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Pulmonary exacerbations in cystic fibrosis: Young children with characteristic signs and symptoms

  • Researchers

    Regelmann WE, Schechter MS, Wagener JS, Morgan WJ, Pasta DJ, Elkin EP, Konstan MW; for the Investigators of the Epidemiologic Study of Cystic Fibrosis.

  • Place of research

    University of Minnesota

  • Publication

    Pediatric Pulmonology / September 2012

  • Subjects

    , , ,

  • What clinical features do doctors use to diagnose a pulmonary exacerbation in young children?

  • Why is this important?

    Pulmonary exacerbations are strongly tied to deterioration in lung function in patients with CF. How do clinicians caring for patients with cystic fibrosis (CF) diagnose a pulmonary exacerbation, especially in children less than 6 years old who cannot perform lung function tests?

  • What did you do?

    Analyzed data from the large Epidemiologic Study of Cystic Fibrosis

  • What did you find?

    We found that the presence of increased cough, increased sputum, new crackles on chest exam and decreased weight-for-age percentile each independently increased the odds that a pulmonary exacerbation would be diagnosed and treated by North American clinicians. Hence, the presence of one or more of these four characteristics identified a pulmonary exacerbation in young CF children. How did those children who had these characteristics fare compared with those who did not? The number of these characteristics present at a single visit before age 6 increased the odds of hospitalization in the next year, and the odds of lower weight, and of lower lung function at age 7. However, it was remarkable that even when three or more of these characteristics were present, 61% were not treated. Those who were diagnosed and treated had a decrease in the proportion with crackles, cough, and Pseudomonas aeruginosa at their short-term follow-up visit compared with those not diagnosed and treated.

  • What does this mean and reasons for caution?

    We conclude that in CF patients under 6 years old, increased cough, increased sputum, new crackles on chest exam and decreased weight-for-age percentile are characteristic of a pulmonary exacerbation, and that diagnosis of pulmonary exacerbation using these characteristics followed by antibiotic treatment improves short term respiratory outcomes.

  • What's next?

    Many children with these characteristics are not treated for a pulmonary exacerbation yet would likely benefit in the short term. Improved therapies are needed to produce long term benefits in young CF children with a pulmonary exacerbation.