title: The clinical effect and the effect on the ciliary motility of oral N-acetylcysteine in patients with cystic fibrosis and primary ciliary dyskinesia.
abstract: The effect of peroral N-acetylcysteine (NAC) in patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) was investigated. 41 CF patients and 13 PCD patients completed the study which was a double-blind, placebo-controlled, cross-over trial. The patients received either NAC or placebo for two periods of three months followed by a three month follow-up period. Active treatment consisted of NAC, either 200 mg x 3 daily (patients weighing less than 30 kg) or 400 mg x 2 daily (greater than 30 kg). The effect was evaluated in terms of a subjective clinical score, weight, sputum bacteriology, blood leucocyte count, sedimentation rate, titres of specific antimicrobial antibodies, lung function parameters and measurement of the ciliary function. No effect was seen in PCD patients, but in CF patients an improved lung function was seen in the period when the patients suffer most from lower airway infections.
authors.affiliation: Paediatric Department, Rigshospitalet, Copenhagen, Denmark.
authors.forename: G
authors.initials: G
authors.lastname: Stafanger
authors.forename: S
authors.initials: S
authors.lastname: Garne
authors.forename: P
authors.initials: P
authors.lastname: Howitz
authors.forename: E
authors.initials: E
authors.lastname: Morkassel
authors.forename: C
authors.initials: C
authors.lastname: Koch
mesh_terms: D000111:Acetylcysteine / Q000627:therapeutic use*; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002923:Cilia / Q000187:drug effects* / Q000502:physiology; D002925:Ciliary Motility Disorders / Q000188:drug therapy / Q000503:physiopathology*; D002986:Clinical Trials as Topic; D003550:Cystic Fibrosis / Q000188:drug therapy / Q000503:physiopathology*; D004311:Double-Blind Method; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D066298:In Vitro Techniques; D007223:Infant; D008297:Male; D010366:Peak Expiratory Flow Rate; D012137:Respiratory System / Q000503:physiopathology; D014797:Vital Capacity
chemical_list: D000111:Acetylcysteine