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  • 1
    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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  • 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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  • 3
    Electronic Resource
    Electronic Resource
    Schwerer Arzneimittel-Ikterus durch das Tuberculostaticum Isonicotinsäurehydrazid (INH) bei einem Kleinkind mit Phenylketonurie (1973)
    Springer
    European journal of pediatrics 115 (1973), S. 49-58 
    Details
    ISSN: 1432-1076
    Keywords: Phenylketonuria ; Seizures ; Isoniazid ; Jaundice ; Electron microscopy ; Degeneration of organelles in liver cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei einem jordanischen Kleinkind, das wegen einer Phenylketonurie mit cerebralen Krampfanfällen seit 1 Jahr mit Primidon und Ospolot behandelt und seit 7 Monaten mit einer phenylalaninarmen Diät unter Zusatz eines Aminosäurengemisches ernährt wurde, entwickelte sich nach 3 monatiger INH-Medikation ein hepatocellulärer Arzneimittelikterus. Dieser erwies sich klinisch und histologisch als reine Form einer toxischen Hepatose ohne entzündliche Symptome. Die elektronemmikroskopische Untersuchung von Leberbiopsiematerial 2 Wochen nach dem Höhepunkt des Ikterus (28 mg% Gesamtbilirubin im Serum) erlaubte den Nachweis eines intracellulären Transportdefektes des bereits konjugierten Bilirubins in den Leberepithelien. Das Pigment wurde analog dem Dubin-Johnson-Syndrom in peribiliären Lysosomen gespeichert. Weitere für die Pathogenese aufschlußreiche Befunde wurden an den Organellen der Hepatocyten erhoben: Hydropische Schwellung des hypertrophierten glatten endoplasmatischen Reticulums bei gleichzeitiger Reduktion des rauhen endoplasmatischen Reticulums, Glykogenschwund und Degeneration der Mitochondrien. Bei der Kontrollbiopsie nach 1/2 Jahr waren alle Befunde bis auf die lysosomale Speicherung normalisiert.
    Notes: Abstract After being treated with isonicotinic acid hydrazine (INH) for 3 months a 3-year-old Jordanian boy with phenylketonuria developed severe hepatocellular jaundice. In addition to INH the patient had received Primidone and Ospolot® for 1 year and a PKU diet for 7 months without any side effects. The liver biopsy 2 weeks after the peak of jaundice revealed no signs of hepatitis on light microscopy. An electron microscope investigation showed a defect in the intracellular transport system of bilirubin; bile pigment was stored in peribiliar lysosomes in the same way as in the Dubin-Johnson Syndrome. The smooth endoplasmatic reticulum was increased and vacuolized, whereas rough endoplasmatic reticulum, mitochondria, and glycogen were reduced. In a control biopsy nearly 6 months after the jaundice the morphologic alterations of the liver had normalized with the exception of the lysosomal changes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00438990
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  • 4
    Electronic Resource
    Electronic Resource
    Glycogenosis type Ib. Further evidence for a membrane disease (1981)
    Springer
    European journal of pediatrics 135 (1981), S. 325-325 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442112
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  • 5
    Electronic Resource
    Electronic Resource
    The effect of sward height on the liveweight gain of farmed yearling red deer (Cervus elaphus) stags (1995)
    Oxford, UK : Blackwell Publishing Ltd
    Grass and forage science 50 (1995), S. 0 
    Details
    ISSN: 1365-2494
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Agriculture, Forestry, Horticulture, Fishery, Domestic Science, Nutrition
    Notes: The effect of sward height on the liveweight gain of yearling farmed red deer stags grazing permanent upland pastures in the UK in two successive years is described. The swards received nitrogen fertilizer at a rate of 218 kg ha−1. Red deer stag calves, which had been weaned in September and housed over winter on a hay and concentrate diet to gain on average 57 g d−1 in year 1 and 75 gd−1 in year 2, grazed at one of fourmean sward heights of 4, 6, 8 and 10cm from the beginning of June until September. Liveweight gains were recorded over a period of 15 weeks. All animals were slaughtered and carcass weights were recorded after the end of the grazing period. Liveweight gains and final live weights and carcass weights were significantly (P〈0·05) lower on the 4-cm sward than on the other swards, but there were no differences between the performance of animals on the 6-, 8- and 10-cm swards.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2494.1995.tb02334.x
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  • 6
    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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  • 7
    Electronic Resource
    Electronic Resource
    Früherkennung des Morbus Wilson (1999)
    Springer
    Monatsschrift Kinderheilkunde 147 (1999), S. 465-468 
    Details
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Morbus Wilson ; Sonographie ; Früherkennung ; Fettleber ; Key words Morbus Wilson ; Sonography ; Fatty liver ; Early diagnosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The following three case studies will prove, that the sonographic diagnosis of a fatty liver is often related to a metabolic disease, beginning at the age of 4 to 5 years in conjunction with the Morbus Wilson. Discussion: Due to te widly spread use of sonography and the meticulous search for morphological changes it becames possible to examine at an early presymptomatic stage the Wilson disease. This and a reliable enforcement of therapy might help to prevent further damage and side effects.
    Notes: Zusammenfassung Anhand von 3 Kasuistiken wird gezeigt, daß die sonongraphische Diagnose einer Fettleber im Kindesalter meist mit einer Stoffwechselerkrankung, ab dem 4. bis 5. Lebensjahr bevorzugt mit einem Morbus Wilson verbunden ist. Diskussion: Aufgrund der weiten Verbreitung der Sonographie ermöglicht die konsequente Abklärung dieser Veränderung neue Perspektiven für eine Frühdiagnose des Morbus Wilson im präsymptomatischen Stadium, dessen symptomatische Phase durch eine konsequente, lebenslange Therapie vermieden werden kann.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001120050446
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  • 8
    Electronic Resource
    Electronic Resource
    Familienuntersuchungen über die Vererbung der Eigenschaft Gma (1962)
    Springer
    International journal of legal medicine 52 (1962), S. 438-442 
    Details
    ISSN: 1437-1596
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine , Law
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00580180
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