Upper Airway Findings and Markers of Lung Disease Progression in Patients with Cystic Fibrosis

Abstract

Introduction Cystic fibrosis (CF) is a genetic disease that limits the quality of life mainly due to respiratory symptoms. The relationship between findings of the upper airways and CF lung disease is not yet completely understood.

Objective The aim of the present study is to describe the most frequent nasal findings and pathogens in patients with CF and investigate the association between the findings of the upper respiratory tract and markers of lung disease progression.

Methods Retrospective study in patients with CF from the Pediatric Pulmonology Department who underwent otorhinolaryngological evaluation between 2015 and 2017. Nasal endoscopy and nasal swab collection were part of the evaluation. The severity markers used were: percentage of predicted forced expiratory volume in the first second (FEV1%), body mass index (BMI) and the Shwachman-Kulczycki (SK) clinical score.

Results A total of 48 patients with CF were included. The mean of the predicted percentage of FEV1% was 83.36 ± 30.04. The average 14 and SK score 89.11 ± 10.50. The bacteriology of the nasal swab was positive in 27 (54.1%) patients. Staphylococcus aureus was positive in 18 patients, Pseudomonas aeruginosa in 5, Pseudomonas cepacea in 3 and Stenotrophomonas maltophila in 1 patient. Nasal polyps were found in nine participants. Nasal polyps were found in nine participants and were associated with lower SK score.

Conclusion The pathogens found in the upper airway were, in order: S. aureus, P. aeruginosa, P. cepacea e S. maltophila. The presence of polyps in the nasal cavity showed statistical significance and appears to have association with the prognostic factor measured by the SK score.

Keywords

cystic fibrosis - respiratory tract infections - nasal polyps - bacteriology

Publikationsverlauf

Eingereicht: 20. Februar 2019

Angenommen: 20. Oktober 2019

Artikel online veröffentlicht:
11. März 2020

• References

• 1 Ratjen F, Döring G. Cystic fibrosis. Lancet 2003; 361 (9358): 681-689
• 2 Castellani C, Assael BM. Cystic fibrosis: a clinical view. Cell Mol Life Sci 2017; 74 (01) 129-140
• 3 Flume PA, O'Sullivan BP, Robinson KA. , et al; Cystic Fibrosis Foundation, Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2007; 176 (10) 957-969
• 4 Weber SAT, Iyomasa RM, Correa CC, Florentino WNM, Ferrari GF. Nasal polyposis in cystic fibrosis: follow-up of children and adolescents for a 3-year period. Rev Bras Otorrinolaringol (Engl Ed) 2017; 83 (06) 677-682
• 5 Cística GBdEeF. Relatório do Registro Brasileiro de Fibrose Cística 2014. 2014
• 6 Plant BJ, Goss CH, Plant WD, Bell SC. Management of comorbidities in older patients with cystic fibrosis. Lancet Respir Med 2013; 1 (02) 164-174
• 7 Aanæs K. Bacterial sinusitis can be a focus for initial lung colonisation and chronic lung infection in patients with cystic fibrosis. J Cyst Fibros 2013; 12 (Suppl. 02) S1-S20
• 8 Ribeiro JD, Fischer GB. Doenças pulmonares obstrutivas crônicas na criança. J Pediatr 2015; 91: S11-S25
• 9 Rosenfeld M, Emerson J, Accurso F. et al. Diagnostic accuracy of oropharyngeal cultures in infants and young children with cystic fibrosis. Pediatr Pulmonol 1999; 28 (05) 321-328
• 10 Ribeiro JD, Ribeiro MA, Ribeiro AF. Controvérsias na fibrose cística--do pediatra ao especialista. J Pediatr (Rio J) 2002; 78 (Suppl. 02) S171-S186
• 11 Freitas MR, Vasconcelos DN, Freitas AE, Maia Filho JH, Castro e Silva Cd. Nasal endoscopic and CT scan alterations of the paranasal sinuses as predictors of severity in patients with cystic fibrosis. Rev Bras Otorrinolaringol (Engl Ed) 2013; 79 (04) 480-486
• 12 Rivas Caldas R, Boisramé S. Upper aero-digestive contamination by Pseudomonas aeruginosa and implications in Cystic Fibrosis. J Cyst Fibros 2015; 14 (01) 6-15
• 13 Mainz JG, Naehrlich L, Schien M. et al. Concordant genotype of upper and lower airways P aeruginosa and S aureus isolates in cystic fibrosis. Thorax 2009; 64 (06) 535-540
• 14 Robertson JM, Friedman EM, Rubin BK. Nasal and sinus disease in cystic fibrosis. Paediatr Respir Rev 2008; 9 (03) 213-219
• 15 Umetsu DT, Moss RB, King VV, Lewiston NJ. Sinus disease in patients with severe cystic fibrosis: relation to pulmonary exacerbation. Lancet 1990; 335 (8697): 1077-1078
• 16 Muhlebach MS, Miller MB, Moore C, Wedd JP, Drake AF, Leigh MW. Are lower airway or throat cultures predictive of sinus bacteriology in cystic fibrosis?. Pediatr Pulmonol 2006; 41 (05) 445-451
• 17 Hansen SK, Rau MH, Johansen HK. et al. Evolution and diversification of Pseudomonas aeruginosa in the paranasal sinuses of cystic fibrosis children have implications for chronic lung infection. ISME J 2012; 6 (01) 31-45
• 18 Rudkjøbing VB, Aanaes K, Wolff TY, von Buchwald C, Johansen HK, Thomsen TR. An exploratory study of microbial diversity in sinus infections of cystic fibrosis patients by molecular methods. J Cyst Fibros 2014; 13 (06) 645-652
• 19 Ratjen F, Walter H, Haug M, Meisner C, Grasemann H, Döring G. Diagnostic value of serum antibodies in early Pseudomonas aeruginosa infection in cystic fibrosis patients. Pediatr Pulmonol 2007; 42 (03) 249-255
• 20 da Silva Filho LV, Tateno AF, Martins KM. et al. The combination of PCR and serology increases the diagnosis of Pseudomonas aeruginosa colonization/infection in cystic fibrosis. Pediatr Pulmonol 2007; 42 (10) 938-944
• 21 Ribeiro JD. Ribeiro MÂGdO, Ribeiro AF. Controvérsias na fibrose cística: do pediatra ao especialista. J Pediatr (Rio J) 2002; 78: 171-186
• 22 Gibson RL, Burns JL, Ramsey BW. Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med 2003; 168 (08) 918-951
• 23 Henriksson G, Westrin KM, Karpati F, Wikström AC, Stierna P, Hjelte L. Nasal polyps in cystic fibrosis: clinical endoscopic study with nasal lavage fluid analysis. Chest 2002; 121 (01) 40-47
• 24 Taylor CJ, McGaw J, Howden R, Duerden BI, Baxter PS. Bacterial reservoirs in cystic fibrosis. Arch Dis Child 1990; 65 (02) 175-177
• 25 Stollar F, Adde FV, Cunha MT, Leone C, Rodrigues JC. Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity. Clinics (São Paulo) 2011; 66 (06) 979-983
• 26 Bittencourt PH, Pimentel CSS, Bonfim BS, Almeida CD, Marostica PJC, de Souza ELS. Incidence and treatment of methicillin-resistant Staphylococcus aureus infection in cystic fibrosis patients: a cohort study. Pediatr Pulmonol 2016; 51: S40
• 27 Smyth AR, Bell SC, Bojcin S. , et al; European Cystic Fibrosis Society. European Cystic Fibrosis Society Standards of Care: Best Practice guidelines. J Cyst Fibros 2014; 13 (Suppl. 01) S23-S42