RESUMO
INTRODUCTION: Chronic rhinosinusitis is very common in patients with cystic fibrosis. This can be explained by the unified airway concept, where the same pathophysiological phenomenon that affects the lungs, affects the paranasal sinuses. The management of these cases is difficult. OBJECTIVE: To describe the teamwork of otolaryngologists and bronchopulmonary specialists in patients with cystic fibrosis. METHOD: We performed a descriptive, retrospective study over the last 17 years, which included 14 patients with chronic rhinosinusitis and cystic fibrosis attended at a private hospital. RESULTS: Of the patients, 64% were male and the median age was 23 years. The most frequent mutations found were ΔF508, M470 and R553. All of the patients with ΔF508 mutation had nasal polyps. 100% of the patients had clinical findings of chronic rhinosinusitis. All the patients had had endoscopic nasal surgery. The median number of endoscopic surgeries was 2. CONCLUSIONS: Given the high prevalence of chronic rhinosinusitis in patients with cystic fibrosis, everyone should have a computed tomography scan of the paranasal sinuses during the initial assessment, considering that sinus germs are the ones that colonise the lower airway. The otolaryngologist should be part of the cystic fibrosis team. Before receiving a lung transplant or in cases of chronic headache, endoscopic surgery should be performed in patients in whom medical treatment fails to clear the sinuses because this infection is the one that colonises the lower airway.
Assuntos
Fibrose Cística/complicações , Rinite/etiologia , Sinusite/etiologia , Adulto , Cloro/análise , Doença Crônica , Fibrose Cística/genética , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Feminino , Humanos , Masculino , Obstrução Nasal/diagnóstico por imagem , Obstrução Nasal/etiologia , Pólipos Nasais/complicações , Pólipos Nasais/genética , Pólipos Nasais/cirurgia , Reoperação , Estudos Retrospectivos , Deleção de Sequência , Sinusite/diagnóstico por imagem , Suor/química , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
La bronquiolitis obliterante (BO) es un síndrome clínico poco frecuente en niños, caracterizado por la obstrucción crónica al flujo de aire asociado a cambios inflamatorios y distintos grados de fibrosis en la vía aérea pequeña. Si bien existen muchas etiologías, la causa mas frecuente se asocia a infeccionesrespiratorias virales, principalmente adenovirus. No existe un consenso para establecer su diagnóstico; sin embargo, se considera un espectro de síntomas persistentes asociados a un patrón en mosaico, bronquiectasias y atelectasias persistentes. El rol de la biopsia pulmonar ha sido cuestionado por subajo rendimiento, invasividad y complicaciones. No existe un tratamiento específico por lo que elmanejo es soporte. Probablemente la mejor estrategia constituya el empleo de antibióticos en forma agresiva, soporte kinésico y nutricional constante y una precoz rehabilitación pulmonar. Estas guías clínicas representan un esfuerzo multidisciplinario, basado en evidencias actuales para brindarherramientas prácticas para el diagnóstico y cuidado de niños y adolescentes con BO post infecciosa.