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

THIEME 434 Original Research Upper Airway Findings and Markers of Lung Disease Progression in Patients with Cystic Fibrosis Luciane Mazzini Steffen1 Leonardo Araujo Pinto3 Luise Sgarabotto Pezzin1 1 Department of Otorhinolaringology, Pontifícia Universidade Católica do Rio Grande do Sul, Porto Alegre, RS, Brazil 2 Department of Pediatrics, Pontifícia Universidade Católica do Rio Grande do Sul, Porto Alegre, RS, Brazil 3 Department of Pediatric Pneumology, Pontifícia Universidade Católica do Rio Grande do Sul, Porto Alegre, RS, Brazil Natassia Sulis2 Nedio Steffen1 Address for correspondence Luise Sgarabotto Pezzin, MD, Department of Otorhinolaringology, Pontifícia Universidade Católica do Rio Grande do Sul, Porto Alegre, RS, 90619900, Brazil (e-mail: ). Int Arch Otorhinolaryngol 2020;24(4):e434–e437. Abstract Keywords ► cystic fibrosis ► respiratory tract infections ► nasal polyps ► bacteriology 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. Introduction Cystic fibrosis (CF) is an autosomal recessive disorder, caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). Cystic fibrosis may have various phenotypic expressions in upper airways and lungs. It affects different systems and may cause life-limiting complications.1–4 received February 20, 2019 accepted October 20, 2019 DOI https://doi.org/ 10.1055/s-0039-3402434. ISSN 1809-9777. The incidence is
1 to 2,000 live births in Caucasians, being less common in African-Americans (1:17,000) and in Eastern countries (1:90,000). The number of registered patients with CF has been growing annually worldwide. This is associated with the introduction of the national or local programs of neonatal screening.5 Copyright © 2020 by Thieme Revinter Publicações Ltda, Rio de Janeiro, Brazil Upper Airway Findings and Markers of Lung Disease Cystic fibrosis is associated with the production of thick secretions, and obstruction exocrine glands in the affected organs.6–8 The respiratory system is mainly responsible for the severity of the disease and the highest morbidity and mortality. The upper airways exhibit a range of conditions such as sinusitis, nasal polyps and mucocele. Lung disease in CF is characterized by respiratory infection and colonization by bacteria that lead to irreversible tissue damage. The microorganisms more frequently involved are: Staphylococcus aureus (SA), Pseudomonas aeruginosa (PA), Pseudomonas cepacea (PC) and Stenotrophomonas maltophilia (SM).9–12 The relationship between the bacteria of the upper or lower respiratory tract is not yet completely understood. Some centers recommend aggressive treatment to infection as well as colonization of the sinuses, especially if there is presence of PA or PC.13–17 To monitor the progression of the disease, some parameters have been currently used as body mass index ([BMI] absolute value or percentile), forced expiratory volume in the first second (FEV1%) and the Shwachman-Kulczycki (SK) score.18 The present study aims to describe the most frequent nasal findings and pathogens in patients with cystic fibrosis. In addition, we investigated the association between the findings of the upper respiratory tract (URT) and markers of lung disease progression. Method This was a retrospective cross-sectional study, involving patients with confirmed diagnosis of CF in accordance with the criteria of the CF Foundation. Patients were followed-up in the CF Clinic at a University Hospital. All of the patients underwent otorhinolaryngological evaluation between 2015 and 2017. The upper airway evaluation included, among the tests, the collection of nasal material through a swab and videonasofibroscopy. All of the patients were evaluated in the outpatient Clinic of Otorhinolaryngology of the same Hospital. The collection was obtained through the nasal swab. (Stuart Swabs, Absorve/CRAL, Sao Paulo/SP, Brazil). Nasal speculum was sterilized in autoclave for opening the nasal cavity and then the swab was inserted into the nostril to the posterior portion (posterior nasal apertures) and directed to the middle meatus. This gathering was preceded by the examination of videonasofibroscopy to prevent contamination of the URT. The material was sent immediately to the microbiology laboratory. The videonasofibroscopy has been performed using a Mashida ENT 30 PIII flexible fiber-optic nasopharyngolaryngoscope with 3.2 mm in diameter (Mashida, Madrid, Spain), coupled to a Toshiba video camera model IKM41A (Toshiba, Tokyo, Japan) and transmitted to a LG TV model 14FK3RB (LG, Seoul, South Korea). A halogen light source of 250 W was used. The present survey allowed the direct assessment of the nasal cavity status. The parameters observed in the nasal endoscopy were: presence, absence and aspect of the meatus secretion, the presence or absence of pharyngeal tonsil and nasal polyps. The results of the oropharyngeal/sputum collection were obtained from the elec- Pezzin et al. tronic patient record, respecting the maximum interval of 90 days, when they perform the routine reviews of multidisciplinary outpatient clinic (Pneumology, Pediatric Gastroenterology and Physical Therapy). Markers of disease severity and progression as FEV1%, BMI, and clinical SK score rating, in addition to the gene mutations, have also been extracted from the records of the patients. Ne (...truncated)