November 1996

Clinical Characteristics and Genotype Analysis of Patients With Cystic Fibrosis and Nasal Polyposis Requiring Surgery

Author Affiliations

From the Departments of Otolaryngology–Head and Neck Surgery (Drs Kingdom and Lee) and Pediatrics (Dr Cropp), University of California, San Francisco, and the Cystic Fibrosis Foundation, Bethesda, Md (Dr FitzSimmons). Dr Kingdom is now with the Division of Otolaryngology–Head and Neck Surgery, Department of Surgery, Emory University School of Medicine, Atlanta, Ga.

Arch Otolaryngol Head Neck Surg. 1996;122(11):1209-1213. doi:10.1001/archotol.1996.01890230055011

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