title: [An alpha 1-antitrypsin aerosol in the treatment of cystic fibrosis].
abstract: Alpha-1-antitrypsin (a-1AT) is a natural inhibitor of the elastase that is released physiologically by neutrophils in the lung. As a result of the increased neutrophil degranulation secondary to chronic epithelial inflammation in cystic fibrosis patients with chronic infections by Pseudomonas aeruginosa, there are larger amounts of elastase in airway secretions. This results in the a-1AT concentration being insufficient to inhibit the destructive proteolytic degradation, culminating in a chronic epithelial burden and a worsening of the cystic fibrosis pulmonary disease. In this preliminary study, we have evaluated the results obtained from the sputum of 4 cystic fibrosis patients treated with a-1AT (Prolastina, Bayer) in aerosol. The levels of a-1AT, neutrophil elastase, antineutrophil elastase activity, IgG, albumin and clinical parameters were measured. The concentration of sputum a-1AT was increased when compared to the same patient after 8 days with treatment (we compared means with Student's t-test and p < 0.05 was considered significant). We did the same with the impairment of neutrophil elastase, although we found no significant results. Nevertheless, antineutrophil elastase activity increased (p < 0.05). These results encourage us to continue the same treatment for a longer period of time to prevent pulmonary disease in CF subjects.
authors.affiliation: Laboratorio de Bioquímica, U. de proteínas, Hospital de Rehabilitación La Fe, Valencia.
authors.forename: J
authors.initials: J
authors.lastname: Ferrer Calvete
authors.forename: J
authors.initials: J
authors.lastname: Aznar Lucea
authors.forename: P
authors.initials: P
authors.lastname: Morales Marín
authors.forename: A
authors.initials: A
authors.lastname: Pereda Pérez
authors.forename: R
authors.initials: R
authors.lastname: Llopis Carles
authors.forename: C
authors.initials: C
authors.lastname: Ribes Koninckx
authors.forename: P
authors.initials: P
authors.lastname: Ferrer González
authors.forename: V
authors.initials: V
authors.lastname: Marco Martínez
mesh_terms: D000336:Aerosols; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism; D004341:Drug Evaluation; D006801:Humans; D011552:Pseudomonas Infections / Q000188:drug therapy / Q000209:etiology / Q000378:metabolism; D015842:Serine Proteinase Inhibitors / Q000008:administration & dosage*; D013183:Sputum / Q000737:chemistry / Q000187:drug effects; D013997:Time Factors; D000515:alpha 1-Antitrypsin / Q000008:administration & dosage*
chemical_list: D000336:Aerosols; D015842:Serine Proteinase Inhibitors; D000515:alpha 1-Antitrypsin