Multiple-breath washout as a lung function test in cystic fibrosis: A cystic fibrosis foundation workshop report

Annals of the American Thoracic Society

Volumn 12, Issue 6, 2015, Pages 932-939

  Subbarao, Padmaja ^a   Milla, Carlos ^b   Aurora, Paul ^c   Davies, Jane C ^d   Davis, Stephanie D ^e   Hall, Graham L ^f   Heltshe, Sonya ^g   Latzin, Philipp ^h   Lindblad, Anders ⁱ   Pittman, Jessica E ^j   Robinson, Paul D ^k   Rosenfeld, Margaret ^g   Singer, Florian ^l   Starner, Tim D ^m   Ratjen, Felix ^a   Morgan, Wayne ⁿ  

^a HOSPITAL FOR SICK CHILDREN RESEARCH INSTITUTE   (Canada)

^b STANFORD UNIVERSITY   (United States)

^c GREAT ORMOND STREET HOSPITAL   (United Kingdom)

^d ROYAL BROMPTON HOSPITAL   (United Kingdom)

^e INDIANA UNIVERSITY SCHOOL OF MEDICINE   (United States)

^f UNIVERSITY OF WESTERN AUSTRALIA   (Australia)

^g UNIVERSITY OF WASHINGTON   (United States)

^h UNIVERSITY CHILDREN S HOSPITAL   (Switzerland)

ⁱ QUEEN SILVIA CHILDREN'S HOSPITAL   (Sweden)

^j WASHINGTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^k UNIVERSITY OF SYDNEY   (Australia)

^l UNIVERSITY CHILDREN S HOSPITAL   (Switzerland)

^m UNIVERSITY OF IOWA CHILDREN S HOSPITAL   (United States)

ⁿ UNIVERSITY OF ARIZONA   (United States)

more..

Author keywords

Cystic fibrosis; Lung clearance index; Multiple breath washout; Pulmonary function tests

Indexed keywords

DORNASE ALFA; PLACEBO; SODIUM CHLORIDE;

ARTICLE; BREATH ANALYSIS; COMPUTER PROGRAM; CONSENSUS; CYSTIC FIBROSIS; EARLY DIAGNOSIS; FORCED EXPIRATORY VOLUME; GROUPS BY AGE; HUMAN; KNOWLEDGE; LUNG CLEARANCE; LUNG CLEARANCE INDEX; LUNG DISEASE; LUNG FUNCTION TEST; LUNG MODEL; MASS SPECTROMETER; MEASUREMENT ACCURACY; MICROCOMPUTER; MULTIPLE BREATH WASHOUT TEST; NORMAL VALUE; NORTH AMERICA; ONLINE ANALYSIS; OUTCOME ASSESSMENT; PSEUDOMONAS AERUGINOSA; RANDOMIZED CONTROLLED TRIAL (TOPIC); REBREATHING; REBREATHING DEVICE; REFERENCE VALUE; RESPIRATORY TRACT PARAMETERS; STANDARDIZATION; WORKSHOP; DEVICES; DISEASE COURSE; FLOWMETER; INFANT; NEWBORN; PATHOPHYSIOLOGY; PHYSIOLOGIC MONITORING; PRESCHOOL CHILD; PROCEDURES; PROGNOSIS; REPRODUCIBILITY;

BREATH TESTS; CHILD, PRESCHOOL; CYSTIC FIBROSIS; DISEASE PROGRESSION; FLOWMETERS; FORCED EXPIRATORY VOLUME; HUMANS; INFANT; INFANT, NEWBORN; MONITORING, PHYSIOLOGIC; OUTCOME ASSESSMENT (HEALTH CARE); PROGNOSIS; REPRODUCIBILITY OF RESULTS; RESPIRATORY FUNCTION TESTS;

EID: 84942254217     PISSN: 23256621     EISSN: None     Source Type: Journal    
DOI: 10.1513/AnnalsATS.201501-021FR     Document Type: Article

References (54)

1. Marshall BC, Nelson EC. Accelerating implementation of biomedical research advances: critical elements of a successful 10 year Cystic Fibrosis Foundation healthcare delivery improvement initiative. BMJ Qual Saf 2014;23:i95-i103.
2. Cystic Fibrosis Foundation. Patient registry 2012 annual data report. Bethesda, MD; 2012.
3. Mott LS, Park J, Murray CP, Gangell CL, de Klerk NH, Robinson PJ, Robertson CF, Ranganathan SC, Sly PD, Stick SM; AREST CF. Progression of early structural lung disease in young children with cystic fibrosis assessed using CT. Thorax 2012;67: 509-516.
4. 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;184:816-821.
5. Rosenfeld M, Allen J, Arets BH, Aurora P, Beydon N, Calogero C, Castile RG, Davis SD, Fuchs S, Gappa M, et al.; American Thoracic Society Assembly on Pediatrics Working Group on Infant and Preschool Lung Function Testing. An official American Thoracic Society workshop report: optimal lung function tests for monitoring cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheezing in children less than 6 years of age. Ann Am Thorac Soc 2013;10: S1-S11.
6. Fowler WS, Cornish ER Jr, Kety SS. Lung function studies: VIII. Analysis of alveolar ventilation by pulmonary N2 clearance curves. J Clin Invest 1952;31:40-50.
7. Robinson PD, Latzin P, Verbanck S, Hall GL, Horsley A, Gappa M, Thamrin C, Arets HG, Aurora P, Fuchs SI, et al. Consensus statement for inert gas washout measurement using multiple-and singlebreath tests. Eur Respir J 2013;41:507-522.
8. Robinson PD, Goldman MD, Gustafsson PM. Inert gas washout: theoretical background and clinical utility in respiratory disease. Respiration 2009;78:339-355.
9. Saidel GM, Salmon RB, Chester EH. Moment analysis of multibreath lung washout. J Appl Physiol 1975;38:328-334.
10. Singer F, Yammine S, Schmidt A, Proietti E, Kieninger E, Barben J, Casaulta C, Regamey N, Gustafsson P, Frey U, et al. Ventilatory response to nitrogen multiple-breath washout in infants. Pediatr Pulmonol 2014;49:342-347.
11. Cross KW, Warner P. The effect of inhalation of high and low oxygen concentrations on the respiration of the newborn infant. J Physiol 1951;114:283-295.
12. Miller HC. Effect of high concentrations of carbon dioxide and oxygen on the respiration of fullterm infants. Pediatrics 1954;14:104-113.
13. Betts KS. Worldwide effort underway to reduce SF6 greenhouse gas emissions. Environ Sci Technol 1998;32:487A-488A.
14. Maiss M, Brenninkmeijer CAM. Atmospheric SF6: trends, sources, and prospects. Environ Sci Technol 1998;32:3077-3086.
15. Hülskamp G, Lum S, Stocks J, Wade A, Hoo AF, Costeloe K, Hawdon J, Deeptha K, Pillow JJ. Association of prematurity, lung disease and body size with lung volume and ventilation inhomogeneity in unsedated neonates: a multicentre study. Thorax 2009;64:240-245.
16. Fuchs SI, Ellemunter H, Eder J, Mellies U, Grosse-Onnebrink J, Tümmler B, Staab D, Jobst A, Griese M, Ripper J, et al. Feasibility and variability of measuring the Lung Clearance Index in a multicenter setting. Pediatr Pulmonol 2012;47:649-657.
17. Houltz B, Green K, Lindblad A, Singer F, Robinson P, Nielsen K, Gustafsson P. Tidal N2 washout ventilation inhomogeneity indices in a reference population aged 7-70 years. Eur Respir J 2012;40:3797.
18. Yammine S, Singer F, Abbas C, Roos M, Latzin P. Multiple-breath washout measurements can be significantly shortened in children. Thorax 2013;68:586-587.
19. Robinson PD, Stocks J, Aurora P, Lum S. Abbreviated multi-breath washout for calculation of lung clearance index. Pediatr Pulmonol 2013;48:336-343.
20. Horsley AR, Macleod KA, Robson AG, Lenney J, Bell NJ, Cunningham S, Greening AP, Gustafsson PM, Innes JA. Effects of cystic fibrosis lung disease on gas mixing indices derived from alveolar slope analysis. Respir Physiol Neurobiol 2008;162:197-203.
21. Verbanck S, Paiva M, Paeps E, Schuermans D, Malfroot A, Vincken W, Vanderhelst E. Lung clearance index in adult cystic fibrosis patients: the role of convection-dependent lung units. Eur Respir J 2013;42: 380-388.
22. Verbanck S, Paiva M, Schuermans D, Malfroot A, Vincken W, Vanderhelst E. Acinar and conductive ventilation heterogeneity in severe CF lung disease: back to the model. Respir Physiol Neurobiol 2013;188:124-132.
23. Stanojevic S, Jensen R, Sundaralingam D, Salazar JG, Yammine S, Singer F, Latzin P, Amin R, Subbarao P, Gustafsson P, et al. Alternative outcomes for the multiple breath washout in children with CF. J Cyst Fibros [online ahead of print] 8 Jan 2015.; DOI: 10.1016/j. jcf.2014.12.008.
24. Jensen R, Stanojevic S, Gibney K, Salazar JG, Gustafsson P, Subbarao P, Ratjen F. Multiple breath nitrogen washout: a feasible alternative to mass spectrometry. PLoS One 2013;8:e56868.
25. Benseler A, Stanojevic S, Jensen R, Gustafsson P, Ratjen F. Effect of equipment dead space on multiple breath washout measures. Respirology 2015;20:459-466.
26. Kraemer R, Blum A, Schibler A, Ammann RA, Gallati S. Ventilation inhomogeneities in relation to standard lung function in patients with cystic fibrosis. Am J Respir Crit Care Med 2005;171:371-378.
27. Wall MA. Moment analysis of multibreath nitrogen washout in young children. J Appl Physiol (1985) 1985;59:274-279.
28. Hall GL, Logie KM, Parsons F, Schulzke SM, Nolan G, Murray C, Ranganathan S, Robinson P, Sly PD, Stick SM, et al.; AREST CF. 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.
29. Lum S, Stocks J, Stanojevic S, Wade A, Robinson P, Gustafsson P, Brown M, Aurora P, Subbarao P, Hoo AF, et al. Age and height dependence of lung clearance index and functional residual capacity. Eur Respir J 2013;41:1371-1377.
30. Fuchs O, Latzin P, Thamrin C, Stern G, Frischknecht P, Singer F, Kieninger E, Proietti E, Riedel T, Frey U. Normative data for lung function and exhaled nitric oxide in unsedated healthy infants. Eur Respir J 2011;37:1208-1216.
31. Verbanck S, Thompson BR, Schuermans D, Kalsi H, Biddiscombe M, Stuart-Andrews C, Hanon S, Van Muylem A, Paiva M, Vincken W, et al. Ventilation heterogeneity in the acinar and conductive zones of the normal ageing lung. Thorax 2012;67:789-795.
32. Aurora P, Bush A, Gustafsson P, Oliver C, Wallis C, Price J, Stroobant J, Carr S, Stocks J; London Cystic Fibrosis Collaboration. Multiplebreath washout as a marker of lung disease in preschool children with cystic fibrosis. Am J Respir Crit Care Med 2005;171:249-256.
33. Ellemunter H, Fuchs SI, Unsinn KM, Freund MC, Waltner-Romen M, Steinkamp G, Gappa M. Sensitivity of Lung Clearance Index and chest computed tomography in early CF lung disease. Respir Med 2010;104:1834-1842.
34. 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;63:129-134.
35. Owens CM, Aurora P, Stanojevic S, Bush A, Wade A, Oliver C, Calder A, Price J, Carr SB, Shankar A, et al.; London Cystic Fibrosis Collaboration. Lung Clearance Index and HRCT are complementary markers of lung abnormalities in young children with CF. Thorax 2011;66:481-488.
36. Singer F, Kieninger E, Abbas C, Yammine S, Fuchs O, Proietti E, Regamey N, Casaulta C, Frey U, Latzin P. Practicability of nitrogen multiple-breath washout measurements in a pediatric cystic fibrosis outpatient setting. Pediatr Pulmonol 2013;48:739-746.
37. Vermeulen F, Proesmans M, Boon M, Havermans T, De Boeck K. Lung clearance index predicts pulmonary exacerbations in young patients with cystic fibrosis. Thorax 2014;69:39-45.
38. Belessis Y, Dixon B, Hawkins G, Pereira J, Peat J, MacDonald R, Field P, Numa A, Morton J, Lui K, et al. Early cystic fibrosis lung disease detected by bronchoalveolar lavage and lung clearance index. Am J Respir Crit Care Med 2012;185:862-873.
39. Gustafsson PM, Aurora P, Lindblad A. Evaluation of ventilation maldistribution as an early indicator of lung disease in children with cystic fibrosis. Eur Respir J 2003;22:972-979.
40. Hoo AF, Thia LP, Nguyen TT, Bush A, Chudleigh J, Lum S, Ahmed D, Balfour Lynn I, Carr SB, Chavasse RJ, et al.; London Cystic Fibrosis Collaboration. Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening. Thorax 2012;67:874-881.
41. Lum S, Gustafsson P, Ljungberg H, Hülskamp G, Bush A, Carr SB, Castle R, Hoo AF, Price J, Ranganathan S, et al.; London Cystic Fibrosis Collaboration. Early detection of cystic fibrosis lung disease: multiplebreath washout versus raised volume tests. Thorax 2007;62:341-347.
42. Nguyen TT, Thia LP, Hoo AF, Bush A, Aurora P, Wade A, et al. Evolution of lung function during the first year of life in newborn screened cystic fibrosis infants. Thorax 2014;69:910-917.
43. Aurora P, Stanojevic S, Wade A, Oliver C, Kozlowska W, Lum S, Bush A, Price J, Carr SB, Shankar A, et al.; London Cystic Fibrosis Collaboration. Lung clearance index at 4 years predicts subsequent lung function in children with cystic fibrosis. Am J Respir Crit Care Med 2011;183:752-758.
44. Ramsey KA, Rosenow T, Skoric B, Adams A, Gallagher CM, Banton GL, Murray C, Ranganathan S, Stick SM, Hall GL. The ability of the Lung Clearance Index to detect bronchiectasis on chest computed tomography in infants and children with cystic fibrosis [abstract]. Pediatr Pulmonol 2014;49:359.
45. Horsley AR, Davies JC, Gray RD, Macleod KA, Donovan J, Aziz ZA, Bell NJ, Rainer M, Mt-Isa S, Voase N, et al. Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation. Thorax 2013;68:532-539.
46. Robinson PD, Cooper P, Van Asperen P, Fitzgerald D, Selvadurai H. Using index of ventilation to assess response to treatment for acute pulmonary exacerbation in children with cystic fibrosis. Pediatr Pulmonol 2009;44:733-742.
47. Kent L, Reix P, Innes JA, Zielen S, Le Bourgeois M, Braggion C, Lever S, Arets HG, Brownlee K, Bradley JM, et al.; European Cystic Fibrosis Society Clinical Trial Network (ECFS-CTN) Standardisation Committee. Lung clearance index: evidence for use in clinical trials in cystic fibrosis. J Cyst Fibros 2014;13:123-138.
48. Amin R, Subbarao P, Jabar A, Balkovec S, Jensen R, Kerrigan S, Gustafsson P, Ratjen F. Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function. Thorax 2010; 65:379-383.
49. Amin R, Subbarao P, Lou W, Jabar A, Balkovec S, Jensen R, Kerrigan S, Gustafsson P, Ratjen F. The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis. Eur Respir J 2011;37: 806-812.
50. Subbarao P, Stanojevic S, Brown M, Jensen R, Rosenfeld M, Davis S, Brumback L, Gustafsson P, Ratjen F. Lung clearance index as an outcome measure for clinical trials in young children with cystic fibrosis: a pilot study using inhaled hypertonic saline. Am J Respir Crit Care Med 2013;188:456-460.
51. Rosenfeld M, Ratjen F, Brumback L, Daniel S, Rowbotham R, McNamara S, Johnson R, Kronmal R, Davis SD; ISIS Study Group. Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: the ISIS randomized controlled trial. JAMA 2012; 307:2269-2277.
52. Davies J, Sheridan H, Bell N, Cunningham S, Davis SD, Elborn JS, Milla CE, Starner TD, Weiner DJ, Lee PS, et al. Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: a randomised controlled trial. Lancet Respir Med 2013;1:630-638.
53. Davis SD, Rosenfeld M, Kerby GS, Brumback L, Kloster MH, Acton JD, Colin AA, Conrad CK, Hart MA, Hiatt PW, et al. Multicenter evaluation of infant lung function tests as cystic fibrosis clinical trial endpoints. Am J Respir Crit Care Med 2010;182:1387-1397.
54. Gray DM, Willemse L, Alberts A, Simpson S, Sly PD, Hall GL, Zar HJ. Lung function in African infants: a pilot study. Pediatr Pulmonol 2015; 50:49-54.