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Cystic fbrosis
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Cystic fbrosis (CF) is a life-threatening multiorgan genetic disease, particularly affecting the lungs, where recurrent infections are the main cause of reduced life expectancy. In CF, mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein impair transepithelial electrolyte and water transport, resulting in airway dehydration, and a thickening of the mucus associated with abnormal viscoelastic properties.
10p
vimackenziebezos
29-11-2021
7
0
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Adult patients with cystic fibrosis (CF) experience daily physical symptoms and disabilities that can be challenging to address for health care teams. Methods: We sought to identify the most frequent topics that CF adults need to discuss with health care teams using a custom questionnaire including 62 items.
8p
vimackenziebezos
29-11-2021
13
1
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Functional Respiratory Imaging (FRI) combines HRCT scans with computational fluid dynamics to provide objective and quantitative information about lung structure and function. FRI has proven its value in pulmonary diseases such as COPD and asthma, but limited studies have focused on cystic fbrosis (CF).
11p
vimackenziebezos
29-11-2021
9
1
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While lung transplant (LTX) can be an effective therapy to provide the survival benefits in selected populations, post-transplant outcome in LTX recipients with bronchiectasis other than cystic fbrosis (CF) has been less studied. Pseudomonas aeruginosa, often associated with exacerbations in bronchiectasis, is the most common micro-organism isolated from LTX recipients.
10p
vimackenziebezos
29-11-2021
9
1
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Handheld oscillating positive expiratory pressure (OPEP) devices have been a mainstay of treatment for patients with hypersecretory conditions such as cystic fbrosis (CF) and chronic obstructive pulmonary disease (COPD) since the 1970s.
11p
vimackenziebezos
29-11-2021
10
1
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High-resolution computed tomography (HRCT) is the gold standard for the evaluation of cystic fbrosis (CF) lung disease; however, lung ultrasound (LUS) is being increasingly used for the assessment of lung in these patients due to its lower cost, availability, and lack of irradiation.
10p
vimackenziebezos
29-11-2021
15
0
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The extend of lung disease remains the most important prognostic factor for survival in patients with cystic fbrosis (CF), and lack of adherence is the main reason for treatment failure. Early detection of deterioration in lung function and optimising adherence are therefore crucial in CF care.
11p
vimackenziebezos
29-11-2021
8
1
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Oscillating Positive Expiratory Pressure (OPEP) devices are important adjuncts to airway clearance therapy in patients with cystic fbrosis (CF). Current devices are typically reusable and require daily, or often more frequent, cleaning to prevent risk of infection by acting as reservoirs of potentially pathogenic organisms.
9p
vijichoo2711
31-05-2021
11
1
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People with cystic fbrosis (pwCF) derive several physiological and psychological benefts from regular physical activity (PA), but the practice is lower than recommended. Knowledge about the facilitators of and barriers to PA at the individual level is important to act positively on PA behaviors.
9p
vianthony2711
16-04-2021
10
2
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While sleep disruption is a common complaint among children with cystic fbrosis (CF), only a few studies have investigated insomnia in adults. The aim of this study was to identify factors associated with insomnia in clinically stable adult CF patients.
10p
vianthony2711
16-04-2021
18
1
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