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  • 1
    Electronic Resource
    Electronic Resource
    Multiple acyl-CoA dehydrogenation deficiency (glutaric aciduria type II), congenital polycystic kidneys, and symmetric warty dysplasia of the cerebral cortex in two brothers (1982)
    Springer
    European journal of pediatrics 139 (1982), S. 56-59 
    Details
    ISSN: 1432-1076
    Keywords: Glutaric aciduria type II ; Multiple acyl-CoA dehydrogenase deficiency ; Organic aciduria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two male siblings suffering from a severe form of glutaric aciduria type II were studied. One patient died within one hour after birth, the other at the age of five days. Both patients presented with respiratory distress soon after birth. They had a variety of congenital morphologic abnormalities. One patient's outstanding “sweaty-feet” odour on the second day of life led to organic acid analysis of urine revealing massive lactic, glutaric, and ethylmalonic aciduria along with a high excretion of various other carboxylic acids and glycine conjugates of the branched chain carboxylic acids. The pattern of metabolites in serum and urine as well as results of degradation studies with various substrates in cultured fibroblasts were consistent with a defect in multiple acyl-CoA dehydrogenation. The morphological abnormalities are presented in a subsequent paper.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442081
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Characterisation of a spark ignition system by planar laser-induced fluorescence of OH at high repetition rates and comparison with chemical kinetic calculations (2000)
    Springer
    Applied physics 70 (2000), S. 287-294 
    Details
    ISSN: 1432-0649
    Keywords: PACS: 07.50.-e; 07.60.-j; 47.11.+j
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Abstract. This study reports the application of a novel, high speed laser-detector system for the time-resolved study of flame propagation in a well-controlled spark ignition system. The ignition system allowed full and reproducible control over the energy deposited during breakdown and the ensuing arc discharge of the spark plasma. Ignition was performed in a closed vessel which was filled with stoichiometric mixtures of methane and air. Four sequential snapshots of two-dimensional OH distributions were recorded during single ignition events by the use of planar laser-induced fluorescence (PLIF). From these OH distributions flame front velocities have been extracted with an accuracy of better than 2%. One-dimensional numerical simulations of the ignition event including detailed chemistry and transport processes have been performed. Experimental results and results from the simulations have been compared to each other with respect to flame front velocities as well as spatial concentration profiles of OH radicals. In general a good agreement was obtained. In this way the ignition system was carefully characterised.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s003400050047
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Flame growth and wrinkling in a turbulent flow (2000)
    Springer
    Applied physics 71 (2000), S. 711-716 
    Details
    ISSN: 1432-0649
    Keywords: PACS: 47.27.-i; 42.62.-b
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Abstract. High-speed planar laser-induced fluorescence (PLIF) and 3-D large eddy simulations (LES) are used to study turbulent flame kernel growth, wrinkling and the formation of separated flame pockets in methane/air mixtures. Turbulence was effected by a set of rotary fans situated in a cylindrical enclosure. Flame wrinkling was followed on sequential 2-D OH images captured at kHz repetition rates. Under stoichiometric conditions and low turbulence levels the flame kernel remains singly connected and close to spherical in shape. By increasing turbulence or reducing the stoichiometry of the mixture the formation of separated pockets could be observed and studied. The mechanisms behind these phenomena are investigated qualitatively by LES of a level-set G-equation describing the flame surface propagation in turbulent flows.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s003400000425
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    Paper (German National Licenses)
    Fulltext
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