Clinical Trial: Inflammatory and Microbiologic Markers in Sputum: Comparing Cystic Fibrosis With Primary Ciliary Dyskinesia

Study Status: Completed
Recruit Status: Completed
Study Type: Interventional

Official Title: Inflammatory and Microbiologic Markers in Sputum in Response to Pulmonary Exacerbation: Comparing Cystic Fibrosis With Primary Ciliary Dyskinesia

Brief Summary: The objective of this study is to compare the lower airways inflammatory response to infection/pulmonary exacerbation among children known to have Primary Ciliary Dyskinesia (PCD) with children known to have Cystic Fibrosis (CF) as measured by the presence of inflammatory mediators in expectorated/induced sputum.

Detailed Summary: The inflammatory response to infection and pulmonary exacerbation in CF is well documented, as is the response to intravenous antibiotic treatment. On the other hand, the inflammatory response to infection and treatment in PCD has not been well characterized. Given differences in disease progression, we hypothesize that children with CF respond to infection with a more exaggerated and prolonged inflammatory response than those with PCD.
Sponsor: The Hospital for Sick Children

Current Primary Outcome:

• Change in sputum bacterial colony count [ Time Frame: Up to 100 days ]

  For the following organisms (Staphylococcus aureus, Haemophilus influenza) in response to a prescribed treatment course of oral antibiotics.

  Colony count will be done at three time points:

  • during respiratory exacerbation (Visit 1 - Day 0),
  • post-antibiotic treatment of exacerbation (Visit 2 - Day 21-42),
  • and on return to clinical baseline (Visit 3 - Day 42-100 (End of Study)).during the study.
• Airway Inflammatory Profile [ Time Frame: Up to 100 days ]

  As measured by sputum interleukin 8 (IL-8) at three time points:

  • during respiratory exacerbation (Visit 1 - Day 0),
  • post-antibiotic treatment of exacerbation (Visit 2 - Day 21-42),
  • and on return to clinical baseline (Visit 3 - Day 42-100 (End of Study)).

Original Primary Outcome: Same as current

Current Secondary Outcome:

• Culture, identification, and antibiotic susceptibility pattern of respiratory pathogens from sputum samples [ Time Frame: Up to 100 days ]

  Will be done at three time points:

  • during respiratory exacerbation (Visit 1 - Day 0),
  • post-antibiotic treatment of exacerbation (Visit 2 - Day 21-42),
  • and on return to clinical baseline (Visit 3 - Day 42-100 (End of Study)).during the study.
• Tolerability and need for sputum induction in Cystic Fibrosis (CF) patients in comparison to Primary Ciliary Dyskinesia (PCD) patients [ Time Frame: Up to 100 days ]

  Sputum will be collected at three time points:

  • during respiratory exacerbation (Visit 1 - Day 0),
  • post-antibiotic treatment of exacerbation (Visit 2 - Day 21-42),
  • and on return to clinical baseline (Visit 3 - Day 42-100 (End of Study)).
• Change in forced expiratory volume in 1 second (FEV1) in response to a treatment course of antibiotics for pulmonary exacerbation. [ Time Frame: Up to 100 days ]

  FEV1 will be measured at three time points:

  • during respiratory exacerbation (Visit 1 - Day 0),
  • post-antibiotic treatment of exacerbation (Visit 2 - Day 21-42),
  • and on return to clinical baseline (Visit 3 - Day 42-100 (End of Study)).during the study.
• Other markers of airway inflammation [ Time Frame: Up to 100 days ]

  Measurement of sputum white cell and neutrophil count (absolute and relative values), neutrophil elastase, nitric oxide (NO), NO metabolites and arginase levels at three time points:

  • during respiratory exacerbation (Visit 1 - Day 0),
  • post-antibiotic treatment of exacerbation (Visit 2 - Day 21-42),
  • and on return to clinical baseline (Visit 3 - Day 42-100 (End of Study)).

Original Secondary Outcome: Same as current

Information By: The Hospital for Sick Children

Dates:
Date Received: June 29, 2010
Date Started: January 2010
Date Completion:
Last Updated: May 21, 2015
Last Verified: May 2015