The DOI matches its Crossref record; 194 of the 195 references it cites were found in a registry or an index.
Cystic fibrosis
Open accessAbstract
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.
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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-citationA 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-citationLumacaftor–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-citationHeijerman, 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
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
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
Cited by
Sorted by citations
Items 601–685 of 685
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Motta, H., Reuwsaat, J. C. V., Lopes, F. C., Viezzer, G., Volpato, F. C. Z., Barth, A. L., de Tarso Roth Dalcin, P., Staats, C. C., Vainstein, M. H., & Kmetzsch, L. (2024) · Figshare · doi:10.6084/m9.figshare.25854655
Motta, H., Reuwsaat, J. C. V., Lopes, F. C., Viezzer, G., Volpato, F. C. Z., Barth, A. L., de Tarso Roth Dalcin, P., Staats, C. C., Vainstein, M. H., & Kmetzsch, L. (2024) · Figshare · doi:10.6084/m9.figshare.26722452
Motta, H., Reuwsaat, J. C. V., Lopes, F. C., Viezzer, G., Volpato, F. C. Z., Barth, A. L., de Tarso Roth Dalcin, P., Staats, C. C., Vainstein, M. H., & Kmetzsch, L. (2024) · Figshare · doi:10.6084/m9.figshare.26722449
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Dasgupta, S., Datta, D., Pal, S., Ghosh, K., & Sett, S. (2025) · Cureus · doi:10.7759/cureus.97373
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Louw, E. H., van Heerden, J. K., Buckley, A., & Allwood, B. (2025) · Respiratory medicine · doi:10.1007/978-3-032-03581-3_12
Blankenship, S., Flueckiger, B. V., Caleb, G., Bailey, J., Baker, E. H., Garcia, B., & Krick, S. (2026) · Therapeutic Advances in Respiratory Disease · doi:10.1177/17534666251410246
Roldán, D. S., León-Póo, M., Melero, E. L., & Shabaka, A. (2026) · The Nephron journals/Nephron journals · doi:10.1159/000550562
Rayudu, S., Manoranjani, M., & D, A. (2026) · International Journal of Applied Pharmaceutics · doi:10.22159/ijap.2026v18i1.55081
Skogeland, U., de Monestrol, I., & Godskesen, T. (2026) · International Journal of Qualitative Studies on Health and Well-Being · doi:10.1080/17482631.2026.2620417
CFTR knock-out mice exhibit impaired renal compensation to dietary NaCl depletion
Berg, P., Rousing, A. Q., Jensen‐Fangel, S., Jeppesen, M., Sørensen, M. V., & Leipziger, J. (2026) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2026.03.004
Liu, J., Ma, L., Yu, W., Zhou, J., Zhou, G., Tuhongjiang, Y., Maitusong, G., Wang, Z., Aizeze, S., Tian, D., et al. (2026) · Orphanet Journal of Rare Diseases · doi:10.1186/s13023-026-04290-w
Chmiel, J. F., McBennett, K., Quon, B. S., Colombo, C., Wachter, E. D., Fajac, I., Mondéjar-López, P., Polineni, D., Robinson, P. J., Sutharsan, S., et al. (2026) · Annals of the American Thoracic Society · doi:10.1093/annalsats/aaoag053
Self-citationAndreo-López, M. C., Contreras-Bolívar, V., Matías-Marín, M. D. S., Cabreja, J. A. Á., Suárez-Catalina, A., Maldonado, F. C., Merlos-Navarro, S., Olveira, C., Olveira, G., Vaquero-Barrios, J. M., et al. (2026) · Journal of Clinical Medicine · doi:10.3390/jcm15062366
Saag, J. L., Bergeron, J., Bailey, J., Monroe, K., Hathorne, H. Y., Solomon, G. M., Lowman, J. D., Bhatt, S. P., Garcia, B., & Krick, S. (2026) · Journal of Functional Morphology and Kinesiology · doi:10.3390/jfmk11020136
Andreev, E. A., Borisov, D. E., Alekseeva, A. I., Vavilov, I. V., Krasovskiy, S., Baranova, I. A., Chuchalin, A. G., & Karyakin, A. A. (2026) · Analytical Chemistry · doi:10.1021/acs.analchem.5c08107
Wall, B. L., Slaven, J. E., Bozic, M. A., Maguiness, K. M., Ren, C. L., Chmiel, J. F., & Sanders, D. B. (2026) · BMJ Open Respiratory Research · doi:10.1136/bmjresp-2025-003576
Réponse clinique aux modulateurs de la protéine CFTR, des mécanismes d'action aux biomarqueurs
Lepissier, A. (2022) · theses.fr (ABES) · doi:10.70675/7f348587z8dfaz45f1z8389z099b3e7b20a0
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Bouderlique, É. (2022) · doi:10.70675/00aca53fz9a20z4df9za5f6zdb830387bf4c
Sergheraert, J. (2023) · theses.fr (ABES) · doi:10.70675/18e53311z2e21z4c03zb0c3z5d816afda432
Cystic fibrosis care today—a narrative review based on views of experts, patients and the public
Singer, F., Schaffer, A. M., Auer, M., Auer, J., Pansy, J., Stadlbauer, V., & Eber, E. (2026) · Wiener klinische Wochenschrift · doi:10.1007/s00508-026-02739-3
Dargham, T. (2024) · theses.fr (ABES) · doi:10.70675/9ba0e68dz056fz48f7z9a02ze01f1e4b1606
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Fan, J., Wu, W., Dou, X., Huo, J., Tang, X., Li, S., & Bao, C. (2026) · Organic & Biomolecular Chemistry · doi:10.1039/d6ob00449k
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Gan‐Or, Z. (2026) · Movement Disorders · doi:10.1002/mds.70328
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Panou, T., Gouveri, E., Popovic, D. S., & Παπάνας, Ν. (2026) · Advances in Therapy · doi:10.1007/s12325-026-03599-z
Sperimentale, S. I. D. B. (2026) · Journal of Biological Research - Bollettino della Società Italiana di Biologia Sperimentale · doi:10.4081/jbr.2026.15415
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Stepanenko, T. A., Moshkova, E. Е., & Dan'shina, E. N. (2026) · PULMONOLOGIYA · doi:10.18093/0869-0189-2026-36-2-288-294
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Ankermann, P., Jenne, S. I., Talarek, J., Heger, L., & Socher, E. (2026) · Computational and Structural Biotechnology Journal · doi:10.34133/csbj.0101
Umbach, A., Santini, A., Bulcaen, M., Guidone, D., Maule, G., Arosio, D., Carrozzo, I., Ciciani, M., Brugnara, E., Ramalho, A. S., et al. (2026) · Science Translational Medicine · doi:10.1126/scitranslmed.adw8886
Santos, G., Montera, L. C., Genaro, L. M., Rodrigues, B. A. G., Leal, R. F., Ribeiro, A. F., Ribeiro, J. D., & Lomazi, E. A. (2026) · Jornal de Pediatria · doi:10.1016/j.jped.2026.101552
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Farinha, C. M. (2026) · European Respiratory Journal · doi:10.1183/13993003.02740-2025
Daly, C., Burgel, P., Sermet‐Gaudelus, I., Baxter, C. A., Payen-Champenois, C., Giraudier, O., Wöhling, H., Pafundi, P. C., Bessou, A., & Vega-Hernandez, G. (2026) · ERJ Open Research · doi:10.1183/23120541.00011-2026
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Linde, L. (2026) · physiopraxis · doi:10.1055/a-2807-6683
Basu, S., Halder, P. P., Roy, P., & Ghosh, A. (2026) · Indian Journal of Precision Medicine and Molecular Medicine · doi:10.4103/ijpmmm.ijpmmm_7_26
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Faverio, P., Capuano, V., Franco, G., Antonio, M., Panetta, E., Zanini, U., Maggioni, E. C., Pozzi, P., Castello, L., Aliberti, S., et al. (2026) · Expert Review of Respiratory Medicine · doi:10.1080/17476348.2026.2675617
Braddish, S., Houghton, S., Ruane‐McAteer, E., & Fortune, D. G. (2026) · Journal of Health Psychology · doi:10.1177/13591053261446886
Reproductive Considerations in Females With Cystic Fibrosis
Weiss, M. S., Chin, J. M., Mody, S. K., & Roe, A. H. (2026) · Endocrine Practice · doi:10.1016/j.eprac.2026.04.021
Fuchs, T., Niedermayr, K., Siedl, A., Gasser, V., Eder, J., Ellemunter, H., & Appelt, D. (2026) · ERJ Open Research · doi:10.1183/23120541.01528-2025
Sangiorgi, G., Cavinato, L., Cristoferi, M., Chiappetta, D., Pastore, V., Cimino, G., Cimino, L., Ascenzioni, F., & Porto, P. D. (2026) · Respiratory Medicine · doi:10.1016/j.rmed.2026.108930
Ueno-Shuto, K., Fukuyama, A., Nakajima, K., Hitora, Y., Tsukamoto, S., Kishimoto, T., Kawano, K., Nishi, K., Suico, M. A., & Shuto, T. (2026) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.64898/2026.06.02.729465
CFTR modulators: a new class of antimicrobials in lung infection?
Erfinanda, L., & Kuebler, W. M. (2026) · American Journal of Physiology-Lung Cellular and Molecular Physiology · doi:10.1152/ajplung.00099.2026
McCarron, A., Reyne, N., Boog, B., Cmielewski, P., & Donnelley, M. (2026) · Gene Therapy · doi:10.1038/s41434-026-00652-y
Akyazı, N., Kemer, S. N., Vardar‐Yağlı, N., Sağlam, M., Damadoğlu, E., Karcıoğlu, O., & Bilgin, S. (2026) · Respiratory Medicine · doi:10.1016/j.rmed.2026.108963
Forgione, F., Canessa, C., Taccetti, G., Fevola, C., Azzari, C., Ricci, S., & Terlizzi, V. (2026) · Human Vaccines & Immunotherapeutics · doi:10.1080/21645515.2026.2693339
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Roberts, J., Moore, J. E., & Millar, B. C. (2026) · Journal of Preventive Diagnostic and Treatment Strategies in Medicine · doi:10.4103/jpdtsm.jpdtsm_41_26
Ke, L.-Q., Guan, L., Bao, Y., & Tang, C. (2026) · Genes · doi:10.3390/genes17070743
Santos, G. A., Daltro, C., & Souza, E. L. (2026) · Revista Paulista de Pediatria · doi:10.1590/1984-0462/2026/44/2025204
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Tupini, C., Capurro, V., Pedemonte, N., Cabrini, G., & Lampronti, I. (2026) · Research Square · doi:10.21203/rs.3.rs-10282441/v1
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Liu, B., & Xie, M. (2026) · Rare Disease and Orphan Drugs Journal · doi:10.20517/rdodj.2025.88
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Terracciano, R., Cerchione, R., Cimbalo, C., Buonpensiero, P., Pasqua, A. D., Rosa, E. D., Salle, F. D., Castaldo, A., Capasso, F., Tosco, A., et al. (2026) · Respiratory Medicine · doi:10.1016/j.rmed.2026.109065
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Kunzelmann, A. K., Naehrig, S., Shad, C., Breuling, M., Goetschke, M., Habler, K., Sommerburg, O., Behr, J., Schatz, L., Starp, J., et al. (2026) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2026.07.1984
Lin, S. J., Lan, R., Zhu, X., Shen, R., Liu, R., Cheng, J., & Liu, X. (2026) · Pharmaceutics · doi:10.3390/pharmaceutics18080945
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Tavares, T. S., Barbosa, D. S. L., de Souza, R. P., da Silva, R., Leal, T. P., de Oliveira, M. D., Silva-Carvalho, C., Marchionni, L., Gouveia, M. H., & Lobo, F. P. (2026) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.64898/2026.08.03.742387
Létourneau, E., Goncalves, O., Côté, J., & Jean-Pierre, F. (2026) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.64898/2026.08.08.743698
Sergienko, D. F. S., Golubtsova, O. I., & Enina, E. A. (2026) · ASTRAKHAN MEDICAL JOURNAL · doi:10.17021/1992-6499-2026-2-173-183
Wanczyk, H., Kosciuszek, N. D., Walker, J., Weiss, D. J., & Finck, C. M. (2026) · International Journal of Bioprinting · doi:10.36922/ijb026280295
Buin, X., Ghanem, R., Pankonien, I., Becq, F., Amaral, M. D., & Montier, T. (2026) · Pharmaceutics · doi:10.3390/pharmaceutics18081040
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Akyazı, N., Kemer, S. N., Vardar‐Yağlı, N., Sağlam, M., Damadoğlu, E., Karcıoğlu, O., & Bilgin, S. (2026) · Hacettepe Üniversitesi Sağlık Bilimleri Fakültesi Dergisi · doi:10.21020/husbfd.1718948
Mier, N. R., Vermeulen, E., Boon, M., Vermeulen, F. L., Devos, A., Aertsen, M., Richter, J., Debeer, A., & Proesmans, M. (2026) · Fetal Diagnosis and Therapy · doi:10.1159/fdt/acuag006
Martin, D., Novel-Catin, E., Raphaële, N.-J., Fouque, D. P., & Durieu, I. (2026) · Journal of Nephrology · doi:10.1093/joneph/aajag182
Boulic, P., Leven, C., Li, D., Zhu, Y., Diniz, J. A., Gouriou, S., Schneider‐Futschik, E. K., & Héry-Arnaud, G. (2026) · Biomedicine & Pharmacotherapy · doi:10.1016/j.biopha.2026.119892
Le, A., Girodon, E., & Sermet‐Gaudelus, I. (2026) · Encyclopédie médico-chirurgicale. Pneumologie · doi:10.1016/s1155-195x(26)47979-7
Rybachok, O. A. (2026) · Spravočnik vrača obŝej praktiki (Journal of Family Medicine) · doi:10.33920/med-10-2608-08
(2026) · Elsevier eBooks · doi:10.1016/b978-3-437-15042-5.00061-8
Shaikhkhalil, A. K., Abu‐Hasan, M., Sharpe, S., & Asfour, F. (2026) · Nutrients · doi:10.3390/nu18193187
Bortolotti, C., Canali, S., Gramegna, A., Aliverti, A., Blasi, F., & Schiaffonati, V. (2026) · Digital Health · doi:10.1177/20552076261465567
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Romano, M., D’Orazio, S., Mattoscio, D., Mucci, M., Plebani, R., Pillarisetti, S., & Recchiuti, A. (2026) · Current topics in microbiology and immunology · doi:10.1007/82_2026_362
SGLT1 inhibition in cystic fibrosis: From preclinical evidence to clinical potential
Liang, X., Liu, Y., Jia, S., Zhang, K., & Xu, J. (2026) · Molecular Therapy Advances · doi:10.1016/j.omta.2026.201868
Warner, S. O., Whitsett, N. H., Dai, Y., SHEANON, N. M., Hoeting, B., Indihar, V., & Winnick, J. J. (2026) · Journal of Cystic Fibrosis · doi:10.1016/j.jcf.2026.09.016
FRAGOSO, E. C., Azevedo, P., Pereira, L., Barbosa, T., Cardoso, A. L., Cruz, C., Amorim, A. A., Azevedo, I., Almeida, L. M., Castanhinha, S., et al. (2026) · Pulmonology · doi:10.1080/25310429.2026.2742057
Li, S., Liu, Y., Wang, S., Zheng, L., & Zhang, L. (2026) · Microchemical Journal · doi:10.1016/j.microc.2026.120101
Metadata
- 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)
- Venue
- The Lancet
- Publisher
- Elsevier BV
- Published
- 2021-06
- Volume
- 397
- Issue
- 10290
- Pages
- 2195-2211
- ISSN
- 0099-5355 (ISSN-L)
- 0140-6736
- 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
- 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 ›
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