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Published ahead of print on August 13, 2003, doi:10.1164/rccm.200303-434OC
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American Journal of Respiratory and Critical Care Medicine Vol 168. pp. 1100-1108, (2003)
© 2003 American Thoracic Society

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

Philip M. Farrell, Zhanhai Li, Michael R. Kosorok, Anita Laxova, Christopher G. Green, Jannette Collins, Hui-Chuan Lai, Michael J. Rock and Mark L. Splaingard

Departments of Pediatrics, Biostatistics/Medical Informatics, Radiology, and Nutritional Sciences, University of Wisconsin, Madison; and Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin

Correspondence and requests for reprints should be addressed to Philip M. Farrell, M.D., Ph.D., University of Wisconsin Medical School, Room 1217 MSC, 1300 University Avenue, Madison, WI 53706-1532. E-mail: pmfarrel{at}facstaff.wisc.edu

Although early diagnosis of cystic fibrosis (CF) can lead to nutritional benefits, there has been uncertainty about pulmonary outcomes. Using a randomized controlled trial with unique unblinding/surveillance, we evaluated patients with CF who received similar treatment after being assigned to an early diagnosis (screened) group or to a standard diagnosis (control) group. When the youngest patient was 7 years of age, we compared outcomes using pulmonary function data and quantitative chest radiology. In the screened group (56 patients), diagnosis was made at a younger age of 12.4 weeks, compared with the diagnosis in control group (47 control patients) at the age of 95.8 weeks, but included a significantly greater proportion of patients with {Delta}F508 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. 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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