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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 70 (1992), S. 637-657 
    ISSN: 1432-1440
    Keywords: Pulmonary surfactant ; Phospholipids ; Surfactant proteins ; Alveolar stability ; Air pollution ; Pulmonary defense ; Adult respiratory distress syndrome ; Surfactant therapy ; Bronchoalveolar lavage
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Pulmonary surfactant is synthesized and secreted by alveolar type II cells and constitutes an important component of the alveolar lining fluid. It comprises a unique mixture of phospholipids and surfactant-specific proteins. More than 30 years after its first biochemical characterization, knowledge of the composition and functions of the surfactant complex has grown considerably. Its classically known role is to decrease surface tension in alveolar air spaces to a degree that facilitates adequate ventilation of the peripheral lung. More recently, other important surfactant functions have come into view. Probably most notable among these, surfactant has been demonstrated to enhance local pulmonary defense mechanisms and to modulate immune responses in the alveolar milieu. These findings have prompted interest in the role and the possible alterations of the surfactant system in a variety of lung diseases and in environmental impacts on the lung. However, only a limited number of studies investigating surfactant changes in human lung disease have hitherto been published. Preliminary results suggest that surfactant analyses, e.g., from bronchoalveolar lavage fluids, may reveal quantitative and qualitative abnormalities of the surfactant system in human lung disorders. It is hypothesized that in the future, surfactant studies may become one of our clinical tools to evaluate the activity and severity of peripheral lung diseases. In certain disorders they may also gain diagnostic significance. Further clinical studies will be necessary to investigate the potential therapeutic benefits of surfactant substitution and the usefulness of pharmacologic manipulation of the secretory activity of alveolar type II cells in pulmonary medicine.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Lineare IgA-Dermatose ; Bullöses Pemphigoid ; Basalmembran ; Bullöse Autoimmundermatosen ; Autoantikörper ; Keywords Linear IgA disease ; Bullous pemphigoid ; Basement membrane ; Autoimmune blistering diseases ; Autoantibodies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract A 29-year-old female patient with an autoimmune subepidermal blistering disease had linear deposits of both IgA and IgG at the basement membrane zone. Clinically, the patient presented with tense blisters on the face, trunk, extremities and oral mucosa. Histologically, we found a subepidermal blister formation and a predominantly neutrophilic infiltrate. Direct immunofluorescence showed linear deposits of IgA along the basement membrane zone, as well as linear deposits of IgG and C3 as typically found in bullous pemphigoid. Indirect immunofluorescence demonstrated circulating IgA and IgG autoantibodies. This case extends previous reports on a subgroup of patients with subepidermal blistering diseases characterized by the presence of both IgA and IgG anti-basement membrane antibodies. These patients reveal clinical, histological and immunopathological features of linear IgA disease and bullous pemphigoid.
    Notes: Zusammenfassung Wir berichten über eine 29jährige Patientin mit einer subepidermal blasenbildenden Autoimmundermatose, die durch lineare Ablagerungen von IgA- und IgG-Antikörpern an der dermoepidermalen Junktionszone gekennzeichnet ist. Klinisch fanden sich pralle Bläschen im Gesicht, am Stamm, an den Extremitäten und an der Mundschleimhaut. Histologisch sahen wir eine subepidermale Blasenbildung und ein neutrophilenreiches Entzündungsinfiltrat. Mittels direkter Immunfluoreszenz fanden sich in der Haut der Patientin lineare IgA-Ablagerungen an der Basalmembranzone. Ungewöhnlich war jedoch der gleichzeitige Nachweis von linearen IgG- und C3-Ablagerungen, die typischerweise beim bullösen Pemphigoid vorkommen. Auch im Serum der Patientin fanden sich Autoantikörper sowohl der IgA- als auch der IgG-Klasse. Der Fall dieser Patientin bestätigt frühere Berichte, daß es bei den subepidermal blasenbildenden Autoimmundermatosen eine Untergruppe von Patienten mit gleichzeitigem Nachweis von IgA- und IgG-Antikörpern gibt, bei denen sich klinische, histologische und immunpathologische Merkmale von linearer IgA-Dermatose und bullösem Pemphigoid überlappen.
    Type of Medium: Electronic Resource
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