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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 102 (1968), S. 62-70 
    ISSN: 1432-1076
    Keywords: Isocitratdehydrogenase ; Enzymuntersuchungen
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei 312 erkrankten Kindern im Alter bis zu 12 Jahren wurde die Aktivität der Isocitratdehydrogenase (ICDH) im Serum ermittelt, um deren mögliche diagnostische Bedeutung für die Pädiatrie festzustellen. Dem Krankengut wurde ein Normalkollektiv von 176 gesunden Kindern gegenübergestellt. Die Normalwerte der ICDH-Aktivität im Serum bei Neugeborenen liegen zwischen 1,88 und 8,76 mU mit einem Mittelwert von 4,8±2,2 mU; bei Kindern bis zu 12 Jahren zwischen 0 und 5,13 mU mit einem Mittelwert von 2,21±0,91 mU. In 80% der Fälle von akuter Virushepatitis zeigte die Serum-ICDH Aktivitätserhöhungen bis zu 59,6 mU/ml, wobei das Ausmaß der Erhöhungen der ICDH bei akuter Virushepatitis keinen sicheren Schluß auf Schwere und Prognose der Erkrankung zuläßt. Der diagnostische Wert der ICDH bei Erkrankungen im Kindesalter liegt vor allem in den akuten Krankheitsverläufen (akute Virusmyokarditis oder Virushepatitis). Im Liquor cerebrospinalis gesunder Kinder war keine Aktivität nachweisbar, im pathologischen Liquor fanden sich unterschiedliche Erhöhungen, die keinen differentialdiagnostischen Rückschluß erlaubten.
    Notes: Summary Serum isocitric dehydrogenase (S-ICD) was determined in 176 healthy children and 312 children with various diseases. In healthy children, the normal value lies at 2.2 mU with a standard deviation of ±0,9 mU. The average value is depending on the age of the patients. In 80% of all cases of acute virus hepatitis the S-ICD showed an increase in activity, but no strict correlation between the increase in activity and the prognosis of the individual case could be found in children suffering from mucoviscidosis, myatonia dystrophica, and progressiv muscular dystrophy. An activity of the S-ICD in the liquor cerebrospinalis is only measureable in cases of meningitis, where an increase can be demonstrated. The diagnostic value of the determination of the S-ICD in pediatrics is limited to the acute stadium of diseases, where high activity can be found.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 102 (1968), S. 230-237 
    ISSN: 1432-1076
    Keywords: Isocitratdehydrogenase ; Erythrocytenenzyme bei Kindern
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei gesunden und kranken Kindern wurden intraerythrocytäre Enzyme unter besonderer Berücksichtigung der ICDH und G-6-PDH gemessen. Die Normalwerte für die ICDH liegen zwischen 14,2 und 42,1 mE/109 Erythrocyten (Höchstwert!), die der G-6-PDH zwischen 240 und 388,3 mE/109 Erythrocyten. Bis auf die Blutgruppenunverträglichkeit konnte bei keiner von uns erfaßten Erkrankung eine signifikante Änderung der ICDH oder G-6-PDH festgestellt werden. Auf das unterschiedliche Verhalten der Enzyme in Neugeborenenerythrocyten und in jungen Erythrocyten Erwachsener wurde nochmals hingewiesen, ebenso auf eine Korrelation zwischen G-6-PDH und ICDH, die statistisch verifiziert wurde.
    Notes: Summary ICD and glucose-6-phosphate dehydrogenase (G-6-PDH) were determined in healthy children as well as in children with various diseases. In healthy children the normal value for the ICD lies between 14.2 and 42.1 mE/109 erythrocytes. For the G-6-PDH the normal values are between 130.1 and 388.3 mE/109 erythrocytes with an average of 240.8 mE/109 erythrocytes. The normal values are significantly wide spread, they are also depending on the age of the examined children. In our cases no significant decreases in the intraerythrocytic activity of the two enzymes could be stated in children with various diseases. Various enzymatic results show that the erythrocytes of newborns and young children are more different from those of adults than previously assumed.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Bare lymphocyte syndrome ; HLA expression ; Severe combined immunodeficiency syndrome ; Neutrophil dysfunction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 4-year-old girl presented with recurrent infections. Immunoglobulin deficiency (serum and secretory IgA, serum IgG3) neutropenia and neutrophil dysfunction (defective spontaneous migration and chemotaxis) were found. T-lymphocyte counts were normal and they responded to phytohaemagglutinin but were not stimulated by Concanavalin A, pokeweed mitogen and microbial antigens in vitro. Delayed cutaneous hypersensitivity testing to purified protein derivative and candidin was negative. Despite bacille Calmette-Guérm vaccination and candidiasis, near normal β-2-microglobulin and human leucocyte antigen (HLA) class I concentrations were detected on mononuclear cells and phytohaemagglutinin-induced lymphoblasts. HLA class II antigens (HLA-DP, −DQ, −DR) were not expressed. These observations indicated a bare lymphocyte syndrome (BLS) type II. This is the first time neutrophil dysfunction has been noted in association with BLS.
    Type of Medium: Electronic Resource
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