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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease cystic fibrosis
Symptom C0024115|lung disease
Sentences 65
PubMedID- 20693248 Outcome measures to assess therapeutic interventions in cystic fibrosis (cf) patients with mild lung disease are lacking.
PubMedID- 23460012 As a result of their underlying lung disease, patients with cystic fibrosis (cf) have a higher risk of developing nontuberculous mycobacteria (ntm) infections compared with the general population.
PubMedID- 22645424 The pathogenesis of lung disease in cystic fibrosis is characterised by decreased airway surface liquid volume and subsequent failure of normal mucociliary clearance.
PubMedID- 23743112 Pseudomonas aeruginosa in patients without cystic fibrosis is strongly associated with chronic obstructive lung disease.
PubMedID- 23748649 Correlation between computed tomography expression of pulmonary hypertension and severity of lung disease in cystic fibrosis patients.
PubMedID- 25963404 Background: cystic fibrosis (cf) leads to advanced lung disease despite aggressive care.
PubMedID- 19931415 Fat-free mass depletion in cystic fibrosis: associated with lung disease severity but poorly detected by body mass index.
PubMedID- 23313410 The use of ivacaftor in an adult with severe lung disease due to cystic fibrosis (deltaf508/g551d).
PubMedID- 21955231 lung disease in cystic fibrosis (cf) is typified by the development of chronic airways infection culminating in bronchiectasis and progression to end-stage respiratory disease.
PubMedID- 23633344 Background: nebuliser systems are used to deliver medications to control the symptoms and the progression of lung disease in people with cystic fibrosis.
PubMedID- 22608703 Background: it is not known whether antibiotic therapy for lung disease in cystic fibrosis (cf) has an influence on circulating polymorphonuclear neutrophil (pmn) function and apoptosis.
PubMedID- 21942462 Objective: guidelines recommend chronic use of tobramycin solution for inhalation (tsi) for cystic fibrosis (cf) patients with moderate-to-severe lung disease and persistent airway pseudomonas aeruginosa.
PubMedID- 25505695 These same challenges are likely to apply to treating infants, or adults with obstructed airways, with aerosolized gene vectors for other lung diseases.figure 3percent of people with cystic fibrosis by age with normal/mild forced expiratory volume in one second (fev1), moderate fev1and severe fev1.
PubMedID- 26047144 Background: lung disease in cystic fibrosis (cf) involves excessive inflammation, repetitive infections and development of bronchiectasis.
PubMedID- 24429095 Early lung disease in cystic fibrosis.
PubMedID- 23226468 The university of north carolina committee for the rights of human subjects approved the collection and use of excess surgical pathology tissue, obtained at the time of lung transplant, from patients with advanced lung disease associated with cystic fibrosis.
PubMedID- 26079395 Does the fef25-75 or the fef75 have any value in assessing lung disease in children with cystic fibrosis or asthma.
PubMedID- 20435528 Inhaled medication and inhalation devices for lung disease in patients with cystic fibrosis: three areas for future research which areas to target.
PubMedID- 20955240 It is the main cause of hospital-acquired pneumonia and of lung disease in patients with cystic fibrosis, as well as infections in patients with neutropenia and burns (pier and ramphal, 2005).
PubMedID- 20717938 Summary: lung disease in patients with cystic fibrosis (cf) is characterized by recurrent bacterial respiratory infections and intense airway inflammation.
PubMedID- 25339567 Mycobacterial lung disease in patients with cystic fibrosis--report of three cases.
PubMedID- 22538801 Rationale: airways obstruction and lung volume restriction, major features of lung disease in cystic fibrosis (cf), may regress independently, causing dysanapsis between these parameters.
PubMedID- 21220215 Reports from the seventies and eighties have shown that cystic fibrosis (cf) patients with severe lung disease have high levels of igg and that this is associated with worse prognosis.
PubMedID- 23076917 Authors' conclusions: there is currently no evidence to support the use of cftr gene transfer reagents as a treatment for lung disease in people with cystic fibrosis.
PubMedID- 25102015 Antioxidant supplementation for lung disease in cystic fibrosis.
PubMedID- 24671311 Polysomnographic differences associated with pulmonary hypertension in patients with advanced lung disease due to cystic fibrosis.
PubMedID- 22960983 What you don't know can hurt you; early asymptomatic lung disease in cystic fibrosis.
PubMedID- 25629612 Changes in the airway microbiome may be important in the pathophysiology of chronic lung disease in patients with cystic fibrosis.
PubMedID- 25070399 We describe the case of a young patient with end-stage lung disease due to of cystic fibrosis and liver cirrhosis who needed combined lung-liver transplantation.
PubMedID- 21382942 cystic fibrosis and survival in patients with advanced lung disease.
PubMedID- 22162514 Regional differences in the evolution of lung disease in children with cystic fibrosis.
PubMedID- 24062613 Background: high-producer tgfbeta1 genotypes are associated with severe lung disease in cystic fibrosis (cf), but studies combining il-8, tnfalpha-, and tgfbeta1(+genotype) levels and their impact on cf lung disease are scarce.
PubMedID- 21154377 Antioxidant micronutrients for lung disease in cystic fibrosis.
PubMedID- 19895389 Lung transplantation is the only life-prolonging therapy available for cystic fibrosis (cf) patients with end-stage lung disease.
PubMedID- 22241570 Background: gastro-esophageal reflux (gor) may contribute to lung disease in children with cystic fibrosis (cf).
PubMedID- 22431492 Background: hypoxemia during sleep is a common finding in cystic fibrosis (cf) patients with more advanced lung disease.
PubMedID- 25861303 cystic fibrosis is associated with chronic lung disease, poor digestion, malabsorption of nutrients, and malnutrition 3.
PubMedID- 22323305 Rationale: unrecognized airway infection and inflammation in young children with cystic fibrosis (cf) may lead to irreversible lung disease; therefore early detection and treatment is highly desirable.
PubMedID- 24884656 Determinants of exercise capacity in cystic fibrosis patients with mild-to-moderate lung disease.
PubMedID- 26022611 Background: to assess the severity of lung disease in cystic fibrosis (cf), scoring systems based on chest radiographs (cxrs), ct and mri have been used extensively, although primarily in research settings rather than for clinical purposes.
PubMedID- 20840889 Inhaled medication and inhalation devices for lung disease in patients with cystic fibrosis: poor adherence and the need to address it.
PubMedID- 26429870 The purpose of this study was to evaluate cardiorespiratory fitness and reasons for exercise curtailment in a contemporary adult cystic fibrosis (cf) cohort with mild lung disease.
PubMedID- 22035707 We describe the successful use of ambulatory single-venous vv ecmo as a bridge to bilateral lung transplantation in 4 patients with end-stage lung disease due to cystic fibrosis who developed acute hypercapnic respiratory failure.
PubMedID- 24282073 Authors' conclusions: there is currently no evidence to support the use of cftr gene transfer agents as a treatment for lung disease in people with cystic fibrosis.
PubMedID- 25478180 Background: lung clearance index (lci) derived from sulfur hexafluoride (sf6) multiple breath washout (mbw) is a sensitive measure of lung disease in people with cystic fibrosis (cf).
PubMedID- 25973994 The spectrum of nocardia lung disease in cystic fibrosis.
PubMedID- 25216826 Mycobacterium abscessus complex is a group of rapidly growing mycobacteria, and an emerging cause of non-tuberculous mycobacterial lung disease in patients with cystic fibrosis and chronic lung diseases, such as bronchiectasis.
PubMedID- 21494150 Purpose of review: lung disease in cystic fibrosis (cf) results from chronic airway infection and inflammation leading to progressive bronchiectasis and respiratory failure.
PubMedID- 24261460 Rationale: previous studies of risk factors for progression of lung disease in cystic fibrosis (cf) have suffered from limitations that preclude a comprehensive understanding of the determinants of cf lung disease throughout childhood.
PubMedID- 25124441 lung disease in people with cystic fibrosis (cf) is initiated by defective host defense that predisposes airways to bacterial infection.

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