Retrospective analysis of chronic rhinosinusitis in patients with cystic fibrosis.

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.

Bacterial pattern in chronic sinusitis and cystic fibrosis.

OBJECTIVE: To assess the association between pathogenic bacteria found in bronchoalveolar lavages and paranasal cavity cultures in patients with cystic fibrosis (CF) who underwent endonasal endoscopic sinus surgery. The authors hypothesized that the pathogenic bacterial pattern of the upper airway would be associated with that of the lower airway. STUDY DESIGN: Prospective case series with planned data collection. SETTING: Tertiary referral center. SUBJECTS AND METHODS: A group of 16 patients with diagnosis of CF who underwent endonasal endoscopic sinus surgery from July 2001 to August 2009 participated in the study. Culture samples were obtained from bronchoalveolar lavages and paranasal cavities. A Fisher exact test was performed to examine the significance of the association between upper airway and lower airway cultures. RESULTS: The most frequent microorganisms in cultures of para-nasal cavities and bronchoalveolar lavages were Pseudomonas aeruginosa, Staphylococcus aureus, and Streptococcus viridans. Statistical analysis revealed a significant association between upper airway cultures (paranasal cavities) and lower airway cavities (bronchoalveolar lavages) for the 2 most frequent microorganisms, P. aeruginosa and S. aureus (P = .007 and P = .030, respectively). CONCLUSION: The authors confirmed their initial hypothesis that a significant association between bronchoalveolar lavages and sinus cultures was established, once more confirming the idea of a unified airway. They found chronic rhinosinusitis with polyps to be a common clinical presentation in patients with CF. Further studies are required to indicate the role of antibiotics and the pathogenesis of the microorganisms as a manifestation of clinical severity.

Guía clínica para el diagnóstico y cuidado de niños/adolescentes con bronquiolitis obliterante post-infecciosa, 2009 / Clinical guidelines for the diagnosis and care of children and adolescents with post-infectious bronchiolitis obliterans, 2009

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.

Análisis retrospectivo de pacientes portadores de rinosinusitis crónica por fibrosis quística / Retrospective analysis of chronic rhinosinusitis in patients with cystic fibrosis

Introducción: La rinosinusitis crónica es muy frecuente en pacientes con fibrosis quística, lo que se puede explicar por el concepto de vía aérea unificada, donde el mismo fenómeno fisiopatológico que afecta a los pulmones, afecta a las cavidades paranasales. El manejo de estos casos es especialmente difícil. Objetivo: Describir el trabajo en equipo de otorrinolaringólogos y neumólogos en pacientes portadores de fibrosis quística. Método: Estudio descriptivo, retrospectivo de 14 pacientes portadores de fibrosis quística con rinosinusitis crónica atendidos durante los últimos 17 años en una clínica privada. Resultados: El 64% es de sexo masculino. La mediana de la edad al punto de corte del estudio es de 23 años. Las mutaciones más frecuentes encontradas son AF508, M470 y R553. El 100% de los pacientes con la mutación AF508 presentan pólipos. El 100% de los pacientes tienen clínica de rinosinusitis crónica. Todos han sido sometidos a cirugía endoscópica. La mediana del número de reintervenciones es de 2 cirugías endoscópicas. Conclusiones: Dada la alta prevalencia de rinosinusitis crónica en estos pacientes, todos deberían tener una tomografía computarizada de cavidades paranasales durante su evaluación inicial considerando que son los gérmenes de las cavidades paranasales los que colonizan la vía aérea baja. El otorrinolaringólogo debe ser parte integral del equipo de fibrosis quística. La cirugía endoscópica debe realizarse en pacientes en que el tratamiento médico no logre limpiar las cavidades paranasales y sea esta infección la que esté colonizando la vía aérea inferior, antes de recibir trasplante de pulmón, o en cefalea crónica(AU)

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 AF508, M470 and R553. All of the patients with AF508 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(AU)