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Lung function in South African children with cystic fibrosis

H J Zar1, B Moore, A Argent

  • 1Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, Cape Town.

South African Medical Journal = Suid-Afrikaanse Tydskrif Vir Geneeskunde
|October 1, 1998
PubMed

Insights

Most cystic fibrosis (CF) patients show mild lung function impairment, with lower airway obstruction (LAO) being common. Pseudomonas aeruginosa (PA) colonization may worsen lung function and disease severity in CF patients.

Area of Science:

  • Pulmonology
  • Genetics
  • Microbiology

Background:

  • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs.
  • Understanding lung function patterns and associated factors is crucial for managing CF.
  • Pseudomonas aeruginosa (PA) colonization is a common complication in CF patients, impacting disease progression.

Purpose of the Study:

  • To characterize lung function patterns in stable cystic fibrosis (CF) patients.
  • To explore the links between abnormal lung function and demographic factors, CF genotype, and PA colonization.
  • To assess bronchodilator responsiveness in CF patients.

Main Methods:

  • A descriptive study was conducted at a dedicated CF clinic.
  • Pulmonary function testing (PFT) was performed on 42 stable CF patients.
  • Data on demographics, CF genotype, and PA colonization were collected and analyzed.

Main Results:

  • The majority of patients (69%) had mild lung disease; 57% showed lower airway obstruction (LAO).
  • Patients with moderate/severe disease were older than those with mild disease (13.3 vs 11.1 years).
  • PA colonization was significantly associated with both lung function patterns and disease severity (P < 0.05).

Conclusions:

  • Mild lung function impairment and LAO are prevalent in stable CF patients.
  • A small proportion of patients demonstrated bronchodilator responsiveness.
  • PA colonization appears linked to the development of abnormal lung function and more severe pulmonary disease in CF.
Abstract

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