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Published ahead of print on August 13, 2003, doi:10.1164/rccm.200303-434OC

Am. J. Respir. Crit. Care Med., Volume 168, Number 9, November 2003, 1100-1108

A more recent version of this article appeared on November 1, 2003
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Submitted on April 9, 2003
Accepted on August 6, 2003

Bronchopulmonary Disease in Children with Cystic Fibrosis After Early or Delayed Diagnosis

Philip M Farrell1*, Zhanhai Li2, Michael R Kosorok2, Anita Laxova1, Christopher G Green1, Jannette Collins3, Hui-Chuan Lai4, Michael J Rock1, and Mark L Splaingard5

1 Pediatrics, University of Wisconsin, Madison, WI, USA, 2 Pediatrics, University of Wisconsin, Madison, WI, USA; Biostatistics/Medical Informatics, University of Wisconsin, Madison, WI, USA, 3 Radiology, University of Wisconsin, Madison, WI, USA, 4 Nutritional Sciences, University of Wisconsin, Madison, WI, USA, 5 Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA

* To whom correspondence should be addressed. E-mail: pmfarrel{at}facstaff.wisc.edu.

Background Although early diagnosis of cystic fibrosis (CF) can lead to nutritional benefits, there has been uncertainty about pulmonary outcomes. Methods Using a randomized controlled trial with unique unblinding/surveillance, we evaluated CF patients who received similar treatment after being assigned to an early diagnosis (screened) group or a standard diagnosis (control) group. When the youngest was 7 years of age, we compared outcomes using pulmonary function data and quantitative chest radiology. Results The screened group (56 patients) was diagnosed at a younger age of 12.4 weeks, compared to 47 controls (95.8 weeks), but included a significantly greater proportion of patients with DF508 genotypes and pancreatic insufficiency. The first chest radiograph showed significantly fewer abnormalities in the screened group, but over time the two groups converged, and after 10 years of age the screened patients showed worse chest x-ray scores associated with earlier acquisition of Pseudomonas aeruginosa. No differences were detected in any measure of pulmonary dysfunction, which was generally mild in each group. Conclusions Although CF neonatal screening provides a potential opportunity for better pulmonary outcomes, it appears that respiratory infections and pancreatic status are the dominant factors in pulmonary prognosis.
Key words: cystic fibrosis, newborn screening, pulmonary disease, Pseudomonas aeruginosa




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