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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 64 (1986), S. 197-205 
    ISSN: 1432-1440
    Keywords: Alpha-1 antitrypsin ; Heterozygous deficiency ; Chronic liver disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Homozygous deficiency of alpha-1 antitrypsin is the most common inborn error or metabolism in Europe. Severe deficiency of this major protease inhibitor in serum is associated with chronic obstructive lung disease, chronic liver disease in adults and neonatal hepatitis. An overview is given of the role of heredity, and the diagnostic criteria and clinical and histological findings in this disorder. Emphysema seems to be caused by the free elastolytic activity of white cells, leading to the degradation of elastin. The pathophysiology of liver disease — less well understood — is discussed with special emphasis on the importance of heterozygous alpha-1 antitrypsin deficiency. Exogenous noxae seem to play an important role in the pathogenesis of heterozygous deficiency. In view of the 7% frequency of heterozygous alpha-1 antitrypsin deficiency in the European population and the role of noxae in the development of pulmonary and liver diseases, improved prophylaxis is mandatory.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 60 (1982), S. 1-7 
    ISSN: 1432-1440
    Keywords: LIver fibrosis ; N-terminal peptide of procollagen type III ; N-acetyl-β-glucosaminidase ; Procollagen prolyl hydroxylase ; Antifibrotic therapy ; Leberfibrose ; N-terminales Peptid des Prokollagen Typ III ; N-Acetyl-β-Glucosaminidase ; Prokollagen-Prolyl-Hydroxylase ; Antifibrotische Therapie
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Im zweiten Teil dieser Übersicht werden die klinischen Aspekte des Bindegewebsstoffwechsels in der fibrotischen Leber dargestellt. Besonders eingegangen wird auf die Möglichkeit, die Aktivität der Leberfibrose durch neuere Laborproben zur Erfassung von Enzymen und Metaboliten des Bindegewebsstoffwechsels zu verfolgen. Für diesen Zweck geeignet erscheinen die Enzyme Prokollagen-Prolyl-Hydroxylase, die lysosomale N-Acetyl-β-Glucosaminidase und das N-terminale Peptid vom Prokollagen Typ III. Die Aktivitäten bzw. Konzentrationen im Serum von Patienten mit chronisch aktiven Leberkrankheiten korrelieren gut mit der histologisch geschätzten Aktivität der Fibrose im punktierten Lebergewebe der gleichen Patienten. Weiterhin werden die therapeutischen Versuche zur Behandlung der Leberfibrose mit bindegewebsspezifischen Substanzen besprochen.
    Notes: Summary In this second part, clinical aspects of connective tissue metabolism in the liver will be described and two main aspects considered. The first is the possibility to monitor the activity of fibrosis by the use of metabolites and enzymes of connective tissue metabolism. In recent studies the qualification for this purpose of enzymes such as procollagen prolyl hydroxylase and lysosomal N-Acetyl-β-glucosaminidase and the N-terminal peptide of procollagen type III has been tested. The serum activities or concentrations of these substances in patients with chronic active liver diseases increase in due relation to the histologically estimated activity of liver fibrosis. The second aspect deals with therapeutic approaches to fibrosis in the liver by using connective tissue specific agents. So far none of the antifibrotic substances such as proline analogues, colchicine, lathyrogens and penicillamine has been used in longer-term antifibrotic treatment.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1440
    Keywords: Prokollagen-Prolyl-Hydroxylase ; N-Acetyl-β-Glucosaminidase ; Leberfibrose ; Leberbiopsie ; Procollagen-Prolyl-Hydroxylase ; N-Acetyl-β-glucosa-minidase ; Fibrosis in the Liver ; Liver Biopsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary In 5 mg of liver tissue obtained by needle biopsy from patients with chronic liver diseases, activity of both Procollagen-prolyl-hydroxylase and N-Acetyl-β-glucosaminidase was measured. The activity of both enzymes increased due to the activity of fibrosis, but prolyhydroxylase increased more. The relation of these two enzyme activities in the development of liver fibrosis is discussed.
    Notes: Zusammenfassung In 5 mg Lebergewebe, gewonnen durch Punktion von Patienten mit chronischen Leberkrankheiten, wurde die Aktivität der Prokollagen-Prolyl-Hydroxylase und der N-Acetyl-β-Glucosaminidase bestimmt. Die Aktivität beider Enzyme ist parallel zum histologisch bestimmten Aktivitätsgrad der Fibrose erhöht. In Relation zur N-Acetyl-β-Glucosaminidase steigt die Aktivität der Prolylhydroxylase stärker an. Die Relation beider Enzymaktivitäten in Zusammenhang mit der Entwicklung einer Leberfibrose wird besprochen.
    Type of Medium: Electronic Resource
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