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  • 1
    Electronic Resource
    Electronic Resource
    Hämolytische Anämie bei Morbus Wilson (Hepatolentikuläre Degeneration) (1972)
    Springer
    Journal of molecular medicine 50 (1972), S. 995-1002 
    Details
    ISSN: 1432-1440
    Keywords: M. Wilson ; Hemolytic anemia ; copper metabolism ; Erythrocyte enzymes ; Allosteric inhibition of enzymes ; Pyruvate kinase ; Glucose-6-phosphate dehydrogenase ; Glutathione reductase ; M. Wilson ; Hämolytische Anämie ; Kupferstoffwechsel ; Erythrocytenenzyme ; Allosterische Hemmung von Enzymen ; Pyruvatkinase ; Glucose-6-phosphatdehydrogenase ; Glutathionreductase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es werden 4 Fälle von Anämie bei Wilsonscher Erkrankung beschrieben, bei denen die hämolytische Anämie der Diagnosestellung des Morbus Wilson zum Teil um Jahre vorausging. Auf die Bedeutung der hämolytischen Anämie als Initial-Symptom des Morbus Wilson wird hingewiesen. Die Ursache der hämolytischen Anämie ist eine akute Kupfervergiftung durch das zu diesem Zeitpunkt erhöhte freie, nicht an Coeruloplasmin gebundene Kupfer. Kupfer-Ionen in einer Konzentration von 3 × 10−5 M, wie sie bei Wilson-Kranken im Frühstadium vorkommen, hemmen reversibel die Aktivität der Pyruvatkinase menschlicher Erythrocyten. Der Mechanismus der Hämolyse beruht nach den beschriebenen experimentellen Ergebnissen eher auf einer Hemmung der Aktivität der Erythrocyten-Pyruvatkinase und anderer glykolytischer Enzyme als auf einer Hemmung der Glucose-6-Phosphatdehydrogenase oder der Glutathion-Reductase.
    Notes: Summary This report on hemolytic anemia in Wilson's disease emphasizes the importance of the initial finding “hemolysis” in Wilson's disease. Four cases are described, in which hemolytic anaemia preceded the diagnosis of M. Wilson, in part, for years. The premature destruction of red cells is caused by acute periodic increases of unbound copper in both plasma and red cells. Copper concentrations of 3×10−5 M observed early in Wilson's disease, reversibly inhibit the pyruvate kinase of human red cells. Fructose-1,6-diphosphate is a potent allosteric effector allowing to overcome the copper effect. The mechanism of hemolysis may be due to the inhibition of pyruvate kinase and other erythrocyte enzymes, not to the inhibition of glucose-6-phosphate dehydrogenase and glutathione reductase, as reported in recent papers.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01486994
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  • 2
    Electronic Resource
    Electronic Resource
    Tumor cell colonies in bone marrow cultures from patients with small cell carcinoma of the lung (1984)
    Springer
    Annals of hematology 48 (1984), S. 227-232 
    Details
    ISSN: 1432-0584
    Keywords: Bone marrow mestastases ; Bone marrow cultures
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Bone marrow specimens from 27 patients with small cell carcinoma of the lung (17 with limited and 10 with extensive disease) were plated in a culture system that supports the growth of multilineage haemopoietic progenitors CFU-GEMM. In five patients (three with extensive and two limited disease) atypical colonies could be observed that were not identifiable as haemopoietic colonies. Cytological staining and cytochemical examination as well as electronic micrographs suggest that these colonies are derived from metastatic carcinoma cells. The histological examination of marrow cells from three out of these five patients revealed no bone marrow involvement. Additional studies might provide further evidence whether bone marrow cultures are a useful probe in order to monitor bone marrow involvement in patients with small cell carcinoma of the lung.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00319814
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  • 3
    Electronic Resource
    Electronic Resource
    Expression of transferrin receptors and intracellular ferritin during terminal differentiation of human monocytes (1984)
    Springer
    Annals of hematology 49 (1984), S. 195-202 
    Details
    ISSN: 1432-0584
    Keywords: Monocyte-Macrophages ; Differentiation ; Transferrin receptors ; Ferritin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Human blood monocytes when cultured on hydrophobic Teflon membranes differentiate into mature macrophages. The expression of transferrin receptors was monitored by monoclonal antibody (OKT9) binding as detected by immunperoxidase staining. Whereas monocytes were negative, an increasing percentage of macrophages, starting from day 2 in culture, labelled with the anti-transferrin receptor antibody as these cells undergo differentiation. After completion of maturation more than 90% of macrophages expressed transferrin receptors. While 90–95% of macrophages from broncho-alveolar lavage fluids labelled with the OKT9 antibody, only a minor portion of macrophages obtained from peritoneal and pleural cavities did so. In parallel, intracellular ferritin in cells of the monocyte-macrophage lineage increased from 10ng/106 cells to 350–1,500ng/106 cells during maturation in vitro. Alveolar macrophages proved to have the highest ferritin content which ranged from 355–8,400ng/106. The results may indicate that iron uptake and storage is a function of cells at late stages of macrophage maturation and that the occurrence of surface receptors for transferrin can be regarded as differentiation dependent marker.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00319822
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  • 4
    Electronic Resource
    Electronic Resource
    Glucosephosphate isomerase deficiency type Liège: A new variant with congenital nonspherocytic hemolytic anemia (1977)
    Springer
    Annals of hematology 35 (1977), S. 187-193 
    Details
    ISSN: 1432-0584
    Keywords: Hemolytic anemia ; Enzyme deficiency ; Glucosephosphate isomerase ; Hämolytische Anämie ; Enzymdefekt ; Glukosephosphat-Isomerase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei einem 3jährigen Mädchen marokkanischer Abstammung, das seit Geburt an einer hämolytischen Anämie leidet, wurde ein GPI-Defekt der Erythroxyten festgestellt. Das defekte Enzym ist sehr thermolabil und zeigt in der Stärkegelelektrophorese eine einzelne Bande mit einer Wanderungsgeschwindigkeit von 96 % der normalen Hauptbande. Nach einer 16000fachen Anreicherung mit einer Ausbeute von 35% war das gereinigte Enzym außergewöhnlich instabil sogar bei niedrigen Temperaturen. Die kinetischen Konstanten der Vor- und Rückreaktion sowie die Inhibitorkonstanten für 2,3-DPG und 6-PG wichen nicht signifikant von den Werten des Wildtyps ab. Die biochemischen Eigenschaften des Enzyms sprechen für eine neue Defektvariante, die nach dem Geburtsort der Patientin Typ Liège genannt wurde.
    Notes: Summary GPI deficiency was detected in a three year old girl of Morrocan origin suffering, since birth, from hemolytic anemia. The defective GPI is very thermolabile and migrates on starch gel electrophoresis as a single band with a mobility of 96 % of the normal main band. The purification of the patient's GPI resulted in a 16000-fold enriched preparation, free of any other enzyme activity. The yield was 35 %. The purified enzyme was very unstable even at low temperature. The kinetic constants of the forward and backward reaction as well as the inhibitory constants of 2,3-DPG and 6-PG do not significantly differ from normal values. The bio-chemical properties of the patient's GPI indicate a new variant designated type Liège.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00999459
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  • 5
    Electronic Resource
    Electronic Resource
    Augsburg-type glucosephosphate isomerase deficiency (1980)
    Springer
    Annals of hematology 40 (1980), S. 107-115 
    Details
    ISSN: 1432-0584
    Keywords: Hemolytic anemia ; Enzyme deficiency ; Glucosephosphate isomerase ; Hämolytische Anämie ; Enzymdefekt ; Glucosephosphat-Isomerase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei einem einjährigen deutschen Jungen wurde als Ursache für eine chronische hämolytische Anämie mit rezidivierenden hämolytischen Krisen ein GPI-Defekt der Erythrozyten entdeckt. Bei Konsanguinität der Eltern handelt es sich um einen homozygoten Defektträger. Die Untersuchung der biochemischen Eigenschaften des Defektenzymes ergab ein verändertes elektrophoretisches Muster, eine deutliche Thermolabilität, eine erhöhte Affinität für G6P und für den kompetitiven Inhibitor 6-PG und leicht zum alkalischen verschobene pH-Optima für beide Substrate. Von der Elektrophorese nach Einfrieren und Auftauen des Hämolysates kann geschlossen werden, daß die genetische Modifikation der GPI-Untereinheit hauptsächlich den Mechanismus betrifft, der beim normalen Enzym die Hauptbande in die Sekundärbanden überführt. Die einmaligen Eigenschaften des Defektenzyms sprechen für eine neue Variante, für die nach dem Geburtsort des Patienten der Name GPI Augsburg vorgeschlagen wird.
    Notes: Summary In a 1-year-old German boy a GPI deficiency was found to be the cause of a chronic nonspherocytic hemolytic anemia with recurrent hemolytic crises. Because of consanguinity of the parents, the patient is true homozygote. The investigation of the biochemical properties of the deficient enzyme revealed an altered electrophoretic behavior, pronounced thermolability, an increased affinity for G6P, an increased affinity for the competitive inhibitor 6-PG, and slightly changed pH optima for both substrates. Electrophoresis after freezing and thawing the hemolysate indicates that the genetic modification of the subunit involves the mechanism of transforming the main band into the secondary bands. The properties of the new deficient GPI indicate a new variant designated GPI Augsburg.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01013693
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  • 6
    Electronic Resource
    Electronic Resource
    Erythrocyte and plasma ferritin in normal subjects, blood donors and iron deficiency anemia patients (1984)
    Springer
    Annals of hematology 48 (1984), S. 131-137 
    Details
    ISSN: 1432-0584
    Keywords: Ferritin ; Iron metabolism ; Erythrocytes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Ferritin concentration has been determined with an immunoradiometric assay in plasma and washed sedimented erythrocytes after hypotonic lysis. There was a gradual decrease of plasma ferritin in the sequence normal males, normal females, blood donors and patients with iron deficiency anemia. Erythrocyte ferritin remained unchanged in normal males and females and in blood donors, but dropped significantly in the anemic patients. Correspondingly, the ratio of erythrocyte to plasma ferritin rose from less than 2 in healthy males up to 8 in persons with iron deficiency. Little, if any effect on plasma and erythrocyte ferritin was observed in 12 male and female volunteers when taking iron for 4 weeks. In 2 patients with iron deficiency anemia the blood counts were normalized within 2–3 months during oral iron substitution, accompanied by a drastic increase of the erythrocyte ferritin concentration to values far above normal. In contrast, the plasma ferritin concentration remained below normal. Thus, in iron deficiency erythrocyte ferritin is synthesized with priority in the presence of iron and, in addition to plasma ferritin, appears to be a useful parameter of the iron status.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00320335
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  • 7
    Electronic Resource
    Electronic Resource
    Ferritin in erythrocytes and plasma of patients with iron overload (1985)
    Springer
    Annals of hematology 51 (1985), S. 25-31 
    Details
    ISSN: 1432-0584
    Keywords: Ferritin ; Iron overload ; Erythrocyte
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Erythrocyte and plasma ferritin was followed in 13 patients with iron overload undergoing phlebotomies for at least 6 months in comparison with untreated patients and normal males. Plasma ferritin was widely scattered with an average of only twice the normal, whereas erythrocyte ferritin was highly elevated to about twelve times the normal (p〈0.0001). — The time course of plasma and erythrocyte ferritin during phlebotomy therapy was analyzed in 3 patients with idiopathic hemochromatosis. Three stages were established: 1. plasma ferritin dropped gradually into the normal range while erythrocyte ferritin remained high, 2. appropriate phlebotomies maintained normal plasma ferritin and high erythrocyte ferritin, and indicated a monthly uptake of dietary iron of 150–200 mg at a steady state, 3. at low plasma ferritin levels, erythrocyte ferritin was rapidly decreased by further intensive phlebotomy therapy. Based on the presumed net removal of iron, 1 μg/l plasma ferritin was equivalent to 3–6 mg of body iron and 1 μg/l erythrocyte ferritin to somewhat less than 1 mg of body iron. — An elevated erythrocyte ferritin during phlebotomy therapy in iron overload not only depends on body iron stores like plasma ferritin but may also be regulated by the activity of erythropoiesis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00320597
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