Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis

Corey, Mary; Edwards, Lloyd J.; Levison, Henry; Knowles, Michael R.

doi:10.1016/s0022-3476(97)70025-8

Cited by 336 publications

(269 citation statements)

References 15 publications

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“…Females with CF have poorer lung function, higher mortality, earlier colonisation with Pseudomonas aeruginosa, and deteriorate with Burkholderia earlier than their male counterparts (Block et al, 2006, Corey et al, 1997, Rosenfeld et al, 1997, Demko et al, 1995, Jackson et al, 2011a, Jackson et al, 2011b. Recent work has focused on the role of steroid sex hormones such as estrogens in this "gender gap" (Chotirmall et al, 2010, Chotirmall et al, 2012a and has been reviewed elsewhere (Chotirmall et al, 2012b, Saint-Criq and Harvey, 2013, Sweezey and Ratjen, 2013.…”

Section: Discussionmentioning

confidence: 99%

Long noncoding RNA are aberrantly expressed in vivo in the cystic fibrosis bronchial epithelium

McKiernan

Molloy

Cryan

et al. 2014

The International Journal of Biochemistry & Cell Biology

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Section: Discussionmentioning

confidence: 99%

Long noncoding RNA are aberrantly expressed in vivo in the cystic fibrosis bronchial epithelium

McKiernan

Molloy

Cryan

et al. 2014

The International Journal of Biochemistry & Cell Biology

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“…24 The FEV 1 data obtained prior to age 6 years or after lung transplantation were excluded. The FEV 1 values in liters were converted into CF-specific percentiles for FEV 1 .…”

Section: Phenotypingmentioning

confidence: 99%

Interactions Between Secondhand Smoke and Genes That Affect Cystic Fibrosis Lung Disease

Collaco¹,

Vanscoy²,

Bremer³

et al. 2008

JAMA

131

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Context Disease variation can be substantial even in conditions with a single gene etiology such as cystic fibrosis (CF). Simultaneously studying the effects of genes and environment may provide insight into the causes of variation.Objective To determine whether secondhand smoke exposure is associated with lung function and other outcomes in individuals with CF, whether socioeconomic status affects the relationship between secondhand smoke exposure and lung disease severity, and whether specific gene-environment interactions influence the effect of secondhand smoke exposure on lung function. Design, Setting, and ParticipantsRetrospective assessment of lung function, stratified by environmental and genetic factors. Data were collected by the US Cystic Fibrosis Twin and Sibling Study with missing data supplemented by the Cystic Fibrosis Foundation Data Registry. All participants were diagnosed with CF, were recruited between October 2000 and October 2006, and were primarily from the United States.Main Outcome Measures Disease-specific cross-sectional and longitudinal measures of lung function. ResultsOf 812 participants with data on secondhand smoke in the home, 188 (23.2%) were exposed. Of 780 participants with data on active maternal smoking during gestation, 129 (16.5%) were exposed. Secondhand smoke exposure in the home was associated with significantly lower cross-sectional (9.8 percentile point decrease; PϽ.001) and longitudinal lung function (6.1 percentile point decrease; P=.007) compared with those not exposed. Regression analysis demonstrated that socioeconomic status did not confound the adverse effect of secondhand smoke exposure on lung function. Interaction between gene variants and secondhand smoke exposure resulted in significant percentile point decreases in lung function, namely in CFTR non-⌬F508 homozygotes (12.8 percentile point decrease; P=.001), TGF␤1−509 TT homozygotes (22.7 percentile point decrease; P=.006), and TGF␤1 codon 10 CC homozygotes (20.3 percentile point decrease; P=.005).Conclusions Any exposure to secondhand smoke adversely affects both crosssectional and longitudinal measures of lung function in individuals with CF. Variations in the gene that causes CF (CFTR) and a CF-modifier gene (TGF␤1) amplify the negative effects of secondhand smoke exposure.

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“…The ability to identify risk factors for low FEV 1 % predicted (FEV 1 expressed as a percentage of the predicted normal value) at age 6 could provide opportunities to intervene and slow the progression of CF lung disease. Previous studies have identified risk factors for low FEV 1 % predicted at age 6, including female sex, poor nutritional status, viral infections, persistent infection with Pseudomonas aeruginosa (P. aeruginosa), respiratory symptoms, pulmonary exacerbations, and low socioeconomic status (3)(4)(5)(6)(7)(8). It is unclear whether these risk factors remain applicable in the current era, as children with CF born today are more likely to be detected by newborn screening (NBS) and treated more aggressively with antibiotics and CF-specific therapies, and to undergo eradication therapy when P. aeruginosa is isolated from respiratory cultures (9)(10)(11).…”

mentioning

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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Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis

Cited by 336 publications

References 15 publications

Long noncoding RNA are aberrantly expressed in vivo in the cystic fibrosis bronchial epithelium

Long noncoding RNA are aberrantly expressed in vivo in the cystic fibrosis bronchial epithelium

Interactions Between Secondhand Smoke and Genes That Affect Cystic Fibrosis Lung Disease

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

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Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis

Cited by 336 publications

References 15 publications

Long noncoding RNA are aberrantly expressed in vivo in the cystic fibrosis bronchial epithelium

Long noncoding RNA are aberrantly expressed in vivo in the cystic fibrosis bronchial epithelium

Interactions Between Secondhand Smoke and Genes That Affect Cystic Fibrosis Lung Disease

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

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