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  • 1
    ISSN: 1432-1440
    Keywords: Osteogenic sarcoma ; Methotrexate ; Radioimmunoassay ; Osteosarkom ; Methotrexat ; Radioimmunoassay
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei 22 Patienten mit osteogenem Sarkom, die 103 hochdosierte Methotrexatinfusionen (6–8,5 g/m2 in 4–6 h) erhielten, wurde mit einem eigenen spezifischen und rasch durchführbaren Radioimmunoassay die Plasmapharmakokinetik des Methotrexats untersucht. Bei nichttoxischen Verläufen lag die Plasmakonzentration nach 24 h unter 8,0 × 10−6 mol/l, nach 48 h unter 8,0 × 10−7 mol/l und nach 72 h unter 4,25 × 10−7 mol/l. Alle Patienten mit 48 h-Werten über 1 × 10−6 mol/l entwickelten schwere toxische Erscheinungen in Form von Knochenmarksdepression und Stomatitis, die durch eine verzögerte Ausscheidung des Methotrexats bedingt war. Der Anstieg des Serumkreatinins war kein zuverlässiges Kriterium für toxische Verläufe. Die Bestimmung der 48- und 72h-Methotrexat-Plasmakonzentrationen erwies sich als zuverlässiger Parameter zur Erfassung von Patienten mit drohender Toxizität. Sie ermöglicht somit, rechtzeitige therapeutische Maßnahmen, z.B. in Form einer zusätzlichen Leukovorintherapie zu ergreifen.
    Notes: Summary In 22 patients with osteogenic sarcoma, treated with 103 high-dose methotrexate infusions (6–8.5 g/m2 in 4–6 h) plasma methotrexate levels were measured with a specific and rapid radioimmunoassay. Nontoxic infusions were associated with methotrexate concentrations below 8.0 × 10−6 mol/l at 24 h, 8.0 × 10−7 mol/l at 48 h and 4.25 × 10−7 mol/l at 72 h. All patients with 48 h methotrexate levels above 1 × 10−6 mol/l manifested severe toxicity with myelosuppression and stomatitis due to delayed methotrexate excretion. Rise of serum creatinine was not reliable to predict oxicity. Determination of 48-and 72-h methotrexate concentrations proved to be a valuable method for identifying patients at high risk for toxic side effects. Additional citrovorum factor may thus be given in time.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Wolman's disease ; Storage ; Cholesterylester ; Triglycerides ; Acid lipase ; Adrenal calcification ; Dyserythropoietic changes ; Foam cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A case of Wolman's disease is described in a German infant who died at the age of 4 months. Hepatosplenomegaly, abdominal distention, gastrointestinal symptoms, dyserythropoietic changes in the bone marrow, but not adrenal calcification on X-ray were present. Stored lipid material could be demonstrated in liver, spleen, intestine, adrenals, thymus, kidneys, blood cells, but not in the central nervous system. Cholesterylesters and triglycerides were markedly increased in liver and spleen. Lysosomal acid lipase was found to be decreased in leucocytes and liver to less than 10% of normal, when measured with synthetic and natural substrates.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 137 (1981), S. 285-290 
    ISSN: 1432-1076
    Keywords: Thalassemia ; Hemosiderosis ; Desferrioxamine ; Liver storage iron ; Ferritin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Ten children with transfusion dependent anemias (thalassemia, sideroblastic anemia, congenital pure red cell aplasia) received either intravenous desferrioxamine (DF) in increasing doses up to 450 mg/kg at the time of transfusion or daily subcutaneous DF up to 110 mg/kg on an outpatient basis. No patient on intravenous DF reached a negative iron balance. All children with a subcutaneous DF dose of more than 60 mg/kg obtained a negative iron balance with a net iron excretion (transfusion iron already substracted) between 206 to 810 mg (mean 496 mg) monthly. The effectiveness of regular subcutaneous DF on liver storage iron could be confirmed in 4 patients by liver biopsy, showing a decrease between 40–60% iron after 12–14 months of chelation therapy. So far the daily iron excretion has remained constant with a given dose of DF over a period up to 15 months. Even if poor compliance in some patients is taken into account, it is possible with this method of treatment to prevent further accumulation of iron in chronically transfused children.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 140 (1983), S. 221-230 
    ISSN: 1432-1076
    Keywords: Familial hemophagocytic lymphohistiocytosis ; Farquhar's disease ; Histiocytosis ; Retichlosis ; Immune deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Familial hemophagocytic lymphohistiocytosis (FHL) is probably a genetically transmitted disease affecting infants and very young children. Cardinal symptoms are fever, hepatosplenomegaly, and pancytopenia. Frequently meningeal involvement is seen, manifested by neurologic symptoms and a lymphohistocytic pleocytosis with increased protein levels in the cerebrospinal fluid. Characteristic laboratory findings in FHL are hypertriglyceridemia and hypofibrinogenemia, which are reversible with treatment. The disease has been rapidly fatal in most patients, but recently longterm remissions have been achieved with cytotoxic agents. Pathohistologic examination shows a widespread infiltrate of lymphocytes and mature macrophages with prominent hemophagocytosis affecting especially liver, spleen, lymph nodes and the central nervous system. Atrophy of the lymphatic tissue is a common finding. From the histologic picture FHL has to be grouped among the histiocytoses of reactive origin since the cells involved show no signs of malignancy. The etiology and pathogenesis of FHL are not known at present. Immunologic studies present evidence for a disturbed function of T lymphocytes, but a secondary immune defect seems to be more likely than primary immune deficiency. Among the broad clinical spectrum of histiocytic disorders especially histiocytic reactions due to infection, histiocytosis X and malignant histiocytosis have to be considered in the differential diagnosis of FHL.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Annals of hematology 37 (1978), S. 89-94 
    ISSN: 1432-0584
    Keywords: Akute lymphoblastische Leukämie ; Prognostische Faktoren bei ALL ; Messung der Zellgröße ; Acute lymphoblastic leukemia ; Prognostic factors in ALL ; Cell size measurement
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The significance of cell size as a prognostic indicator in acute lymphoblastic leukemia (ALL) is controversial. Accuracy in measurement of cell size can be improved by determination of cell areas instead of single cell diameters. In the present study cell areas of 200 cells were determined in pretreatment bone marrows of 35 children with ALL. For better comparison with other studies which had used cell diameters only, the measured area was expressed as circle area from which the circle diameter was calculated. Cells with a diameter of 〉 12μm were defined as macrolymphoblasts (MLB). Several clinical characteristics considered to be risk factors in ALL were ascertained for each patient. The duration of first complete remission was used to assess the prognostic significance of cell size and of number of risk factors. In contrast to previous reports patients with more than 25% MLB had longer remissions. However, nearly all patients of this group had no or one risk factor only. When patients with more than one risk factor were excluded from statistical analysis, the group with more than 25 % MLB had no longer a better prognosis compared to the group with 25% MLB or less. Thus, in this study the percentage of MLB was not an independent prognostic indicator for risk of relapse in ALL.
    Notes: Zusammenfassung Die Bedeutung der Zellgröße als prognostischer Faktor bei Patienten mit akuter lymphoblastischer Leukämie (ALL) ist umstritten. Die Messung der Zellfläche anstelle des Zelldurchmessers ergibt genauere Werte. In der vorliegenden Studie wurde bei 35 Kindern mit ALL die Zellfläche von 200 Zellen in den Knochenmarksausstrichen vor Therapiebeginn bestimmt. Aus Vergleichsgründen mit anderen Arbeiten, die lediglich Zelldurchmesser benützt hatten, wurde die gemessene Fläche einer Kreisfläche gleichgesetzt und der Kreisdurchmesser bestimmt. Zellen mit einem Durchmesser von 〉 12μm wurden als Makrolymphoblasten (MLB) bezeichnet. Für jeden Patienten wurden die klinischen Merkmale, die als Risikofaktoren bei der ALL gelten, ermittelt. Die Dauer der ersten Remission diente als Maßstab für die Prognose. Im Gegensatz zu vorhergehenden Berichten hatten Patienten mit mehr als 25% MLB eine bessere Prognose, was Frührezidive anbelangte. Fast alle Patienten dieser Gruppe hatten jedoch keinen oder nur einen Risikofaktor. Wenn bei der statistischen Analyse nur Patienten mit keinem oder einem Risikofaktor berücksichtigt wurden, zeigte die Gruppe mit mehr als 25% MLB keine bessere Prognose mehr gegenüber der Gruppe mit 25 % MLB und weniger. In der vorliegenden Studie war der Anteil der Makrolymphoblasten kein unabhängiger prognostischer Parameter für das Rezidivrisiko bei ALL.
    Type of Medium: Electronic Resource
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