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For: Rosenfeld M, Cunningham S, Harris WT, Lapey A, Regelmann WE, Sawicki GS, Southern KW, Chilvers M, Higgins M, Tian S, Cooke J, Davies JC; KLIMB study group. 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;18:838-43. [PMID: 31053538 DOI: 10.1016/j.jcf.2019.03.009] [Cited by in Crossref: 38] [Cited by in F6Publishing: 32] [Article Influence: 12.7] [Reference Citation Analysis]
Number Citing Articles
1 Mcgarry ME, Gibb ER, Oates GR, Schechter MS. Left Behind: The Potential Impact of CFTR Modulators on Racial and Ethnic Disparities in Cystic Fibrosis. Paediatric Respiratory Reviews 2021. [DOI: 10.1016/j.prrv.2021.12.001] [Reference Citation Analysis]
2 Guerra L, Favia M, Di Gioia S, Laselva O, Bisogno A, Casavola V, Colombo C, Conese M. The preclinical discovery and development of the combination of ivacaftor + tezacaftor used to treat cystic fibrosis. Expert Opin Drug Discov 2020;15:873-91. [PMID: 32290721 DOI: 10.1080/17460441.2020.1750592] [Cited by in Crossref: 9] [Cited by in F6Publishing: 7] [Article Influence: 4.5] [Reference Citation Analysis]
3 Hoppe JE, Chilvers M, Ratjen F, McNamara JJ, Owen CA, Tian S, Zahigian R, Cornell AG, McColley SA. Long-term safety of lumacaftor-ivacaftor in children aged 2-5 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a multicentre, phase 3, open-label, extension study. Lancet Respir Med 2021:S2213-2600(21)00069-2. [PMID: 33965000 DOI: 10.1016/S2213-2600(21)00069-2] [Cited by in Crossref: 1] [Article Influence: 1.0] [Reference Citation Analysis]
4 Bell SC, Mall MA, Gutierrez H, Macek M, Madge S, Davies JC, Burgel P, Tullis E, Castaños C, Castellani C, Byrnes CA, Cathcart F, Chotirmall SH, Cosgriff R, Eichler I, Fajac I, Goss CH, Drevinek P, Farrell PM, Gravelle AM, Havermans T, Mayer-hamblett N, Kashirskaya N, Kerem E, Mathew JL, Mckone EF, Naehrlich L, Nasr SZ, Oates GR, O'neill C, Pypops U, Raraigh KS, Rowe SM, Southern KW, Sivam S, Stephenson AL, Zampoli M, Ratjen F. The future of cystic fibrosis care: a global perspective. The Lancet Respiratory Medicine 2020;8:65-124. [DOI: 10.1016/s2213-2600(19)30337-6] [Cited by in Crossref: 183] [Cited by in F6Publishing: 82] [Article Influence: 91.5] [Reference Citation Analysis]
5 Somayaji R, Nichols DP, Bell SC. Cystic fibrosis - Ten promising therapeutic approaches in the current era of care. Expert Opin Investig Drugs 2020;29:1107-24. [PMID: 32744089 DOI: 10.1080/13543784.2020.1805733] [Cited by in Crossref: 3] [Cited by in F6Publishing: 3] [Article Influence: 1.5] [Reference Citation Analysis]
6 Guimbellot JS, Taylor-Cousar JL. Combination CFTR modulator therapy in children and adults with cystic fibrosis. Lancet Respir Med 2021;9:677-9. [PMID: 33965001 DOI: 10.1016/S2213-2600(21)00176-4] [Reference Citation Analysis]
7 Aurora P, Duncan JA, Lum S, Davies G, Wade A, Stocks J, Viviani L, Raywood E, Pao C, Ruiz G, Bush A. Early Pseudomonas aeruginosa predicts poorer pulmonary function in preschool children with cystic fibrosis. Journal of Cystic Fibrosis 2022. [DOI: 10.1016/j.jcf.2022.04.013] [Reference Citation Analysis]
8 Daines CL, Morgan WJ. The Future of Highly Effective Modulator Therapy in Cystic Fibrosis. Am J Respir Crit Care Med 2021;203:1453-5. [PMID: 33901406 DOI: 10.1164/rccm.202104-0850ED] [Reference Citation Analysis]
9 Lee AJ, Huffmyer JL, Thiele EL, Zeitlin PL, Chatterjee D. The Changing Face of Cystic Fibrosis: An Update for Anesthesiologists. Anesth Analg 2022. [PMID: 35020677 DOI: 10.1213/ANE.0000000000005856] [Reference Citation Analysis]
10 Flume PA. From the Editor's Desk. J Cyst Fibros 2022;21:197-8. [PMID: 35337599 DOI: 10.1016/j.jcf.2022.03.003] [Reference Citation Analysis]
11 Savant AP, McColley SA. Cystic fibrosis year in review 2019: Section 1 CFTR modulators. Pediatr Pulmonol 2020;55:3236-42. [PMID: 32833326 DOI: 10.1002/ppul.25039] [Cited by in Crossref: 2] [Cited by in F6Publishing: 3] [Article Influence: 1.0] [Reference Citation Analysis]
12 Gariepy CE, Ooi CY, Maqbool A, Ellery KM. Demographics and risk factors for pediatric recurrent acute pancreatitis. Curr Opin Gastroenterol 2021;37:491-7. [PMID: 34120130 DOI: 10.1097/MOG.0000000000000764] [Reference Citation Analysis]
13 Meoli A, Fainardi V, Deolmi M, Chiopris G, Marinelli F, Caminiti C, Esposito S, Pisi G. State of the Art on Approved Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators and Triple-Combination Therapy. Pharmaceuticals (Basel) 2021;14:928. [PMID: 34577628 DOI: 10.3390/ph14090928] [Reference Citation Analysis]
14 Sergeev V, Chou FY, Lam GY, Hamilton CM, Wilcox PG, Quon BS. The Extrapulmonary Effects of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Cystic Fibrosis. Ann Am Thorac Soc 2020;17:147-54. [PMID: 31661636 DOI: 10.1513/AnnalsATS.201909-671CME] [Cited by in Crossref: 24] [Cited by in F6Publishing: 7] [Article Influence: 24.0] [Reference Citation Analysis]
15 King JA, Nichols A, Bentley S, Carr SB, Davies JC. An Update on CFTR Modulators as New Therapies for Cystic Fibrosis. Pediatr Drugs. [DOI: 10.1007/s40272-022-00509-y] [Reference Citation Analysis]
16 Laselva O, Guerra L, Castellani S, Favia M, Di Gioia S, Conese M. Small-molecule drugs for cystic fibrosis: Where are we now? Pulm Pharmacol Ther 2021;:102098. [PMID: 34793977 DOI: 10.1016/j.pupt.2021.102098] [Reference Citation Analysis]
17 Guimbellot JS, Baines A, Paynter A, Heltshe SL, VanDalfsen J, Jain M, Rowe SM, Sagel SD; GOAL-e2 Investigators. Long term clinical effectiveness of ivacaftor in people with the G551D CFTR mutation. J Cyst Fibros 2021;20:213-9. [PMID: 33249004 DOI: 10.1016/j.jcf.2020.11.008] [Cited by in Crossref: 4] [Cited by in F6Publishing: 3] [Article Influence: 2.0] [Reference Citation Analysis]
18 Flume PA, Castellani C, Davies J. JCF Year in Review. J Cyst Fibros 2021;20:1-2. [PMID: 33526211 DOI: 10.1016/j.jcf.2021.01.004] [Reference Citation Analysis]
19 Dang Y, van Heusden C, Nickerson V, Chung F, Wang Y, Quinney NL, Gentzsch M, Randell SH, Moulton HM, Kole R, Ni A, Juliano RL, Kreda SM. Enhanced delivery of peptide-morpholino oligonucleotides with a small molecule to correct splicing defects in the lung. Nucleic Acids Res 2021;49:6100-13. [PMID: 34107015 DOI: 10.1093/nar/gkab488] [Cited by in Crossref: 1] [Article Influence: 1.0] [Reference Citation Analysis]
20 van Koningsbruggen-Rietschel S. Getting Near to "Closing the Gap" in the Pediatric Age Group for the First Personalized Treatment of Cystic Fibrosis. Am J Respir Crit Care Med 2021;203:536-7. [PMID: 33113333 DOI: 10.1164/rccm.202010-3848ED] [Reference Citation Analysis]
21 Munce D, Lim M, Akong K. Persistent recovery of pancreatic function in patients with cystic fibrosis after ivacaftor. Pediatr Pulmonol 2020;55:3381-3. [PMID: 32910556 DOI: 10.1002/ppul.25065] [Cited by in Crossref: 5] [Cited by in F6Publishing: 4] [Article Influence: 2.5] [Reference Citation Analysis]
22 McDonald CM, Alvarez JA, Bailey J, Bowser EK, Farnham K, Mangus M, Padula L, Porco K, Rozga M. Academy of Nutrition and Dietetics: 2020 Cystic Fibrosis Evidence Analysis Center Evidence-Based Nutrition Practice Guideline. J Acad Nutr Diet 2021;121:1591-1636.e3. [PMID: 32565399 DOI: 10.1016/j.jand.2020.03.015] [Cited by in Crossref: 3] [Cited by in F6Publishing: 2] [Article Influence: 1.5] [Reference Citation Analysis]
23 Davies JC, Wainwright CE, Sawicki GS, Higgins MN, Campbell D, Harris C, Panorchan P, Haseltine E, Tian S, Rosenfeld M. Ivacaftor in Infants Aged 4 to <12 Months with Cystic Fibrosis and a Gating Mutation. Results of a Two-Part Phase 3 Clinical Trial. Am J Respir Crit Care Med 2021;203:585-93. [PMID: 33023304 DOI: 10.1164/rccm.202008-3177OC] [Cited by in Crossref: 11] [Cited by in F6Publishing: 4] [Article Influence: 11.0] [Reference Citation Analysis]
24 McBennett KA, Davis PB, Konstan MW. Increasing life expectancy in cystic fibrosis: Advances and challenges. Pediatr Pulmonol 2021. [PMID: 34672432 DOI: 10.1002/ppul.25733] [Cited by in Crossref: 1] [Article Influence: 1.0] [Reference Citation Analysis]
25 Konrad J, Eber E, Stadlbauer V. Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators. Paediatr Respir Rev 2020:S1526-0542(20)30181-0. [PMID: 33485777 DOI: 10.1016/j.prrv.2020.12.001] [Cited by in Crossref: 1] [Cited by in F6Publishing: 1] [Article Influence: 0.5] [Reference Citation Analysis]
26 Bergeron C, Cantin AM. New Therapies to Correct the Cystic Fibrosis Basic Defect. Int J Mol Sci 2021;22:6193. [PMID: 34201249 DOI: 10.3390/ijms22126193] [Cited by in F6Publishing: 1] [Reference Citation Analysis]
27 Nichols AL, Davies JC, Jones D, Carr SB. Restoration of exocrine pancreatic function in older children with cystic fibrosis on ivacaftor. Paediatr Respir Rev 2020;35:99-102. [PMID: 32386958 DOI: 10.1016/j.prrv.2020.04.003] [Cited by in Crossref: 3] [Cited by in F6Publishing: 4] [Article Influence: 1.5] [Reference Citation Analysis]
28 Lahiri T, Sullivan JS. Recent advances in the early treatment of cystic fibrosis: Bridging the gap to highly effective modulator therapy. Pediatr Pulmonol 2021. [PMID: 34473419 DOI: 10.1002/ppul.25660] [Reference Citation Analysis]
29 Bass R, Brownell JN, Stallings VA. The Impact of Highly Effective CFTR Modulators on Growth and Nutrition Status. Nutrients 2021;13:2907. [PMID: 34578785 DOI: 10.3390/nu13092907] [Cited by in Crossref: 1] [Cited by in F6Publishing: 1] [Article Influence: 1.0] [Reference Citation Analysis]
30 Egan ME. Cystic fibrosis transmembrane conductance receptor modulator therapy in cystic fibrosis, an update. Curr Opin Pediatr 2020;32:384-8. [PMID: 32374578 DOI: 10.1097/MOP.0000000000000892] [Cited by in Crossref: 8] [Cited by in F6Publishing: 4] [Article Influence: 4.0] [Reference Citation Analysis]
31 Miller MJ, Foroozan R. Papilledema and hypervitaminosis A after elexacaftor/tezacaftor/ivacaftor for cystic fibrosis. Can J Ophthalmol 2021:S0008-4182(21)00162-9. [PMID: 34058144 DOI: 10.1016/j.jcjo.2021.04.018] [Reference Citation Analysis]
32 Lopes-Pacheco M. CFTR Modulators: The Changing Face of Cystic Fibrosis in the Era of Precision Medicine. Front Pharmacol 2019;10:1662. [PMID: 32153386 DOI: 10.3389/fphar.2019.01662] [Cited by in Crossref: 63] [Cited by in F6Publishing: 56] [Article Influence: 31.5] [Reference Citation Analysis]
33 Bratcher PE, Yadav S, Shaughnessy CA, Thornell IM, Zeitlin PL. Effect of apical chloride concentration on the measurement of responses to CFTR modulation in airway epithelia cultured from nasal brushings. Physiol Rep 2020;8:e14603. [PMID: 33038073 DOI: 10.14814/phy2.14603] [Cited by in Crossref: 2] [Cited by in F6Publishing: 3] [Article Influence: 2.0] [Reference Citation Analysis]
34 Aoyama BC, Mogayzel PJ. Ivacaftor for the treatment of cystic fibrosis in children under six years of age. Expert Rev Respir Med 2020;14:547-57. [PMID: 32154747 DOI: 10.1080/17476348.2020.1741352] [Cited by in Crossref: 2] [Article Influence: 1.0] [Reference Citation Analysis]
35 Karb DB, Cummings LC. The Intestinal Microbiome and Cystic Fibrosis Transmembrane Conductance Regulator Modulators: Emerging Themes in the Management of Gastrointestinal Manifestations of Cystic Fibrosis. Curr Gastroenterol Rep 2021;23:17. [PMID: 34448955 DOI: 10.1007/s11894-021-00817-2] [Reference Citation Analysis]
36 Wright BA, Ketchen NK, Rasmussen LN, Bartels AR, Singh SB. Impact of elexacaftor/tezacaftor/ivacaftor on vitamin D absorption in cystic fibrosis patients. Pediatr Pulmonol 2021. [PMID: 34859619 DOI: 10.1002/ppul.25781] [Reference Citation Analysis]
37 Jeyaratnam J, van der Meer R, Berkers G, Heijerman HG, Beekman JM, van der Ent CK. Breast development in a 7 year old girl with CF treated with ivacaftor: An indication for personalized dosing? J Cyst Fibros 2021:S1569-1993(21)01285-6. [PMID: 34175243 DOI: 10.1016/j.jcf.2021.06.001] [Reference Citation Analysis]
38 Bush A, Pavord ID. Challenging the paradigm: moving from umbrella labels to treatable traits in airway disease. Breathe (Sheff) 2021;17:210053. [PMID: 35035544 DOI: 10.1183/20734735.0053-2021] [Cited by in Crossref: 1] [Article Influence: 1.0] [Reference Citation Analysis]
39 Nissenbaum C, Davies G, Horsley A, Davies JC. Monitoring early stage lung disease in cystic fibrosis. Current Opinion in Pulmonary Medicine 2020;26:671-8. [DOI: 10.1097/mcp.0000000000000732] [Cited by in Crossref: 3] [Article Influence: 1.5] [Reference Citation Analysis]
40 McColley SA. Predicting the course of nutrition and lung disease in infants and children with cystic fibrosis. J Cyst Fibros 2020;19:847-9. [PMID: 32674985 DOI: 10.1016/j.jcf.2020.07.006] [Reference Citation Analysis]
41 Caudri D, Turkovic L, de Klerk NH, Rosenow T, Murray CP, Steyerberg EW, Ranganathan SC, Sly P, Stick SM, Breuer O; AREST CF Study Group. A screening tool to identify risk for bronchiectasis progression in children with cystic fibrosis. Pediatr Pulmonol 2022;57:122-31. [PMID: 34596357 DOI: 10.1002/ppul.25712] [Reference Citation Analysis]
42 Sellers ZM. Pancreatic complications in children with cystic fibrosis. Curr Opin Pediatr 2020;32:661-7. [PMID: 32773577 DOI: 10.1097/MOP.0000000000000934] [Cited by in Crossref: 2] [Article Influence: 1.0] [Reference Citation Analysis]
43 Farrell PM, Rock MJ, Baker MW. The Impact of the CFTR Gene Discovery on Cystic Fibrosis Diagnosis, Counseling, and Preventive Therapy. Genes (Basel) 2020;11:E401. [PMID: 32276344 DOI: 10.3390/genes11040401] [Cited by in Crossref: 6] [Cited by in F6Publishing: 4] [Article Influence: 3.0] [Reference Citation Analysis]
44 Bailey J, Rozga M, McDonald CM, Bowser EK, Farnham K, Mangus M, Padula L, Porco K, Alvarez JA. Effect of CFTR Modulators on Anthropometric Parameters in Individuals with Cystic Fibrosis: An Evidence Analysis Center Systematic Review. J Acad Nutr Diet 2021;121:1364-1378.e2. [PMID: 32532673 DOI: 10.1016/j.jand.2020.03.014] [Cited by in Crossref: 5] [Cited by in F6Publishing: 3] [Article Influence: 2.5] [Reference Citation Analysis]
45 Dumas MP, Xia S, Bear CE, Ratjen F. Perspectives on the translation of in-vitro studies to precision medicine in Cystic Fibrosis. EBioMedicine 2021;73:103660. [PMID: 34740114 DOI: 10.1016/j.ebiom.2021.103660] [Reference Citation Analysis]
46 Haq I, Almulhem M, Soars S, Poulton D, Brodlie M. Precision Medicine Based on CFTR Genotype for People with Cystic Fibrosis. PGPM 2022;Volume 15:91-104. [DOI: 10.2147/pgpm.s245603] [Reference Citation Analysis]
47 Hutchinson I, McNally P. Appearance of Pancreatic Sufficiency and Discontinuation of Pancreatic Enzyme Replacement Therapy in Children with Cystic Fibrosis on Ivacaftor. Ann Am Thorac Soc 2021;18:182-3. [PMID: 32931706 DOI: 10.1513/AnnalsATS.202006-614RL] [Cited by in Crossref: 4] [Cited by in F6Publishing: 2] [Article Influence: 4.0] [Reference Citation Analysis]
48 Barry PJ, Taylor-Cousar JL. Triple combination cystic fibrosis transmembrane conductance regulator modulator therapy in the real world - opportunities and challenges. Curr Opin Pulm Med 2021;27:554-66. [PMID: 34420018 DOI: 10.1097/MCP.0000000000000819] [Reference Citation Analysis]
49 Coverstone AM, Ferkol TW. Early Diagnosis and Intervention in Cystic Fibrosis: Imagining the Unimaginable. Front Pediatr 2020;8:608821. [PMID: 33505947 DOI: 10.3389/fped.2020.608821] [Cited by in Crossref: 1] [Article Influence: 1.0] [Reference Citation Analysis]