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Article |

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

Todd T. Kingdom, MD; Kelvin C. Lee, MD; Stacey C. Fitz Simmons, PhD; Gerd J. Cropp, MD, PhD
Arch Otolaryngol Head Neck Surg. 1996;122(11):1209-1213. doi:10.1001/archotol.1996.01890230055011.
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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


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