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Sökning: WFRF:(Berk M.)

  • Resultat 11-20 av 33
  • Föregående 1[2]34Nästa
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18.
  • Berk, H. L., et al. (författare)
  • Explanation of the JET n=0 chirping mode
  • 2006
  • Ingår i: Nuclear Fusion. - 0029-5515 .- 1741-4326. ; 46:10, s. S888-S897
  • Tidskriftsartikel (refereegranskat)abstract
    • <p>Persistent rapid up and down frequency chirping modes with a toroidal mode number of zero (n = 0) are observed in the JET tokamak when energetic ions, in the range of several hundred keV, are created by high field side ion cyclotron resonance frequency heating. Fokker-Planck calculations demonstrate that the heating method enables the formation of an energetically inverted ion distribution which supplies the free energy for the ions to excite a mode related to the geodesic acoustic mode. The large frequency shifts of this mode are attributed to the formation of phase space structures whose frequencies, which are locked to an ion orbit bounce resonance frequency, are forced to continually shift so that energetic particle energy can be released to counterbalance the energy dissipation present in the background plasma.</p>
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  • Berk, JL, et al. (författare)
  • The diflunisal trial : update on study drug tolerance and disease progression
  • 2011
  • Ingår i: Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis. - 1744-2818. ; 18:Suppl. 1, s. 191-192
  • Tidskriftsartikel (refereegranskat)abstract
    • <p>Abstract: Familial amyloidotic polyneuropathy (FAP) is a lethal genetic disorder that affects the peripheral and autonomic nervous systems, heart, gastro-intestinal (GI) tract, and soft tissues. Disease progression is increasingly reported following liver transplantation, the only proven treatment for FAP. Small molecule thyroxine mimetics stabilize transthyretin, inhibiting FAP amyloid fibril formation under stringent <em>in vitro</em> conditions. We report on the progress of an international, randomized placebo-controlled study designed to determine the effect of diflunisal, a thyroxine mimetic, on neurologic disease progression in patients with active FAP. Our experience to date indicates diflunisal is well tolerated by this study cohort and that neurologic disease advances more rapidly in FAP than it does in diabetes mellitus.</p> <p>Background: Transthyretin-related familial amyloidotic polyneuropathy (FAP) is a lethal autosomal dominant genetic disorder that predominantly affects the peripheral nervous system. FAP amyloid fibrils result from the misfolding of transthyretin, a transport protein predominantly produced by the liver. Although liver transplantation effectively treats patients with certain FAP mutations and limited disease, reports increasingly document progressive amyloid deposition following transplantation [1,2]. Alternative treatments are needed. <em>In vitro</em> investigations and a phase I clinical trial have demonstrated that thyroxine and small molecule mimetics, e.g. diflunisal, inhibit tetrameric transthyretin dissociation and suppress amyloid fibril formation [3,4].</p> <p>Methods: To examine the effect of diflunisal on disease progression in FAP, we designed a randomized, placebo controlled, double blind, multicenter international study employing the validated diabetic (DM) polyneuropathy metric, Neurologic Impairment Score + 7 attributes (NIS+7®), as the primary endpoint. A two-point change in NIS+7 correlates with clinically detectable progression of peripheral neuropathy among diabetics [5]. Entry criteria include proven FAP genotype, biopsy-proven amyloid deposits, and peripheral or autonomic neuropathy. Patients with alternate causes of neuropathy, other NSAID use, severe heart or kidney dysfunction, or previous liver transplantion are excluded. Study evaluations occur at entry, 6, 12, and 24 months. Adverse are collected by monthly telephone interviews, diary entries, and study site visit interactions. Relatedness of adverse events to study drug is assigned according to documentation in the investigational brochure, the protocol, the informed consent form; or at the investigator's discretion.</p> <p>Results: To date, 90 subjects have enrolled – 62 men and 28 women with median age 63 years (range 27–76 years). Adverse events tabulated by affected organ systems predominantly involved gastrointestinal events, more often attributed to disease complications than study drug side effects (Table 1). Although rare events, congestive heart failure in two subjects and GI bleeding in another prompted study drug discontinuation. Two disease-related deaths have occurred, both off study drug. Aggregate data from all study subjects (placebo and active drug arms) followed for at least 12 months identified a 3.2 point increase in median NIS+7 summated scores. In contrast, Dyck et al. [6] reported an annual 0.85 point increase in NIS+7 median scores in a large cohort of diabetics with polyneuropathy. Taken together, NIS+7 detected neurologic disease progression in this FAP cohort after 12 months observation. Additionally, NIS+7 measured disease advanced <img src="http://informahealthcare.com/na101/home/literatum/publisher/ashley/journals/entities/223C.gif" />3.5 times faster in our aggregate FAP study population than previously reported in DM.</p> <p>Conclusions: Diflunisal is well tolerated in FAP patients participating in the study. NIS+7, a composite scoring system, appears to be an effective study instrument for ATTR neuropathy, detecting significant change over 12 months observation. Neurologic disease progresses more rapidly in FAP than DM cohorts. The exact rate of disease progression in untreated FAP subjects detected by NIS+7 awaits unblinding of the data. These data will provide basis for future study design in FAP patients.</p>
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  • Borysov, Stanislav S., et al. (författare)
  • Online search tool for graphical patterns in electronic band structures
  • 2018
  • Ingår i: Npj Computational Materials. - 2057-3960. ; 4
  • Tidskriftsartikel (refereegranskat)abstract
    • <p>Many functional materials can be characterized by a specific pattern in their electronic band structure, for example, Dirac materials, characterized by a linear crossing of bands; topological insulators, characterized by a Mexican hat pattern or an effectively free electron gas, characterized by a parabolic dispersion. To find material realizations of these features, manual inspection of electronic band structures represents a relatively easy task for a small number of materials. However, the growing amount of data contained within modern electronic band structure databases makes this approach impracticable. To address this problem, we present an automatic graphical pattern search tool implemented for the electronic band structures contained within the Organic Materials Database. The tool is capable of finding user-specified graphical patterns in the collection of thousands of band structures from high-throughput calculations in the online regime. Using this tool, it only takes a few seconds to find an arbitrary graphical pattern within the ten electronic bands near the Fermi level for 26,739 organic crystals. The source code of the developed tool is freely available and can be adapted to any other electronic band structure database.</p>
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  • Resultat 11-20 av 33
  • Föregående 1[2]34Nästa
 
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