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  • 1
    ISSN: 1432-1076
    Keywords: Mevalonate kinase deficiency ; Mevalonic aciduria ; Heterozygote ; Autosomal-recessive inheritance ; Cholesterol bisoynthesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Mevalonate kinase deficiency has been documented in an 8-year-old child who presented with cerebellar ataxia, hypotonia and mevalonic aciduria. The activity of mevalonate kinase in extracts of cultured skin fibroblasts derived from the patient was approximately 2% of the mean value for controls. Family studies were carried out on the mother, the father and a sister, all of whom were clinically well. Mevalonate kinase activity in extracts of cultured skin fibroblasts and transformed lymphoblasts derived from the parents of the patient were 43%–52% of the mean control values. These data are consistent with an autosomal recessive mode of inheritance for mevalonate kinase deficiency.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Diagnostic value ; Gliadin IgG, IgA, and IgE antibody determinations ; Coeliac disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The diagnostic value of gliadin IgG, IgA and IgE antibody (AB) determinations using the fluorescent immunosorbent test was examined in 586 children with malabsorptive disorders and/or failure to thrive. All patients underwent jejunal biopsy and were on a gluten-containing diet. IgG AB were found in all patients (331/331) with untreated coeliac disease (CD) in our study, but IgA AB in only 295/331 (89%). Therefore a screening test based only on IgA AB determinations is not recommended. By contrast, 203 (80%) of 255 children with other malabsorptive disorders had no gliadin AB, 43 (16.5%) had only IgG AB and only 9 (3.5%) had IgG and IgA AB. IgE AB proved to be of no additional value as a diagnostic tool because they were found in a quarter of the children without CD. Statistical evaluation of combined IgG and IgA AB determination showed at least 96% sensitivity and a specificity of 97%. The subjective (“Bayesian”) probability that an actual patient with a given AB test result has CD, is considered: a patient very probably has CD in the case of positive IgG and IgA AB, and no CD in the case of a negative AB result. In the case of negative IgA AB but positive IgG AB the physician's judgement (“prior probability”) influences the (“posterior”) probability of CD for an actual patient. In contrast to IgG AB, IgA AB decline rapidly after the introduction of a gluten-free diet and may be used for diet control after diagnosis. Antibodies against cow's milk proteins, though present in 72% of the 331 patients with CD, are of no therapeutic significance in CD and are of no value for its diagnosis.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Coeliac disease ; Different dietary conditions ; Fluorescent immunosorbent test for IgG gliadin antibodies ; Leucocyte migration inhibition test with gluten subfractions B2 and B3
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The diagnostic value of the fluorescent immunosorbent test for IgG gliadin antibodies (FIST) has been investigated in comparison with the LIF test—the competence of the gluten subfractions B2 and B3 in releasing lymphokines from peripheral lymphocytes in vitro—in 96 patients with coeliac disease (CD) under various dietary conditions. In untreated children with CD during their first 2 years of life, the FIST showed 100% sensitivity with 95% specificity, whilst the LIF test showed only 70% sensitivity and 73% specificity. Therefore it can be concluded that the FIST as a screening test is superior to the LIF. In older children with a proved recurrence of the mucosal abnormality after reintroduction of a normal diet, only 44% showed increased IgG gliadin antibody titres whereas 70% proved to be positive in the LIF test. Under a controlled gluten challenge all six patients reacted with a distinct increase in gliadin antibody titres whereas the LIF test changed from positive to negative and vice versa without following any clear principle. These results emphasize the inadequacy of the LIF test as a diagnostic method, both in untreated CD and under controlled gluten challenge.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    [S.l.] : American Institute of Physics (AIP)
    Physics of Fluids 31 (1988), S. 3109-3115 
    ISSN: 1089-7666
    Source: AIP Digital Archive
    Topics: Physics
    Notes: The stimulated Brillouin scattering spectrum from a plasma is determined by the ion acoustic normal mode frequencies, which are dependent on the ion temperature and flow velocity. Thus these quantities are indirectly measured by the scattered light. The frequencies of ion acoustic normal modes and stimulated Brillouin scattered light are calculated for a model plasma formed by collisionlessly interpenetrating ion beams by assuming the ion–ion two-stream instability is marginally stable. The spectrum differs measurably from the spectrum expected from the hot, stationary plasma created by collisional stagnation. Comparison of the scattered light spectra for these two limiting cases suggests that careful observation of the scattered light near the laser frequency is a potentially useful diagnostic of the collisionality of interpenetrating ion beams.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    New York, NY : American Institute of Physics (AIP)
    Physics of Fluids 1 (1989), S. 1850-1854 
    ISSN: 1089-7666
    Source: AIP Digital Archive
    Topics: Physics
    Notes: The (3)/(2) ω0 harmonic emission has been observed from a laser-produced plasma using exploding foil targets illuminated by 1.053 μm laser light with a 300 psec square pulse. The shift of the measured wavelength from (2)/(3) λ0 is found to depend markedly on the target thickness. Variation of the flow velocity and thus the Doppler shifts of the wave frequency with target thickness is found to be more important than variation of the electron temperature. Adjusting the measured wavelength by Doppler shifts calculated with flow velocities from simulations brings the measurements from different target thicknesses into agreement. These results indicate that previous predictions of electron temperatures from analyses of half-harmonic spectra from inertial confinement fusion plasmas are up to a factor of 2 too small.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    New York, NY : American Institute of Physics (AIP)
    Physics of Fluids 1 (1989), S. 414-421 
    ISSN: 1089-7666
    Source: AIP Digital Archive
    Topics: Physics
    Notes: The flux of radiation emitted from an inhomogeneous plasma by stimulated Raman and Brillouin scattering is calculated with a source that includes both bremsstrahlung and longitudinal plasma wave noise sources. Significant enhancement of the flux above that found for a bremsstrahlung source alone are found for stimulated Brillouin scattering and for nonthermal but stable velocity distributions.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    [S.l.] : American Institute of Physics (AIP)
    Physics of Fluids 28 (1985), S. 2895-2909 
    ISSN: 1089-7666
    Source: AIP Digital Archive
    Topics: Physics
    Notes: Calculations have been made of the spectrum of light emitted at half-integer harmonics of the laser light frequency ω0. The emitted light results from either the Raman scattering of laser light by Langmuir waves or by the direct conversion of Langmuir waves to light. The Langmuir wave amplitude is calculated from convective instability theory for two-plasmon decay in an inhomogeneous, plane-stratified medium. The Raman scattering process generates harmonics at (n/2) ω0 where n=1,3,5 ...; however, only for n=1 and 3 is the emission level significant. The conversion process produces (1/2) ω0 light with a comparable efficiency to the Raman scattering process near the two-plasmon instability threshold. Results of the calculation are in agreement with the general features of measured emission spectra.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    [S.l.] : American Institute of Physics (AIP)
    Physics of Fluids 29 (1986), S. 583-593 
    ISSN: 1089-7666
    Source: AIP Digital Archive
    Topics: Physics
    Notes: Stimulated Raman scattering (SRS) was studied in experiments with laser-irradiated Au disk targets. Typically the laser energy was 150 J in a 1.0 nsec pulse at a wavelength of 526 nm. Laser intensity at the target was ∼7×1014 W/cm2. The spectrum, angular distribution, onset time, duration, and intensity of the Raman light were measured. In addition the electron density distribution was determined by holographic interferometry and images of the Raman emission were obtained. These data show that the Raman light is primarily backscattered and that scattering occurs at electron plasma densities 0.01 nc ≤n≤0.15nc. SRS occurred at laser intensities which are below theoretical thresholds, given the plasma conditions. Otherwise, most of the data support the present understanding of Raman scattering.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    [S.l.] : American Institute of Physics (AIP)
    Physics of Fluids 28 (1985), S. 2419-2431 
    ISSN: 1089-7666
    Source: AIP Digital Archive
    Topics: Physics
    Notes: The behavior of the convective two-plasmon decay instability is studied using a kinetic theory formulation which is applicable to high-temperature plasmas. Theoretical values for the Langmuir wave amplification are obtained in several limits. The temperature-dependent effects of Landau damping and kinetic theory corrections to the coupling coefficients are studied. Landau damping significantly reduces the spectrum of unstable modes for T〉1 keV, while kinetic corrections become important for T〉10 keV. The nonresonant scattering of Langmuir waves by thermal electrons is calculated and is found to be comparable to the resonant convective process for T〉20 keV.
    Type of Medium: Electronic Resource
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