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  • Title: Clinical characteristics and genotype analysis of patients with cystic fibrosis and nasal polyposis requiring surgery.
    Author: Kingdom TT, Lee KC, FitzSimmons SC, Cropp GJ.
    Journal: Arch Otolaryngol Head Neck Surg; 1996 Nov; 122(11):1209-13. PubMed ID: 8906056.
    Abstract:
    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 20198 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 = 19383) 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 delta-F508/delta-F508 (57.5% vs 49.9%, P = .01) and the delta-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 delta-F508/delta-F508 and the delta-F508/G551D genotypes in this patient group.
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