title: [Pilot study of amiloride inhalation in children with cystic fibrosis].
abstract: Bronchial clearance is impaired in cystic fibrosis (CF). Respiratory secretions contain less sodium and chlorid, resulting in reduced airway water content. Aerosolized amiloride, a sodium transport blocker, was shown to normalize the amount of sodium in bronchial mucus, leading to an improvement in mucociliary clearance. In a controlled, double blind crossover study on nine CF children we tried to assess the efficacy of twice daily inhaled amiloride (10(-3)M) on sputum weight, consistency of sputum and lung function (FEF1, FVC, FEF50, FEF25, PEF). Each treatment period (amiloride versus 0.9% saline) lasted for two months. We could show that inhaled amiloride was able to increase mean sputum weight per day from 11.75 g (+/- 5.96) up to 18.5 g (+/- 10.34). This was equal to an increase of 57%. Some children felt that sputum expectoration lasted longer while using amiloride and that even for some hours after inhalation they expectorated a sputum-like fluid. We were able to detect, at least in some patients, that their sputum consisted of two parts, one showing more solid contents, the other more fluid-like contents. This was, however, not a consistent feature. No significant or clinical important differences were found for pulmonary function test data. There were no pulmonary or extra pulmonary side effects from treatment with amiloride. Further studies should be undertaken to assess the efficacy of longer lasting amiloride inhalation on the course of the disease in CF patients.
authors.affiliation: Kinderspital Salzburg.
authors.forename: J
authors.initials: J
authors.lastname: Riedler
authors.forename: I
authors.initials: I
authors.lastname: Huttegger
mesh_terms: D000280:Administration, Inhalation; D000293:Adolescent; D000584:Amiloride / Q000008:administration & dosage*; D002648:Child; D003550:Cystic Fibrosis / Q000188:drug therapy*; D004311:Double-Blind Method; D005260:Female; D006801:Humans; D008176:Lung Volume Measurements; D008297:Male; D009079:Mucociliary Clearance / Q000187:drug effects*; D010865:Pilot Projects; D013183:Sputum / Q000187:drug effects
chemical_list: D000584:Amiloride
vernacular_title: Pilotstudie zur Amiloridinhalation bei Kindern mit cystischer Fibrose.