{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,30]],"date-time":"2026-06-30T06:45:49Z","timestamp":1782801949989,"version":"3.54.5"},"reference-count":72,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T00:00:00Z","timestamp":1782518400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Genes"],"abstract":"<jats:p>Cystic fibrosis (CF) is an autosomal recessive disorder caused by pathogenic variants in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. It is increasingly understood through a genomics-informed framework linking variant architecture to phenotype, diagnosis, and therapeutic eligibility. This review summarizes current evidence on CFTR structure, variant interpretation, genotype-phenotype heterogeneity, diagnostic workflows, and modern management. We highlight how full-gene sequencing, curated disease-liability databases, functional testing, and organoid-informed theratyping refine diagnosis and treatment selection; how disrupted chloride and bicarbonate transport drives muco-obstructive airway disease and multisystem complications; and why modulator therapy must still be integrated with respiratory, nutritional, endocrine, hepatobiliary, reproductive, and psychosocial care. We also outline unresolved challenges in rare variants, residual organ damage, ancestry-related diagnostic gaps, and mutation-agnostic therapeutic development.<\/jats:p>","DOI":"10.3390\/genes17070743","type":"journal-article","created":{"date-parts":[[2026,6,30]],"date-time":"2026-06-30T05:58:04Z","timestamp":1782799084000},"page":"743","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Cystic Fibrosis: Modern Diagnostic and Therapeutic Advances from Molecular Pathogenesis to Multidisciplinary Management"],"prefix":"10.3390","volume":"17","author":[{"given":"Liqin","family":"Ke","sequence":"first","affiliation":[{"name":"Children\u2019s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Children and Adolescents\u2019 Health and Diseases, Hangzhou 310052, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lijun","family":"Guan","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Children and Adolescents\u2019 Health and Diseases, Hangzhou 310052, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-2185-2122","authenticated-orcid":false,"given":"Yiyao","family":"Bao","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Children and Adolescents\u2019 Health and Diseases, Hangzhou 310052, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5072-5849","authenticated-orcid":false,"given":"Chao","family":"Tang","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Children and Adolescents\u2019 Health and Diseases, Hangzhou 310052, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,6,27]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2519","DOI":"10.1016\/S0140-6736(16)00576-6","article-title":"Cystic fibrosis","volume":"388","author":"Elborn","year":"2016","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1038\/s41572-024-00538-6","article-title":"Cystic fibrosis","volume":"10","author":"Mall","year":"2024","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"15010","DOI":"10.1038\/nrdp.2015.10","article-title":"Cystic fibrosis","volume":"1","author":"Ratjen","year":"2015","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"2195","DOI":"10.1016\/S0140-6736(20)32542-3","article-title":"Cystic fibrosis","volume":"397","author":"Shteinberg","year":"2021","journal-title":"Lancet"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1055\/s-0039-1694021","article-title":"Cystic Fibrosis: Pathophysiology of Lung Disease","volume":"40","author":"Bergeron","year":"2019","journal-title":"Semin. Respir. Crit. Care Med."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1757","DOI":"10.1152\/physrev.00004.2021","article-title":"Physiology and pathophysiology of human airway mucus","volume":"102","author":"Hill","year":"2022","journal-title":"Physiol. Rev."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1056\/NEJMra1300109","article-title":"Origins of cystic fibrosis lung disease","volume":"372","author":"Stoltz","year":"2015","journal-title":"N. Engl. J. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1016\/j.jcf.2018.02.006","article-title":"ECFS best practice guidelines: The 2018 revision","volume":"17","author":"Castellani","year":"2018","journal-title":"J. Cyst. Fibros."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"S4","DOI":"10.1016\/j.jpeds.2016.09.064","article-title":"Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation","volume":"181S","author":"Farrell","year":"2017","journal-title":"J. Pediatr."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1016\/j.jcf.2024.05.007","article-title":"Standards for the care of people with cystic fibrosis (CF); Planning for a longer life","volume":"23","author":"Gramegna","year":"2024","journal-title":"J. Cyst. Fibros."},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"McGarry, M.E., Raraigh, K.S., Farrell, P., Shropshire, F., Padding, K., White, C., Dorley, M.C., Hicks, S., Ren, C.L., and Tullis, K. (2025). Cystic Fibrosis Newborn Screening: A Systematic Review-Driven Consensus Guideline from the United States Cystic Fibrosis Foundation. Int. J. Neonatal Screen., 11.","DOI":"10.3390\/ijns11020024"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1016\/j.jcf.2021.07.019","article-title":"Pregnancy in cystic fibrosis: Review of the literature and expert recommendations","volume":"21","author":"Jain","year":"2022","journal-title":"J. Cyst. Fibros."},{"key":"ref_13","doi-asserted-by":"crossref","unstructured":"Kayani, K., Mohammed, R., and Mohiaddin, H. (2018). Cystic Fibrosis-Related Diabetes. Front. Endocrinol., 9.","DOI":"10.3389\/fendo.2018.00020"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"210157","DOI":"10.1183\/20734735.0157-2021","article-title":"Transition from paediatric to adult care in cystic fibrosis","volume":"18","author":"Office","year":"2022","journal-title":"Breathe"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1136\/thoraxjnl-2015-207488","article-title":"International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety","volume":"71","author":"Quittner","year":"2016","journal-title":"Thorax"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1220","DOI":"10.1097\/HEP.0000000000000646","article-title":"Cystic fibrosis screening, evaluation, and management of hepatobiliary disease consensus recommendations","volume":"79","author":"Sellers","year":"2024","journal-title":"Hepatology"},{"key":"ref_17","first-page":"23","article-title":"Transition to adult care in cystic fibrosis: The challenges and the structure","volume":"41","author":"Singh","year":"2022","journal-title":"Paediatr. Respir. Rev."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1038\/nrg3849","article-title":"Cystic fibrosis genetics: From molecular understanding to clinical application","volume":"16","author":"Cutting","year":"2015","journal-title":"Nat. Rev. Genet."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1016\/j.biocel.2014.02.023","article-title":"Genetics of cystic fibrosis: CFTR mutation classifications toward genotype-based CF therapies","volume":"52","author":"Fanen","year":"2014","journal-title":"Int. J. Biochem. Cell. Biol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1185","DOI":"10.1016\/S0140-6736(23)01608-2","article-title":"The future of cystic fibrosis treatment: From disease mechanisms to novel therapeutic approaches","volume":"402","author":"Graeber","year":"2023","journal-title":"Lancet"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"eS13","DOI":"10.1016\/S0929-693X(20)30045-2","article-title":"Variant classifications, databases and genotype-phenotype correlations","volume":"27","author":"Raynal","year":"2020","journal-title":"Arch. Pediatr."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1160","DOI":"10.1038\/ng.2745","article-title":"Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene","volume":"45","author":"Sosnay","year":"2013","journal-title":"Nat. Genet."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1171","DOI":"10.1016\/S0140-6736(23)01609-4","article-title":"CFTR modulator therapy: Transforming the landscape of clinical care in cystic fibrosis","volume":"402","author":"Robinson","year":"2023","journal-title":"Lancet"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1940","DOI":"10.1016\/S0140-6736(19)32597-8","article-title":"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","volume":"394","author":"Heijerman","year":"2019","journal-title":"Lancet"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1612","DOI":"10.1056\/NEJMoa1807120","article-title":"VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles","volume":"379","author":"Keating","year":"2018","journal-title":"N. Engl. J. Med."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1056\/NEJMoa1908639","article-title":"Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele","volume":"381","author":"Middleton","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1056\/NEJMoa1105185","article-title":"A CFTR potentiator in patients with cystic fibrosis and the G551D mutation","volume":"365","author":"Ramsey","year":"2011","journal-title":"N. Engl. J. Med."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"2024","DOI":"10.1056\/NEJMoa1709847","article-title":"Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis","volume":"377","author":"Rowe","year":"2017","journal-title":"N. Engl. J. Med."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"2013","DOI":"10.1056\/NEJMoa1709846","article-title":"Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del","volume":"377","author":"Munck","year":"2017","journal-title":"N. Engl. J. Med."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1056\/NEJMoa1409547","article-title":"Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR","volume":"373","author":"Wainwright","year":"2015","journal-title":"N. Engl. J. Med."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"815","DOI":"10.1056\/NEJMoa2100665","article-title":"VX18-445-104 Study Group. Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes","volume":"385","author":"Barry","year":"2021","journal-title":"N. Engl. J. Med."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"888","DOI":"10.1016\/S2213-2600(24)00208-X","article-title":"The expanded French compassionate programme for elexacaftor-tezacaftor-ivacaftor use in people with cystic fibrosis without a F508del CFTR variant: A real-world study","volume":"12","author":"Burgel","year":"2024","journal-title":"Lancet Respir. Med."},{"key":"ref_33","doi-asserted-by":"crossref","unstructured":"Regard, L., Martin, C., Burnet, E., Da Silva, J., and Burgel, P.R. (2022). CFTR Modulators in People with Cystic Fibrosis: Real-World Evidence in France. Cells, 11.","DOI":"10.3390\/cells11111769"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1016\/S2213-2600(21)00454-9","article-title":"Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: A 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial","volume":"10","author":"Sutharsan","year":"2022","journal-title":"Lancet Respir. Med."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1522","DOI":"10.1164\/rccm.202102-0509OC","article-title":"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","volume":"203","author":"Zemanick","year":"2021","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/j.jcf.2019.03.002","article-title":"Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines","volume":"18","author":"Ramos","year":"2019","journal-title":"J. Cyst. Fibros."},{"key":"ref_37","first-page":"100291","article-title":"Treatment of cystic fibrosis related bone disease","volume":"27","author":"Ullal","year":"2022","journal-title":"J. Clin. Transl. Endocrinol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1136\/jmg-2022-108501","article-title":"Genetic spectrum of Chinese children with cystic fibrosis: Comprehensive data analysis from the main referral centre in China","volume":"60","author":"Shen","year":"2022","journal-title":"J. Med. Genet."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1038\/ejhg.2008.136","article-title":"Best practice guidelines for molecular genetic diagnosis of cystic fibrosis and CFTR-related disorders\u2014Updated European recommendations","volume":"17","author":"Dequeker","year":"2009","journal-title":"Eur. J. Hum. Genet."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1016\/j.jcf.2021.10.011","article-title":"Complete CFTR gene sequencing in 5,058 individuals with cystic fibrosis informs variant-specific treatment","volume":"21","author":"Raraigh","year":"2022","journal-title":"J. Cyst. Fibros."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"S27","DOI":"10.1016\/j.jpeds.2016.09.063","article-title":"Applying Cystic Fibrosis Transmembrane Conductance Regulator Genetics and CFTR2 Data to Facilitate Diagnoses","volume":"181","author":"Sosnay","year":"2017","journal-title":"J. Pediatr."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"949","DOI":"10.1016\/j.jcf.2020.03.019","article-title":"Penetrance is a critical parameter for assessing the disease liability of CFTR variants","volume":"19","author":"Boussaroque","year":"2020","journal-title":"J. Cyst. Fibros."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"S33","DOI":"10.1016\/j.jpeds.2016.09.065","article-title":"Diagnosis of Cystic Fibrosis in Screened Populations","volume":"181","author":"Farrell","year":"2017","journal-title":"J. Pediatr."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"e2023064657","DOI":"10.1542\/peds.2023-064657","article-title":"Cystic Fibrosis Foundation Evidence-Based Guideline for the Management of CRMS\/CFSPID","volume":"153","author":"Green","year":"2024","journal-title":"Pediatrics"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"898","DOI":"10.1373\/clinchem.2017.274670","article-title":"Advances in the Diagnosis and Management of Cystic Fibrosis in the Genomic Era","volume":"64","author":"Wiencek","year":"2018","journal-title":"Clin. Chem."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"e240008","DOI":"10.1148\/rg.240008","article-title":"Update on the Role of Chest Imaging in Cystic Fibrosis","volume":"44","author":"Bugenhagen","year":"2024","journal-title":"RadioGraphics"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1398","DOI":"10.1164\/rccm.202402-0389CI","article-title":"Lentiviral Gene Therapy for Cystic Fibrosis: A Promising Approach and First-in-Human Trial","volume":"210","author":"Davies","year":"2024","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_48","doi-asserted-by":"crossref","unstructured":"Pankonien, I., Quaresma, M.C., Rodrigues, C.S., and Amaral, M.D. (2022). CFTR, Cell Junctions and the Cytoskeleton. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms23052688"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"105614","DOI":"10.1016\/j.compbiomed.2022.105614","article-title":"Analysis of CFTR gene expression as an immunological and prognostic biomarker in pan-cancers","volume":"146","author":"Wang","year":"2022","journal-title":"Comput. Biol. Med."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"e31422","DOI":"10.1002\/jcp.31422","article-title":"The interactome of cystic fibrosis transmembrane conductance regulator and its role in male fertility: A critical review","volume":"239","author":"Ribeiro","year":"2024","journal-title":"J. Cell. Physiol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1038\/s41392-022-01280-9","article-title":"Structural insights into substrate recognition and translocation of human peroxisomal ABC transporter ALDP","volume":"8","author":"Xiong","year":"2023","journal-title":"Signal Transduct. Target. Ther."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1042\/BST20200710","article-title":"Structural dynamics of ABC transporters: Molecular simulation studies","volume":"49","author":"Furuta","year":"2021","journal-title":"Biochem. Soc. Trans."},{"key":"ref_53","doi-asserted-by":"crossref","unstructured":"Conti, J., Sorio, C., and Melotti, P. (2023). Organoid Technology and Its Role for Theratyping Applications in Cystic Fibrosis. Children, 10.","DOI":"10.3390\/children10010004"},{"key":"ref_54","doi-asserted-by":"crossref","unstructured":"M\u00e9sin\u00e8le, J., Ruffin, M., Guillot, L., and Corvol, H. (2022). Modifier Factors of Cystic Fibrosis Phenotypes: A Focus on Modifier Genes. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms232214205"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"240068","DOI":"10.1183\/16000617.0068-2024","article-title":"Changing profile of bacterial infection and microbiome in cystic fibrosis: When to use antibiotics in the era of CFTR-modulator therapy","volume":"33","author":"Milczewska","year":"2024","journal-title":"Eur. Respir. Rev."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"e00138-18","DOI":"10.1128\/CMR.00138-18","article-title":"Cystic Fibrosis and Pseudomonas aeruginosa: The Host-Microbe Interface","volume":"32","author":"Malhotra","year":"2019","journal-title":"Clin. Microbiol. Rev."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"1640","DOI":"10.1513\/AnnalsATS.201404-166OC","article-title":"Cystic Fibrosis Foundation pulmonary guideline. pharmacologic approaches to prevention and eradication of initial Pseudomonas aeruginosa infection","volume":"11","author":"Mogayzel","year":"2014","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"1292","DOI":"10.1053\/j.gastro.2023.07.007","article-title":"AGA Clinical Practice Update on the Epidemiology, Evaluation, and Management of Exocrine Pancreatic Insufficiency: Expert Review","volume":"165","author":"Whitcomb","year":"2023","journal-title":"Gastroenterology"},{"key":"ref_59","doi-asserted-by":"crossref","unstructured":"Pietrzykowska, A., Darmon, J., Szymocha, K., Donocik, W., Tarasiewicz, M., Stadnik, J., and Jarosz-Chobot, P. (2025). Cystic fibrosis-related diabetes in the era of modern treatment using CFTR modulators in pediatric patients-a systematic review. Front. Pediatr., 13.","DOI":"10.3389\/fped.2025.1688862"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"1148","DOI":"10.1002\/alr.22953","article-title":"The management of cystic fibrosis chronic rhinosinusitis: An evidenced-based review with recommendations","volume":"12","author":"Spielman","year":"2022","journal-title":"Int. Forum Allergy Rhinol."},{"key":"ref_61","first-page":"275","article-title":"Role of mucociliary clearance system in respiratory diseases","volume":"48","author":"Wu","year":"2023","journal-title":"J. Cent. South Univ. Med. Sci."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"121054","DOI":"10.1016\/j.ijpharm.2021.121054","article-title":"The effect of ingredients commonly used in nasal and inhaled solutions on the secretion of mucus in vitro","volume":"608","author":"Ayoub","year":"2021","journal-title":"Int. J. Pharm."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"1604","DOI":"10.1016\/j.chest.2021.05.013","article-title":"Mucociliary Clearance Differs in Mild Asthma by Levels of Type 2 Inflammation","volume":"160","author":"Corcoran","year":"2021","journal-title":"Chest"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1007\/s12055-021-01223-z","article-title":"Non-pulmonary complications after lung transplantation: Part I","volume":"38","author":"Kanade","year":"2022","journal-title":"Indian J. Thorac. Cardiovasc. Surg."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1007\/s12055-021-01231-z","article-title":"Non-pulmonary complications after lung transplantation: Part II","volume":"38","author":"Kanade","year":"2022","journal-title":"Indian J. Thorac. Cardiovasc. Surg."},{"key":"ref_66","doi-asserted-by":"crossref","unstructured":"Duloquin, G., Pommier, T., Georges, M., Giroud, M., Guenancia, C., B\u00e9jot, Y., Laurent, G., and Rabec, C. (2024). Is COVID-19 Infection a Multiorganic Disease? Focus on Extrapulmonary Involvement of SARS-CoV-2. J. Clin. Med., 13.","DOI":"10.3390\/jcm13051397"},{"key":"ref_67","first-page":"538","article-title":"Cystic fibrosis in childhood and adulthood","volume":"65","author":"Syunyaeva","year":"2024","journal-title":"Inn. Med."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"e002606","DOI":"10.1136\/bmjresp-2024-002606","article-title":"Psychosocial and mental health in cystic fibrosis in the modern era of care: Time to evolve","volume":"12","author":"Harrigan","year":"2025","journal-title":"BMJ Open Respir. Res."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"1569","DOI":"10.1056\/NEJMoa2411664","article-title":"Phase 3 Trial of the DPP-1 Inhibitor Brensocatib in Bronchiectasis","volume":"392","author":"Chalmers","year":"2025","journal-title":"N. Engl. J. Med."},{"key":"ref_70","doi-asserted-by":"crossref","unstructured":"Kouadri, A., Lyko, C., El Hajjar, C., Cormenier, J., Kevin Gemy, K., Alfaidy, N., and Benharouga, M. (2025). Inflammatory response in CF airway epithelial cells: A comparative study of modulators and wild-type CFTR rescue. Front. Pharmacol., 16.","DOI":"10.3389\/fphar.2025.1657688"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"118936","DOI":"10.1016\/j.biopha.2025.118936","article-title":"Trikafta restores thermodynamic coupling between two nucleotide binding domains for potentiating CFTR activity","volume":"194","author":"Wang","year":"2026","journal-title":"Biomed. Pharmacother."},{"key":"ref_72","doi-asserted-by":"crossref","unstructured":"Vitiello, A., Blasi, F., Sabbatucci, M., Zovi, A., Miele, F., Ponzo, A., Langella, R., and Boccellino, M. (2024). The Impact of Antimicrobial Resistance in Cystic Fibrosis. J. Clin. Med., 13.","DOI":"10.3390\/jcm13061711"}],"container-title":["Genes"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2073-4425\/17\/7\/743\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,30]],"date-time":"2026-06-30T06:16:49Z","timestamp":1782800209000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2073-4425\/17\/7\/743"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,27]]},"references-count":72,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2026,7]]}},"alternative-id":["genes17070743"],"URL":"https:\/\/doi.org\/10.3390\/genes17070743","relation":{},"ISSN":["2073-4425"],"issn-type":[{"value":"2073-4425","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,27]]}}}