Skip to main content
Top
Published in: European Radiology 4/2007

01-04-2007 | Pediatric

Assessment of lung disease in children with cystic fibrosis using hyperpolarized 3-Helium MRI: comparison with Shwachman score, Chrispin-Norman score and spirometry

Authors: Edwin J. R. van Beek, Catherine Hill, Neil Woodhouse, Stanislao Fichele, Sally Fleming, Bridget Howe, Sandra Bott, Jim M. Wild, Christopher J. Taylor

Published in: European Radiology | Issue 4/2007

Login to get access

Abstract

This study assesses the feasibility of hyperpolarized 3-Helium MRI in children with cystic fibrosis (CF) and correlates the findings with standard clinical parameters based on chest radiograph (CXR) and pulmonary function tests (PFT). An uncontrolled, observational study in eighteen children with cystic fibrosis aged 5 – 17 years (median 12.1 years), with different severity of disease was carried out. All subjects underwent routine clinical assessment including PFT and standard auxology; CXR was obtained and Shwachman and Chrispin-Norman scores calculated. Hyperpolarized 3-He magnetic resonance imaging (MRI) was carried out using a spin-exchange polarizer and a whole body 1.5 T scanner. Ventilation distribution images were obtained during a 21-second breath-hold and scored according to previously defined criteria. Spearman’s non-parametric correlations test was performed to assess for statistical significance at the p<0.05 level. The children tolerated the procedure well. No desaturation events were observed during 3-He MRI. A significant, albeit moderate, correlation was found between MRI score and FEV1% predicted (r=−0.41; p=0.047) and FVC% predicted (r=−0.42; p=0.04), while there were trends of correlations between Shwachman score and MRI score (r=−0.38; p=0.06) and Shwachman score and FEV1% predicted (r=0.39; p=0.055). The feasibility of hyperpolarized 3-He MRI in children with CF was demonstrated. MRI appears to be able to demonstrate functional lung changes, although correlations with routine clinical tests are only moderate to poor. This non-ionising radiation technique could be useful for monitoring lung disease and assessing therapy in this patient population.
Literature
2.
go back to reference Davis PB, Drumm M, Konstan MW (1996) Cystic fibrosis: state of the art. Am J Respir Crit Care Med 145:1229–1256 Davis PB, Drumm M, Konstan MW (1996) Cystic fibrosis: state of the art. Am J Respir Crit Care Med 145:1229–1256
3.
go back to reference Mastella G, Zanolla L, Castellani C et al (2001) Neonatal screening for cystic fibrosis: long-term clinical balance. Pancreatology 1:531–537PubMedCrossRef Mastella G, Zanolla L, Castellani C et al (2001) Neonatal screening for cystic fibrosis: long-term clinical balance. Pancreatology 1:531–537PubMedCrossRef
4.
go back to reference Landau LI, Mellis CM, Phelan PD, Bristowe B, McLennan L (1979) “Small airways disease” in children: no test is best. Thorax 34(2):217–223, AprPubMedCrossRef Landau LI, Mellis CM, Phelan PD, Bristowe B, McLennan L (1979) “Small airways disease” in children: no test is best. Thorax 34(2):217–223, AprPubMedCrossRef
5.
go back to reference Tiddens HAWM (2002) Detecting early structural lung damage in cystic fibrosis. Pediatr Pulmonol 34:228–231PubMedCrossRef Tiddens HAWM (2002) Detecting early structural lung damage in cystic fibrosis. Pediatr Pulmonol 34:228–231PubMedCrossRef
6.
go back to reference Terheggen-Lagro S, Truyijens N, van Poppel N, Gulmans V, van der Laag J, van der Ent C (2003) Correlation of six different cystic fibrosis chest radiograph scoring systems with clinical parameters. Pediatr Pulmonol 35:441–445PubMedCrossRef Terheggen-Lagro S, Truyijens N, van Poppel N, Gulmans V, van der Laag J, van der Ent C (2003) Correlation of six different cystic fibrosis chest radiograph scoring systems with clinical parameters. Pediatr Pulmonol 35:441–445PubMedCrossRef
7.
go back to reference Stanbrook MB, Corey M, Tullis DE (2004) The repeatability of forced expiratory volume measurements in adults with cystic fibrosis. Chest 125:150–155PubMedCrossRef Stanbrook MB, Corey M, Tullis DE (2004) The repeatability of forced expiratory volume measurements in adults with cystic fibrosis. Chest 125:150–155PubMedCrossRef
8.
go back to reference Rosenberg SM, Howatt MF, Grum CM (1992) Spirometry and chest roentgenographic appearances in adults with cystic fibrosis. Chest 101:961–964PubMedCrossRef Rosenberg SM, Howatt MF, Grum CM (1992) Spirometry and chest roentgenographic appearances in adults with cystic fibrosis. Chest 101:961–964PubMedCrossRef
9.
go back to reference Recommendations of a Working Group (1996) Clinical guidelines for cystic fibrosis care. Cystic fibrosis trust. British Paediatric Association. British Thoracic Society Recommendations of a Working Group (1996) Clinical guidelines for cystic fibrosis care. Cystic fibrosis trust. British Paediatric Association. British Thoracic Society
10.
go back to reference de Jong PA, Mayo JR, Golmohammadi K et al (2006) Estimation of cancer mortality associated with repetitive computed tomography scanning. Am J Respir Crit Care Med 173:199–203PubMedCrossRef de Jong PA, Mayo JR, Golmohammadi K et al (2006) Estimation of cancer mortality associated with repetitive computed tomography scanning. Am J Respir Crit Care Med 173:199–203PubMedCrossRef
11.
go back to reference Santamaria F, Grillo G, Guidi G et al (1998) Cystic fibrosis: when should high-resolution computed tomography of the chest be obtained? Pediatrics 101:908–913PubMedCrossRef Santamaria F, Grillo G, Guidi G et al (1998) Cystic fibrosis: when should high-resolution computed tomography of the chest be obtained? Pediatrics 101:908–913PubMedCrossRef
12.
go back to reference Nathanson I, Conboy K, Murphy S, Afshani E, Kuhn JP (1991) Ultrafast computerized tomography of the chest in cystic fibrosis: a new scoring system. Pediatr Pulmonol 11:81–86PubMedCrossRef Nathanson I, Conboy K, Murphy S, Afshani E, Kuhn JP (1991) Ultrafast computerized tomography of the chest in cystic fibrosis: a new scoring system. Pediatr Pulmonol 11:81–86PubMedCrossRef
13.
go back to reference Helbich TH, Heinz-Peer G, Eichler I et al (1999) Cystic fibrosis: CT assessment of lung involvement in children and adults. Radiology 213:55537–55544 Helbich TH, Heinz-Peer G, Eichler I et al (1999) Cystic fibrosis: CT assessment of lung involvement in children and adults. Radiology 213:55537–55544
14.
go back to reference Robinson TE, Leung AN, Northway WH et al (2003) Composite spirometric-computed tomography outcome measure in early cystic fibrosis lung disease. Am J Respir Crit Care Med 168:588–593PubMedCrossRef Robinson TE, Leung AN, Northway WH et al (2003) Composite spirometric-computed tomography outcome measure in early cystic fibrosis lung disease. Am J Respir Crit Care Med 168:588–593PubMedCrossRef
15.
go back to reference Brody AS, Molina PL, Klein JS, Ramagopal M, Swartz DR (1999) High-resolution computed tomography of the chest in children with cystic fibrosis: support for use as an outcome surrogate. Pediatr Radiol 29:731–735PubMedCrossRef Brody AS, Molina PL, Klein JS, Ramagopal M, Swartz DR (1999) High-resolution computed tomography of the chest in children with cystic fibrosis: support for use as an outcome surrogate. Pediatr Radiol 29:731–735PubMedCrossRef
16.
go back to reference de Jong PA, Ottink MD, Robben SGF et al (2004) Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements. Radiology 231:434–439PubMedCrossRef de Jong PA, Ottink MD, Robben SGF et al (2004) Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements. Radiology 231:434–439PubMedCrossRef
17.
go back to reference Bhalla M, Turcios N, Aponte V et al (1991) Cystic fibrosis: scoring system with thin-section CT. Radiology 179:783–788PubMed Bhalla M, Turcios N, Aponte V et al (1991) Cystic fibrosis: scoring system with thin-section CT. Radiology 179:783–788PubMed
18.
go back to reference Shwachman H, Kulczycki LL (1958) Long term study of 105 patients with CF. Am J Dis Child 96:6–15 Shwachman H, Kulczycki LL (1958) Long term study of 105 patients with CF. Am J Dis Child 96:6–15
19.
go back to reference Chrispin AR, Norman AP (1974) The systematic evaluation of a chest radiograph in CF. Pediatr Radiol 2:101–106PubMedCrossRef Chrispin AR, Norman AP (1974) The systematic evaluation of a chest radiograph in CF. Pediatr Radiol 2:101–106PubMedCrossRef
20.
go back to reference Kauczor HU, Hofmann D, Kreitner KF et al (1996) Normal and abnormal pulmonary ventilation: visualization at hyperpolarized He-3 MR imaging. Radiology 201:564–568PubMed Kauczor HU, Hofmann D, Kreitner KF et al (1996) Normal and abnormal pulmonary ventilation: visualization at hyperpolarized He-3 MR imaging. Radiology 201:564–568PubMed
21.
go back to reference Salerno M, de Lange EE, Altes TA, Truwit JD, Brookeman JR, Mugler JP III (2002) Emphysema: Hyperpolarized helium 3 diffusion MR imaging of the lungs compared with spirometric indexes - Initial experience. Radiology 222:252–260PubMedCrossRef Salerno M, de Lange EE, Altes TA, Truwit JD, Brookeman JR, Mugler JP III (2002) Emphysema: Hyperpolarized helium 3 diffusion MR imaging of the lungs compared with spirometric indexes - Initial experience. Radiology 222:252–260PubMedCrossRef
22.
go back to reference Samee S, Altes TA, Powers P, de Lange EE, Knight-Scott J, Rakes G, Mugler JP, Ciambotti JM, Alford BA, Brookeman JR, Platts-Mills TAE (2003) Imaging the lungs in asthmatics using hyperpolarized Helium-3 MR: assesment of response to methacholine and exercise challenge. J Allergy Clin Immunol 111:1205–1211PubMedCrossRef Samee S, Altes TA, Powers P, de Lange EE, Knight-Scott J, Rakes G, Mugler JP, Ciambotti JM, Alford BA, Brookeman JR, Platts-Mills TAE (2003) Imaging the lungs in asthmatics using hyperpolarized Helium-3 MR: assesment of response to methacholine and exercise challenge. J Allergy Clin Immunol 111:1205–1211PubMedCrossRef
23.
go back to reference Zaporozhan J, Gast KK, Ley S et al (2004) Functional analysis in single lung transplant recipients. A comparative study of high resolution CT, 3He MRI, and pulmonary function tests. Chest 125:173–181PubMedCrossRef Zaporozhan J, Gast KK, Ley S et al (2004) Functional analysis in single lung transplant recipients. A comparative study of high resolution CT, 3He MRI, and pulmonary function tests. Chest 125:173–181PubMedCrossRef
24.
go back to reference Donnelly LF, MacFall JR, McAdams HP et al (1999) Cystic fibrosis: combined hyperpolarized 3He-enhanced and conventional proton MR imaging in the lung - preliminary observation. Radiology 212:885–889PubMed Donnelly LF, MacFall JR, McAdams HP et al (1999) Cystic fibrosis: combined hyperpolarized 3He-enhanced and conventional proton MR imaging in the lung - preliminary observation. Radiology 212:885–889PubMed
25.
go back to reference Van Beek EJ, Schmiedeskamp J, Wild JM et al (2003) Hyperpolarized 3-helium MR imaging of the lungs: testing the concept of a central production facility. Eur Radiol 13:2583–2586PubMedCrossRef Van Beek EJ, Schmiedeskamp J, Wild JM et al (2003) Hyperpolarized 3-helium MR imaging of the lungs: testing the concept of a central production facility. Eur Radiol 13:2583–2586PubMedCrossRef
26.
go back to reference Mentore K, Froh DK, de Lange EE, Brookeman JR, Paget-Brown AO, Altes AA (2005) Hyperpolarized HHe 3 MRI of the lung in cystic fibrosis: assessment at baseline and after bronchodilator and airway clearance treatment. Acad Radiol 12:1423–1429PubMedCrossRef Mentore K, Froh DK, de Lange EE, Brookeman JR, Paget-Brown AO, Altes AA (2005) Hyperpolarized HHe 3 MRI of the lung in cystic fibrosis: assessment at baseline and after bronchodilator and airway clearance treatment. Acad Radiol 12:1423–1429PubMedCrossRef
27.
go back to reference Koumellis P, van Beek EJR, Woodhouse N, Fichele S, Paley MNJ, Hill C, Taylor C, Wild JM (2005) Quantitative analysis of regional airways obstruction using dynamic hyperpolarized 3He MRI - preliminary results in children with cystic fibrosis. J Magn Reson Imaging 22:420–426PubMedCrossRef Koumellis P, van Beek EJR, Woodhouse N, Fichele S, Paley MNJ, Hill C, Taylor C, Wild JM (2005) Quantitative analysis of regional airways obstruction using dynamic hyperpolarized 3He MRI - preliminary results in children with cystic fibrosis. J Magn Reson Imaging 22:420–426PubMedCrossRef
28.
go back to reference McMahon CJ, Dodd JD, Hill C, Woodhouse N, Wild JM, Fichele S, Gallagher CG, Skehan SJ, Masterson JB, van Beek EJR. Hyperpolarized 3Helium magnetic resonance imaging of the lung in cystic fibrosis: comparison with high resolution CT and spirometry. Eur Radiol DOI 10.1007/s00330-006-0311-5 McMahon CJ, Dodd JD, Hill C, Woodhouse N, Wild JM, Fichele S, Gallagher CG, Skehan SJ, Masterson JB, van Beek EJR. Hyperpolarized 3Helium magnetic resonance imaging of the lung in cystic fibrosis: comparison with high resolution CT and spirometry. Eur Radiol DOI 10.1007/s00330-006-0311-5
29.
go back to reference Polgar G, Promadlist U (1971) Pulmonary function testing in children. WB Saunders Co, Philadelphia Polgar G, Promadlist U (1971) Pulmonary function testing in children. WB Saunders Co, Philadelphia
30.
go back to reference Wild JM, Woodhouse N, Paley MN, Said Z, Fichele S, Kasuboski L, van Beek EJR (2004) Comparison between 2D and 3D gradient echo sequences for MR of human lung ventilation with hyperpolarized 3Helium. Magn Reson Med 52:673–678PubMedCrossRef Wild JM, Woodhouse N, Paley MN, Said Z, Fichele S, Kasuboski L, van Beek EJR (2004) Comparison between 2D and 3D gradient echo sequences for MR of human lung ventilation with hyperpolarized 3Helium. Magn Reson Med 52:673–678PubMedCrossRef
31.
go back to reference de Jong PA, Nakano Y, Lequin MH et al (2004) Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis. Eur Respir J 23:93–97PubMedCrossRef de Jong PA, Nakano Y, Lequin MH et al (2004) Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis. Eur Respir J 23:93–97PubMedCrossRef
32.
go back to reference Robinson TE, Leung AN, Northway WH et al (2001) Spirometer-triggered high-resolution computed tomography and pulmonary function measurements during an acute exacerbation in patients with cystic fibrosis. J Pediatr 138:553–559PubMedCrossRef Robinson TE, Leung AN, Northway WH et al (2001) Spirometer-triggered high-resolution computed tomography and pulmonary function measurements during an acute exacerbation in patients with cystic fibrosis. J Pediatr 138:553–559PubMedCrossRef
33.
go back to reference Brody AS, Tiddens HAWM, Castile RG et al (2005) Computed tomography in the evaluation of cystic fibrosis lung disease. Am J Respir Crit Care Med 172:1246–1252PubMedCrossRef Brody AS, Tiddens HAWM, Castile RG et al (2005) Computed tomography in the evaluation of cystic fibrosis lung disease. Am J Respir Crit Care Med 172:1246–1252PubMedCrossRef
34.
go back to reference Woodhouse N, Wild JM, Paley MNJ, Fichele S, Said Z, Swift A, van Beek EJR (2005) Combined Helium-3/Proton magnetic resonance imaging measurement of ventilated lung volumes in smokers compared to never-smokers. J Magn Reson Imaging 21:365–369PubMedCrossRef Woodhouse N, Wild JM, Paley MNJ, Fichele S, Said Z, Swift A, van Beek EJR (2005) Combined Helium-3/Proton magnetic resonance imaging measurement of ventilated lung volumes in smokers compared to never-smokers. J Magn Reson Imaging 21:365–369PubMedCrossRef
35.
go back to reference Woodhouse N, van Beek EJ, Fichele S, Hill C, Taylor C, Wild JM (2005) Reproducibility of lung volume measurements in hyperpolarized 3-Helium MRI of children with cystic fibrosis. [abstract] Proc Radiol Soc North America :685 Woodhouse N, van Beek EJ, Fichele S, Hill C, Taylor C, Wild JM (2005) Reproducibility of lung volume measurements in hyperpolarized 3-Helium MRI of children with cystic fibrosis. [abstract] Proc Radiol Soc North America :685
Metadata
Title
Assessment of lung disease in children with cystic fibrosis using hyperpolarized 3-Helium MRI: comparison with Shwachman score, Chrispin-Norman score and spirometry
Authors
Edwin J. R. van Beek
Catherine Hill
Neil Woodhouse
Stanislao Fichele
Sally Fleming
Bridget Howe
Sandra Bott
Jim M. Wild
Christopher J. Taylor
Publication date
01-04-2007
Publisher
Springer-Verlag
Published in
European Radiology / Issue 4/2007
Print ISSN: 0938-7994
Electronic ISSN: 1432-1084
DOI
https://doi.org/10.1007/s00330-006-0392-1

Other articles of this Issue 4/2007

European Radiology 4/2007 Go to the issue