Document Type : original article


1 Taleghani Pediatric Hospital, Golestan Univesity of Medical Sciences, Gorgan, Iran

2 Taleghani Pediatric Hospital, Golestan University of Medical Sciences, Gorgan, Iran.

3 1. Golestan University of Medical Sciences, Gorgan, Iran. 2. Endocrinology and Metabolism Research Center, Endocrinology and Metabolism Clinical Sciences Institute, Tehran University of Medical Sciences, Tehran, Iran.

4 1.Golestan University of Medical Sciences, Gorgan, Iran 2.Endocrinology and Metabolism Research Instituite, Tehran University of Medical Sciences, Tehran, Iran

5 Taleghani Pediatric Hospital, Golestan Univesity of Medical Sciences, Gorgan,Iran


Background: Cystic Fibrosis (CF) is an autosomal recessive disease that has a negative impact on the quality of life in patients. Various methods for assessing lung function and airway obstruction in patients with CF can be used to diagnose and recognize the progression of the disease.
Methods: This is a cross-sectional study with a descriptive approach conducted on 45 CF patients aged 5-18 years. This study investigated the relation between pulmonary disease severity in lung computed tomography (CT) scans, and pulmonary function tests in cystic fibrosis patients referred to our paediatric hospital.
Results: The average age of the patients was 8.33 ± 2.95 years. 73.3% had bronchiectasis with different degrees. In oscillometry (IOS) evaluation, 8.9% had central obstruction, 28.9% had total airway obstruction, 37.8% had peripheral obstruction, and 24.4% had no obstruction. The spirometry results revealed that 53.3% of patients had normal spirometry, 35.6% showed a mild obstruction pattern, and 11.1% had a moderate obstruction. The results obtained from spirometry and IOS revealed that the total resistance in moderate airway obstruction was significantly higher than other resistances, and there was a significant increase in the severity of moderate obstruction of total airway resistance (P=0.022).
Conclusion: Our study showed that CT scan could reveal the complications of cystic fibrosis earlier than pulmonary tests, and it is a reliable tool in evaluating the progress of cystic fibrosis complications and should be considered in the follow-up of patients. Moreover, IOS can help interpret spirometry findings.


  1. 1.Bayfield KJ, Douglas TA, Rosenow T, Davies JC, Elborn SJ, Mall M, Paproki A, Ratjen F, Sly PD, Smyth AR, Stick S. Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis. Thorax. 2021 Dec 1; 76(12):1255-65.

    1. Kapnadak SG, Dimango E, Hadjiliadis D, Hempstead SE, Tallarico E, Pilewski JM, Faro A, Albright J, Benden C, Blair S, Dellon EP. Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease. Journal of Cystic Fibrosis. 2020 May 1; 19(3):344-54.
    2. Schlüter DK, Southern KW, Dryden C, Diggle P, Taylor-Robinson D. Impact of newborn screening on outcomes and social inequalities in cystic fibrosis: a UK CF registry-based study. Thorax. 2020 Feb 1; 75(2):123-31.
    3. Kaminsky DA, Simpson SJ, Berger KI, Calverley P, de Melo PL, Dandurand R, Dellacà RL, Farah CS, Farré R, Hall GL, Ioan I. Clinical significance and applications of oscillometry. European Respiratory Review. 2022 Mar 31; 31(163).
    4. Turkovic L, Caudri D, Rosenow T, Breuer O, Murray C, Tiddens HA, Ramanauskas F, Ranganathan SC, Hall GL, Stick SM. Structural determinants of long-term functional outcomes in young children with cystic fibrosis. European Respiratory Journal. 2020 May 1; 55(5).
    5. Sakarya A, Uyan ZS, Baydemir C, Anık Y, Erdem E, Gokdemir Y, Karadag B, Karakoc F, Ersu R. Evaluation of children with cystic fibrosis by impulse oscillometry when stable and at exacerbation. Paediatric pulmonology. 2016 Nov; 51(11):1151-87.
    6. Wamosy RM, Assumpção MS, Parazzi PL, Ribeiro JD, Roesler H, Schivinski CI. Reliability of impulse oscillometry parameters in healthy children and in children with cystic fibrosis. International Journal of Clinical Practice. 2021 Apr; 75(4):e13715.
    7. Moreau L, Crenesse D, Berthier F, Albertini M. Relationship between impulse oscillometry and spirometric indices in cystic fibrosis children. Acta Pædiatrica. 2009 Jun; 98(6):1019-23.
    8. de Oliveira Jorge PP, de Lima JH, e Silva DC, Medeiros D, Solé D, Wandalsen GF. Impulse oscillometry in the assessment of children's lung function. Allergologia et Immunopathologia. 2019 May 1; 47(3):295-302.
    9. Tomalak W, Radliński J, Pawlik J, Latawier W, Pogorzelski A. Impulse oscillometry vs. body plethysmography in assessing respiratory resistance in children. Paediatric Pulmonology. 2006 Jan; 41(1):50-4.
    10. Buchs C, Coutier L, Vrielynck S, Jubin V, Mainguy C, Reix P. An impulse oscillometry system is less efficient than spirometry in tracking lung function improvements after intravenous antibiotic therapy in paediatric patients with cystic fibrosis. Paediatric Pulmonology. 2015 Nov; 50(11):1073-81.
    11. van Straten M, Brody AS, Ernst C, Guillerman RP, Tiddens HA, Nagle SK. Guidance for computed tomography (CT) imaging of the lungs for patients with cystic fibrosis (CF) in research studies. Journal of Cystic Fibrosis. 2020 Mar 1; 19(2):176-83.
    12. Pollak M, Shaw M, Balkovec S, Wilson D, Kowalik K, Subbarao P, Ratjen F. Infant spirometry as a predictor of lung function at early childhood in cystic fibrosis patients. Journal of Cystic Fibrosis. 2021 Nov 1; 20(6):937-40.
    13. Tiddens HA. Chest computed tomography scans should be considered as a routine investigation in cystic fibrosis. Paediatric respiratory reviews. 2006 Sep 1; 7(3):202-8.
    14. de Jong PA, Nakano Y, Lequin MH, Mayo JR, Woods R, Pare PD, Tiddens HA. Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis. European Respiratory Journal. 2004 Jan 1; 23(1):93-7.
    15. de Jong PA, Lindblad A, Rubin L, Hop WC, de Jongste JC, Brink M, Tiddens HA. Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax. 2006 Jan 1; 61(1):80-5.
    16. Schaedel C, De Monestrol I, Hjelte L, Johannesson M, Kornfält R, Lindblad A, Strandvik B, Wahlgren L, Holmberg L. Predictors of deterioration of lung function in cystic fibrosis. Paediatric pulmonology. 2002 Jun; 33(6):483-91.
    17. Loeve M, van Hal PT, Robinson P, De Jong PA, Lequin MH, Hop WC, Williams TJ, Nossent GD, Tiddens HA. The spectrum of structural abnormalities on CT scans from patients with CF with severe advanced lung disease. Thorax. 2009 Oct 1; 64(10):876-82.
    18. Montella S, Santamaria F, Salvatore M, Pignata C, Maglione M, Iacotucci P, Mollica C. Assessment of chest high-field magnetic resonance imaging in children and young adults with noncystic fibrosis chronic lung disease: comparison to high-resolution computed tomography and correlation with pulmonary function. Investigative radiology. 2009 Sep 1; 44(9):532-8.
    19. Robinson TE, Leung AN, Northway WH, Blankenberg FG, Bloch DA, Oehlert JW, Al-Dabbagh H, Hubli S, Moss RB. Spirometer-triggered high-resolution computed tomography and pulmonary function measurements during an acute exacerbation in patients with cystic fibrosis. The Journal of pediatrics. 2001 Apr 1; 138(4):553-9.
    20. Ring AM, Carlens J, Bush A, Castillo-Corullón S, Fasola S, Gaboli MP, Griese M, Koucky V, La Grutta S, Lombardi E, Proesmans M. Pulmonary function testing in children's interstitial lung disease. European Respiratory Review. 2020 Sep 30; 29(157).
    21. Fretzayas A, Loukou I, Moustaki M, Douros K. Correlation of computed tomography findings and lung function in children and adolescents with cystic fibrosis. World Journal of Pediatrics. 2021 Jun; 17(3):221-6.
    22. Bortoluzzi CF, Pontello E, Pintani E, de Winter-de Groot KM, D'Orazio C, Assael BM, Hunink MM, Tiddens HA, Caudri D, Belessis Y, Bremont F. The impact of chest computed tomography and chest radiography on clinical management of cystic fibrosis lung disease. Journal of Cystic Fibrosis. 2020 Jul 1; 19(4):641-6.
    23. Gökçen DT, Polat SE, Hızal MG, Özsezen BK, Güneş A, Cinel G. The Evaluation of Lung Involvement in Patients with Cystic Fibrosis by Using Mediastinal Magnetic Resonance Imaging. Turk Toraks Dergisi. 2019 Apr 1; 20:55.