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Author Ellerman, A.; Bisgaard, H. url  openurl
  Title Longitudinal study of lung function in a cohort of primary ciliary dyskinesia Type Journal Article
  Year (down) 1997 Publication The European Respiratory Journal Abbreviated Journal Eur Respir J  
  Volume 10 Issue 10 Pages 2376-2379  
  Keywords Adolescent; Adult; Age Distribution; Bacterial Infections/diagnosis/epidemiology/*physiopathology; Child; Child, Preschool; Ciliary Motility Disorders/diagnosis/epidemiology/*physiopathology; Cohort Studies; Denmark/epidemiology; Disease Progression; Female; Follow-Up Studies; Forced Expiratory Volume; Humans; Longitudinal Studies; Male; Middle Aged; Prognosis; *Respiratory Function Tests; Respiratory Tract Infections/diagnosis/*physiopathology; Time Factors; Vital Capacity  
  Abstract Patients with primary ciliary dyskinesia (PCD) have pronounced stasis of their respiratory secretions and therefore recurrent lower airway infections, which raises concerns for the development of lung function. Twenty four patients with PCD have been studied prospectively with a standardized regime in our clinic for 2-16 yrs with clinic visits, including spirometry 2-4 times per year, daily physiotherapy and monthly sputum cultures with subsequent specific antibiotic treatment. Lung function was significantly lower in the 12 PCD patients entering the cohort as adults when compared to the PCD patients entering as children (forced vital capacity (FVC) 70 versus 85% predicted; forced expiratory volume in one second (FEV1) 59 versus 72% pred). The lung damage did not relate to the type of ciliary dyskinesia. During the subsequent surveillance of the groups for a median of 14 and 7 yrs, respectively, the lung function remained stable in most patients. It is concluded that primary ciliary dyskinesia is accompanied by a progressive deterioration in lung function if undertreated, but lung function can be maintained with appropriate antibiotic treatment and regular physiotherapy. This emphasizes the need for early diagnosis of primary ciliary dyskinesia.  
  Address  
  Corporate Author Thesis  
  Impact Factor 07,636 First Author Ellerman, A. Editor  
  Language English Summary Language Original Title  
  Series Editor Series Title Abbreviated Series Title  
  Series Volume Series Issue Senior Author Bisgaard, H.  
  ISSN 0903-1936 ISBN Medium  
  Area Expedition Conference  
  Notes PMID:9387968 Approved no  
  Call Number Serial 270  
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