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  • 1
    Digitale Medien
    Digitale Medien
    Springer
    Journal of molecular medicine 37 (1959), S. 255-258 
    ISSN: 1432-1440
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    Digitale Medien
    Digitale Medien
    Springer
    Journal of molecular medicine 37 (1959), S. 237-240 
    ISSN: 1432-1440
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Zusammenfassung Von 5 Kindern mit einer sicheren Cöliakie konnten in 3 Fällen im Serum unter einer Glutenbelastung Peptide nachgewiesen werden, die bei stoffwechselgesunden Kindern und bei Cöliakiekranken unter glutenfreier Kost nicht nachweisbar waren. Es handelt sich in diesen 3 Fällen um chromatographisch schnellwandernde Peptide, die kein Prolin enthielten. Bei einem Kind mit einer fraglichen Cöliakie wurde ebenfalls ein derartiges Peptid beobachtet.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 3
    Digitale Medien
    Digitale Medien
    Springer
    Journal of molecular medicine 33 (1955), S. 35-39 
    ISSN: 1432-1440
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Zusammenfassung Die Bedeutung des Elektrolytund Wasserhaushaltes für die Anfallsperiodik von Epileptikern wird an Hand bekannter Befunde erläutert. Eigene Untersuchungen zeigten, daß durch enteralen Na-Entzug mit Hilfe von Kationenaustauschern eine Beeinflussung der Krampftätigkeit bei Epileptikern möglich ist. Für die Dauer der Behandlung konnte ein Sistieren der Anfälle und eine weitgehende Rückbildung der epileptischen EEG-Veränderungen erzielt werden.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 4
    Digitale Medien
    Digitale Medien
    Springer
    European journal of nutrition 16 (1977), S. 211-215 
    ISSN: 1436-6215
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Land- und Forstwirtschaft, Gartenbau, Fischereiwirtschaft, Hauswirtschaft , Medizin
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 5
    Digitale Medien
    Digitale Medien
    Springer
    Virchows Archiv 385 (1979), S. 109-116 
    ISSN: 1432-2307
    Schlagwort(e): Shwachman's syndrome ; Leukaemia ; Pancreatic atrophy
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary The clinical and morphological characteristics of Shwachman's syndrome (exocrine pancreatic insufficiency, pancytopenia, skeletal changes) were observed in a boy who, at the age of 8 years, developed a juvenile form of chronic myeloic leukemia which did not respond to cytostatic treatment. Autopsy revealed a striking lipomatous atrophy of the pancreas, defects in the ossification zones of the bones and marked dwarfism. In addition there was leukaemic infiltration of the pancreas, the spleen, the liver and the lymph nodes. The association of Shwachman's syndrome with leukaemia is a rare, but remarkable complication of this entity because of its relationship to the preceeding pancytopenia. Thorough follow-up of the haematological status of patients with Shwachman's syndrome is recommended.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 6
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 130 (1979), S. 155-164 
    ISSN: 1432-1076
    Schlagwort(e): Coeliac disease ; Diarrhoea, infantile ; Fluorescent antibody technique ; Gliadin ; Immunology ; Malabsorption syndromes
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract An immunofluorescent gliadin antibody assay is described using pyruvic aldehyde-stabilized human erythrocytes coated with gliadin. Fifty coeliac children all had high serum IgG-antigliadin titres during a normal diet or a challenge with gluten. On a gluten-free diet (30 children), titres were much lower. In patients followed-up for one year on a gluten-free diet, an initial rise in titres was followed by a slow decline. On challenge, IgG-antigliadin titres showed a slow rise or persistence at the same level in most patients. Fifty-two percent of control children with malabsorptive disorders, but without the typical “flat” mucosal lesion on jejunal biopsy, were shown to have positive titres in their sera, as were 6% of normal children and 4% of adult blood donors. The fluorescent antibody technique was compared with methods commonly used to detect wheat-protein antibodies, and was found to be superior to all of them. The immunofluorescent gliadin antibody assay appears to be useful in following-up children with coeliac disease, and in selecting patients for jejunal biopsy, although it does not replace biopsy.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 7
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 143 (1985), S. 203-208 
    ISSN: 1432-1076
    Schlagwort(e): Farber disease ; Lipogranulomatosis ; Acid ceramidase deficiency ; Arthropathy ; Hoarseness ; Ultrastructure
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract A 20-month-old girl showed typical clinical signs of Farber disease: hoarseness since birth, and periarticular subcutaneous painful nodules. Complete deficiency of acid ceramidase activity was found in cultured skin fibroblasts. An electron microscopic examination of a dermal nodule disclosed pathognomonic tubular inclusions in histiocytes. In epidermal cells zebra-body-like and needle-like lysosomal inclusions were found. Their ultrastructure is different from that of the intrahistiocytic lysosomal inclusions. Probably three clinical types of Farber disease may be distinguished according to the symptomatology and the course of the discase: a severe type, an intermediate type and a relatively mild type. The activity of acid ceramidase does not correlate with prognosis of the disease, while a correlation between first appearance of dermal nodules and clinical course appears likely.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 8
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 135 (1980), S. 31-36 
    ISSN: 1432-1076
    Schlagwort(e): Antigen handling ; Coeliac disease ; Family study ; Fluorescent antibody technique ; Gliadin antibodies ; Reticulin antibodies ; Monozygotic twins
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract The familial occurence of coeliac disease is well known. In every day practice, however, diagnosis of coeliac disease is not frequently established in the relatives of patients. As it did not seem practicable to biopsy all relatives, several tests were investigated in selecting individuals for intestinal biopsy in a family study. 55 index patients out of 54 families with biopsy-proven coeliac disease and 165 of their first grade relatives underwent the study. Immunofluorescent gliadin and reticulin antibodies were determined, and additionally laboratory tests were done. These included haemoglobin, serum iron, serum protein and albumin, serum immunoglobulins and blood xylose. The immunofluorescent gliadin antibody assay using red cells coated with gliadin proved to be superior to the other methods. False negatives came to 8.7%, and false positives 10.9%, in healthy relatives. Gliadin antibodies could be found five times more frequently in healthy relatives than in normal controls. This finding indicates a genetic predisposition to the formation of gliadin antibodies in coeliac families. Ninety-one percent of index coeliac children had IgG-antigliadin in their sera while on a normal diet. During gluten-free diet, and in adult patients, results were less convincing. All relatives with antigliadin titres greater than 8 have been biopsied, and all with titres above 64 were shown to have coeliac disease. The prevalence of coeliac disease found in this study was 5.5%. In the active state of coeliac disease in children, gliadin antibody determination thus is a valuable diagnostic tool but in selecting relatives for biopsy there are limitations to the wide application of the test. Although reticulin antibodies are more specific for coeliac disease than gliadin antibodies, determination of antireticulin proved to be much less sensitive.
    Materialart: Digitale Medien
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  • 9
    ISSN: 1432-1076
    Schlagwort(e): Acrodermatitis enteropathica ; Diarrhoea, protracted ; Monosaccharide malabsorption ; Total parenteral nutrition ; Zinc deficiency
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Protracted diarrhoea in early infancy gives rise to many diagnostic and therapeutic problems. Jejunal biopsy often reveals villous atrophy of varying degrees. Severe reduction of small intestinal absorptive area causes secondary monosaccharide malabsorption, as well as secondary disaccharide deficiency, consequences which are relevant in any attempts at oral feeding. Morphologic, metabolic, endocrinological and microbiological studies have to be undertaken in order to establish a definitive diagnosis in protracted diarrhoea, but these studies often fail to reveal the aetiology of the disease. Immunologic abnormalities like phagocyte dysfunction, thymic atrophy and hypoplasia of B-cell regions in lymph nodes might be secondary events, but some types of immunodeficiency are of primary importance in the development of protracted diarrhoea. Total parenteral nutrition in many cases has to be instituted, with all its implications and hazards: septicaemia is the most dangerous of these. Zinc deficiency and acrodermatitis enteropathica may occur during total parenteral nutrition, and zinc deficiency secondarily contributes to the symptoms of diarrhoea. Parenteral administration of zinc is able to overcome these effects.
    Materialart: Digitale Medien
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  • 10
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 82 (1959), S. 548-559 
    ISSN: 1432-1076
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Zusammenfassung Die Normalwerte der MDH liegen auch im Kindesalter zwischen 1–7 E, die der SGOT und der SGPT zwischen 10 und 40 E. Findet man Fermentaktivitäten zwischen 40 und 50 E, so handelt es sich um Grenzwerte, die einer Wiederholung bedürfen. Für die Neugeborenen- und Säuglingsperiode gelten im Prinzip die gleichen Normalwerte. Die MDH und die SGOT liegen allerdings an der oberen Grenze des Normbereiches. Die SGOT sollte jedoch auch bei diesen Kindern 50 E nicht überschreiten. Auch für das Kindesalter hat sich die Bestimmung der Transaminasen zur Verlaufsbeurteilung der Hepatitis als sehr empfindlicher Test erwiesen, der alle Schwankungen im Krankheitsverlauf genau anzeigt. Zur Abgrenzung verschiedener Ikterusformen im Neugeborenen- und Säuglingsalter liegen bisher noch keine ausreichenden Erfahrungen vor. Doch sprechen einige Befunde dafür, daß auch hier die Bestimmung der Fermentspiegel in differentialdiagnostischer Hinsicht eine Bedeutung erlangen wird.
    Materialart: Digitale Medien
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