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1
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3343023861
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Early intervention and prevention of lung disease in cystic fibrosis: A European consensus
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Doring G, Hoiby N, for the Consensus Study Group. Early intervention and prevention of lung disease in cystic fibrosis: a European consensus. J Cyst Fibros 2004; 3:67-91. Review article summarizing the current evidence (or lack of it) for treatment of the young child with cystic fibrosis. Highlights that the evidence base for what we do is gravely lacking.
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(2004)
J Cyst Fibros
, vol.3
, pp. 67-91
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Doring, G.1
Hoiby, N.2
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2
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0035830289
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Airway function in infants newly diagnosed with cystic fibrosis
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Ranganathan S, Dezateux C, Bush A, et al. Airway function in infants newly diagnosed with cystic fibrosis [research letter]. Lancet 2001; 358:1964-1965.
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(2001)
Lancet
, vol.358
, pp. 1964-1965
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Ranganathan, S.1
Dezateux, C.2
Bush, A.3
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3
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0037111312
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Relative ability of full and partial forced expiratory manoeuvres to identify diminished airway function in infants with cystic fibrosis
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Ranganathan S, Bush A, Dezateux C, et al. Relative ability of full and partial forced expiratory manoeuvres to identify diminished airway function in infants with cystic fibrosis. Am J Respir Crit Care Med 2002; 166:1350-1357.
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(2002)
Am J Respir Crit Care Med
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Ranganathan, S.1
Bush, A.2
Dezateux, C.3
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4
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11144355490
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The evolution of airway function in early childhood following clinical diagnosis of cystic fibrosis
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Ranganathan SC, Stocks J, Oezateux C, et al. The evolution of airway function in early childhood following clinical diagnosis of cystic fibrosis. Am J Respir Crit Care Med 2004; 169:928-933. The previous paper demonstrated that lung function as measured by the RVRTC was abnormal at diagnosis even in babies with cystic fibrosis with no apparent respiratory disease. This follow-up 6 months later in the same group of cystic fibrosis patients and controls shows no catch-up growth despite intensive therapy in specialist cystic fibrosis clinics.
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(2004)
Am J Respir Crit Care Med
, vol.169
, pp. 928-933
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Ranganathan, S.C.1
Stocks, J.2
Oezateux, C.3
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5
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0028914085
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Early pulmonary inflammation in infants with cystic fibrosis
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Khan TZ, Wagener JS, Boat T, et al. Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med 1995; 151:1075-1082.
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(1995)
Am J Respir Crit Care Med
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Khan, T.Z.1
Wagener, J.S.2
Boat, T.3
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6
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0027651801
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Chronic modification of lung and heart development in glucocorticoid treated newborn rats exposed to hyperoxia or room air
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Thibeault DW, Heimes B, Rezaiekhaligh M, Mabry S. Chronic modification of lung and heart development in glucocorticoid treated newborn rats exposed to hyperoxia or room air. Pediatr Pulmonol 1993; 16:81-88.
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(1993)
Pediatr Pulmonol
, vol.16
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Thibeault, D.W.1
Heimes, B.2
Rezaiekhaligh, M.3
Mabry, S.4
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7
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0028834931
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Influence of postnatally administered glucocorticoids on rat lung growth
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Tschanz SA, Damke BM, Burri PH. Influence of postnatally administered glucocorticoids on rat lung growth. Biol Neonate 1995; 68:229-245.
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(1995)
Biol Neonate
, vol.68
, pp. 229-245
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Tschanz, S.A.1
Damke, B.M.2
Burri, P.H.3
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8
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0031887382
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Early treatment with Dornase alpha in cystic fibrosis: What are the issues?
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Bush A. Early treatment with Dornase alpha in cystic fibrosis: what are the issues? Pediatr Pulmonol 1998; 25:79-82.
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(1998)
Pediatr Pulmonol
, vol.25
, pp. 79-82
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Bush, A.1
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10
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0347363613
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Preserved diffusion capacity in children with cystic fibrosis
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Merkus PJ, Govaere ES, Hop WH, et al. Preserved diffusion capacity in children with cystic fibrosis. Pediatr Pulmonol 2004; 37:56-60. Although it might be predicted that DLco might be affected in cystic fibrosis due to interference with alveolar growth, in fact this parameter is well preserved until late. An important negative paper.
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(2004)
Pediatr Pulmonol
, vol.37
, pp. 56-60
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Merkus, P.J.1
Govaere, E.S.2
Hop, W.H.3
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11
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2542496991
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Serial lung function and responsiveness in cystic fibrosis during early childhood
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Nielsen KG, Pressler T, Klug B, et al. Serial lung function and responsiveness in cystic fibrosis during early childhood. Am J Respir Crit Care Med 2004; 169:1209-1216. Serial longitudinal follow-up of cystic fibrosis children through the preschool years, demonstrating that there is airway obstruction in cystic fibrosis from infancy, which does not improve even with intensive therapy over several years.
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(2004)
Am J Respir Crit Care Med
, vol.169
, pp. 1209-1216
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Nielsen, K.G.1
Pressler, T.2
Klug, B.3
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12
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9644259076
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Multiple breath inert gas washout as a measure of ventilation distribution in children with cystic fibrosis
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Aurora P, Gustafsson PM, Bush A, et al. Multiple breath inert gas washout as a measure of ventilation distribution in children with cystic fibrosis. Thorax 2004; 59:1068-1073. Cross-sectional study establishing that LCI is a robust measurement that is transferable between institutions and also that it appears to be more sensitive than spirometry in older children with cystic fibrosis.
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(2004)
Thorax
, vol.59
, pp. 1068-1073
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Aurora, P.1
Gustafsson, P.M.2
Bush, A.3
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13
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12744277962
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Multiple-breath washout as a marker of lung disease in preschool children with cystic fibrosis
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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 2005; 171:249-256. Cross-sectional study in preschool children comparing spirometry, airway resistance measurements by plethysmography, and LCI, showing that LCI was the most sensitive technique of the three in detecting abnormalities of lung function.
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(2005)
Am J Respir Crit Care Med
, vol.171
, pp. 249-256
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Aurora, P.1
Bush, A.2
Gustafsson, P.3
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14
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13544250572
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Ventilation inhomogeneities in relation to standard lung function in patients with cystic fibrosis
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Kraemer R, Blum A, Schibler A, et al. Ventilation inhomogeneities in relation to standard lung function in patients with cystic fibrosis. Am J Respir Crit Care Med 2005; 171:371-378. Extremely important longitudinal study over 14 years in 142 children who acquired chronic infection with P. aeruginosa. They showed that at all stages of disease studied, LCI was the most sensitive and earliest affected parameter of lung function. More data are needed to confirm this in severe cystic fibrosis lung disease.
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(2005)
Am J Respir Crit Care Med
, vol.171
, pp. 371-378
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Kraemer, R.1
Blum, A.2
Schibler, A.3
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15
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3242693509
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High-resolution computed tomography in young patients with cystic fibrosis: Distribution of abnormalities and correlation with pulmonary function tests
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Brody AS, Klein JS, Molina PL, et al. High-resolution computed tomography in young patients with cystic fibrosis: distribution of abnormalities and correlation with pulmonary function tests. J Pediatr 2004; 145:32-38. Cross-sectional study of HRCT scored semiquantitatively compared with spirometry. Normal spirometry did not preclude bronchiectasis and air trapping on HRCT. Only a quarter of the scans were normal.
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(2004)
J Pediatr
, vol.145
, pp. 32-38
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Brody, A.S.1
Klein, J.S.2
Molina, P.L.3
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16
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0347766074
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Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis
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de Jong PA, Nakano Y, Lequin MH, et al. Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis. Eur Respir J 2004; 23:93-97. Longitudinal study showing that progressive changes on HRCT can occur in the face of normal and preserved spirometry. Weaknesses include semiquantitative scoring (although the same conclusions would be reached by any of the five systems they used) and lack of attempts at clinical correlation.
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(2004)
Eur Respir J
, vol.23
, pp. 93-97
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De Jong, P.A.1
Nakano, Y.2
Lequin, M.H.3
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17
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7044272783
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Quantitative air-trapping analysis in children with mild cystic fibrosis lung disease
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Bonnel AS, Song SM, Kesavarju K, et al. Quantitative air-trapping analysis in children with mild cystic fibrosis lung disease. Pediatr Pulmonol 2004; 38:396-405. Important proof-of-concept paper demonstrating the quantitative information that can be derived digitally from HRCT scans at total lung capacity and FRC. Surely the way forward if radiation dose can be reduced to acceptable levels for repeated scans.
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(2004)
Pediatr Pulmonol
, vol.38
, pp. 396-405
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Bonnel, A.S.1
Song, S.M.2
Kesavarju, K.3
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18
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0034991110
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Technique and clinical applications of full-inflation and end-exhalation, controlled ventilation chest CT in infants and young children
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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 2001; 31:413-422.
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(2001)
Pediatr Radiol
, vol.31
, pp. 413-422
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Long, F.R.1
Castile, R.G.2
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19
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0042877071
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Composite spirometric-computed tomography outcome measure in early cystic fibrosis lung disease
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Robinson TE, Leung AN, Northway WH, et al. Composite spirometric-computed tomography outcome measure in early cystic fibrosis lung disease. Am J Respir Crit Care Med 2003; 168:588-593.
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(2003)
Am J Respir Crit Care Med
, vol.168
, pp. 588-593
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Robinson, T.E.1
Leung, A.N.2
Northway, W.H.3
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20
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0037420207
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Cancer risk in nontransplanted and transplanted cystic fibrosis patients: A 10-year study
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Maisonneuve P, FitzSimmons SC, Neglia JP, et al. Cancer risk in nontransplanted and transplanted cystic fibrosis patients: a 10-year study. J Natl Cancer Inst 2003; 95:381-387.
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(2003)
J Natl Cancer Inst
, vol.95
, pp. 381-387
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Maisonneuve, P.1
Fitzsimmons, S.C.2
Neglia, J.P.3
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21
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0034764166
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Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis
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Rosenfeld M, Gibson RL, McNamara S, et al. Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis. Pediatr Pulmonol 2001; 32:356-366.
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(2001)
Pediatr Pulmonol
, vol.32
, pp. 356-366
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Rosenfeld, M.1
Gibson, R.L.2
McNamara, S.3
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22
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0030665499
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Regional variation of lung inflammation in cystic fibrosis
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Meyer KC, Sharma A. Regional variation of lung inflammation in cystic fibrosis. Am J Respir Crit Care Med 1997; 156:1536-1540.
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(1997)
Am J Respir Crit Care Med
, vol.156
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Meyer, K.C.1
Sharma, A.2
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23
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0033979508
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Fractional analysis of bronchoalveolar lavage fluid cytology in cystic fibrosis patients with normal lung function: Bronchoalveolar Lavage for the Evaluation of Anti-Inflammatory Treatment (BEAT) study group
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Ratjen F, Rietschel E, Griese M, et al. Fractional analysis of bronchoalveolar lavage fluid cytology in cystic fibrosis patients with normal lung function: Bronchoalveolar Lavage for the Evaluation of Anti-Inflammatory Treatment (BEAT) study group. Eur Respir J 2000; 15:141-145.
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Eur Respir J
, vol.15
, pp. 141-145
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Ratjen, F.1
Rietschel, E.2
Griese, M.3
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24
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0030698259
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Longitudinal study of lung function in a cohort of primary ciliary dyskinesia
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Ellerman A, Bisgaard H. Longitudinal study of lung function in a cohort of primary ciliary dyskinesia. Eur Respir J 1997; 10:2376-2379.
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(1997)
Eur Respir J
, vol.10
, pp. 2376-2379
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Ellerman, A.1
Bisgaard, H.2
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25
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0442313529
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Primary ciliary dyskinesia: Diagnostic and phenotypic features
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Noone PG, Leigh MW, Sannuti A, et al. Primary ciliary dyskinesia: diagnostic and phenotypic features. Am J Respir Crit Care Med 2004; 169:459-467.
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(2004)
Am J Respir Crit Care Med
, vol.169
, pp. 459-467
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Noone, P.G.1
Leigh, M.W.2
Sannuti, A.3
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26
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18844380742
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Asthma research: The real action is in children
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Bush A. Asthma research: the real action is in children. Paediatr Respir Rev 2005; 6:101-110. Includes a review of the ethics of bronchoscopic and other research in children.
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(2005)
Paediatr Respir Rev
, vol.6
, pp. 101-110
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Bush, A.1
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27
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0346751942
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The safety and quality of endobronchial biopsy in children under five years old
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Saglani S, Payne DN, Nicholson AG, et al. The safety and quality of endobronchial biopsy in children under five years old. Thorax 2003; 58:1053-1057.
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(2003)
Thorax
, vol.58
, pp. 1053-1057
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Saglani, S.1
Payne, D.N.2
Nicholson, A.G.3
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28
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0348012895
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Safety of endobronchial biopsy in 170 children with chronic respiratory symptoms
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Salva PS, Theroux C, Schwartz D. Safety of endobronchial biopsy in 170 children with chronic respiratory symptoms. Thorax 2003; 58:1058-1060.
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(2003)
Thorax
, vol.58
, pp. 1058-1060
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Salva, P.S.1
Theroux, C.2
Schwartz, D.3
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29
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14944365002
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Structural changes to airway smooth muscle in cystic fibrosis
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Hays SR, Ferrando RE, Carter R, et al. Structural changes to airway smooth muscle in cystic fibrosis. Thorax 2005; 60:226-228. A small study in an important but neglected area, structural airway wall changes. The authors used endobronchial biopsy to study airway smooth muscle and demonstrated hypertrophy but not hyperplasia of airway smooth muscle. We need to perform more endobronchial biopsies at the time of clinically indicated bronchoscopy, to explore the changes of airway remodeling in cystic fibrosis further.
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(2005)
Thorax
, vol.60
, pp. 226-228
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Hays, S.R.1
Ferrando, R.E.2
Carter, R.3
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30
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Transforming growth factor β1 and airway remodeling in children with cystic fibrosis
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Milliard TN, Shute J, Nicholson AG, et al. Transforming growth factor β1 and airway remodeling in children with cystic fibrosis. Thorax 2004; 59(Suppl 11):ii25-ii26.
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(2004)
Thorax
, vol.59
, Issue.SUPPL. 11
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Milliard, T.N.1
Shute, J.2
Nicholson, A.G.3
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