Abstract
Background
The timely and appropriate monitoring of pulmonary status is of utmost importance for patients with cystic fibrosis (CF). Computed tomography (CT) has been used in clinical and research settings for tracking lung involvement in CF patients. However, as CT delivers a considerable amount of radiation, its sequential use in CF patients remains a concern. The application of CT, therefore, should take into account its potential risks. This review aims to understand whether and to what extent the CT findings correlate with the findings from other monitoring tools in CF lung disease.
Data sources
PubMed was searched for articles about the correlation of chest CT findings with spirometric indices and with lung clearance index in children and adolescents with CF. The most relevant articles were reviewed and are presented herein.
Results
Most studies have shown that forced expiratory volume in the first second (FEV1) and other spirometric indices correlate moderately with CT structural lung damage. However, at the individual level, there were patients with FEV1 within the normal range and abnormal CT and vice versa. Furthermore, longitudinal studies have indicated that the deterioration of structural lung damage does not occur in parallel with the progression of lung function. Lung clearance index is a better predictor of CT findings.
Conclusions
In general, the existing studies do not support the use of lung function tests as surrogates of chest CT.
Similar content being viewed by others
References
Lynch DA, Brasch RC, Hardy KA, Webb WR. Pediatric pulmonary disease: assessment with high-resolution ultrafast CT. Radiology. 1990;176:243–8.
Hansell DM, Strickland B. High-resolution computed tomography in pulmonary cystic fibrosis. Br J Radiol. 1989;62:1–5.
de Jong PA, Mayo JR, Golmohammadi K, Nakano Y, Lequin MH, Tiddens HA, et al. Estimation of cancer mortality associated with repetitive computed tomography scanning. Am J Respir Crit Care Med. 2006;173:199–203.
Loeve M, van Hal PT, Robinson P, de Jong PA, Lequin MH, Hop WC, et al. The spectrum of structural abnormalities on CT scans from patients with CF with severe advanced lung disease. Thorax. 2009;64:876–82.
Calder AD, Bush A, Brody AS, Owens CM. Scoring of chest CT in children with cystic fibrosis: state of the art. Pediatr Radiol. 2014;44:1496–506.
Bhalla M, Turcios N, Aponte V, Jenkins M, Leitman BS, McCauley DI, et al. Cystic fibrosis: scoring system with thin-section CT. Radiology. 1991;179:783–8.
Santamaria F, Grillo G, Guidi G, Rotondo A, Raia V, de Ritis G, et al. Cystic fibrosis: when should high-resolution computed tomography of the chest Be obtained? Pediatrics. 1998;101:908–13.
Helbich TH, Heinz-Peer G, Eichler I, Wunderbaldinger P, Gotz M, Wojnarowski C, et al. Cystic fibrosis: CT assessment of lung involvement in children and adults. Radiology. 1999;213:537–44.
Brody AS, Klein JS, Molina PL, Quan J, Bean JA, Wilmott RW. High-resolution computed tomography in young patients with cystic fibrosis: distribution of abnormalities and correlation with pulmonary function tests. J Pediatr. 2004;145:32–8.
de Jong PA, Ottink MD, Robben SG, Lequin MH, Hop WC, Hendriks JJ, et al. Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements. Radiology. 2004;231:434–9.
Stick SM, Brennan S, Murray C, Douglas T, von Ungern-Sternberg BS, Garratt LW, et al. Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening. J Pediatr. 2009;155:e621.
Rosenow T, Oudraad MC, Murray CP, Turkovic L, Kuo W, de Bruijne M, et al. PRAGMA-CF. A quantitative structural lung disease computed tomography outcome in young children with cystic fibrosis. Am J Respir Crit Care Med. 2015;191:1158–65.
Aziz ZA, Davies JC, Alton EW, Wells AU, Geddes DM, Hansell DM. Computed tomography and cystic fibrosis: promises and problems. Thorax. 2007;62:181–6.
Pereira FF, Ibiapina Cda C, Alvim CG, Camargos PA, Figueiredo R, Pedrosa JF. Correlation between Bhalla score and spirometry in children and adolescents with cystic fibrosis. Rev Assoc Med Bras. 1992;2014:216–21.
Rybacka A, Gozdzik-Spychalska J, Rybacki A, Piorunek T, Batura-Gabryel H, Karmelita-Katulska K. Congruence between pulmonary function and computed tomography imaging assessment of cystic fibrosis severity. Adv Exp Med Biol. 2018;1114:67–766.
Dakin CJ, Pereira JK, Henry RL, Wang H, Morton JR. Relationship between sputum inflammatory markers, lung function, and lung pathology on high-resolution computed tomography in children with cystic fibrosis. Pediatr Pulmonol. 2002;33:475–82.
Oikonomou A, Manavis J, Karagianni P, Tsanakas J, Wells AU, Hansell DM, et al. Loss of FEV1 in cystic fibrosis: correlation with HRCT features. Eur Radiol. 2002;12:2229–35.
Demirkazik FB, Ariyurek OM, Ozcelik U, Gocmen A, Hassanabad HK, Kiper N. High resolution CT in children with cystic fibrosis: correlation with pulmonary functions and radiographic scores. Eur J Radiol. 2001;37:54–9.
Loeve M, Rosenow T, Gorbunova V, Hop WC, Tiddens HA, de Bruijne M. Reversibility of trapped air on chest computed tomography in cystic fibrosis patients. Eur J Radiol. 2015;84:1184–90.
de Jong PA, Nakano Y, Lequin MH, Mayo JR, Woods R, Pare PD, et al. Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis. Eur Respir J. 2004;23:93–7.
de Jong PA, Lindblad A, Rubin L, Hop WC, de Jongste JC, Brink M, et al. Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax. 2006;61:80–5.
Sanders DB, Li Z, Brody AS, Farrell PM. Chest computed tomography scores of severity are associated with future lung disease progression in children with cystic fibrosis. Am J Respir Crit Care Med. 2011;184:816–21.
Cademartiri F, Luccichenti G, Palumbo AA, Maffei E, Pisi G, Zompatori M, et al. Predictive value of chest CT in patients with cystic fibrosis: a single-center 10-year experience. AJR Am J Roentgenol. 2008;190:1475–80.
Svedberg M, Gustafsson P, Tiddens H, Imberg H, Pivodic A, Lindblad A. Risk factors for progression of structural lung disease in school-age children with cystic fibrosis. J Cyst Fibros. 2020;19:910–6.
Turkovic L, Caudri D, Rosenow T, Breuer O, Murray C, Tiddens H, et al. Structural determinants of long term functional outcomes in young children with cystic fibrosis. Eur Respir J. 2020;55:5.
Robinson TE, Goris ML, Moss RB, Tian L, Kan P, Yilma M, et al. Mucus plugging, air trapping, and bronchiectasis are important outcome measures in assessing progressive childhood cystic fibrosis lung disease. Pediatr Pulmonol. 2020;55:929–38.
Tiddens HA. Detecting early structural lung damage in cystic fibrosis. Pediatr Pulmonol. 2002;34:228–31.
Quanjer PH, Stanojevic S, Cole TJ, Baur X, Hall GL, Culver BH, et al. Multi-ethnic reference values for spirometry for the 3–95-yr age range: the global lung function 2012 equations. Eur Respir J. 2012;40:1324–43.
Quanjer PH, Weiner DJ, Pretto JJ, Brazzale DJ, Boros PW. Measurement of FEF25-75% and FEF75% does not contribute to clinical decision-making. Eur Respir J. 2014;43:1051–8.
Gibson RL, Burns JL, Ramsey BW. Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med. 2003;168:918–51.
Robinson PD, Lindblad A, Gustafsson PM. Comparison of the utility of multiple breath inert gas washout parameters in cystic fibrosis. Thorax. 2010;65:659.
Fretzayas A, Douros K, Moustaki M, Loukou I. Applications of lung clearance index in monitoring children with cystic fibrosis. World J Clin Pediatr. 2019;8:15–22.
Gustafsson PM, De Jong PA, Tiddens HA, Lindblad A. Multiple-breath inert gas washout and spirometry versus structural lung disease in cystic fibrosis. Thorax. 2008;63:129–34.
Ellemunter H, Fuchs SI, Unsinn KM, Freund MC, Waltner-Romen M, Steinkamp G, et al. Sensitivity of Lung Clearance Index and chest computed tomography in early CF lung disease. Respir Med. 2010;104:1834–42.
Fuchs SI, Gappa M, Eder J, Unsinn KM, Steinkamp G, Ellemunter H. Tracking lung clearance index and chest CT in mild cystic fibrosis lung disease over a period of three years. Respir Med. 2014;108:865–74.
Yammine S, Ramsey KA, Skoric B, King L, Latzin P, Rosenow T, et al. Single-breath washout and association with structural lung disease in children with cystic fibrosis. Pediatr Pulmonol. 2019;54:587–94.
Hall GL, Logie KM, Parsons F, Schulzke SM, Nolan G, Murray C, et al. Air trapping on chest CT is associated with worse ventilation distribution in infants with cystic fibrosis diagnosed following newborn screening. PLoS ONE. 2011;6:e23932.
Ramsey KA, Rosenow T, Turkovic L, Skoric B, Banton G, Adams AM, et al. Lung clearance index and structural lung disease on computed tomography in early cystic fibrosis. Am J Respir Crit Care Med. 2016;193:60–7.
Ferris H, Twomey M, Moloney F, O’Neill SB, Murphy K, O'Connor OJ, et al. Computed tomography dose optimisation in cystic fibrosis: a review. World J Radiol. 2016;8:331–41.
Kuo W, Ciet P, Tiddens HA, Zhang W, Guillerman RP, van Straten M. Monitoring cystic fibrosis lung disease by computed tomography. Radiation risk in perspective. Am J Respir Crit Care Med. 2014;189:1328–36.
Funding
No financial or non-financial benefits have been received or will be received from any party related directly or indirectly to the subject of this article.
Author information
Authors and Affiliations
Contributions
Dr. Loukou and Dr. Moustaki wrote the initial draft. Prof Fretzayas and Assoc. Prof Douros critically revised the manuscript. All authors approved the final version of the manuscript.
Corresponding author
Ethics declarations
Ethical approval
Ethical approval non-applicable.
Conflict of interest
No conflict of interest to be declared.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Fretzayas, A., Loukou, I., Moustaki, M. et al. Correlation of computed tomography findings and lung function in children and adolescents with cystic fibrosis. World J Pediatr 17, 221–226 (2021). https://doi.org/10.1007/s12519-020-00388-8
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12519-020-00388-8