Defining an exacerbation of pulmonary disease in cystic fibrosis

Pediatr Pulmonol. 2001 Jun;31(6):436-42. doi: 10.1002/ppul.1072.


Despite the importance of pulmonary exacerbations in CF in both clinical and research settings, both published evidence and consensus are lacking concerning the criteria used to define an exacerbation. The use of hospitalization as a surrogate measure presupposes uniformity among clinicians in diagnosis and treatment of exacerbations. Our aims were to evaluate consensus among clinicians about the variables considered helpful in diagnosing an exacerbation requiring treatment. A comprehensive list of symptoms, signs, and investigations used to define exacerbations was compiled from published trials. A written self-administered questionnaire included the list in age-appropriate groups to survey opinion about the helpfulness of each item, and the estimated proportion of patients admitted within a month of diagnosis of an exacerbation. This was sent to all clinicians managing CF patients in Australia. There were replies from 59/91 clinicians (65%), 41/60 (68%) from those managing children and 18/31 (58%) from those managing adults. Responses of those managing children and adults differed for 7/32 variables (Mann-Whitney test, P < 0.05). Clinic grouping did not show greater consensus among responses of pediatricians (Kruskal-Wallis test, P = 0.362). Consensus, >74% or <26% of respondents rating a variable helpful/very helpful, was found in only 50% of variables listed. Estimated admission rate within a month of diagnosis was 61% (30-100%) for those managing adults and 48% %5-100%) for pediatricians. A lack of consensus was found among clinicians managing CF about the variables considered in diagnosing an exacerbation. The estimated proportion admitted within a month of diagnosis was very variable. This demonstrated inhomogeneity in approach to diagnosis and management of an exacerbation suggests a significant heterogeneity of clinical care.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Cough
  • Cystic Fibrosis / classification
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / pathology
  • Delphi Technique*
  • Diagnosis, Differential
  • Disease Progression
  • Female
  • Humans
  • Lung Diseases / classification*
  • Lung Diseases / etiology
  • Lung Diseases / pathology
  • Male
  • Physicians
  • Respiratory Function Tests
  • Severity of Illness Index