Predictors of sinonasal disease onset, progression, and severity in pediatric cystic fibrosis patients

About 50% of patients with cystic fibrosis (CF) have sinonasal complications, which include inferior turbinate hypertrophy (NTH) and/or nasal polyposis (NP), and different degrees of lung disease, which represents the main cause of mortality. Monitoring of sinonasal disease requires complex instrumental procedures, while monitoring of lung inflammation requires invasive collection of bronchoalveolar lavage fluid. The aim of this study was to investigate the associations between salivary cytokines levels and CF-related airway diseases. Salivary biochemical parameters and cytokines, i.e., interleukin-6 (IL-6), IL-8, and tumor necrosis factor alpha (TNF-α), were analyzed in resting saliva from healthy subjects and patients with CF. Patients with CF showed significantly higher levels of salivary chloride, IL-6, IL-8, and TNF-α and lower calcium levels than healthy subjects. Among patients with CF, IL-6 and IL-8 were significantly higher in patients with NTH, while TNF-α was significantly lower in patients with NP. A decreasing trend of TNF-α in patients with severe lung disease was also observed. On the other hand, we did not find significant correlation between cytokine levels and Pseudomonas aeruginosa or Stenotrophomonas maltophilia colonization. These preliminary results suggest that salivary IL-6 and IL-8 levels increase during the acute phase of sinonasal disease (i.e., NTH), while the end stages of pulmonary disease and sinonasal disease (i.e., NP) show decreased TNF-α levels.

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Cystic Fibrosis Diagnosed Using Indigenously Wrapped Sweating Technique: First Large-Scale Study Reporting Socio-Demographic, Clinical, and Laboratory Features among the Children in Bangladesh A Lower Middle Income Country

Global Pediatric Health ◽

10.1177/2333794x20967585 ◽

2020 ◽

Vol 7 ◽

pp. 2333794X2096758

Author(s):

ARM Luthful Kabir ◽

Sudipta Roy ◽

Rahat Bin Habib ◽

Kazi Selim Anwar ◽

Md. Abid Hossain Mollah ◽

...

Keyword(s):

Cystic Fibrosis ◽

Large Scale ◽

Tertiary Care ◽

Disease Onset ◽

Severe Malnutrition ◽

Sweat Test ◽

Cross Sectional ◽

Middle Income ◽

Sweat Chloride ◽

Radio Imaging

Due to lack of robust data on childhood cystic fibrosis (CF) in Bangladesh we sought to evaluate their clinico-epidemiology. A cross-sectional observation was conducted adopting CF-foundation consensus-panel-diagnostic criteria in 3 tertiary-care-hospitals in Bangladesh from 2000 to 2017. Clinically suspected 95 CF-cases were subjected to sweat-chloride testing using locally-developed a fast, cheap and effective indigenously body-wrapped sweating technique measured by US-Easy Lyte-automated microprocessor-controlled analyzer marking ≥60 mmol/L as positive. Mean-age of CF-cases at disease-onset was 16.9 ± 26.6 months that significantly differed with age-at-diagnosis ( P < .02). Pulmonary syndromes included chronic wet cough in 100%, respiratory distress in 90.5%, digital-clubbing in 78%, mucopurulent-sputum in 74%-cases, and crepitation in 82%. Radio-imaging revealed bronchiectasis in 60%, hyperinflation/peribronchial-thickening in 22% and, pan-sinusitis in 89%-cases. While 37% had history-of malabsorption, high-fecal-fat revealed in 53%-cases. Malnutrition prevailed as severe-underweight in 87%-cases and all CF-cases (100%) had high sweat-chloride (mean = 118 ± 53.34 mmol/L). Thus, children with pulmonary features coupled with severe malnutrition and associated radio-imaging bronchiectasis should be screened for CF with a fast, cheap and effective sweat test in resource poor settings.

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Respiratory Viral Detection in the Paranasal Sinuses of Patients with Cystic Fibrosis

American Journal of Rhinology and Allergy ◽

10.2500/ajra.2017.31.4422 ◽

2017 ◽

Vol 31 (2) ◽

pp. 105-108 ◽

Cited By ~ 2

Author(s):

Nicholas R. Rowan ◽

Eric W. Wang ◽

Alyssa Kanaan ◽

Nivedita Sahu ◽

John V. Williams ◽

...

Keyword(s):

Cystic Fibrosis ◽

Paranasal Sinuses ◽

Viral Infections ◽

Respiratory Viruses ◽

Clinical Severity ◽

Healthy Controls ◽

Viral Detection ◽

Respiratory Viral Infections ◽

Clinical Measures ◽

Sinonasal Disease

Background Pulmonary colonization with antibiotic-resistant organisms in patients with cystic fibrosis (CF) is often preceded by upper-airway infections. Although there is a well-described relationship between pulmonary respiratory viral infections and overall disease progression of CF, the pathogenicity of respiratory viral infections in the paranasal sinuses of patients with CF remains unknown. With recent advances in respiratory virus detection techniques, this study sought to detect the presence of respiratory viruses in the paranasal sinuses of patients with CF in comparison with healthy controls and to correlate the viral presence with clinical measures of sinonasal disease. Methods This prospective individual cohort study compared 24 patients with CF with 14 healthy controls. Basic demographics, clinical measures of disease and respiratory viral screens (commercial multiplex) obtained directly from the paranasal sinuses were compared between the two groups. Results Respiratory viruses were detected in 33% of patients with CF (8/24) compared with 0% of the healthy controls (0/14) (p = 0.017). Respiratory viruses were only detected during the winter months, and the most commonly identified were influenza A and human rhinovirus strains. There was no statistical difference in the 22-Item Sino-Nasal Outcome Test (SNOT-22) scores (p = 0.93) or modified Lund-Kennedy scores (p = 0.74) between patients with CF with a positive viral test and those without a positive result. Conclusions Respiratory viral detection is more commonly detected in the paranasal sinuses of patients with CF compared with healthy controls. Although respiratory viral presence did not correlate with a worse clinical severity of sinonasal disease, these findings may provide insight into the pathophysiology of CF and open new avenues for potential targeted therapy.

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Pediatric Sinusitis: The Role of Endoscopic Sinus Surgery in Cystic Fibrosis and Other Forms of Sinonasal Disease

Archives of Otolaryngology - Head and Neck Surgery ◽

10.1001/archotol.1991.01870160076013 ◽

1991 ◽

Vol 117 (4) ◽

pp. 422-426 ◽

Cited By ~ 54

Author(s):

J. K. Duplechain ◽

J. A. White ◽

R. H. Miller

Keyword(s):

Cystic Fibrosis ◽

Endoscopic Sinus Surgery ◽

Sinus Surgery ◽

Pediatric Sinusitis ◽

Sinonasal Disease

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Cystic fibrosis - general review on sinonasal complications and case report

Romanian Journal of Rhinology ◽

10.1515/rjr-2017-0004 ◽

2017 ◽

Vol 7 (25) ◽

pp. 33-37

Author(s):

Claudiu Manea ◽

Alina Diaconescu

Keyword(s):

Quality Of Life ◽

Cystic Fibrosis ◽

Case Report ◽

Life Expectancy ◽

Multiple Organ ◽

Lower Airway ◽

General Review ◽

Organ Systems ◽

Sinonasal Disease

Abstract An irreversible disease, cystic fibrosis (CF), is responsible for affecting multiple organ systems containing epithelia. It is well known that the sinonasal disease caused by CF has consequences for the incidence of the lower airway exacerbations, as well as affecting the quality of life of those patients. This review provides an update by evaluating the available literature regarding pathogenesis, management and treatment of cystic fibrosis patients. To gain a better view of the disease and obtain a higher life expectancy, further studies are needed.

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