Comparison of normal infants and infants with cystic fibrosis using forced expiratory flows breathing air and heliox

Davis, Stephanie D.; Jones, Marcus Herbert; Kisling, Jeffrey; Howard, John M.; Tepper, Robert S.

doi:10.1002/1099-0496(200101)31:1<17::aid-ppul1002>3.0.co;2-8

Cited by 24 publications

(9 citation statements)

References 33 publications

(14 reference statements)

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“…17 A similar method was applied recently in infants, 97 but has not been found to increase the sensitivity of raised volume forced expiratory maneuvers in detecting differences between infants with CF and normal controls. 98 Lamarre et al 14 studied 19 children with CF, all of whom would have been reported as having normal lung function on the basis of spirometric and plethysmographic measurements. In contrast, ventilation distribution and V/Q ratio were abnormal, reinforcing the notion that the current``best methods'' may still be relatively insensitive tools.…”

Section: Discussionmentioning

confidence: 99%

Lung function testing in infants with cystic fibrosis: Lessons from the past and future directions

Gappa

Ranganathan²,

Stocks³

2001

Pediatric Pulmonology

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Despite the increasing awareness of the need to identify early pulmonary changes in cystic fibrosis (CF) noninvasively, the role of lung function testing in infancy and early childhood remains less clear than in older children with CF. The aim of this review is to summarize available data, discuss the information gained from these publications, and put this information into perspective with more recent developments of lung function testing in both infants and older children with CF. While some of the available data have been the foundation of the current level of understanding of respiratory physiology in CF, interpretation of other data has been hampered by differences between centers with regard to the methods and equipment used, patient selection, small number of subjects, and lack of appropriate reference data. A structured multicenter approach based on recently published recommendations for the measurement of lung function in infancy, together with pursuit of recent developments such as assessment of raised lung volume flow volume curves and ventilation inhomogeneity may help to more effectively utilize lung function tests in infants in the future.

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Section: Discussionmentioning

confidence: 99%

Lung function testing in infants with cystic fibrosis: Lessons from the past and future directions

Gappa

Ranganathan²,

Stocks³

2001

Pediatric Pulmonology

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“…However, the role of PFTs in very young patients with CF has been less well documented. Available data show that airway obstruction and/or hyperinflation can be present not only in infants with CF-related respiratory symptoms but also in asymptomatic CF infants (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15). That the ΔF508/ΔF508 mutation may have a deleterious effect on early lung function has been suggested by a study in which ΔF508/ΔF508 CF infants had significantly higher airflow resistance than CF infants with other mutations (6).…”

Section: Introductionmentioning

confidence: 99%

Pulmonary Function Tests in Preschool Children with Cystic Fibrosis

Beydon¹,

Amsallem²,

Bellet³

et al. 2002

Am J Respir Crit Care Med

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Pulmonary function tests have rarely been assessed in preschool children with cystic fibrosis (CF). The objective of this multicenter study was to compare pulmonary function in 39 preschool children with CF (height, 90-130 cm; 16 homozygous Delta F508) and in 79 healthy control children. Functional residual capacity (helium dilution technique) and expiratory interrupter resistance (Rint(exp)) (interrupter technique) were measured. As compared with control children, children with CF had significantly higher Rint(exp), expressed as absolute values and as Z-scores (1.05 +/- 0.36 versus 0.80 +/- 0.20 kPa.L(-1). second, p < 0.0001; and 1.31 +/- 1.72 versus 0.19 +/- 0.97, p < 0.0001), and significantly lower specific expiratory interrupter conductance (1.29 +/- 0.34 versus 1.63 +/- 0.43 kPa(-1). second, p < 0.0001). The effect of the bronchodilator salbutamol on Rint(exp) was not significantly different between children with CF and control children. Rint(exp) Z-scores were significantly higher in children with CF who were exposed to passive smoke (n = 8) (p < 0.03). Children with CF and with a history of respiratory symptoms (n = 31) had significantly higher functional residual capacity Z-scores (p < 0.02) and lower specific expiratory interrupter conductance Z-scores (p < 0.04). Genotype did not influence the data. We conclude that Rint(exp) and functional residual capacity measurements may help to follow young children with CF who are unable to perform reproducible forced expiratory maneuvers.

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“…[1][2][3][4][5][6] Parameters derived from forced expiratory manoeuvres have traditionally been used to monitor lung function in older patients with cystic fibrosis and forced expiratory volume in 1 second (FEV 1 ) is still considered to be a predictor of prognosis in patients with moderate to severe cystic fibrosis lung disease. 7 8 Although infants cannot perform such manoeuvres, forced expiratory flow-volume (FEFV) loops can be obtained by substituting voluntary effort with application of an external pressure to the chest and abdomen to force expiration.…”

mentioning

confidence: 99%

Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests

Lum¹,

Gustafsson

Ljungberg³

et al. 2007

Thorax

199

211

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Background: Lung clearance index (LCI), a measure of ventilation inhomogeneity derived from the multiplebreath inert gas washout (MBW) technique, has been shown to detect abnormal lung function more readily than spirometry in preschool children with cystic fibrosis, but whether this holds true during infancy is unknown. Objectives: To compare the extent to which parameters derived from the MBW and the raised lung volume rapid thoraco-abdominal compression (RVRTC) techniques identify diminished airway function in infants with cystic fibrosis when compared with healthy controls. Methods: Measurements were performed during quiet sleep, with the tidal breathing MBW technique being performed before the forced expiratory manoeuvres. Results: Measurements were obtained in 39 infants with cystic fibrosis (mean (SD) age 41.4 (22.0) weeks) and 21 controls (37.0 (15.1) weeks). Infants with cystic fibrosis had a significantly higher respiratory rate (38 (10) vs 32 (5) bpm) and LCI (8.4 (1.5) vs 7.2 (0.3)), and significantly lower values for all forced expiratory flow-volume parameters compared with controls. Girls with cystic fibrosis had significantly lower forced expiratory volume ) than boys (mean (95% CI girls-boys): -1.2 (-2.1 to 20.3) for FEV 0.5 Z score; FEF 25-75 : -1.2 (-2.2 to 20.15)). When using both the MBW and RVRTC techniques, abnormalities were detected in 72% of the infants with cystic fibrosis, with abnormalities detected in 41% using both techniques and a further 15% by each of the two tests performed. Conclusions: These findings support the view that inflammatory and/or structural changes in the airways of children with cystic fibrosis start early in life, and have important implications regarding early detection and interventions. Monitoring of early lung disease and functional status in infants and young children with cystic fibrosis may be enhanced by using both MBW and the RVRTC.

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Comparison of normal infants and infants with cystic fibrosis using forced expiratory flows breathing air and heliox

Cited by 24 publications

References 33 publications

Lung function testing in infants with cystic fibrosis: Lessons from the past and future directions

Lung function testing in infants with cystic fibrosis: Lessons from the past and future directions

Pulmonary Function Tests in Preschool Children with Cystic Fibrosis

Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests

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