Chronic rhinosinusitis and nasal polyposis in cystic fibrosis: update on diagnosis and treatment

Kang, Suzie Hyeona; Dalcin, Paulo de Tarso Roth; Piltcher, Otávio Bejzman; Migliavacca, Raphaella de Oliveira

doi:10.1590/s1806-37132015000100009

Cited by 48 publications

(70 citation statements)

References 58 publications

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“…In the present study, the most common complaint in children was nasal congestion (61.7%). In addition, there was no relationship between symptoms and age, sex, height, and weight that was consistent with the most recent studies (9,17,18). In our study, 31.9% of nasal polyps were diagnosed using rigid endoscope examination.…”

Section: Discussionsupporting

confidence: 93%

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Sinonasal Manifestations in Children with Cystic Fibrosis

2019

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Background: Involvement of the upper respiratory tract and pathological changes in the nose and paranasal sinuses are common in patients with cystic fibrosis. Objectives: We aimed to identify sinonasal manifestations in Iranian children with cystic fibrosis. Methods: Forthy seven children with cystic fibrosis were enrolled and symptoms related to the nose and paranasal sinuses were recorded using a questionnaire. The patients' nasal and sinus CT scan were investigated in terms of findings related to cystic fibrosis. Results: The most common complaint was nasal congestion (61.7%). Ground glass opacity in the nasal cavity was found in 63.8% of the patients. Postnasal drip was observed in 36.2% of the patients. In nasal endoscopic examination, 15 (31.9%) patients had clear polyps in the nasal cavity. Conclusions: History, CT scans, and initial clinical examination (rhinorrhea in anterior rhinoscopy) does not allow accurate diagnosis of chronic rhino sinusitis or nasal polyps in children with cystic fibrosis.

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Section: Discussionsupporting

confidence: 93%

“…Many patients do not report Sino nasal symptoms. Involvement of the upper respiratory tract occurs as chronic rhino sinusitis and nasal polyps that can affect the quality of life in these patients (6)(7)(8)(9). Nasal polyps are the most common finding on physical examination.…”

Section: Introductionmentioning

confidence: 99%

Sinonasal Manifestations in Children with Cystic Fibrosis

2019

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“…It has been suggested that this is related to these patients lack of a “normal” baseline for comparison. Others have proposed that sinonasal symptoms are reported to be low because they are overshadowed by other systemic symptoms, such as significant pulmonary and/or gastrointestinal symptoms . Regardless of the exact reasoning as to why CF patients do not report a high level of sinonasal morbidity, the low symptom burden in these patients makes the assessment of response to treatment difficult.…”

Section: Discussionmentioning

confidence: 99%

Ivacaftor improves rhinologic, psychologic, and sleep‐related quality of life in G551D cystic fibrosis patients

McCormick

Cho

Lampkin

et al. 2018

Int Forum Allergy Rhinol

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Background Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator that improves pulmonary function in cystic fibrosis (CF) patients with at least 1 copy of the G551D CFTR mutation. The purpose of this study is to evaluate the impact of ivacaftor on chronic rhinosinusitis (CRS) symptoms in this population. Methods The G551D Observational (GOAL) study was a multicenter prospective cohort study enrolling CF patients ≥6 years with at least 1 G551D mutation. Subjects were provided 20‐item Sino‐Nasal Outcome Test (SNOT‐20) questionnaires prior to ivacaftor therapy and at 1, 3, and 6 months afterward. The impact on rhinologic (R), psychological (P), sleep (S), and ear/facial (E) quality of life (QOL) domains was evaluated separately. Results Of 153 subjects, 129 (84%) completed all questionnaires. Typical baseline symptom burden was low (75% with scores <1) and degree of improvement (ie, reduced scores) was greater with higher baseline scores. SNOT‐20 decreased, reflecting improvement, at all follow‐up intervals (1 month: [mean change ± standard deviation] –0.25 ± 0.53, p < 0.01; 3 months; –0.29 ± 0.58, p < 0.01; 6 months: –0.21 ± 0.58, p = 0.02), but less than the prespecified minimal clinically important difference (0.8). Significant improvement was observed at 1, 3, and 6 months in the R domain (1 month: –0.24, p < 0.01; 3 months: –0.34, p < 0.01; 6 months: –0.25, p < 0.01) and P domain (1 month: –0.25, p < 0.01; 3 months: –0.32, p < 0.01; 6 months: –0.26, p < 0.01), and 1 and 3 months in the S domain (1 months: –0.35, p < 0.01; 3 months: –0.32, p < 0.01; 6 months: –0.18, p = 0.07). There was no improvement in the E domain at any time point. Conclusion Ivacaftor improves QOL in the R, P, and S domains in G551D CF patients, although QOL instruments validated for CRS may not translate well to CF CRS patients because symptom burden was surprisingly low.

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“…In the 6 studies included in our review, the delivery of dornase was via different inhalational devices and the impact this may have had on outcomes and the comparison of those outcomes is unknown. The PARI‐SINUS™ device delivers vibrating aerosol particles with pressure gradients in nasal and paranasal cavities, allowing for aerosol diffusion into passively ventilated areas . The standard nasal device does not sufficiently deliver drugs to the nasal cavity and goes mainly to the posterior nasopharynx.…”

Section: Discussionmentioning

confidence: 99%