Study Results

• What We Learned:

• Primary Findings:

  Effectiveness:

  The data evaluated for this publication included 889 children with CF =12 years of age from the EPIC Observational Study who had no isolation of Pa from respiratory cultures. The primary endpoint for the analysis was age at initial Pa acquisition, defined as the age at first isolation of Pa from a clinically-collected respiratory culture. CFTR mutations with minimal function were associated with earlier Pa acquisition compared to mutations with residual function; the median age at Pa acquisition was 2.9 years among participants with minimal CFTR function vs.10.3 years for those with residual CFTR function (hazard ratio (HR) comparing minimal to residual CFTR function 2.87 (95% CI 1.88, 4.39)). Home environmental exposure as possible risk factors was evaluated. None of these factors, including cigarette smoke, hot tub use, breastfeeding, or daycare attendance, was associated with age at initial Pa acquisition. Newborn screening was not associated with age at Pa acquisition. Key associations were validated in a CF Foundation National Patient Registry replication cohort.

   

  Safety:

  Because this was an observational study no safety measures were applicable.

• Citation:

  Pediatr Pulmonol 2010;DOI 10.1002/ppul.21279;45(9):934-44

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