Airway function in infants newly diagnosed with cystic fibrosis

Ranganathan, Sarath; Dezateux, Carol; Bush, Andrew; Carr, S.B.; Castle, RA; Madge, Su; Price, John; Stroobant, John; Wade, Angie; Wallis, Colin; Stocks, Janet

doi:10.1016/s0140-6736(01)06970-7

Cited by 114 publications

(91 citation statements)

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“…Others have described cross-sectional (23)(24)(25)(26)(27) and longitudinal associations (18) between lower lung function and second-hand smoke exposure, but the effect of maternal smoking during pregnancy on lung function in children with CF has not to our knowledge been examined elsewhere. The effect of maternal smoking during pregnancy has been shown to be associated with lower lung function in healthy infants and adolescents and more symptoms in young children with asthma (28)(29)(30).…”

Section: Original Researchmentioning

confidence: 99%

Early Childhood Risk Factors for Decreased FEV₁at Age 6-7 Years in Young Children with Cystic Fibrosis

et al. 2015

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Rationale: There are limited objective measures of the severity of lung disease before children are able to routinely perform spirometry, generally at age 6 years. Identifying risk factors for reduced lung function at age 6 provides opportunities to intervene and slow the progression of cystic fibrosis (CF) lung disease.Objectives: To evaluate early childhood predictors of lung function at age 6-7 in a large U.S. CF cohort in the current era of widespread early eradication therapy for Pseudomonas aeruginosa (P. aeruginosa).Methods: Participants were children with CF enrolled before age 4 in the Early Pseudomonas Infection Control (EPIC) Observational Study, a multicenter, longitudinal study that enrolled P. aeruginosa-negative children not exceeding 12 years of age. Linear regression was used to estimate the association between potential early childhood risk factors and the best FEV 1 % predicted at age 6-7 years.Measurements and Main Results: Four hundred and eightyfour children (of 1,797 enrolled in the EPIC Observational Study) met the eligibility criteria for this analysis. Mean (SD) age at enrollment was 2.0 (1.3) years. In a multivariable model adjusted for age at enrollment, the following risk factors were significantly associated with lower mean (95% confidence interval) FEV 1 % predicted at age 6-7: weight percentile less than 10% during the year of enrollment (-5.3 [-9.1, -1.5]), P. aeruginosa positive during the year of enrollment (-2. Conclusions:In this large U.S. cohort, we identified several early childhood risk factors for lower FEV 1 at age 6-7 years, most of which are modifiable. Clinical trial registered with www.clinicaltrials.gov (NCT00097773).Keywords: cystic fibrosis; lung function; microbiology; tobacco smoke pollution Correspondence and requests for reprints should be addressed to Don B. Sanders, M.D., M.S.,

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Section: Original Researchmentioning

confidence: 99%

Early Childhood Risk Factors for Decreased FEV₁at Age 6-7 Years in Young Children with Cystic Fibrosis

et al. 2015

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“…9 Forced flows and volumes obtained over an extended volume range (from near total lung capacity) have been shown to be more sensitive in detecting diminished airway function in infants with cystic fibrosis than the tidal rapid thoracic compression technique. [10][11][12] By contrast, several studies have shown that many younger children with cystic fibrosis have spirometry results that fall within the normal range, 4 13 14 and that indices of gas mixing derived from inert gas multiplebreath washout (MBW) may be more sensitive than spirometry in detecting early airway disease in both preschool and schoolage children with cystic fibrosis. 1 4 15 Reduced gas mixing efficiency or ''increased inhomogeneity in ventilation'' can result from differences in specific ventilation between lung units joined at branch points in the conducting airways.…”

mentioning

confidence: 99%

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

Lum¹,

Gustafsson

Ljungberg³

et al. 2007

Thorax

198

210

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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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“…A number of studies using bronchoalveolar lavage (BAL) markers of infection and inflammation, lung function tests or computed tomography of the chest have documented that significant lung damage occurs very early in many, even asymptomatic, infants (Khan et al, 1995;Ranganathan et al, 2001;Davis et al, 2007;Mott et al, 2009). The results of two recent studies concerning very young infants who were investigated routinely after CF diagnosis by NBS are particularly striking (Sly et al, 2009;Stafler et al, 2011).…”

Section: Early Diagnosis: Cf Newborn Screening (Nbs)mentioning

confidence: 99%

The Prognosis of Cystic Fibrosis - A Clinician's Perspective

Lebecque¹

2012

Cystic Fibrosis - Renewed Hopes Through Research

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Airway function in infants newly diagnosed with cystic fibrosis

Cited by 114 publications

References 4 publications

Early Childhood Risk Factors for Decreased FEV₁at Age 6-7 Years in Young Children with Cystic Fibrosis

Early Childhood Risk Factors for Decreased FEV₁at Age 6-7 Years in Young Children with Cystic Fibrosis

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

The Prognosis of Cystic Fibrosis - A Clinician's Perspective

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Airway function in infants newly diagnosed with cystic fibrosis

Cited by 114 publications

References 4 publications

Early Childhood Risk Factors for Decreased FEV1at Age 6-7 Years in Young Children with Cystic Fibrosis

Early Childhood Risk Factors for Decreased FEV1at Age 6-7 Years in Young Children with Cystic Fibrosis

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

The Prognosis of Cystic Fibrosis - A Clinician's Perspective

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Early Childhood Risk Factors for Decreased FEV₁at Age 6-7 Years in Young Children with Cystic Fibrosis

Early Childhood Risk Factors for Decreased FEV₁at Age 6-7 Years in Young Children with Cystic Fibrosis