title: [The protease inhibitor system in children (in diseases of the lung, liver and hereditary alpha 1-antitrypsin deficiency)].
abstract: The balance of the protease inhibitor system is decisive for the pulmonary conditions of children. Our investigations showed that an imbalance of this system can be interpreted as manifestation of a severe pulmonary inflammation (e.g. deficit of inter-alpha-antitrypsin inhibitor in case of dyspnoe) or it can be regarded itself as a severe disease (alpha 1 antitrypsin deficit). A largely normal protease inhibitor system in children with pulmonary diseases is a point in favour of a clinically bland progression (for instance no protease inhibitor depression in group II = lung diseases). The efforts to compensate for existing inhibitor deficits are distinct (e.g. by the compensatory mechanism of alpha 2 macroglobulin). How and to which extent hepatopathies or the still immature liver in early childhood, for instance by synthesis disturbances, have an effect on the protease inhibitor system cannot be regarded as clarified. For characterization pulmonary inflammatory diseases and alpha 1 antitrypsin deficiency analysis of inhibitors can be efficient.
authors: Medizinische Universitätsklinik und Poliklinik der Berufsgenossenschaftlichen Krankenanstalten, Bergmannsheil Bochum.
authors: A
authors: A
authors: Gillissen
authors: B
authors: B
authors: Rasche
authors: W T
authors: WT
authors: Ulmer
mesh_terms: D000293:Adolescent; D000510:Alpha-Globulins / Q000172:deficiency; D001991:Bronchitis / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008107:Liver Diseases / Q000235:genetics*; D008171:Lung Diseases / Q000235:genetics*; D008297:Male; D010641:Phenotype; D011014:Pneumonia / Q000235:genetics; D011480:Protease Inhibitors / Q000172:deficiency*; D012127:Respiratory Distress Syndrome, Newborn / Q000235:genetics; D012307:Risk Factors; D019896:alpha 1-Antitrypsin Deficiency; D000511:alpha-Macroglobulins / Q000172:deficiency
chemical_list: D000510:Alpha-Globulins; D011480:Protease Inhibitors; D000511:alpha-Macroglobulins; C011877:inter-alpha-inhibitor
vernacular_title: Das Proteasen-Inhibitorsystem bei Kindern (bei Erkrankungen der Lunge, der Leber und beim hereditären alpha 1-Antitrypsinmangel).