Abstract
As principais manifestacoes otorrinolaringologicas da Fibrose Cistica sao a rinossinusite cronica e a polipose nasossinusal, com diferentes apresentacoes clinicas. OBJETIVO: Caracterizar, do ponto de vista nasossinusal, as criancas e adolescentes com fibrose cistica por meio de um questionario, do exame clinico e da endoscopia nasal. FORMA DE ESTUDO: Clinico descritivo transversal. MATERIAL E METODO: Avaliacao de 100 criancas e adolescentes com fibrose cistica por meio de um questionario especifico, exame fisico otorrinolaringologico, endoscopia nasal e estadiamento endoscopico dos polipos nasais. RESULTADOS: Os sintomas mais frequentes foram: tosse (45%), respiracao oral (44%), disturbios do sono (42%) e obstrucao nasal (37%). Vinte e oito pacientes (28%) apresentaram secrecao mucopurulenta nasal e 41% apresentaram abaulamento medial da parede lateral do nariz. Os polipos nasais foram identificados em apenas 14% dos casos, nenhum deles era obliterante. CONCLUSAO: O questionario, o exame clinico e especialmente a endoscopia nasal permitiram uma avaliacao detalhada das caracteristicas nasais das criancas e adolescentes com fibrose cistica. Alguns achados foram discordantes da literatura, principalmente a baixa prevalencia encontrada de polipos nasais, e parecem estar relacionados com caracteristicas proprias da populacao estudada. A melhor caracterizacao desse grupo de pacientes, do ponto de vista otorrinolaringologico, contribui para uma adequada abordagem multidisciplinar.
Highlights
Cystic fibrosis (CF) is the most common inherited autosomal recessive disorder in Caucasians.[1,2,3,4,5,6] Its prevalence ranges from 1:6.000 to 1:2.000 of live births in white populations of developed countries;[1,3,4,6,7,8,9] it is rare in black (1:30.000) and Asian (1:90.000)[10,11] populations
In Brazil, the incidence ranges from 1:9.500 in Parana state,[12] to 1:8.700 in Santa Catarina state,[13] and 1:10.000 in Minas Gerais state.[14]. This disease results from mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR), located on the q31 region of the long arm of chromosome 7.15-17 This gene, which was described in 1989,15-17 encodes for a protein that functions as a chloride channel; dysfunction of this channel results in altered sodium, chloride and water transport across the apical membrane of epithelial cells in the respiratory tract and in exocrine glands
Our results shown that all patients with nasal polyps had nasal discharges, there was no relation between the presence of nasal polyps and symptoms such as coughing, rhinorrhea, mouth breathing, agitated sleep, headaches, and nasal block, even though certain authors[1,3,24,28] have described nasal block as a typical symptom in CF patients with nasal polyps
Summary
Cystic fibrosis (CF) is the most common inherited autosomal recessive disorder in Caucasians.[1,2,3,4,5,6] Its prevalence ranges from 1:6.000 to 1:2.000 of live births in white populations of developed countries;[1,3,4,6,7,8,9] it is rare in black (1:30.000) and Asian (1:90.000)[10,11] populations. In Brazil, the incidence ranges from 1:9.500 in Parana state,[12] to 1:8.700 in Santa Catarina state,[13] and 1:10.000 in Minas Gerais state.[14]. This disease results from mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR), located on the q31 region of the long arm of chromosome 7.15-17 This gene, which was described in 1989,15-17 encodes for a protein that functions as a chloride channel; dysfunction of this channel results in altered sodium, chloride and water transport across the apical membrane of epithelial cells in the respiratory tract and in exocrine glands. Mucosal edema ensues, compromising ciliary function and favoring bacterial colonization, usually by Staphylococcus aureus and Pseudomonas aeruginosa.[3,6,18,26]
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