Effect of Aerosolized Uridine-5 ′ -Triphosphate on Airway Clearance with Cough in Patients with Primary Ciliary Dyskinesia
نویسندگان
چکیده
منابع مشابه
Changes in airway inflammation during pulmonary exacerbations in patients with cystic fibrosis and primary ciliary dyskinesia.
Lung disease in patients with both primary ciliary dyskinesia (PCD) or cystic fibrosis (CF) is associated with impaired mucociliary clearance; however, clinical outcomes are typically worse in CF patients. We assessed whether CF and PCD patients differ in inflammatory response in the airways during pulmonary exacerbation.We first studied clinically stable PCD patients with a spectrum of bacteri...
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Primary ciliary dyskinesia is a rare, autosomal recessive disease, with diminished ciliary motility resulting in chronic disease of the upper and lower airway. Due to its low prevalence, there are few studies with significant numbers of cases, and a lack of guidelines for the management of this group of patients. Most patients present chronic rhinosinusitis at diagnosis and while there is impro...
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Primary ciliary dyskinesia (PCD) leads to recurrent/chronic respiratory infections, resulting in chronic inflammation and potentially in chronic pulmonary disease with bronchiectasis. We analyzed longitudinal data on body length/height and body mass index (BMI) for 29 children and young adults with PCD aging 1.5-24 years (median, 14.5) who had been diagnosed at the age of 0.5-17 years (median, ...
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Primary ciliary dyskinesia (PCD) is a genetic condition affecting one in 10 000–40 000 people from birth [1]; cilia fail to beat, and the airway clearance of mucus and debris is severely impaired. If untreated, this results in progressive lung infection leading to bronchiectasis and ultimately respiratory failure. Additionally, delayed diagnosis has implications for genetic counselling, appropr...
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ژورنال
عنوان ژورنال: American Journal of Respiratory and Critical Care Medicine
سال: 1999
ISSN: 1073-449X,1535-4970
DOI: 10.1164/ajrccm.160.1.9806146