Lower airway inflammation in infants and young children with cystic fibrosis

Am J Respir Crit Care Med. 1997 Oct;156(4 Pt 1):1197-204. doi: 10.1164/ajrccm.156.4.96-11058.


Airway inflammation is an important component of cystic fibrosis (CF) lung disease. To determine whether this begins early in the illness, before the onset of infection, we examined bronchoalveolar lavage (BAL) fluid from 46 newly diagnosed infants with CF under the age of 6 mo identified by a neonatal screening program. These infants were divided into three groups: 10 had not experienced respiratory symptoms or received antibiotics and pathogens were absent in their BAL fluid; 18 had clear evidence of lower respiratory viral or bacterial (> or = 10(5) CFU/ml) infection; and the remaining 18 had either respiratory symptoms, taken antibiotics, or had < 10(5) CFU/ml of respiratory pathogens. Their BAL cytology, interleukin-8, and elastolytic activity were compared with those from 13 control subjects. In a longitudinal study to assess if inflammation develops or persists in the absence of infection, the results of 56 paired annual BAL specimens from 44 CF infants were grouped according to whether they showed absence, development, clearance, or persistence of infection. In newly diagnosed infants with CF, those without infection had BAL profiles comparable with control subjects while those with a lower respiratory infection had evidence of airway inflammation. In older children, the development and persistence of infection was accompanied by increased inflammatory markers, whereas these were decreased in the absence, or with the clearance, of infection. We conclude that airway inflammation follows respiratory infection and, in young children, improves when pathogens are eradicated from the airways.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anti-Bacterial Agents
  • Bacterial Infections / complications*
  • Bacterial Infections / drug therapy
  • Biomarkers
  • Bronchoalveolar Lavage Fluid / cytology
  • Bronchoalveolar Lavage Fluid / microbiology
  • Bronchoalveolar Lavage Fluid / virology
  • Child, Preschool
  • Cross-Sectional Studies
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / diagnosis
  • Cystic Fibrosis / genetics
  • Drug Therapy, Combination / therapeutic use
  • Female
  • Humans
  • Infant
  • Inflammation / etiology
  • Inflammation / metabolism
  • Interleukin-8 / metabolism
  • Leukocyte Count
  • Leukocyte Elastase / metabolism
  • Longitudinal Studies
  • Male
  • Neutrophils / enzymology
  • Neutrophils / pathology
  • Pneumonia, Bacterial / complications*
  • Pneumonia, Bacterial / metabolism
  • Pneumonia, Viral / complications*
  • Pneumonia, Viral / metabolism
  • Respiratory Tract Infections / complications*
  • Respiratory Tract Infections / drug therapy
  • Virus Diseases / complications*
  • Virus Diseases / drug therapy


  • Anti-Bacterial Agents
  • Biomarkers
  • Interleukin-8
  • Leukocyte Elastase