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  • 1
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Primäre biliäre Zirrhose ; Chronische aktive Hepatitis ; Antigenpräsentierende Zellen ; Key words Primary biliary cirrhosis ; Chronic active hepatitis ; Antigen-presenting cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Liver tissue from 14 female patients with primary biliary cirrhosis (PBC; stages I–IV) was systematically investigated for the prevalence and distribution of dendritic antigen-presenting cells. Dendritic cells (DCs) were immunhistochemically identified by use of anti-S 100 protein and KiMlp antibodies. We confirm previous findings that, in early PBC, S 100 protein-positive DCs can be detected within the lining of bile duct epithelia. However, the present study disclosed that S 100 protein- and KiMlp-positive DCs regularly occur in piecemeal necroses (PMNs) developing in PBC-associated chronic hepatitis. DCs in PMNs were observed in all PBC stages, but were most prominent in late-stage PBC. These findings suggest that autoimmune tissue damage in PBC may not be limited to bile ducts, but may also ensue in hepatic parenchyma, producing the pattern of chronic hepatitis with signs of activity.
    Notes: Zusammenfassung In der vorliegenden retrospektiven Untersuchung wurden Lebergewebsproben von 14 Patientinnen mit gesicherter primärer biliärer Zirrhose (PBZ) der Stadien I bis IV hinsichtlich Häufigkeit und Verteilung dendritischer, antigenpräsentierender Zellen systematisch untersucht. Dendritische Zellen (DZ) wurden immunhistochemisch mittels Anti-S-100-Proteinantikörper und KiMlp-Antikörper identifiziert. Wir bestätigen frühere Beobachtungen, daß sich bei der PBZ innerhalb von Gallengangsepithelien S-100-Protein-positive DZ nachweisen lassen. Als neuer und pathogenetisch wahrscheinlich wichtiger Befund zeigte sich indessen, daß DZ (S-100-Ag+ und/oder KiMlp+) auch in Piecemealnekrosen (PMN), welche die mit PBZ assoziierten hepatischen Veränderungen kennzeichnen, vorkommen. DZ in PMN treten in allen PBZ-Stadien auf, finden sich indessen gehäuft in den späten Stadien. Aufgrund dieser Beobachtung formulieren wir die Hypothese, daß bei der PBZ Autoimmunprozesse nicht nur im Bereich der Gallengänge, sondern auch im hepatozytären Parenchym stattfinden.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Osteogenesis imperfecta type II ; Collagen type I ; Genetic defect ; Alkaline phosphatase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have examined collagen from a patient with the rare type IIC form of perinatally lethal osteogenesis imperfecta, in whom biochemical characteristics of hypophosphatasia were also apparent. In addition to normal α1(I) and α2(I) chains, there were chains overmodified along their lengths. Unexpectedly, the thermal stability of molecules containing these chains was normal. This suggests the existence of a structural mutation causing delayed triple helix formation, situated in either the α1(I) or α2(I) C-terminal propeptide. Since collagen synthesised by fibroblasts from each of the patient's parents was normal, the mutation was probably newly arising and dominant. In contrast to other reported cases of lethal osteogenesis imperfecta, not only was the secretion of collagen by cultured fibroblasts considerably retarded, but that of non-collagenous proteins was also severely impaired.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 150 (1991), S. 557-559 
    ISSN: 1432-1076
    Keywords: Sclerosing cholangitis ; Crohn disease ; Liver cirrhosis ; Adolescence
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 17-year-old patient suffering from Crohn disease (CD) and liver cirrhosis is presented. At an advanced stage of the disease, he died of a concomitant urosepsis. Autopsy showed that the liver cirrhosis was caused by sclerosing cholangitis. This very rare complication of CD in adolescence is discussed.
    Type of Medium: Electronic Resource
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