Objective:
To analyze the clinical characteristics and genotypes of patients with cystic fibrosis (CF) and nasal polyposis who require surgery.
Design:
Cross-sectional analysis of a large patient database.
Setting:
Data obtained from the National CF Patient Registry of the Cystic Fibrosis Foundation, Bethesda, Md.
Patients:
Clinical and genotype data on 20 198 patients with CF who were registered in 1992 and 1993 were analyzed. The study group (n=815) consisted of patients with CF who had undergone surgical procedures for the treatment of nasal polyposis. The comparison group (n=19 383) comprised the remainder of the patients in the database.
Results:
Statistical analysis revealed that patients with CF and nasal polyposis who required surgery had better pulmonary function (higher percent-predicted forced expiratory volume in 1 second and forced vital capacity), better nutritional status, a higher rate of Pseudomonas aeruginosa colonization, more office visits, more hospitalizations, and a higher rate of acute exacerbations per year (P<.001 for each) than did the comparison group. Among the patients who had mutation analysis performed, patients with nasal polyposis who required surgery were significantly associated with 2 specific genotypes: the Δ-F508/Δ-F508 (57.5% vs 49.9%, P=.01) and the Δ-F508/G551D (12% vs 8%, P=.05) genotypes.
Conclusions:
Patients with CF and nasal polyposis who require surgery may constitute a clinical subgroup within the spectrum of the disease. These patients appear to have slightly better pulmonary function and nutritional status; yet, they seem to have a higher degree of health care utilization. The higher rate of P aeruginosa respiratory infection in this patient group suggests an association with the presence of nasal polyposis. Genotype analysis showed a higher prevalence of the Δ-F508/Δ-F508 and the Δ-F508/G551D genotypes in this patient group.Arch Otolaryngol Head Neck Surg. 1996;122:1209-1213