ライフサイエンスコーパス: cystic fibrosis

1 h underlying diseases including HIV/AIDS and cystic fibrosis.

2 gy of chronic lung infections in people with cystic fibrosis.

3 tract, such as inflammatory bowel disease or Cystic Fibrosis.

4 y within a few breath holds in patients with cystic fibrosis.

5 patients with inflammatory bowel disease or cystic fibrosis.

6 ch as pulmonary hypertension, hypoxemia, and cystic fibrosis.

7 constipation including that associated with cystic fibrosis.

8 rm decline of lung function in patients with cystic fibrosis.

9 s in patients with bronchiectasis not due to cystic fibrosis.

10 few options for a cure for all patients with cystic fibrosis.

11 t often infects open wounds or patients with cystic fibrosis.

12 al for its mucolytic action in patients with cystic fibrosis.

13 t unique host-pathogen interactions exist in cystic fibrosis.

14 en proposed as a target for the treatment of cystic fibrosis.

15 nhomogeneity-in children aged 3-6 years with cystic fibrosis.

16 to the development of secretory diarrhea and cystic fibrosis.

17 to control mucus-related pathologies such as cystic fibrosis.

18 liver transplant recipient who does not have cystic fibrosis.

19 n from respiratory secretions of people with cystic fibrosis.

20 unocompromised individuals and patients with cystic fibrosis.

21 e with burns, surgical wounds or people with cystic fibrosis.

22 olding are transforming the clinical care of cystic fibrosis.

23 resemble the airway defects associated with cystic fibrosis.

24 stone disease, urinary tract infection, and cystic fibrosis.

25 rane regulator characteristic of the disease cystic fibrosis.

26 y epithelial cells cultured from people with cystic fibrosis.

27 air respiratory host defences in people with cystic fibrosis(1-3).

28 CFTR mutations cause cystic fibrosis, a lethal incurable disease.

29 le ion channels can restore host defences in cystic fibrosis airway epithelia via a mechanism that is

30 upregulated in response to P. aeruginosa by cystic fibrosis airway epithelia.

31 s and Main Results: Ceramide is increased in cystic fibrosis airway epithelium owing to differential

32 show that in cystic fibrosis, airway gland mucus gels form under cond

33 igible if they had a documented diagnosis of cystic fibrosis and a minimum of two cystic fibrosis cli

34 enesis of T(H) 1-directed lung diseases like cystic fibrosis and acute lung injury.

35 olated from the sputum of an individual with Cystic Fibrosis and assembled in a fully factorial desig

36 in a multicentre paediatric population with cystic fibrosis and associated with reduced lung functio

37 as putative treatments for diseases such as cystic fibrosis and cancer.

38 le for significant morbidity in persons with cystic fibrosis and chronic granulomatous disease, repre

39 elastase (NE) activity in tissues, including cystic fibrosis and chronic obstructive pulmonary diseas

40 rged as a growing threat to individuals with cystic fibrosis and other pre-existing chronic lung dise

41 a homozygous disadvantage, as in the case of cystic fibrosis and sickle cell disease.

42 s: To investigate sphingolipid metabolism in cystic fibrosis and the effects of treatment with recomb

43 ts in a large burden of care for people with cystic fibrosis and their families.

44 pathogen and a common cause of infection in cystic fibrosis and ventilator-associated pneumonia and

45 management of lung diseases such as asthma, cystic fibrosis, and chronic obstructive pulmonary disea

46 a of asthma, chloride channel dysfunction of cystic fibrosis, and ciliary defects of primary ciliary

47 s, contribute to airway mucus obstruction in cystic fibrosis, and facilitate tumor metastasis after d

48 prevalent in chronic lung disease, including cystic fibrosis, and infections are characterized by neu

49 a variety of human diseases, such as cancer, cystic fibrosis, and inflammatory bowel diseases.

50 genetic testing supporting the diagnosis of cystic fibrosis, and the development of therapies target

51 ts living with the disease, complications of cystic fibrosis are becoming increasingly common.

52 coronary artery disease, multiple sclerosis, cystic fibrosis, asthma, cancer, neurological disorders,

53 le: Chronic azithromycin is commonly used in cystic fibrosis based on short controlled clinical trial

54 ble gap in health outcomes for patients with cystic fibrosis between high-income countries, and low-i

55 Rationale: Non-cystic fibrosis bronchiectasis is characterized by airwa

56 rtonic saline inhalation acutely reduced non-cystic fibrosis bronchiectasis mucus concentration by 5%

57 frequency, and clinical significance in non-cystic fibrosis bronchiectasis remain unclear.

58 Studies in patients with cystic fibrosis bronchiectasis were excluded.

59 recommended for long-term management of non-cystic fibrosis bronchiectasis with frequent exacerbatio

60 Eligible patients had non-cystic fibrosis bronchiectasis, had had at least two pul

61 In patients with non-cystic fibrosis bronchiectasis, lung infection with Pseu

62 l symptoms and restrict the complications of cystic fibrosis, but advances in CFTR modulator therapie

63 le for fragment-based drug discovery and the cystic fibrosis C2-corrector clinical candidate ABBV-322

64 es associated with the changing landscape of cystic fibrosis care and the opportunities available for

65 nd its delivery (section 2); the building of cystic fibrosis care globally (section 3); novel therape

66 iratory Medicine Commission on the future of cystic fibrosis care was established at a time of great

67 In considering the future of cystic fibrosis care, the Commission focused on five key

68 ess, providing a blueprint for the future of cystic fibrosis care.

69 ia cepacia complex and often associated with cystic fibrosis, carries a high mortality rate.

70 ultures of airway epithelia from people with cystic fibrosis caused by different mutations, including

71 nion channel activity conferred by the major cystic fibrosis-causing mutation, F508del, in in vitro s

72 essing and gating defects of the predominant cystic fibrosis-causing mutation.

73 ighly qualified professionals are present in cystic fibrosis centres to meet the needs of ageing pati

74 study of children aged 6-16 years at five US cystic fibrosis centres, using culture methods sensitive

75 le: Despite therapeutic progress in treating cystic fibrosis (CF) airway disease, airway inflammation

76 important implications in disorders such as cystic fibrosis (CF) and asthma.

77 Oral fluid from patients with cystic fibrosis (CF) and healthy controls (HCs) were stu

78 ied differentiated primary cultures of human cystic fibrosis (CF) and non-CF airway epithelia.

79 ve therapeutic approach for the treatment of cystic fibrosis (CF) and other mucoobstructive diseases.

80 Mutations in these genes cause cystic fibrosis (CF) and sickle cell disease (SCD), resp

81 Over the past 30 years, a range of cystic fibrosis (CF) animal models have been generated f

82 Animal models of cystic fibrosis (CF) are essential for investigating dis

83 ned survival in patients with advanced-stage cystic fibrosis (CF) are not included in the lung alloca

84 Children with cystic fibrosis (CF) can develop life-threatening infect

85 odulators provide no therapeutic benefit for cystic fibrosis (CF) caused by many loss-of-function mut

86 The gold standard for cystic fibrosis (CF) diagnosis is the determination of c

87 dministration, such as pulmonary delivery in cystic fibrosis (CF) disease, remains a significant chal

88 Patients with cystic fibrosis (CF) do not respond with increased urina

89 om a 1993-1997 cohort from the United States Cystic Fibrosis (CF) Foundation Patient Registry to asse

90 en Pseudomonas aeruginosa from patients with cystic fibrosis (CF) frequently contain mutations in the

91 ress in the development of new therapies for cystic fibrosis (CF) has benefited from therapeutically

92 Adults with cystic fibrosis (CF) have been reported to be at five to

93 Patients with cystic fibrosis (CF) have increased risk of vitamin D de

94 Most infants with cystic fibrosis (CF) have pancreatic exocrine insufficie

95 bination recently approved for patients with cystic fibrosis (CF) homozygous for the Phe508del mutati

96 nary exercise testing (CPET) for survival in cystic fibrosis (CF) in the context of current clinical

97 Cystic fibrosis (CF) is a familial autosomal recessive d

98 Cystic fibrosis (CF) is a genetic disease caused by muta

99 Cystic fibrosis (CF) is a genetic disease caused by muta

100 Cystic fibrosis (CF) is a genetic disorder caused by def

101 Cystic fibrosis (CF) is a genetic disorder of the epithe

102 Rationale: Cystic fibrosis (CF) is a life-shortening, multisystem h

103 Cystic fibrosis (CF) is a life-threatening autosomal rec

104 Cystic fibrosis (CF) is a life-threatening chronic infla

105 Cystic Fibrosis (CF) is a monogenic disease caused by mu

106 Cystic fibrosis (CF) is a monogenic disorder caused by m

107 Cystic Fibrosis (CF) is a multi-organ progressive geneti

108 Cystic fibrosis (CF) is a multiorgan disease caused by m

109 Cystic fibrosis (CF) is a multisystem disorder, but prog

110 Cystic fibrosis (CF) is an autosomal recessive disorder

111 Cystic fibrosis (CF) is caused by defective Cystic Fibro

112 Cystic fibrosis (CF) is caused by loss-of-function mutat

113 Cystic fibrosis (CF) is caused by mutations in the CF tr

114 Cystic fibrosis (CF) is caused by mutations in the cysti

115 The devastating inherited disease cystic fibrosis (CF) is caused by mutations of the Cysti

116 Cystic fibrosis (CF) is characterized by chronic bacteri

117 creasing life expectancy of individuals with Cystic Fibrosis (CF) is likely to be associated with new

118 The pathogenesis of airway infection in cystic fibrosis (CF) is poorly understood.

119 The leading cause of cystic fibrosis (CF) is the deletion of phenylalanine 50

120 Cystic fibrosis (CF) lung disease is characterized by an

121 e characteristics, onset, and progression of cystic fibrosis (CF) lung disease.

122 People with the genetic disease cystic fibrosis (CF) often carry a deletion mutation Del

123 ause severe lung infections in patients with cystic fibrosis (CF) or chronic granulomatous disease (C

124 Diabetes is associated with worse cystic fibrosis (CF) outcomes.

125 Chronic lung infection in cystic fibrosis (CF) patients by Staphylococcus aureus i

126 airway pathogens to enter the circulation of cystic fibrosis (CF) patients during chronic infective s

127 Cystic fibrosis (CF) patients experience heightened leve

128 is increasingly recognized as a colonizer of cystic fibrosis (CF) patients, but the role that A. xylo

129 species are opportunistic lung pathogens of cystic fibrosis (CF) patients.

130 at causes pulmonary disease, particularly in cystic fibrosis (CF) patients.

131 In cystic fibrosis (CF) pigs, loss of cystic fibrosis trans

132 Rationale: Cystic fibrosis (CF) pulmonary disease is characterized

133 their abundance and functional relevance in cystic fibrosis (CF) remain poorly understood.

134 Cystic fibrosis (CF) remains the most common life-shorte

135 study was that Hdac6 depletion would restore cystic fibrosis (CF) responses to bacterial challenge to

136 ator (CFTR) associated with a severe form of cystic fibrosis (CF) reveal the importance and heterogen

137 udomonas aeruginosa infection, especially in cystic fibrosis (CF) sufferers.

138 Median cystic fibrosis (CF) survival has increased dramatically

139 Diabetes is a common complication of cystic fibrosis (CF) that affects approximately 20% of a

140 Improvements in management of cystic fibrosis (CF) through specialist centres in the U

141 Protein interactions that stabilize the cystic fibrosis (CF) transmembrane conductance regulator

142 The role of epithelial cystic fibrosis (CF) transmembrane conductance regulator

143 formation in neutrophils from patients with cystic fibrosis (CF) was normal during early phagocytosi

144 the morbidity and mortality associated with cystic fibrosis (CF), a condition that predisposes patie

145 Cystic fibrosis (CF), a most deadly genetic disorder, is

146 nt localization to the plasma membrane cause cystic fibrosis (CF), an inherited and eventually lethal

147 thogen in burn patients and individuals with cystic fibrosis (CF), and a leading cause of nosocomial

148 is increasingly observed in patient sputa in cystic fibrosis (CF), and while existing epidemiology in

149 Conclusion In patients with cystic fibrosis (CF), automated quantification of lung M

150 Cystic fibrosis (CF), caused by mutations to CFTR, leads

151 In persons with cystic fibrosis (CF), decreased airway microbial diversi

152 ted with Pseudomonas aeruginosa infection in cystic fibrosis (CF), non-CF bronchiectasis (BE), and ch

153 Background In patients with cystic fibrosis (CF), pulmonary structures with high MRI

154 Background In cystic fibrosis (CF), recurrent imaging and pulmonary fu

155 Autosomal recessive diseases, such as cystic fibrosis (CF), require inheritance of 2 mutated g

156 on in some early infections in children with Cystic Fibrosis (CF), suggesting these isolates may have

157 Cystic fibrosis (CF)-related liver disease (CFLD) is a c

158 rotein that is defective in individuals with cystic fibrosis (CF).

159 s of growing clinical concern in people with cystic fibrosis (CF).

160 c obstructive pulmonary disease, asthma, and cystic fibrosis (CF).

161 used to improve lung health in patients with cystic fibrosis (CF).

162 ronic infections in the lungs of people with cystic fibrosis (CF).

163 recruited to the airways of individuals with cystic fibrosis (CF).

164 ve ventilation measurements in patients with cystic fibrosis (CF).

165 CFTR) disrupt epithelial secretion and cause cystic fibrosis (CF).

166 y diarrheas, whereas CFTR mutations underlie cystic fibrosis (CF).

167 els that underlie long QT syndrome (LQT) and cystic fibrosis (CF).

168 side-induced nephrotoxicity in children with Cystic Fibrosis (CF).

169 lent respiratory infections in children with cystic fibrosis (CF).

170 circulations have been identified in asthma, cystic fibrosis, chronic thromboembolism and primary car

171 osis of cystic fibrosis and a minimum of two cystic fibrosis clinic visits and two respiratory cultur

172 se of serious infections in individuals with cystic fibrosis, compromised immune systems, or severe b

173 ve inhaled Antibiotics in Bronchiectasis and Cystic Fibrosis Consortium, European Respiratory Society

174 epithelial cells derived from subjects with cystic fibrosis (DeltaF508/DeltaF508 and DeltaF508/-) ba

175 fic therapeutic targets include scleroderma, cystic fibrosis, dermatomyositis, and lupus, all of whic

176 Notably, CFTR potentiators used to treat cystic fibrosis effectively rescue CFTR function and mar

177 r necrosis factor receptor 1 is increased in cystic fibrosis epithelia and activates NF-kappaB signal

178 ll tolerated in children aged 2-5 years with cystic fibrosis for 24 weeks.

179 The Cystic Fibrosis Foundation and the National Institutes o

180 ic Registry of Transplant Recipients and the Cystic Fibrosis Foundation Patient Registry.

181 his retrospective cohort study used the U.S. cystic fibrosis Foundation Patient Registry.

182 er, although life expectancy for people with cystic fibrosis has increased substantially, the disease

183 tor therapies to address the basic defect of cystic fibrosis have been remarkable and the field is ev

184 phangioleiomyomatosis, Loeys-Dietz syndrome, cystic fibrosis, homocystinuria, and cutis laxa, among o

185 FTR corrector and potentiator in people with cystic fibrosis homozygous for the F508del mutation.

186 shed in patients aged 6 years and older with cystic fibrosis, homozygous for the F508del-CFTR mutatio

187 , chronic obstructive pulmonary disease, and cystic fibrosis; however, its presence, frequency, and c

188 As the median age of patients with cystic fibrosis increases, with a rapid increase in the

189 cal studies suggest that airway infection in cystic fibrosis initiates with Staphylococcus aureus and

190 Cystic fibrosis is a condition caused by mutations in th

191 Cystic fibrosis is characterized by dehydration of the a

192 Since the main organ that is affected by cystic fibrosis is the lung, the delivery of drugs direc

193 ucus obstruction and related phenotypes in a cystic fibrosis-like lung disease model (i.e., Scnn1b-Tg

194 enic (Scnn1b-Tg(+)) mice, which recapitulate cystic fibrosis-like mucoinflammatory airway disease, de

195 Using the cystic fibrosis lung as an example, we cultured an avera

196 dase ameliorates the two pivotal features of cystic fibrosis lung disease, inflammation and infection

197 rom biofilm-associated infections, including cystic fibrosis lung infection(4), as well as medical de

198 rincipal pathogens associated with wound and cystic fibrosis lung infections.

199 Culture-independent studies of cystic fibrosis lung microbiota have provided few mechan

200 (desert soil biocrust wetting) and clinical (cystic fibrosis lung) examples, our ability to recover m

201 tection of bacteria in explanted whole human Cystic Fibrosis lungs.

202 In contrast to cystic fibrosis macrophages, smoke-exposed THP-1 and AMP

203 hildren: infective pulmonary exacerbation of cystic fibrosis (n=2), gastroenteritis viral (n=1), and

204 ough morphotypes of M. abscessus However, in cystic fibrosis neutrophils, wortmannin inhibited killin

205 ry airway diseases such as asthma, COPD, and cystic fibrosis, novel strategies are needed to avoid ke

206 dies in the past two decades have shown that cystic fibrosis occurs and is more frequent than was pre

207 duces pulmonary exacerbations in people with cystic fibrosis older than age 6 years.

208 uses on pulmonary disease in adults (without cystic fibrosis or human immunodeficiency virus infectio

209 Patients with bronchiectasis due to cystic fibrosis or traction bronchiectasis associated wi

210 nicity of this bacterium in individuals with cystic fibrosis, our results highlight that the O(2)-ind

211 d in this way to measure CFTR function using cystic fibrosis patient-derived iPSC lines before and af

212 as of a nonresistance strain isolated from a cystic fibrosis patient.

213 evaluated on respiratory specimens from non-cystic fibrosis patients and compared to the mycobacteri

214 ude exhaled breath condensate collected from cystic fibrosis patients as well as in vitro-cultured hu

215 model, and in sputum samples recovered from cystic fibrosis patients that contain multiple mixed bac

216 gladioli strains isolated from the lungs of cystic fibrosis patients were found to produce unusual l

217 Eight cystic fibrosis patients with A. xylosoxidans were treat

218 um abscessus are increasing in prevalence in cystic fibrosis patients.

219 also a risk factor for lung exacerbations in cystic fibrosis patients.

220 within the extracellular DNA of sputum from cystic fibrosis patients.

221 ctions and are the leading cause of death in cystic fibrosis patients.

222 and BCC1622, both isolated from the lungs of cystic fibrosis patients.

223 aracteristics of this pathogen among the non-cystic fibrosis population and the importance of early r

224 estral haplotype and delayed colonization in Cystic Fibrosis, postulating that downregulation of RNF5

225 nce of Mendelian inheritance, for example in cystic fibrosis, primary ciliary dyskinesia (PCD), and s

226 ently, Food and Drug Administration-approved cystic fibrosis protein trafficking chaperone, lumacafto

227 (AC), to quantify the aggregation levels of cystic fibrosis Pseudomonas aeruginosa (CF-PA) isolates

228 63% lower, a respiratory domain score on the Cystic Fibrosis Questionnaire-Revised (range, 0 to 100,

229 s were absolute change in sweat chloride and Cystic Fibrosis Questionnaire-Revised respiratory domain

230 n of small-colony variant prevalence data in cystic fibrosis registries, should be considered for ong

231 Cystic fibrosis-related diabetes (CFRD) worsens CF lung

232 in mucoinflammatory airway diseases, such as cystic fibrosis, remain unknown.

233 dam Annotated Grid Morphometric Analysis for Cystic Fibrosis scoring system.

234 in this report: the changing epidemiology of cystic fibrosis (section 1); future challenges of clinic

235 g to develop sgNIPTs of sickle cell disease, cystic fibrosis, spinal muscular atrophy, alpha-thalasse

236 However, in cystic fibrosis, stimulating submucosal glands has the o

237 untreated neutrophils from either healthy or cystic fibrosis subjects.

238 lian Respiratory Early Surveillance Team for Cystic Fibrosis surveillance program between 2000 and 20

239 Rationale: In cystic fibrosis the major cause of morbidity and mortali

240 haled antibiotics is the standard of care in cystic fibrosis, there is insufficient evidence to suppo

241 noz and Yu et al. explored the role that the cystic fibrosis transmembrane conductance regulator (CFT

242 (-) drinking test to assess the role of the cystic fibrosis transmembrane conductance regulator (CFT

243 The cystic fibrosis transmembrane conductance regulator (CFT

244 The cystic fibrosis transmembrane conductance regulator (CFT

245 is is a condition caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFT

246 acterizations of three nonsense mutations of cystic fibrosis transmembrane conductance regulator (CFT

247 Cigarette smoke decreased AMPhi cystic fibrosis transmembrane conductance regulator (CFT

248 ultiorgan disease caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFT

249 Loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator (CFT

250 The cystic fibrosis transmembrane conductance regulator (CFT

251 proved methods are needed to reliably assess Cystic Fibrosis Transmembrane Conductance Regulator (CFT

252 Cystic fibrosis transmembrane conductance regulator (CFT

253 irst nucleotide-binding domain (NBD1) of the cystic fibrosis transmembrane conductance regulator (CFT

254 opment of therapies targeting defects in the cystic fibrosis transmembrane conductance regulator (CFT

255 (-) secretion, through the chloride channels cystic fibrosis transmembrane conductance regulator (CFT

256 hannels involved in Cl(-) extrusion, such as cystic fibrosis transmembrane conductance regulator (CFT

257 onogenic disorder caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFT

258 A pathway for cystic fibrosis transmembrane conductance regulator (CFT

259 demonstrated that VX-809, a corrector of the cystic fibrosis transmembrane conductance regulator (CFT

260 ER) protein that regulates the biogenesis of cystic fibrosis transmembrane conductance regulator (CFT

261 Cystic fibrosis (CF) is caused by defective Cystic Fibrosis Transmembrane Conductance Regulator (CFT

262 f the trypsin inhibitor gene (SPINK1) or the cystic fibrosis transmembrane conductance regulator (CFT

263 irst nucleotide-binding domain (NBD1) of the cystic fibrosis transmembrane conductance regulator (CFT

264 The cystic fibrosis transmembrane conductance regulator (CFT

265 genetic disease caused by loss of functional cystic fibrosis transmembrane conductance regulator (CFT

266 ) is caused by loss-of-function mutations of cystic fibrosis transmembrane conductance regulator (CFT

267 enoceptor (beta(2)AR)-mediated activation of cystic fibrosis transmembrane conductance regulator (CFT

268 monogenic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFT

269 Mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFT

270 (cAMP)-mediated active Cl- secretion via the cystic fibrosis transmembrane conductance regulator (CFT

271 The cystic fibrosis transmembrane conductance regulator (CFT

272 fibrosis (CF) is caused by mutations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFT

273 ed by many loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator (CFT

274 at increasing arginine would enhance F508del-cystic fibrosis transmembrane conductance regulator (CFT

275 creting goblet cells, motile ciliated cells, cystic fibrosis transmembrane conductance regulator (CFT

276 The Cystic Fibrosis Transmembrane Conductance Regulator (CFT

277 fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFT

278 d chloride channels (TMEM16A), including the cystic fibrosis transmembrane conductance regulator (CFT

279 In cystic fibrosis (CF) pigs, loss of cystic fibrosis transmembrane conductance regulator (CFT

280 Reduced expression of cystic fibrosis transmembrane conductance regulator and

281 ain whether an acidic pH produced by loss of cystic fibrosis transmembrane conductance regulator anio

282 hase 1, sodium/glucose co-transporter-1, and cystic fibrosis transmembrane conductance regulator in t

283 that CF is caused by mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gen

284 Rationale: Lumacaftor-ivacaftor is a CFTR (cystic fibrosis transmembrane conductance regulator) mod

285 ut does not replicate a human-relevant CFTR (cystic fibrosis transmembrane conductance regulator) var

286 Rationale: Enhancing non-CFTR (cystic fibrosis transmembrane conductance regulator)-med

287 n essential cargo for lumen formation, CFTR (cystic fibrosis transmembrane conductance regulator).

288 n 19, epithelial cellular adhesion molecule, cystic fibrosis transmembrane conductance regulator, and

289 cal resistance (TEER) (>400 Ohms.cm(2)), and cystic fibrosis transmembrane conductance regulator-medi

290 ed efficiency and specificity to correct the cystic fibrosis transmembrane regulator (CFTR) function

291 ly lower expression of the gene encoding the cystic fibrosis transmembrane regulator characteristic o

292 c Reticulum-associated degradation (ERAD) of Cystic fibrosis transmembrane-conductance regulator (CFT

293 Conclusion In lungs with cystic fibrosis, ultrashort echo time oxygen-enhanced MR

294 Studies in cystic fibrosis were excluded.

295 d 36-72 months; had a confirmed diagnosis of cystic fibrosis; were able to comply with medication use

296 rovements in health outcomes for people with cystic fibrosis, which was once a fatal disease of infan

297 ive improvements in the lives of people with cystic fibrosis who are homozygous for the F508del mutat

298 at enrolment, with a confirmed diagnosis of cystic fibrosis who were homozygous for the F508del-CFTR

299 or in patients 12 years of age or older with cystic fibrosis with Phe508del-minimal function genotype

300 r-ivacaftor was efficacious in patients with cystic fibrosis with Phe508del-minimal function genotype