Longitudinal study of lung function in a cohort of primary ciliary dyskinesia

A Ellerman, H Bisgaard

230 Citations (Scopus)

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.

Original languageEnglish
JournalEuropean Respiratory Journal
Volume10
Issue number10
Pages (from-to)2376-9
Number of pages4
ISSN0903-1936
Publication statusPublished - Oct 1997

Keywords

  • Adolescent
  • Adult
  • Age Distribution
  • Bacterial Infections
  • Child
  • Child, Preschool
  • Ciliary Motility Disorders
  • Cohort Studies
  • Denmark
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Forced Expiratory Volume
  • Humans
  • Longitudinal Studies
  • Male
  • Middle Aged
  • Prognosis
  • Respiratory Function Tests
  • Respiratory Tract Infections
  • Time Factors
  • Vital Capacity

Fingerprint

Dive into the research topics of 'Longitudinal study of lung function in a cohort of primary ciliary dyskinesia'. Together they form a unique fingerprint.

Cite this