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  • 1
    Electronic Resource
    Electronic Resource
    Esthesioneuroblastoma: Ultrastructural, immunohistological and biochemical investigation of one case (1984)
    Springer
    European archives of oto-rhino-laryngology and head & neck 239 (1984), S. 133-144 
    Details
    ISSN: 1434-4726
    Keywords: Esthesioneuroblastoma ; Intermediate filaments ; Secretory granules ; Ultrastructure ; Immunohistology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of esthesioneuroblastoma, the pathological diagnosis of which almost always causes great difficulties, was investigated ultrastructurally, biochemically, and immunohistologically, using antibodies against the five known types of intermediate filaments [keratin, vimentin, desmin, glial fibrillary acidic protein (GFAP) and neurofilaments]. The tumour cells did not react with antibodies against any of the five intermediate filament proteins. Ultrastructural investigations showed dense cored secretory granules in the cytoplasm and cell processes. Thus, immunohistology offers by “exclusion” a differential diagnosis to avoid often misdiagnosed tumours (undifferentiated carcinomas, embryonal rhabdomyosarcomas, and malignant lymphomas), since carcinomas react with antikeratin, embryonal rhabdomyosarcomas with antibodies to desmin and malignant lymphomas show immunofluorescence with antibodies to vimentin. The biological behaviour (age distribution, tendency to metastasize), the normal values of biochemical parameters, homovanillic acid and vanilmandelic acid (HVA, VMA), and the absence of neurofilaments distinguish this type of tumour from the peripheral sympathetic neuroblastoma.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00463554
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Effect of age on homovanillic and 4-hydroxy-3-methoxymandelic acid levels in plasma (1986)
    Springer
    European journal of pediatrics 145 (1986), S. 555-557 
    Details
    ISSN: 1432-1076
    Keywords: Homovanillic acid ; 4-hydroxy-3-methoxymandelic acid ; Catecholamine acidic metabolites ; Plasma ; Mass fragmentography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The plasma levels of homovanillic and 4-hydroxy-3-methoxymandelic acids have been determined in 524 normal subjects ranging from birth to 49 years of age using a mass fragmentographic method. The mean concentration of homovanillic acid in plasma declines exponentially from the age of 1 day (x=2342.0 nmol/l, 426.6 ng/ml) to adulthood (x=60 nmol/l, 10.9 ng/ml). 4-Hydroxy-3-methoxymandelic acid behaves in a similar although not so extreme manner (392 nmol/l, 77.6 ng/ml–50 nmol/l, 10 ng/ml).
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02429064
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Neonatal pyruvate dehydrogenase deficiency with lipoate responsive lactic acidaemia and hyperammonaemia (1989)
    Springer
    European journal of pediatrics 148 (1989), S. 543-547 
    Details
    ISSN: 1432-1076
    Keywords: Pyruvate dehydrogenase deficiency ; Hyperammonaemia ; Peritoneal dialysis ; Lipoic acid ; Plasma amino acids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 2-day-old girl developed a severe lactic acidosis with a normal lactate/pyruvate ratio and hyperammonaemia. Plasma arginine and citrulline levels were below the limit of detection. In muscle total pyruvate dehydrogenase complex (PDHC) and pyruvate decarboxylase (E1) activities were reduced to a fraction of lower control values. The acute neonatal period was bridged with peritoneal dialysis, dichloroacetate therapy, supplements of arginine and branched chain amino acids, a complete vitamin B complex and lipoic acid. Lactate homeostasis responded to pharmacological supplements of lipoic acid. At age 1 year the child was hypotonic, showed severe developmental retardation, optic atrophy and cranial dysmorphism. She died aged 1 year 8 months with signs of respiratory paralysis but with normal lactate levels under assisted breathing. Pathological findings at autopsy were suggestive of Leigh syndrome, interstitial pneumonia and extensive fatty infiltration of hepatocytes. Regression analysis of data from 187 plasma amino acid determinations from the patient over a period of 1 year 8 months revealed a persistent imbalance involving alanine, glutamic acid, glutamine, proline, citrulline and branched chain amino acids. Aspects of acute and long-term therapy in this patient and some implications of the imbalances in plasma amino acids are discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441554
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    Paper (German National Licenses)
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