[PDF] Top 20 BPIFB1 (LPLUNC1) is upregulated in cystic fibrosis lung disease
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BPIFB1 (LPLUNC1) is upregulated in cystic fibrosis lung disease
... the disease. As mentioned previously, BPIFA1, but not BPIFB1, has been shown to inhibit the biochemical activation of ENaC sodium channels by preventing pro- teolytic processing and therefore activation of ... See full document
10
Neutrophil elastase and matrix metalloproteinase 12 in cystic fibrosis lung disease
... structural lung damage and that other factors contribute to emphysema forma- tion in βENaC-Tg mice ...the lung tissues was used as a bottom-up approach to search for candidate genes and pathways responsible ... See full document
7
Impact of pseudomonas aeruginosa Liverpool epidemic strain (LES) on cystic fibrosis patients
... with lung disease, most notably CF ...CF lung is a very hostile and heterogeneous environment in which the colonising microorganisms have to adapt to ... See full document
171
Lung function imaging methods in Cystic Fibrosis pulmonary disease
... Europe Cystic Fibrosis affects 1 to 3500 new-borns ...is lung disease, it is critical that we improve pulmonary diagnosis in patients to better predict adverse events and monitor therapies ... See full document
11
Myocardial infarction in an adult with cystic fibrosis and heart and lung transplant
... The most effective treatment of CTV is prevention and modification of risk factors using antihypertensive agents (e.g. ACE inhibitors and calcium channel blockers), statins (e.g. pravastatin) and strict diabetic control. ... See full document
5
Mycobacterial infections in a large Virginia hospital, 2001 2009
... NTM lung species, including age, gender, chronic obstructive pulmonary disease, cystic fibrosis, other lung diseases, diabetes, end stage renal disease, collagen vascular ... See full document
7
Potential of anti-inflammatory treatment for cystic fibrosis lung disease
... Abstract: Cystic fibrosis (CF) is the most common life-shortening genetic disorder in Caucasians. With improved diagnosis and treatment, survival has steadily increased. Unfortunately, the overwhelming ... See full document
14
No benefit of longer eradication therapy of Pseudomonas aeruginosa primoinfections in pediatric cystic fibrosis
... acute lung disease were excluded ...with cystic fibrosis was recently discussed [15– ...the lung func- tion (FEV1) before implementation of the eradication protocol were similar to ... See full document
6
Molecular screening of R117H mutation in non caucasian cystic fibrosis patients in the north of Iran
... including lung epithelium, submucosal gland of intestine, stomach, pancreas, gallbladder, liver, sweat ducts and reproductive ...obstructive lung disease and chronic bacterial infections leading to ... See full document
6
Effect of Shear Stress on Pseudomonas aeruginosa Isolated from the Cystic Fibrosis Lung
... be upregulated during growth of ...significantly upregulated in response to growth under low fluid shear conditions (see Table S1 in the supplemental ...be upregulated under envelope stress con- ... See full document
16
The innate immune system in cystic fibrosis lung disease
... An assessment of the relative contribution of individ- ual proteins or peptides to innate immune responses is quite challenging. Biochemical methods have been used to isolate and detect the molecules from biological sam- ... See full document
6
DNA methylation at modifier genes of lung disease severity is altered in cystic fibrosis
... HMOX1 encodes a protein that is important for iron homeostasis and cell protection from oxidative damage during stress. Activating and repressive factors regulate the HMOX1 basal expression by interacting with the ... See full document
15
Bacterial activity in cystic fibrosis lung infections
... In certain cases, the T-RFLP profile and RT-T-RFLP profile were found by visual comparison to be similar. To provide a more robust analysis, Hierarchical Cluster Analysis (HCA) was used. HCA demonstrated that there was ... See full document
12
Challenging the diagnosis of Cystic Fibrosis in a patient carrying the 186-8T/C allelic variant in the CF Transmembrane Conductance Regulator gene
... Here we report the clinical case of a 38 year-old Italian woman who, since 2010, had been treated as affected by CF in a European CF Center. This had followed diagnosis based on repeated sweat tests (chloride 60 and 74 ... See full document
7
Inhaled protein/peptide-based therapies for respiratory disease
... pulmonary disease (COPD), and cystic fibrosis (CF) are all chronic pulmonary diseases, albeit with different etiologies, that are characterized by airflow limitation, chronic inflammation, and ... See full document
5
Complex two gene modulation of lung disease severity in children with cystic fibrosis
... CF lung disease by 2 genes, MBL2 and TGFB1, as well as providing evidence for their interaction in controlling clinical variation of pulmonary phenotype in pediatric CF ... See full document
11
Determinants of exercise capacity in cystic fibrosis patients with mild-to-moderate lung disease
... The patients had a range of disease severities and were recruited from four centers in France. Patients from dif- ferent centers all had characteristics consistent with the French CF Registry 2009 Report [28] and ... See full document
8
Epithelial mesenchymal transition (EMT): a universal process in lung diseases with implications for cystic fibrosis pathophysiology
... Pulmonary Disease (COPD) results from chronic inflammation, pulmonary remodeling, per- manent airflow obstruction, and air trapping that leads to difficulties breathing [78, ... See full document
10
Hemorrhagic Retinopathy in a Patient With Cystic Fibrosis
... A 21-year-old woman with a history of chronic lung disease secondary to cystic fibrosis (GF) developed an acute hemorrhagic retinopathy during exposure to moder-.. ately high altitude.[r] ... See full document
5
Nutritional advances in patients with respiratory diseases
... in disease management from diagnosis and treatment to prediction and ...for disease prevention but also contributing to heterogeneity in disease progression and ...pulmonary disease (COPD), ... See full document
6
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