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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease cystic fibrosis
Comorbidity C0024115|lung disease
Sentences 64
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- 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- 20717938 Summary: lung disease in patients with cystic fibrosis (cf) is characterized by recurrent bacterial respiratory infections and intense airway inflammation.
PubMedID- 23272037 It is known that low doses of dexamethasone slow the progression of lung disease in patients with cystic fibrosis through its role in reducing the inflammation associated with the bacterial infection [26].
PubMedID- 25725986 Background: although recent studies have begun to elucidate how airway microbial community structure relates to lung disease in cystic fibrosis (cf), microbial community activity and the host's response to changes in this activity are poorly understood.
PubMedID- 20840889 Inhaled medication and inhalation devices for lung disease in patients with cystic fibrosis: poor adherence and the need to address it.
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- 23378408 Hrct has an advantage over conventional chest x-rays in the evaluation of the stage and progression of lung disease in patients with 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- 25339567 [mycobacterial lung disease in patients with cystic fibrosis--report of three cases].
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- 20435528 Inhaled medication and inhalation devices for lung disease in patients with cystic fibrosis: three areas for future research which areas to target.
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- 21462361 Genetic studies of lung disease in cystic fibrosis (cf) are hampered by the lack of a severity measure that accounts for chronic disease progression and mortality attrition.
PubMedID- 25222938 Pulmonary hypertension in cystic fibrosis with advanced lung disease.
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- 19931415 Fat-free mass depletion in cystic fibrosis: associated with lung disease severity but poorly detected by body mass index.
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- 25102015 Antioxidant supplementation for lung disease in cystic fibrosis.
PubMedID- 25984165 Recent data indicate that azithromycin is beneficial in lung disease of cystic fibrosis because it directly suppresses excessive inflammation [3].
PubMedID- 24170579 Malnutrition is one of the main burdens of disease in cystic fibrosis (cf) along with lung disease.
PubMedID- 20693248 Outcome measures to assess therapeutic interventions in cystic fibrosis (cf) patients with mild lung disease are lacking.
PubMedID- 21382942 cystic fibrosis and survival in patients with advanced lung disease.
PubMedID- 20811560 Tuberculosis, including tuberculosis bronchiectasis, bronchogenic carcinoma, and chronic inflammatory lung disease due to bronchiectasis, cystic fibrosis, or aspergillosis are the most common causes of massive hemoptysis [19].
PubMedID- 25963404 Background: cystic fibrosis (cf) leads to advanced lung disease despite aggressive care.
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- 24884656 Determinants of exercise capacity in cystic fibrosis patients with mild-to-moderate lung disease.
PubMedID- 24429095 Early lung disease in cystic fibrosis.
PubMedID- 22431492 Background: hypoxemia during sleep is a common finding in cystic fibrosis (cf) patients with more advanced lung disease.
PubMedID- 26453627 Further issues that require attention are organ transplantation and end of life management.lung disease in adults with cystic fibrosis drives most of the clinical care requirements, and major life-threatening complications, such as respiratory infection, respiratory failure, pneumothorax and haemoptysis, and the management of lung transplantation require expertise from trained respiratory physicians.
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- 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- 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- 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- 23313410 The use of ivacaftor in an adult with severe lung disease due to cystic fibrosis (deltaf508/g551d).
PubMedID- 25973994 The spectrum of nocardia lung disease in cystic fibrosis.
PubMedID- 23235684 Authors' conclusions: this review did not find any evidence for the effectiveness of different antimicrobial treatment for nontuberculous mycobacteria lung disease in people with cystic fibrosis.
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- 23743112 Pseudomonas aeruginosa in patients without cystic fibrosis is strongly associated with chronic obstructive lung disease.
PubMedID- 20634941 Most of the morbidity and mortality of cystic fibrosis patients results from the lung disease [3].
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- 23748649 Correlation between computed tomography expression of pulmonary hypertension and severity of lung disease in cystic fibrosis patients.
PubMedID- 26079395 Does the fef25-75 or the fef75 have any value in assessing lung disease in children with cystic fibrosis or asthma.
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- 26047144 Background: lung disease in cystic fibrosis (cf) involves excessive inflammation, repetitive infections and development of bronchiectasis.
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- 19895389 Lung transplantation is the only life-prolonging therapy available for cystic fibrosis (cf) patients with end-stage lung disease.
PubMedID- 24671311 Polysomnographic differences associated with pulmonary hypertension in patients with advanced lung disease due to cystic fibrosis.
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- 25629612 Changes in the airway microbiome may be important in the pathophysiology of chronic lung disease in patients with cystic fibrosis.

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