01

The management of refractory recurrent pericarditis is challenging. Previous clinical reports have noted a beneficial effect of high-dose intravenous human immunoglobulins (IvIgs) in isolated and systemic inflammatory disease-related forms. In this article, we analyzed retrospectively our clinical experience with IvIg therapy in a series of clinical cases of pericarditis refractory to conventional treatment. We retrospectively analyzed 9 patients (1994 to 2010) with refractory recurrent pericarditis, who received high-dose IvIg as a part of their medical treatment. Nonsteroidal anti-inflammatory drugs (NSAIDs), steroids, or colchicine treatment was not discontinued during IvIg treatment. No patients had a history of autoimmune or connective tissue diseases. During an average period of 11 months from the first recurrence, patients had experienced a mean of 5 relapses before the first IvIg treatment. In 4 cases, patients showed complete clinical remission with no further relapse after the first IvIg cycle. Two patients experienced a single minor relapse, responsive to short-term nonsteroidal anti-inflammatory drugs. In 2 patients, we performed a second cycle of IvIg after a recurrence of pericarditis, with subsequent complete remission. One patient did not respond to 3 cycles of IvIg and subsequently underwent pericardial window and long-term immunosuppressive treatment. No major adverse effect was observed in consequence of IvIg administration in all the cases. In conclusion, although IvIg mode of action is still poorly understood in this setting, this treatment can be considered as an option in patients with recurrent pericarditis refractory to conventional medical treatment and, in our small series, has proved to be effective in 8 of 9 cases. Copyright © 2013 Elsevier Inc. All rights reserved.

Plasticity enhancement mechanisms in refractory metals and intermetallics

International Nuclear Information System (INIS)

Gibala, R.; Chang, H.; Czarnik, C.M.; Edwards, K.M.; Misra, A.

1993-01-01

Plasticity enhancement associated with surface films and precipitates or dispersoids in bcc refractory metals is operative in ordered intermetallic compounds. Some results are given for NiAl and MoSi 2 -based materials. The monotonic and cyclic plasticity of NiAl at room temperature can be enhanced by surface films. Ductile second phases also enhance the plasticity of NiAl. MoSi 2 exhibits similar effects of surface films and dispersoids, but primarily at elevated temperatures. The plasticity enhancement is associated with enhanced dislocation generation from constrained deformation at the film-substrate or precipitate/dispersoid-matrix interface of the composite systems

Low-Chrome/Chrome Free Refractories for Slagging Gasifiers

International Nuclear Information System (INIS)

Bennett, J.P.; Kwong, K.-S.; Powell, C.P.; Thomas, H.; Petty, A.V. Jr.

2007-01-01

Gasifiers are containment vessels used to react carbon-containing materials with oxygen and water, producing syngas (CO and H2) that is used in chemical and power production. It is also a potential source of H2 in a future hydrogen economy. Air cooled slagging gasifiers are one type of gasifier, operating at temperatures from 1275-1575 C and at pressures of 400 psi or higher. They typically use coal or petroleum coke as the carbon source, materials which contain ash impurities that liquefy at the gasification temperatures, producing liquid slag in quantities of 100 or more tons/day, depending on the carbon fed rate and the percent ash present in the feedstock. The molten slag is corrosive to refractory linings, causing chemical dissolution and spalling. The refractory lining is composed of chrome oxide, alumina, and zirconia; and is replaced every 3-24 months. Gasifier users would like greater on-line availability and reliability of gasifier liners, something that has impacted gasifier acceptance by industry. Research is underway at NETL to improve refractory service life and to develop a no-chrome or low-chrome oxide alternative refractory liner. Over 250 samples of no- or low-chrome oxide compositions have been evaluated for slag interactions by cup testing; with potential candidates for further studies including those with ZrO2, Al2O3, and MgO materials. The development of improved liner materials is necessary if technologies such as IGCC and DOE's Near Zero Emissions Advanced Fossil Fuel Power Plant are to be successful and move forward in the marketplace

Long-term evaluation of treatment of chronic, therapeutically refractory tinnitus by neurostimulation

NARCIS (Netherlands)

Staal, M. J.; Holm, A. F.; Mooij, J. J. A.; Albers, F. W. J.; Bartels, H.

2007-01-01

Objective: Long-term evaluation of treatment of chronic, therapeutically refractory tinnitus by means of chronic electrical stimulation of the vestibulocochlear nerve. Patients: Inclusion criteria were severe, chronic, therapeutically refractory, unilateral tinnitus and severe hearing loss at the

Super-Refractory Status Epilepticus: Report of a Case and Review of the Literature.

Science.gov (United States)

Lapenta, Leonardo; Frisullo, Giovanni; Vollono, Catello; Brunetti, Valerio; Giannantoni, Nadia Mariagrazia; Sandroni, Claudio; Di Lella, Giuseppe; Della Marca, Giacomo

2015-10-01

Super-refractory status epilepticus (SE; ie, SE continuing or recurring despite 24 hours of general anesthesia) is a severe condition with high percentage of mortality and morbidity. Usually, this condition occurs because of serious brain damage; nevertheless, some patients develop super-refractory SE without identifiable etiology. Although not uncommonly encountered in neurointensive care, scientific data on this condition are still lacking in terms of treatment and prognosis. Herein, we report a case of super-refractory SE with recovery after 50 days, despite electroencephalographic (EEG) and magnetic resonance imaging (MRI) signs traditionally related to poor prognosis. A review of the literature on super-refractory SE is also presented. © EEG and Clinical Neuroscience Society (ECNS) 2014.

Tioguanine in the treatment of refractory coeliac disease--a single centre experience.

Science.gov (United States)

Tack, G J; van Asseldonk, D P; van Wanrooij, R L J; van Bodegraven, A A; Mulder, C J

2012-08-01

Refractory coeliac disease type I is a complicated form of coeliac disease characterised by primary or secondary resistance to a gluten-free diet with persisting or reoccurring intestinal villous atrophy and symptoms of malabsorption. Besides corticosteroids, azathioprine has been advocated for the treatment of refractory coeliac disease type I. However, tioguanine (TG) might be better tolerated and more efficacious owing to a simpler metabolism towards bioactivation. To evaluate tolerability and effectiveness of the nonconventional thiopurine derivative TG in refractory coeliac disease type I. Refractory coeliac disease type I patients treated with TG between June 2001 and November 2010 with a follow-up period of at least 1 year were included. Adverse events, laboratory values, 6-thioguanine nucleotide concentrations and rates of both clinical and histological response were evaluated at baseline and during follow-up. Twelve adult refractory coeliac disease type I patients were included. The median TG treatment duration was 14 months. Ten patients tolerated TG treatment on the long term, whereas two patients withdrew treatment due to adverse events. No nodular regenerative hyperplasia of the liver was observed. During follow-up clinical and histological response was observed in 83% and 78%, respectively. Corticosteroid dependency decreased by 50%. Tioguanine appears to be a convenient drug for the treatment of refractory coeliac disease type I based on higher histological and similar clinical response rates as compared with historical conventional therapies. © 2012 Blackwell Publishing Ltd.

Refractory, Abrasive and Other Industrial Mineral Operations

Data.gov (United States)

Department of Homeland Security — This map layer includes refractory, abrasive, and other industrial minerals operations in the United States. The data represent commodities covered by the Minerals...

Evaluation of nutritional status in children with refractory epilepsy

Directory of Open Access Journals (Sweden)

Testolin G

2006-04-01

Full Text Available Abstract Background children affected by refractory epilepsy could be at risk of malnutrition because of feeding difficulties (anorexia, chewing, swallowing difficulties or vomiting and chronic use of anticonvulsants, which may affect food intake and energy metabolism. Moreover, their energy requirement may be changed as their disabilities would impede normal daily activities. The aim of the present study was to evaluate nutritional status, energy metabolism and food intake in children with refractory epilepsy. Methods 17 children with refractory epilepsy (13 boys and 4 girls; mean age 9 ± 3,2 years; Body Mass Index 15,7 ± 3,6 underwent an anthropometric assessment, body composition evaluation by dual-energy X-ray absorptiometry, detailed dietetic survey and measurement of resting energy expenditure by indirect calorimetry. Weight-for-age, height-for-age (stunting and weight-for-height (wasting were estimated compared to those of a reference population of the same age. Results 40% of children were malnourished and 24% were wasted. The nutritional status was worse in the more disabled children. Dietary intake resulted unbalanced (18%, 39%, 43% of total daily energy intake derived respectively from protein, lipid and carbohydrate. Adequacy index [nutrient daily intake/recommended allowance (RDA × 100] was Conclusion many children with refractory epilepsy would benefit from individual nutritional assessment and management as part of their overall care.

Improvement of castable refractories for RH snorkel; RH shinshitsukanyo futeikeizai no kaizen

Energy Technology Data Exchange (ETDEWEB)

Nishi, K.; Obana, T.; Fijii, T.; Shimizu, I. [Harima Ceramics Corp., Hyogo (Japan)

1999-11-01

Relating to corrosion of RH submerged nozzle, resistance against iron oxide of monolithic refractories was examined. Corrosion and seepage of refractories were measured by rotating corrosion, refractories include alumina-spinel castable, which is applied to RH under tank, and alumina-magnesia castable, which is generally used for RH, submerged nozzle. Alumina-spinel castable is superior in resistance against iron oxide than alumina-magnesia castable, and the resistance decreased with increase of stainless fiber addition to castable. Alumina-spinel castable without stainless fiber was suitable for bottom end of the dipping pipe. (NEDO)

Application of monolithic lining to tundish and extension of tundish life with improvement of refractories; Tandeisshu futeiseika to taikabutsu kaizen ni yoru jumyo kojo

Energy Technology Data Exchange (ETDEWEB)

Mori, Eiichiro; Fujta, Takashi; Tanikawa, Kanji [Kobe Steel Corp., Hyogo (Japan)

1999-06-01

As a part of realization of monolithic liming of a tundish lining refractory, a study on 2CC and 3CC tundish monolithic refractories was carried out to improve placing efficiency and extend its life. Main inspection points were set on (1) casting facility specifications and (2) structure and material. A force feeding pump system with a high degree of freedom for placing layout was selected. It was judged preferable than a hopper dolly type ladle repair facility in the degree of freedom for a placing site and facility expenses, though little inferior at maintenance. A force feeding pump is combined with a boom to enable the facility to cast for plural objects. Low expansion and creep properties were given to the refractory by reviewing the addition of the expansive material and the structure was improved by setting anchor blocks at corner parts. The cost of refractories was reduced and labor saving of refractory placing workers was achieved by widely extending the life. (NEDO)

Effectiveness of intravenous levetiracetam as an adjunctive treatment in pediatric refractory status epilepticus.

Science.gov (United States)

Kim, Jon Soo; Lee, Jeong Ho; Ryu, Hye Won; Lim, Byung Chan; Hwang, Hee; Chae, Jong-Hee; Choi, Jieun; Kim, Ki Joong; Hwang, Yong Seung; Kim, Hunmin

2014-08-01

Intravenous levetiracetam (LEV) has been shown to be effective and safe in treating adults with refractory status epilepticus (SE). We sought to investigate the efficacy and safety of intravenous LEV for pediatric patients with refractory SE. We performed a retrospective medical-record review of pediatric patients who were treated with intravenous LEV for refractory SE. Clinical information regarding age, sex, seizure type, and underlying neurological status was collected. We evaluated other anticonvulsants that were used prior to administration of intravenous LEV and assessed loading dose, response to treatment, and any adverse events from intravenous LEV administration. Fourteen patients (8 boys and 6 girls) received intravenous LEV for the treatment of refractory SE. The mean age of the patients was 4.4 ± 5.5 years (range, 4 days to 14.6 years). Ten of the patients were neurologically healthy prior to the refractory SE, and the other 4 had been previously diagnosed with epilepsy. The mean loading dose of intravenous LEV was 26 ± 4.6 mg/kg (range, 20-30 mg/kg). Seizure termination occurred in 6 (43%) of the 14 patients. In particular, 4 (57%) of the 7 patients younger than 2 years showed seizure termination. No immediate adverse events occurred during or after infusions. The current study demonstrated that the adjunctive use of intravenous LEV was effective and well tolerated in pediatric patients with refractory SE, even in patients younger than 2 years. Intravenous LEV should be considered as an effective and safe treatment option for refractory SE in pediatric patients.

Neuroimaging in refractory epilepsy. Current practice and evolving trends

Energy Technology Data Exchange (ETDEWEB)

Ramli, N. [Department of Biomedical Imaging, University Malaya Research Imaging Centre (Malaysia); Rahmat, K., E-mail: katt_xr2000@yahoo.com [Department of Biomedical Imaging, University Malaya Research Imaging Centre (Malaysia); Lim, K.S.; Tan, C.T. [Neurology Unit, Department of Medicine, University Malaya, Kuala Lumpur (Malaysia)

2015-09-15

Highlights: • Neuroimaging is imperative in diagnostic work up and therapeutic assessment of refractory epilepsy. • Identification of epileptogenic zone on EEG, MRI and functional imaging improves the success of surgery. • High performance MRI greatly enhanced metabolic information and elucidate brain functions. • Optimisation of epilepsy protocols in structural and functional MRI are presented in this article. - Abstract: Identification of the epileptogenic zone is of paramount importance in refractory epilepsy as the success of surgical treatment depends on complete resection of the epileptogenic zone. Imaging plays an important role in the locating and defining anatomic epileptogenic abnormalities in patients with medically refractory epilepsy. The aim of this article is to present an overview of the current MRI sequences used in epilepsy imaging with special emphasis of lesion seen in our practices. Optimisation of epilepsy imaging protocols are addressed and current trends in functional MRI sequences including MR spectroscopy, diffusion tensor imaging and fusion MR with PET and SPECT are discussed.

Neuroimaging in refractory epilepsy. Current practice and evolving trends

International Nuclear Information System (INIS)

Ramli, N.; Rahmat, K.; Lim, K.S.; Tan, C.T.

2015-01-01

Highlights: • Neuroimaging is imperative in diagnostic work up and therapeutic assessment of refractory epilepsy. • Identification of epileptogenic zone on EEG, MRI and functional imaging improves the success of surgery. • High performance MRI greatly enhanced metabolic information and elucidate brain functions. • Optimisation of epilepsy protocols in structural and functional MRI are presented in this article. - Abstract: Identification of the epileptogenic zone is of paramount importance in refractory epilepsy as the success of surgical treatment depends on complete resection of the epileptogenic zone. Imaging plays an important role in the locating and defining anatomic epileptogenic abnormalities in patients with medically refractory epilepsy. The aim of this article is to present an overview of the current MRI sequences used in epilepsy imaging with special emphasis of lesion seen in our practices. Optimisation of epilepsy imaging protocols are addressed and current trends in functional MRI sequences including MR spectroscopy, diffusion tensor imaging and fusion MR with PET and SPECT are discussed

Human adipose cells in vitro are either refractory or responsive to insulin, reflecting host metabolic state.

Directory of Open Access Journals (Sweden)

Vladimir A Lizunov

Full Text Available While intercellular communication processes are frequently characterized by switch-like transitions, the endocrine system, including the adipose tissue response to insulin, has been characterized by graded responses. Yet here individual cells from adipose tissue biopsies are best described by a switch-like transition between the basal and insulin-stimulated states for the trafficking of the glucose transporter GLUT4. Two statistically-defined populations best describe the observed cellular heterogeneity, representing the fractions of refractive and responsive adipose cells. Furthermore, subjects exhibiting high systemic insulin sensitivity indices (SI have high fractions of responsive adipose cells in vitro, while subjects exhibiting decreasing SI have increasing fractions of refractory cells in vitro. Thus, a two-component model best describes the relationship between cellular refractory fraction and subject SI. Since isolated cells exhibit these different response characteristics in the presence of constant culture conditions and milieu, we suggest that a physiological switching mechanism at the adipose cellular level ultimately drives systemic SI.

Evidence for Tocilizumab as a Treatment Option in Refractory Uveitis Associated with Juvenile Idiopathic Arthritis.

Science.gov (United States)

Tappeiner, Christoph; Mesquida, Marina; Adán, Alfredo; Anton, Jordi; Ramanan, Athimalaipet V; Carreno, Ester; Mackensen, Friederike; Kotaniemi, Kaisu; de Boer, Joke H; Bou, Rosa; de Vicuña, Carmen García; Heiligenhaus, Arnd

2016-12-01

To report on experience using the anti-interleukin 6 receptor antibody tocilizumab (TCZ) to treat severe and therapy-refractory uveitis associated with juvenile idiopathic arthritis (JIA). Retrospective data were gathered from patients with JIA receiving TCZ treatment for uveitis. JIA and related uveitis data (disease onset, activity, structural complications, and topical and systemic antiinflammatory treatment) were evaluated at the start of TCZ (baseline) and every 3 months during TCZ therapy. A total of 17 patients (14 women) with active uveitis were included (mean age 15.3 ± 6.9 yrs, mean followup time 8.5 mos). In all patients, uveitis had been refractory to previous topical and systemic corticosteroids, methotrexate (MTX), and other synthetic and biological disease-modifying antirheumatic drugs, including ≥ 1 tumor necrosis factor-α (TNF-α) inhibitor. Uveitis inactivity was achieved in 10 patients after a mean of 5.7 months of TCZ treatment (in 3 of them, it recurred during followup) and persisted in the remaining 7 patients. By using TCZ, systemic corticosteroids or immunosuppressives could be spared in 7 patients. Macular edema was present in 5 patients at baseline and improved in all of them under TCZ treatment. Arthritis was active in 11 patients at the initial and in 6 at the final followup visit. TCZ appears to represent a therapeutic option for severe JIA-associated uveitis that has been refractory to MTX and TNF-α inhibitors in selected patients. The present data indicate that inflammatory macular edema responds well to TCZ in patients with JIA-associated uveitis.

The effect of various sintering temperature on used refractory towards its physical properties

Science.gov (United States)

Sudibyo; Wulandari, Y. R.; Amin, M.; Azhar

2018-01-01

The used magnesia refractory from the kiln of cement industry was successfully recycled to new refractory using Kaolin as an adhesive. In this work, the temperatures of sintering were varied from 1000°C to 1500°C. The result shows that the increment temperature effects in sintering process will enhance refractory physical properties such as bulk density, cold crushing strength or pressure strength and thermal conductivity. Meanwhile, the porosity was decreased as the increase of the sintering temperature.

Rapid pacing results in changes in atrial but not in ventricular refractoriness

NARCIS (Netherlands)

Schoonderwoerd, BA; Van Gelder, IC; Tieleman, RG; Bel, KJ; Crijns, HJGM

It is well known that atrial tachycardia causes atrial electrical remodeling, characterized by shortening of atrial effective refractory periods (AERPs) and loss of physiological adaptation of AERP to rate. However, the nature and time course of changes in ventricular effective refractory periods

Spanish consensus for the management of patients with advanced radioactive iodine refractory differentiated thyroid cancer.

Science.gov (United States)

Riesco-Eizaguirre, Garcilaso; Galofré, Juan Carlos; Grande, Enrique; Zafón Llopis, Carles; Ramón y Cajal Asensio, Teresa; Navarro González, Elena; Jiménez-Fonseca, Paula; Santamaría Sandi, Javier; Gómez Sáez, José Manuel; Capdevila, Jaume

2016-04-01

Approximately one third of the patients with differentiated thyroid cancer (DTC) who develop structurally-evident metastatic disease are refractory to radioactive iodine (RAI). Most deaths from thyroid cancer occur in these patients. The main objective of this consensus is to address the most controversial aspects of management of these patients. On behalf of the Spanish Society of Endocrinology & Nutrition (SEEN) and the Spanish Group for Orphan and Infrequent Tumors (GETHI), the Spanish Task Force for Thyroid Cancer, consisting of endocrinologists and oncologists, reviewed the relevant literature and prepared a series of clinically relevant questions related to management of advanced RAI-refractory DTC. Ten clinically relevant questions were identified by the task force. In answering to these 10 questions, the task force included recommendations regarding the best definition of refractoriness; the best therapeutic options including watchful waiting, local therapies, and systemic therapy (e.g. kinase inhibitors), when sodium iodide symporter (NIS) restoration may be expected; and how recent advances in molecular biology have increased our understanding of the disease. In response to our appointment as a task force by the SEEN and GHETI, we developed a consensus to help in clinical management of patients with advanced RAI-refractory DTC. We think that this consensus will provide helpful and current recommendations that will help patients with this disorder to get optimal medical care. Copyright © 2015 SEEN. Published by Elsevier España, S.L.U. All rights reserved.

Transient refractory material dissolution by a volumetrically-heated melt

Energy Technology Data Exchange (ETDEWEB)

Seiler, Jean Marie, E-mail: jean-marie.seiler@cea.fr [CEA, DEN, DTN, 17 Rue des Martyrs, 38054 Grenoble Cedex 9 (France); Ratel, Gilles [CEA, DEN, DTN, 17 Rue des Martyrs, 38054 Grenoble Cedex 9 (France); Combeau, Hervé [Institut Jean Lamour, UMR 7198, Lorraine University, Ecole des Mines de Nancy, Parc de Saurupt, 54042 Nancy Cedex (France); Gaus-Liu, Xiaoyang; Kretzschmar, Frank; Miassoedov, Alexei [Karlsruhe Institut of Technology, Hermann-von-Helmholtz-Platz 1, 76344 Eggenstein-Leopoldshafen (Germany)

2014-12-15

Highlights: • We describe a test investigating ceramic dissolution by a molten non-eutectic melt. • The evolution of the interface temperature between melt and refractory is measured. • A theoretical model describing dissolution kinetics is proposed. • When dissolution stops, interface temperature is the liquidus temperature of the melt. - Abstract: The present work addresses the question of corium–ceramic interaction in a core catcher during a core-melt accident in a nuclear power plant. It provides an original insight into transient aspects concerning dissolution of refractory material by a volumetrically heated pool. An experiment with simulant material (LIVECERAM) is presented. Test results clearly show that dissolution of solid refractory material can occur in a non-eutectic melt at a temperature which is lower than the melting temperature of the refractory material. During the dissolution transient, the interface temperature rises above the liquidus temperature, corresponding to the instantaneous average composition of the melt pool. With constant power dissipation in the melt and external cooling of the core-catcher, a final steady-state situation is reached. Dissolution stops when the heat flux (delivered by the melt to the refractory) can be removed by conduction through the residual thickness of the ceramic, with T{sub interface} = T{sub liquidus} (calculated for the average composition of the final liquid pool). The final steady state corresponds to a uniform pool composition and uniform interface temperature distribution. Convection in the pool is governed by natural thermal convection and the heat flux distribution is therefore similar to what would be obtained for a single component pool. An interpretation of the experiment with two model-based approaches (0D and 1D) is presented. The mass transfer kinetics between the interface and the bulk is controlled by a diffusion sublayer within the boundary layer. During the dissolution transient

Surgical treatment for medically refractory focal epilepsy in a patient with fragile X syndrome.

Science.gov (United States)

Kenmuir, Cynthia; Richardson, Mark; Ghearing, Gena

2015-10-01

Medication resistant temporal lobe epilepsy occurs in a small population of patients with fragile X syndrome. We present the case of a 24-year-old man with medically refractory temporal lobe epilepsy and fragile X syndrome who underwent left anterior temporal lobectomy resulting in cessation of seizures. Our patient was diagnosed with fragile X syndrome with a fully mutated, fully methylated FMR1 gene resulting in 572 CGG repeats. He developed seizures initially controlled with Depakote monotherapy, but progressed to become medically refractive to combination treatment with Depakote, lamotrigine and zonisamide. Prolonged video EEG monitoring revealed interictal left temporal sharp waves and slowing as well as subclinical and clinical seizures, each with left temporal onset. 3T MRI was consistent with left mesial temporal sclerosis. After discussing the case in our multidisciplinary surgical epilepsy conference, he was referred for presurgical evaluation including neuropsychological testing and Wada testing. He underwent an asleep left anterior temporal lobectomy, sparing the superior temporal gyrus. Pathology showed neuronal loss and gliosis in the hippocampus and amygdala. Twelve months after surgery, the patient has not experienced a seizure. He is described by his parents as less perseverative and less restless. We have presented the case of a 24 year-old-man with fragile X syndrome who underwent successful left anterior temporal lobectomy for the treatment of medically refractory epilepsy who is now seizure free without further functional impairment. This case report demonstrates the feasibility of surgical treatment for a patient with comorbid fragile X syndrome and mesial temporal sclerosis. Copyright © 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

A randomized controlled trial of the ketogenic diet in refractory childhood epilepsy

NARCIS (Netherlands)

Lambrechts, D.A.J.E.; de Kinderen, R.J.A.; Vles, J.S.H.; de Louw, A.J.A.; Aldenkamp, A.P.; Majoie, H.J.M.

2017-01-01

Objective: To evaluate the efficacy and tolerability of the ketogenic diet (KD) during the first 4 months of a randomized controlled trial (RCT) in refractory epilepsy patients aged 1–18 years. Methods: Children and adolescents with refractory epilepsy, not eligible for epilepsy surgery, were

Ixabepilone: a new chemotherapeutic option for refractory metastatic breast cancer

Directory of Open Access Journals (Sweden)

Shannon Puhalla

2008-09-01

Full Text Available Shannon Puhalla, Adam BrufskyUPMC Magee-Womens Cancer Program, University of Pittsburgh, Pittsburgh, Pennsylvania, USAAbstract: Taxane therapy is commonly used in the treatment of metastatic breast cancer. However, most patients will eventually become refractory to these agents. Ixabepilone is a newly approved chemotherapeutic agent for the treatment of metastatic breast cancer. Although it targets microtubules similarly to docetaxel and paclitaxel, ixabepilone has activity in patients that are refractory to taxanes. This review summarizes the pharmacology of ixapebilone and clinical trials with the drug both as a single agent and in combination. Data were obtained using searches of PubMed and abstracts of the annual meetings of the American Society of Clinical Oncology and the San Antonio Breast Cancer Symposium from 1995 to 2008. Ixapebilone is a semi-synthetic analog of epothilone B that acts to induce apoptosis of cancer cells via the stabilization of microtubules. Phase I clinical trials have employed various dosing schedules ranging from daily to weekly to 3-weekly. Dose-limiting toxicites included neuropathy and neutropenia. Responses were seen in a variety of tumor types. Phase II studies verified activity in taxane-refractory metastatic breast cancer. The FDA has approved ixabepilone for use as monotherapy and in combination with capecitabine for the treatment of metastatic breast cancer. Ixabepilone is an efficacious option for patients with refractory metastatic breast cancer. The safety profile is similar to that of taxanes, with neuropathy and neutropenia being dose-limiting. Studies are ongoing with the use of both iv and oral formulations and in combination with other chemotherapeutic and biologic agents.Keywords: ixabepilone, epothilone, metastatic breast cancer, taxane-refractory

Clinical outcomes of intravenous immunoglobulin therapy in refractory uveitis.

Science.gov (United States)

Garcia-Geremias, M; Carreño, E; Epps, S J; Lee, R W J; Dick, A D

2015-04-01

Intravenous immunoglobulin (IVIg) therapy has multiple mechanisms of immunomodulatory action. We wished therefore to assess its efficacy in a spectrum of patients with refractory uveitis. Retrospective review of clinical charts was conducted to document response to IVIg treatment in consecutive patients with treatment-refractory uveitis. Main outcome measures were control of intraocular inflammation, visual acuity, progression of the disease, and complications. Four (two male) patients, with a mean age at the beginning of the treatment of 47 years (range: 39-64), were included in the study. Indication for treatment was patients with active non-infectious uveitis refractory to steroids and immunomodulatory therapy. All patients received a course of 0.5 g/kg per day of IVIg for three consecutive days, repeating this course at a mean of 11 week (range: 2-39 weeks) intervals when indicated clinically. The median duration of the IVIg therapy was 7 months (range: 3-14 months). In three patients treatment resulted in stabilisation and prevention of progression of the disease, and additionally in two patients it facilitated a decrease in prednisolone dose. Treatment failed to induce long-term remission in one patient with recurrence of macular oedema. IVIg was well tolerated with neither immediate nor longer-term adverse events observed. In three out of four cases IVIg was an effective adjunctive therapy and well tolerated for the management of treatment-refractory uveitis.

Advances in refractories for the metallurgical industries III: proceedings of the international symposium

Energy Technology Data Exchange (ETDEWEB)

Allaire, C.; Rigaud, M. [eds.] [Ecole Polytchnique, Montreal, PQ (Canada)

1999-07-01

In the three years since the last symposium key innovations have been made in the penetration of monoliths in all sectors of the metallurgical industries. The symposium was presented within the 38th Annual Conference of Metallurgists of the Canadian Institute of Mining, Metallurgy and Petroleum. Broad international coverage was maintained with papers originating from 12 different countries including: Australia, Canada, China, France, Germany, India, Iran, Japan, Korea, Mexico, New Zealand, Norway, and the United States. The 27 papers presented are divided into seven sessions to reflect the interests of the participants. The sessions included mold fluxes I and II, aluminum refractories, alumina castables, andalusite castables, continuous casting of refractories, aluminum refractories II and copper-nickel refineries. One paper on refractories for exhaust gas scrubbers is abstracted separately.

Evaluation of refractory materials for a nuclear waste incinerator

International Nuclear Information System (INIS)

Grotzky, V.K.; Kneale, P.A.; Teter, A.R.

1980-01-01

An experiment to find a suitable refractory lining for a nuclear waste incinerator has been completed. Eleven brick and six castable products were analyzed by optical and scanning microscopy. All the materials were fashioned into cup shapes and subjected to temperatures ranging from 800 to 1200 0 C for as long as six weeks. Some of the cups were charged weekly with pellets made from ash materials that would contact an incinerator liner. Refractory products containing a high percentage of aluminum oxide had the greatest resistance to cracking and slag buildup. 35 figures

Refractory status epilepticus and glutamic acid decarboxylase antibodies in adults: presentation, treatment and outcomes.

Science.gov (United States)

Khawaja, Ayaz M; Vines, Brannon L; Miller, David W; Szaflarski, Jerzy P; Amara, Amy W

2016-03-01

Glutamic acid decarboxylase antibodies (GAD-Abs) have been implicated in refractory epilepsy. The association with refractory status epilepticus in adults has been rarely described. We discuss our experience in managing three adult patients who presented with refractory status epilepticus associated with GAD-Abs. Case series with retrospective chart and literature review. Three patients without pre-existing epilepsy who presented to our institution with generalized seizures between 2013 and 2014 were identified. Seizures proved refractory to first and second-line therapies and persisted beyond 24 hours. Patient 1 was a 22-year-old female who had elevated serum GAD-Ab titres at 0.49 mmol/l (normal: status epilepticus. Causation cannot be established since GAD-Abs may be elevated secondary to concurrent autoimmune diseases or formed de novo in response to GAD antigen exposure by neuronal injury. Based on this report and available literature, there may be a role for immuno- and chemotherapy in the management of refractory status epilepticus associated with GAD-Abs.

Benchmarking infrastructure for mutation text mining.

Science.gov (United States)

Klein, Artjom; Riazanov, Alexandre; Hindle, Matthew M; Baker, Christopher Jo

2014-02-25

Experimental research on the automatic extraction of information about mutations from texts is greatly hindered by the lack of consensus evaluation infrastructure for the testing and benchmarking of mutation text mining systems. We propose a community-oriented annotation and benchmarking infrastructure to support development, testing, benchmarking, and comparison of mutation text mining systems. The design is based on semantic standards, where RDF is used to represent annotations, an OWL ontology provides an extensible schema for the data and SPARQL is used to compute various performance metrics, so that in many cases no programming is needed to analyze results from a text mining system. While large benchmark corpora for biological entity and relation extraction are focused mostly on genes, proteins, diseases, and species, our benchmarking infrastructure fills the gap for mutation information. The core infrastructure comprises (1) an ontology for modelling annotations, (2) SPARQL queries for computing performance metrics, and (3) a sizeable collection of manually curated documents, that can support mutation grounding and mutation impact extraction experiments. We have developed the principal infrastructure for the benchmarking of mutation text mining tasks. The use of RDF and OWL as the representation for corpora ensures extensibility. The infrastructure is suitable for out-of-the-box use in several important scenarios and is ready, in its current state, for initial community adoption.

Benchmarking infrastructure for mutation text mining

Science.gov (United States)

2014-01-01

Background Experimental research on the automatic extraction of information about mutations from texts is greatly hindered by the lack of consensus evaluation infrastructure for the testing and benchmarking of mutation text mining systems. Results We propose a community-oriented annotation and benchmarking infrastructure to support development, testing, benchmarking, and comparison of mutation text mining systems. The design is based on semantic standards, where RDF is used to represent annotations, an OWL ontology provides an extensible schema for the data and SPARQL is used to compute various performance metrics, so that in many cases no programming is needed to analyze results from a text mining system. While large benchmark corpora for biological entity and relation extraction are focused mostly on genes, proteins, diseases, and species, our benchmarking infrastructure fills the gap for mutation information. The core infrastructure comprises (1) an ontology for modelling annotations, (2) SPARQL queries for computing performance metrics, and (3) a sizeable collection of manually curated documents, that can support mutation grounding and mutation impact extraction experiments. Conclusion We have developed the principal infrastructure for the benchmarking of mutation text mining tasks. The use of RDF and OWL as the representation for corpora ensures extensibility. The infrastructure is suitable for out-of-the-box use in several important scenarios and is ready, in its current state, for initial community adoption. PMID:24568600

An unusual case of refractory wheeze

Science.gov (United States)

Ramesh, Venkat; Acharya, Vishak; Pai, Narasimha; Krishnan, Ananda

2015-01-01

A 37-year-old man presented with a history of episodic wheeze and breathlessness of 3 years’ duration refractory to treatment. Physical examination revealed diffuse expiratory polyphonic rhonchi while the remainder of the examination including the cardiac examination was reported as normal. Pulmonary function testing revealed mild obstruction with bronchodilator reversibility. The patient was discharged on a 6-month course of antitubercular treatment (ATT) as bronchial brush cytology (obtained via bronchoscopy) was positive for acid-fast bacilli. The patient presented after completing 6 months of ATT with persistent symptoms, a loud S1 and a mid-diastolic murmur at the apex. High-resolution CT of the chest showed bilateral dependent ground glass opacities. An echocardiogram revealed a left atrial myxoma, and normal RV size and pressures. The patient underwent successful surgical removal of the same, and made a complete recovery. Refractory wheeze is a very unusual presentation of a left atrial myxoma. PMID:25733086

Severe nivolumab-induced pneumonitis preceding durable clinical remission in a patient with refractory, metastatic lung squamous cell cancer: a case report

Directory of Open Access Journals (Sweden)

Hong Li

2017-02-01

Full Text Available Abstract Background Programmed cell death 1 (PD-1 and its ligand 1 (PD-L1 inhibitors have quickly become standard of care for patients with advanced non-small cell lung cancer and increasing numbers of other cancer types. In this report, we discuss the clinical history, pathological evaluation, and genomic findings in a patient with metastatic lung squamous cell cancer (SCC who developed severe nivolumab-induced pneumonitis preceding durable clinical remission after three doses of nivolumab. Case presentation A patient with chemotherapy-refractory, metastatic lung SCC developed symptomatic pneumonitis by week 4 after nivolumab treatment, concurrently with onset of a potent antitumor response. Despite discontinuation of nivolumab after three doses and the use of high dose oral corticosteroids for grade 3 pneumonitis, continued tumor response to a complete remission by 3 months was evident by radiographic assessment. At the time of this submission, the patient has remained in clinical remission for 14 months. High PD-L1 expression by immunohistochemistry staining was seen in intra-alveolar macrophages and viable tumor cells in the pneumonitis and recurrent tumor specimens, respectively. Tumor genomic profiling by FoundationOne targeted exome sequencing revealed a very high tumor mutation burden (TMB corresponding to 95–96 percentile in lung SCC, i.e., 87.4–91.0 and 82.9 mut/Mb, respectively, in pre- and post-nivolumab tumor specimens. Except for one, the 13 functional genomic alterations remained the same in the diagnostic, recurrent, and post-treatment, relapsed tumor specimens, suggesting that nivolumab reset the patient’s immune system against one or more preexisting tumor-associated antigens (TAAs. One potential TAA candidate is telomerase reverse transcriptase (TERT in which an oncogenic promoter -146C>T mutation was detected. Human leukocyte antigen (HLA typing revealed HLA-A*0201 homozygosity, which is the prevalent HLA class I

Systemic mastocytosis uncommon in KIT D816V mutation positive core-binding factor acute myeloid leukemia

DEFF Research Database (Denmark)

Kristensen, Thomas; Preiss, Birgitte; Broesby-Olsen, Sigurd

2012-01-01

Abstract The KIT D816V mutation is detected in the vast majority of adult cases of systemic mastocytosis (SM). The mutation is also frequently detected in core-binding factor acute myeloid leukemia (CBF-AML) defined by the presence of t(8;21)(q22;q22); RUNX1-RUNX1T1 or inv(16)(p13.1;q22)/t(16;16)(p...

Preparation and characterization of clay bonded high strength silica refractory by utilizing agriculture waste

International Nuclear Information System (INIS)

Bhardwaj, A.; Hossain, S.K.S.; Majh, M.R.

2017-01-01

Clay bonded silica refractory was prepared by utilizing agriculture waste called rice husk ash (RHA) and refractory grog. Various samples were prepared with different compositions based upon partial replacement of quartz by RHA. Rectangular samples were prepared by following semi dry process prior to pressing in a uniaxial hydraulic press and sintering at a temperature of 1200°C in air atmosphere. Various physical, mechanical and thermal characterizations were done like X-ray diffraction (XRD), scanning electron microscope (SEM), apparent porosity (AP), bulk density (BD), cold crushing strength (CCS), refractoriness and thermal conductivity measurement. The sample utilizing 30% of RHA was considered most optimum composition which produced cold crushing strength of 38MPa and thermal conductivity of 2.08W/mK at 800°C with a considerable good refractoriness. Enhancement in the mechanical as well as thermal properties may be considered as attributed to the amorphous silica which has reacted more easily and efficiently with other material surrounding giving rise to the densification and produced stable crystalline phase to the refractory material. These promising characteristics suggests that the RHA may lead to be used as a potential material for the preparation of clay bonded high strength silica refractories. [es

Enhancement of the life of refractories through the operational experience of plasma torch melter

Energy Technology Data Exchange (ETDEWEB)

Moon, Young Pyo [Technology Institute, Korea Radioactive waste Agency (KORAD), Daejeon (Korea, Republic of); Choi, Jaang Young [Chungnam National University, Daejeon (Korea, Republic of)

2016-06-15

The properties of wastes for melting need to be considered to minimize the maintenance of refractory and to discharge the molten slags smoothly from a plasma torch melter. When the nonflammable wastes from nuclear facilities such as concrete debris, glass, sand, etc., are melted, they become acid slags with low basicity since the chemical composition has much more acid oxides than basic oxides. A molten slag does not have good characteristics of discharge and is mainly responsible for the refractory erosion due to its low liquidity. In case of a stationary plasma torch melter with a slant tapping port on the wall, a fixed amount of molten slags remains inside of tapping hole as well as the melter inside after tapping out. Nonmetallic slags keep the temperature higher than melting point of metal because metallic slags located on the bottom of melter by specific gravity difference are simultaneously melted when dual mode plasma torch operates in transferred mode. In order to minimize the refractory erosion, the compatible refractories are selected considering the temperature inside the melter and the melting behavior of slags whether to contact or noncontact with molten slags. An acidic refractory shall not be installed in adjacent to a basic refractory for the resistibility against corrosion.

Refractory heartburn: comparison of intercellular space diameter in documented GERD vs. functional heartburn.

Science.gov (United States)

Vela, Marcelo F; Craft, Brandon M; Sharma, Neeraj; Freeman, Janice; Hazen-Martin, Debra

2011-05-01

Refractory heartburn despite acid suppression may be explained by ongoing gastroesophageal reflux disease (GERD) or functional heartburn (FH), i.e., symptoms without evidence of GERD. Impedance-pH monitoring (impedance-pH) detects acid and nonacid reflux and is useful for evaluating acid-suppressed, refractory patients. Intercellular space diameter (ISD) of esophageal epithelium measured by transmission electron microscopy (TEM) is a marker of epithelial damage present in both erosive and nonerosive reflux disease. ISD has not been used to study refractory heartburn or FH. Our aim was to compare ISD in healthy controls and refractory heartburn patients with GERD and FH. In refractory heartburn patients (heartburn more than twice/week for at least 2 months despite proton pump inhibitor (PPI) b.i.d.), erosive esophagitis and/or abnormal impedance-pH (increased acid exposure or positive symptom index) defined GERD; normal esophagogastroduodenoscopy (EGD)/impedance-pH defined FH. Asymptomatic, healthy controls had normal EGD and pH-metry. Mean ISD in each subject, determined by blinded TEM of esophageal biopsies, was the average of 100 measurements (10 measurements in each of 10 micrographs). In all, 11 healthy controls, 11 FH, and 15 GERD patients were studied. Mean ISD was significantly higher in GERD compared with controls (0.87 vs. 0.32 μm, P=0.003) and FH (0.87 vs. 0.42 μm, P=0.012). Mean ISD was similar in FH and controls (0.42 vs. 0.32 μm, P=0.1). The proportion of patients with abnormal ISD was significantly higher for GERD compared with FH (60 vs. 9%, P=0.014). ISD is increased in refractory heartburn patients with GERD but not those with FH. Our findings suggest that measurement of ISD by TEM might be a useful tool to distinguish GERD from FH in patients with refractory heartburn.

Regional cerebral blood flow abnormalities in late-life depression. Relation to refractoriness and chronification

Energy Technology Data Exchange (ETDEWEB)

Awata, Shuichi; Konno, Michiko; Sato, Mitsumoto [Tohoku Univ., Sendai (Japan). School of Medicine; Ito, Hiroshi; Ono, Shuichi; Kawashima, Ryuta; Fukuda, Hiroshi

1998-02-01

We examined patterns of regional cerebral blood flow (rCBF) abnormalities in 18 patients with major depressive disorder in late life using single photon emission computed tomography (SPECT) and {sup 99m}Tc-hexamethyl-propylenamine oxime ({sup 99m}Tc-HMPAO). Compared with 13 age-matched controls, relative rCBF was significantly decreased bilaterally in the anterior cingulate gyrus, the prefrontal cortex, the temporal cortex, the parietal cortex, the hippocampus and the caudate nucleus. However, it was not correlated with the severity of depression or global cognitive dysfunction. In 10 patients with a prolonged depressive episode or prolonged residual symptoms (the refractory subgroup), robust and extensive decreases in rCBF were found compared with controls and the rCBF decreased significantly in the anterior cingulate gyrus and the prefrontal cortex compared with that in the non-refractory subgroup. In the non-reflactory subgroup, rCBF decreased significantly in the caudate nucleus and tended to decrease in the anterior cingulate gyrus compared with controls. These findings indicate that dysfunction of the limbic system, the cerebral association cortex and the caudate nucleus may be implicated in late-life depression and that robust and extensive hypoperfusion, especially in the anterior cingulate and the prefrontal regions, may relate to refractoriness or chronification of depression. (author). 60 refs.

Regional cerebral blood flow abnormalities in late-life depression. Relation to refractoriness and chronification

International Nuclear Information System (INIS)

Awata, Shuichi; Konno, Michiko; Sato, Mitsumoto; Ito, Hiroshi; Ono, Shuichi; Kawashima, Ryuta; Fukuda, Hiroshi

1998-01-01

We examined patterns of regional cerebral blood flow (rCBF) abnormalities in 18 patients with major depressive disorder in late life using single photon emission computed tomography (SPECT) and 99m Tc-hexamethyl-propylenamine oxime ( 99m Tc-HMPAO). Compared with 13 age-matched controls, relative rCBF was significantly decreased bilaterally in the anterior cingulate gyrus, the prefrontal cortex, the temporal cortex, the parietal cortex, the hippocampus and the caudate nucleus. However, it was not correlated with the severity of depression or global cognitive dysfunction. In 10 patients with a prolonged depressive episode or prolonged residual symptoms (the refractory subgroup), robust and extensive decreases in rCBF were found compared with controls and the rCBF decreased significantly in the anterior cingulate gyrus and the prefrontal cortex compared with that in the non-refractory subgroup. In the non-reflactory subgroup, rCBF decreased significantly in the caudate nucleus and tended to decrease in the anterior cingulate gyrus compared with controls. These findings indicate that dysfunction of the limbic system, the cerebral association cortex and the caudate nucleus may be implicated in late-life depression and that robust and extensive hypoperfusion, especially in the anterior cingulate and the prefrontal regions, may relate to refractoriness or chronification of depression. (author). 60 refs

The induction of somatic mutations by high-LET radiation observed using the Drosophila assay system

International Nuclear Information System (INIS)

Yoshikawa, Isao; Takatsuji, Toshihiro; Nagano, Masaaki; Hoshi, Masaharu; Takada, Jun; Endo, Satoru

1999-01-01

To evaluate the mutagenic potential of high-LET radiation, an analysis was made on the production of somatic mutations by 252 Cf fission neutron s and heavy particle ions accelerated by a synchrotron. A Drosophila strain that allows simultaneous detection of two types of mutations in an identical fly was constructed. One was a wing-hair mutation and the other was an eye-color mosaic spot mutation. Measurements were made using a combined assay system of both mutation assays. Larvae were exposed to radiation at the age of post-ovipositional day-3. The efficiency of 252 Cf neutrons for inducing wing-hair mosaic spots was very high, the relative biological effectiveness (RBE) = 8.5, but the efficiency for eye-color mosaic spot was nearly equal (RBE = 1.2) to that of 137 Cs γ-rays. The RBE of carbon ions for inducing wing-hair mosaic spots increased as an increase in LET values. The RBE for the induction of eye-color mutants did not change with LET. These relationships suggest that more complex types of DNA damages such as non-rejoinable strand break or clustered double strand break, which increase with LET may be responsible for the induction of wing-hair mutation, while simpler forms of molecular damage may induce a reversion in the white-ivory allele. (M.N.)

Destruction of Refractory Carbon in Protoplanetary Disks

Energy Technology Data Exchange (ETDEWEB)

Anderson, Dana E.; Blake, Geoffrey A. [Division of Geological and Planetary Sciences, California Institute of Technology, 1200 E. California Blvd., Pasadena, CA 91125 (United States); Bergin, Edwin A. [Department of Astronomy, University of Michigan, 1085 S. University, Ann Arbor, MI 48109-1107 (United States); Ciesla, Fred J. [Department of Geophysical Sciences, The University of Chicago, 5734 South Ellis Ave., Chicago, IL 60637 (United States); Visser, Ruud [European Southern Observatory, Karl-Schwarzschild-Str. 2, D-85748, Garching (Germany); Lee, Jeong-Eun [School of Space Research, Kyung Hee University, 1732, Deogyeong-daero, Giheung-gu, Yongin-si, Gyeonggi-do 17104 (Korea, Republic of)

2017-08-10

The Earth and other rocky bodies in the inner solar system contain significantly less carbon than the primordial materials that seeded their formation. These carbon-poor objects include the parent bodies of primitive meteorites, suggesting that at least one process responsible for solid-phase carbon depletion was active prior to the early stages of planet formation. Potential mechanisms include the erosion of carbonaceous materials by photons or atomic oxygen in the surface layers of the protoplanetary disk. Under photochemically generated favorable conditions, these reactions can deplete the near-surface abundance of carbon grains and polycyclic aromatic hydrocarbons by several orders of magnitude on short timescales relative to the lifetime of the disk out to radii of ∼20–100+ au from the central star depending on the form of refractory carbon present. Due to the reliance of destruction mechanisms on a high influx of photons, the extent of refractory carbon depletion is quite sensitive to the disk’s internal radiation field. Dust transport within the disk is required to affect the composition of the midplane. In our current model of a passive, constant- α disk, where α = 0.01, carbon grains can be turbulently lofted into the destructive surface layers and depleted out to radii of ∼3–10 au for 0.1–1 μ m grains. Smaller grains can be cleared out of the planet-forming region completely. Destruction may be more effective in an actively accreting disk or when considering individual grain trajectories in non-idealized disks.

Influence of molybdenum impurity on the electronic properties of refractory phases

International Nuclear Information System (INIS)

Ivanovskij, A.L.

1992-01-01

Results of calculations of electronic structure modification of refractory phases - TiC, TiN - during dissolution in their volume of molybdenum as the element, dominating in the processes of formation of ring transition area (K-phase) are presented. It is shown that reconstruction of local state density (LSD) of Mo in the system Ni:Mo reflects the effects of impurity state and nickel valency area hybridization. LSD of Mo in MoC, MoN acquires quite a new form

Use of ustekinumab for severe refractory atopic dermatitis in a young teenager.

Science.gov (United States)

Wlodek, C; Hewitt, H; Kennedy, C T

2016-08-01

When conventional systemic immunosuppressive treatments fail in the setting of severe eczema, unlike in psoriasis, there are limited treatment options and only anecdotal evidence to help guide clinicians. There is a growing body of evidence for the use of certain biologic agents for moderate to severe eczema. We report the youngest case to date successfully and safely treated with ustekinumab for severe refractory atopic dermatitis. © 2016 British Association of Dermatologists.

Colectomy for refractory constipation

DEFF Research Database (Denmark)

Raahave, Dennis; Loud, Franck Bjørn; Christensen, Elsebeth

2010-01-01

OBJECTIVE: This study evaluated the type of colectomy, postoperative complications, functional results, and satisfaction in patients with constipation refractory to conservative therapy. Further, colonic transit time (CTT), faecal load (coprostasis), and colon length (redundancies) were compared...... had at hemicolectomy, 11 patients a subtotal colectomy and 3 patients an ileostomy. Two patients had an anastomotic leak and one died. In 11 patients, further surgery was necessary, because of recurrent constipation. Abdominal pain disappeared and defecation patterns improved significantly to 1-4 per...

Identification of novel mutations including a double mutation in patients with inherited cardiomyopathy by a targeted sequencing approach using the Ion Torrent PGM system.

Science.gov (United States)

Zhao, Yue; Cao, Hong; Song, Yindi; Feng, Yue; Ding, Xiaoxue; Pang, Mingjie; Zhang, Yunmei; Zhang, Hong; Ding, Jiahuan; Xia, Xueshan

2016-06-01

Inherited cardiomyopathy is the major cause of sudden cardiac death (SCD) and heart failure (HF). The disease is associated with extensive genetic heterogeneity; pathogenic mutations in cardiac sarcomere protein genes, cytoskeletal protein genes and nuclear envelope protein genes have been linked to its etiology. Early diagnosis is conducive to clinical monitoring and allows for presymptomatic interventions as needed. In the present study, the entire coding sequences and flanking regions of 12 major disease (cardiomyopathy)-related genes [namely myosin, heavy chain 7, cardiac muscle, β (MYH7); myosin binding protein C, cardiac (MYBPC3); lamin A/C (LMNA); troponin I type 3 (cardiac) (TNNI3); troponin T type 2 (cardiac) (TNNT2); actin, α, cardiac muscle 1 (ACTC1); tropomyosin 1 (α) (TPM1); sodium channel, voltage gated, type V alpha subunit (SCN5A); myosin, light chain 2, regulatory, cardiac, slow (MYL2); myosin, heavy chain 6, cardiac muscle, α (MYH6); myosin, light chain 3, alkali, ventricular, skeletal, slow (MYL3); and protein kinase, AMP-activated, gamma 2 non-catalytic subunit  (PRKAG2)] in 8 patients with dilated cardiomyopathy (DCM) and in 8 patients with hypertrophic cardiomyopathy (HCM) were amplified and then sequenced using the Ion Torrent Personal Genome Machine (PGM) system. As a result, a novel heterozygous mutation (MYH7, p.Asn885Thr) and a variant of uncertain significance (TNNT2, p.Arg296His) were identified in 2 patients with HCM. These 2 missense mutations, which were absent in the samples obtained from the 200 healthy control subjects, altered the amino acid that was evolutionarily conserved among a number of vertebrate species; this illustrates that these 2 non-synonymous mutations play a role in the pathogenesis of HCM. Moreover, a double heterozygous mutation (PRKAG2, p.Gly100Ser plus MYH7, p.Arg719Trp) was identified in a patient with severe familial HCM, for the first time to the best of our

Method for hot pressing irregularly shaped refractory articles

Science.gov (United States)

Steinkamp, William E.; Ballard, Ambrose H.

1982-01-01

The present invention is directed to a method for hot pressing irregularly haped refractory articles with these articles of varying thickness being provided with high uniform density and dimensional accuracy. Two partially pressed compacts of the refractory material are placed in a die cavity between displaceable die punches having compact-contacting surfaces of the desired article configuration. A floating, rotatable block is disposed between the compacts. The displacement of the die punches towards one another causes the block to rotate about an axis normal to the direction of movement of the die punches to uniformly distribute the pressure loading upon the compacts for maintaining substantially equal volume displacement of the powder material during the hot pressing operation.

Clinical and etiological profile of refractory rickets from western India.

Science.gov (United States)

Joshi, Rajesh R; Patil, Shailesh; Rao, Sudha

2013-07-01

To present clinical and etiological profile of refractory rickets from Mumbai. Case records of 36 patients presenting over 2½ y with refractory rickets were evaluated with respect to clinical presentation, biochemical, radiological features and where needed, ophthalmological examination, ultrasonography and special tests on blood and urine. Twenty three (63 %) patients had renal tubular acidosis (RTA)-distal RTA in 20 and proximal RTA in 3 patients; 5 (14 %) had vitamin D dependent rickets (VDDR I in 2 and VDDR II in 3 patients), 4 (11 %) had chronic renal failure (CRF) and 2 each (6 %) had hypophosphatemic rickets and chronic liver disease as cause of refractory rickets. A significant proportion of patients with RTA and VDDR showed skeletal changes of rickets in the first 2 y of life, while those with hypophosphatemic rickets presented later. Patients with hypophosphatemic rickets had predominant involvement of lower limbs, normal blood calcium and PTH levels and phosphorus leak in urine. All patients with RTA presented with failure to thrive, polyuria and marked rickets; blood alkaline phosphatase levels being normal in almost 50 % patients. Three (75 %) patients with rickets due to CRF had GFR rickets inspite of taking high dose of vitamin D orally. Refractory rickets is a disorder of multiple etiologies; a good history and clinical examination supplemented with appropriate investigations helps to determine its cause.

USE OF STRUCTURAL MRI IN PATIENTS WITH MEDICALLY REFRACTORY SEIZURES

Directory of Open Access Journals (Sweden)

Ara G. Kaprelyan

2012-12-01

Full Text Available Introduction: Refractory epilepsy is common in patients with structural brain lesions including acquired disorders and genetic abnormalities. Recently, MRI is a precise diagnostic tool for recognition of different structural causes underlying medically intractable seizures.Objective: To evaluate the usefulness of MRI for detection of brain lesions associated with refractory epilepsy.Material and methods: 49 patients (20M and 29F; aged 48.6±24.7 years with refractory epilepsy were included in the study. They presented with partial (46.0%, secondary (31.0% or primary (23.0% generalized tonic-clonic seizures. Clinical diagnosis was based on the revised criteria of ILAE. Structural neuroimaging (MRI, EEG recording, and neurological examination were performed.Results: MRI detected different structural brain abnormalities totally in 36 (73.5% patients, including cerebral tumors (21p, cerebrovascular accidents (5p, hyppocampal sclerosis (3p, developmental malformations (2p, postencephalitic lesions (2p, arachnoid cysts (2p, and tuberous sclerosis (1p. Neuroimaging revealed normal findings in 13 (27.5% cases. EEG recordings showed focal epileptic activity in 38 (77.6% patients, including 33 cases with and 5 without structural brain abnormalities.Conclusion: This study revealed that structural brain lesions are commonly associated with refractory epilepsy. We suggested that MRI is a useful diagnostic method for assessment of patients with uncontrolled seizures or altered epileptic pattern.

Penetration and Effectiveness of Micronized Copper in Refractory Wood Species.

Science.gov (United States)

Civardi, Chiara; Van den Bulcke, Jan; Schubert, Mark; Michel, Elisabeth; Butron, Maria Isabel; Boone, Matthieu N; Dierick, Manuel; Van Acker, Joris; Wick, Peter; Schwarze, Francis W M R

2016-01-01

The North American wood decking market mostly relies on easily treatable Southern yellow pine (SYP), which is being impregnated with micronized copper (MC) wood preservatives since 2006. These formulations are composed of copper (Cu) carbonate particles (CuCO3·Cu(OH)2), with sizes ranging from 1 nm to 250 μm, according to manufacturers. MC-treated SYP wood is protected against decay by solubilized Cu2+ ions and unreacted CuCO3·Cu(OH)2 particles that successively release Cu2+ ions (reservoir effect). The wood species used for the European wood decking market differ from the North American SYP. One of the most common species is Norway spruce wood, which is poorly treatable i.e. refractory due to the anatomical properties, like pore size and structure, and chemical composition, like pit membrane components or presence of wood extractives. Therefore, MC formulations may not suitable for refractory wood species common in the European market, despite their good performance in SYP. We evaluated the penetration effectiveness of MC azole (MCA) in easily treatable Scots pine and in refractory Norway spruce wood. We assessed the effectiveness against the Cu-tolerant wood-destroying fungus Rhodonia placenta. Our findings show that MCA cannot easily penetrate refractory wood species and could not confirm the presence of a reservoir effect.

Wettability between TiN,TiC Containing Carbon Composite Refractory and Molten Slag or Hot Metal

Institute of Scientific and Technical Information of China (English)

SHIYue－xun; LIYingand; 等

1994-01-01

In order to develop a new-type TiC-TiN containing carbon composite refractory so as to improve the service life of blast furnace hearth,the wettability between the carbon refractory and molten slag or metal has been mea-sured.It was indicated that the carbon refractory is wet-ted by slag(θ≤90°） when(TiC+TiN)>33.52%,The effects of TiN or TiC on wetting behavior are basi-cally identical.When the amount of TiC in the carbon com-posite refractory is greater than 60% it will be wetted by hot metal;therefore,the carbon composite refractory will be wetted by slag but not permeated by hot metal when the amount of TiC is restricted.

Nuclear Space Power Systems Materials Requirements

International Nuclear Information System (INIS)

Buckman, R.W. Jr.

2004-01-01

High specific energy is required for space nuclear power systems. This generally means high operating temperatures and the only alloy class of materials available for construction of such systems are the refractory metals niobium, tantalum, molybdenum and tungsten. The refractory metals in the past have been the construction materials selected for nuclear space power systems. The objective of this paper will be to review the past history and requirements for space nuclear power systems from the early 1960's through the SP-100 program. Also presented will be the past and present status of refractory metal alloy technology and what will be needed to support the next advanced nuclear space power system. The next generation of advanced nuclear space power systems can benefit from the review of this past experience. Because of a decline in the refractory metal industry in the United States, ready availability of specific refractory metal alloys is limited

Refractory Hypertension: Determination of Prevalence, Risk Factors and Comorbidities in a Large, Population-Based Cohort

Science.gov (United States)

Calhoun, David A.; Booth, John N.; Oparil, Suzanne; Irvin, Marguerite R.; Shimbo, Daichi; Lackland, Daniel T.; Howard, George; Safford, Monika M.; Muntner, Paul

2014-01-01

Refractory hypertension is an extreme phenotype of antihypertensive treatment failure. Participants in the REasons for Geographic And Racial Differences in Stroke (REGARDS) Study, a large (n=30,239), population-based cohort were evaluated to determine the prevalence of refractory hypertension and associated cardiovascular risk factors and comorbidities. Refractory hypertension was defined as uncontrolled blood pressure (systolic/diastolic ≥ 140/90 mm Hg) on ≥ 5 antihypertensive drug classes. Participants with resistant hypertension (systolic/diastolic ≥140/90 mm Hg on ≥ 3 orhypertensive participants served as comparator groups. Of 14,809 REGARDS participants receiving antihypertensive treatment, 78 (0.5%) had refractory hypertension. The prevalence of refractory hypertension was 3.6% among participants with resistant hypertension(n=2,144) and 41.7% among participants on 5 or more antihypertensive drug classes. Among all hypertensive participants, African American race, male gender, living in the stroke belt or buckle, higher body mass index, lower heart rate, reduced estimated glomerular filtration rate, albuminuria, diabetes and history of stroke and coronary heart disease were associated with refractory hypertension. Compared to resistant hypertension, prevalence ratios for refractory hypertension were increased for African Americans (3.00, 95% CI 1.68 – 5.37) and those with albuminuria (2.22, 95% CI 1.40 – 3.52) and diabetes (2.09, 95% CI 1.32 – 3.31). The median 10-year Framingham risk for coronary heart disease and stroke was higher among participants with refractory hypertension compared to either comparator group. These data indicate that while resistant hypertension is relatively common among treated hypertensive patients, true antihypertensive treatment failure is rare. PMID:24324035

Super-refractory status epilepticus in West China.

Science.gov (United States)

Tian, L; Li, Y; Xue, X; Wu, M; Liu, F; Hao, X; Zhou, D

2015-07-01

This study aims to determine the general frequency, mortality, and risk factors of super-refractory status epilepticus (SRSE) versus non-refractory status epilepticus (NRSE) and refractory status epilepticus (RSE). This work is a retrospective study. Clinical data of patients who were diagnosed with status epilepticus (SE) in the neurological ward and neuro-intensive care unit of West China Hospital from January 2009 to December 2012 were collected and analyzed. A total of 98 patients were included in the study. The percentages of NRSE, RSE, and SRSE were 67.3%, 20.4%, and 12.2%, respectively. Convulsive SE was the main seizure type among the three groups. The most common cause of NRSE was related to epilepsy (EP). However, 67.7% of SRSE cases were caused by acute encephalitis. Moreover, 47% of SE and 40% of RSE cases had a history of EP, whereas only 8.3% of SRSE cases had such history (P  0.05). The general mortality of SE was 7.1%, whereas that of SRSE was 50%. During follow-up, most SRSE patients who survived have developed symptomatic EP. This study was the first to use the statistical percentage of SRSE. Approximately 12.2% of SE cases will result in SRSE, which is a challenging medical situation for doctors. Patients with first episodes and acute encephalitis were also prone to develop SRSE. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Refractory experience in circulating fluidized bed combustors, Task 7

Energy Technology Data Exchange (ETDEWEB)

Vincent, R.Q.

1989-11-01

This report describes the results of an investigation into the status of the design and selection of refractory materials for coal-fueled circulating fluidized-bed combustors. The survey concentrated on operating units in the United States manufactured by six different boiler vendors: Babcock and Wilcox, Combustion Engineering, Foster Wheeler, Keeler Dorr-Oliver, Pyropower, and Riley Stoker. Information was obtained from the boiler vendors, refractory suppliers and installers, and the owners/operators of over forty units. This work is in support of DOE's Clean Coal Technology program, which includes circulating fluidized-bed technology as one of the selected concepts being evaluated.

Refractory metal component technology for in-core sensor design

International Nuclear Information System (INIS)

Cannon, C.P.

1986-02-01

Within recent years, an increasing concern over reactor safety has prompted tests that characterize reactor core environments during transient conditions. Such tests include the Loss-of-Fluid-Tests (Idaho National Engineering Lab (INEL)), Severe Fuel Damage Tests (INEL), Core Debris Rubble Tests (Sandia National Laboratories (SNL)), and similar tests performed by foreign nations. The in-core sensors for these tests require refractory metal components to be compatible with electrical insulator materials as well as materials comprising highly corrosive service mediums. This paper presents the refractory metal technology utilized to provide basic sensor designs in the above mentioned reactor tests

Keratomycosis due to Tintelnotia destructans refractory to common therapy treated successfully with systemic and local terbinafine in combination with polyhexamethylene biguanide.

Science.gov (United States)

Behrens-Baumann, Wolfgang J; Hofmüller, Wolfram; Tammer, Ina; Tintelnot, Kathrin

2018-04-28

To report on a wearer of rigid gas-permeable contact lenses with a keratomycosis due to Tintelnotia-a new genus of Phaeosphaeriaceae-treated with terbinafine and polyhexamethylene biguanide. Chart review of a patient with fungal keratitis treated additionally with systemic and topical terbinafine 0.25% after symptoms increased under conventional antimycotic therapy with voriconazole. Antifungal susceptibility had been tested in vitro. After starting an additional treatment with systemic and topical terbinafine, the severe corneal infection was sufficiently resolved. The drug was well tolerated without any neurological, dermatological or gastroenterological problems. Terbinafine revealed a marked in vitro antifungal activity of 0.12 µg/ml. The fungus was identified as Tintelnotia destructans. Terbinafine might be considered as a therapeutic option in severe cases of fungal keratitis refractory to common antifungal therapy.

The HIVToolbox 2 web system integrates sequence, structure, function and mutation analysis.

Directory of Open Access Journals (Sweden)

David P Sargeant

Full Text Available There is enormous interest in studying HIV pathogenesis for improving the treatment of patients with HIV infection. HIV infection has become one of the best-studied systems for understanding how a virus can hijack a cell. To help facilitate discovery, we previously built HIVToolbox, a web system for visual data mining. The original HIVToolbox integrated information for HIV protein sequence, structure, functional sites, and sequence conservation. This web system has been used for almost 40,000 searches. We report improvements to HIVToolbox including new functions and workflows, data updates, and updates for ease of use. HIVToolbox2, is an improvement over HIVToolbox with new functions. HIVToolbox2 has new functionalities focused on HIV pathogenesis including drug-binding sites, drug-resistance mutations, and immune epitopes. The integrated, interactive view enables visual mining to generate hypotheses that are not readily revealed by other approaches. Most HIV proteins form multimers, and there are posttranslational modification and protein-protein interaction sites at many of these multimerization interfaces. Analysis of protease drug binding sites reveals an anatomy of drug resistance with different types of drug-resistance mutations regionally localized on the surface of protease. Some of these drug-resistance mutations have a high prevalence in specific HIV-1 M subtypes. Finally, consolidation of Tat functional sites reveals a hotspot region where there appear to be 30 interactions or posttranslational modifications. A cursory analysis with HIVToolbox2 has helped to identify several global patterns for HIV proteins. An initial analysis with this tool identifies homomultimerization of almost all HIV proteins, functional sites that overlap with multimerization sites, a global drug resistance anatomy for HIV protease, and specific distributions of some DRMs in specific HIV M subtypes. HIVToolbox2 is an open-access web application available at

Mechanical activation of ceramic powders and its influence on the quality of the refractory linings

International Nuclear Information System (INIS)

Ceganac, Z.; Acimovic, Z.; Andric, Lj.; Petrov, M.; Mihajlovic, S.

2003-01-01

In the paper we present results of the investigation of the influence of mechanical activation of ceramic powders-talc and alumina, on the quality of refractory linings for casting applications. It is shown that additional fine grinding of ceramic powders increases the quality of the refractory lining. Grinding and activation of the particles with various granulations contributes to the creation of the homogenous continuous layer of refractory lining on the sand moulds and cores, as well as on the evaporating polystyrene patterns for applications in the Lost foam casting process. Refractory linings obtained within the activation of particles procedure have better adherence to moulds, cores and evaporating patterns. (Original)

High Throughput Drug Sensitivity Assay and Genomics- Guided Treatment of Patients With Relapsed or Refractory Acute Leukemia

Science.gov (United States)

2018-02-28

Acute Leukemia of Ambiguous Lineage; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Refractory Acute Myeloid Leukemia; Refractory Adult Acute Lymphoblastic Leukemia; Refractory Childhood Acute Lymphoblastic Leukemia

Testing Consent Order on Refractory Ceramic Fibers

Science.gov (United States)

This notice announces that EPA has signed signed an enforceable testing consent order under the Toxic Substances Control Act (TSCA), 15 U.S.C. section 2601 at seq., with three of the primary producers of refractory ceramic fibers (RCF).

Improving outcomes of refractory celiac disease – current and emerging treatment strategies

Directory of Open Access Journals (Sweden)

Woodward J

2016-08-01

Full Text Available Jeremy Woodward Department of Gastroenterology and Clinical Nutrition, Addenbrooke’s Hospital, Cambridge, UK Abstract: Intestinal inflammation and symptoms of celiac disease (CD usually respond well to gluten withdrawal, but rare cases are refractory to diet. Two types of refractory CD are discriminated on the basis of the presence or absence of an atypical population of mucosal lymphocytes that may progress to enteropathy-associated T-cell lymphoma. Challenges remain in the secure diagnosis of both types of refractory disease, and evidence on which to base treatment recommendations is flawed by the small numbers of reported patients and the use of different diagnostic strategies. Recent advances in our understanding of the mechanisms of the condition in conjunction with the development of immunomodulatory agents for managing other inflammatory diseases are helping to shape future approaches to targeted therapy. Progression will depend on collaboration and recruitment to trials. In the meantime, there is evidence to suggest that earlier diagnosis and better follow-up and management of CD may prevent the development of refractoriness. Keywords: celiac disease, gluten, small intestine, lymphoma, lymphocytes

Service life prediction for refractory materials

Czech Academy of Sciences Publication Activity Database

Boccaccini, D. N.; Cannio, M.; Volkov-Husović, T. D.; Kamseu, E.; Romagnoli, M.; Veronesi, P.; Leonelli, C.; Dlouhý, Ivo; Boccaccini, A. R.

2008-01-01

Roč. 43, č. 12 (2008), s. 4079-4090 ISSN 0022-2461 R&D Projects: GA ČR(CZ) GA106/05/0495 Institutional research plan: CEZ:AV0Z20410507 Keywords : refractory materials * ultrasonic velocity * fracture toughness Subject RIV: JH - Ceramics, Fire-Resistant Materials and Glass Impact factor: 1.181, year: 2008

Refractory chronic cluster headache

DEFF Research Database (Denmark)

Mitsikostas, Dimos D; Edvinsson, Lars; Jensen, Rigmor H

2014-01-01

Chronic cluster headache (CCH) often resists to prophylactic pharmaceutical treatments resulting in patients' life damage. In this rare but pragmatic situation escalation to invasive management is needed but framing criteria are lacking. We aimed to reach a consensus for refractory CCH definition...... for clinical and research use. The preparation of the final consensus followed three stages. Internal between authors, a larger between all European Headache Federation members and finally an international one among all investigators that have published clinical studies on cluster headache the last five years...

Study on the efficiency of ceramic coating for avoiding oxidation in carbon refractories

International Nuclear Information System (INIS)

Santos, I.M.G.; Cruz Junior, F.; Paskocimas, C.A.; Leite, E.R.; Longo, Elson; Varela, J.A.

1997-01-01

A ceramic coating made of sodium phosphossilicate and clay was developed to the protection of refractories against carbon oxidation during the pre-heating of siderurgical equipment. This search has the objective of comparing the refractory behaviour with and without coating, according to temperature, time and atmosphere. The results show that the coating is more efficient at higher temperatures. An important point is that the efficiency is smaller after long thermal is that the efficiency is smaller after long thermal treatments and at very aggressive conditions. In spite of this the oxidation is still smaller than in refractory without coating. (author)

Second-hit mosaic mutation in mTORC1 repressor DEPDC5 causes focal cortical dysplasia-associated epilepsy.

Science.gov (United States)

Ribierre, Théo; Deleuze, Charlotte; Bacq, Alexandre; Baldassari, Sara; Marsan, Elise; Chipaux, Mathilde; Muraca, Giuseppe; Roussel, Delphine; Navarro, Vincent; Leguern, Eric; Miles, Richard; Baulac, Stéphanie

2018-04-30

DEP domain-containing 5 protein (DEPDC5) is a repressor of the recently recognized amino acid-sensing branch of the mTORC1 pathway. So far, its function in the brain remains largely unknown. Germline loss-of-function mutations in DEPDC5 have emerged as a major cause of familial refractory focal epilepsies, with case reports of sudden unexpected death in epilepsy (SUDEP). Remarkably, a fraction of patients also develop focal cortical dysplasia (FCD), a neurodevelopmental cortical malformation. We therefore hypothesized that a somatic second-hit mutation arising during brain development may support the focal nature of the dysplasia. Here, using postoperative human tissue, we provide the proof of concept that a biallelic 2-hit - brain somatic and germline - mutational mechanism in DEPDC5 causes focal epilepsy with FCD. We discovered a mutation gradient with a higher rate of mosaicism in the seizure-onset zone than in the surrounding epileptogenic zone. Furthermore, we demonstrate the causality of a Depdc5 brain mosaic inactivation using CRISPR-Cas9 editing and in utero electroporation in a mouse model recapitulating focal epilepsy with FCD and SUDEP-like events. We further unveil a key role of Depdc5 in shaping dendrite and spine morphology of excitatory neurons. This study reveals promising therapeutic avenues for treating drug-resistant focal epilepsies with mTORC1-targeting molecules.

Determination of D816V mutation in the c-kIt gene in the Slovenian patients with acute myeloid leukemia and systemic mastocytosis

Directory of Open Access Journals (Sweden)

Martina Fink

2012-12-01

Full Text Available Background: D816V mutation in the C-KIT gene is present in more than 90 % of patients with systemic mastocytosis (SM and 2–7 % of patients with acute myeloid leukemia (AML. D816V mutation is caused by the substitution of adenine with thymine at 2447 nucleotide sequence in the C-KIT gene. This nucleotide substitution causes the replacment of aspartate acid by valine at codon 816 of the KIT protein. KIT protein with D816V mutation acts as constitutively active tyrosine kinase that promotes cell proliferation and inhibits apoptosis. The purpose of our study was to determine the incidence of D816V mutation in the C-KIT gene in Slovenian patients with AML and in patients with suspected systemic mastocytosis. Patients and methods: In the retrospective study, 71 patients with AML and 25 patients with suspected systemic mastocytosis were included. D816V mutation in the C-KIT gene was determined by polymerase chain reaction (PCR and the resulting PCR products were analyzed by agarose gel electrophoresis. Results: D816V mutation in KIT protein was determined in 7 % of patients with AML and in 32 % patients with suspected systemic mastocytosis. Conclusions: Identification of D816V mutation in the C-KIT gene must always be performed in patients with suspected systemic mastocytosis. The determination of this mutation contributes to the diagnosis and treatment selection. The finding of D816V mutation in the C-KIT gene in patients with AML and concomitant genetic modifications RUNX-RUNX1T1 (typical translocation t(8; 21 (q22, q22 or CBFB-MYH11, which is the result of inversion on chromosome 16–(inv (16 (p13, q22, however, indicates a faster, more aggressive course of the disease and predicts a worse outcome. The finding of the mutation in other patients with AML may indicate the presence of concomitant AML and SM, which was not found in our patients.

SLC6A1 Mutation and Ketogenic Diet in Epilepsy With Myoclonic-Atonic Seizures.

Science.gov (United States)

Palmer, Samantha; Towne, Meghan C; Pearl, Phillip L; Pelletier, Renee C; Genetti, Casie A; Shi, Jiahai; Beggs, Alan H; Agrawal, Pankaj B; Brownstein, Catherine A

2016-11-01

Epilepsy with myoclonic-atonic seizures, also known as myoclonic-astatic epilepsy or Doose syndrome, has been recently linked to variants in the SLC6A1 gene. Epilepsy with myoclonic-atonic seizures is often refractory to antiepileptic drugs, and the ketogenic diet is known for treating medically intractable seizures, although the mechanism of action is largely unknown. We report a novel SLC6A1 variant in a patient with epilepsy with myoclonic-atonic seizures, analyze its effects, and suggest a mechanism of action for the ketogenic diet. We describe a ten-year-old girl with epilepsy with myoclonic-atonic seizures and a de novo SLC6A1 mutation who responded well to the ketogenic diet. She carried a c.491G>A mutation predicted to cause p.Cys164Tyr amino acid change, which was identified using whole exome sequencing and confirmed by Sanger sequencing. High-resolution structural modeling was used to analyze the likely effects of the mutation. The SLC6A1 gene encodes a transporter that removes gamma-aminobutyric acid from the synaptic cleft. Mutations in SLC6A1 are known to disrupt the gamma-aminobutyric acid transporter protein 1, affecting gamma-aminobutyric acid levels and causing seizures. The p.Cys164Tyr variant found in our study has not been previously reported, expanding on the variants linked to epilepsy with myoclonic-atonic seizures. A 10-year-old girl with a novel SLC6A1 mutation and epilepsy with myoclonic-atonic seizures had an excellent clinical response to the ketogenic diet. An effect of the diet on gamma-aminobutyric acid reuptake mediated by gamma-aminobutyric acid transporter protein 1 is suggested. A personalized approach to epilepsy with myoclonic-atonic seizures patients carrying SLC6A1 mutation and a relationship between epilepsy with myoclonic-atonic seizures due to SLC6A1 mutations, GABAergic drugs, and the ketogenic diet warrants further exploration. Copyright © 2016 Elsevier Inc. All rights reserved.

Mixed Uranium/Refractory Metal Carbide Fuels for High Performance Nuclear Reactors

International Nuclear Information System (INIS)

Knight, Travis; Anghaie, Samim

2002-01-01

Single phase, solid-solution mixed uranium/refractory metal carbides have been proposed as an advanced nuclear fuel for advanced, high-performance reactors. Earlier studies of mixed carbides focused on uranium and either thorium or plutonium as a fuel for fast breeder reactors enabling shorter doubling owing to the greater fissile atom density. However, the mixed uranium/refractory carbides such as (U, Zr, Nb)C have a lower uranium densities but hold significant promise because of their ultra-high melting points (typically greater than 3700 K), improved material compatibility, and high thermal conductivity approaching that of the metal. Various compositions of (U, Zr, Nb)C were processed with 5% and 10% metal mole fraction of uranium. Stoichiometric samples were processed from the constituent carbide powders, while hypo-stoichiometric samples with carbon-to-metal (C/M) ratios of 0.92 were processed from uranium hydride, graphite, and constituent refractory carbide powders. Processing techniques of cold uniaxial pressing, dynamic magnetic compaction, sintering, and hot pressing were investigated to optimize the processing parameters necessary to produce high density (low porosity), single phase, solid-solution mixed carbide nuclear fuels for testing. This investigation was undertaken to evaluate and characterize the performance of these mixed uranium/refractory metal carbides for high performance, ultra-safe nuclear reactor applications. (authors)

Identifying pathways affected by cancer mutations.

Science.gov (United States)

Iengar, Prathima

2017-12-16

Mutations in 15 cancers, sourced from the COSMIC Whole Genomes database, and 297 human pathways, arranged into pathway groups based on the processes they orchestrate, and sourced from the KEGG pathway database, have together been used to identify pathways affected by cancer mutations. Genes studied in ≥15, and mutated in ≥10 samples of a cancer have been considered recurrently mutated, and pathways with recurrently mutated genes have been considered affected in the cancer. Novel doughnut plots have been presented which enable visualization of the extent to which pathways and genes, in each pathway group, are targeted, in each cancer. The 'organismal systems' pathway group (including organism-level pathways; e.g., nervous system) is the most targeted, more than even the well-recognized signal transduction, cell-cycle and apoptosis, and DNA repair pathway groups. The important, yet poorly-recognized, role played by the group merits attention. Pathways affected in ≥7 cancers yielded insights into processes affected. Copyright © 2017 Elsevier Inc. All rights reserved.

Approaches to refractory epilepsy

Directory of Open Access Journals (Sweden)

Jerome Engel

2014-01-01

Full Text Available Epilepsy is one of the most common serious neurological conditions, and 30 to 40% of people with epilepsy have seizures that are not controlled by medication. Patients are considered to have refractory epilepsy if disabling seizures continue despite appropriate trials of two antiseizure drugs, either alone or in combination. At this point, patients should be referred to multidisciplinary epilepsy centers that perform specialized diagnostic testing to first determine whether they are, in fact, pharmacoresistant, and then, if so, offer alternative treatments. Apparent pharmacoresistance can result from a variety of situations, including noncompliance, seizures that are not epileptic, misdiagnosis of the seizure type or epilepsy syndrome, inappropriate use of medication, and lifestyle issues. For patients who are pharmacoresistant, surgical treatment offers the best opportunity for complete freedom from seizures. Surgically remediable epilepsy syndromes have been identified, but patients with more complicated epilepsy can also benefit from surgical treatment and require more specialized evaluation, including intracranial EEG monitoring. For patients who are not surgical candidates, or who are unwilling to consider surgery, a variety of other alternative treatments can be considered, including peripheral or central neurostimulation, ketogenic diet, and complementary and alternative approaches. When such alternative treatments are not appropriate or effective, quality of life can still be greatly improved by the psychological and social support services offered by multidisciplinary epilepsy centers. A major obstacle remains the fact that only a small proportion of patients with refractory epilepsy are referred for expert evaluation and treatment.

Refractory overactive bladder: Beyond oral anticholinergic therapy

Science.gov (United States)

Glinski, Ronald W.; Siegel, Steven

2007-01-01

Objectives: In this review, we discuss the treatment of refractory overactive bladder (OAB) that has not adequately responded to medication therapy and we propose an appropriate care pathway to the treatment of OAB. We also attempt to address the cost of OAB treatments. Materials and Methods: A selective expert review of the current literature on the subject of refractory OAB using MEDLINE was performed and the data is summarized. We also review our experience in treating refractory OAB. The role and outcomes of various treatment options for refractory OAB are discussed and combined therapy with oral anticholinergics is explored. Emerging remedies including intravesical botulinum toxin injection and pudendal neuromodulation are also reviewed, along with conventional surgical options. Results: In general behavioral therapy, pelvic floor electrical stimulation, magnetic therapy and posterior tibial nerve stimulation (PTNS), have shown symptom decreases in 50-80% of patients with OAB. Depending on the study, combination therapy with oral anticholinergics seems to improve efficacy of behavioral therapy and PTNS in approximately 10-30%. In multicenter, long-term randomized controlled trials, sacral neuromodulation has been shown to improve symptoms of OAB and OAB incontinence in up to 80% of the patients treated. Studies involving emerging therapies such as pudendal serve stimulation suggest that there may be a 15-20% increase in efficacy over sacral neuromodulation, but long-term studies are not yet available. Another emerging therapy, botulinum toxin, is also showing similar success in reducing OAB symptoms in 80-90% of patients. Surgical approaches, such as bladder augmentation, are a last resort in the treatment of OAB and are rarely used at this point unless upper tract damage is a concern and all other treatment options have been exhausted. Conclusion: The vast majority of OAB patients can be managed successfully by behavioral options with or without

Refractory overactive bladder: Beyond oral anticholinergic therapy

Directory of Open Access Journals (Sweden)

Ronald W Glinski

2007-01-01

Full Text Available Objectives: In this review, we discuss the treatment of refractory overactive bladder (OAB that has not adequately responded to medication therapy and we propose an appropriate care pathway to the treatment of OAB. We also attempt to address the cost of OAB treatments. Materials and Methods: A selective expert review of the current literature on the subject of refractory OAB using MEDLINE was performed and the data is summarized. We also review our experience in treating refractory OAB. The role and outcomes of various treatment options for refractory OAB are discussed and combined therapy with oral anticholinergics is explored. Emerging remedies including intravesical botulinum toxin injection and pudendal neuromodulation are also reviewed, along with conventional surgical options. Results: In general behavioral therapy, pelvic floor electrical stimulation, magnetic therapy and posterior tibial nerve stimulation (PTNS, have shown symptom decreases in 50-80% of patients with OAB. Depending on the study, combination therapy with oral anticholinergics seems to improve efficacy of behavioral therapy and PTNS in approximately 10-30%. In multicenter, long-term randomized controlled trials, sacral neuromodulation has been shown to improve symptoms of OAB and OAB incontinence in up to 80% of the patients treated. Studies involving emerging therapies such as pudendal serve stimulation suggest that there may be a 15-20% increase in efficacy over sacral neuromodulation, but long-term studies are not yet available. Another emerging therapy, botulinum toxin, is also showing similar success in reducing OAB symptoms in 80-90% of patients. Surgical approaches, such as bladder augmentation, are a last resort in the treatment of OAB and are rarely used at this point unless upper tract damage is a concern and all other treatment options have been exhausted. Conclusion: The vast majority of OAB patients can be managed successfully by behavioral options with or

The induction of somatic mutations by high-LET radiations using the drosophila assay system

International Nuclear Information System (INIS)

Yoshikawa, Isao; Takatsuji, Toshihiro

2004-01-01

Two types of somatic mutation in Drosophila melanogaster were examined to evaluate the relative biological effectiveness (RBE) of 252 Cf neutrons and heavy ions (carbon ions and neon ions) accelerated with a synchrotron for inducing mutations as a function of linear energy transfer (LET). One is the loss of heterozygosity for wing-hair mutations and the other the reversion of the mutant white-ivory. The measurements were made using a combined mutation assay system; so that induced mutant wing-hair clones as well as revertant eye-color clones could be detected simultaneously in the same fly. Larvae were irradiated at the age of 3 days post-oviposition. The efficiency of 252 Cf neutrons for inducing wing-hair mosaic spots is very high, RBE=8.5, but that for eye-color mosaic spot is almost equal (RBE=1.2) to that of 137 Cs γ-rays. RBE-LET relationships were obtained for the induction of wing-hair and eye-color mosaic spots. The RBE of carbon and neon ions for producing wing-hair mosaic spots increased with increasing LET values. The RBE for the induction of eye-color mutants did not change with LET. These relationships suggest that more complex types of DNA damage such as non-rejoinable strand breaks or clustered double strand breaks that increase with LET may be responsible for inducing the wing-hair mutation, while simpler forms of molecular damage may induce reversion in the white-ivory allele. (author)

Bone hydatid disease refractory to nitazoxanide treatment

NARCIS (Netherlands)

Schipper, Hans G.; Simsek, Suat; van Agtmael, Michiel A.; van Lienden, Krijn P.

2009-01-01

We report a patient with bone hydatid disease that was refractory to both long-term daily treatment with albendazole, combined with cimetidine or administered as monotherapy ( approximately 15 years) and a relatively short course of nitazoxanide combined with albendazole (3 months). Despite

Hyperthermia-induced alteration of yeast susceptibility to mutation

International Nuclear Information System (INIS)

Mitchel, R.E.J.; Morrison, D.P.

1985-01-01

Diploid yeast (s. cerevisiae) were examined for alterations in susceptibility to induced mutation following hyperthermia treatment. In cells grown at 23 0 C, a non-lethal heat exposure (38 0 C, 30 min) markedly suppressed mutation induced by a subsequent non-killing dose of MNNG of MNU. Mutation by ENU, 8-MOP + UVA, or γ-rays was not affected. An intermediate level of mutation suppression was observed for mutation by 254nm UV or MMS. Mutation by MNNG was not suppressed by the same heat treatment delivered after the mutagen exposure. In a split dose experiment (two MNNG treatments separated by a heat exposure) no suppression of mutation was observed. Treatment with cycloheximide mimicked the effect of heat treatment. These data suggest that mutation induction by MNNG or MNU is protein synthesis dependent, i.e. an error-prone repair system is induced by exposure to MNNG or MNU but not by ENU, 8-MOP+UVA or γ-irradiation. We propose that hyperthermia treatment, by inducing stress protein synthesis at the expense of normal protein synthesis, precludes induction of this error-prone system. Therefore, in heat treated cells, DNA lesions produced by MNNG or MNU exposure must be resolved by an essentially constitutive system which is less error-prone than the inducible one

Morphometric brain characterization of refractory obsessive-compulsive disorder: diffeomorphic anatomic registration using exponentiated Lie algebra.

Science.gov (United States)

Tang, Wanjie; Li, Bin; Huang, Xiaoqi; Jiang, Xiaoyu; Li, Fei; Wang, Lijuan; Chen, Taolin; Wang, Jinhui; Gong, Qiyong; Yang, Yanchun

2013-10-01

Few studies have used neuroimaging to characterize treatment-refractory obsessive-compulsive disorder (OCD). This study sought to explore gray matter structure in patients with treatment-refractory OCD and compare it with that of healthy controls. A total of 18 subjects with treatment-refractory OCD and 26 healthy volunteers were analyzed by MRI using a 3.0-T scanner and voxel-based morphometry (VBM). Diffeomorphic anatomical registration using exponentiated Lie algebra (DARTEL) was used to identify structural changes in gray matter associated with treatment-refractory OCD. A partial correlation model was used to analyze whether morphometric changes were associated with Yale-Brown Obsessive-Compulsive Scale scores and illness duration. Gray matter volume did not differ significantly between the two groups. Treatment-refractory OCD patients showed significantly lower gray matter density than healthy subjects in the left posterior cingulate cortex (PCC) and mediodorsal thalamus (MD) and significantly higher gray matter density in the left dorsal striatum (putamen). These changes did not correlate with symptom severity or illness duration. Our findings provide new evidence of deficits in gray matter density in treatment-refractory OCD patients. These patients may show characteristic density abnormalities in the left PCC, MD and dorsal striatum (putamen), which should be verified in longitudinal studies. © 2013. Published by Elsevier Inc. All rights reserved.

LINX®, a novel treatment for patients with refractory asthma complicated by gastroesophageal reflux disease: a case report.

Science.gov (United States)

Sriratanaviriyakul, Narin; Kivler, Celeste; Vidovszky, Tamas J; Yoneda, Ken Y; Kenyon, Nicholas J; Murin, Susan; Louie, Samuel

2016-05-24

Gastroesophageal reflux disease is one of the most common comorbidities in patients with asthma. Gastroesophageal reflux disease can be linked to difficult-to-control asthma. Current management includes gastric acid suppression therapy and surgical antireflux procedures. The LINX® procedure is a novel surgical treatment for patients with gastroesophageal reflux disease refractory to medical therapy. To the best of our knowledge, we report the first case of successful treatment of refractory asthma secondary to gastroesophageal reflux disease using the LINX® procedure. Our patient was a 22-year-old white woman who met the American Thoracic Society criteria for refractory asthma that had remained poorly controlled for 5 years despite progressive escalation to step 6 treatment as recommended by National Institutes of Health-National Asthma Education and Prevention Program guidelines, including high-dose oral corticosteroids, high-dose inhaled corticosteroid plus long-acting β2-agonist, leukotriene receptor antagonist, and monthly omalizumab. Separate trials with azithromycin therapy and roflumilast did not improve her asthma control, nor did bronchial thermoplasty help. Additional consultations with two other university health systems left the patient with few treatment options for asthma, which included cyclophosphamide. Instead, the patient underwent a LINX® procedure after failure of maximal medical therapy for gastroesophageal reflux disease with the additional aim of improving asthma control. After she underwent LINX® treatment, her asthma improved dramatically and was no longer refractory. She had normal exhaled nitric oxide levels and loss of peripheral eosinophilia after LINX® treatment. Prednisone was discontinued without loss of asthma control. The only immediate adverse effects due to the LINX® procedure were bloating, nausea, and vomiting. LINX® is a viable alternative to the Nissen fundoplication procedure for the treatment of patients with

Medical versus surgical treatment for refractory or recurrent peptic ulcer.

Science.gov (United States)

Gurusamy, Kurinchi Selvan; Pallari, Elena

2016-03-29

Refractory peptic ulcers are ulcers in the stomach or duodenum that do not heal after eight to 12 weeks of medical treatment or those that are associated with complications despite medical treatment. Recurrent peptic ulcers are peptic ulcers that recur after healing of the ulcer. Given the number of deaths due to peptic ulcer-related complications and the long-term complications of medical treatment (increased incidence of fracture), it is unclear whether medical or surgical intervention is the better treatment option in people with recurrent or refractory peptic ulcers. To assess the benefits and harms of medical versus surgical treatment for people with recurrent or refractory peptic ulcer. We searched the specialised register of the Cochrane Upper GI and Pancreatic Diseases group, the Cochrane Central Register of Controlled Trials (CENTRAL) in the Cochrane Library, MEDLINE, EMBASE, Science Citation Index Expanded, and trials registers until September 2015 to identify randomised trials and non-randomised studies, using search strategies. We also searched the references of included studies to identify further studies. We considered randomised controlled trials and non-randomised studies comparing medical treatment with surgical treatment in people with refractory or recurrent peptic ulcer, irrespective of language, blinding, or publication status for inclusion in the review. Two review authors independently identified trials and extracted data. We planned to calculate the risk ratio, mean difference, standardised mean difference, or hazard ratio with 95% confidence intervals using both fixed-effect and random-effects models with Review Manager 5 based on intention-to-treat analysis. We included only one non-randomised study published 30 years ago in the review. This study included 77 participants who had gastric ulcer and in whom medical therapy (histamine H2 receptor blockers, antacids, and diet) had failed after an average duration of treatment of 29 months. The

Sorption techniques for production of high purity refractory metals

International Nuclear Information System (INIS)

Shatalov, V.V.; Peganov, V.A.; Logvinenko, I.A.; Molchanova, T.V.

2004-01-01

A consideration is given to potentialities of sorption processes tot provide a high quality of refractory metal and their alloys when using hydrometallurgical methods for raw material processing. The efficiency of application of ion exchange technology is shown for complex solutions reprocessing for various types of polymetallic raw materials, among them uranium ores, enriched concentrates of refractory metal ores, intermediate products, waste solutions. Based on investigation results on the behaviour of elements in process solutions and the mechanism of their sorption and elution, the process of pure chemical compounds production are developed which provide thereafter manufacturing compact metals. The flowsheets developed are mastered on a commercial scale [ru

Low-temperature plasma spheroidizing of polydisperse powders of refractory materials

International Nuclear Information System (INIS)

Tsymbalist, M.M.; Rudenskaya, N.A.; Kuz'min, B.P.; Pan'kov, V.A.

2003-01-01

A model is developed for heating and melting of a spherical particle, when powder processing in low temperature plasma, with the aim of estimation of the dependence of the degree of fusion on particle size for various materials. Spheroidizing of various refractory material powders close in shape and size composition is experimentally performed. Experimental and calculation estimates of spheroidizing criteria for the materials studied are in a satisfactory agreement. The influence of basic physical properties of refractory materials and plasma processing parameters on the degree of particle spheroidizing is analyzed [ru

Effect of spinal cord stimulation on myocardial perfusion reserve in patients with refractory angina pectoris.

Science.gov (United States)

Saraste, Antti; Ukkonen, Heikki; Varis, Antti; Vasankari, Tuija; Tunturi, Satu; Taittonen, Markku; Rautakorpi, Pirkka; Luotolahti, Matti; Airaksinen, K E Juhani; Knuuti, Juhani

2015-04-01

Epidural spinal cord stimulation (SCS) provides symptom relief in refractory angina pectoris, but its mechanism of action remains incompletely understood. We studied effects of short-term SCS therapy on myocardial ischaemia tolerance, myocardial perfusion reserve (MPR), and endothelium-mediated vasodilatation induced by cold pressor test (CPT) in patients with refractory angina pectoris. We prospectively recruited 18 patients with refractory angina pectoris and studied them after implantation of SCS device at baseline before starting the therapy and after 3 weeks of continuous SCS therapy. Myocardial ischaemia was evaluated by dobutamine stress echocardiography. Global and regional myocardial blood flow (MBF) were measured using positron emission tomography and (15)O-water at rest, during adenosine stress, and in response to CPT. Systemic haemodynamics were comparable before and after 3 weeks of SCS at rest, during adenosine stress and during CPT. Appearance of angina pectoris induced by dobutamine stress was delayed after SCS therapy. Global MPR increased (P = 0.02) from 1.7 ± 0.6 at baseline to 2.0 ± 0.6 after 3-week SCS therapy. This was associated with a significant reduction in global MBF at rest and increase in MBF induced by adenosine in the ischaemic regions. Global MBF response to CPT was improved after SCS (0.27 ± 0.20 vs. 0.40 ± 0.15, P = 0.03). Short-term SCS therapy improved myocardial ischaemia tolerance, absolute MPR, and endothelium-mediated vasomotor function in refractory angina pectoris, indicating that this therapy can alleviate myocardial perfusion abnormalities in advanced CAD. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.

PMS2 gene mutation results in DNA mismatch repair system failure in a case of adult granulosa cell tumor.

Science.gov (United States)

Wang, Wen-Chung; Lee, Ya-Ting; Lai, Yen-Chein

2017-03-27

Granulosa cell tumors are rare ovarian malignancies. Their characteristics include unpredictable indolent growth with malignant potential and late recurrence. Approximately 95% are of adult type. Recent molecular studies have characterized the FOXL2 402C > G mutation in adult granulosa cell tumor. Our previous case report showed that unique FOXL2 402C > G mutation and defective DNA mismatch repair system are associated with the development of adult granulosa cell tumor. In this study, the DNA sequences of four genes, MSH2, MLH1, MSH6, and PMS2, in the DNA mismatch repair system were determined via direct sequencing to elucidate the exact mechanism for the development of this granulosa cell tumor. The results showed that two missense germline mutations, T485K and N775L, inactivate the PMS2 gene. The results of this case study indicated that although FOXL2 402C > G mutation determines the development of granulosa cell tumor, PMS2 mutation may be the initial driver of carcinogenesis. Immunohistochemistry-based tumor testing for mismatch repair gene expression may be necessary for granulosa cell tumors to determine their malignant potential or if they are part of Lynch syndrome.

Colonic exclusion and combined therapy for refractory constipation.

Science.gov (United States)

Peng, Hong-Yun; Xu, Ai-Zhong

2006-12-28

To investigate the therapeutic effectiveness of colonic exclusion and combined therapy for refractory constipation. Thirty-two patients with refractory constipation were randomly divided into treatment group (n = 14) and control group (n = 18). Fourteen patients in treatment group underwent colonic exclusion and end-to-side colorectal anastomosis. Eighteen patients in control group received subtotal colectomy and end-to-end colorectal anastomosis. The therapeutic effects of the operations were assessed by comparing the surgical time, incision length, volume of blood losses, hospital stay, recovery rate and complication incidence. All patients received long-term follow-up. All operations were successful and patients recovered fully after the operations. In comparison of treatment group and control group, the surgical time (h), incision length (cm), volume of blood losses (mL), hospital stay (d) were 87 +/- 16 min vs 194 +/- 23 min (t = 9.85), 10.4 +/- 0.5 cm vs 21.2 +/- 1.8 cm (t = 14.26), 79.5 +/- 31.3 mL vs 286.3 +/- 49.2 mL (t = 17.24), and 11.8 +/- 2.4 d vs 18.6 +/- 2.6 d (t = 6.91), respectively (P 0.05), 21.4% vs 33.3% (P = 0.73 > 0.05), respectively. Colonic exclusion has better therapeutic efficacy on refractory constipation. It has many advantages such as shorter surgical time, smaller incision, fewer blood losses and shorter hospital stay.

The Effects of Mg/Si on the Exoplanetary Refractory Oxygen Budget

Science.gov (United States)

Unterborn, Cayman T.; Panero, Wendy R.

2017-08-01

Solar photospheric abundances of refractory elements mirror the Earth’s to within ˜10 mol% when normalized to the dominant terrestrial-planet-forming elements Mg, Si, and Fe. This allows for the adoption of solar composition as an order-of-magnitude proxy for Earth’s. It is not known, however, the degree to which this mirroring of stellar and terrestrial planet abundances holds true for other star-planet systems without determination of the composition of initial planetesimals via condensation sequence calculations and post condensation processes. We present the open-source Arbitrary Composition Condensation Sequence calculator (ArCCoS) to assess how the elemental composition of a parent star affects that of the planet-building material, including the extent of oxidation within the planetesimals. We demonstrate the utility of ArCCoS by showing how variations in the abundance of the stellar refractory elements Mg and Si affect the condensation of oxygen, a controlling factor in the relative proportions of planetary core and silicate mantle material. This thereby removes significant degeneracy in the interpretation of the structures of exoplanets, as well as provides observational tests for the validity of this model.

Lightweight cordierite–mullite refractories with low coefficients of ...

Indian Academy of Sciences (India)

of thermal conductivity and high mechanical properties ... compositions and microstructures of lightweight refractories were measured by X-ray .... of matrices were determined by calibration with EDAX ZAF .... Guzman I Ya 2003 Glass Ceram.

Antipsychotic Polypharmacy in a Treatment-Refractory Schizophrenia Population Receiving Adjunctive Treatment With Electroconvulsive Therapy

DEFF Research Database (Denmark)

Kristensen, Diana; Hageman, Ida; Bauer, Jeanett

2013-01-01

Antipsychotic polypharmacy (APP) is frequent, but its pattern is unknown in treatment-refractory schizophrenia-spectrum patients receiving electroconvulsive therapy (ECT).......Antipsychotic polypharmacy (APP) is frequent, but its pattern is unknown in treatment-refractory schizophrenia-spectrum patients receiving electroconvulsive therapy (ECT)....

Morphological differentiation of severe aplastic anaemia from hypocellular refractory cytopenia of childhood

DEFF Research Database (Denmark)

Baumann, Irith; Führer, Monika; Behrendt, Sonja

2012-01-01

To evaluate the reproducibility and reliability of the histomorphological criteria differentiating severe aplastic anaemia (SAA) and hypoplastic refractory cytopenia of childhood (RCC), the most frequently acquired hypocellular bone marrow conditions of childhood.......To evaluate the reproducibility and reliability of the histomorphological criteria differentiating severe aplastic anaemia (SAA) and hypoplastic refractory cytopenia of childhood (RCC), the most frequently acquired hypocellular bone marrow conditions of childhood....

Refractory Depression, Fatigue, Irritable Bowel Syndrome, and Chronic Pain: A Functional Medicine Case Report.

Science.gov (United States)

Plotnikoff, Gregory; Barber, Melissa

2016-01-01

Single-disorder or single-organ-system clinical practice guidelines are often of limited usefulness in guiding effective management of patients with chronic multidimensional signs and symptoms. The presence of multiple long-standing medical problems in a given patient despite intensive medical effort suggests that addressing systemic core imbalances could complement more narrowly focused approaches. A 72-year-old man experiencing longstanding depression, fatigue, irritable bowel syndrome, and chronic pain in the context of additional refractory illnesses was assessed and treated, guided by a system-oriented approach to underlying core imbalances termed functional medicine. This patient was referred from a team of clinicians representing primary care, cardiology, gastroenterology, hematology, and psychology. Prior treatment had been unsuccessful in managing multiple chronic comorbidities. Diagnostic assessment included comprehensive stool and nutritional/metabolic laboratory testing. The blood-, urine-, or stool-based measurements of relevant markers for multiple systemic issues, including digestion/absorption, inflammation, oxidative stress, and methylation, identified previously unrecognized root causes of his constellation of symptoms. These functional measurements guided rational recommendations for dietary choices and supplementation. The patient experienced steady and significant improvement in his mental health, fatigue, chronic pain, and irritable bowel syndrome-as well as the unexpected resolution of his chronic idiopathic pancytopenia. The success in this case suggests that other patients with chronic, complex, and treatment-refractory illness may benefit from a system-oriented assessment of core imbalances guided by specialized nutritional/metabolic and digestive laboratory testing.

Advances in multidisciplinary individualized treatment of refractory hepatic alveolar echinococcosis

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ABUDUAINI Abulizi

2015-04-01

Full Text Available Hepatic alveolar echinococcosis (HAE is a zoonotic parasitic disease that seriously threatens the population in western China and compromises patients′ quality of life. With the continuous improvement in radical resection rate in recent years, late-stage HAE patients that were incurable in the past now have the opportunity for radical resection. However, patients who are not suitable candidates for radical resection still suffer from various complications and poor quality of life. Therefore, HAE is still considered a refractory and complex disease. The simple empirical treatment model provided by traditional professional discussion is unable to satisfy the treatment of advanced refractory HAE as it is unable to integrate specialized, standardized clinical skills for diagnosis and treatment. Multidisciplinary individualized treatment (MDT organically integrates the advantages of the available treatment into a reasonable individualized comprehensive treatment regimen. This review summarizes the advances in MDT for HAE as the best option to increase long-term survival, and suggests MDT as the first-line treatment for late-stage refractory hepatic alveolar echinococcosis.

Immunomodulatory therapy in refractory/recurrent ovarian cancer

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Chao-Yu Chen

2015-04-01

Conclusion: IMT alone or add-on to palliative/salvage chemotherapy for refractory/recurrent EOC achieves a substantial disease stabilizing rate without severe toxicity, which might be a potential option in selected patients. The ALC 1 month after IMT could be an early indicator to disease stabilization.

Decision Making during the Psychological Refractory Period

NARCIS (Netherlands)

Zylberberg, Ariel; Ouellette, Brian; Sigman, Mariano; Roelfsema, Pieter R.

2012-01-01

In spite of its massively parallel architecture [1], the human brain is fundamentally limited if required to perform two tasks at the same time [2, 3]. This limitation can be studied with the psychological refractory period (PRP) paradigm, where two stimuli that require speeded responses occur in

Ways to improve physical and thermal performance of refractory lining materials

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Khlystov A.I.

2017-01-01

Full Text Available Refractory lining materials, which include ceramic refractories and nonfired heat-resistant concretes, have a very short lifespan during the turnaround time measured in years and sometimes months. Therefore, increasing the service life of thermal generating units by 1.5-2 times will bring significant economic benefits. The main factor that determines the durability of refractory lining materials is the thermal resistance. It is possible to increase the thermal resistance by improving such physical and mechanical properties as strength and density. As for the ceramic refractory performance improvement, such technological methods as their structural and chemical modification by phosphate binder impregnation, as well as introduction of phosphate components into the ceramic batches during the molding process increase, in particular, their thermal stability. The use of aluminous and high-alumina cements contributes to a significant increase of not only strength, but also physical and thermal performance of heat-resistant concretes with different fillers. Switching to the use of chemical binders in the compositions of heat-resistant concretes (liquid glass with effective hardeners; silicate-block and phosphate binders enables to develop high-heat resistant materials which do not soften in a wide range of heating temperatures from 400 °С to 1600 °С. The positive results on increasing the thermal resistance of heat-resistant composites can be obtained by reinforcing them with high temperature fibers.

CAR-T cells and allogeneic hematopoietic stem cell transplantation for relapsed/refractory B-cell acute lymphoblastic leukemia.

Science.gov (United States)

Liu, Jun; Zhang, Xi; Zhong, Jiang F; Zhang, Cheng

2017-10-01

Relapsed/refractory acute lymphoblastic leukemia (ALL) has a low remission rate after chemotherapy, a high relapse rate and poor long-term survival even when allogeneic hematopoietic stem cell transplantation (allo-HSCT) is performed. Chimeric antigen receptors redirected T cells (CAR-T cells) can enhance disease remission with a favorable outcome for relapsed/refractory ALL, though some cases quickly relapsed after CAR-T cell treatment. Thus, treatment with CAR-T cells followed by allo-HSCT may be the best way to treat relapsed/refractory ALL. In this review, we first discuss the different types of CAR-T cells. We then discuss the treatment of relapsed/refractory ALL using only CAR-T cells. Finally, we discuss the use of CAR-T cells, followed by allo-HSCT, for the treatment of relapsed/refractory ALL.

Rate of primary refractory disease in B and T-cell non-Hodgkin's lymphoma: correlation with long-term survival.

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Corrado Tarella

Full Text Available BACKGROUND: Primary refractory disease is a main challenge in the management of non-Hodgkin's Lymphoma (NHL. This survey was performed to define the rate of refractory disease to first-line therapy in B and T-cell NHL subtypes and the long-term survival of primary refractory compared to primary responsive patients. METHODS: Medical records were reviewed of 3,106 patients who had undergone primary treatment for NHL between 1982 and 2012, at the Hematology Centers of Torino and Bergamo, Italy. Primary treatment included CHOP or CHOP-like regimens (63.2%, intensive therapy with autograft (16.9%, or other therapies (19.9%. Among B-cell NHL, 1,356 (47.8% received first-line chemotherapy with rituximab. Refractory disease was defined as stable/progressive disease, or transient response with disease progression within six months. RESULTS: Overall, 690 (22.2% patients showed primary refractory disease, with a higher incidence amongst T-cell compared to B-cell NHL (41.9% vs. 20.5%, respectively, p<0.001. Several other clinico-pathological factors at presentation were variably associated with refractory disease, including histological aggressive disease, unfavorable clinical presentation, Bone Marrow involvement, low lymphocyte/monocyte ration and male gender. Amongst B-cell NHL, the addition of rituximab was associated with a marked reduction of refractory disease (13.6% vs. 26.7% for non-supplemented chemotherapy, p<0.001. Overall, primary responsive patients had a median survival of 19.8 years, compared to 1.3 yr. for refractory patients. A prolonged survival was consistently observed in all primary responsive patients regardless of the histology. The long life expectancy of primary responsive patients was documented in both series managed before and after 2.000. Response to first line therapy resulted by far the most predictive factor for long-term outcome (HR for primary refractory disease: 16.52, p<0.001. CONCLUSION: Chemosensitivity to primary

Clinical observation on Ahmed glaucoma valve implantation for teenagers with refractory glaucoma

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Dong-Dao Song

2016-06-01

Full Text Available AIM:To observe the clinical effect of Ahmed glaucoma valve(AGVimplantation for teenagers with refractory glaucoma. METHODS: Twenty-seven patients(27 eyeswith refractory glaucoma were treated with AGV implantation in our hospital from October 2012 to October 2014. The patients were followed up for 12mo. The success rate of the operation, postoperative intraocular pressure, the best corrected visual acuity, as well as complications were recorded. RESULTS: The success rate of the operation was 85%. The intraocular pressure of the 27 patients decreased from 48.3±8.3mmHg before operations to 21.4±8.1mmHg(PPCONCLUSION: AGV implantation is characterized by a high success rate, simple operation, less complications and is an effective treatment for refractory glaucoma in adolescents.

Corrosion of K-3 glass-contact refractory in sodium-rich aluminosilicate melts

International Nuclear Information System (INIS)

Lu, X.D.; Gan, H.; Buechele, A.C.; Pegg, I.L.

1999-01-01

The corrosion of the glass-contact refractory Monofrax K-3 in two sodium-rich aluminosilicate melts has been studied at 1,208 and 1,283 C using a modified ASTM procedure with constant agitation of the melt by air bubbling. The results for the monolithic refractory indicate a fast initial stage involving phase dissolution and transformation and a later passivated stage in which the surface of the refractory has been substantially modified. The composition of the stable spinel phase in the altered layer on monolithic coupons of K-3 is almost identical to the equilibrium composition bracketed by the dissolution of powdered K-3 into under-saturated melts on the other. The temperature and melt shear viscosity were found to have significant effects on the rates of K-3 dissolution and transformation

Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN)

DEFF Research Database (Denmark)

Howard, James F; Utsugisawa, Kimiaki; Benatar, Michael

2017-01-01

BACKGROUND: Complement is likely to have a role in refractory generalised myasthenia gravis, but no approved therapies specifically target this system. Results from a phase 2 study suggested that eculizumab, a terminal complement inhibitor, produced clinically meaningful improvements in patients...... with anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis. We further assessed the efficacy and safety of eculizumab in this patient population in a phase 3 trial. METHODS: We did a phase 3, randomised, double-blind, placebo-controlled, multicentre study (REGAIN) in 76...... hospitals and specialised clinics in 17 countries across North America, Latin America, Europe, and Asia. Eligible patients were aged at least 18 years, with a Myasthenia Gravis-Activities of Daily Living (MG-ADL) score of 6 or more, Myasthenia Gravis Foundation of America (MGFA) class II-IV disease...

Infantile Systemic Hyalinosis: A Case Report with a Novel Mutation

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Siham Al Sinani

2013-01-01

Full Text Available Infantile Systemic Hyalinosis (ISH (OMIM 236490 is a rare, progressive and fatal autosomal recessive disorder characterized by multiple subcutaneous skin nodules, gingival hypertrophy, osteopenia, joint contractures, failure to thrive, diarrhea with protein losing enteropathy, and frequent infections. There is diffuse deposition of hyaline material in the skin, gastrointestinal tract, muscle and endocrine glands. It is caused by mutations in the ANTXR2 (also known as CMG2 gene, which encodes a trans-membranous protein involved in endothelial development and basement membrane-extracellular matrix assembly. We describe a child with classical features of ISH presenting in infancy with severe chronic debilitating pain and progressive joint contractures. The diagnosis was confirmed by molecular DNA sequencing of ANTXR2 gene which revealed a novel homozygous mutation not previously reported; 79 bp deletion of the entire exon 11 (c.867_945del, p.E289DfsX22. Although this is the first reported case of ISH in Oman, we believe that the disease is under-diagnosed since children affected with this lethal disease pass away early in infancy prior to establishing a final diagnosis.

New applications and novel processing of refractory metal alloys

International Nuclear Information System (INIS)

Briant, C.L.

2001-01-01

Refractory metals have often been limited in their application because of their propensity to oxidize and to undergo a loos of yield strength at elevated temperatures. However, recent developments in both processing and alloy composition have opened the possibility that these materials might be used in structural applications that were not considered possible in the past. At the same time, the use of refractory metals in the electronics industry is growing, particularly with the use of tantalum as a diffusion barrier for copper metallization. Finally, the application of grain boundary engineering to the problem of intergranular fracture in these materials may allow processes to be developed that will produce alloys with a greater resistance to fracture. (author)

Classical management of refractory adult immune (idiopathic) thrombocytopenic purpura.

Science.gov (United States)

McMillan, R

2002-03-01

Treatment of chronic immune (idiopathic) thrombocytopenic purpura with corticosteroids and/or splenectomy results in safe platelet counts in over 70% of patients without additional treatment. Therapy of patients who are refractory to these two treatments may be difficult. The treatment approach to refractory ITP patients, described in this report, is arbitrarily divided into four levels: levels 1 through 3 represent treatments with increasing side effects; level 4 therapy may be tried when the others have failed. Patients undergoing these treatments may require concomitant intravenous gammaglobulin, high-dose corticosteroids or platelets, to maintain the platelet count in the setting of mucosal bleeding or severe thrombocytopenia. Copyright 2002, Elsevier Science Ltd. All rights reserved.

Behaviour of inorganics and refractory lining design in a entrained flow reactor for the production of BTL

Energy Technology Data Exchange (ETDEWEB)

Berjonneau, J.; Poirier, J.; Colombel, L. (CNRS CRMHT, Orleans (France)); Pichavant, M. (CNRS ISTO, Orleans (France)); Defoort, F. (CEA LPTM DTN/SE2T/LPTM, Grenoble (France))

2007-07-01

During the gasification of biomass, inorganic species are produced and constitute an important obstacle in this process. The aim of this study is to understand the behaviour of the inorganic species particularly the production of ashes. These ashes will coat the reactor wall and will play at the same time the role of protection of the refractory layer against external attacks and of thermal insulation. To define the operating conditions and to design the refractory structure, melting temperature of the slag is one of the most important properties. To know this characteristic a thermodynamic approach is used. The database used for the calculation is firstly validated by comparison with systems available in the literature and with an experimental approach. (orig.)

Refractory Septic Shock Treated with Nephrectomy under the Support of Extracorporeal Membrane Oxygenation

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Young Kun Lee

2015-08-01

Full Text Available Conventional medical therapies have not been very successful in treating adults with refractory septic shock. The effects of direct hemoperfusion using polymyxin B and veno-arterial extracorporeal membrane oxygenation (ECMO for refractory septic shock remain uncertain. A 66-year-old man was admitted to the emergency department and suffered from sepsis-induced hemodynamic collapse. For hemodynamic improvement, we performed direct hemoperfusion using polymyxin B. Computed tomography scan of this patient revealed emphysematous pyelonephritis (EPN, for which he underwent emergent nephrectomy with veno-arterial ECMO support. To the best of our knowledge, this is the first report of successful treatment of EPN with refractory septic shock using polymyxin B hemoperfusion and nephrectomy under the support of ECMO.

Clinical features and gene mutational spectrum of CDKL5-related diseases in a cohort of Chinese patients.

Science.gov (United States)

Zhao, Ying; Zhang, Xiaoying; Bao, Xinhua; Zhang, Qingping; Zhang, Jingjing; Cao, Guangna; Zhang, Jie; Li, Jiarui; Wei, Liping; Pan, Hong; Wu, Xiru

2014-02-25

Mutations in the cyclin-dependent kinase-like 5 (CDKL5) (NM_003159.2) gene have been associated with early-onset epileptic encephalopathies or Hanefeld variants of RTT(Rett syndrome). In order to clarify the CDKL5 genotype-phenotype correlations in Chinese patients, CDKL5 mutational screening in cases with early-onset epileptic encephalopathies and RTT without MECP2 mutation were performed. The detailed clinical information including clinical manifestation, electroencephalogram (EEG), magnetic resonance imaging (MRI), blood, urine amino acid and organic acid screening of 102 Chinese patients with early-onset epileptic encephalopathies and RTT were collected. CDKL5 gene mutations were analyzed by PCR, direct sequencing and multiplex ligation-dependent probe amplification (MLPA). The patterns of X-chromosome inactivation (XCI) were studied in the female patients with CDKL5 gene mutation. De novo CDKL5 gene mutations were found in ten patients including one missense mutation (c.533G > A, p.R178Q) which had been reported, two splicing mutations (ISV6 + 1A > G, ISV13 + 1A > G), three micro-deletions (c.1111delC, c.2360delA, c.234delA), two insertions (c.1791 ins G, c.891_892 ins TT in a pair of twins) and one nonsense mutation (c.1375C > T, p.Q459X). Out of ten patients, 7 of 9 females with Hanefeld variants of RTT and the remaining 2 females with early onset epileptic encephalopathy, were detected while only one male with infantile spasms was detected. The common features of all female patients with CDKL5 gene mutations included refractory seizures starting before 4 months of age, severe psychomotor retardation, Rett-like features such as hand stereotypies, deceleration of head growth after birth and poor prognosis. In contrast, the only one male patient with CDKL5 mutation showed no obvious Rett-like features as females in our cohort. The X-chromosome inactivation patterns of all the female patients were random. Mutations in CDKL5 gene are responsible for 7 with

Iatrogenic colorectal Kaposi sarcoma complicating a refractory ...

African Journals Online (AJOL)

Kaposi sarcoma is a mesenchymal tumor associated to a human herpes virus-8. It often occurs in human immunodeficiency virus-positive subjects. Colorectal localization is rare. We report the case of a colorectal Kaposi sarcoma complicating a refractory ulcerative colitis treated with surgery after the failure of ...

Efficacy and Safety of Rituximab in Children with Refractory Nephrotic Syndrome; A Multicenter Clinical Trial

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Yo Han Ahn

2014-06-01

Conclusions: In this interim analysis of clinical trial to evaluate the efficacy and safety of RTX in children with refractory NS, RTX treatment for refractory NS was safe and effective, especially in patients with DNS.

Evaluation of nutritional status in children with refractory epilepsy.

Science.gov (United States)

Bertoli, S; Cardinali, S; Veggiotti, P; Trentani, C; Testolin, G; Tagliabue, A

2006-04-26

children affected by refractory epilepsy could be at risk of malnutrition because of feeding difficulties (anorexia, chewing, swallowing difficulties or vomiting) and chronic use of anticonvulsants, which may affect food intake and energy metabolism. Moreover, their energy requirement may be changed as their disabilities would impede normal daily activities. The aim of the present study was to evaluate nutritional status, energy metabolism and food intake in children with refractory epilepsy. 17 children with refractory epilepsy (13 boys and 4 girls; mean age 9 +/- 3,2 years; Body Mass Index 15,7 +/- 3,6) underwent an anthropometric assessment, body composition evaluation by dual-energy X-ray absorptiometry, detailed dietetic survey and measurement of resting energy expenditure by indirect calorimetry. Weight-for-age, height-for-age (stunting) and weight-for-height (wasting) were estimated compared to those of a reference population of the same age. 40% of children were malnourished and 24% were wasted. The nutritional status was worse in the more disabled children. Dietary intake resulted unbalanced (18%, 39%, 43% of total daily energy intake derived respectively from protein, lipid and carbohydrate). Adequacy index [nutrient daily intake/recommended allowance (RDA) x 100] was nutritional assessment and management as part of their overall care.

Adalimumab for the treatment of refractory noninfectious paediatric uveitis.

Science.gov (United States)

Muñoz-Gallego, Alicia; Barral, Estefanía; Enríquez, Eugenia; Tejada, Pilar; Barceló, Ana; de Inocencio, Jaime

2017-06-01

To report the experience of our center with the use of adalimumab (ADA) for the treatment of severe refractory noninfectious paediatric uveitis. The study is a retrospective case series of all paediatric patients with refractory uveitis who were treated with ADA at the Paediatric Uveitis Unit of our center from 2008 to 2015. We present 12 patients (6 Juvenile idiopathic arthritis-associated uveitis, 4 idiopathic panuveitis, 1 early-onset sarcoidosis-associated panuveitis, and 1 intermediate uveitis), with uveitis in 19/24 eyes. Once ADA therapy was started, all the patients presented improved activity according to Standardization of Uveitis Nomenclature (SUN) criteria. Nine out of the 12 patients had structural damage before ADA could be started: cataract (n = 4), glaucoma (n = 2), cystic macular edema (n = 1), exudative retinal detachment (n = 1), and optic disk edema (n = 5). Visual acuity improved or maintained stable in 17/19 affected eyes, and only 2 eyes decreased its visual acuity because of structural damage, which was already present before ADA therapy. In our experience, ADA presents a good safety profile and is efficacious in the treatment of paediatric patients with different forms of refractory noninfectious uveitis.

Evaluation of nutritional status in children with refractory epilepsy

Science.gov (United States)

Bertoli, S; Cardinali, S; Veggiotti, P; Trentani, C; Testolin, G; Tagliabue, A

2006-01-01

Background children affected by refractory epilepsy could be at risk of malnutrition because of feeding difficulties (anorexia, chewing, swallowing difficulties or vomiting) and chronic use of anticonvulsants, which may affect food intake and energy metabolism. Moreover, their energy requirement may be changed as their disabilities would impede normal daily activities. The aim of the present study was to evaluate nutritional status, energy metabolism and food intake in children with refractory epilepsy. Methods 17 children with refractory epilepsy (13 boys and 4 girls; mean age 9 ± 3,2 years; Body Mass Index 15,7 ± 3,6) underwent an anthropometric assessment, body composition evaluation by dual-energy X-ray absorptiometry, detailed dietetic survey and measurement of resting energy expenditure by indirect calorimetry. Weight-for-age, height-for-age (stunting) and weight-for-height (wasting) were estimated compared to those of a reference population of the same age. Results 40% of children were malnourished and 24% were wasted. The nutritional status was worse in the more disabled children. Dietary intake resulted unbalanced (18%, 39%, 43% of total daily energy intake derived respectively from protein, lipid and carbohydrate). Adequacy index [nutrient daily intake/recommended allowance (RDA) × 100] was nutritional assessment and management as part of their overall care. PMID:16640779

Refractory metal particles in refractory inclusions in the Allende meteorite

International Nuclear Information System (INIS)

Fuchs, L.H.; Blander, M.

1980-01-01

An examination of refractory metal particles in five calcium-aluminum-rich inclusions in the Allende meteorite indicates a complex variety of compositions and large departures from equilibrium. These particles appear to have been primordial condensates which were isolated from the nebula and from each other at different times by cocondensing oxides. Selective diffusion and/or oxidation of the more oxidizable metals (Mo, W, Fe and Ni), phase segregations into different alloy phases (fcc, bcc, hcp and perhaps ordered phases) and the formation of metastable condensates appears to have been involved in the modification of these materials to their present state. Only a small fraction of our observations cannot be reconciled with this picture because of a lack of knowledge of some of the phase equilibria which might have bee involved

A Case of Refractory Pulmonary Coccidioidomycosis Successfully Treated with Posaconazole Therapy

Science.gov (United States)

Patel, RH; Pandya, S; Nanjappa, S; Greene, JN

2018-01-01

Coccidioidomycosis is an endemic fungal infection caused by the inhalation of the spores of Coccidioides species. Patients with underlying immunosuppressive illness can contract chronic or disseminated disease which requires prolonged systemic therapy. Pulmonary coccidioidomycosis remains as an illusory and abstruse disease, with increased prevalence that poses as a challenge for clinicians in developing an effective strategy for treatment. Here, we report successful treatment of a refractory case of chronic relapsing pulmonary coccidioidomycosis in a 50-year old woman with a thin-walled cavitary lung lesion who was ultimately treated with posaconazole.

Sub-micrometer refractory carbonaceous particles in the polar stratosphere

Science.gov (United States)

Schütze, Katharina; Wilson, James Charles; Weinbruch, Stephan; Benker, Nathalie; Ebert, Martin; Günther, Gebhard; Weigel, Ralf; Borrmann, Stephan

2017-10-01

Eleven particle samples collected in the polar stratosphere during SOLVE (SAGE III Ozone loss and validation experiment) from January until March 2000 were characterized in detail by high-resolution transmission and scanning electron microscopy (TEM/SEM) combined with energy-dispersive X-ray microanalysis. A total of 4202 particles (TEM = 3872; SEM = 330) were analyzed from these samples, which were collected mostly inside the polar vortex in the altitude range between 17.3 and 19.9 km. Particles that were volatile in the microscope beams contained ammonium sulfates and hydrogen sulfates and dominated the samples. Some particles with diameters ranging from 20 to 830 nm were refractory in the electron beams. Carbonaceous particles containing additional elements to C and O comprised from 72 to 100 % of the refractory particles. The rest were internal mixtures of these materials with sulfates. The median number mixing ratio of the refractory particles, expressed in units of particles per milligram of air, was 1.1 (mg air)-1 and varied between 0.65 and 2.3 (mg air)-1. Most of the refractory carbonaceous particles are completely amorphous, a few of the particles are partly ordered with a graphene sheet separation distance of 0.37 ± 0.06 nm (mean value ± standard deviation). Carbon and oxygen are the only detected major elements with an atomic O/C ratio of 0.11 ± 0.07. Minor elements observed include Si, S, Fe, Cr and Ni with the following atomic ratios relative to C: Si/C: 0.010 ± 0.011; S/C: 0.0007 ± 0.0015; Fe/C: 0.0052 ± 0.0074; Cr/C: 0.0012 ± 0.0017; Ni/C: 0.0006 ± 0.0011 (all mean values ± standard deviation).High-resolution element distribution images reveal that the minor elements are distributed within the carbonaceous matrix; i.e., heterogeneous inclusions are not observed. No difference in size, nanostructure and elemental composition was found between particles collected inside and outside the polar vortex. Based on chemistry and nanostructure

Mutation analysis of β-thalassemia in East-Western Indian population: a recent molecular approach

Directory of Open Access Journals (Sweden)

Shah PS

2017-05-01

Full Text Available Parth S Shah,1 Nidhi D Shah,2 Hari Shankar P Ray,3 Nikunj B Khatri,3 Ketan K Vaghasia,3 Rutvik J Raval,4 Sandip C Shah,3 Mandava V Rao5 1Department of Medicine, Lahey Hospital and Medical Center, Boston, MA, 2Department of Pediatrics, Nassau University Medical Centre, New York, NY, USA; 3Supratech Micropath Laboratory and Research Institute, 4Department of Zoology, 5School of Sciences, Gujarat University, Ahmedabad, Gujarat, India Background: β-Thalassemia is the most prevalent genetic disorder in India. Its traits and coinheritance vary from mild to severe conditions, resulting in thalassemia minor, intermediate, and major, depending upon many factors.Purpose: The objective of this study was to identify the incidence of β-thalassemia traits, their coinheritance, and mutations, as well as to support the patients already diagnosed with β-thalassemia in East-Western Indian population for better management.Patients and methods: Seventy-five referral cases for β-thalassemia were analyzed for various β-thalassemia traits, heterozygosity, and homozygosity conditions. Blood phenotypic parameters using cell counter and capillary electrophoresis were investigated. Analyses of eight common mutations of thalassemia in India were carried out using polymerase chain reaction-amplification refractory mutation system, end point polymerase chain reaction, and DNA sequencing methods.Results: Of these (75 referral cases from East-Western Indian region, 68 were positive for β-thalassemia (90.67%. The majority of case types were of β-thalassemia minor (49, 65.33%, followed by HbE traits (6, 8.0% and β-thalassemia major, including heterozygous and homozygous (5, 6.66%; 4, 5.33% types and then HbE homozygous (2, 2.66%, as well as one each of the HbE/β-thalassemia and HbD/β-thalassemia (1, 1.34% combination. Mutation analysis also revealed that the highest frequency of mutation was c.92+5G>C (41, 60.29% followed by deletion 619bp (9, 13.23% and c.79G>A (8, 11

Trace Element Abundances in an Unusual Hibonite-Perovskite Refractory Inclusion from Allende

Science.gov (United States)

Mane, Prajkta; Wadhwa, M.; Keller, L. P.

2013-01-01

Calcium-aluminum-rich refractory inclusions (CAIs) are thought to be the first-formed solids in the Solar protoplanetary disk and can provide information about the earliest Solar System processes (e.g., [1]). A hibonite-perovskitebearing CAI from the Allende CV3 chondrite (SHAL, [2]) contains a single of 500 micrometers hibonite grain and coarse-grained perovskite. The mineralogy and oxygen isotopic composition of this CAI shows similarities with FUN inclusions, especially HAL [2]. Here we present trace element abundances in SHAL.

Brief Report: Human Acute Myeloid Leukemia Reprogramming to Pluripotency Is a Rare Event and Selects for Patient Hematopoietic Cells Devoid of Leukemic Mutations.

Science.gov (United States)

Lee, Jong-Hee; Salci, Kyle R; Reid, Jennifer C; Orlando, Luca; Tanasijevic, Borko; Shapovalova, Zoya; Bhatia, Mickie

2017-09-01

Induced pluripotent stem cell reprogramming has provided critical insights into disease processes by modeling the genetics and related clinical pathophysiology. Human cancer represents highly diverse genetics, as well as inter- and intra-patient heterogeneity, where cellular model systems capable of capturing this disease complexity would be invaluable. Acute myeloid leukemia (AML) represents one of most heterogeneous cancers and has been divided into genetic subtypes correlated with unique risk stratification over the decades. Here, we report our efforts to induce pluripotency from the heterogeneous population of human patients that represents this disease in the clinic. Using robust optimized reprogramming methods, we demonstrate that reprogramming of AML cells harboring leukemic genomic aberrations is a rare event with the exception of those with de novo mixed-lineage leukemia (MLL) mutations that can be reprogrammed and model drug responses in vitro. Our findings indicate that unlike hematopoietic cells devoid of genomic aberrations, AML cells harboring driver mutations are refractory to reprogramming. Expression of MLL fusion proteins in AML cells did not contribute to induced reprogramming success, which continued to select for patient derived cells devoid of AML patient-specific aberrations. Our study reveals that unanticipated blockades to achieving pluripotency reside within the majority of transformed AML patient cells. Stem Cells 2017;35:2095-2102. © 2017 AlphaMed Press.

Refractory compounds for repairing coke-oven brickwork

Energy Technology Data Exchange (ETDEWEB)

Hanganu, M.; Dragomir, C. (and others)

1984-01-01

A number of refractory compounds are presented, based on aluminium phosphate, phosphoric acid, sodium silicate and fire-clay and mullitic zirconium mastic, including their composition, properties and application (by impregnation, spray application or pouring), which are suitable for repairing various types of coke-oven brickwork. The results obtained are given.

Bolus versus continuous low dose of enalaprilat in congestive heart failure with acute refractory decompensation.

Science.gov (United States)

Podbregar, M; Voga, G; Horvat, M; Zuran, I; Krivec, B; Skale, R; Pareznik, R

1999-01-01

The first dose of angiotensin-converting enzyme (ACE) inhibitors may trigger a considerable fall of blood pressure in chronic heart failure. The response may be dose-related. To determine hemodynamic and systemic oxygenation effects of low-dose enalaprilat, we administered intravenous enalaprilat (0.004 mg/kg) as bolus (group B) or continuous 1-hour infusion (group C) in 20 patients with congestive heart failure due to ischemic heart disease with acute decompensation refractory to inotropic, vasodilator and diuretic therapy. Hemodynamic and systemic oxygenation variables were recorded at baseline (+0 min), +30, +60, +120, +180, and +360 min after the start of intervention. Mean arterial pressure (MAP) (p < 0. 001), mean pulmonary artery pressure (MPAP) (p < 0.001), pulmonary artery occlusion pressure (PAOP) (p < 0.001), oxygen extraction ratio (ER) (p < 0.026) decreased regardless of enalaprilat application. Compared to group B, there was in group C prolonged decrease of MAP, MPAP, PAOP, ER and increase of pulmonary artery oxyhemoglobin saturation in regard to baseline values. Cardiac index, heart rate, central venous pressure and oxygen consumption index did not change. A low dose of intravenous enalaprilat (0.004 mg/kg) can be used to safely improve hemodynamics and systemic oxygenation in congestive heart failure due to ischemic heart disease with acute refractory decompensation.

Multifunctional Metallic and Refractory Materials for Energy Efficient Handling of Molten Metals

Energy Technology Data Exchange (ETDEWEB)

Xingbo Liu; Ever Barbero; Bruce Kang; Bhaskaran Gopalakrishnan; James Headrick; Carl Irwin

2009-02-06

The goal of the project was to extend the lifetime of hardware submerged in molten metal by an order of magnitude and to improve energy efficiency of molten metal handling process. Assuming broad implementation of project results, energy savings in 2020 were projected to be 10 trillion BTU/year, with cost savings of approximately $100 million/year. The project team was comprised of materials research groups from West Virginia University and the Missouri University of Science and Technology formerly University of Missouri – Rolla, Oak Ridge National Laboratory, International Lead and Zinc Research Organization, Secat and Energy Industries of Ohio. Industry partners included six suppliers to the hot dip galvanizing industry, four end-user steel companies with hot-dip Galvanize and/or Galvalume lines, eight refractory suppliers, and seven refractory end-user companies. The results of the project included the development of: (1) New families of materials more resistant to degradation in hot-dip galvanizing bath conditions were developed; (2) Alloy 2020 weld overlay material and process were developed and applied to GI rolls; (3) New Alloys and dross-cleaning procedures were developed for Galvalume processes; (4) Two new refractory compositions, including new anti-wetting agents, were identified for use with liquid aluminum alloys; (5) A new thermal conductivity measurement technique was developed and validated at ORNL; (6) The Galvanizing Energy Profiler Decision Support System (GEPDSS)at WVU; Newly Developed CCW Laser Cladding Shows Better Resistance to Dross Buildup than 316L Stainless Steel; and (7) A novel method of measuring the corrosion behavior of bath hardware materials. Project in-line trials were conducted at Southwire Kentucky Rod and Cable Mill, Nucor-Crawfordsville, Nucor-Arkansas, Nucor-South Carolina, Wheeling Nisshin, California Steel, Energy Industries of Ohio, and Pennex Aluminum. Cost, energy, and environmental benefits resulting from the project

Treatment of refractory/relapsed adult acute lymphoblastic leukemia with bortezomib- based chemotherapy

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Zhao J

2015-06-01

Full Text Available Junmei Zhao,* Chao Wang,* Yongping Song, Yuzhang Liu, Baijun FangHenan Key Lab of Experimental Haematology, Henan Institute of Haematology, Henan Tumor Hospital, Zhengzhou University, Zhengzhou, People’s Republic of China  *These authors contributed equally to this work Abstract: Nine pretreated patients aged >19 years with relapsed/refractory acute lymphoblastic leukemia (ALL were treated with a combination of bortezomib plus chemotherapy before allogeneic hematopoietic stem cell transplantation (allo-HSCT. Eight (88.9% patients, including two Philadelphia chromosome-positive ALL patients, achieved a complete remission. Furthermore, the evaluable patients have benefited from allo-HSCT after response to this reinduction treatment. We conclude that bortezomib-based chemotherapy was highly effective for adults with refractory/relapsed ALL before allo-HSCT. Therefore, this regimen deserves a larger series within prospective trials to confirm these results. Keywords: acute lymphoblastic leukemia, refractory, relapsed, bortezomib

The Denver Tube Combined with Antiviral Drugs In the Treatment of HBV-related Cirrhosis with Refractory Ascites: A Report of Three Cases

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Wang Xiao-jin

2014-03-01

Full Text Available Treatment of nucleos(tide antiviral drugs for decompensated HBV-related cirrhosis can significantly improve the prognosis. But those patients with refractory ascites possibly deteriorate due to the complications of ascites before any benefit from anti-viral drugs could be observed. Therefore, it is important to find a way to help the patients with HBV-related cirrhosis and refractory ascites to receive the full benefits from antiviral therapy. Peritoneovenous shunt (PVS using Denver tube enables ascites to continuously bypass into systemic circulation, thereby reducing ascites and albumin input and improving quality of life. We report herein 3 cases of decompensated HBV-related cirrhosis with refractory ascites, PVS using Denver tube was combined with lamivudine for antiviral treatment before and after. Then, ascites was alleviated significantly or disapeared and viral responsed well. All patients achieved a satisfactory long-term survival from 6.7 to 14.7 years. It was suggested that the Denver shunt could be used as an adjuvant method to antiviral drugs for decompensated HBV-related cirrhosis with refractory ascites to help the patients reap the full benefits and maximize efficacy of antiviral treatment.

Long-term effects of electrical neurostimulation in patients with unstable angina : Refractory to conventional therapies

NARCIS (Netherlands)

de Vries, Jessica; DeJongste, Mike J. L.; Zijlstra, Felix; Staal, Michiel

2007-01-01

Background. Patients with unstable angina pectoris may become refractory to conventional therapies. Electrical neurostimulation with transcutaneous electrical stimulation and/or spinal cord stimulation has been shown to be effective for patients with refractory unstable angina pectoris in hospital

Degradation of Alumina and Magnesia Chrome refractory bricks in Portland cement kiln – Corrected version*

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Ben Addi K.

2014-05-01

Full Text Available In cement plants, the refractory products are particularly confronted to partially liquid oxide phases at temperature ranging between 900°C and 1700°C. All constituents of these products have to resist not only to thermal constraints, but also to the thermochemical solicitations which result from contact material/coating. In order to study the phenomenon of degradation of refractory bricks in cement kilns and to identify the causes of their degradation, we proceed to the examination of industrial cases in cement kiln. Many chemical tests of the degraded refractory bricks have been done and the results acquired were compared to the ones not used. The analysis of the results is doing using different techniques (Loss of ignition, X-ray Fluorescence, X-ray Diffraction. The results show that the degradation of the used bricks in the clinkering and cooling zone is due to the infiltration of aggressive elements such us sulphur, alkali (Na2O, K2O .... The chemical interaction between the Portland clinker phases and refractory material has also an importance on the stability of the coating and consequently on the life of the refractories.

Intravenous Foscarnet With Topical Cidofovir for Chronic Refractory Genital Herpes in a Patient With AIDS.

Science.gov (United States)

Usoro, Agnes; Batts, Alfreda; Sarria, Juan C

2015-01-01

Few case reports have documented the use of topical cidofovir for refractory genital herpes simplex virus (HSV) ulcers in human immunodeficiency virus (HIV) infected patients. This drug formulation lacks a standardized concentration or even a procedural outline as to how it should be compounded. We aim to discuss the utilization of topical cidofovir in addition to presenting a procedural means of compounding it for treatment of refractory genital HSV ulcers. Our patient completed 21 days of intravenous foscarnet and 13 days of topical cidofovir with clinical improvement in the penile and scrotal ulcers. Genital herpes is a concern in patients with HIV because it generally manifests as a persistent infection. Physicians should be aware that when patients fail to respond to the conventional treatment regimens for genital HSV in a timely manner, other options are available, such as topical cidofovir as an adjuvant to systemic antivirals.

Intravenous Foscarnet With Topical Cidofovir for Chronic Refractory Genital Herpes in a Patient With AIDS

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Agnes Usoro BSN

2015-12-01

Full Text Available Few case reports have documented the use of topical cidofovir for refractory genital herpes simplex virus (HSV ulcers in human immunodeficiency virus (HIV infected patients. This drug formulation lacks a standardized concentration or even a procedural outline as to how it should be compounded. We aim to discuss the utilization of topical cidofovir in addition to presenting a procedural means of compounding it for treatment of refractory genital HSV ulcers. Our patient completed 21 days of intravenous foscarnet and 13 days of topical cidofovir with clinical improvement in the penile and scrotal ulcers. Genital herpes is a concern in patients with HIV because it generally manifests as a persistent infection. Physicians should be aware that when patients fail to respond to the conventional treatment regimens for genital HSV in a timely manner, other options are available, such as topical cidofovir as an adjuvant to systemic antivirals.

Pembrolizumab and Vorinostat in Treating Patients With Relapsed or Refractory Diffuse Large B-Cell Lymphoma, Follicular Lymphoma, or Hodgkin Lymphoma

Science.gov (United States)

2018-04-23

Grade 3a Follicular Lymphoma; Grade 3b Follicular Lymphoma; Recurrent Classical Hodgkin Lymphoma; Recurrent Diffuse Large B-Cell Lymphoma; Recurrent Follicular Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Mediastinal (Thymic) Large B-Cell Cell Lymphoma; Refractory Classical Hodgkin Lymphoma; Refractory Diffuse Large B-Cell Lymphoma; Refractory Follicular Lymphoma; Refractory Mediastinal (Thymic) Large B-Cell Cell Lymphoma

Two-Step Oxidation of Refractory Gold Concentrates with Different Microbial Communities.

Science.gov (United States)

Wang, Guo-Hua; Xie, Jian-Ping; Li, Shou-Peng; Guo, Yu-Jie; Pan, Ying; Wu, Haiyan; Liu, Xin-Xing

2016-11-28

Bio-oxidation is an effective technology for treatment of refractory gold concentrates. However, the unsatisfactory oxidation rate and long residence time, which cause a lower cyanide leaching rate and gold recovery, are key factors that restrict the application of traditional bio-oxidation technology. In this study, the oxidation rate of refractory gold concentrates and the adaption of microorganisms were analyzed to evaluate a newly developed two-step pretreatment process, which includes a high temperature chemical oxidation step and a subsequent bio-oxidation step. The oxidation rate and recovery rate of gold were improved significantly after the two-step process. The results showed that the highest oxidation rate of sulfide sulfur could reach to 99.01 % with an extreme thermophile microbial community when the pulp density was 5%. Accordingly, the recovery rate of gold was elevated to 92.51%. Meanwhile, the results revealed that moderate thermophiles performed better than acidophilic mesophiles and extreme thermophiles, whose oxidation rates declined drastically when the pulp density was increased to 10% and 15%. The oxidation rates of sulfide sulfur with moderate thermophiles were 93.94% and 65.73% when the pulp density was increased to 10% and 15%, respectively. All these results indicated that the two-step pretreatment increased the oxidation rate of refractory gold concentrates and is a potential technology to pretreat the refractory sample. Meanwhile, owing to the sensitivity of the microbial community under different pulp density levels, the optimization of microbial community in bio-oxidation is necessary in industry.

MgAl2O4 spinel refractory as containment liner for high-temperature alkali salt containing environments

Science.gov (United States)

Peascoe-Meisner, Roberta A [Knoxville, TN; Keiser, James R [Oak Ridge, TN; Hemric, James G [Knoxville, TN; Hubbard, Camden R [Oak Ridge, TN; Gorog, J Peter [Kent, WA; Gupta, Amul [Jamestown, NY

2008-10-21

A method includes containing a high-temperature alkali salt containing environment using a refractory containment liner containing MgAl.sub.2O.sub.4 spinel. A method, includes forming a refractory brick containing MgAl.sub.2O.sub.4 spinel having an exterior chill zone defined by substantially columnar crystallization and an interior zone defined by substantially equiaxed crystallization; and removing at least a portion of the exterior chill zone from the refractory brick containing MgAl.sub.2O.sub.4 spinel by scalping the refractory brick containing MgAl.sub.2O.sub.4 spinel to define at least one outer surface having an area of substantially equiaxed crystallization. A product of manufacture includes a refractory brick containing MgAl.sub.2O.sub.4 spinel including an interior zone defined by substantially equiaxed crystallization; and at least one outer surface having an area of substantially equiaxed crystallization.

Performance Evaluation of Refractory Composite Coatings in Potassium Rich Environment

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Kristina BRINKIENĖ

2016-09-01

Full Text Available A laboratory scale method was used to study the performance of reinforced cement composites in potassium rich environment of biomass combustion. Buckwheat husk (BH was used as potential source of unexploited biomass product applicable as biomass derived fuel. In order to enhance the alkali effect on the properties of the investigated materials, the solution of potassium carbonate (K2CO3 was selected as potassium rich aggressive environment. Two reinforced cement composites as potential repair coatings for restoration of damaged refractory surfaces with different composition of aggregate were used in corrosion tests. Performance of refractory coatings was evaluated by analysing the microstructure of the treated composites as well as mechanical properties. Energy-dispersive X-ray spectroscopy (SEM/EDS and optical microscopy were used to study the microstructure in the corroded region of the refractory coatings. Long term studies in the solution of 1M K2CO3 for 56 months have demonstrated that composite with the additive of fluid cracking catalyst of oil refinery and petrochemical industries is more durable in the potassium rich environment.DOI: http://dx.doi.org/10.5755/j01.ms.22.3.8348

Treatment of refractory chylothorax with externalized pleuroperitoneal shunts in children.

Science.gov (United States)

Wolff, A B; Silen, M L; Kokoska, E R; Rodgers, B M

1999-09-01

Traditional therapy for refractory chylothorax in the pediatric population has included pleurodesis and thoracic duct ligation. These procedures are associated with high morbidity and questionable success rates. We retrospectively reviewed our experience with 15 patients who underwent treatment for chylous effusions using pleuroperitoneal shunts with exteriorized pump chambers. Mean patient age at time of shunt placement was 2.1 (0.1 to 11.5) years and the most common indication (7 of 15) was refractory chylothorax following surgical correction of congenital heart disease. Mean chylothorax duration before shunt placement was 76 (5 to 810) days and shunts were in place for an average of 104 (12 to 365) days. A total of 19 chylous effusions (pleural or pericardial) were treated with shunts. Nine of 11 right-sided chylothoraces, 5 of 6 left-sided chylothoraces, and 2 of 2 chylopericardia resolved with shunt therapy (84% total). Pleuroperitoneal shunting failed to clear the effusion in 3 children. There were six episodes of shunt malfunction that were repaired and two episodes of infection. Inguinal or umbilical hernia developed in 4 patients. Externalized pleuroperitoneal shunting is a safe, effective, and minimally invasive treatment for children with refractory chylous effusions.

NBAS mutations cause a multisystem disorder involving bone, connective tissue, liver, immune system, and retina

NARCIS (Netherlands)

Segarra, Nuria Garcia; Ballhausen, Diana; Crawford, Heather; Perreau, Matthieu; Campos-Xavier, Belinda; van Spaendonck-Zwarts, Karin; Vermeer, Cees; Russo, Michel; Zambelli, Pierre-Yves; Stevenson, Brian; Royer-Bertrand, Beryl; Rivolta, Carlo; Candotti, Fabio; Unger, Sheila; Munier, Francis L.; Superti-Furga, Andrea; Bonafé, Luisa

2015-01-01

We report two unrelated patients with a multisystem disease involving liver, eye, immune system, connective tissue, and bone, caused by biallelic mutations in the neuroblastoma amplified sequence (NBAS) gene. Both presented as infants with recurrent episodes triggered by fever with vomiting,

Disease: H01278 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available H01278 Iron-refractory iron deficiency anemia (IRIDA) Iron-refractory iron deficiency anemia...LE ... Mutations in TMPRSS6 cause iron-refractory iron deficiency anemia (IRIDA). ... JOURNAL ... Nat Genet 40:569-71 (2008) DOI:10.1038/ng.130 ...

Rikkunshito improves globus sensation in patients with proton-pump inhibitor-refractory laryngopharyngeal reflux.

Science.gov (United States)

Tokashiki, Ryoji; Okamoto, Isaku; Funato, Nobutoshi; Suzuki, Mamoru

2013-08-21

To investigate the effect of rikkunshito on laryngopharyngeal reflux (LPR) symptoms and gastric emptying in patients with proton-pump inhibitor (PPI)-refractory LPR. In total, 22 patients with LPR were enrolled. Following a 2-wk treatment with PPI monotherapy, PPI-refractory LPR patients were randomly divided into two treatment groups (rikkunshito alone or rikkunshito plus the PPI, lansoprazole). LPR symptoms were assessed using a visual analog scale (VAS) score, gastrointestinal symptoms were assessed using the gastrointestinal symptom rating scale (GSRS), and gastric emptying was assessed using the radio-opaque marker method prior to and 4 wk following treatments. The 4-wk treatment with rikkunshito alone and with rikkunshito plus the PPI significantly decreased the globus sensation VAS scores. The VAS score for sore throat was significantly decreased following treatment with rikkunshito plus PPI but not by rikkunshito alone. Neither treatment significantly changed the GSRS scores. Rikkunshito improved delayed gastric emptying. We found a significant positive correlation between improvements in globus sensation and in gastric emptying (r² = 0.4582, P sensation in patients with PPI-refractory LPR, in part, because of stimulation of gastric emptying. Thus, rikkunshito is an effective treatment for PPI-refractory LPR.

CLINICAL SIGNIFICANCE OF ANTIPHOSPHOLIPID ANTIBODIES AND GENE MUTATIONS IN HEMOSTASIS OF CHILDREN WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND JUVENILE DERMATOMYOSITIS

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O.A. Solntseva

2006-01-01

Full Text Available Thrombophilia in children with diffuse connective tissue disorders as systemic lupus erythematosus (SLE and juvenile dermatomyositis (JDM could arise from various causes including peripherial blood circulation of antiphospholipid antibodies (APH and genetic mutations in the system of hemostasis. Thrombosis is a serious and prognostically unfavorable complication that has negative impact on the underlying disease course. The study included 96 children, 65 of them had diagnosed SLE and the other 31 had JDM. The Elisa method was used to detect antiphospholipid antibodies, coagulation method was used to detect lupus anticoagulant (LAC and antibodies to cardiolipins (anticl, ?2:glycoprotein 1 (anti ? 2 gp 1 and prothrombin (APT. The PCR method (DNA diagnostics was used to detect DNA mutations as factor resistance to of activated protein c (Leiden 5,10 methylen tetrahydrofolate reductase (MTHFR gene polymorphism. The incidence of APL antibodies was registered in 61.5% patients with SLE and in 32.2% of patients with JDM. Ac ligg, anti ?2 gp 1 Igg were clinically significant in thrombotic events in patients with SLE and JDM, and so was LAC in patients with SLE. The prevalence of the hemostasis system mutations is concordant with reported data. Conclusion thrombophilia is frequently associated with APH antibodies or combination of APH antibodies with genetic abnormalities. Sole genetic mutations are salient in patients with JDM.Key words: thrombophilia, systemic lupus erythematosus, juvenile dermatomyositis, antiphospholipid antibodies, lupus anticoagulant, leiden, prothrombin, methylentet rahydrofolate reductase.

Engineering high power induction plasma unit at BARC for mass synthesis of refractory nano-ceramics

International Nuclear Information System (INIS)

Ghorui, S.; Sahasrabudhe, S.N.; Dhamale, G.; Das, A.K.

2013-01-01

Atmospheric pressure RF thermal plasma sources are gaining increasing importance for production of high purity novel nano-materials in different high-end technological applications. Inherent electrode-less features of the discharge together with the large volume and high energy density of the produced plasma ensures contamination free process environment and mass production ability. Reported herewith is the development of an indigenous induction plasma system for mass synthesis of nanopowders of refractory ceramic materials. The system has been tested for continuous synthesis of Al 2 O 3 nano-powder at a rate of more than 600 gm per hour and checked for its viability for bulk production of nano-particles of other refractory ceramics like Yttrium oxide and Neodymium Oxide. From collected evidences, the process of formation of the nano-particles is identified as the evaporation and subsequent homogeneous nucleation. Major features observed for alumina are complete conversion into highly spherical nano-sized particles, small particle sizes, very narrow size distribution, highly crystallite nature and mixed phases depending on the zone of collection. For alumina, the particles are found to exhibit a uni-modal distribution with peak near 15 nm

Phenylketonuria mutation analysis in Northern Ireland: A rapid stepwise approach

Energy Technology Data Exchange (ETDEWEB)

Zschocke, J.; Graham, C.A.; Nevin, N.C. [Queen`s Univ., Belfast (Australia)] [and others

1995-12-01

We present a multistep approach for the rapid analysis of phenylketonuria (PKU) mutations. In the first step, three common mutations and a polymorphic short tandem repeat (STR) system are rapidly analyzed with a fluorescent multiplex assay. In the second step, minihaplotypes combining STR and VNTR data are used to determine rare mutations likely to be present in an investigated patient, which are then confirmed by restriction enzyme analysis. The remaining mutations are analyzed with denaturant gradient-gel electrophoresis and sequencing. The first two steps together identify both mutations in 90%-95% of PKU patients, and results can be obtained within 2 d. We have investigated 121 Northern Irish families with hyperphenylalaninemia, including virtually all patients born since 1972, and have found 34 different mutations on 241 of the 242 mutant alleles. Three mutations (R408W, 165T, and F39L) account for 57.5% of mutations, while 14 mutations occur with a frequency of 1%-6%. The present analysis system is efficient and inexpensive and is particularly well suited to routine mutation analysis in a diagnostic setting. 19 refs., 5 tabs.

[Nursing management of a refractory cardiac death donor].

Science.gov (United States)

Kaufmann, Marion

2016-09-01

The nursing management of a refractory circulatory death donor is a new procedure which forms an integral part of patient care. It comprises technical and organisational aspects, and requires a conceptual, ethical and deontological effort. Copyright © 2016. Published by Elsevier Masson SAS.

Pulsed radiofrequency of brachial plexus under ultrasound guidance for refractory stump pain: a case report

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Zheng B

2017-11-01

Full Text Available Bixin Zheng, Li Song, Hui Liu Department of Pain Management, West China Hospital of Sichuan University, Chengdu, China Abstract: The post-amputation (pain syndrome, including stump pain, phantom limb sensation, and phantom limb pain is common but difficult to treat. Refractory stump pain in the syndrome is an extremely challenging and troublesome clinical condition. Patients respond poorly to drugs, nerve blocks, and other effective treatments like spinal cord stimulation and surgery. Pulsed radiofrequency (PRF technique has been shown to be effective in reducing neuropathic pain. This report describes a patient with persistent and refractory upper limb stump pain being successfully relieved with PRF of brachial plexus under ultrasound guidance after a 6-month follow-up period, suggesting that PRF may be considered as an alternative treatment for refractory stump-neuroma pain. Keywords: ultrasound guidance, pulsed radiofrequency, brachial plexus, refractory stump pain 

Development of industrial x-ray computed tomography and its application to refractories

International Nuclear Information System (INIS)

Aiba, Yoshiro; Oki, Kazuo; Nakamura, Shigeo; Fujii, Masashi.

1985-01-01

An industrial X-ray computed tomography was developed under the influence of the rapid spread of the use of the X-ray CT scanner in the medical field and improvements of the equipment. Although current nondestructive testing machines of refractories use the ultrasonic inspection method or the X-ray fluoroscopic method, these equipments cannot produce a tomogram or cannot carry out quantitative evaluation. By using an industrial X-ray computed tomography, submerged nozzles for continuous casting of steel were analyzed with interesting results. The features of the industrial X-ray computed tomography applied for refractory nozzles are as follows: (1) It promptly detects interior defects. (2) It can measure dimensions and shapes. (3) It can numerically express the distribution of density. Accordingly, it is expected that the industrial X-ray computed tomography will widely be used in the fields of development and quality control of refractories and advanced ceramic materials. (author)

Diode and Nd:YAG laser in a case of refractory acne keloidalis nuchae

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Ravi Kumar Chittoria

2015-03-01

Full Text Available Acne keloidalis nuchae (AKN is a disease of unclear etiology that mainly affects males. Medical treatment of AKN is difficult, with refractory cases often requiring ablation by laser or surgical resection. We report herein, a 23-year-old male with refractory AKN treated successfully with combined laser ablation, using an 810-nm diode laser and a 1064-nm Nd:YAG laser.

Outcomes of Ahmed valve surgery for refractory glaucoma in Dhahran, Saudi Arabia

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Tariq Alasbali

2015-06-01

Full Text Available AIM:To evaluate the outcomes of Ahmed glaucoma valve (AGV implantation surgery for refractory glaucoma.METHODS:This one-armed historical cohort study was conducted in 2011. Refractory glaucoma was defined as eyes with an intraocular pressure (IOP greater than 21 mm Hg with maximally tolerated glaucoma medications, failed surgeries, or both. For all eyes with refractory glaucoma that underwent AGV implantation, data were collected on IOP, the best corrected visual acuity (BCVA and glaucoma medications preoperatively and 4, 6, 12, 24 and 56wk postoperatively. Logarithm values of IOP were calculated and compared.RESULTS: The study group was comprised of 30 patients (30 eyes, 16 males and 14 females with refractory glaucoma. Mean preoperative IOP was 39.3±13.8 mm Hg. Postoperative mean IOP was 15.7±7.1 mm Hg, 19.6±12.8 mm Hg and 13.9±14.2 mm Hg at 12, 24 and 56wk respectively. BCVA was ≥ 6/60 in 11 eyes preoperatively, and five eyes had BCVA≥6/60 at 56wk postoperatively. Preoperatively, more than four medications were used to treat glaucoma in 21 eyes. At 12wk postoperatively, no medications were required to control IOP in 20 eyes. At 56wk postoperatively, at least one medication was required to control IOP in 10 eyes. Over the entire follow up period, four eyes were treated with yttrium aluminium garnet (YAG laser and 14 eyes required a second surgery. The AGV was removed in four eyes.CONCLUSION: AGV implantation reduced IOP and the number of medications required to control refractory glaucoma. However, there was a higher risk of decreased vision. Long-term follow up and prompt intervention are recommended.

Determination of the resistance to tensile fracture of refractory mixtures of gunite

International Nuclear Information System (INIS)

Gomez Sanchez, A; Tomba Martinez, A.G

2004-01-01

The determination of the mechanical properties of cooled refractory mixtures is useful since it allows the materials to be compared for the purposes of selection and reports on their degree of internal cohesion, green or calcination, so that their structural ability can be estimated, especially during installation. Given the testing difficulties originating in the fragility of the ceramic materials, the tension test is not generally used in refractories. However, ASTM C-307 94 determines the tensile strength of cured chemical-resistant materials, for which this work considered the possibility of testing cement-based monolithic refractories in this non conventional condition. The tensile resistance to the fracture of three different refractory mixtures (A, B 1 and B 2 ), used in heat repairing by gunite in coking ovens, that were characterized by chemical, granulometric, and mineralogical analysis pycnometric density measurements. The pieces for the tests ('bone' type: length = 75 mm, maximum width = 40 mm, minimum width = 25 mm, thickness = 10 - 25 mm) were prepared by ramming of mixtures of material/water in a metallic mold; they were sinterized (1200 o C, 1h) and characterized by measures of bulk density, porosity and observation of the surface texture, in green and calcinated. The tensile tests, based on ASTM C-307 94, were performed in an Instron model 4467 machine in open air, at room temperature and position control (0.5 mm/min). The following values were obtained, in kPa: AW347±308; B 1 W738±130; B 2 W604±64. These values were lower than those for the tensile fracture module (MOR), although they displayed an equivalent order: A≤B 2 ≤B 1 . This was related to the characteristics of each refractory mixture and at the end of the pieces tested (CW)

An unusual case of refractory wheeze

OpenAIRE

Ramesh, Venkat; Acharya, Vishak; Pai, Narasimha; Krishnan, Ananda

2015-01-01

A 37-year-old man presented with a history of episodic wheeze and breathlessness of 3?years? duration refractory to treatment. Physical examination revealed diffuse expiratory polyphonic rhonchi while the remainder of the examination including the cardiac examination was reported as normal. Pulmonary function testing revealed mild obstruction with bronchodilator reversibility. The patient was discharged on a 6-month course of antitubercular treatment (ATT) as bronchial brush cytology (obtaine...

Supra-Tenon Capsule Implantation of the Ahmed Glaucoma Valve in Refractory Pediatric Glaucoma.

Science.gov (United States)

Elhefney, Eman M; Al-Sharkawy, Hossam T; Kishk, Hanem M

2016-09-01

To evaluate the efficacy of supra-Tenon capsule implantation of an Ahmed glaucoma valve (AGV) as a measure to decrease the fibrotic potential of the Tenon capsule on bleb formation and its subsequent effect on intraocular pressure (IOP) control in children with refractory glaucoma. Mansoura Ophthalmic Centre, Faculty of Medicine, Mansoura University, Egypt. A prospective interventional study. Twenty-two eyes of 12 children with refractory glaucoma underwent supra-Tenon capsule implantation of AGV. Ophthalmic examinations under general anesthesia including measurement of the corneal diameter and the IOP with Perkin's tonometer were performed preoperatively, on the first postoperative day, the first postoperative week, weekly for the first month, 2-weekly for the following 3 months, and monthly for at least 18 months. Postoperative complications and the number of glaucoma medications used preoperatively and postoperatively were recorded. The paired Student t test was used to compare preoperative and postoperative data. There were 12 eyes (54.6%) with refractory congenital glaucoma, 7 eyes (31.8%) with refractory pseudophakic glaucoma, and 3 eyes (13.6%) with refractory aphakic glaucoma. Patients included 10 male (83.3%) and 2 female (16.7%) children with a mean age of 16.3±9.7 months. The mean follow-up duration was 24.1±4.3 months. There was a statistically significant difference between the mean preoperative IOP (30.7±2.88 mm Hg) and the mean postoperative IOP (16.1±3.60 mm Hg) (t=16.22 and P=0.000, with a mean decrease in the IOP by 47.6%). The difference between the mean number of antiglaucoma medications before surgery (1.86±0.4) and after surgery (1.0±0.9) was also statistically significant (t=4.31 and P=0.000). Total success was achieved in 18 eyes (81.9%). Postoperative complications included tube exposure and slippage (10%), hypotony (10%), and hyphema (5%). Supra-Tenon capsule implantation of the AGV was successful in controlling the IOP with few

Spontaneous mutation by mutagenic repair of spontaneous lesions in DNA

International Nuclear Information System (INIS)

Hastings, P.J.; Quah, S.-K.; Borstel, R.C. von

1976-01-01

It is stated that strains of yeast carrying mutations in many of the steps in pathways repairing radiation-induced damage to DNA have enhanced spontaneous mutation rates. Most strains isolated because they have enhanced spontaneous mutation carry mutations in DNA repair systems. This suggests that much spontaneous mutation arises by mutagenic repair of spontaneous lesions. (author)

Refractory chronic migraine

DEFF Research Database (Denmark)

Martelletti, Paolo; Katsarava, Zaza; Lampl, Christian

2014-01-01

The debate on the clinical definition of refractory Chronic Migraine (rCM) is still far to be concluded. The importance to create a clinical framing of these rCM patients resides in the complete disability they show, in the high risk of serious adverse events from acute and preventative drugs...... and in the uncontrolled application of therapeutic techniques not yet validated.The European Headache Federation Expert Group on rCM presents hereby the updated definition criteria for this harmful subset of headache disorders. This attempt wants to be the first impulse towards the correct identification...... of these patients, the correct application of innovative therapeutic techniques and lastly aim to be acknowledged as clinical entity in the next definitive version of the International Classification of Headache Disorders 3 (ICHD-3 beta)....

Single nucleotide polymorphism array lesions, TET2, DNMT3A, ASXL1 and CBL mutations are present in systemic mastocytosis.

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Fabiola Traina

Full Text Available We hypothesized that analysis of single nucleotide polymorphism arrays (SNP-A and new molecular defects may provide new insight in the pathogenesis of systemic mastocytosis (SM. SNP-A karyotyping was applied to identify recurrent areas of loss of heterozygosity and bidirectional sequencing was performed to evaluate the mutational status of TET2, DNMT3A, ASXL1, EZH2, IDH1/IDH2 and the CBL gene family. Overall survival (OS was analyzed using the Kaplan-Meier method. We studied a total of 26 patients with SM. In 67% of SM patients, SNP-A karyotyping showed new chromosomal abnormalities including uniparental disomy of 4q and 2p spanning TET2/KIT and DNMT3A. Mutations in TET2, DNMT3A, ASXL1 and CBL were found in 23%, 12%, 12%, and 4% of SM patients, respectively. No mutations were observed in EZH2 and IDH1/IDH2. Significant differences in OS were observed for SM mutated patients grouped based on the presence of combined TET2/DNMT3A/ASXL1 mutations independent of KIT (P = 0.04 and sole TET2 mutations (P<0.001. In conclusion, TET2, DNMT3A and ASXL1 mutations are also present in mastocytosis and these mutations may affect prognosis, as demonstrated by worse OS in mutated patients.

Influence of phase transformations on the mechanical behaviour of refractory ceramics at high temperature

International Nuclear Information System (INIS)

Schmitt, N.; Poirier, J.

2009-01-01

Refractories used at high temperature are subjected to high chemical and mechanical stresses. The mastery of their microstructure as well as the phase changes occurring in service is essential to ensure resistance to wear and failure of refractory linings. Great progress has been made: combining efficient techniques for the investigation of the microstructure with powerful numerical tools (thermochemical and thermo-mechanical computations) provides information (e.g., degradation mechanisms) that cannot be obtained directly. Also multi-physical and multi-scale models developing materials with high-performance for higher temperature and with longer lifetime. In this paper, through several examples we show some interactions between the mechanical behavior and the microstructure transformations of refractory ceramics. The tools developed to characterize their microstructure change in situ (e.g., at high temperature) and to identify their kinetics are described. Some methodologies and tools developed in recent years, today, provide a better understanding of in-service behavior of refractories while identifying the critical material and process parameters likely to increase life-time. (authors)

Primary pulmonary lymphoma mimicking a refractory lung abscess: A case report.

Science.gov (United States)

Matsumoto, Takeshi; Otsuka, Kojiro; Funayama, Yuki; Imai, Yukihiro; Tomii, Keisuke

2015-04-01

The current study presents a case of primary pulmonary lymphoma (PPL) mimicking refractory lung abscess that was diagnosed at autopsy. An 80-year-old male with clinically inapparent aspiration presented with a large cavitated mass and pleural effusion. A lung abscess and empyema was diagnosed, therefore, antibiotics were administered and the pleural effusion was drained. Various examinations, including a biopsy, yielded no specific diagnosis. The lesion was considered inoperable due to the poor general condition of the patient. Subsequently, the mass that had been diagnosed as a refractory lung abscess became enlarged and a repeat biopsy resulted in a diagnosis of diffuse large B-cell lymphoma. The patient succumbed to sudden respiratory failure, and the final diagnosis of PPL was confirmed at autopsy. PPL is a rare disease that accounts for 0.45% of all pulmonary malignant tumors and is difficult to diagnose in inoperable cases. Therefore, patients with PPL who do not undergo surgery can be misdiagnosed and consequently treated inappropriately. PPL should therefore be considered in the differential diagnosis of a refractory lung abscess.

Chemical compositions of refractory inclusions in the Murchison C2 chondrite

International Nuclear Information System (INIS)

Ekambaram, V.; Kawabe, I.; Tanaka, T.; Davis, A.M.; Grossman, L.

1984-01-01

Samples from ten refractory inclusions in Murchison, some of which are splits of inclusions whose mineralogical and petrographic characteristics are known, have been analysed for thirty-six elements by neutron activation. Six inclusions have group II or group III patterns or variants of such patterns. Two inclusions, BB-5 and MUCH-1, have large negative Yb anomalies unaccompanied by correspondingly large negative Eu anomalies. It is possible that the latter condensed originally with group III patterns and preferentially took up Eu in later exchange processes under reducing conditions. One inclusion, SH-2, has heavy REE enrichment factors that increase with the refractoriness of the REE, indicating the presence of an extremely high-temperature, or ultrarefractory, REE condensate, but it also has a heavy REE/light REE ratio that indicates mixing of that component with a lower-temperature REE condensate. The frequency of highly fractionated REE patterns and absence of group I patterns suggest that refractory inclusions in Murchison stopped equilibrating with the nebular gas at higher temperatures than most Allende-coarse-grained inclusions. The results are discussed. (author)

Benefit–risk assessment of golimumab in the treatment of refractory ulcerative colitis

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Pugliese D

2016-02-01

Full Text Available Daniela Pugliese, Carla Felice, Rosario Landi, Alfredo Papa, Luisa Guidi, Alessandro Armuzzi Inflammatory Bowel Disease Unit, Complesso Integrato Columbus, Catholic University, Rome, Italy Abstract: Significant advances in the management of patients with ulcerative colitis (UC have been made since the introduction of anti-tumor necrosis factor (TNF-alpha agents, especially for those who fail or do not tolerate conventional therapies. Two drugs, infliximab first, then adalimumab afterward, showed effectiveness in inducing and maintaining long-term remission both in pivotal trials as well as in clinical practice. However, approximately 25% of patients with UC, who fail or do not tolerate all available therapies, require a colectomy for refractory disease. The therapeutic scenario of UC has been recently upgraded by the introduction of golimumab, the latest anti TNF-alpha agent to be approved. Golimumab is a totally humanized monoclonal antibody, administered by a subcutaneous injection every 4 weeks. Treatment with golimumab has shown to be effective to induce sustained clinical benefit in tough-to-treat patients with UC, including steroid and/or immunosuppressive refractory and steroid-dependent patients. In this review, we summarize all available efficacy and safety data of golimumab in UC, analyzing the potential therapeutic position for the treatment of refractory patients with UC. Keywords: ulcerative colitis, refractoriness, anti-TNF-alpha, golimumab 

HPA antibodies in Algerian multitransfused patients: Prevalence and involvement in platelet refractoriness.

Science.gov (United States)

Brouk, Hacene; Bertrand, Gérald; Zitouni, Selma; Djenouni, Amel; Martageix, Corinne; Griffi, Fatiha; Kaplan, Cecile; Ouelaa, Hanifa

2015-06-01

Patients receiving cellular blood components may form HLA or HPA antibodies. The frequency and the specificity of HPA antibodies after a series of blood transfusions have never been reported in the Algerian population which is ethnically diverse and runs a higher risk of platelet alloimmunization due to high b allelic frequencies observed for the HPA systems. 117 polytransfused patients were included in this study; the detection of HPA antibodies was performed by the Monoclonal Antibody-specific Immobilization of Platelet Antigens method (MAIPA). Post-transfusion platelet effectiveness was evaluated by the calculation of corrected count increment (CCI). The antibodies against platelets were detected in 10.26% of the patients. In this study, the platelet systems concerned by the alloimmunizations were specifically HPA-1, -3 and -5 with particular predominance of HPA-1. Twenty two patients were refractory to platelet transfusion, as assessed by a CCI; in which 64% have factors associated with increased platelet consumption. Platelet Immunization was found in 14% of platelet refractoriness (PTR) cases. 03 Anti-platelet antibodies were directed against GPIb-IX (n = 1), anti-HPA-1b (n = 1) and anti HPA-5b (n = 1) associated with anti-HLA antibodies in two cases. HLA and HPA alloimmunization is common among chronically transfused patients. PTR detection, identification of the underlying causes, and selection of the appropriate product for transfusion are fundamental to reduce the risk of major bleedings. Copyright © 2014 Elsevier Ltd. All rights reserved.

Sub-micrometer refractory carbonaceous particles in the polar stratosphere

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K. Schütze

2017-10-01

Full Text Available Eleven particle samples collected in the polar stratosphere during SOLVE (SAGE III Ozone loss and validation experiment from January until March 2000 were characterized in detail by high-resolution transmission and scanning electron microscopy (TEM/SEM combined with energy-dispersive X-ray microanalysis. A total of 4202 particles (TEM  =  3872; SEM  =  330 were analyzed from these samples, which were collected mostly inside the polar vortex in the altitude range between 17.3 and 19.9 km. Particles that were volatile in the microscope beams contained ammonium sulfates and hydrogen sulfates and dominated the samples. Some particles with diameters ranging from 20 to 830 nm were refractory in the electron beams. Carbonaceous particles containing additional elements to C and O comprised from 72 to 100 % of the refractory particles. The rest were internal mixtures of these materials with sulfates. The median number mixing ratio of the refractory particles, expressed in units of particles per milligram of air, was 1.1 (mg air−1 and varied between 0.65 and 2.3 (mg air−1. Most of the refractory carbonaceous particles are completely amorphous, a few of the particles are partly ordered with a graphene sheet separation distance of 0.37 ± 0.06 nm (mean value ± standard deviation. Carbon and oxygen are the only detected major elements with an atomic O∕C ratio of 0.11 ± 0.07. Minor elements observed include Si, S, Fe, Cr and Ni with the following atomic ratios relative to C: Si∕C: 0.010 ± 0.011; S∕C: 0.0007 ± 0.0015; Fe∕C: 0.0052 ± 0.0074; Cr∕C: 0.0012 ± 0.0017; Ni∕C: 0.0006 ± 0.0011 (all mean values ± standard deviation.High-resolution element distribution images reveal that the minor elements are distributed within the carbonaceous matrix; i.e., heterogeneous inclusions are not observed. No difference in size, nanostructure and elemental composition was found between

Classical Hodgkin's lymphoma: the Lymphoma Study Association guidelines for relapsed and refractory adult patients eligible for transplant.

Science.gov (United States)

Van Den Neste, Eric; Casasnovas, Olivier; André, Marc; Touati, Mohamed; Senecal, Delphine; Edeline, Véronique; Stamatoullas, Aspasia; Fornecker, Luc; Deau, Bénédicte; Gastinne, Thomas; Reman, Oumédaly; Gaillard, Isabelle; Borel, Cécile; Brice, Pauline; Fermé, Christophe

2013-08-01

The Hodgkin's Lymphoma Committee of the Lymphoma Study Association (LYSA) gathered in 2012 to prepare guidelines on the management of transplant-eligible patients with relapsing or refractory Hodgkin's lymphoma. The working group is made up of a multidisciplinary panel of experts with a significant background in Hodgkin's lymphoma. Each member of the panel of experts provided an interpretation of the evidence and a systematic approach to obtain consensus was used. Grades of recommendation were not required since levels of evidence are mainly based on phase II trials or standard practice. Data arising from randomized trials are emphasized. The final version was endorsed by the scientific council of the LYSA. The expert panel recommends a risk-adapted strategy (conventional treatment, or single/double transplantation and/or radiotherapy) based on three risk factors at progression (primary refractory disease, remission duration < 1 year, stage III/IV), and an early evaluation of salvage chemosensitivity, including (18)fluorodeoxy glucose-positron emission tomography interpreted according to the Deauville scoring system. Most relapsed or refractory Hodgkin's lymphoma patients chemosensitive to salvage should receive high-dose therapy and autologous stem-cell transplantation as standard. Efforts should be made to increase the proportion of chemosensitive patients by alternating non-cross-resistant chemotherapy lines or exploring the role of novel drugs.

Unusual cause of respiratory distress misdiagnosed as refractory asthma

Directory of Open Access Journals (Sweden)

Al-Otair Hadil

2006-01-01

Full Text Available We report a young lady, who was labeled as a case of refractory asthma for a few years, based on history of shortness of breath on minimal exertion, noisy breathing and normal chest radiograph. Repeated upper airway exam by an otolaryngologist and computerized tomography scan, were normal. On presentation to our hospital, she was diagnosed to have fixed upper airway obstruction, based on classical flow-volume loop findings. Fibroptic bronchoscopy revealed a web-shaped subglottic stenosis. The histopathology of a biopsy taken from that area, showed non-specific inflammation. No cause for this stenosis could be identified. The patient was managed with rigid bronchoscopy dilatation, without recurrence. We report this case as idiopathic subglottic stenosis, that was misdiagnosed as refractory bronchial asthma, stressing the importance of performing spirometry in the clinic.

Somatic mutations in Tradescantia as a model system for studying the effects of the environmental agents

International Nuclear Information System (INIS)

Cebulska-Wasilewska, A.

1986-01-01

The application of the plant model system for studying the biological effects of ionizing radiation and chemical mutagens is presented. The model system is based on the somatic mutation frequency in stamen hair cells of Tradescantia clones heterosygous for flower color. The interaction of chemical mutagens with radiation in the induction of somatic mutations was investigated. The results demonstrate the synergistic interaction between radiation and chemical mutagens like ethyl methanesulfonate and di-bromoethane. The synergistic effect is clearly manifested after combined treatment with radiation and chemicals. In the low dose region the effect depends on the radiation dose and chemical exposure. Other results show the influence of the fluoride treatment on the radiation effect. The fluoride treatment is likely to alter the DNA double strand breaks repair processes. Additionally the usefulness of the model system for studying the mutagenic effectiveness of the pollution in the ambient air is presented. 148 refs. (author)

Percutaneous radiofrequency treatment for refractory anteromedial pain of osteoarthritic knees.

Science.gov (United States)

Ikeuchi, Masahiko; Ushida, Takahiro; Izumi, Masashi; Tani, Toshikazu

2011-04-01

Although severe knee osteoarthritis with refractory pain is commonly treated surgically, this is often not an option for patients with poor health status or unwillingness to undergo major surgery. We examined the efficacy of radiofrequency application to sensory nerves as a novel alternative treatment for refractory knee pain. This study was an open-label, nonrandomized, and controlled study. Patients complaining of refractory anteromedial knee pain associated with radiological osteoarthritis (moderate or severe) were included. They were assigned to one of two groups: those receiving radiofrequency thermocoagulation (N = 18) or those receiving nerve block (N = 17), depending on the time period that they were referred to the clinic. Radiofrequency current or local anesthetics was applied to the medial retinacular nerve and the infrapatellar branch of the saphenous nerve. Western Ontario McMaster Universities osteoarthritis index score, pain visual analog scale (VAS), and patient's global assessment were assessed with a minimum follow-up of 6 months.   Radiofrequency treatment significantly decreased knee pain as measured by VAS for 12 weeks compared with the control group. In terms of responders, more patients in the RF group responded to the treatment than in the control group. The differences were statistically significant at 4 weeks, 8 weeks, and 12 weeks in pain VAS. Eight patients (44%) treated with radiofrequency rated excellent or good but only three (18%) in the control group rated good, although the difference was not statistically significant. Some patients were able to benefit substantially from radiofrequency treatment. Even if its effective period is limited, radiofrequency application is a promising treatment to alleviate refractory anteromedial knee pain with osteoarthritis. Further experience and technical improvements are needed to establish its role in the management of knee osteoarthritis. Wiley Periodicals, Inc.

Pembrolizumab in Treating Younger Patients With Recurrent, Progressive, or Refractory High-Grade Gliomas, Diffuse Intrinsic Pontine Gliomas, Hypermutated Brain Tumors, Ependymoma or Medulloblastoma

Science.gov (United States)

2018-06-18

Constitutional Mismatch Repair Deficiency Syndrome; Lynch Syndrome; Malignant Glioma; Progressive Ependymoma; Progressive Medulloblastoma; Recurrent Brain Neoplasm; Recurrent Childhood Ependymoma; Recurrent Diffuse Intrinsic Pontine Glioma; Recurrent Medulloblastoma; Refractory Brain Neoplasm; Refractory Diffuse Intrinsic Pontine Glioma; Refractory Ependymoma; Refractory Medulloblastoma

Lenvatinib in Advanced Radioiodine-Refractory Thyroid Cancer - A Retrospective Analysis of the Swiss Lenvatinib Named Patient Program.

Science.gov (United States)

Balmelli, Catharina; Railic, Nikola; Siano, Marco; Feuerlein, Kristin; Cathomas, Richard; Cristina, Valerie; Güthner, Christiane; Zimmermann, Stefan; Weidner, Sabine; Pless, Miklos; Stenner, Frank; Rothschild, Sacha I

2018-01-01

Purpose: Differentiated thyroid cancer (DTC) accounts for approximately 95% of thyroid carcinomas. In the metastatic RAI-refractory disease, chemotherapy has very limited efficacy and is associated with substantial toxicity. With increasing knowledge of the molecular pathogenesis of DTC, novel targeted therapies have been developed. Lenvatinib is a tyrosine kinase inhibitor (TKI) with promising clinical activity based on the randomized phase III SELECT trial. In Switzerland, a Named Patient Program (NPP) was installed to bridge the time gap to Swissmedic approval. Here, we report the results from the Swiss Lenvatinib NPP including patients with metastatic RAI-refractory DTC. Methods: Main inclusion criteria for the Swiss NPP were RAI-refractory DTC, documented disease progression, Eastern Cooperative Oncology Group (ECOG) performance status 0-3. The number of previous therapies was not limited. The Swiss Lenvatinib NPP was initiated in June 2014 and was closed in October 2015 with the approval of the drug. Results: Between June 2014 and October 2015, 13 patients with a median age of 72 years have been enrolled. Most patients (69%) had at least one prior systemic therapy, mainly sorafenib. 31% of patients showed a PR and 31% SD. Median progression free survival was 7.2 months and the median overall survival was 22.7 months. Dose reduction due to adverse events was necessary in 7 patients (53%). At the time of analysis 6 patients (47%) were still on treatment with a median time on treatment of 9.98 months. Conclusions: Our results show that lenvatinib has reasonable clinical activity in unselected patients with RAI-refractory thyroid cancer with nearly two-third of patients showing clinical benefit. The toxicity profile of lenvatinib is manageable.

Handbook of industrial refractories technology principles, types, properties and applications

CERN Document Server

Caniglia, Stephen

1989-01-01

Encompasses the entire range of industrial refractory materials and forms: properties and their measurement, applications, manufacturing, installation and maintenance techniques, quality assurance, and statistical process control.

Chloroplast mutations induced by 9-aminoacridine hydrochloride are independent of the plastome mutator in Oenothera.

Science.gov (United States)

GuhaMajumdar, M; Baldwin, S; Sears, B B

2004-02-01

Oenothera plants homozygous for the recessive plastome mutator allele ( pm) show chloroplast DNA (cpDNA) mutation frequencies that are about 1,000-fold higher than spontaneous levels. The pm-encoded gene product has been hypothesized to have a function in cpDNA replication, repair and/or mutation avoidance. Previous chemical mutagenesis experiments with the alkylating agent nitroso-methyl urea (NMU) showed a synergistic effect of NMU on the induction of mutations in the pm line, suggesting an interaction between the pm-encoded gene product and one of the repair systems that corrects alkylation damage. The goal of the experiments described here was to examine whether the pm activity extends to the repair of damage caused by non-alkylating mutagens. To this end, the intercalating mutagen, 9-aminoacridine hydrochloride (9AA) was tested for synergism with the plastome mutator. A statistical analysis of the data reported here indicates that the pm-encoded gene product is not involved in the repair of the 9AA-induced mutations. However, the recovery of chlorotic sectors in plants derived from the mutagenized seeds shows that 9AA can act as a mutagen of the chloroplast genome.

The ketogenic diet in two paediatric patients with refractory myoclonic status epilepticus.

Science.gov (United States)

Caraballo, Roberto Horacio; Valenzuela, Gabriela Reyes; Armeno, Marisa; Fortini, Sebastian; Mestre, Graciela; Cresta, Araceli

2015-12-01

We describe two patients with refractory myoclonic status epilepticus treated with the ketogenic diet. Between May 1, 2014 and January 1, 2015, two patients who met the diagnostic criteria for refractory myoclonic status epilepticus, seen at our department, were placed on the ketogenic diet and followed for a minimum of six months. One patient with myoclonic epilepsy of unknown aetiology had a 75-90% seizure reduction, and the other with progressive encephalopathy associated with myoclonic epilepsy had a 50% seizure reduction. Both patients retained good tolerability for the diet. At the last control, one patient had isolated myoclonias and EEG showed occasional generalized spike-and-polyspike waves; the patient is now successfully attending kindergarten. The quality of life of the second patient improved significantly. In both cases, the number of antiepileptic drugs was reduced. The ketogenic diet is an effective and well-tolerated treatment option for patients with refractory myoclonic status epilepticus and should be considered earlier in the course of treatment.

Refractory experience in circulating fluidized bed combustors, Task 7. Final report

Energy Technology Data Exchange (ETDEWEB)

Vincent, R.Q.

1989-11-01

This report describes the results of an investigation into the status of the design and selection of refractory materials for coal-fueled circulating fluidized-bed combustors. The survey concentrated on operating units in the United States manufactured by six different boiler vendors: Babcock and Wilcox, Combustion Engineering, Foster Wheeler, Keeler Dorr-Oliver, Pyropower, and Riley Stoker. Information was obtained from the boiler vendors, refractory suppliers and installers, and the owners/operators of over forty units. This work is in support of DOE`s Clean Coal Technology program, which includes circulating fluidized-bed technology as one of the selected concepts being evaluated.

Refractory organic matter in coastal salt marshes-effect on C sequestration calculations.

Science.gov (United States)

Leorri, Eduardo; Zimmerman, Andrew R; Mitra, Siddhartha; Christian, Robert R; Fatela, Francisco; Mallinson, David J

2018-08-15

The age and ability of salt marshes to accumulate and sequester carbon is often assessed using the carbon isotopic signatures (Δ 14 C and δ 13 C) of sedimentary organic matter. However, transfers of allochthonous refractory carbon (C RF ) from the watershed to marshes would not represent new C sequestration. To better understand how refractory carbon (C RF ) inputs affect assessments of marsh age and C sequestration, Δ 14 C and δ 13 C of both total organic carbon (TOC), C RF , and non-C RF organic matter fractions were measured in salt marshes from four contrasting systems on the North Atlantic coast. To our knowledge, no salt marsh sediment study has considered refractory or allochthonous carbon in carbon budget calculations or the impact on chronologies. Stable and radiogenic isotope data suggest that while TOC was dominated by autochthonous plant inputs, C RF was dominated by locally recycled or allochthonous C, the delivery of which was controlled by the size and slope of each watershed. Steep-gradient rivers analyzed delivered Δ 14 C-depleted C RF to their estuarine marshes, while the site located in the low-gradient river was associated with larger C RF content. Finally, the marsh isolated from riverine input contained the least fraction of TOC as C RF . Laterally transported C RF caused only a small offset in Δ 14 C in relation to TOC in low-gradient systems (average Δ 14 C offset was -44.4 and -24.2‰ at each location). However, the presence of allochthonous Δ 14 C-depleted C RF in sediments of steep-gradient rivers led to large overestimates of the time of organic matter deposition (i.e. apparent age was older than the 'true' time of deposition) (Δ 14 C offset ranged from -170.6 to -528.9‰). Further, reliance on TOC or loss on ignition analyses to calculate C sequestration by marshes might produce overestimates of at least as much as 10 to 20% since neither account for the lateral transport of allochthonous carbon. Copyright © 2018 Elsevier B

Intraarticular Pulsed Radiofrequency to Treat Refractory Lumbar Facet Joint Pain in Patients with Low Back Pain.

Science.gov (United States)

Chang, Min Cheol; Cho, Yun-Woo; Ahn, Da Hyun; Do, Kyung Hee

2018-04-01

Many treatment techniques have been used for refractory lumbar facet joint pain; however, their efficacy has been controversial. In this study, we investigated the clinical efficacy and safety of intra-articular pulsed radiofrequency for the treatment of refractory lumbar facet joint pain in patients with low back pain. Twenty patients with refractory lumbar facet joint pain were recruited, and each patient was treated via intra-articular pulsed radiofrequency. The treatment effects were measured with a numerical rating scale, and the technical accuracy of intra-articular pulsed radiofrequency treatment was evaluated independently by 2 radiologists. Any adverse events or complications also were checked. We performed intra-articular pulsed radiofrequency treatment at 48 levels of the lumbar facet joints in 20 patients (5 men and 15 women; mean age, 64.50 ± 10.65 years) with refractory lumbar facet joint pain. Pain scores were significantly reduced at 1 month, 3 months, and 6 months after treatment (P radiofrequency results in all 20 patients, without any serious adverse effects. Treatment using intra-articular pulsed radiofrequency is an alternative to other techniques in patients with refractory lumbar facet joint pain. Copyright © 2018 Elsevier Inc. All rights reserved.

Comparison of somatic mutation frequencies at HGPRT locus induced by radiation and chemical pollutant from energy system

International Nuclear Information System (INIS)

Xu Honglan; Cao Yi; Duan Zhikai; Wu Qiqing; Chen Ying; Zhang Shuxian

1998-12-01

The somatic induction frequencies of mutation at the hypoxanthine-guanine phosphoribosyl transferase (HGPRT) locus induced by 60 Co γ-rays and Benzo-a-pyrene (B(a)P), which are representative of hazardous emission and pollutant from nuclear energy cycle and fossil-fuelled energy cycle respectively, were detected by using forward mutation assay and cloning technique in both V 79 Chinese hamster cells and human peripheral blood T-lymphocytes. Resistant mutants were selected with 6-thioguanine (6-TG). Dose-response curves and mathematical expressions were obtained for mutation frequencies and survival following γ-ray and B(a)P(+S 9 ) treatments. The dose ranges for the two mutagens were compared when they induced the same mutation frequencies. In V 79 /HGPRT assay system, when the mutation frequencies were 5∼35 mutants/10 6 cells the response of γ-rays in the dose range from 0.93∼4.96 Gy at dose rate of 1.16 Gy/min is nearly equivalent to that in the B(a)P dose range from 0.52∼4.27 μg/ml. By using cloning technique in T-lymphocytes, when the mutation frequencies were 1∼14 mutants/10 5 cells the response of γ-rays in the dose range from 0.05∼4.77 Gy at dose rate of 1.03 Gy/min is nearly equivalent to that in the B(a)P dose range from 0.15∼7.36 μg/ml. When the survival fraction is 37%, the mutation frequency induced by B(a)P is higher than that induced by 60 Co γ-rays

Processing of Refractory Metal Alloys for JOYO Irradiations

International Nuclear Information System (INIS)

RF Luther; ME Petrichek

2006-01-01

This is a summary of the refractory metal processing experienced by candidate Prometheus materiats as they were fabricated into specimens destined for testing within the JOYO test reactor, ex-reactor testing at Oak Ridge National Laboratory (ORNL), or testing within the NRPCT. The processing is described for each alloy from the point of inception to the point where processing was terminated due to the cancellation of Naval Reactor's involvement in the Prometheus Project. The alloys included three tantalum-base alloys (T-111, Ta-10W, and ASTAR-811C), a niobium-base alloy, (FS-85), and two molybdenum-rhenium alloys, one containing 44.5 w/o rhenium, and the other 47.5 w/o rhenium. Each of these alloys was either a primary candidate or back-up candidate for cladding and structural applications within the space reactor. Their production was intended to serve as a forerunner for large scale production ingots that were to be procured from commercial refractory metal vendors such as Wah Chang

Towards linked open gene mutations data

Science.gov (United States)

2012-01-01

Background With the advent of high-throughput technologies, a great wealth of variation data is being produced. Such information may constitute the basis for correlation analyses between genotypes and phenotypes and, in the future, for personalized medicine. Several databases on gene variation exist, but this kind of information is still scarce in the Semantic Web framework. In this paper, we discuss issues related to the integration of mutation data in the Linked Open Data infrastructure, part of the Semantic Web framework. We present the development of a mapping from the IARC TP53 Mutation database to RDF and the implementation of servers publishing this data. Methods A version of the IARC TP53 Mutation database implemented in a relational database was used as first test set. Automatic mappings to RDF were first created by using D2RQ and later manually refined by introducing concepts and properties from domain vocabularies and ontologies, as well as links to Linked Open Data implementations of various systems of biomedical interest. Since D2RQ query performances are lower than those that can be achieved by using an RDF archive, generated data was also loaded into a dedicated system based on tools from the Jena software suite. Results We have implemented a D2RQ Server for TP53 mutation data, providing data on a subset of the IARC database, including gene variations, somatic mutations, and bibliographic references. The server allows to browse the RDF graph by using links both between classes and to external systems. An alternative interface offers improved performances for SPARQL queries. The resulting data can be explored by using any Semantic Web browser or application. Conclusions This has been the first case of a mutation database exposed as Linked Data. A revised version of our prototype, including further concepts and IARC TP53 Mutation database data sets, is under development. The publication of variation information as Linked Data opens new perspectives

Towards linked open gene mutations data.

Science.gov (United States)

Zappa, Achille; Splendiani, Andrea; Romano, Paolo

2012-03-28

With the advent of high-throughput technologies, a great wealth of variation data is being produced. Such information may constitute the basis for correlation analyses between genotypes and phenotypes and, in the future, for personalized medicine. Several databases on gene variation exist, but this kind of information is still scarce in the Semantic Web framework. In this paper, we discuss issues related to the integration of mutation data in the Linked Open Data infrastructure, part of the Semantic Web framework. We present the development of a mapping from the IARC TP53 Mutation database to RDF and the implementation of servers publishing this data. A version of the IARC TP53 Mutation database implemented in a relational database was used as first test set. Automatic mappings to RDF were first created by using D2RQ and later manually refined by introducing concepts and properties from domain vocabularies and ontologies, as well as links to Linked Open Data implementations of various systems of biomedical interest. Since D2RQ query performances are lower than those that can be achieved by using an RDF archive, generated data was also loaded into a dedicated system based on tools from the Jena software suite. We have implemented a D2RQ Server for TP53 mutation data, providing data on a subset of the IARC database, including gene variations, somatic mutations, and bibliographic references. The server allows to browse the RDF graph by using links both between classes and to external systems. An alternative interface offers improved performances for SPARQL queries. The resulting data can be explored by using any Semantic Web browser or application. This has been the first case of a mutation database exposed as Linked Data. A revised version of our prototype, including further concepts and IARC TP53 Mutation database data sets, is under development.The publication of variation information as Linked Data opens new perspectives: the exploitation of SPARQL searches on

Determination of Unit Pressure Force in Material Volume in the Course of Refractory Stamping Press Moulding

Directory of Open Access Journals (Sweden)

Orłowicz A.W.

2016-06-01

Full Text Available The paper presents results of assessment of the unit pressure force within the refractory material volume in the course press-moulding of stampings for refractory precast shapes. The force was evaluated with the use of physical simulation of deformation undergone by lead balls placed in the raw refractory mass subjected to pressing in a metal die. To determine the value of unit pressure force applied to the aggregate grains in the course of stamping press-moulding, physical model of deformation of a sphere induced by the uniaxial stress state was used.

Normal and refractory concretes for LMFBR applications. Volume 1. Review of literature on high-temperature behavior of portland cement and refractory concretes. Final report

International Nuclear Information System (INIS)

Bazant, Z.P.; Chern, J.C.; Abrams, M.S.; Gillen, M.P.

1982-06-01

The extensive literature on the properties and behavior at elevated temperature of portland cement concrete and various refractory concretes was reviewed to collect in concise form the physical and chemical properties of castable refractory concretes and of conventional portland cement concretes at elevated temperature. This survey, together with an extensive bibliography of source documents, is presented in Volume 1. A comparison was made of these properties, the relative advantages of the various concretes was evaluated for possible liquid metal fast breeder reactor applications, and a selection was made of several materials of interest for such applications. Volume 2 concludes with a summary of additional knowledge needed to support such uses of these materials together with recommendations on research to provide that knowledge

The thermo-technical optimization in the evaporator by a targeted selection of refractory systems. Calculations and evaluation, installation of a tailor-made refractory system, results from the practice; Waermetechnische Optimierung im Dampferzeuger durch gezielte Auswahl von Feuerfestsystemen. Berechnungen und Bewertungen, Installation des massgeschneiderten Feuerfestsystems, Ergebnisse aus der Praxis

Energy Technology Data Exchange (ETDEWEB)

Martin, Karl-Ulrich [Mokesa AG, Birsfelden (Switzerland). Marketing/Verkauf; Hofmans, Erik [ISVAG, Wilrijk (Belgium). Technischer Dienst/Instandhaltung; Kern, Tobias [Mokesa AG, Birsfelden (Switzerland); Hoeff, Jos van der [Mokesa Benelux B.V., Limmen (Netherlands)

2013-03-01

The thermo-technical optimization of existing incinerator boilers with the benefit of a stabilised combustion and a low consumption of fossil fuels in order to meet the rules at a temperature of 850 Celsius for a time period of two seconds even at partial load and low-calorific fuel (waste) is state of the art by means of CFD modelling and its successful transformation into the practice with proven and tailor-made refractory systems. The direct, straight and sustainable economical use is in an enhanced throughput of waste materials, a reduced consumption of fuels and thus a conservation of CO{sub 2}. The stabile combustion control is with a poor readjustment is an important and assuring advantage for the operations manager.

The first USH2A mutation analysis of Japanese autosomal recessive retinitis pigmentosa patients: a totally different mutation profile with the lack of frequent mutations found in Caucasian patients.

Science.gov (United States)

Zhao, Yang; Hosono, Katsuhiro; Suto, Kimiko; Ishigami, Chie; Arai, Yuuki; Hikoya, Akiko; Hirami, Yasuhiko; Ohtsubo, Masafumi; Ueno, Shinji; Terasaki, Hiroko; Sato, Miho; Nakanishi, Hiroshi; Endo, Shiori; Mizuta, Kunihiro; Mineta, Hiroyuki; Kondo, Mineo; Takahashi, Masayo; Minoshima, Shinsei; Hotta, Yoshihiro

2014-09-01

Retinitis pigmentosa (RP) is a highly heterogeneous genetic disease. The USH2A gene, which accounts for approximately 74-90% of Usher syndrome type 2 (USH2) cases, is also one of the major autosomal recessive RP (arRP) causative genes among Caucasian populations. To identify disease-causing USH2A gene mutations in Japanese RP patients, all 73 exons were screened for mutations by direct sequencing. In total, 100 unrelated Japanese RP patients with no systemic manifestations were identified, excluding families with obvious autosomal dominant inheritance. Of these 100 patients, 82 were included in this present study after 18 RP patients with very likely pathogenic EYS (eyes shut homolog) mutations were excluded. The mutation analysis of the USH2A revealed five very likely pathogenic mutations in four patients. A patient had only one very likely pathogenic mutation and the others had two of them. Caucasian frequent mutations p.C759F in arRP and p.E767fs in USH2 were not found. All the four patients exhibited typical clinical features of RP. The observed prevalence of USH2A gene mutations was approximately 4% among Japanese arRP patients, and the profile of the USH2A gene mutations differed largely between Japanese patients and previously reported Caucasian populations.

Design of Refractory High-Entropy Alloys

Science.gov (United States)

Gao, M. C.; Carney, C. S.; Doğan, Ö. N.; Jablonksi, P. D.; Hawk, J. A.; Alman, D. E.

2015-11-01

This report presents a design methodology for refractory high-entropy alloys with a body-centered cubic (bcc) structure using select empirical parameters (i.e., enthalpy of mixing, atomic size difference, Ω-parameter, and electronegativity difference) and CALPHAD approach. Sixteen alloys in equimolar compositions ranging from quinary to ennead systems were designed with experimental verification studies performed on two alloys using x-ray diffraction, energy-dispersive spectroscopy, and scanning electron microscopy. Two bcc phases were identified in the as-cast HfMoNbTaTiVZr, whereas multiple phases formed in the as-cast HfMoNbTaTiVWZr. Observed elemental segregation in the alloys qualitatively agrees with CALPHAD prediction. Comparisons of the thermodynamic mixing properties for liquid and bcc phases using the Miedema model and CALPHAD are presented. This study demonstrates that CALPHAD is more effective in predicting HEA formation than empirical parameters, and new single bcc HEAs are suggested: HfMoNbTiZr, HfMoTaTiZr, NbTaTiVZr, HfMoNbTaTiZr, HfMoTaTiVZr, and MoNbTaTiVZr.

Time-resolved analysis of particle emissions from residential biomass combustion - Emissions of refractory black carbon, PAHs and organic tracers

Science.gov (United States)

Nielsen, Ingeborg E.; Eriksson, Axel C.; Lindgren, Robert; Martinsson, Johan; Nyström, Robin; Nordin, Erik Z.; Sadiktsis, Ioannis; Boman, Christoffer; Nøjgaard, Jacob K.; Pagels, Joakim

2017-09-01

Time-resolved particle emissions from a conventional wood stove were investigated with aerosol mass spectrometry to provide links between combustion conditions, emission factors, mixing state of refractory black carbon and implications for organic tracer methods. The addition of a new batch of fuel results in low temperature pyrolysis as the fuel heats up, resulting in strong, short-lived, variable emission peaks of organic aerosol-containing markers of anhydrous sugars, such as levoglucosan (fragment at m/z 60). Flaming combustion results in emissions dominated by refractory black carbon co-emitted with minor fractions of organic aerosol and markers of anhydrous sugars. Full cycle emissions are an external mixture of larger organic aerosol-dominated and smaller thinly coated refractory black carbon particles. A very high burn rate results in increased full cycle mass emission factors of 66, 2.7, 2.8 and 1.3 for particulate polycyclic aromatic hydrocarbons, refractory black carbon, total organic aerosol and m/z 60, respectively, compared to nominal burn rate. Polycyclic aromatic hydrocarbons are primarily associated with refractory black carbon-containing particles. We hypothesize that at very high burn rates, the central parts of the combustion zone become air starved, leading to a locally reduced combustion temperature that reduces the conversion rates from polycyclic aromatic hydrocarbons to refractory black carbon. This facilitates a strong increase of polycyclic aromatic hydrocarbons emissions. At nominal burn rates, full cycle emissions based on m/z 60 correlate well with organic aerosol, refractory black carbon and particulate matter. However, at higher burn rates, m/z 60 does not correlate with increased emissions of polycyclic aromatic hydrocarbons, refractory black carbon and organic aerosol in the flaming phase. The new knowledge can be used to advance source apportionment studies, reduce emissions of genotoxic compounds and model the climate impacts of

The effects of baroreflex activation therapy on blood pressure and sympathetic function in patients with refractory hypertension

DEFF Research Database (Denmark)

Gordin, Daniel; Fadl Elmula, Fadl Elmula M; Andersson, Bert

2017-01-01

). Results: The primary end point is the reduction in 24-hour systolic ABPM by BAT at 8 months, as compared to pharmacotherapy. Secondary and tertiary endpoints are effects of BAT on home and office blood pressures, measures of indices of cardiac and vascular structure and function during follow......Objective: To explore the effects of baroreflex activation therapy (BAT) on hypertension in patients with treatment resistant or refractory hypertension. Methods: This investigator-initiated randomized, double-blind, 1:1 parallel-design clinical trial will include 100 patients with refractory...... hypertension from 6 tertiary referral hypertension centers in the Nordic countries. A Barostim Neo System will be implanted and after 1 month patients will be randomized to either BAT for 16 months or continuous pharmacotherapy (BAT off) for 8 months followed by BAT for 8 months. A second randomization...

Identification of GLI Mutations in Patients With Hirschsprung Disease That Disrupt Enteric Nervous System Development in Mice.

Science.gov (United States)

Liu, Jessica Ai-Jia; Lai, Frank Pui-Ling; Gui, Hong-Sheng; Sham, Mai-Har; Tam, Paul Kwong-Hang; Garcia-Barcelo, Maria-Mercedes; Hui, Chi-Chung; Ngan, Elly Sau-Wai

2015-12-01

Hirschsprung disease is characterized by a deficit in enteric neurons, which are derived from neural crest cells (NCCs). Aberrant hedgehog signaling disrupts NCC differentiation and might cause Hirschsprung disease. We performed genetic analyses to determine whether hedgehog signaling is involved in pathogenesis. We performed deep-target sequencing of DNA from 20 patients with Hirschsprung disease (16 men, 4 women), and 20 individuals without (controls), and searched for mutation(s) in GLI1, GLI2, GLI3, SUFU, and SOX10. Biological effects of GLI mutations were tested in luciferase reporter assays using HeLa or neuroblastoma cell lines. Development of the enteric nervous system was studied in Sufu(f/f), Gli3(Δ699), Wnt1-Cre, and Sox10(NGFP) mice using immunohistochemical and whole-mount staining procedures to quantify enteric neurons and glia and analyze axon fasciculation, respectively. NCC migration was studied using time-lapse imaging. We identified 3 mutations in GLI in 5 patients with Hirschsprung disease but no controls; all lead to increased transcription of SOX10 in cell lines. SUFU, GLI, and SOX10 form a regulatory loop that controls the neuronal vs glial lineages and migration of NCCs. Sufu mutants mice had high Gli activity, due to loss of Sufu, disrupting the regulatory loop and migration of enteric NCCs, leading to defective axonal fasciculation, delayed gut colonization, or intestinal hypoganglionosis. The ratio of enteric neurons to glia correlated inversely with Gli activity. We identified mutations that increase GLI activity in patients with Hirschsprung disease. Disruption of the SUFU-GLI-SOX10 regulatory loop disrupts migration of NCCs and development of the enteric nervous system in mice. Copyright © 2015 AGA Institute. Published by Elsevier Inc. All rights reserved.

Removal of Refractory Organics from Biologically Treated Landfill Leachate by Microwave Discharge Electrodeless Lamp Assisted Fenton Process

Directory of Open Access Journals (Sweden)

Jiuyi Li

2015-01-01

Full Text Available Biologically treated leachate usually contains considerable amount of refractory organics and trace concentrations of xenobiotic pollutants. Removal of refractory organics from biologically treated landfill leachate by a novel microwave discharge electrodeless lamp (MDEL assisted Fenton process was investigated in the present study in comparison to conventional Fenton and ultraviolet Fenton processes. Conventional Fenton and ultraviolet Fenton processes could substantially remove up to 70% of the refractory organics in a membrane bioreactor treated leachate. MDEL assisted Fenton process achieved excellent removal performance of the refractory components, and the effluent chemical oxygen demand concentration was lower than 100 mg L−1. Most organic matters were transformed into smaller compounds with molecular weights less than 1000 Da. Ten different polycyclic aromatic hydrocarbons were detected in the biologically treated leachate, most of which were effectively removed by MDEL-Fenton treatment. MDEL-Fenton process provides powerful capability in degradation of refractory and xenobiotic organic pollutants in landfill leachate and could be adopted as a single-stage polishing process for biologically treated landfill leachate to meet the stringent discharge limit.

Role of rectal myomectomy in refractory chronic constipation ...

African Journals Online (AJOL)

Background: To assess the role of diagnostic and therapeutic value of anorectal myectomy in cases of chronic refractory constipation. Materials and Methods: Twenty-eight patients 11 months to 9 years of age presenting with chronic constipation, with contrast enema showing dilated rectum and sigmoid colon were included ...

Atypical Presentation of Herpes Simplex Virus Type 2 Infection Refractory to Treatment With Acyclovir in 2 Hematologic Patients.

Science.gov (United States)

Nieto Rodríguez, D; Sendagorta Cudós, E; Rueda Carnero, J M; Herranz Pinto, P

2017-12-01

Herpesvirus infections are not uncommon in hematologic patients. Our first patient, diagnosed with chronic lymphatic leukemia, presented extensive genital herpes infection refractory to treatment with acyclovir and with a partial response to foscarnet, which had to be withdrawn due to systemic adverse effects. The second patient, diagnosed with follicular Hodgkin lymphoma, presented hypertrophic herpes infection refractory to treatment with acyclovir but that responded to intralesional cidofovir and topical imiquimod. As in other immunodepressed patients, herpesvirus infection in hematologic patients can present atypical manifestations, as well as resistance to treatments that act via the viral thymidine kinase. A high level of clinical suspicion is therefore needed to make an early diagnosis, together with extensive knowledge of the different treatments available. Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.