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References
- Cystic fibrosis.Lancet. 2021; 397: 2195-2211
Cystic Fibrosis Foundation Patient Registry 2020 Annual Data Report Bethesda, Maryland ©2021 Cystic Fibrosis Foundation.
- Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.N Engl J Med. 2019; 381: 1809-1819
- Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial.Lancet. 2019; 394: 1940-1948
- Rapid improvement after starting elexacaftor-tezacaftor-ivacaftor in patients with cystic fibrosis and advanced pulmonary disease.Am J Respir Crit Care Med. 2021; https://doi.org/10.1164/rccm.202011–204153OC
- Efficacy of elexacaftor/tezacaftor/ivacaftor in advanced cystic fibrosis lung disease.Ann Am Thorac Soc. 2021; https://doi.org/10.1513/AnnalsATS.202102–202220RL
- Elexacaftor-tezacaftor-ivacaftor: the first triple-combination cystic fibrosis transmembrane conductance regulator modulating therapy.J Pediatr Pharmacol Ther. 2020; 25: 192-197
- Patient perspectives following initiation of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis and advanced lung disease.Respir Med. 2021; 80: 100829
- Cystic fibrosis transmembrane conductance receptor modulator therapy in cystic fibrosis, an update.Curr Opin Pediatr. 2020; 32: 384-388
- Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis.J Cyst Fibros. 2021; 20: 460-463
- Clinical effectiveness of elexacaftor/Tezacftor/ivacaftor in people with cystic fibrosis.Am J Respir Crit Care Med. 2021; https://doi.org/10.1164/rccm.202108–1986OC
- Gender gap in cystic fibrosis mortality.Am J Epidemiol. 1997; 145: 794-803
- A prospective study of the effects of sex hormones on lung function and inflammation in women with cystic fibrosis.Ann Am Thorac Soc. 2021; https://doi.org/10.1513/AnnalsATS.202008–1064OC
- Gender differences in cystic fibrosis: Pseudomonas aeruginosa infection.J Clin Epidemiol. 1995; 48: 1041-1049
- Determinants of mortality from cystic fibrosis in Canada, 1970–1989.Am J Epidemiol. 1996; 143: 1007-1017
- Effect of estrogen on pseudomonas mucoidy and exacerbations in cystic fibrosis.N Engl J Med. 2012; 366: 1978-1986
- Immune, inflammatory and infectious consequences of estrogen in women with cystic fibrosis.Expert Rev Respir Med. 2012; 6: 573-575
- Effects of puberty on cystic fibrosis related pulmonary exacerbations in women versus men.Pediatr Pulmonol. 2014; 49: 28-35
- Females with cystic fibrosis demonstrate a differential response profile to ivacaftor compared with males.Am J Respir Crit Care Med. 2020; 201: 996-998
- Correlation of sweat chloride and percent predicted FEV1 in cystic fibrosis patients treated with ivacaftor.J Cyst Fibros. 2017; 16: 41-44
- Females with cystic fibrosis have a larger decrease in sweat chloride in response to lumacaftor/ivacaftor compared to males.J Cyst Fibros. 2021; 20: e7-e11
- Elexacaftor/tezacaftor/ivacaftor improved clinical outcomes in an N1303K-CFTR patient based on in vitro Experimental evidence.Am J Respir Crit Care Med. 2021; https://doi.org/10.1164/rccm.202101–0090LE
- Triple therapy for cystic fibrosis Phe508del–gating and –residual function Genotypes.N Engl J Med. 2021; 385: 815-825
- Standards for the clinical care of children and adults with cystic fibrosis in the UK–.Cystic Fibrosis Trust. 2011; 1: 46
- ECFS best practice guidelines: the 2018 revision.J Cyst Fibros. 2018; 17: 153-178
- Patient and provider experience with cystic fibrosis Telemedicine clinic.Front Pediatr. 2021; 9: 784692
- Real-world outcomes in cystic fibrosis Telemedicine clinical care in a time of a Global pandemic.Chest. 2021; https://doi.org/10.1016/j.chest.2021.11.035
- Telemedicine and cystic fibrosis: do we still need face-to-face clinics?.Paediatr Respir Rev. 2021; https://doi.org/10.1016/j.prrv.2021.05.002
- A Feasibility study of Urgent Implementation of cystic fibrosis multidisciplinary Telemedicine clinic in the face of COVID-19 pandemic: single-Center experience. Telemed.J E Health. 2020; 26: 978-984
- Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations.J Cyst Fibros. 2019; 18: 102-109
- Disease progression in patients with cystic fibrosis treated with ivacaftor: data from national US and UK registries.J Cyst Fibros. 2020; 19: 68-79
- Clinical characteristics of Pseudomonas and Aspergillus co-infected cystic fibrosis patients: a UK registry study.J Cyst Fibros. 2021; https://doi.org/10.1016/j.jcf.2021.04.007
- Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function.J Cyst Fibros. 2021; 20: 220-227
- Neutrophil elastase activity is associated with exacerbations and lung function decline in bronchiectasis.Am J Respir Crit Care Med. 2017; 195: 1384-1393
- Association between pulmonary function and sputum biomarkers in cystic fibrosis.Am J Respir Crit Care Med. 2007; 175: 822-828
- Restoring cystic fibrosis transmembrane conductance regulator function reduces airway bacteria and inflammation in people with cystic fibrosis and chronic lung infections.Am J Respir Crit Care Med. 2017; 195: 1617-1628
- Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.Am J Respir Crit Care Med. 2014; 190: 175-184
- Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2–5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study.Lancet Respir Med. 2016; 4: 107-115
- An open-label extension study of ivacaftor in children with CF and a CFTR gating mutation initiating treatment at age 2–5 years (KLIMB).J Cyst Fibros. 2019; 18: 838-843
- Understanding the natural progression in %FEV1decline in patients with cystic fibrosis: a longitudinal study.Thorax. 2012; 67: 860-866
- Predicting disease progression in cystic fibrosis.Expert Rev Respir Med. 2018; 12: 905-917
- Sensitivity of lung clearance index and chest computed tomography in early cf lung disease.Respir Med. 2010; 104: 1834-1842
- Comparison of lung clearance index and magnetic resonance imaging for assessment of lung disease in children with cystic fibrosis.Am J Respir Crit Care Med. 2017; 195: 349-359
- Lung clearance index: evidence for use in clinical trials in cystic fibrosis.J Cyst Fibros. 2014; 13: 123-138
- Longitudinal assessment of lung clearance index to monitor disease progression in children and adults with cystic fibrosis.Thorax. 2021; https://doi.org/10.1136/thoraxjnl-2021–216928
- Effect of lumacaftor-ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis: results from the PROSPECT MCC sub-study.J Cyst Fibros. 2021; https://doi.org/10.1016/j.jcf.2021.05.004
- Toward the establishment of new clinical endpoints for cystic fibrosis: the role of lung clearance index and cardiopulmonary exercise testing.Front Pediatr. 2021; 9: 635719
- WS7. 6 Effect of ivacaftor on lung function in subjects with CF who have the G551D-CFTR mutation and mild lung disease: a comparison of lung clearance index (LCI) vs. spirometry.J Cyst Fibros. 2012; S15
Amin R, Subbarao P, Jabar A, Balkovec S, Jensen R, Kerrigan S, Gustafsson P, Ratjen F. Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function. Thorax. 2010 May;65(5):379-83. doi:10.1136/thx.2009.125831. PMID: 20435858.
- Withdrawal of dornase alfa increases ventilation inhomogeneity in children with cystic fibrosis.J Cystic Fibrosis. 2021; https://doi.org/10.1016/j.jcf.2021.02.004
- The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis.Eur Respir J. 2011; 37: 806-812
- Effect of hypertonic saline on lung clearance index in infants and preschool children with cf: a pilot study: 223.Pediatr Pulmonol. 2012; 47: 301-302
- Lung clearance index is a sensitive, repeatable and practical measure of airways disease in adults with cystic fibrosis.Thorax. 2008; 63: 135-140
- Multiple-breath washout as a marker of lung disease in preschool children with cystic fibrosis.Am J Respir Crit Care Med. 2005; 171: 249-256
- Lung clearance index in adult cystic fibrosis patients: the role of convection-dependent lung units.Eur Respir J. 2013; 42: 380-388
- Airway inflammation in cystic fibrosis: molecular mechanisms and clinical implications.Thorax. 2013; 68: 1157-1162
- Multiple-breath washout outcomes are sensitive to inflammation and infection in children with cystic fibrosis.Ann Am Thorac Soc. 2017; 14: 1436-1442
- Low levels of exhaled nitric oxide are associated with impaired lung function in cystic fibrosis.Pediatr Pulmonol. 2010; 45: 241-248
- Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation.Thorax. 2013; 68: 532-539
- Lung clearance index in the assessment of airways disease.Respir Med. 2009; 103: 793-799
- Peripheral airway involvement in CF and asthma compared by inert gas washout.Pediatr Pulmonol. 2007; 42: 168-176
- Abnormal preschool Lung Clearance Index (LCI) reflects clinical status and predicts lower spirometry later in childhood in cystic fibrosis.J Cyst Fibros. 2019; 18: 721-727
- Lung clearance index at 4 years predicts subsequent lung function in children with cystic fibrosis.Am J Respir Crit Care Med. 2011; 183: 752-758
- An observational study of the lung clearance index throughout childhood in cystic fibrosis: early years matter.Eur Respir J. 2020; 56: 2000006
- In cystic fibrosis, lung clearance index is sensitive to detecting abnormalities appearing at exercise in children with normal spirometry.Respir Physiol Neurobiol. 2018; 247: 9-11
- Can LCI predict new Pseudomonas aeruginosa colonization among CF patients?.Eur Respir J. 2017; 50
- Clinical value of lung clearance index (LCI) among patients with cystic fibrosis.Eur Respir J. 2014; 44
- Lung clearance index to Track acute respiratory Events in school-age children with cystic fibrosis.Am J Respir Crit Care Med. 2021; 203: 977-986
- Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D- CFTR mutation and preserved spirometry: a randomised controlled trial.Lancet Respir Med. 2013; 1: 630-638
- A phase 3, double-blind, parallel-group study to evaluate the efficacy and safety of tezacaftor in combination with ivacaftor in participants 6 through 11 years of age with cystic fibrosis homozygous for F508del or heterozygous for the F508del-CFTR mutation and a residual function mutation.J Cystic Fibrosis. 2021; 20: 68-77
- Changes in LCI in F508del/F508del patients treated with lumacaftor/ivacaftor: results from the prospect study.J Cyst Fibros. 2020; 19: 931-933
- A phase 3 open-label study of elexacaftor/tezacaftor/ivacaftor in children 6 through 11 years of age with cystic fibrosis and at least one F508del allele. Am. J. Respir. Crit.Care Med. 2021; 203: 1522-1532
- Natural variability of clinically measured lung clearance index in children with cystic fibrosis.Eur Respir J. 2021; 58
- Variability of lung clearance index in clinically stable cystic fibrosis lung disease in school age children.J Cystic Fibrosis. 2018; 17: 236-241
- Air trapping on chest CT is associated with worse ventilation distribution in infants with cystic fibrosis diagnosed following newborn screening.PLoS One. 2011; 6: e23932
- High resolution computed tomography of the chest in cystic fibrosis (CF): is simplification of scoring systems feasible?.Eur Radiol. 2008; 18: 538-547
- Chest imaging in cystic fibrosis studies: what counts, and can be counted?.J Cyst Fibros. 2017; 16: 175-185
- Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis.Eur Respir J. 2004; 23: 93-97
- Computed tomography in the evaluation of cystic fibrosis lung disease.Am J Respir Crit Care Med. 2005; 172: 1246-1252
- Lung Clearance Index and HRCT are complementary markers of lung abnormalities in young children with CF.Thorax. 2011; 66: 481-488
- Tracking CF disease progression with CT and respiratory symptoms in a cohort of children aged 6-19 years.Pediatr Pulmonol. 2014; 49: 1182-1189
- Lung clearance index and structural lung disease on computed tomography in early cystic fibrosis.Am J Respir Crit Care Med. 2016; 193: 60-67
- PRAGMA-CF. A quantitative structural lung disease computed tomography outcome in young children with cystic fibrosis.Am J Respir Crit Care Med. 2015; 191: 1158-1165
- The short-term effects of ORKAMBI (lumacaftor/ivacaftor) on regional and distal lung structures using functional respiratory imaging.Ther Adv Respir Dis. 2021; 15 (17534666211046774)
- High-resolution computed tomography of the chest in children with cystic fibrosis: support for use as an outcome surrogate.Pediatr Radiol. 1999; 29: 731-735
- High-resolution computed tomography in young patients with cystic fibrosis: distribution of abnormalities and correlation with pulmonary function tests.J Pediatr. 2004; 145: 32-38
- Effect of bronchoalveolar lavage–Directed therapy on Pseudomonas aeruginosa infection and structural lung Injury in children with cystic fibrosis: a randomized trial.JAMA. 2011; 306: 163-171
- Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis.Eur Respir J. 2013; 42: 371-379
- WS08. 3 CF-CT and PRAGMA-CF scoring techniques compared using quantification with objective airway and artery dimensions of children with cystic fibrosis.J Cyst Fibros. 2016; 1: S13
- Radiation exposure from CT scans in childhood and subsequent risk of leukaemia and brain tumours: a retrospective cohort study.Lancet. 2012; 380: 499-505
- Inhaled hypertonic saline in preschool children with cystic fibrosis (SHIP): a multicentre, randomised, double-blind, placebo-controlled trial.Lancet Respir Med. 2019; 7: 802-809
- 539: effect of inhaled hypertonic saline on structural lung disease in preschool children with cystic fibrosis. The SHIP-CT study.J Cyst Fibros. 2021; 20: S255
- P058 the effect of hypertonic saline treatment in pre-schoolers with cystic fibrosis on lung structure as measured by chest computed tomography. SHIP-CT study.J Cyst Fibros. 2021; 20: S56
- Lung morphology assessment of cystic fibrosis using MRI with ultra-short echo time at submillimeter spatial resolution.Eur Radiol. 2016; 26: 3811-3820
- Current state of the art MRI for the longitudinal assessment of cystic fibrosis.J Magn Reson Imaging. 2020; 52: 1306-1320
- Magnetic resonance imaging detects progression of lung disease and impact of newborn screening in preschool children with cystic fibrosis.Am J Respir Crit Care Med. 2021; https://doi.org/10.1164/rccm.202102–0278OC
Chung, J. et al. Increased Inflammatory Markers Detected in Nasal Lavage Correlate with Paranasal Sinus Abnormalities at MRI in Adolescent Patients with Cystic Fibrosis. Antioxidants (Basel) 10, (2021).
- Comparison of chest-MRI to chest-ct to monitor cystic fibrosis lung disease.Pediatric Pulmonology Supplement. 2010; 33: 362
- Lung morphology assessment using MRI: a robust ultra-short TR/TE 2D steady state free precession sequence used in cystic fibrosis patients.Magn Reson Med. 2009; 61: 299-306
- Spirometer-controlled cine magnetic resonance imaging used to diagnose tracheobronchomalacia in paediatric patients.Eur Respir J. 2014; 43: 115-124
- Repeatability and reproducibility of quantitative whole-lung perfusion magnetic resonance imaging.J Thorac Imaging. 2011; 26: 230-239
- Regional structure-function in cystic fibrosis lung disease using hyperpolarized 129Xe and Ultrashort echo magnetic resonance imaging.Am J Respir Crit Care Med. 2020; 202: 290-292
- The assessment of short- and long-term changes in lung function in cystic fibrosis using 129Xe MRI.Eur Respir J. 2020; 56
- Comparison of hyperpolarized 3He and 129Xe MR imaging in patients with cystic fibrosis. A108. Pathophysiology in DIFFUSE PARENCHYMAL.LUNG DISEASES. 2019; https://doi.org/10.1164/ajrccm-conference.2019.199.1_meetingabstracts.a2567
- Hyperpolarized 3He MR for sensitive imaging of ventilation function and treatment efficiency in young cystic fibrosis patients with normal lung function.Radiology. 2010; 255: 225-232
- The clinical Use of lung MRI in cystic fibrosis: what, now, how?.Chest. 2021; 159: 2205-2217
- Diagnostic value of chest ultrasound in children with cystic fibrosis - pilot study.PLoS One. 2019; 14: e0215786
- How useful is the lung ultrasound in cystic fibrosis?.Eur Respir J. 2016; 48: 1261
- Lung clearance index and lung ultrasound in cystic fibrosis children.Eur Respir J. 2018; 52: OA4988
- The Use of ultrasound as a tool to evaluate pulmonary disease in cystic fibrosis. Respir.Care. 2020; 65: 293-303
- Development and validation of the Cystic Fibrosis Questionnaire in the United States: a health-related quality-of-life measure for cystic fibrosis.Chest. 2005; 128: 2347-2354
- Content validity—establishing and reporting the evidence in newly developed patient-reported outcomes (PRO) instruments for medical product evaluation: ISPOR PRO Good Research Practices Task Force report: part 2—assessing respondent understanding.Value Health. 2011; 14: 978-988
- Translation and linguistic validation of a disease-specific quality of life measure for cystic fibrosis.J Pediatr Psychol. 2000; 25: 403-414
- Psychometric evaluation of the cystic fibrosis questionnaire-Revised in a national sample.Qual Life Res. 2012; 21: 1267-1278
- Development of a disease specific health related quality of life measure for adults and adolescents with cystic fibrosis.Thorax. 2000; 55: 946-954
- Patient-reported respiratory symptoms in cystic fibrosis.J Cyst Fibros. 2009; 8: 245-252
- Anxiety, depression, and life satisfaction in parents caring for children with cystic fibrosis.Pediatr Pulmonol. 2011; 46: 672-682
- Depressive symptoms in children with cystic fibrosis and parents and its effects on adherence to airway clearance.Pediatr Pulmonol. 2010; 45: 756-763
- Questions on life satisfaction for adolescents and adults with cystic fibrosis: development of a disease-specific questionnaire.Chest. 2003; 123: 42-48
- ‘Il faut continuer à poser des questions’ patient reported outcome measures in cystic fibrosis: an anthropological perspective.J Cyst Fibros. 2021; 20: e108-e113
- Quality of life is poorly correlated to lung disease severity in school-aged children with cystic fibrosis.J Cyst Fibros. 2021; https://doi.org/10.1016/j.jcf.2021.11.005
- Impact of CFTR therapy on chronic rhinosinusitis and health status: Deep learning CT analysis and patient reported outcomes.Ann Am Thorac Soc. 2021; https://doi.org/10.1513/AnnalsATS.202101–203057OC
- Impact of novel CFTR modulator on sinonasal quality of life in adult patients with cystic fibrosis.Int Forum Allergy Rhinol. 2021; 11: 201-203
- Designing the GALAXY study: Partnering with the cystic fibrosis community to optimize assessment of gastrointestinal symptoms.J Cyst Fibros. 2021; 20: 598-604
- Utilization of electronic patient-reported outcome measures in cystic fibrosis research: application to the GALAXY study.J Cyst Fibros. 2021; 20: 605-611
- Cardiopulmonary exercise testing provides additional prognostic information in cystic fibrosis.Am J Respir Crit Care Med. 2019; 199: 987-995
- The 1-minute Sit-to-Stand test in adults with cystic fibrosis: correlations with cardiopulmonary exercise test, 6-minute walk test, and Quadriceps Strength. Respir.Care. 2016; 61: 1620-1628
- Six-minute walk test vs cardiopulmonary exercise test in the assessment of exercise tolerance in adults with cystic fibrosis.Cystic Fibrosis. 2017; https://doi.org/10.1183/1393003.congress-2017.pa1349
- ERS statement on standardisation of cardiopulmonary exercise testing in chronic lung diseases.Eur Respir Rev. 2019; 28: 180101
- Exercise standards for testing and training: a scientific statement from the American Heart Association.Circulation. 2013; 128: 873-934
- Assessment of exercise pulmonary function.Pediatr Lab Exerc Test. 1993; : 141-163
- Exercise performance and breathing patterns in cystic fibrosis: Male-female differences and influence of resting pulmonary function.Pediatr Pulmonol. 1991; 10: 101-105
- The prognostic value of exercise testing in patients with cystic fibrosis. N. Engl.J Med. 1992; 327: 1785-1788
- Reproducibility of maximal cardiopulmonary exercise testing for young cystic fibrosis patients.J Cyst Fibros. 2013; 12: 644-650
- Lung clearance index (LCI) as a predictor of exercise limitation among CF patients.Pediatr Pulmonol. 2018; 53: 81-87
- Ventilation efficiency to exercise in patients with cystic fibrosis.Pediatr Pulmonol. 2019; 54: 1584-1590
- Does Pseudomonas aeruginosa colonization Affect exercise capacity in CF?.Pulm Med. 2019; 2019: 3786245
- Improvement in exercise duration, lung function and well-being in G551D-cystic fibrosis patients: a double-blind, placebo-controlled, randomized, cross-over study with ivacaftor treatment.Clin Sci. 2017; 131: 2037-2045
- Exercise testing in cystic fibrosis: who and why?.Paediatr Respir Rev. 2018; 27: 28-32
- Exercise testing in pediatric lung transplant candidates with cystic fibrosis.Pediatr Transpl. 2011; 15: 294-299
- Peak oxygen uptake and mortality in cystic fibrosis: systematic review and meta-analysis. Respir.Care. 2019; 64: 91-98
- Peak oxygen uptake and mortality in children with cystic fibrosis.Thorax. 2005; 60: 50-54
- Patients with cystic fibrosis and advanced lung disease benefit from lumacaftor/ivacaftor treatment.Pediatr Pulmonol. 2020; 55: 3364-3370
- The effect of ivacaftor in adolescents with cystic fibrosis (G551D mutation): an exercise physiology perspective.Pediatr Phys Ther. 2014; 26: 454-461
- Exercise testing for children with cystic fibrosis: a systematic review. Pediatr.Pulmonol. 2020; 55: 1996-2010
- ATS statement on six-minute walk test.Am J Respir Crit Care Med. 2003; 167: 1287
- The repeatability of submaximal endurance exercise testing in cystic fibrosis.Pediatr Pulmonol. 2007; 42: 75-82
- The 6-min walk test: a quick measure of functional status in elderly adults.Chest. 2003; 123: 387-398
- Six-minute walk test in children and adolescents with cystic fibrosis.Pediatr Pulmonology. 2006; 41: 618-622
- Six minute walk test Z score: correlations with cystic fibrosis severity markers.J Cyst Fibros. 2012; 11: 253-256
- Six-minute walk test as a determinant of the functional capacity of children and adolescents with cystic fibrosis: a systematic review. Respir.Med. 2018; 137: 83-88
- Measuring the impact of CFTR modulation on sweat chloride in cystic fibrosis: Rationale and design of the CHEC-SC study.J Cyst Fibros. 2021; 20: 965-971
- CFTR modulator-induced sweat chloride changes across the cystic fibrosis population: first results from the CHEC-SC study.Pediatric Pulmonology. 2019; 54 (WILEY 111 RIVER ST, HOBOKEN 07030-5774, NJ USA): S229
- 555: clinical trial interest after establishment of modulator therapy: Interim CHEC-SC survey results.J Cyst Fibros. 2021; 20: S262
- Data from the US and UK cystic fibrosis registries support disease modification by CFTR modulation with ivacaftor.Thorax. 2018; 73: 731-740
- Real-world outcomes among patients with cystic fibrosis treated with ivacaftor: 2012–2016 experience.Pulm Ther. 2020; 6: 141-149
- Single-cell transcriptional archetypes of airway inflammation in cystic fibrosis.Am J Respir Crit Care Med. 2020; 202: 1419-1429
- A single-cell atlas of the airway epithelium reveals the CFTR-rich pulmonary ionocyte.Nature. 2018; 560: 377-381
- Secretory cells dominate airway CFTR expression and function in human airway superficial epithelia.Am J Respir Crit Care Med. 2021; 203: 1275-1289
- Multiparameter single cell profiling of airway inflammatory cells.Cytometry B Clin Cytom. 2017; 92: 12-20
- CFTR modulator therapy enhances peripheral blood monocyte contributions to immune responses in people with cystic fibrosis. Front.Pharmacol. 2020; 11: 1219
- Pseudomonas aeruginosa infection, but not mono or dual-combination CFTR modulator therapy affects circulating regulatory T cells in an adult population with cystic fibrosis.J Cyst Fibros. 2021; https://doi.org/10.1016/j.jcf.2021.05.001
- Airway inflammation and host responses in the era of CFTR modulators.Int J Mol Sci. 2020; 21
- Inflammatory markers of lung disease in adult patients with cystic fibrosis.Pediatr Pulmonol. 2007; 42: 256-262
- Mechanisms and markers of airway inflammation in cystic fibrosis.Eur Respir J. 2002; 19: 333-340
- A screening tool to identify risk for bronchiectasis progression in children with cystic fibrosis.Pediatr Pulmonol. 2021; https://doi.org/10.1002/ppul.25712
- RNA sequencing data from neutrophils of patients with cystic fibrosis reveals potential for developing biomarkers for pulmonary exacerbations.J Cystic Fibrosis. 2019; 18: 194-202
- A Short extension to multiple breath washout provides additional signal of distal airway disease in people with CF: a pilot study.J Cyst Fibros. 2021; https://doi.org/10.1016/j.jcf.2021.06.013
- Mapping the lung proteome in cystic fibrosis.J Proteome Res. 2009; 8: 3020-3028
- SPLUNC1: a novel marker of cystic fibrosis exacerbations.Eur Respir J. 2021; https://doi.org/10.1183/13993003.00507–02020
- Anaerobic bacteria cultured from cystic fibrosis airways correlate to milder disease: a multisite study.Eur Respir J. 2018; 52
- Association between genetically determined pancreatic status and lung disease in adult cystic fibrosis patients.Chest. 2002; 121: 73-80
- Differences in the pattern of structural abnormalities on CT scan in patients with cystic fibrosis and pancreatic sufficiency or insufficiency.Chest. 2013; 144: 208-214
Coffey MJ, Ooi CY. Pancreatitis in Cystic Fibrosis and CFTR-Related Disorder. In: Rodrigo, L., editor. Acute Pancreatitis [Internet]. London: IntechOpen; 2012 [cited 2022 Jul 11]. Available from: https://www.intechopen.com/chapters/26187 doi: 10.5772/27861.
- Clinical practice and genetic counseling for cystic fibrosis and CFTR-related disorders.Genet Med. 2008; 10: 851-868
- Atypical cystic fibrosis and CFTR-related diseases.Clin Rev Allergy Immunol. 2008; 35: 116-123
- Longitudinal monitoring of disease progression in children with mild CF using hyperpolarised gas MRI and LCI.Eur Respir J. 2016; 48
- Detection of early subclinical lung disease in children with cystic fibrosis by lung ventilation imaging with hyperpolarised gas MRI.Thorax. 2017; 72: 760-762
- Characterizing responses to CFTR-modulating drugs using rectal organoids derived from subjects with cystic fibrosis.Sci Transl Med. 2016; 8: 344ra84
- Might brushed nasal cells be a surrogate for CFTR modulator clinical response?.Am J Respir Crit Care Med. 2019; 199: 123-126
- Electronic monitoring reveals highly variable adherence patterns in patients prescribed ivacaftor.J Cyst Fibros. 2015; 14: 621-626
- Soft, skin-interfaced sweat stickers for cystic fibrosis diagnosis and management.Sci Transl Med. 2021; 13
- Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators.Sci Rep. 2017; 7: 7375
- CFTR modulator theratyping: current status, gaps and future directions.J Cyst Fibros. 2019; 18: 22-34
- Theratyping cystic fibrosis in vitro in ALI-culture and organoid models generated from patient-derived nasal epithelial Conditionally Reprogrammed Stem Cells.Eur. Respir J. 2021; https://doi.org/10.1183/13993003.00908–02021
- The dynamic response of sweat chloride to changes in exercise Load measured by a wearable sweat sensor.Scientific Rep. 2020; 10
- Wearable Analytical platform with Enzyme-Modulated dynamic range for the Simultaneous Colorimetric detection of sweat Volume and sweat biomarkers.ACS Sens. 2021; 6: 130-136
- Rationale and design of a randomized trial of home electronic symptom and lung function monitoring to detect cystic fibrosis pulmonary exacerbations: the early intervention in cystic fibrosis exacerbation (eICE) trial.Contemp Clin Trials. 2013; 36: 460-469
- High treatment burden in adults with cystic fibrosis: challenges to disease self-management.J Cyst Fibros. 2009; 8: 91-96
- Tackling the increasing complexity of CF care.Pediatr Pulmonol. 2015; 40: S74-S79
- Drug interactions and treatment burden as survival improves.Curr Opin Pulm Med. 2015; 21: 617-625
- Evaluating the impact of stopping chronic therapies after modulator drug therapy in cystic fibrosis: the SIMPLIFY clinical trial study design.Ann Am Thorac Soc. 2021; 18: 1397-1405
- Adherence to therapies in cystic fibrosis: a targeted literature review.Expert Rev Respir Med. 2017; 11: 129-145
- Characterising burden of treatment in cystic fibrosis to identify priority areas for clinical trials.J Cyst Fibros. 2020; 19: 499-502
- Safety of repeated intermittent courses of aerosolized recombinant human deoxyribonuclease in patients with cystic fibrosis.J Pediatr. 1997; 131: 118-124
- Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis.N. Engl. J Med. 1994; 331: 637-642
- Mucus clearance and lung function in cystic fibrosis with hypertonic saline.N. Engl. J Med. 2006; 354: 241-250
- A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis.N. Engl. J Med. 2006; 354: 229-240
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