title: Granulocyte elastase-alpha 1-antiproteinase complex in cystic fibrosis: sensitive plasma assay for monitoring pulmonary infections.
abstract: Elastase in plasma was determined as a complex of granulocyte elastase and alpha 1-antiproteinase (GEC) by an enzyme-linked immunosorbent assay in 67 patients with cystic fibrosis. The patients were observed for 1 years, when clinically infected and noninfected. Although noninfected patients had GEC levels within the normal range, the mean value was significantly higher than the mean values in patients in an optimal noninfectious state (P less than 0.01) and in healthy controls (P less than 0.001). Clinical signs of (in most cases) low-grade infection were paralleled by significant increases in GEC levels (P less than 0.001). There was no correlation with different types of bacteria or age of patients. No cross reaction to Pseudomonas elastase could be found in vitro or in vivo. Significant decreases of GEC values were seen after intravenously administered antimicrobial therapy (P less than 0.001), and although the white blood cell count and erythrocyte sedimentation rate were rarely increased above the normal range, they decreased congruently (P less than 0.001), and lung function improved (P less than 0.001). The sensitivity of GEC was significantly higher than that for erythrocyte sedimentation rate (P less than 0.01) and white blood cell count (P less than 0.05). Determination of GEC values seems to be a simple and sensitive measurement for monitoring treatment in cystic fibrosis, especially of low-grade infections in patients with normal standard blood tests.
mesh_terms: D000293:Adolescent; D000328:Adult; D001798:Blood Proteins; D002648:Child; D002675:Child, Preschool; D004796:Clinical Enzyme Tests / Q000379:methods*; D003550:Cystic Fibrosis / Q000150:complications / Q000175:diagnosis*; D004797:Enzyme-Linked Immunosorbent Assay; D005069:Evaluation Studies as Topic; D005260:Female; D006098:Granulocytes / Q000201:enzymology*; D006801:Humans; D007223:Infant; D008297:Male; D008991:Monitoring, Physiologic / Q000379:methods*; D011446:Prospective Studies; D011480:Protease Inhibitors / Q000097:blood*; D011552:Pseudomonas Infections / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D013203:Staphylococcal Infections / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D013997:Time Factors; D000515:alpha 1-Antitrypsin
chemical_list: D001798:Blood Proteins; D011480:Protease Inhibitors; D000515:alpha 1-Antitrypsin