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Cystic fibrosis gastrointestinal disease includes nutrient malabsorption and intestinal inflammation. We show that the abundances of Escherichia coli in fecal microbiota were significantly higher in young children with cystic fibrosis than in controls and correlated with fecal measures of nutrient malabsorption and inflammation, suggesting that E. coli(More)
Home spirometry is gaining acceptance in the medical community because of its ability to detect pulmonary exacerbations and improve outcomes of chronic lung ailments. However, cost and usability are significant barriers to its widespread adoption. To this end, we present SpiroSmart, a low-cost mobile phone application that performs spirometry sensing using(More)
Although the median survival for patients with cystic fibrosis (CF) is 32.9 years, a small group of patients live much longer. We analyzed the genotype and phenotype of CF patients 40 years and older seen between 1992 and 2004 at the National Jewish Medical and Research Center (n = 55). These patients were divided into two groups according to age at(More)
BACKGROUND Computer analysis of high-resolution CT (HRCT) scans may improve the assessment of structural lung injury in children with cystic fibrosis (CF). The goal of this cross-sectional pilot study was to validate automated, observer-independent image analysis software to establish objective, simple criteria for bronchiectasis and air trapping. METHODS(More)
RATIONALE Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator recently approved for patients with CF age 6 and older with the G551D mutation. OBJECTIVES To evaluate ivacaftor in a postapproval setting and determine mechanism of action and response of clinically relevant markers. METHODS We conducted a longitudinal(More)
Because pesticides may operate through different mechanisms, the authors studied the risk of prostate cancer associated with specific pesticides in the Agricultural Health Study (1993-2007). With 1,962 incident cases, including 919 aggressive prostate cancers among 54,412 applicators, this is the largest study to date. Rate ratios and 95% confidence(More)
The lung clearance index (LCI) is a lung function parameter derived from the multiple-breath washout (MBW) test. Although first developed 60 years ago, the technique was not widely used for many years. Recent technological advances in equipment design have produced gains in popularity for this test among cystic fibrosis (CF) researchers and clinicians,(More)
BACKGROUND CF infants often do not grow as expected which adversely affects later clinical outcomes, thus sensitive early measures of growth deficiency are important. This study compared attained growth for age with velocity standards to determine which better predicts growth deficits at 24 months of age. METHODS Growth deficiency in infancy based on(More)
BACKGROUND The prognostic value of Pseudomonas aeruginosa serology for antibiotic therapy in cystic fibrosis patients is not well understood. METHODS Using five antigens from two ELISAs, we assessed whether positive serology in CF patients participating in the multi-center Early Pseudomonas Infection in Children (EPIC) trial would predict treatment(More)
BACKGROUND There are limited data assessing the predictive value of fraction of exhaled nitric oxide (FENO ) for persistence of wheezing, exacerbations, or lung function change over time in infants/toddlers with recurrent wheezing. OBJECTIVES In an ongoing longitudinal cohort of infants and toddlers with recurrent wheezing, we compared predictive values(More)