The remaining barriers to normalcy in CF: Advances in assessment of CF lung disease

Despite early diagnosis of cystic fibrosis (CF) through newborn screening, a substantial proportion of infants and young children with CF still demonstrate physiologic and structural evidence of lung disease progression, such as obstructive airway disease and bronchiectasis. The growing availability of highly effective CF transmembrane conductance regulatory modulator therapy to the vast majority of people with CF has led to the potential to alter the natural history of CF lung disease, but to assess the full impact of these therapies on CF lung disease and to help guide treatment, sensitive measures of early and mild disease are needed. Chest imaging using computed tomography or magnetic resonance imaging is one approach, but technologic barriers and/or concern about exposure to ionizing radiation may limit its use. However, advances in physiologic measurement techniques and exhaled breath analysis offer another option for assessment of CF lung disease

• Publication year

  2020

• Venue

  Pediatric Pulmonology

• Publication date

  2020-06-26

• Fields of study

  Medicine

• Identifiers
  DOI 10.1002/ppul.24929PMID 32589821
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Exhaled breath testing - A tool for the clinician and researcher.

  2019cited by this paper
• Respiratory rate in infants with cystic fibrosis throughout the first year of life and association with lung clearance index measured shortly after birth.

  2019cited by this paper
• Inhaled hypertonic saline in preschool children with cystic fibrosis (SHIP): a multicentre, randomised, double-blind, placebo-controlled trial.

  2019cited by this paper
• Identifying pediatric lung disease: A comparison of forced oscillation technique outcomes

  2019cited by this paper
• The lung clearance index as a monitoring tool in cystic fibrosis: ready for the clinic?

  2018cited by this paper
• Airway infection, systemic inflammation and lung clearance index in children and adults with cystic fibrosis

  2018cited by this paper
• The use of multiple breath washout for assessing cystic fibrosis in infants

  2017cited by this paper
• Feasibility of Early Detection of Cystic Fibrosis Acute Pulmonary Exacerbations by Exhaled Breath Condensate Metabolomics: A Pilot Study.

  2017cited by this paper
• Pulmonary function deficits in newborn screened infants with cystic fibrosis managed with standard UK care are mild and transient

  2017influential reference
• Efficacy and safety of lumacaftor and ivacaftor in patients aged 6-11 years with cystic fibrosis homozygous for F508del-CFTR: a randomised, placebo-controlled phase 3 trial.

  2017cited by this paper
• Progression of Lung Disease in Preschool Patients with Cystic Fibrosis

  2017cited by this paper
• Physiologic endpoints for clinical studies for cystic fibrosis.

  2016cited by this paper
• Long-term improvement of lung clearance index in patients with mild cystic fibrosis lung disease: Does hypertonic saline play a role?

  2016cited by this paper
• Lung Clearance Index in Adults and Children With Cystic Fibrosis.

  2016cited by this paper
• Evaluation of children with cystic fibrosis by impulse oscillometry when stable and at exacerbation

  2016cited by this paper
• Lung Clearance Index and Structural Lung Disease on Computed Tomography in Early Cystic Fibrosis.

  2015cited by this paper
• Dexmedetomidine for infant pulmonary function testing

  2015cited by this paper
• Multiple-Breath Washout as a Lung Function Test in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report.

  2015cited by this paper
• Tracking Lung Clearance Index and chest CT in mild cystic fibrosis lung disease over a period of three years.

  2014influential reference
• Lung clearance index: evidence for use in clinical trials in cystic fibrosis.

  2014cited by this paper
• Chloral hydrate sedation for infant pulmonary function testing

  2014cited by this paper
• Lung clearance index predicts pulmonary exacerbations in young patients with cystic fibrosis

  2013cited by this paper
• Evolution of lung function during the first year of life in newborn screened cystic fibrosis infants

  2013cited by this paper
• Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: a randomised controlled trial.

  2013cited by this paper
• Association of lung function, chest radiographs and clinical features in infants with cystic fibrosis

  2013cited by this paper
• Consensus statement for inert gas washout measurement using multiple- and single- breath tests

  2013cited by this paper
• An official American Thoracic Society workshop report: optimal lung function tests for monitoring cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheezing in children less than 6 years of age.

  2013cited by this paper
• Lung clearance index as an outcome measure for clinical trials in young children with cystic fibrosis. A pilot study using inhaled hypertonic saline.

  2013cited by this paper
• Early cystic fibrosis lung disease detected by bronchoalveolar lavage and lung clearance index.

  2012cited by this paper
• Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening

  2012cited by this paper
• Improvement in pulmonary function following antibiotics in infants with cystic fibrosis

  2012cited by this paper
• Analysis of the associations between lung function and clinical features in preschool children with Cystic Fibrosis

  2012cited by this paper
• Lung function distinguishes preschool children with CF from healthy controls in a multi‐center setting

  2012cited by this paper
• Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: the ISIS randomized controlled trial.

  2012cited by this paper
• Air Trapping on Chest CT Is Associated with Worse Ventilation Distribution in Infants with Cystic Fibrosis Diagnosed following Newborn Screening

  2011influential reference
• Infection, inflammation, and lung function decline in infants with cystic fibrosis.

  2011cited by this paper
• The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis

  2011cited by this paper
• Lung Clearance Index and HRCT are complementary markers of lung abnormalities in young children with CF

  2011cited by this paper
• Lung clearance index at 4 years predicts subsequent lung function in children with cystic fibrosis.

  2011cited by this paper
• Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function

  2010cited by this paper
• Lung Disease at Diagnosis in Infants with Cystic Fibrosis Detected by Newborn Screening

  2010cited by this paper
• Multicenter evaluation of infant lung function tests as cystic fibrosis clinical trial endpoints.

  2010cited by this paper
• Longitudinal assessment of lung function from infancy to childhood in patients with cystic fibrosis

  2009cited by this paper
• Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening.

  2009cited by this paper
• Inert Gas Washout: Theoretical Background and Clinical Utility in Respiratory Disease

  2009cited by this paper
• Biomarkers in exhaled breath condensate indicate presence and severity of cystic fibrosis in children

  2008cited by this paper
• Lung function in infants with cystic fibrosis diagnosed by newborn screening.

  2008cited by this paper
• Lung function in 3–5‐year‐old children with cystic fibrosis

  2008cited by this paper
• An official American Thoracic Society/European Respiratory Society statement: pulmonary function testing in preschool children.

  2007cited by this paper
• Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests

  2007cited by this paper
• Respiratory impedance in children with cystic fibrosis using forced oscillations in clinic

  2007cited by this paper
• Multiple-breath inert gas washout and spirometry versus structural lung disease in cystic fibrosis

  2007cited by this paper
• Spirometry in early childhood in cystic fibrosis patients.

  2007cited by this paper
• Advances in cystic fibrosis therapies

  2006cited by this paper
• Changes in lung function measured by spirometry and the forced oscillation technique in cystic fibrosis patients undergoing treatment for respiratory tract exacerbation

  2006cited by this paper
• ATS/ERS statement: raised volume forced expirations in infants: guidelines for current practice.

  2005cited by this paper
• Multiple-breath washout as a marker of lung disease in preschool children with cystic fibrosis.

  2005cited by this paper
• Serial lung function and responsiveness in cystic fibrosis during early childhood.

  2004influential reference
• Tidal Breathing Analysis

  2004cited by this paper
• The evolution of airway function in early childhood following clinical diagnosis of cystic fibrosis.

  2004cited by this paper
• The forced oscillation technique in clinical practice: methodology, recommendations and future developments

  2003cited by this paper
• Assessment of tidal breathing parameters in infants with cystic fibrosis

  2003cited by this paper
• Pathophysiology and management of pulmonary infections in cystic fibrosis.

  2003cited by this paper
• Spirometry in 3- to 6-year-old children with cystic fibrosis.

  2002cited by this paper
• Exhaled breath condensate: an evolving tool for noninvasive evaluation of lung disease.

  2002cited by this paper
• Pulmonary function tests in preschool children with cystic fibrosis.

  2002cited by this paper
• Adult‐type pulmonary function tests in infants without respiratory disease

  2000cited by this paper
• Tidal expiratory flow patterns in airflow obstruction.

  1981cited by this paper

• State-of-the-art review of lung imaging in cystic fibrosis with recommendations for pulmonologists and radiologists from the “iMAging managEment of cySTic fibROsis” (MAESTRO) consortium

  2022cites this paper