Cystic fibrosis: The role of the small airways

Journal of Aerosol Medicine and Pulmonary Drug Delivery

Volumn 25, Issue 5, 2012, Pages 261-264

  Ratjen, Felix ^a  

^a HOSPITAL FOR SICK CHILDREN RESEARCH INSTITUTE   (Canada)

Author keywords

Cyctic fibrosis; Nebulizer; Small airways

Indexed keywords

DORNASE ALFA; SODIUM CHLORIDE;

AEROSOL; ARTICLE; DIAGNOSTIC ACCURACY; DIAGNOSTIC TEST; FORCED EXPIRATORY VOLUME; HUMAN; LUNG CLEARANCE; LUNG CLEARANCE INDEX; LUNG FIBROSIS; LUNG FUNCTION TEST; LUNG VOLUME; MEDICAL NEBULIZER; MULTIPLE BREATH WASHOUT; NUCLEAR MAGNETIC RESONANCE IMAGING; RESPIRATORY AIRFLOW; RESPIRATORY TRACT PARAMETERS; SENSITIVITY AND SPECIFICITY; SMALL AIRWAY DISEASE; SPIROMETRY;

ADMINISTRATION, INHALATION; AEROSOLS; ANIMALS; CYSTIC FIBROSIS; HUMANS; INFANT; LUNG; MUCUS; RESPIRATORY FUNCTION TESTS; RESPIRATORY SYSTEM ABNORMALITIES;

EID: 84867087485     PISSN: 19412711     EISSN: 19412703     Source Type: Journal    
DOI: 10.1089/jamp.2011.0923     Document Type: Article

References (37)

1. Ratjen FA: Cystic fibrosis: pathogenesis and future treatment strategies. Respir Care. 2009;54:595-605.
2. Ulrich M, Worlitzsch D, Viglio S, Siegmann N, Iadarola P, Shute JK, Geiser M, Pier GB, Friedel G, Barr ML, Schuster A, Meyer KC, Ratjen F, Bjarnsholt T, Gulbins E, and Doring G: Alveolar inflammation in cystic fibrosis. J Cyst Fibros. 2010; 9:217-227.
3. Kreda SM, Mall M, Mengos A, Rochelle L, Yankaskas J, Riordan JR, and Boucher RC: Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epithelia. Mol Biol Cell. 2005;16:2154-2167.
4. Meyerholz D, Stoltz D, Namati E, Ramachandran S, Pezzulo A, Smith A, Rector M, Suter M, Kao S, McLennan G, Tearney G, Zabner J, McCray PJ, and Welsh M: Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children. Am J Respir Crit Care Med. 2010;182:1251-1261.
5. Ballard ST, Trout L, Bebok Z, Sorscher EJ, and Crews A: CFTR involvement in chloride, bicarbonate, and liquid secretion by airway submucosal glands. Am J Physiol. 1999; 277:L694-L699.
6. Chen J, Stoltz D, Karp P, Ernst S, Pezzulo A, Moninger T, Rector M, Reznikov L, Launspach J, Chaloner K, Zabner J, and Welsh M: Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia. Cell. 2010;143:911-923.
7. Tarran R, Button B, Picher M, Paradiso A, Ribeiro C, Lazarowski E, Zhang L, Collins P, Pickles R, Fredberg J, and Boucher R: Normal and cystic fibrosis airway surface liquid homeostasis. The effects of phasic shear stress and viral infections. J Biol Chem. 2005;280:35751-35759.
8. van Ewijk B, van der Zalm M, Wolfs T, and van der Ent C: Viral respiratory infections in cystic fibrosis. J Cyst Fibros. 2005;Aug:31-36.
9. Oppenheimer E: Similarity of the tracheobronchial mucous glands and epithelium in infants with and without cystic fibrosis. Hum Pathol. 1981;12:36-48.
10. Bedrossian C, Greenberg S, Singer D, Hansen J, and Rosenberg H: The lung in cystic fibrosis: a quantitative study including prevalence of pathologic findings among different age groups. Hum Pathol. 1976;7:195-204.
11. Sturgess J, and Imrie J: Quantitative evaluation of the development of tracheal submucosal glands in infants with cystic fibrosis and control infants. Am J Pathol. 1982;106: 303-311.
12. Zuelzer W, and Newton WJ: The pathogenesis of fibrocystic disease of the pancreas; a study of 36 cases with special reference to the pulmonary lesions Pediatrics. 1949; 4:53-69.
13. Weibel E, and Gomez D: Arhitecture of the human lung. Use of quantitative methods establishes fundamental relations between size and number of lung structures. Science 1962;24:577-585.
14. Burgel PR: The role of small airways in obstructive airway diseases. Eur Respir Rev. 2011;20:23-33.
15. Allen TC: Pathology of small airways disease. Arch Pathol Lab Med. 2010;134:702-718.
16. Tiddens H, Donaldson S, Rosenfeld M, and Paré P: Cystic fibrosis lung disease starts in the small airways: can we treat it more effectively? Pediatr Pulmonol. 2010;45:107-117.
17. Burgel PR, Montani D, Danel C, Dusser DJ, and Nadel JA: A morphometric study of mucins and small airway plugging in cystic fibrosis. Thorax. 2007;62:153-161.
18. Griese M, Essl R, Schmidt R, Rietschel E, Ratjen F, Ballmann M, and Paul K: Pulmonary surfactant, lung function, and endobronchial inflammation in cystic fibrosis. Am J Respir Crit Care Med. 2004;170:1000-1005.
19. Linnane B, Robinson P, Ranganathan S, Stick S, and Murray C: Role of high-resolution computed tomography in the detection of early cystic fibrosis lung disease. Paediatr Respir Rev. 2008;9:168-174.
20. Loeve M, Lequin MH, de Bruijne M, Hartmann IJ, Gerbrands K, van Straten M, Hop WC, and Tiddens HA: Cystic fibrosis: are volumetric ultra-low-dose expiratory CT scans sufficient for monitoring related lung disease? Radiology. 2009;253:223-229.
21. O'Connor OJ, Vandeleur M, McGarrigle AM, Moore N, McWilliams SR, McSweeney SE, O'Neill M, Ni Chroinin M, and Maher MM: Development of low-dose protocols for thin-section CT assessment of cystic fibrosis in pediatric patients. Radiology. 2010;257:820-829.
22. Eichinger M, Heussel CP, Kauczor HU, Tiddens H, and Puderbach M: Computed tomography and magnetic resonance imaging in cystic fibrosis lung disease. J Magn Reson Imaging. 2010;32:1370-1378.
23. Regamey N, Jeffery PK, Alton EW, Bush A, and Davies JC: Airway remodelling and its relationship to inflammation in cystic fibrosis. Thorax. 2011;66:624-629.
24. Kerem E, Reisman J, Corey M, Canny GJ, and Levison H: Prediction of mortality in patients with cystic fibrosis. N Engl J Med. 1992;326:1187-1191.
25. Cooper PJ, Robertson CF, Hudson IL, and Phelan PD: Variability of pulmonary function tests in cystic fibrosis. Pediatr Pulmonol. 1990;8:16-22.
26. Quan JM, Tiddens HA, Sy JP, McKenzie SG, Montgomery MD, Robinson PJ, Wohl ME, and Konstan MW: A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities. J Pediatr. 2001;139:813-820.
27. Bakker EM, Volpi S, Salonini E, van der Wiel-Kooij EC, Sintnicolaas CJ, Hop WC, Assael BM, Merkus PJ, and Tiddens HA. Improved treatment response to dornase alfa in cystic fibrosis patients using controlled inhalation. Eur Respir J. 2011 Jul 7 [Epub ahead of print].
28. Verbanck S, Schuermans D, Van Muylem A, Melot C, Noppen M, Vincken W, and Paiva M: Conductive and acinar lung-zone contributions to ventilation inhomogeneity in COPD. Am J Respir Crit Care Med. 1998;157:1573-1577.
29. Aurora P, Gustafsson P, Bush A, Lindblad A, Oliver C, Wallis CE, and Stocks J: Multiple breath inert gas washout as a measure of ventilation distribution in children with cystic fibrosis. Thorax. 2004;59:1068-1073.
30. Aurora P, Bush A, and Gustafsson P: Multiple-breath washout as a marker of lung disease in preschool children with cystic fibrosis. Am J Respir Crit Care Med. 2005;171: 249-256.
31. Kraemer R, Blum A, Schibler A, Ammann RA, and 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.
32. Gustafsson PM, De Jong PA, Tiddens HA, and Lindblad A: Multiple-breath inert gas washout and spirometry versus structural lung disease in cystic fibrosis. Thorax. 2008;63: 129-134.
33. Aurora P, Stanojevic S, Wade A, Oliver C, Kozlowska W, Lum S, Bush A, Price J, Carr S, Shankar A, Stocks J, and 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.
34. Amin R, Subbarao P, Jabar A, Balkovec S, Jensen R, Kerrigan S, Gustafsson P, and Ratjen F: Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function. Thorax. 2010;65:379-383.
35. Amin R, Subbarao P, Lou W, Jabar A, Balkovec S, Jensen R, Kerrigan S, Gustafsson P, and Ratjen F: The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis. Eur Respir J. 2010;37:806-812.
36. Heijerman H, Westerman E, Conway S, Touw D, Döring G, and Consensus working group: Inhaled medication and inhalation devices for lung disease in patients with cystic fibrosis: A European consensus. J Cyst Fibros. 2009;8:295-315.
37. Ratjen F: Restoring airway surface liquid in cystic fibrosis. N Engl J Med. 2006;354:291-293.