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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 133 (1980), S. 287-292 
    ISSN: 1432-1076
    Keywords: Goldenhar syndrome ; Hemifacial microsomia ; Developmental field complex ; Mental retardation ; Multiple congenital anomalies syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Three patients with oculoauriculovertebral dysplasia (Goldenhar) or hemifacial microsomia are presented. One had ocular, oral and auricular anomalies; another had vertebral malformations in addition to ocular and oro-auricular anomalies, and in a third only oro-auricular malformations were evident. The oculoauriculovertebral malformation complex is regarded as a variety of bilateral hemifacial microsomia, with the vertebral defects, the rare occipital encephalocele and cleft of lip and palate presumably representing midline interaction between the 2 fields. Hemifacial microsomia is a causally non-specific developmental field complex (DFC) which usually occurs sporadically, but can also be seen as an autosomal dominant trait and as a component manifestation in the 18 trisomy syndrome. Pathogenetic and therapeutic considerations are also discussed.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1573-7217
    Keywords: clearance ; cystic disease fluid protein ; fibrinogen ; GCDFP-15 ; glycoprotein ; Kupffer cells ; immunocytochemistry ; metabolism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Gross Cystic Disease Fluid Protein (GCDFP-15) is a 60,000 dalton glycoprotein isolated from human breast cyst fluid, composed of four 15,000 dalton monomers. Carbohydrate analysis indicates that each monomer has a single carbohydrate chain of the complex type. GCDFP-15 intravenously injected into rats showed a rapid circulatory clearance, the rate of clearance being faster in female animals [t1/2 = 12.8 (± 2.0) min. females, and 16.7 (± 2.6) min. males]. The major organs of clearance were the liver (70%) and kidneys (15%). Immunoperoxidase staining showed localization in Kupffer cells and the proximal convoluted tubules of the kidney. Removal of sialic acid from GCDFP-15 resulted in a more rapid clearance (t1/2 = 2.2 min) by the liver (85%). This clearance was inhibited by coinjection of asialo alpha1 acid glycoprotein. About 3% of GCDFP-15 was excreted in bile with a transit time through the liver of 38 min. Examination of the uptake of GCDFP-15 by isolated rat Kupffer cells showed that yeast mannan, fucosylated BSA, and carcinoembryonic antigen (CEA) failed to inhibit uptake, though the binding of GCDFP-15 was clearly saturable. This suggests that a novel receptor system on the rat Kupffer cell may be responsible for GCDFP-15 clearance.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1573-0794
    Keywords: Comets ; missions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Geosciences , Physics
    Notes: Abstract In 1997, the COmet Nucleus TOUR (CONTOUR) was selected byNASA for a new start as part of the Discovery line. In this paper, we review the status of the mission, the mission timeline and the instruments to be flown. Detail is given of the science goals and how they are to be accomplished.
    Type of Medium: Electronic Resource
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