Incidência e evolução da polipose nasal em crianças e adolescentes com fibrose cística

Abstract

Nasal polyps are a clinical sign of alert for investigatingCystic Fibrosis (CF). Aims: To study the incidence of nasal polyps in children and adolescents with cystic fibrosis, its possible association with age, gender, clinical manifestations, genotype and sweat chlorine level, and its evolution with topical steroid therapy. Methods: Clinical symptoms, sweat chlorine level and genotype were studied in 23 cystic fibrosis patients. Nasal polyps were diagnosed by nasal endoscopy and treated with topical steroids during 6 months, followed by a second nasal endoscopy. Fisher test was used for statistical analysis. Results: Nasal polyps were found in 39.1% of the patients (five bilateral, four unilateral), all older than six years, recurrent pneumonia in 82.6%, pancreatic insufficiency in 87% and malnutrition in 74%. No association was seen between nasal polyps and sweat chlorine level, genotype, clinical sings of severity and nasal symptoms. Seven patients improved in their nasal polyps with topical steroids, six showed complete resolution. Conclusion: The study showed a high incidence of nasal polyps in older children, who span the entire range of clinical severity, even in the absence of clinical nasal symptoms. Topical steroid therapy showed good results. An interaction among pediatricians and otolaryngologists is necessary for diagnosis and follow-up.

Avaliação endoscópica nasal de crianças e adolescentes com fibrose cística

“…From 1961 to 2005, the prevalence of nasal polyps increased from 5 to 57%. 26,29,[31][32][33][34][35][36][37][38][40][41][42] Explanations include increased survival rates of CF patients in developed countries, the selection of patients, sample sizes, different age groups in these studies, and especially the more recent routine use of nasal endoscopy as a diagnostic tool. 3,19,48 Brihaye et al 29 showed that over 25% of polyps were not diagnosed in the clinical examination with anterior rhinoscopy without nasal endoscopy in CF patients.…”

“…[28][29][30] The frequency of nasal polyposis varies in different populations, and depends on the evaluation technique. 26,29,[31][32][33][34][35][36][37][38][39][40][41][42] It is thought that nasosinusal involvement may worsen pulmonary manifestations; 20 thus, otorhinolaryngologists have become more involved in evaluating these patients. Recent studies in Brazil 26,40,41 have shown a growing concern with characterizing in more detail the nasosinusal findings of CF patients, because this disorder is genetically very heterogeneous, with many types of mutations and a wide variety of clinical presentations, 28 which may be explained by specific phenotypic features of the Brazilian population and even within populations of any given region in this country.…”

Nasal polyposis in cystic fibrosis: follow-up of children and adolescents for a 3-year period

“…According to data from the literature and the study previously conducted in our service, 23 a need for better characterization of the evolution of UAW involvement in these patients was identified.…”

Nasal endoscopic evaluation of children and adolescents with cystic fibrosis

“… 28 , 29 , 30 The frequency of nasal polyposis varies in different populations, and depends on the evaluation technique. 26 , 29 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 …”

“…It is thought that nasosinusal involvement may worsen pulmonary manifestations; 20 thus, otorhinolaryngologists have become more involved in evaluating these patients. Recent studies in Brazil 26 , 40 , 41 have shown a growing concern with characterizing in more detail the nasosinusal findings of CF patients, because this disorder is genetically very heterogeneous, with many types of mutations and a wide variety of clinical presentations, 28 which may be explained by specific phenotypic features of the Brazilian population and even within populations of any given region in this country.…”