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Cystic fibrosis (CF) is the most common genetic disease within the Caucasian population and leads to premature respiratory failure. Approximately 60,000 individuals are currently living with CF in North America and Europe, 40% of whom are adults. The life span of these patients has increased from approximately 2 to 32 yr of age over the last three decades.(More)
Cystic fibrosis is a common inherited fatal disease. As the life expectancy of affected individuals continues to increase with advances in disease management, this disease is no longer limited to the pediatric population. Currently, 40% of patients with cystic fibrosis are adults. In addition, patients may not present until adulthood and frequently have(More)
RATIONALE In cystic fibrosis (CF), conventional antibiotic susceptibility results correlate poorly with clinical outcomes. We hypothesized that biofilm testing would more accurately reflect the susceptibilities of bacteria infecting CF airways. METHODS A multicenter randomized pilot trial was conducted to assess the efficacy and safety of using biofilm(More)
Pseudomonas aeruginosa (PA) is a major pathogen causing morbidity and ultimately mortality in patients afflicted with cystic fibrosis (CF) lung disease. One important virulence factor, pyocyanin (PCN), is a blue, redox-active compound that is secreted in such copious amounts by PA in the CF lungs that it determines the colour of expectorated sputum. In this(More)
A review of literature for first metatarsocuneiform joint exostosis, accessory navicular bone, Haglund's syndrome, and os trigonum syndrome does not reveal any significant changes in the process of diagnosis and treatment. The surgical procedures are simple to perform but complications do occur that necessitate the understanding of the pathology and anatomy(More)
BACKGROUND Childhood interstitial lung disease (ILD) is a spectrum of diseases including many different rare lung conditions. We present a family with an unusual presentation of ILD in association with rheumatologic and immunologic abnormalities. METHODS Eight children with a common father were evaluated for evidence of lung disease in association with(More)
BACKGROUND Patients with cystic fibrosis (CF) may be at risk for micronutrient depletion, particularly during periods of illness and infection. The purpose of this study was to investigate serum micronutrient status over time in adults with CF initially hospitalized with a pulmonary exacerbation. MATERIALS AND METHODS This was an ancillary study of a(More)
Vitamin D deficiency is highly prevalent in children and adults with cystic fibrosis (CF). Recent studies have found an association between vitamin D status and risk of pulmonary exacerbations in children and adults with CF. The ongoing Vitamin D for enhancing the Immune System in Cystic fibrosis (DISC) study is a multi-center, double-blind, randomized,(More)
Chronic illness is a significant stressor; the majority of Americans cope utilizing spirituality. Numerous studies demonstrate links between spiritual coping and health outcomes. The purpose of this study was to determine whether persons diagnosed with cystic fibrosis (CF) as adults use spirituality to cope and influence disease management. Semi-structured(More)
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