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  • 1
    ISSN: 1432-1076
    Keywords: Key words 3-Oxo-Δ4-steroid 5β-reductase deficiency ; 3-oxo-Δ4 bile acid ; Immunoblot analysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 3-oxo-Δ4-steroid 5β-reductase (5β-reductase) deficiency is difficult to diagnose because severe liver damage can result in a similar pattern of metabolite excretion. We investigated the usefulness of immunoblot analysis for diagnosis of 5β-reductase deficiency and quantitatively analysed urinary bile acids by gas chromatography-mass spectrometry in a 5-month-old Japanese boy with severe neonatal cholestasis associated with hypertyrosinaemia. A liver sample was examined by immunoblot analysis using monoclonal antibodies against 5β-reductase. Urinary 3-oxo-Δ4 bile acids accounted for 88.3% of total bile acids, 5α-bile acids for 0.9%, and primary bile acids for 9.1%. Immunoblot analysis of the liver tissue showed an indistinct band of 5β-reductase. Conclusions These findings suggest that this patient had a secondary 5β-reductase deficiency due to severe liver damage, even though 3-oxo-Δ4 bile acids constituted more than 70% of total urinary bile acids. However, the patient may possibly have had an inherited 5β-reductase deficiency.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0630
    Keywords: PACS: 78.20.Hp; 42.30.Va; 65.90.+i
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: Abstract. The noncontact imaging of the buried structures is carried out in the open-air atmosphere by using the photothermal deflection (PTD) method. We applied these techniques to the layered samples. Besides the PTD images for the optically opaque buried structures, the parameters of the materials such as thermal diffusivity can also be calculated from the PTD amplitude and phase signal in the PTD scanning images. When the PTD signals at two different modulation frequencies are used, the thermal diffusivity of the buried structure can be obtained from the PTD signal outside of the sample nondestructively.
    Type of Medium: Electronic Resource
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