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  • 1
    ISSN: 1432-2307
    Keywords: Liver ; Copper storage ; Indian childhood cirrhosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A female child of non-consanguineous, healthy German parents fell ill at the age of 7 months with a progressive liver disease leading to irreversible hepatic failure 3 months later. Histological examination revealed severe liver cell necrosis, excessive Mallory body formation and veno-occlusive-like changes associated with massive storage of copper, similar to Indian childhood cirrhosis (ICC). Chronic copper contamination of drinking water was the only detectable aetiological factor. The study illustrates that ICC most probably is an environmental disease, also occurring outside the Indian subcontinent, and is likely to be underdiagnosed in the Western world.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Graefe's archive for clinical and experimental ophthalmology 174 (1968), S. 367-376 
    ISSN: 1435-702X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary In the cortex of clear and cataractuous human lenses the activity of glucose-6-phosphate-dehydrogenase and of 6-phospho-gluconate-dehydrogenase was determined by a standardised UV-method (Bücher et al.). We measured a higher elevated activity of glucose-6-phosphatedehydrogenase in the lens than in the liver, the activity of 6-phospho-gluconate-dehydrogenase was a little lower. Relating the activities of both enzymes to soluble lens protein, in cataractuous human lenses there is an increased activity which imposes particularly in so called “Permeabilitätskatarakt” and in totally opaque lenses. Relating the enzyme-activities to the data of a glycolytic enzyme (GAPDH) we get a significant relative increase in activity of the enzymes of the pentose-phosphate-cycle. Relating the enzyme activities to wet-weight, totally opaque lenses show significant lower data than other forms of cataract and clear lenses.
    Notes: Zusammenfassung In der Binde klarer und getrübter menschlicher Linsen wurden die Aktivitäten der Glucose-6-phosphat-Dehydrogenase und der 6-Phosphogluconat-Dehydrogenase im standardisierten optischen Test bestimmt. In der Linse konnten etwas höhere Aktivitäten der Glucose-6-phosphat-Dehydrogenase als in der Leber gemessen werden. Die Aktivität der 6-Phosphogluconat-Dehydrogenase liegt etwas unter den für die Leber angegebenen Werten. Beide Enzyme zeigen bei Bezug ihrer Aktivität auf das lösliche Linsenprotein in getrübten menschlichen Linsen eine Aktivitätssteigerung, die besonders ausgeprägt bei der Permeabilitätskatarakt und bei total getrübten Linsen auftritt. Werden die Enzymaktivitäten auf die Werte eines glykolytischen Enzyms (GAPDH) bezogen, so ergibt sich in Permeabilitätskatarakten und total getrübten Linsen eine deutliche relative Steigerung der Aktivität der Enzyme des Pentose-Phosphat-Cyclus.
    Type of Medium: Electronic Resource
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