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  • Acute hepatitis  (1)
  • Autoimmune haemolytic anaemia  (1)
  • Dystrophie  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Alteration of the lung parenchyma associated with autoimmune hepatitis (1991)
    Springer
    Virchows Archiv 419 (1991), S. 153-157 
    Details
    ISSN: 1432-2307
    Keywords: Autoimmune hepatitis ; Chronic interstitial pneumonitis ; Autoimmune haemolytic anaemia ; Endogenous lectins
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinical history, radiological and histomorphological alterations of the lung parenchyma associated with chronic active autoimmune hepatitis are described. A 6-month-old female infant developed chronic active autoimmune hepatitis associated with autoimmune haemolytic anaemia. She was treated with immunosuppressive drugs, including steroids, for more than 6 years and developed symptoms and radiological signs of interstitial pneumonitis 4 years after onset of the autoimmune hepatitis. Associated bronchiectasis was detected 1 year later. No abnormalities of lung defence mechanisms could be demonstrated. Resection of the sixth left segment and of the basal parts of the left lower lobe revealed honeycombing with changes in the lung parenchyma which included chronic interstitial pneumonitis with multinucleate giant cells, seen predominantly in the distal airways, marked diffuse interstitial mononuclear infiltrates and mild diffuse interstitial fibrosis as well as bronchiectasis and organizing pneumonia. Granulomatous lesions, angiitis and necrotic areas were absent. Immunohistochemistry for immunoglobulins was negative for IgA, IgG and IgM and positive for IgD in the multinucleate giant cells. A strong positive reaction to HLA-DR-specific monoclonal antibody was noted, whereas no specific sugar receptors (endogenous lectins) could be detected by use of biotinylated glyconeoproteins.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01600230
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Quantitative determination of single serum proteins during acute hepatitis in childhood (1978)
    Springer
    European journal of pediatrics 128 (1978), S. 123-128 
    Details
    ISSN: 1432-1076
    Keywords: Serum proteins ; Acute hepatitis ; Childhood
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fifteen serum proteins were estimated by linear immunodiffusion in blood samples from children with acute hepatitis. Blood was drawn at the beginning of the disease and three weeks later. The results were compared with results obtained from a group of age-matched normal children. At the beginning of the disease prealbumin and beta-2-glycoprotein I were depressed, whereas alpha-1-acid-glycoprotein, alpha-1-antitrypsin, ceruloplasmin and alpha-2-HS-glycoprotein were found to be elevated. Alpha-2-macroglobulin, transferrin and beta-lipoprotein showed a significant elevation after three weeks. Beta-1-A/C, IgM and IgG remained elevated during the time of observation. Albumin, haptoglobin and IgA were similar in patients and controls and did not change during the period of observation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00496997
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    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    Erhöhte Phytansäurespiegel in Plasma und Leber bei einem Kleinkind mit unklarem Hirnschaden (1974)
    Springer
    Journal of molecular medicine 52 (1974), S. 651-653 
    Details
    ISSN: 1432-1440
    Keywords: Dystrophy ; cerebral damage ; alpha-oxydation ; phytanic acid ; Refsum's disease ; Dystrophie ; Hirnschaden ; alpha-Oxydation ; Phytansäure ; Refsum-Krankheit
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung In Serum und Leber eines Kleinkindes mit unklarem Hirnschaden konnten größere Mengen von 3,7,11,15-Tetramethylhexadecansäure (Phytansäure) nachgewiesen und mittels Gaschromatographie und Massenspektroskopie (GCMS) identifiziert werden. Unter den Fettsäuren der Serumtriglyceride betrug ihre Konzentration maximal 8,5%. Bisher wurde Phytansäure nur bei der Heredopathia atactica polyneuritiformis (Refsum-Krankheit) vermehrt gefunden, für die ihre Speicherung pathognomonisch ist. Unser Patient weist die charakteristischen Symptome der Refsum-Krankheit nicht auf. Es handelt sich damit um den ersten Fall einer Phytansäurespeicherung, ohne daß eine Refsum-Krankheit vorliegt.
    Notes: Summary Considerable amounts of 3,7,11,15-tetramethylhexadecanoic acid (phytanic acid) could be identified by gasliquid chromatography and mass spectrometry (GCMS) in serum and liver of an infant with cerebral damage of unknown etiology. The concentration of phytanic acid in serum triglyceride fatty acids was 8.5%. Up to now, increased levels of phytanic acid was found only in heredopathia atactica polyneuritiformis (Refsum's syndrome) for which it's occurrence was considered pathognomonic. Our patient does not exhibit the typical symptoms of Refsum's disease and represents the first case of phytanic acid storage without heredopathia atactica polyneuritiformis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01468803
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    Paper (German National Licenses)
    Fulltext
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