Abstract

IntroductionNasal polyposis is often found in patients with cystic fibrosis. ObjectiveTo assess the incidence of nasal polyposis, the response to medical treatment, recurrence and the need for surgical intervention in children and adolescents with cystic fibrosis during a three-year follow-up. MethodsClinical symptoms (pulmonary, pancreatic insufficiency, malnutrition, nasal obstruction), two positive sweat chloride tests, and genotype findings in 23 patients with cystic fibrosis were analyzed. All patients underwent nasal endoscopy every 12 months from January 2005 to December 2007, to assess the presence and grade of Nasal Polyps. Nasal polyposis, when present, were treated with topical corticosteroids for 6–12 months, with progress being evaluated within the 3 years of follow-up. ResultsIn the first evaluation, nasal polyposis was diagnosed in 30.43% of patients (3 bilateral and 4 unilateral), recurrent pneumonia in 82.6%, pancreatic insufficiency in 87%, and malnutrition in 74%. The presence of nasal polyposis was not associated with chloride values in the sweat, genotype, clinical signs of severity of cystic fibrosis, or nasal symptoms. In the three-year period of follow up, 13 patients (56.52%) had at least one event of polyposis, with the youngest being diagnosed at 32 months of age. Only one patient underwent surgery (polypectomy), and there was one diagnosis of nasopharyngeal carcinoma. ConclusionThe study showed a high incidence of nasal polyposis. Monitoring through routine endoscopy in patients with cystic fibrosis, even in the absence of nasal symptoms, is highly recommended. The therapy with topical corticosteroids achieved good results. Thus, an interaction between pediatricians and otolaryngologists is necessary.

Highlights

  • Nasal polyposis is often found in patients with cystic fibrosis

  • The initial sample consisted of 23 patients (20 males), aged 1 year and 9 months to 22 years and 8 months, followed at the Cystic Fibrosis Reference Center of Pediatrics Pneumology Department of the institution concerned

  • Cystic fibrosis (CF) diagnosis was confirmed in all subjects through the sweat test

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Summary

Introduction

Nasal polyposis is often found in patients with cystic fibrosis. Objective: To assess the incidence of nasal polyposis, the response to medical treatment, recurrence and the need for surgical intervention in children and adolescents with cystic fibrosis during a three-year follow-up. Methods: Clinical symptoms (pulmonary, pancreatic insufficiency, malnutrition, nasal obstruction), two positive sweat chloride tests, and genotype findings in 23 patients with cystic fibrosis were analyzed. Results: In the first evaluation, nasal polyposis was diagnosed in 30.43% of patients (3 bilateral and 4 unilateral), recurrent pneumonia in 82.6%, pancreatic insufficiency in 87%, and malnutrition in 74%. Cystic fibrosis (CF) is an autosomal recessive disease that affects the exocrine glands, involving multiple organs and progressing chronically and progressively It is the most common lethal genetic disease in Caucasians, with an average frequency of 1:2000 live births.[1,2] In Brazil, studies have revealed an incidence of 1:9500 live births in the state of Parana,3 1:8700 in Santa Catarina[4] and. Mortality has been reduced in recent years due to earlier diagnosis, greater attention to prophylaxis of recurrent airway infections, and better control of patients in specialized services.[1,2]

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