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1 r degeneration (AMD), diabetes mellitus, and cystic fibrosis).
2 logy of many respiratory diseases, including cystic fibrosis.
3 mbrane regulator (CFTR) mutation that causes cystic fibrosis.
4 sweat diagnostics with reliable detection of cystic fibrosis.
5 k and lower life expectancy in patients with cystic fibrosis.
6 yperoxaluria observed in this mouse model of cystic fibrosis.
7 Cystic fibrosis.
8 ss 23 sites of the lungs from a patient with cystic fibrosis.
9 6-mediated oxalate secretion is defective in cystic fibrosis.
10 underlying cause of disease in patients with cystic fibrosis.
11 del mutation, which is the dominant cause of cystic fibrosis.
12 ted with an aggressive clinical phenotype in cystic fibrosis.
13 k and lower life expectancy in patients with cystic fibrosis.
14 function decline and increased mortality in cystic fibrosis.
15 notably prevalent among young children with cystic fibrosis.
16 for further investigation as treatments for cystic fibrosis.
17 tation that is associated with mild forms of cystic fibrosis.
18 ch is associated with the pathophysiology of cystic fibrosis.
19 een increase airway surface liquid volume in cystic fibrosis.
20 ts with hospital-acquired infections or with cystic fibrosis.
21 ic validity of attenuating IL-17 activity in cystic fibrosis.
22 s of several respiratory diseases, including cystic fibrosis.
23 of-function chloride channelopathies such as cystic fibrosis.
24 te stone formation observed in patients with cystic fibrosis.
25 ision therapies for airway disorders such as cystic fibrosis.
26 ight be promising as co-adjuvant therapy for cystic fibrosis.
27 icted to be cost-effective for patients with cystic fibrosis.
28 tricted evidence available for patients with cystic fibrosis.
29 elopathies including cardiac arrhythmias and cystic fibrosis.
30 l CRC screening strategies for patients with cystic fibrosis.
32 of lumacaftor and ivacaftor in patients with cystic fibrosis aged 6-11 years homozygous for F508del-C
33 A longitudinal cohort study of patients with cystic fibrosis aged 6-21 years was conducted using the
35 sms fail in CFTR(-/-) swine, suggesting that cystic fibrosis airways do not respond to inhaled pathog
36 g 8 kg or more with a confirmed diagnosis of cystic fibrosis and a CFTR gating mutation on at least o
39 ic rhinosinusitis, and exacerbations of both cystic fibrosis and chronic obstructive pulmonary diseas
41 R) activity and lung function in people with cystic fibrosis and G551D-CFTR mutations but does not re
42 nts in patients aged 12 years and older with cystic fibrosis and homozygous for F508del-CFTR, but it
43 ible if they were at least 12 years old with cystic fibrosis and homozygous for the F508del-CFTR muta
44 acrophages in human lungs from patients with cystic fibrosis and induced in mouse macrophages in resp
46 teractions in A. fumigatus and patients with cystic fibrosis and the ongoing validation of novel labo
47 48 patients 12 years of age or older who had cystic fibrosis and were heterozygous for the Phe508del
48 in patients 12 years of age or older who had cystic fibrosis and were homozygous for the CFTR Phe508d
49 in patients 12 years of age or older who had cystic fibrosis and were homozygous for the CFTR Phe508d
50 lso to treat conformational diseases such as cystic fibrosis, and Alpha-1 antitrypsin deficiency.
57 onal and experimental approach to rescue the cystic-fibrosis-associated protein cystic fibrosis trans
60 ombinations may have therapeutic efficacy in cystic fibrosis caused by the W1282X mutation, although
66 They are major pathogens in patients with cystic fibrosis (CF) and can cause severe necrotizing pn
67 onization in chronic lung disease, including cystic fibrosis (CF) and chronic obstructive pulmonary d
68 mechanism in airways, and it is impaired in cystic fibrosis (CF) and other obstructive lung diseases
69 urkholderia dolosa caused an outbreak in the cystic fibrosis (CF) clinic at Boston Children's Hospita
70 erimental measurements made using normal and cystic fibrosis (CF) cultured human airway epithelium.
74 es are reminiscent of the pathophysiology of cystic fibrosis (CF) in which loss-of-function mutations
75 progression of lung disease in children with cystic fibrosis (CF) indicates that sensitive noninvasiv
82 teria (NTM) from the sputum of patients with cystic fibrosis (CF) is challenging due to overgrowth by
86 B-OprM efflux system, naturally occurring in cystic fibrosis (CF) isolates, have been previously show
88 mains an important pathogen in patients with cystic fibrosis (CF) lung disease as well as non-CF bron
90 ommunity of three temperate phages active in cystic fibrosis (CF) lung infections, including the tran
91 lize a panel of P. aeruginosa burn wound and cystic fibrosis (CF) lung isolates to demonstrate that P
92 respiratory virus infections predispose the cystic fibrosis (CF) lung to chronic bacterial colonizat
93 y adaptation during chronic infection of the cystic fibrosis (CF) lung, including reduced production
94 bacteria rarely reported in patients without cystic fibrosis (CF) or immunocompromising conditions.
95 istic pathogen that persists in the lungs of cystic fibrosis (CF) patients and may be responsible for
96 es chronic lung infections in the airways of cystic fibrosis (CF) patients as well as other immune-co
99 e been implemented for health care visits by cystic fibrosis (CF) patients in an attempt to prevent t
100 with pulmonary exacerbations, especially in cystic fibrosis (CF) patients, and the importance of thi
102 the effects of NBD2 mutations identified in cystic fibrosis (CF) patients, demonstrating that mutant
108 n strategies to prevent lung damage in early cystic fibrosis (CF) requires objective outcome measures
112 . aeruginosa lung infections associated with cystic fibrosis (CF) will be advanced by an improved und
113 ma membrane of secretory epithelia and cause cystic fibrosis (CF) with variable disease severity.
114 Akt signaling is suppressed in patients with cystic fibrosis (CF), a disease characterized by hyper-i
115 flammatory responses in mice and humans with cystic fibrosis (CF), a life-threatening disorder of the
117 ole in chronic inflammatory diseases such as cystic fibrosis (CF), and targeting ER stress may be use
118 rane conductance regulator (CFTR) gene cause cystic fibrosis (CF), but are not good predictors of lun
119 sweat is an important diagnostic marker for cystic fibrosis (CF), but the implementation of point-of
120 tly in the sputum of pediatric patients with cystic fibrosis (CF), by combining the high sensitivity
121 tracellular pathogen killing is defective in cystic fibrosis (CF), despite abundant production of rea
122 ), which is defective in the genetic disease cystic fibrosis (CF), forms a gated pathway for chloride
123 important global threat to individuals with cystic fibrosis (CF), in whom M. abscessus accelerates i
124 rin in inflammatory lung diseases, including cystic fibrosis (CF), perhaps by regulation of airway su
125 ion of universal newborn screening (NBS) for cystic fibrosis (CF), the timing and magnitude of growth
126 in sepsis, pneumonia, wound infections, and cystic fibrosis (CF), which is caused by mutations of th
127 e, but their potential role in patients with cystic fibrosis (CF)-associated lung disease remains unc
146 tural lung abnormalities in individuals with cystic fibrosis (CF); however, the associations between
149 ormation of the temperature-sensitive mutant cystic fibrosis channel (F508-CFTR) at the plasma membra
152 o reducing the detrimental health effects of cystic fibrosis could be the identification of proteins
154 138 mimic or siRNA against SIN3A to cultured cystic fibrosis (DeltaF508/DeltaF508) airway epithelia p
155 studies were performed in the context of the cystic fibrosis diagnosis and preliminary investigation
157 t chloride is of interest as a biomarker for cystic fibrosis, electrolyte metabolism disorders, elect
162 ian Cystic Fibrosis Registry (CCFR) and U.S. Cystic Fibrosis Foundation Patient Registry (CFFPR) betw
164 ood Institute/National Institutes of Health, Cystic Fibrosis Foundation, the University of Alabama at
165 sits in the first 12 months of life at 28 US Cystic Fibrosis Foundation-accredited Care Centers from
166 r mutant cells were grown as biofilms on the Cystic Fibrosis genotype bronchial epithelial cells.
167 muscular dystrophy, spinal muscular atrophy, cystic fibrosis, haemophilia and sickle cell disease.
170 al therapies that target the basic defect in cystic fibrosis have recently been developed and are eff
171 ed studies and patients aged 6-11 years with cystic fibrosis homozygous for F508del-CFTR in an open-l
173 cacy in patients aged 12 years or older with cystic fibrosis homozygous for F508del-cystic fibrosis t
176 Median age of survival in patients with cystic fibrosis increased in both countries between 1990
180 to tissue remodeling and respiratory disease.Cystic fibrosis is caused by mutations in the CFTR chlor
183 nstrate the in vivo contribution of IL-17 in cystic fibrosis lung disease and the therapeutic validit
185 ocepacia is an opportunistic pathogen of the cystic fibrosis lung that elicits a strong inflammatory
187 nome analyses of B. cenocepacia infection in cystic fibrosis lungs and serves as a valuable resource
190 s cathepsin C inhibitor for the treatment of cystic fibrosis, noncystic fibrosis bronchiectasis, ANCA
193 gladioli BCC0238, a clinical isolate from a cystic fibrosis patient, led to the discovery of gladiol
194 directed approach, we were able to generate cystic fibrosis patient-specific iPSC-derived airway org
197 ed the elevated sweat electrolyte content of cystic fibrosis patients compared with that of healthy c
198 coccus aureus-specific serum IgG compared to cystic fibrosis patients despite recurrent S. aureus inf
199 idly growing mycobacteria from the sputum of cystic fibrosis patients has recently been reported.
200 ed autophagy has previously been reported in cystic fibrosis patients with the common F508del-CFTR mu
201 tant strains of P. aeruginosa (isolated from cystic fibrosis patients) indicating a potential therape
202 ed Burkholderia cenocepacia isolates from 16 cystic fibrosis patients, spanning a period of 2-20 yr a
206 t least 15 kg, with a confirmed diagnosis of cystic fibrosis, percent predicted forced expiratory vol
207 bably the most relevant structural change in cystic fibrosis) peribronchial thickening, mucous pluggi
209 TTs), respectively, in pancreatic-sufficient cystic fibrosis (PS-CF), PI-CF, and normal control subje
211 e (+0.15; 95% CI, 0.08 to 0.22; P < 0.0001), Cystic Fibrosis Questionnaire-Revised respiratory domain
212 Scores on the respiratory domain of the Cystic Fibrosis Questionnaire-Revised, a quality-of-life
213 a well-established in vivo clinical test for cystic fibrosis, reflects transepithelial cation and ani
218 the median age of survival of patients with cystic fibrosis reported in the United States was 36.8 y
220 morphological abnormalities in patients with cystic fibrosis, such as bronchiectasis (which is progre
222 retion in wild-type but not in pig models of cystic fibrosis, suggesting an impaired response to path
224 To use a standardized approach to calculate cystic fibrosis survival estimates and to explore differ
226 caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFT
227 n autosomal recessive disorder affecting the cystic fibrosis transmembrane conductance regulator (CFT
228 modifies the local translation speed of the cystic fibrosis transmembrane conductance regulator (CFT
229 d selectivity against other proteins such as cystic fibrosis transmembrane conductance regulator (CFT
230 ase is caused by the loss of function of the cystic fibrosis transmembrane conductance regulator (CFT
231 letion of phenylalanine 508 (F508del) in the cystic fibrosis transmembrane conductance regulator (CFT
235 antivirals and as correctors of the F508del-cystic fibrosis transmembrane conductance regulator (CFT
236 kA), the alginate transporter (AlgE) and the cystic fibrosis transmembrane conductance regulator (CFT
239 cond nucleotide-binding domain (NBD2) of the cystic fibrosis transmembrane conductance regulator (CFT
240 ed phenylquinoxalinone CFTRact-J027 (4) as a cystic fibrosis transmembrane conductance regulator (CFT
241 ch are homologous to the gating mutations of cystic fibrosis transmembrane conductance regulator (CFT
243 t is in part regulated by apically expressed cystic fibrosis transmembrane conductance regulator (CFT
244 (IL-8) secretion and decreased apical cilia, cystic fibrosis transmembrane conductance regulator (CFT
245 not dependent upon special properties of the cystic fibrosis transmembrane conductance regulator (CFT
246 e determined the amino acids inserted at the cystic fibrosis transmembrane conductance regulator (CFT
248 is (CF), which is caused by mutations of the cystic fibrosis transmembrane conductance regulator (Cft
249 diseases.The F508 deletion (F508del) in the cystic fibrosis transmembrane conductance regulator (CFT
250 and specific domain interaction between the cystic fibrosis transmembrane conductance regulator (CFT
251 reased intestinal permeability and decreased cystic fibrosis transmembrane conductance regulator (Cft
253 tive degradation of the common mutant of the cystic fibrosis transmembrane conductance regulator (CFT
255 ector, rAAV2/HBoV1, expressing a full-length cystic fibrosis transmembrane conductance regulator (CFT
256 ent protein kinase (PKG), and opening of the cystic fibrosis transmembrane conductance regulator (CFT
257 cessfully to identify the interactome of the cystic fibrosis transmembrane conductance regulator (CFT
259 n genetic disease caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFT
260 irway epithelia partially restored DeltaF508-cystic fibrosis transmembrane conductance regulator (CFT
261 ng (PDE1), control of cell proliferation and cystic fibrosis transmembrane conductance regulator (CFT
262 caused by loss-of-function mutations of the cystic fibrosis transmembrane conductance regulator (CFT
263 (MRPs), and an ATP-gated anion channel, the cystic fibrosis transmembrane conductance regulator (CFT
264 ism of action of modulator compounds for the cystic fibrosis transmembrane conductance regulator (CFT
265 with cystic fibrosis homozygous for F508del-cystic fibrosis transmembrane conductance regulator (CFT
267 ystic fibrosis results from mutations in the cystic fibrosis transmembrane conductance regulator (CFT
268 nstrate that mice carrying the most frequent cystic fibrosis transmembrane conductance regulator (CFT
271 vious work indicates that ivacaftor improves cystic fibrosis transmembrane conductance regulator (CFT
272 caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFT
274 e expression, stability, and function of the cystic fibrosis transmembrane conductance regulator (CFT
276 as I507-ATC-->ATT, in deletion of Phe508 in cystic fibrosis transmembrane conductance regulator (Del
277 d hydrostatic pressure resulted in decreased cystic fibrosis transmembrane conductance regulator acti
278 uodenal HCO3(-) secretion appears to require cystic fibrosis transmembrane conductance regulator and
280 a secreted P. aeruginosa epoxide hydrolase, cystic fibrosis transmembrane conductance regulator inhi
281 n, an effect which was partially reversed by cystic fibrosis transmembrane conductance regulator pote
283 To determine the feasibility of using a cystic fibrosis transmembrane conductance regulator pote
284 quently caused by the retention of the CFTR (cystic fibrosis transmembrane conductance regulator) mut
285 escue the cystic-fibrosis-associated protein cystic fibrosis transmembrane conductance regulator, whi
286 e within the airway as a result of defective cystic fibrosis transmembrane receptor (CFTR) expression
288 d lower expression of chloride channel 2 and cystic fibrosis transmembrane regulator in diabetic corn
290 say for early identification of infants with cystic fibrosis was first recognised, the performance of
291 lidation cohort included adult patients with cystic fibrosis who had CT imaging performed between Jan
293 timal colonoscopy strategy for patients with cystic fibrosis who never received an organ transplant;
294 timal colonoscopy strategy for patients with cystic fibrosis who never received an organ transplant;
295 RCTs)-TRAFFIC and TRANSPORT-in patients with cystic fibrosis who were aged 12 years or older and homo
296 aftor alone was efficacious in patients with cystic fibrosis who were heterozygous for the Phe508del
297 ion therapy in patients aged 6-11 years with cystic fibrosis who were homozygous for F508del-CFTR.
298 s to be safe in children aged 2-5 years with cystic fibrosis with a gating mutation followed up for 2
300 t was seen predominantly in patients without cystic fibrosis with MAC and was sustained 1 year after
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