Cited by 685Cystic Fibrosis Research AdvancesGenetic and Kidney Cyst DiseasesNeonatal Respiratory Health Research

The DOI matches its Crossref record; 194 of the 195 references it cites were found in a registry or an index.

Cystic fibrosis

Open access

Shteinberg, Michal, Haq, Iram J, Polineni, Deepika, Davies, Jane C·2021·The Lancet

Open this paper in CoChat — free account

Abstract

Cystic fibrosis is a monogenic disease considered to affect at least 100 000 people worldwide. Mutations in CFTR, the gene encoding the epithelial ion channel that normally transports chloride and bicarbonate, lead to impaired mucus hydration and clearance. Classical cystic fibrosis is thus characterised by chronic pulmonary infection and inflammation, pancreatic exocrine insufficiency, male infertility, and might include several comorbidities such as cystic fibrosis-related diabetes or cystic fibrosis liver disease. This autosomal recessive disease is diagnosed in many regions following newborn screening, whereas in other regions, diagnosis is based on a group of recognised multiorgan clinical manifestations, raised sweat chloride concentrations, or CFTR mutations. Disease that is less easily diagnosed, and in some cases affecting only one organ, can be seen in the context of gene variants leading to residual protein function. Management strategies, including augmenting mucociliary clearance and aggressively treating infections, have gradually improved life expectancy for people with cystic fibrosis. However, restoration of CFTR function via new small molecule modulator drugs is transforming the disease for many patients. Clinical trial pipelines are actively exploring many other approaches, which will be increasingly needed as survival improves and as the population of adults with cystic fibrosis increases. Here, we present the current understanding of CFTR mutations, protein function, and disease pathophysiology, consider strengths and limitations of current management strategies, and look to the future of multidisciplinary care for those with cystic fibrosis.

Integrity signals

Registry

Crossref · 10.1016/s0140-6736(20)32542-3

Cites

194 of 194 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

We couldn’t retrieve this paper’s full text, so its claims and self-check weren’t run. The registry and citation checks still apply.

Standardisation of spirometry
Miller, M. R., Hankinson, J., Brusasco, V., Burgos, F., Casaburi, R., Coates, A., Crapo, R., Enright, P., van der Grinten, C. P. M., Gustafsson, P., et al. (2005) · European Respiratory Journal · doi:10.1183/09031936.05.00034805 · Cited by 15,627
Identification of the Cystic Fibrosis Gene: Genetic Analysis
Kerem, B.-S., Rommens, J. M., Buchanan, J. A., Markiewicz, D., Cox, T. K., Chakravarti, A., Buchwald, M., & Tsui, L.-C. (1989) · Science · doi:10.1126/science.2570460 · Cited by 4,142
Antibiotic resistance in Pseudomonas aeruginosa: mechanisms and alternative therapeutic strategies
Pang, Z., Raudonis, R., Glick, B. R., Lin, T., & Cheng, Z. (2018) · Biotechnology Advances · doi:10.1016/j.biotechadv.2018.11.013 · Cited by 2,520
Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele
Middleton, P. G., Mall, M. A., Dřevínek, P., Lands, L. C., McKone, E. F., Polineni, D., Ramsey, B. W., Taylor-Cousar, J. L., Tullis, E., Vermeulen, F., et al. (2019) · New England Journal of Medicine · doi:10.1056/nejmoa1908639 · Cited by 2,198
Self-citation
A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation
Ramsey, B. W., Davies, J., McElvaney, N. G., Tullis, E., Bell, S. C., Dřevínek, P., Griese, M., McKone, E. F., Wainwright, C. E., Konstan, M. W., et al. (2011) · New England Journal of Medicine · doi:10.1056/nejmoa1105185 · Cited by 2,195
Self-citation
Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR
Wainwright, C. E., Elborn, J. S., Ramsey, B. W., Marigowda, G., Huang, X., Cipolli, M., Colombo, C., Davies, J. C., De Boeck, K., Flume, P. A., et al. (2015) · New England Journal of Medicine · doi:10.1056/nejmoa1409547 · Cited by 1,622
Self-citation
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
Heijerman, H. G. M., McKone, E. F., Downey, D. G., Van Braeckel, E., Rowe, S. M., Tullis, E., Mall, M. A., Welter, J. J., Ramsey, B. W., McKee, C. M., et al. (2019) · The Lancet · doi:10.1016/s0140-6736(19)32597-8 · Cited by 1,414
Intermittent Administration of Inhaled Tobramycin in Patients with Cystic Fibrosis
Ramsey, B. W., PEPE, M. S., Quan, J. M., Otto, K., Montgomery, A. B., Williams‐Warren, J., Vasiljev-K, M., Borowitz, D., Bowman, C. M., Marshall, B. C., et al. (1999) · New England Journal of Medicine · doi:10.1056/nejm199901073400104 · Cited by 1,296
The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Thirty-sixth adult lung and heart–lung transplantation Report—2019; Focus theme: donor and recipient size match
Chambers, D. C., Cherikh, W. S., Harhay, M. O., Hayes, D., Hsich, E., Khush, K. K., Meiser, B., Potena, L., Rossano, J. W., Toll, A. E., et al. (2019) · The Journal of Heart and Lung Transplantation · doi:10.1016/j.healun.2019.08.001 · Cited by 1,269
The future of cystic fibrosis care: a global perspective
Bell, S. C., Mall, M. A., Gutierrez, H., Macek, M., Madge, S. L., Davies, J. C., Burgel, P., Tullis, E., Castaños, C., Castellani, C., et al. (2019) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(19)30337-6 · Cited by 1,081
Self-citation

Show all 195 references

Research works printed but not linked: 2

Saunders, 2016, Developments in multibreath washout testing in children with cystic fibrosis, Curr Med Res Opin, 33, 1
2015, Symposium Summaries, Pediatr Pulmonol, 50, S108

Sorted by citations

Items 101–200 of 685

Elexacaftor-Tezacaftor-Ivacaftor as a Final Frontier in the Treatment of Cystic Fibrosis: Definition of the Clinical and Microbiological Implications in a Case-Control Study
Migliorisi, G., Collura, M., Ficili, F., Pensabene, T., Bongiorno, D., Collura, A., Bernardo, F. D., & Stefani, S. (2022) · Pharmaceuticals · doi:10.3390/ph15050606 · Cited by 23
Immunoreactive Trypsinogen in Infants Born to Women with Cystic Fibrosis Taking Elexacaftor–Tezacaftor–Ivacaftor
Patel, P., Yeley, J., Brown, C. D., Wesson, M., Lesko, B. G., Slaven, J. E., Chmiel, J. F., Jain, R., & Sanders, D. B. (2023) · International Journal of Neonatal Screening · doi:10.3390/ijns9010010 · Cited by 23
Automatic bronchus and artery analysis on chest computed tomography to evaluate the effect of inhaled hypertonic saline in children aged 3-6 years with cystic fibrosis in a randomized clinical trial
Chen, Y., Lv, Q., Andrinopoulou, E., Estrella, L. G., Charbonnier, J., Caudri, D., Davis, S. D., Rosenfeld, M., Ratjen, F. A., Kronmal, R. A., et al. (2023) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2023.05.013 · Cited by 23
Fibrosis quística: epidemiología, clínica, diagnóstico y tratamiento
Cáceres, L. D., & Zamarrón, E. (2023) · Medicina Clínica · doi:10.1016/j.medcli.2023.06.006 · Cited by 23
In utero and postnatal ivacaftor/lumacaftor therapy rescues multiorgan disease in CFTR-F508del ferrets
Evans, I. A., Sun, X., Liang, B., Vegter, A. R., Guo, L., Lynch, T. J., Zhang, Y., Zhang, Y., Yi, Y., Yu, Y., et al. (2024) · JCI Insight · doi:10.1172/jci.insight.157229 · Cited by 22
Risk factors for severe COVID-19 in people with cystic fibrosis: A systematic review
Terlizzi, V., Motisi, M. A., Pellegrino, R., Padoan, R., & Chiappini, E. (2022) · Frontiers in Pediatrics · doi:10.3389/fped.2022.958658 · Cited by 22
A review of causal discovery methods for molecular network analysis
Kelly, J., Berzuini, C., Keavney, B. D., Tomaszewski, M., & Guo, H. (2022) · Molecular Genetics & Genomic Medicine · doi:10.1002/mgg3.2055 · Cited by 25
Cystic Fibrosis Bone Disease: The Interplay between CFTR Dysfunction and Chronic Inflammation
Fonseca, Ó., Gomes, M. S., Amorim, M. A., & Gomes, A. C. (2023) · Biomolecules · doi:10.3390/biom13030425 · Cited by 22
Reversal of cylindrical bronchial dilatations in a subset of adults with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor
Cazier, P., Chassagnon, G., Dhôte, T., Silva, J. D., Kanaan, R., Honoré, I., Carlier, N., Revel, M., Canniff, E., Martin, C., et al. (2024) · European Respiratory Journal · doi:10.1183/13993003.01794-2023 · Cited by 22
Reduction of systemic inflammation by elexacaftor/tezacaftor/ivacaftor correlates with lung function improvement in cystic fibrosis
Halle, O., Graeber, S. Y., Kontsendorn, J., Kessemeier, C., Falke, J.-N., Schwabe, J. D., Schütz, K., Pallenberg, S. T., Dalferth, R., Grychtol, R. M., et al. (2025) · European Respiratory Journal · doi:10.1183/13993003.00150-2025 · Cited by 21
Review article: epidemiology, pathogenesis and management of liver disease in adults with cystic fibrosis
Scott, J. A., Jones, A. M., Hanley, K. P., & Athwal, V. S. (2022) · Alimentary Pharmacology & Therapeutics · doi:10.1111/apt.16749 · Cited by 20
The Impact of Air Pollution on the Course of Cystic Fibrosis: A Review
Blayac, M., Coll, P., Urbach, V., Fanen, P., Epaud, R., & Lanone, S. (2022) · Frontiers in Physiology · doi:10.3389/fphys.2022.908230 · Cited by 20
Challenged Urine Bicarbonate Excretion as a Measure of Cystic Fibrosis Transmembrane Conductance Regulator Function in Cystic Fibrosis
Berg, P., Sørensen, M. V., Rousing, A. Q., Olesen, H. V., Jensen‐Fangel, S., Jeppesen, M., & Leipziger, J. (2022) · Annals of Internal Medicine · doi:10.7326/m22-1741 · Cited by 20
Lipidomic alterations in human saliva from cystic fibrosis patients
Caterino, M., Fedele, R., Carnovale, V., Castaldo, A., Gelzo, M., Iacotucci, P., Ruoppolo, M., & Castaldo, G. (2023) · Scientific Reports · doi:10.1038/s41598-022-24429-6 · Cited by 20
Effect of elexacaftor/tezacaftor/ivacaftor on systemic inflammation in cystic fibrosis
Maher, R. E., Cytlak, U., Aleem, S., Barry, P. J., Brice, D. P., Caamaño‐Gutiérrez, E., Driver, K., Emmott, E., Rothwell, A., Smith, E., et al. (2025) · Thorax · doi:10.1136/thorax-2024-222242 · Cited by 19
Precision Medicine Based on CFTR Genotype for People with Cystic Fibrosis
Haq, I., Almulhem, M. M., Soars, S., Poulton, D., & Brodlie, M. (2022) · Pharmacogenomics and Personalized Medicine · doi:10.2147/pgpm.s245603 · Cited by 20
Self-citation
Extracellular Vesicles’ Role in the Pathophysiology and as Biomarkers in Cystic Fibrosis and COPD
Gioia, S. D., Daniello, V., & Conese, M. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms24010228 · Cited by 20
Role of the Mitochondrial E3 Ubiquitin Ligases as Possible Therapeutic Targets in Cancer Therapy
Gregorio, J. D., Appignani, M., & Flati, V. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms242417176 · Cited by 19
Comparative microbiome analysis in cystic fibrosis and non-cystic fibrosis bronchiectasis.
Motta, H., Reuwsaat, J. C. V., Lopes, F. C., Viezzer, G., Volpato, F. C. Z., Barth, A. L., Dalcin, P. D. T. R., Staats, C. C., Vainstein, M. H., & Kmetzsch, L. (2024) · Respiratory Research · doi:10.1186/s12931-024-02835-w · Cited by 19
Different management approaches and outcome for infants with an inconclusive diagnosis following newborn screening for cystic fibrosis (CRMS/CFSPID) and Pseudomonas aeruginosa isolation
Dolce, D., Claut, L. E., Colombo, C., Tosco, A., Castaldo, A., Padoan, R., Timpano, S., Fabrizzi, B., Bonomi, P., Taccetti, G., et al. (2022) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2022.07.007 · Cited by 18
Research progress and application of liver organoids for disease modeling and regenerative therapy
Hu, Y., Geng, Q., Wang, L., Wang, Y., Huang, C., Fan, Z., & Kong, D. (2024) · Journal of Molecular Medicine · doi:10.1007/s00109-024-02455-3 · Cited by 21
Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations
Strandvik, B. (2022) · Nutrients · doi:10.3390/nu14040853 · Cited by 18
Exploring the impact of elexacaftor-tezacaftor-ivacaftor treatment on opinions regarding airway clearance techniques and nebulisers: TEMPO a qualitative study in children with cystic fibrosis, their families and healthcare professionals
Almulhem, M. M., Harnett, N., Graham, S., Haq, I. J., Visram, S., Ward, C., & Brodlie, M. (2022) · BMJ Open Respiratory Research · doi:10.1136/bmjresp-2022-001420 · Cited by 18
Self-citation
Compendium of causative genes and their encoded proteins for common monogenic disorders
Apgar, T. L., & Sanders, C. R. (2021) · Protein Science · doi:10.1002/pro.4183 · Cited by 21
Longitudinal assessment of renal function after lung transplantation for cystic fibrosis: transition from post-operative acute kidney injury to acute kidney disease and chronic kidney failure
Scaravilli, V., Merrino, A., Bichi, F., Madotto, F., Morlacchi, L. C., Nosotti, M., Lissoni, A., Rosso, L., Blasi, F., Pesenti, A. M., et al. (2022) · Journal of Nephrology · doi:10.1007/s40620-022-01392-z · Cited by 17
Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis
Welsner, M., Dietz-Terjung, S., Stehling, F., Schulte, T., Niehammer, U., Gahbiche, F.-E., Taube, C., Straßburg, S., Schoebel, C., Weinreich, G., et al. (2022) · BMC Pulmonary Medicine · doi:10.1186/s12890-022-02243-0 · Cited by 17
The Clinical Association between Aspergillus fumigatus and Respiratory Outcomes in Adolescents and Adults with Cystic Fibrosis
O’Dea, A. L., Feng, R., Glaser, L. J., Kubrak, C., Rubenstein, R. C., Dorgan, D. J., Hadjiliadis, D., Kawut, S. M., & Hong, G. (2023) · Annals of the American Thoracic Society · doi:10.1513/annalsats.202210-852oc · Cited by 17
Use of elexacaftor/tezacaftor/ivacaftor combination in pregnancy
Cimino, G. G., Sorrenti, S., Murciano, M., Galoppi, P., Ascenzioni, F., Botta, B., Brunelli, R., Cavinato, L., Cerrato, A., Cianfoni, G., et al. (2023) · Archives of Gynecology and Obstetrics · doi:10.1007/s00404-023-06962-5 · Cited by 17
Multimodal analysis of granulocytes, monocytes, and platelets in patients with cystic fibrosis before and after Elexacaftor–Tezacaftor–Ivacaftor treatment
Schmidt, H., Höpfer, L. M., Wohlgemuth, L., Knapp, C. L., Mohamed, A. O. K., Stukan, L., Münnich, F., Hüsken, D., Koller, A. S., Stratmann, A. E. P., et al. (2023) · Frontiers in Immunology · doi:10.3389/fimmu.2023.1180282 · Cited by 17
Small molecule modulators of cystic fibrosis transmembrane conductance regulator (CFTR): Structure, classification, and mechanisms
Cao, L., Wu, Y., Gong, Y., & Zhou, Q. (2024) · European Journal of Medicinal Chemistry · doi:10.1016/j.ejmech.2023.116120 · Cited by 18
Backbone cationized highly branched poly(β-amino ester)s as enhanced delivery vectors in non-viral gene therapy
Li, Y., Qiu, B., Li, Z., Wang, X., He, Z., Manzanares, D., Song, R., Sigen, A., Zhao, C., Johnson, M., et al. (2024) · Journal of Controlled Release · doi:10.1016/j.jconrel.2024.01.046 · Cited by 17
Mechanisms of Lung Cancer Development in Cystic Fibrosis Patients: The Role of Inflammation, Oxidative Stress, and Lung Microbiome Dysbiosis
Pagliaro, R., Scialò, F., Schiattarella, A., Cianci, R., Campbell, S. F. M., Perrotta, F., Bianco, A., & Castaldo, G. (2025) · Biomolecules · doi:10.3390/biom15060828 · Cited by 17
Bacterial Subversion of Autophagy in Cystic Fibrosis
Flores-Vega, V. R., Vargas-Roldán, S. Y., Lezana, J. L., Lascuraín, R., Santos, J. I., & Rosales-Reyes, R. (2021) · Frontiers in Cellular and Infection Microbiology · doi:10.3389/fcimb.2021.760922 · Cited by 16
The role of indolyl substituents in squaramide-based anionophores
Picci, G., Carreira‐Barral, I., Alonso-Carrillo, D., Busonera, C., Milia, J., Quesada, R., & Caltagirone, C. (2022) · Organic & Biomolecular Chemistry · doi:10.1039/d2ob01444k · Cited by 16
Effect of 18 months elexacaftor-tezacaftor-ivacaftor on body mass index and glycemic control in adults with cystic fibrosis
Taelman, V., Declercq, D., Biervliet, S. V., Weygaerde, Y. V., Lapauw, B., & Braeckel, E. V. (2023) · Clinical Nutrition ESPEN · doi:10.1016/j.clnesp.2023.08.028 · Cited by 16
The role of vasoactive intestinal peptide in pulmonary diseases
Zhong, H.-L., Li, P.-Z., Li, D., Guan, C., & Zhou, Y. (2023) · Life Sciences · doi:10.1016/j.lfs.2023.122121 · Cited by 16
The relationship between lung disease severity and the sputum proteome in cystic fibrosis
Maher, R. E., Barrett, E., Beynon, R. J., Harman, V. M., Jones, A. M., McNamara, P. S., Smith, J. A., & Lord, R. W. (2022) · Respiratory Medicine · doi:10.1016/j.rmed.2022.107002 · Cited by 16
Tissue-localized immune responses in people with cystic fibrosis and respiratory nontuberculous mycobacteria infection
Hayes, D., Shukla, R. K., Cheng, Y., Gecili, E., Merling, M. R., Szczesniak, R. D., Ziady, A. G., Woods, J. C., Hall‐Stoodley, L., Liyanage, N. P. M., et al. (2022) · JCI Insight · doi:10.1172/jci.insight.157865 · Cited by 15
Advances in the pathogenesis and personalised treatment of paediatric asthma
Scotney, E., Fleming, L., Saglani, S., Sonnappa, S., & Bush, A. (2023) · BMJ Medicine · doi:10.1136/bmjmed-2022-000367 · Cited by 16
Reproductive Counseling and Care in Cystic Fibrosis: A Multidisciplinary Approach for a New Therapeutic Era
McGlynn, J., DeCelie-Germana, J., Kier, C., & Langfelder-Schwind, E. (2023) · Life · doi:10.3390/life13071545 · Cited by 15
Qualitative and quantitative evaluation of computed tomography changes in adults with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor: a retrospective observational study
Dettmer, S., Weinheimer, O., Sauer‐Heilborn, A., Lammers, O., Wielpütz, M. O., Fuge, J., Welte, T., Wacker, F. K., & Ringshausen, F. C. (2023) · Frontiers in Pharmacology · doi:10.3389/fphar.2023.1245885 · Cited by 15
Long-Term Impact of Lumacaftor/Ivacaftor Treatment on Cystic Fibrosis Disease Progression in Children 2–5 Years of Age Homozygous for F508del-CFTR : A Phase 2, Open-Label Clinical Trial
Stahl, M., Roehmel, J. F., Eichinger, M., Doellinger, F., Naehrlich, L., Kopp, M., Dittrich, A., Sommerburg, O., Ray, P., Maniktala, A., et al. (2024) · Annals of the American Thoracic Society · doi:10.1513/annalsats.202402-201oc · Cited by 15
Real-world impact of Elexacaftor-Tezacaftor-Ivacaftor treatment in young people with Cystic Fibrosis: A longitudinal study
Connett, G. J., Maguire, S., Larcombe, T., Scanlan, N., Shinde, S. S., Muthukumarana, T., Bevan, A., Keogh, R. H., & Legg, J. P. (2024) · Respiratory Medicine · doi:10.1016/j.rmed.2024.107882 · Cited by 15
Antibacterial contact-dependent proteins secreted by Gram-negative cystic fibrosis respiratory pathogens
Crisan, C. V., & Goldberg, J. B. (2022) · Trends in Microbiology · doi:10.1016/j.tim.2022.03.009 · Cited by 15
Pseudomonas aeruginosa aggregation and Psl expression in sputum is associated with antibiotic eradication failure in children with cystic fibrosis
Morris, A. J., Yau, Y. C. W., Park, S., Eisha, S., McDonald, N., Parsek, M. R., Howell, P. L., Hoffman, L. R., Nguyen, D. M., DiGiandomenico, A., et al. (2022) · Scientific Reports · doi:10.1038/s41598-022-25889-6 · Cited by 15
Sensing of Chloride Ions in Sweat by Means of Printed Extended‐Gate Organic Electrochemical Transistors
Bortolotti, C., Ottomaniello, A., Parlanti, P., Mattoli, V., Natali, D., Aliverti, A., Kyndiah, A., & Caironi, M. (2025) · Advanced Functional Materials · doi:10.1002/adfm.202511675 · Cited by 15
A Model of Intracellular Persistence of Pseudomonas aeruginosa in Airway Epithelial Cells
Malet, J. K., Hennemann, L. C., Hua, E. M.-L., Faure, E., Waters, V. J., Rousseau, S., & Nguyen, D. M. (2022) · Cellular Microbiology · doi:10.1155/2022/5431666 · Cited by 14
Genetic therapies in cystic fibrosis
Taylor‐Cousar, J. L., Boyd, A. C., Alton, E. W. F. W., & Polineni, D. (2023) · Current Opinion in Pulmonary Medicine · doi:10.1097/mcp.0000000000001019 · Cited by 14
Self-citation
Bacteriophage Therapy of Multidrug-resistant Achromobacter in an 11-Year-old Boy With Cystic Fibrosis Assessed by Metagenome Analysis
Bradley, J. S., Hajama, H., Akong, K., Jordan, M., Stout, D., Rowe, R. S., Conrad, D. J., Hingtgen, S., & Segall, A. M. (2023) · The Pediatric Infectious Disease Journal · doi:10.1097/inf.0000000000004000 · Cited by 14
Prominent role of gut dysbiosis in the pathogenesis of cystic fibrosis-related liver disease in mice
Bertolini, A., Nguyen, M., Zehra, S. A., Taleb, S. A., Bauer-Pisani, T., Palm, N., Strazzabosco, M., & Fiorotto, R. (2024) · Journal of Hepatology · doi:10.1016/j.jhep.2024.03.041 · Cited by 13
Inflammation in preschool cystic fibrosis is of mixed phenotype, extends beyond the lung and is differentially modified by CFTR modulators
Shanthikumar, S., Gubbels, L., Wong, A. T. C., Walker, H., Ranganathan, S., & Neeland, M. R. (2025) · Thorax · doi:10.1136/thorax-2024-221634 · Cited by 13
The Microbiology of Non-aeruginosa Pseudomonas Isolated From Adults With Cystic Fibrosis: Criteria to Help Determine the Clinical Significance of Non-aeruginosa Pseudomonas in CF Lung Pathology
Moore, J. E., McCaughan, J., Rendall, J. C., & Millar, B. C. (2022) · British Journal of Biomedical Science · doi:10.3389/bjbs.2022.10468 · Cited by 13
The manipulation of cell signaling and host cell biology by cholera toxin
White, C., Bader, C., & Teter, K. (2022) · Cellular Signalling · doi:10.1016/j.cellsig.2022.110489 · Cited by 13
Resolvin D1 improves airway inflammation and exercise capacity in cystic fibrosis lung disease
Ferri, G., Serano, M., Isopi, E., Mucci, M., Mattoscio, D., Pecce, R., Protasi, F., Mall, M. A., Romano, M., & Recchiuti, A. (2023) · The FASEB Journal · doi:10.1096/fj.202301495r · Cited by 13
Case Report: Guanfacine and methylphenidate improved chronic lower back pain in autosomal dominant polycystic kidney disease with comorbid attention deficit hyperactivity disorder and autism spectrum disorder
Kasahara, S., Kanda, S., Takahashi, M., Fujioka, M., Morita, T., Matsudaira, K., Sato, N. S., Hattori, M., Momose, T., Niwa, S., et al. (2023) · Frontiers in Pediatrics · doi:10.3389/fped.2023.1283823 · Cited by 13
CFTR is required for zinc-mediated antibacterial defense in human macrophages
Gupta, K. D., Curson, J. E. B., Tarique, A. A., Kapétanovic, R., Schembri, M. A., Fantino, E., Sly, P. D., & Sweet, M. J. (2024) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.2315190121 · Cited by 14
Unmet challenges in cystic fibrosis treatment with modulators
Corrao, F., Kelly, M., Sermet‐Gaudelus, I., & Semeraro, M. (2024) · Expert Review of Respiratory Medicine · doi:10.1080/17476348.2024.2357210 · Cited by 14
Nutritional status in the era of highly effective CFTR modulators
Bass, R., & Alvarez, J. A. (2024) · Pediatric Pulmonology · doi:10.1002/ppul.26806 · Cited by 14
Adhesive-Based Fabrication Technique for Culture of Lung Airway Epithelial Cells with Applications in Cell Patterning and Microfluidics
Dabaghi, M., Tiessen, N., Cao, Q., Chandiramohan, A., Saraei, N., Kim, Y., Gupta, T., Selvaganapathy, P. R., & Hirota, J. A. (2021) · ACS Biomaterials Science & Engineering · doi:10.1021/acsbiomaterials.1c01200 · Cited by 13
Treatment of cystic fibrosis related bone disease
Ullal, J., KUTNEY, K. A., Williams, K. M., & Weber, D. R. (2021) · Journal of Clinical & Translational Endocrinology · doi:10.1016/j.jcte.2021.100291 · Cited by 15
Modulator Therapy in Cystic Fibrosis Patients with cis Variants in F508del Complex Allele: A Short-Term Observational Case Series
Terlizzi, V., Centrone, C., Ferrari, B., Castellani, C., Gunawardena, T. N. A., Taccetti, G., & Laselva, O. (2022) · Journal of Personalized Medicine · doi:10.3390/jpm12091421 · Cited by 13
Managing cystic fibrosis in children aged 6-11yrs: a critical review of elexacaftor/tezacaftor/ivacaftor combination therapy
Jordan, K., Zemanick, E. T., Taylor‐Cousar, J. L., & Hoppe, J. E. (2023) · Expert Review of Respiratory Medicine · doi:10.1080/17476348.2023.2179989 · Cited by 13
Role of vitamin D3 in selected pulmonary diseases with particular emphasis on lung fibrosis
Chojnacki, M., & Lemieszek, M. K. (2023) · Annals of Agricultural and Environmental Medicine · doi:10.26444/aaem/161583 · Cited by 14
Profile of the intestinal microbiota of patients with cystic fibrosis: A systematic review
Furtado, J. D. S., Brasiel, P. G. D. A., & Luquetti, S. C. P. D. (2023) · Clinical Nutrition ESPEN · doi:10.1016/j.clnesp.2023.04.008 · Cited by 12
Loss of CFTR function in macrophages alters the cell transcriptional program and delays lung resolution of inflammation
Wellems, D. L., Hu, Y., Jennings, S., & Wang, G. (2023) · Frontiers in Immunology · doi:10.3389/fimmu.2023.1242381 · Cited by 13
Mycobacterium abscessus extracellular vesicles increase mycobacterial resistance to clarithromycin in vitro
Vermeire, C. A., Tan, X., Liang, Y., Kotey, S. K., Rogers, J., Hartson, S. D., Liu, L., & Cheng, Y. (2024) · PROTEOMICS · doi:10.1002/pmic.202300332 · Cited by 12
Various mutations in icaR, the repressor of the icaADBC locus, occur in mucoid Staphylococcus aureus isolates recovered from the airways of people with cystic fibrosis
Bianca, S., Rumpf, C. H., Hait, R. J., Janssen, T., Deiwick, S., Schwierzeck, V., Mellmann, A., & Kahl, B. C. (2024) · Microbes and Infection · doi:10.1016/j.micinf.2024.105306 · Cited by 12
Ocular Changes in Cystic Fibrosis: A Review
Liberski, S., Confalonieri, F., Cofta, S., Petrovski, G., & Kocięcki, J. (2024) · International Journal of Molecular Sciences · doi:10.3390/ijms25126692 · Cited by 12
Highly branched poly β-amino ester/CpG-depleted CFTR plasmid nanoparticles for non-viral gene therapy in lung cystic fibrosis disease
Qiu, B., Manzanares, D., Li, Y., Wang, X., Li, Z., Terreau, S., He, Z., Lyu, J., Wang, W., & Lara‐Sáez, I. (2024) · Molecular Therapy — Methods & Clinical Development · doi:10.1016/j.omtm.2024.101292 · Cited by 12
Definitions of pulmonary exacerbation in people with cystic fibrosis: a scoping review
Almulhem, M. M., Ward, C., Haq, I., Gray, R. D., & Brodlie, M. (2024) · BMJ Open Respiratory Research · doi:10.1136/bmjresp-2024-002456 · Cited by 12
Self-citation
Genetic engineering drives the breakthrough of pig models in liver disease research
Xu, C., Fang, X., Xu, X., & Wei, X. (2024) · Liver Research · doi:10.1016/j.livres.2024.09.003 · Cited by 12
Population Pharmacokinetics of Elexacaftor, Tezacaftor and Ivacaftor in a Real-World Cohort of Adults with Cystic Fibrosis
Magnas, P., Bouazza, N., Foissac, F., Bournaud, L. F., Lui, G., Carlier, N., Silva, J. D., Fesenbeckh, J., Kanaan, R., Honoré, I., et al. (2025) · Clinical Pharmacokinetics · doi:10.1007/s40262-025-01516-1 · Cited by 12
Anticipating New Treatments for Cystic Fibrosis: A Global Survey of Researchers
Cabral, B. P., Terlizzi, V., Laselva, O., Filho, C. G. C., & Mota, F. B. (2022) · Journal of Clinical Medicine · doi:10.3390/jcm11051283 · Cited by 11
SLC6A14 Impacts Cystic Fibrosis Lung Disease Severity via mTOR and Epithelial Repair Modulation
Mercier, J., Calmel, C., Mésinèle, J., Sutanto, E. N., Merabtene, F., Longchampt, É., Sage, É., Kicic, A., Boëlle, P., Corvol, H., et al. (2022) · Frontiers in Molecular Biosciences · doi:10.3389/fmolb.2022.850261 · Cited by 12
Targeting HuR-Vav3 mRNA interaction prevents Pseudomonas aeruginosa adhesion to the cystic fibrosis airway epithelium
Badaoui, M., Sobolewski, C., Lüscher, A., Bacchetta, M., Köhler, T., van Delden, C., Foti, M., & Chanson, M. (2023) · JCI Insight · doi:10.1172/jci.insight.161961 · Cited by 12
Home Spirometry in Children with Cystic Fibrosis
Berlinski, A., Leisenring, P., Willis, L. C., & King, S. E. (2023) · Bioengineering · doi:10.3390/bioengineering10020242 · Cited by 12
The Role of a Cholecystokinin Receptor Antagonist in the Management of Chronic Pancreatitis: A Phase 1 Trial
Ciofoaia, V., Chen, W., Bakain, T. W., Gay, M. D., Shivapurkar, N., & Smith, J. P. (2024) · Pharmaceutics · doi:10.3390/pharmaceutics16050611 · Cited by 12
Pancreatic Crosstalk in the Disease Setting: Understanding the Impact of Exocrine Disease on Endocrine Function
Villaca, C. B. P., & Mastracci, T. L. (2024) · Comprehensive physiology · doi:10.1002/cphy.c230008 · Cited by 13
Ocular Surface Ion Transport and Dry Eye Disease
Lindgren, E. S., Çil, O., Verkman, A. S., & Pasricha, N. D. (2022) · Current Ophthalmology Reports · doi:10.1007/s40135-022-00295-3 · Cited by 11
Using generic drug elexacaftor/tezacaftor/ivacaftor+ ivacaftor in patients with cystic fidosis in routine clinical practice
Krasovskiy, S., & Kagazezhev, R. U. (2023) · PULMONOLOGIYA · doi:10.18093/0869-0189-2023-33-6-781-791 · Cited by 11
Accuracy, Reproducibility, and Responsiveness to Treatment of Home Spirometry in Cystic Fibrosis: Multicenter, Retrospective, Observational Study
Oppelaar, M. C., van Helvoort, H. A., Bannier, M. A. G. E., Reijers, M. H. E., van der Vaart, H., van der Meer, R., Altenburg, J., Conemans, L. H., Rottier, B. L., Nuijsink, M., et al. (2024) · Journal of Medical Internet Research · doi:10.2196/60892 · Cited by 11
Antibiotic treatment of bacterial lung infections in cystic fibrosis
Taccetti, G., Terlizzi, V., Campana, S., Dolce, D., Ravenni, N., Fevola, C., Francalanci, M., Galici, V., & Neri, A. S. (2024) · European Journal of Pediatrics · doi:10.1007/s00431-024-05905-9 · Cited by 11
Redox Imbalance in Cystic Fibrosis: The Multifaceted Role of Oxidative Stress
Artusi, I., Rubin, M., & Cozza, G. (2025) · Pharmaceuticals · doi:10.3390/ph18060784 · Cited by 11
Bicarbonate defective CFTR variants increase risk for chronic pancreatitis: A meta-analysis
Berke, G., Gede, N., Szadai, L., Ocskay, K., Hegyi, P., Sahin‐Tóth, M., & Hegyi, E. (2022) · PLoS ONE · doi:10.1371/journal.pone.0276397 · Cited by 11
Lung-on-chip: Its current and future perspective on pharmaceutical and biomedical applications
De, A., Paul, S., Reddy, Y. N., Sharma, V., Bhaumik, J., & Tippavajhala, V. K. (2022) · Journal of Drug Delivery Science and Technology · doi:10.1016/j.jddst.2022.103930 · Cited by 11
Cell signaling and regulation of CFTR expression in cystic fibrosis cells in the era of high efficiency modulator therapy
Ghigo, A., Santi, C. D., Hart, M., Mitash, N., & Swiatecka‐Urban, A. (2023) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2022.12.015 · Cited by 11
Extracellular vesicles-derived miRNAs as mediators of pulmonary exacerbation in pediatric cystic fibrosis
Stachowiak, Z., Wojsyk‐Banaszak, I., Jończyk‐Potoczna, K., Narożna, B., Langwiński, W., & Szczepankiewicz, A. (2023) · Journal of Breath Research · doi:10.1088/1752-7163/acb792 · Cited by 11
The natural history of cystic fibrosis liver disease a prospective cohort study
Rowland, M., Drummond, J., Connolly, L., Daly, E., McCormick, P. A., Bourke, B., Amjad, A., Broderick, A., Brian, C., Cox, D. W., et al. (2023) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2023.07.002 · Cited by 11
Nutritional management of people living with cystic fibrosis throughout life and disease continuum: Changing times, new challenges
Mailhot, G., Denis, M., Beauchamp‐Parent, C., & Jomphe, V. (2023) · Journal of Human Nutrition and Dietetics · doi:10.1111/jhn.13214 · Cited by 12
Efficacy and safety profile of elexacaftor-tezacaftor-ivacaftor triple therapy on cystic fibrosis: a systematic review and single arm meta-analysis
Xu, W., Wu, T., Zhou, Z., & Zuo, Z. (2023) · Frontiers in Pharmacology · doi:10.3389/fphar.2023.1275470 · Cited by 11
Therapeutic Drug Monitoring of Elexacaftor, Tezacaftor, and Ivacaftor in Adult People with Cystic Fibrosis
Naehrig, S., Shad, C., Breuling, M., Goetschke, M., Habler, K., Sieber, S., Kastenberger, J., Kunzelmann, A. K., Sommerburg, O., Liebchen, U., et al. (2024) · Journal of Personalized Medicine · doi:10.3390/jpm14101065 · Cited by 12
Trends in and Outcomes of Deliveries Complicated by Cystic Fibrosis
Schmidt, C. N., Friedman, A. M., DiMango, E., Linder, A. H., Sobhani, N. C., D’Alton, M. E., & Wen, T. (2022) · Obstetrics and Gynecology · doi:10.1097/aog.0000000000004959 · Cited by 10
Supramolecular Ion Channel with Controlled K+/Na+ Transport Behaviors
He, L., Luo, Y., Zhang, Y., Yan, T., & Liu, J. (2024) · Chemical Research in Chinese Universities · doi:10.1007/s40242-024-4149-7 · Cited by 10
“Shifting sands in cystic fibrosis”: impacts of CFTR modulators on reproductive health in people with cystic fibrosis and challenges related to in utero exposure
Felton, I. C., Downes, A., Bokobza, I., Weitnauer, L., & Davies, J. C. (2024) · Expert Opinion on Pharmacotherapy · doi:10.1080/14656566.2024.2426677 · Cited by 10
Self-citation
CRISPR for cystic fibrosis: Advances and insights from a systematic review
Nicosia, L., & Harrison, P. T. (2025) · Molecular Therapy · doi:10.1016/j.ymthe.2025.06.021 · Cited by 11
SLC26A9 as a Potential Modifier and Therapeutic Target in Cystic Fibrosis Lung Disease
Gorrieri, G., Zara, F., & Scudieri, P. (2022) · Biomolecules · doi:10.3390/biom12020202 · Cited by 10
CFTR Modulator Therapy for Rare CFTR Mutants
Mergiotti, M., Murabito, A., Prono, G., & Ghigo, A. (2022) · Journal of Respiration · doi:10.3390/jor2020005 · Cited by 11
Genetic Testing in Acute and Chronic Pancreatitis
Piseddu, I., Vielhauer, J., & Mayerle, J. V. (2022) · Current Treatment Options in Gastroenterology · doi:10.1007/s11938-022-00383-0 · Cited by 10
Long-Term Follow-Up of Health-Related Quality of Life and Short-Term Intervention with CFTR Modulator Therapy in Adults with Cystic Fibrosis: Evaluation of Changes over Several Years with or without 33 Weeks of CFTR Modulator Therapy
Gruber, W., Welsner, M., Blosch, C., Dillenhoefer, S., Olivier, M., Brinkmann, F., Koerner‐Rettberg, C., Sutharsan, S., Mellies, U., Taube, C., et al. (2023) · Healthcare · doi:10.3390/healthcare11212873 · Cited by 10
Hyperpolarized Gas Imaging in Lung Diseases: Functional and Artificial Intelligence Perspective
Zhang, Z., Li, H., Xiao, S., Zhou, Q., Liu, S., Zhou, X. H., & Fan, L. (2024) · Academic Radiology · doi:10.1016/j.acra.2024.01.014 · Cited by 10

Authors: Shteinberg, M., Haq, I. J., Polineni, D., & Davies, J. C.
Affiliations: Technion – Israel Institute of Technology (IL)
Newcastle upon Tyne Hospitals NHS Foundation Trust (GB)
University of Kansas Medical Center (US)
Guy's and St Thomas' NHS Foundation Trust (GB)
ORCID: Shteinberg, Michal
Haq, Iram J
Polineni, Deepika
Davies, Jane C
Venue: The Lancet
Publisher: Elsevier BV
Published: 2021-06
Volume: 397
Issue: 10290
Pages: 2195-2211
ISSN: 0099-5355 (ISSN-L)
0140-6736
DOI: 10.1016/s0140-6736(20)32542-3
OpenAlex: W4205612199
Cited by: 682
References: 195
Citations by year: 2026: 96 · 2025: 132 · 2024: 147 · 2023: 160 · 2022: 131 · 2021: 14
FWCI: 18.21
Type: journal article
Language: en
Open access: green
doi.org
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
www.elsevier.com/legal/tdmrep-license (tdm)
Locations: The Lancet (publishedVersion)
PubMed (publishedVersion)
Newcastle University ePrints (Newcastle Univesity)
Funders: National Institute for Health and Care Research (NIHR201402, CL-2018-01-002)
Cystic Fibrosis Trust (SRC 014)
Academy of Medical Sciences (SGL024\1027)
Topics: Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Kidney Cyst Diseases
Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Neonatal Respiratory Health Research
Keywords: cystic fibrosis, CFTR mutations, CFTR function, mucus hydration, chronic lung infection, exocrine pancreatic insufficiency, male infertility, cystic fibrosis-related diabetes, cystic fibrosis liver disease, newborn screening, sweat test, CFTR modulator therapy, mucociliary clearance, disease pathophysiology, multidisciplinary care
MeSH terms: Bicarbonates, Chlorides, Cystic Fibrosis, Humans, Life Expectancy, Mucociliary Clearance, Mutation, Exocrine Pancreatic Insufficiency, Genetic Therapy, Cystic Fibrosis Transmembrane Conductance Regulator, Disease Management

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review

Cystic fibrosis

Abstract

Overview

Integrity signals

Verification

Claims the paper makes

Citations

Cites

Cited by

Related

Metadata

Record

About these checks