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Am J Epidemiol 2004; 159:537-546.
Copyright © 2004 by the Johns Hopkins Bloomberg School of Public Health

ORIGINAL CONTRIBUTIONS

Association between Initial Disease Presentation, Lung Disease Outcomes, and Survival in Patients with Cystic Fibrosis

HuiChuan J. Lai1,2 , Yu Cheng2, Hyungjun Cho2, Michael R. Kosorok2,3 and Philip M. Farrell3

1 Department of Nutritional Sciences, University of Wisconsin College of Agriculture and Life Sciences, Madison, WI.
2 Department of Biostatistics and Medical Informatics, University of Wisconsin School of Medicine, Madison, WI.
3 Department of Pediatrics, University of Wisconsin School of Medicine, Madison, WI.

This US study was conducted to determine whether mode of diagnosis and initial disease presentation influence lung disease and survival in patients with cystic fibrosis. The study population included 27,703 patients reported to the 1986–2000 Cystic Fibrosis Foundation Registry. Patients were segregated into four diagnostic categories: meconium ileus (MI), prenatal/neonatal screening (SCREEN), positive family history (FH), and symptoms other than meconium ileus (SYMPTOM). When compared with patients in the SCREEN group, those in the MI or SYMPTOM group were found to have significantly greater risks of shortened survival, Pseudomonas aeruginosa acquisition, and forced expiratory volume in 1 second (FEV1) below 70% of predicted. In the SYMPTOM group, the greatest risks of shortened survival, P. aeruginosa acquisition, and FEV1 <70% occurred for patients presenting with combined respiratory and gastrointestinal symptoms, followed by respiratory or gastrointestinal symptoms alone; the best outcomes were in patients with other presenting features. Additionally, patients with presumably "severe" genotypes ({Delta}F508 plus other class I, II, III mutations in both alleles) had greater risks of shortened survival and P. aeruginosa acquisition compared with patients with presumably "mild" genotypes (class IV or V mutations in one or both alleles).

cystic fibrosis; diagnosis; genotype; lung diseases; registries; signs and symptoms; signs and symptoms, respiratory; survival

Abbreviations: Abbreviations: CFTR, cystic fibrosis transmembrane regulator gene; CI, confidence interval; FEV1, forced expiratory volume in 1 second; FH, group of patients with a positive family history without symptoms; GI, group of patients with gastrointestinal symptoms; MI, group of patients with meconium ileus; OR, odds ratio; OTHER, group of patients with other symptoms; RESP, group of patients with respiratory symptoms; SCREEN, group of patients without meconium ileus and identified via prenatal/neonatal screening; SYMPTOM, group of patients with symptoms other than meconium ileus.


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