Assessment and monitoring of cystic fibrosis lung disease in infants and young children

References

• Davis PB, Drumm M, Konstan MW. Cystic fibrosis. Am. J. Respir. Crit. Care Med.154, 1229–1256 (1996).
   PubMed Web of Science ®Google Scholar
• Riordan JR, Rommens JM, Kerem B et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science245, 1066–1073 (1989).
   PubMed Web of Science ®Google Scholar
• Boucher RC. Airway surface dehydration in cystic fibrosis: pathogenesis and therapy. Annu. Rev. Med.58, 157–170 (2007).
   PubMed Web of Science ®Google Scholar
• Gibson RL, Burns JL, Ramsey BW. Pathophysiology and management of pulmonary infections in cystic fibrosis. Am. J. Respir. Crit. Care Med.168, 918–951 (2003).
   PubMed Web of Science ®Google Scholar
• Chmiel JF, Berger M, Konstan MW. The role of inflammation in the pathophysiology of CF lung disease. Clin. Rev. Allergy Immunol.23, 5–27 (2002).
   PubMed Web of Science ®Google Scholar
• Deterding RR, Lavange LM, Engels JM et al. Phase 2 randomized safety and efficacy trial of nebulized denufosol tetrasodium in cystic fibrosis. Am. J. Respir. Crit. Care Med.176, 362–369 (2007).
   PubMed Web of Science ®Google Scholar
• Elkins MR, Robinson M, Rose BR et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N. Engl. J. Med.354, 229–240 (2006).
   PubMed Web of Science ®Google Scholar
• Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, Boucher RC. Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N. Engl. J. Med.354, 241–250 (2006).
   PubMed Web of Science ®Google Scholar
• Fuchs HJ, Borowitz DS, Christiansen DH et al. Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group. N. Engl. J. Med.331, 637–642 (1994).
   PubMed Web of Science ®Google Scholar
• Retsch-Bogart GZ, Burns JL, Otto KL et al. A Phase 2 study of aztreonam lysine for inhalation to treat patients with cystic fibrosis and Pseudomonas aeruginosa infection. Pediatr. Pulmonol.43, 47–58 (2007).
   Google Scholar
• Ramsey BW, Pepe MS, Quan JM et al. Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. Cystic Fibrosis Inhaled Tobramycin Study group. N. Engl. J. Med.340, 23–30 (1999).
   PubMed Web of Science ®Google Scholar
• Grosse SD, Boyle CA, Botkin JR et al. Newborn screening for cystic fibrosis: evaluation of benefits and risks and recommendations for state newborn screening programs. MMWR Recomm. Rep.53, 1–36 (2004).
   Google Scholar
• 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.145, 32–38 (2004).
   PubMed Web of Science ®Google Scholar
• Davis SD, Fordham LA, Brody AS et al. Computed tomography reflects lower airway inflammation and tracks changes in early cystic fibrosis. Am. J. Respir. Crit. Care Med.175, 943–950 (2007).
   PubMed Web of Science ®Google Scholar
• de Jong PA, Ottink MD, Robben SG et al. Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements. Radiology231, 434–439 (2004).
   Google Scholar
• Nasr SZ, Kuhns LR, Brown RW, Hurwitz ME, Sanders GM, Strouse PJ. Use of computerized tomography and chest x-rays in evaluating efficacy of aerosolized recombinant human DNase in cystic fibrosis patients younger than age 5 years: a preliminary study. Pediatr. Pulmonol.31, 377–382 (2001).
   PubMed Web of Science ®Google Scholar
• Brody AS, Kosorok MR, Li Z et al. Reproducibility of a scoring system for computed tomography scanning in cystic fibrosis. J. Thorac. Imaging21, 14–21 (2006).
   PubMed Web of Science ®Google Scholar
• Bonnel AS, Song SM, Kesavarju K et al. Quantitative air-trapping analysis in children with mild cystic fibrosis lung disease. Pediatr. Pulmonol.38, 396–405 (2004).
   Google Scholar
• Long FR, Williams RS, Adler BH, Castile RG. Comparison of quiet breathing and controlled ventilation in the high-resolution CT assessment of airway disease in infants with cystic fibrosis. Pediatr. Radiol.35, 1075–1080 (2005).
   PubMed Web of Science ®Google Scholar
• Long FR, Castile RG. Technique and clinical applications of full-inflation and end-exhalation controlled-ventilation chest CT in infants and young children. Pediatr. Radiol.31, 413–422 (2001).
   Web of Science ®Google Scholar
• Long FR, Castile RG, Brody AS et al. Lungs in infants and young children: improved thin-section CT with a noninvasive controlled-ventilation technique – initial experience. Radiology212, 588–593 (1999).
   Google Scholar
• Frush DP. Review of radiation issues for computed tomography. Semin. Ultrasound CT MR25, 17–24 (2004).
   Google Scholar
• Brenner DJ, Hall EJ. Computed tomography – an increasing source of radiation exposure. N. Engl. J. Med.357, 2277–2284 (2007).
   PubMed Web of Science ®Google Scholar
• Kauczor HU. Hyperpolarized helium-3 gas magnetic resonance imaging of the lung. Top. Magn. Reson. Imaging14, 223–230 (2003).
   Google Scholar
• Mentore K, Froh DK, de Lange EE, Brookeman JR, Paget-Brown AO, Altes TA. Hyperpolarized HHe 3 MRI of the lung in cystic fibrosis: assessment at baseline and after bronchodilator and airway clearance treatment. Acad. Radiol.12, 1423–1429 (2005).
   Google Scholar
• Mayer-Hamblett N, Aitken ML, Accurso FJ et al. Association between pulmonary function and sputum biomarkers in cystic fibrosis. Am. J. Respir. Crit. Care Med.175, 822–828 (2007).
   PubMed Web of Science ®Google Scholar
• Konstan MW, Hilliard KA, Norvell TM, Berger M. Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation. Am. J. Respir. Crit. Care Med.150, 448–454 (1994).
   PubMed Web of Science ®Google Scholar
• Khan TZ, Wagener JS, Bost T, Martinez J, Accurso FJ, Riches DW. Early pulmonary inflammation in infants with cystic fibrosis. Am. J. Respir. Crit. Care Med.151, 1075–1082 (1995).
   PubMed Web of Science ®Google Scholar
• Armstrong DS, Hook SM, Jamsen KM et al. Lower airway inflammation in infants with cystic fibrosis detected by newborn screening. Pediatr. Pulmonol.40, 500–510 (2005).
   PubMed Web of Science ®Google Scholar
• McGrath LT, Mallon P, Dowey L et al. Oxidative stress during acute respiratory exacerbations in cystic fibrosis. Thorax54, 518–523 (1999).
   Google Scholar
• Konstan MW, Byard PJ, Hoppel CL, Davis PB. Effect of high-dose ibuprofen in patients with cystic fibrosis. N. Engl. J. Med.332, 848–854 (1995).
   PubMed Web of Science ®Google Scholar
• Konstan MW, Schluchter MD, Xue W, Davis PB. Clinical use of Ibuprofen is associated with slower FEV1 decline in children with cystic fibrosis. Am. J. Respir. Crit. Care Med.176, 1084–1089 (2007).
   PubMed Web of Science ®Google Scholar
• Lands LC, Milner R, Cantin AM, Manson D, Corey M. High-dose ibuprofen in cystic fibrosis: Canadian safety and effectiveness trial. J. Pediatr.151, 249–254 (2007).
   PubMed Web of Science ®Google Scholar
• Eigen H, Rosenstein BJ, FitzSimmons S, Schidlow DV. A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group. J. Pediatr.126, 515–523 (1995).
   PubMed Web of Science ®Google Scholar
• Salva PS, Doyle NA, Graham L, Eigen H, Doerschuk CM. TNF-α, IL-8, soluble ICAM-1, and neutrophils in sputum of cystic fibrosis patients. Pediatr. Pulmonol.21, 11–19 (1996).
   PubMedGoogle Scholar
• Greally P, Hussain MJ, Vergani D, Price JF. Serum interleukin-1 a and soluble interleukin-2 receptor concentrations in cystic fibrosis. Arch. Dis. Child68, 785–787 (1993).
   PubMedGoogle Scholar
• Greally P, Hussein MJ, Cook AJ, Sampson AP, Piper PJ, Price JF. Sputum tumour necrosis factor-a and leukotriene concentrations in cystic fibrosis. Arch. Dis. Child.68, 389–392 (1993).
   PubMed Web of Science ®Google Scholar
• Bonfield TL, Panuska JR, Konstan MW et al. Inflammatory cytokines in cystic fibrosis lungs. Am. J. Respir. Crit. Care Med.152, 2111–2118 (1995).
   PubMed Web of Science ®Google Scholar
• Konstan MW, Hilliard KA, Norvell TM, Berger M. Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation. Am. J. Respir. Crit. Care Med.150, 448–454 (1994).
   PubMed Web of Science ®Google Scholar
• Konstan MW, Walenga RW, Hilliard KA, Hilliard JB. Leukotriene B4 markedly elevated in the epithelial lining fluid of patients with cystic fibrosis. Am. Rev. Respir. Dis.148, 896–901 (1993).
   PubMed Web of Science ®Google Scholar
• Dakin CJ, Numa AH, Wang H, Morton JR, Vertzyas CC, Henry RL. Inflammation, infection, and pulmonary function in infants and young children with cystic fibrosis. Am. J. Respir. Crit. Care Med.165, 904–910 (2002).
   PubMed Web of Science ®Google Scholar
• Rosenfeld M, Gibson RL, McNamara S et al. Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis. Pediatr. Pulmonol.32, 356–366 (2001).
   PubMed Web of Science ®Google Scholar
• Kharitonov SA, Barnes PJ. Exhaled markers of pulmonary disease. Am. J. Respir. Crit. Care Med.163, 1693–1722 (2001).
   PubMed Web of Science ®Google Scholar
• Carpagnano GE, Barnes PJ, Geddes DM, Hodson ME, Kharitonov SA. Increased leukotriene B4 and interleukin-6 in exhaled breath condensate in cystic fibrosis. Am. J. Respir. Crit. Care Med.167, 1109–1112 (2003).
   PubMed Web of Science ®Google Scholar
• Cunningham S, McColm JR, Ho LP, Greening AP, Marshall TG. Measurement of inflammatory markers in the breath condensate of children with cystic fibrosis. Eur. Respir. J.15, 955–957 (2000).
   PubMed Web of Science ®Google Scholar
• Carpagnano GE, Barnes PJ, Francis J, Wilson N, Bush A, Kharitonov SA. Breath condensate pH in children with cystic fibrosis and asthma: a new noninvasive marker of airway inflammation? Chest125, 2005–2010 (2004).
   PubMed Web of Science ®Google Scholar
• Jobsis Q, Raatgeep HC, Schellekens SL, Kroesbergen A, Hop WC, de Jongste JC. Hydrogen peroxide and nitric oxide in exhaled air of children with cystic fibrosis during antibiotic treatment. Eur. Respir. J.16, 95–100 (2000).
   PubMedGoogle Scholar
• Celio S, Troxler H, Durka SS et al. Free 3-nitrotyrosine in exhaled breath condensates of children fails as a marker for oxidative stress in stable cystic fibrosis and asthma. Nitric Oxide15, 226–232 (2006).
   Google Scholar
• Rosias PP, Den Hartog GJ, Robroeks CM et al. Free radicals in exhaled breath condensate in cystic fibrosis and healthy subjects. Free Radic. Res.40, 901–909 (2006).
   PubMed Web of Science ®Google Scholar
• Horak F Jr, Moeller A, Singer F et al. Longitudinal monitoring of pediatric cystic fibrosis lung disease using nitrite in exhaled breath condensate. Pediatr. Pulmonol.42, 1198–1206 (2007).
   Google Scholar
• Franklin P, Moeller A, Hall GL, Horak F Jr, Patterson H, Stick SM. Variability of nitric oxide metabolites in exhaled breath condensate. Respir. Med.100, 123–129 (2006).
   Google Scholar
• Grasemann H, Michler E, Wallot M, Ratjen F. Decreased concentration of exhaled nitric oxide (NO) in patients with cystic fibrosis. Pediatr. Pulmonol.24, 173–177 (1997).
   PubMed Web of Science ®Google Scholar
• Grasemann H, Ratjen F. Cystic fibrosis lung disease: the role of nitric oxide. Pediatr. Pulmonol.28, 442–448 (1999).
   Google Scholar
• Kelley TJ, Drumm ML. Inducible nitric oxide synthase expression is reduced in cystic fibrosis murine and human airway epithelial cells. J. Clin. Invest.102, 1200–1207 (1998).
   PubMed Web of Science ®Google Scholar
• Meng QH, Springall DR, Bishop AE et al. Lack of inducible nitric oxide synthase in bronchial epithelium: a possible mechanism of susceptibility to infection in cystic fibrosis. J. Pathol.184, 323–331 (1998).
   PubMed Web of Science ®Google Scholar
• Moeller A, Horak F Jr, Lane C et al. Inducible NO synthase expression is low in airway epithelium from young children with cystic fibrosis. Thorax61, 514–520 (2006).
   PubMed Web of Science ®Google Scholar
• Grasemann H, Lax H, Treseler JW, Colin AA. Dornase a and exhaled NO in cystic fibrosis. Pediatr. Pulmonol.38, 379–385 (2004).
   PubMed Web of Science ®Google Scholar
• Gaston B, Ratjen F, Vaughan JW et al. Nitrogen redox balance in the cystic fibrosis airway: effects of antipseudomonal therapy. Am. J. Respir. Crit. Care Med.165, 387–390 (2002).
   PubMedGoogle Scholar
• Ho LP, Innes JA, Greening AP. Exhaled nitric oxide is not elevated in the inflammatory airways diseases of cystic fibrosis and bronchiectasis. Eur. Respir. J.12, 1290–1294 (1998).
   PubMed Web of Science ®Google Scholar
• Jaffe A, Slade G, Rae J, Laverty A. Exhaled nitric oxide increases following admission for intravenous antibiotics in children with cystic fibrosis. J. Cyst. Fibros.2, 143–147 (2003).
   PubMedGoogle Scholar
• Linnane SJ, Keatings VM, Costello CM et al. Total sputum nitrate plus nitrite is raised during acute pulmonary infection in cystic fibrosis. Am. J. Respir. Crit. Care Med.158, 207–212 (1998).
   Google Scholar
• Elphick HE, Demoncheaux EA, Ritson S, Higenbottam TW, Everard ML. Exhaled nitric oxide is reduced in infants with cystic fibrosis. Thorax56, 151–152 (2001).
   PubMedGoogle Scholar
• Franklin PJ, Hall GL, Moeller A, Horak F Jr, Brennan S, Stick SM. Exhaled nitric oxide is not reduced in infants with cystic fibrosis. Eur. Respir. J.27, 350–353 (2006).
   PubMed Web of Science ®Google Scholar
• Martinez T, Weist A, Williams T, Clem C, Silkoff P, Tepper RS. Assessment of exhaled nitric oxide kinetics in healthy infants. J. Appl. Physiol.94, 2384–2390 (2003).
   Google Scholar
• Tepper RS, Montgomery GL, Ackerman V, Eigen H. Longitudinal evaluation of pulmonary function in infants and very young children with cystic fibrosis. Pediatr. Pulmonol.16, 96–100 (1993).
   Google Scholar
• Clayton RG Sr, Diaz CE, Bashir NS, Panitch HB, Schidlow DV, Allen JL. Pulmonary function in hospitalized infants and toddlers with cystic fibrosis. J. Pediatr.132, 405–408 (1998).
   Google Scholar
• Castile RG, Iram D, McCoy KS. Gas trapping in normal infants and in infants with cystic fibrosis. Pediatr. Pulmonol.37, 461–469 (2004).
   PubMedGoogle Scholar
• Berge MT, Wiel E, Tiddens HA, Merkus PJ, Hop WC, de Jongste JC. DNase in stable cystic fibrosis infants: a pilot study. J. Cyst. Fibros.2, 183–188 (2003).
   PubMedGoogle Scholar
• Brennan S, Hall GL, Horak F et al. Correlation of forced oscillation technique in preschool children with cystic fibrosis with pulmonary inflammation. Thorax60, 159–163 (2005).
   PubMed Web of Science ®Google Scholar
• Respiratory mechanics in infants: physiologic evaluation in health and disease. American Thoracic Society/European Respiratory Society. Am. Rev. Respir. Dis.147, 474–496 (1993).
   Google Scholar
• ATS/ERS Statement: raised volume forced expirations in infants: guidelines for current practice. Am. J. Respir. Crit. Care Med.172, 1463–1471 (2005).
   PubMed Web of Science ®Google Scholar
• Feher A, Castile R, Kisling J et al. Flow limitation in normal infants: a new method for forced expiratory maneuvers from raised lung volumes. J. Appl. Physiol.80, 2019–2025 (1996).
   Google Scholar
• Turner DJ, Stick SM, Lesouef KL, Sly PD, Lesouef PN. A new technique to generate and assess forced expiration from raised lung volume in infants. Am. J. Respir. Crit. Care Med.151, 1441–1450 (1995).
   PubMed Web of Science ®Google Scholar
• Ranganathan SC, Bush A, Dezateux C et al. Relative ability of full and partial forced expiratory maneuvers to identify diminished airway function in infants with cystic fibrosis. Am. J. Respir. Crit. Care Med.166, 1350–1357 (2002).
   PubMed Web of Science ®Google Scholar
• Turner DJ, Lanteri CJ, Lesouef PN, Sly PD. Improved detection of abnormal respiratory function using forced expiration from raised lung volume in infants with cystic fibrosis. Eur. Respir. J.7, 1995–1999 (1994).
   Google Scholar
• Nixon GM, Armstrong DS, Carzino R et al. Early airway infection, inflammation, and lung function in cystic fibrosis. Arch. Dis. Child.87, 306–311 (2002).
   PubMedGoogle Scholar
• Rosenfeld M, Brumback L, Knutzen S et al. Multicenter, longitudinal evaluation of raised volume forced expiratory flows in infants with CF is feasible. Pediatr. Pulmonol.28(Suppl.), 313 (2007).
   Google Scholar
• Linnane B, Hall G, Nolan G et al. Lung function is diminished in infants with CF diagnosed by newborn screening regardless of pulmonary infection detected in broncho-alveolar lavage. Pediatr. Pulmonol.30(Suppl.), 332 (2007).
   Google Scholar
• Sharp J, Sheehan D, Ren CL. Lung function in infants with CF diagnosed by newborn screening. Pediatr. Pulmonol.30(Suppl.), 334 (2007).
   Google Scholar
• Beydon N, Davis SD, Lombardi E et al. An official American Thoracic Society/European Respiratory Society statement: pulmonary function testing in preschool children. Am. J. Respir. Crit. Care Med.175, 1304–1345 (2007).
   PubMed Web of Science ®Google Scholar
• Eigen H, Bieler H, Grant D et al. Spirometric pulmonary function in healthy preschool children. Am. J. Respir. Crit. Care Med.163, 619–623 (2001).
   PubMed Web of Science ®Google Scholar
• Aurora P, Stocks J, Oliver C et al. Quality control for spirometry in preschool children with and without lung disease. Am. J. Respir. Crit. Care Med.169, 1152–1159 (2004).
   PubMed Web of Science ®Google Scholar
• Marostica PJ, Weist AD, Eigen H et al. Spirometry in 3- to 6-year-old children with cystic fibrosis. Am. J. Respir. Crit. Care Med.166, 67–71 (2002).
   Google Scholar
• Vilozni D, Bentur L, Efrati O et al. Spirometry in early childhood in cystic fibrosis patients. Chest131, 356–361 (2007).
   Google Scholar
• Martin TR, Feldman HA, Fredberg JJ, Castile RG, Mead J, Wohl ME. Relationship between maximal expiratory flows and lung volumes in growing humans. J. Appl. Physiol.65, 822–828 (1988).
   Google Scholar
• Guilbert TW, Morgan WJ, Zeiger RS et al. Long-term inhaled corticosteroids in preschool children at high risk for asthma. N. Engl. J. Med.354, 1985–1997 (2006).
   PubMed Web of Science ®Google Scholar
• Dubois AB, Brody AW, Lewis DH, Burgess BF Jr. Oscillation mechanics of lungs and chest in man. J. Appl. Physiol.8, 587–594 (1956).
   PubMed Web of Science ®Google Scholar
• Goldman MD. Clinical application of forced oscillation. Pulm. Pharmacol. Ther.14, 341–350 (2001).
   PubMed Web of Science ®Google Scholar
• Oostveen E, MacLeod D, Lorino H et al. The forced oscillation technique in clinical practice: methodology, recommendations and future developments. Eur. Respir. J.22, 1026–1041 (2003).
   PubMed Web of Science ®Google Scholar
• Frei J, Jutla J, Kramer G, Hatzakis GE, Ducharme FM, Davis GM. Impulse oscillometry: reference values in children 100 to 150 cm in height and 3 to 10 years of age. Chest128, 1266–1273 (2005).
   PubMed Web of Science ®Google Scholar
• Hall GL, Sly PD, Fukushima T et al. Respiratory function in healthy young children using forced oscillations. Thorax62, 521–526 (2007).
   PubMedGoogle Scholar
• Ren CL, Brucker JL, Rovitelli AK, Bordeaux KA. Changes in lung function measured by spirometry and the forced oscillation technique in cystic fibrosis patients undergoing treatment for respiratory tract exacerbation. Pediatr. Pulmonol.41, 345–349 (2006).
   PubMed Web of Science ®Google Scholar
• Nielsen KG, Pressler T, Klug B, Koch C, Bisgaard H. Serial lung function and responsiveness in cystic fibrosis during early childhood. Am. J. Respir. Crit. Care Med.169, 1209–1216 (2004).
   PubMedGoogle Scholar
• Gangell CL, Horak F Jr, Patterson HJ, Sly PD, Stick SM, Hall GL. Respiratory impedance in children with cystic fibrosis using forced oscillations in clinic. Eur. Respir. J.30, 892–897 (2007).
   PubMedGoogle Scholar
• Marotta A, Klinnert MD, Price MR, Larsen GL, Liu AH. Impulse oscillometry provides an effective measure of lung dysfunction in 4-year-old children at risk for persistent asthma. J. Allergy Clin. Immunol.112, 317–322 (2003).
   PubMed Web of Science ®Google Scholar
• Beydon N, Amsallem F, Bellet M et al. Pulmonary function tests in preschool children with cystic fibrosis. Am. J. Respir. Crit. Care Med.166, 1099–1104 (2002).
   PubMedGoogle Scholar
• Bisgaard H, Nielsen KG. Plethysmographic measurements of specific airway resistance in young children. Chest128, 355–362 (2005).
   PubMedGoogle Scholar
• Klug B, Bisgaard H. Measurement of the specific airway resistance by plethysmography in young children accompanied by an adult. Eur. Respir. J.10, 1599–1605 (1997).
   Google Scholar
• Gustafsson PM. Inert gas washout in preschool children. Paediatr. Respir. Rev.6, 239–245 (2005).
   PubMedGoogle Scholar
• Aurora P, Bush A, Gustafsson P et al. Multiple-breath washout as a marker of lung disease in preschool children with cystic fibrosis. Am. J. Respir. Crit. Care Med.171, 249–256 (2005).
   PubMed Web of Science ®Google Scholar
• Lum S, Gustafsson P, Ljungberg H et al. Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests. Thorax62, 341–347 (2007).
   PubMed Web of Science ®Google Scholar