Sinonasal alterations in computed tomography scans in cystic fibrosis: a literature review of observational studies

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

doi:10.1002/alr.21266

Cited by 20 publications

(16 citation statements)

References 35 publications

(78 reference statements)

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“…11,12 The challenge, however, is determining which patients have clinically relevant sinusitis, as there is a disconnect between symptoms and objective findings of sinusitis in CF patients. 2,13,14 CFQ-R 14+ scores were also statistically worse in the exacerbation cohort compared with the baseline cohort. Factors such as physical functioning, emotion, treatment burden, and respiratory symptoms are understandably worse for exacerbation patients, as these are likely acutely affected.…”

Section: Discussionmentioning

confidence: 87%

“…This indicates that, on average, CF patients have worse SNOT‐22 scores than non‐CF, non‐CRS patients, even if they have not been previously diagnosed with CRS . The challenge, however, is determining which patients have clinically relevant sinusitis, as there is a disconnect between symptoms and objective findings of sinusitis in CF patients …”

Section: Discussionmentioning

confidence: 99%

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Sinonasal quality‐of‐life declines in cystic fibrosis patients with pulmonary exacerbations

Safi

DiMango

Keating

et al. 2019

Int Forum Allergy Rhinol

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Background In cystic fibrosis (CF), the relationship between chronic rhinosinusitis (CRS) and pulmonary disease is poorly understood. The purpose of this study was to evaluate the relationship between scores on the 22‐item Sino‐Nasal Outcome Test (SNOT‐22) and CF Questionnaire―revised for adolescents and adults over 14 (CFQ‐R 14+), and pulmonary function tests in 2 cohorts of CF patients: those at their baseline health and those with a pulmonary exacerbation. Methods Patients >18 years old seen in a Cystic Fibrosis Foundation‒accredited clinic completed the SNOT‐22 and CFQ‐R 14+ instruments. Patients presenting for routine care represented the baseline cohort. Patients diagnosed with a pulmonary exacerbation represented the exacerbation cohort. Average SNOT‐22 and CFQ‐R 14+ scores for both groups were compared using a 2‐sample t test, and correlation coefficient was calculated. Results One hundred three patients were enrolled over 3 months (30 exacerbations and 73 baseline). Patients’ mean age was 32 years (56% female and 44% male). Average SNOT‐22 and CFQ‐R 14+ scores were significantly worse for exacerbation patients (p = 0.001 and p = 0.0003, respectively). Percent predicted forced expiratory volume in 1 second and forced vital capacity were both higher for baseline patients (p = 0.002 and p = 0.001, respectively). Average SNOT‐22 score for all patients was worse than the average score for non‐CF, non‐CRS patients. Conclusion CF patients with pulmonary exacerbations have worse SNOT‐22 and CFQ‐R 14+ scores than CF patients at their baseline health. This finding suggests a temporal relationship between sinonasal and pulmonary quality of life, and that worsening of both is associated with reduced pulmonary function.

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Section: Discussionmentioning

confidence: 87%

Section: Discussionmentioning

confidence: 99%

Sinonasal quality‐of‐life declines in cystic fibrosis patients with pulmonary exacerbations

Safi

DiMango

Keating

et al. 2019

Int Forum Allergy Rhinol

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“…[29]. In der Bildgebung finden sich bei fast allen Mukoviszidose-Patienten Veränderungen, die mit einer chronischen Rhinosinusitis vereinbar sind [30]. Viele Patienten leiden unter nasaler Kongestion, Gesichtsschmerz und verstärktem Sekretfluss [31].…”

Section: Genetik Der Mukoviszidoseunclassified

Einführung des deutschlandweiten Neugeborenenscreenings für Mukoviszidose

Heinemann

Hentschel

Becker

et al. 2016

LaboratoriumsMedizin

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ZusammenfassungDie Mukoviszidose oder Cystische Fibrose (CF) ist eine autosomal rezessiv vererbte Stoffwechselerkrankung und mit einer regional schwankenden Inzidenz von ca. 1:3.300–1:5.800 eine der häufigsten angeborenen Stoffwechselerkrankungen in Deutschland. Durch eine mutationsbedingte verminderte oder fehlende Funktion von Chloridkanälen kommt es hier zu einer Veränderung der Sekretzusammensetzung aller exokrinen Drüsen. Die mittlere Lebenserwartung von Mukoviszidose-Patienten konnte durch verbesserte Behandlungsstrategien auf mittlerweile über 40 Jahre erheblich gesteigert werden. Es hat sich dabei gezeigt, dass eine frühzeitige Diagnosestellung einen positiven Einfluss auf Krankheitsverlauf, Lebensqualität und Lebenserwartung der betroffenen Patienten hat. Diese Erkenntnis führte in den letzten 10 Jahren europaweit zur Aufnahme der Mukoviszidose in regionale und nationale Neugeborenenscreening-Programme. Mit dem Beschluss des Gemeinsamen Bundesausschusses zur Einführung des Mukoviszidosescreenings im August 2015 wurde Mukoviszidose nun auch in Deutschland als weitere Zielkrankheit in die Kinderrichtlinien aufgenommen und ist nach Veröffentlichung im Bundesanzeiger somit bundeseinheitlich als Bestandteil des deutschen Neugeborenenscreening-Programms vorgeschrieben. Das Procedere beinhaltet ein Stufenscreening mit der Kombination von Immunreaktivem Trypsin (IRT) und Pankreatitis-assoziiertem Protein (PAP) mit zusätzlicher Mutationsanalytik. Dank einer deutschlandweit früheren Diagnosestellung wird ein verbessertes Langzeitoutcome von Mukoviszidose-Patienten erwartet.

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“…Almost all patients with cystic fibrosis have radiological and sinus changes in their nose, however, these changes are not associated with disease severity (10,11). Nasal and paranasal sinus opacity, medialization of the lateral nasal wall, demineralization of uncinate excrescence, hypoplasia, and agenesis of frontal and sphenoid sinuses are among the most common CT findings in these patients (12). With respect to cystic fibrosis, few studies have been carried out in Iran.…”

Section: Introductionmentioning

confidence: 99%

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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Sinonasal alterations in computed tomography scans in cystic fibrosis: a literature review of observational studies

Abstract: There are few studies in the CF adult population regarding sinonasal CT alterations. Many studies report specific pathological features in CF upper airways that could help in the diagnosis of doubtful cases.

Cited by 20 publications

References 35 publications

Sinonasal quality‐of‐life declines in cystic fibrosis patients with pulmonary exacerbations

Sinonasal quality‐of‐life declines in cystic fibrosis patients with pulmonary exacerbations

Einführung des deutschlandweiten Neugeborenenscreenings für Mukoviszidose

Sinonasal Manifestations in Children with Cystic Fibrosis

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