Chronic rhinosinusitis and nasal polyposis in cystic fibrosis: update on diagnosis and treatment / Rinossinusite crônica e polipose nasossinusal na fibrose cística: atualização sobre diagnóstico e tratamento

Although cystic fibrosis (CF) is an irreversible genetic disease, advances in treatment have increased the life expectancy of CF patients. Upper airway involvement, which is mainly due to pathological changes in the paranasal sinuses, is prevalent in CF patients, although many are only mildly symptomatic (with few symptoms). The objective of this literature review was to discuss the pathophysiology and current therapeutic management of chronic rhinosinusitis (CRS) in CF patients. The review was based on current evidence, which was classified in accordance with the Oxford Centre for Evidence-Based Medicine criteria. When symptomatic, CRS with nasal polyps can affect quality of life and can lead to pulmonary exacerbations, given that the paranasal sinuses can be colonized with pathogenic bacteria, especially Pseudomonas aeruginosa. Infection with P. aeruginosa plays a crucial role in morbidity and mortality after lung transplantation in CF patients. Although clinical treatment of the upper airways is recommended as initial management, this recommendation is often extrapolated from studies of CRS in the general population. When sinonasal disease is refractory to noninvasive therapy, surgery is indicated. Further studies are needed in order to gain a better understanding of upper airway involvement and improve the management of CRS in CF patients, with the objective of preserving lung function and avoiding unnecessary invasive procedures.

A fibrose cística (FC) é uma doença genética irreversível, mas os avanços no tratamento têm aumentado a expectativa de vida dos pacientes. O acometimento das vias aéreas superiores, principalmente por alterações patológicas dos seios paranasais, é prevalente nesses pacientes, embora muitos apresentem poucos sintomas. O objetivo desta revisão é discutir a fisiopatologia e o manejo terapêutico atual da rinossinusite crônica (RSC) na FC. A revisão fundamentou-se nas evidências mais recentes, classificadas em conformidade com os critérios do Oxford Centre for Evidence-Based Medicine. Quando sintomática, a RSC com pólipos nasais pode afetar a qualidade de vida e as exacerbações pulmonares, já que os seios paranasais podem ser colonizados por bactérias patogênicas, principalmente a Pseudomonas aeruginosa. Essa bactéria tem papel crucial na morbidade e mortalidade após o transplante pulmonar em pacientes com FC. Embora o tratamento clínico das vias aéreas superiores seja indicado no manejo inicial, a indicação é muitas vezes extrapolada de estudos sobre RSC na população geral. A cirurgia é a alternativa quando o quadro nasossinusal é refratário à terapia não invasiva. Mais estudos são necessários para compreender melhor o acometimento das vias aéreas superiores e melhorar o manejo da RSC na FC, a fim de preservar a função pulmonar e evitar procedimentos invasivos desnecessários.

An experimental model of chronic rhinosinusitis in rabbits without bacterial inoculation

PURPOSE: Evaluate and compare two different experimental techniques of maxillary sinus ostium occlusion using N-butyl cyanoacrylate in developing chronic histological findings without the inoculation of pathogenic bacteria among rabbits. METHODS: In a randomized study, sixteen New Zealand rabbits were assigned for occlusion of the right maxillary sinus through a transmaxillary approach or through the roof of the nasal cavity. The contralateral sinus served as a control. After 12 weeks, the animals were sacrificed for blinded histopathological analysis of the maxillary sinus mucosa. RESULTS: Histopathological changes consistent with CRS were found in eight (100%) of the maxillary sinuses approached transmaxillary and three of those through the roof of the nasal cavity (37.5%), p 0.008 and 0.250, respectively, comparing with the control side. Chronic mucosal changes were significantly better induced using the transmaxillary approach (p 0.026). CONCLUSION: It is possible to induce a model of chronic sinusitis among rabbits with transmaxillary sinus occlusion without bacterial inoculation. This model can be replicated for future cellular studies. .

Rinossinusite fúngica invasiva aguda com desfecho favorável / Acute invasive fungal rhinosinusitis with favorable outcome

A rinossinusite fúngica invasiva aguda é uma patologia que afeta principalmente pacientes imunocomprometidos, tendo morbidade e mortalidade elevadas.Apresentamos um caso de aspergilose invasiva aguda em paciente imunocomprometido, que apresentou bom desfecho pelo diagnóstico precoce e rápida intervenção. Relato de caso: Menina de 13 anos com diagnóstico de leucemia mieloide aguda interna por neutropenia febril após quimioterapia. TCseios da face mostrou opacificação parcial dos seios à esquerda. Leucograma revelou contagem de 880 leucócitos totais. À endoscopia nasal, constatou-se corneto médio isquêmico à esquerda. Levada de urgência ao bloco cirúrgico e realizado debridamento amplo. Anatomopatológico revelou áreas de necrose isquêmica e estruturas fúngicas angioinvasivas, compatíveis com aspergilose.Exame de cultura de fungo confirmou Aspergillus flavus. Iniciado tratamento com voriconazol. Paciente realiza acompanhamento com a Oncologia Pediátrica,mantendo-se em remissão oncológica e sem recidiva fúngica. A frequência das infecções micóticas do nariz e seios paranasais vem aumentando nas últimas décadas. Há um quadro febril, com ou sem sintomas nasais, sem resposta a antibióticos. Endoscopia nasal é o exame mais importante. O corneto médio costuma ser mais acometido. Não há sinais patognomônicos na TC, servindo mais como um instrumento de diagnóstico diferencial, planejamento emonitoramento pós-terapêutico. A correção concomitante de qualquer distúrbio metabólico ou imunológico subjacente é o fator prognóstico mais importante.Deve ser introduzida terapia antifúngica associada ao debridamento cirúrgico. O risco de contaminação fúngica pode ser minimizado com cuidados de exposição do paciente neutropênico e com a implantação de unidades de internação com filtros HEPA.

Acute invasive fungal sinusitis is a disease that affects primarily immunocompromised patients causing high morbidity and mortality. We present a case of acute invasive aspergillosis in an immunocompromised patient that evolved with a good outcome resulting from early diagnosis and rapid intervention. Case report: a thirteen year-old girl diagnosed with acute myeloid leukemia admitted for febrile neutropenia after chemotherapy. Sinus CT revealed partial opacification of left sinuses. Blood tests showed a number of total leukocytes, count 880. In the nasal endoscopy, we found a left middle turbinate ischemia. Patient was taken to surgical emergency and submitted to extensive debridement. Pathological examination revealed areas of ischemic necrosis and angioinvasive fungal structures consistent with aspergillosis. Examination confirmed Aspergillus flavus; Voriconazole was introduced. Follow-up was performed by pediatric oncology; cancer kept in remission without fungal recurrence. The incidence of fungal infections of the nose and paranasal sinuses has been increasing in recent decades. Fever unresponsive to antibiotics,with or without nasal symptoms, is the most suggestive sign of this disease that typically affects the middle turbinate. Nasal endoscopy is its most effective diagnostic test. There are no pathognomonic signs using CT, which serves moreas a post-treatment tool for differential diagnosis, planning, and monitoring.Concomitant correction of any underlying metabolic or immune disorder is the most important prognostic factor. Antifungal therapy should be introduced and associated with surgical debridement. Risk of fungal contamination can be minimized with care of exposure of neutropenic patients, and with the implantation of hospitalization units with HEPA filters.

Características dos pacientes adolescentes e adultos com fibrose cística do Hospital de Clínicas de Porto Alegre / Characteristics of adolescent and adult patients with cystic fibrosis of Hospital de Clínicas de Porto Alegre

Introdução: A expectativa de vida dos pacientes com fibrose cística (FC) tem aumentado progressivamente nas últimas décadas. Objetivos: Determinar as características clínicas dos pacientes com FC em acompanhamento com a equipe de adultos do Hospital de Clínicas de Porto Alegre (HCPA) e determinar quais características estão associadas com a gravidade da obstrução do fluxo aéreo. Pacientes e métodos: Estudo transversal dos pacientes com FC (idade ≥ 16 anos) em acompanhamento na equipe de adultos do HCPA. Foram coletados dados demográficos, clínicos, nutricionais, função pulmonar, testes laboratoriais, achados radiológicos e microbiologia do escarro. Resultados: Trinta e nove pacientes (21 masculinos/18 femininos) consultavam com a equipe de adultos em 2003. A idade mediana foi 22,3 anos e a freqüência da raça branca, 97,4%. Análise genética foi realizada em 27 pacientes. Sete pacientes (25,9%) foram homozigotos para mutação delta F508 e 10 (37%) tinham apenas uma mutação delta F508. O escore clínico de Shwachman-Kulczycki mediano foi 80, o escore de Brasfield mediano foi 14 e o VEF1 médio foi 51,1% do previsto. Quatorze pacientes tinham distúrbio ventilatório obstrutivo (DVO) ausente ou leve, 11 tinham DVO moderado e 14 tinham DVO grave. A gravidade do DVO associou-se com a saturação de oxigênio, escore de dispnéia, escore clínico e escore radiológico. Conclusão: Descrevemos um grupo jovem de pacientes adultos com FC com doença pulmonar moderada a grave, mas com performance boa a excelente na sua atividade diária. A obstrução do fluxo aéreo associou-se à saturação de oxigênio, escore de dispnéia, escore clínico e escore radiológico (AU)

Introduction: Life expectancy of cystic fibrosis (CF) patients has been greatly increased over past decades. Objective: To determine the clinical characteristics of CF patients attending the adult team of Hospital de Clínicas de Porto Alegre (HCPA) and to determine which characteristics are associated with the severity of the air-flow obstruction. Patients and methods: The study comprised a cross-sectional survey of CF patients (age ≥16 years) attending to the adult team of HCPA. Data collected included patient demographics, clinical data, nutritional status, pulmonary function, laboratory tests, radiological findings and sputum microbiology. Results: Thirty-nine patients (21 male/18 female) were attending to the adult team during 2003. The median age was 22.3 years and the frequency of white race was 97.4%. Genetic analysis was performed in 27 patients. Seven patients (25.9%) were homozygous for delta F508 mutation and 10 (37%) had one delta F508 mutation. The median Shwachman-Kulczycki clinical score was 80, the median Brasfield score was 14 and the mean FEV1 was 51.1% predicted. Fourteen patients had normal or mild obstructive ventilatory disorder (OVD), 11 patients had moderate OVD and 14 patients had severe OVD. The severity of the OVD was associated with oxygen saturation, dyspnea score, clinical score and radiographic score. Conclusion: We describe a young group of CF adults with moderate to severe pulmonary disease, but with a good to excellent performance in their daily activity. The air-flow obstruction was associated with oxygen saturation, dyspnea score, clinical score and radiographic score (AU)