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Items: 1 to 50 of 82

1.

The future of cystic fibrosis care: a global perspective.

Bell SC, Mall MA, Gutierrez H, Macek M, Madge S, Davies JC, Burgel PR, 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.

Lancet Respir Med. 2019 Sep 27. pii: S2213-2600(19)30337-6. doi: 10.1016/S2213-2600(19)30337-6. [Epub ahead of print] Review.

PMID:
31570318
2.

Amikacin liposome inhalation suspension for chronic Pseudomonas aeruginosa infection in cystic fibrosis.

Bilton D, Pressler T, Fajac I, Clancy JP, Sands D, Minic P, Cipolli M, Galeva I, Solé A, Quittner AL, Liu K, McGinnis JP 2nd, Eagle G, Gupta R, Konstan MW; CLEAR-108 Study Group.

J Cyst Fibros. 2019 Aug 23. pii: S1569-1993(19)30833-1. doi: 10.1016/j.jcf.2019.08.001. [Epub ahead of print]

PMID:
31451351
3.

An automated computed tomography score for the cystic fibrosis lung.

Chassagnon G, Martin C, Burgel PR, Hubert D, Fajac I, Paragios N, Zacharaki EI, Legmann P, Coste J, Revel MP.

Eur Radiol. 2018 Dec;28(12):5111-5120. doi: 10.1007/s00330-018-5516-x. Epub 2018 Jun 4.

PMID:
29869171
4.

No easy road to better cystic fibrosis care in Eastern Europe?

De Boeck K, Fajac I.

J Cyst Fibros. 2018 Jul;17(4):423-424. doi: 10.1016/j.jcf.2018.05.007. Epub 2018 May 31. No abstract available.

PMID:
29859834
5.

Cystic fibrosis: Current aspects and perspectives.

Burgel PR, Fajac I.

Presse Med. 2017 Jun;46(6 Pt 2):e85-e86. doi: 10.1016/j.lpm.2017.06.001. French. No abstract available.

PMID:
28641709
6.

New treatments targeting the basic defects in cystic fibrosis.

Fajac I, Wainwright CE.

Presse Med. 2017 Jun;46(6 Pt 2):e165-e175. doi: 10.1016/j.lpm.2017.01.024. Epub 2017 May 26. Review.

PMID:
28554723
7.

A multiple reader scoring system for Nasal Potential Difference parameters.

Solomon GM, Liu B, Sermet-Gaudelus I, Fajac I, Wilschanski M, Vermeulen F, Rowe SM.

J Cyst Fibros. 2017 Sep;16(5):573-578. doi: 10.1016/j.jcf.2017.04.011. Epub 2017 Apr 29.

8.

Bacteria-driven peribronchial lymphoid neogenesis in bronchiectasis and cystic fibrosis.

Frija-Masson J, Martin C, Regard L, Lothe MN, Touqui L, Durand A, Lucas B, Damotte D, Alifano M, Fajac I, Burgel PR.

Eur Respir J. 2017 Apr 26;49(4). pii: 1601873. doi: 10.1183/13993003.01873-2016. Print 2017 Apr.

9.

New horizons for cystic fibrosis treatment.

Fajac I, De Boeck K.

Pharmacol Ther. 2017 Feb;170:205-211. doi: 10.1016/j.pharmthera.2016.11.009. Epub 2016 Dec 1. Review.

PMID:
27916649
10.

Long-term computed tomographic changes in cystic fibrosis patients treated with ivacaftor.

Chassagnon G, Hubert D, Fajac I, Burgel PR, Revel MP; investigators.

Eur Respir J. 2016 Jul;48(1):249-52. doi: 10.1183/13993003.01918-2015. Epub 2016 May 26. No abstract available.

11.

Multicentre chest computed tomography standardisation in children and adolescents with cystic fibrosis: the way forward.

Kuo W, Kemner-van de Corput MP, Perez-Rovira A, de Bruijne M, Fajac I, Tiddens HA, van Straten M; ECFS-CTN/SCIFI CF study group.

Eur Respir J. 2016 Jun;47(6):1706-17. doi: 10.1183/13993003.01601-2015. Epub 2016 Apr 13.

12.

Disease-specific clinical trials networks: the example of cystic fibrosis.

De Boeck K, Bulteel V, Fajac I.

Eur J Pediatr. 2016 Jun;175(6):817-24. doi: 10.1007/s00431-016-2712-z. Epub 2016 Mar 15.

13.

[Demographic growth and targeted therapies: The changing face of cystic fibrosis].

Fajac I, Burgel PR.

Rev Mal Respir. 2016 Oct;33(8):645-647. doi: 10.1016/j.rmr.2015.12.001. Epub 2016 Jan 6. French. No abstract available.

PMID:
26777401
14.

[FUTURE THERAPIES FOR CYSTIC FIBROSIS].

Fajac I.

Rev Prat. 2015 Oct;65(8):1106-8. French.

PMID:
26749719
15.

Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial.

Kerem E, Konstan MW, De Boeck K, Accurso FJ, Sermet-Gaudelus I, Wilschanski M, Elborn JS, Melotti P, Bronsveld I, Fajac I, Malfroot A, Rosenbluth DB, Walker PA, McColley SA, Knoop C, Quattrucci S, Rietschel E, Zeitlin PL, Barth J, Elfring GL, Welch EM, Branstrom A, Spiegel RJ, Peltz SW, Ajayi T, Rowe SM; Cystic Fibrosis Ataluren Study Group.

Lancet Respir Med. 2014 Jul;2(7):539-47. doi: 10.1016/S2213-2600(14)70100-6. Epub 2014 May 15.

16.

A new Clinical Trials Directive to boost academic trials?

De Boeck K, Tiddens H, Elborn S, Fajac I; European Cystic Fibrosis Society (ECFS) and the ECFS Clinical Trial Network.

Lancet Respir Med. 2013 Apr;1(2):99-101. doi: 10.1016/S2213-2600(13)70041-9. Epub 2013 Mar 21. No abstract available.

PMID:
24429080
17.

Prognostic value of six minute walk test in cystic fibrosis adults.

Martin C, Chapron J, Hubert D, Kanaan R, Honoré I, Paillasseur JL, Aubourg F, Dinh-Xuan AT, Dusser D, Fajac I, Burgel PR.

Respir Med. 2013 Dec;107(12):1881-7. doi: 10.1016/j.rmed.2013.10.001. Epub 2013 Oct 11.

18.

[Cystic fibrosis: new treatments targeting the CFTR protein].

Fajac I, Sermet-Gaudelus I.

Rev Mal Respir. 2013 Apr;30(4):255-61. doi: 10.1016/j.rmr.2012.10.631. Epub 2013 Jan 11. Review. French.

PMID:
23664284
19.

CFTR dysfunction induces vascular endothelial growth factor synthesis in airway epithelium.

Martin C, Coolen N, Wu Y, Thévenot G, Touqui L, Prulière-Escabasse V, Papon JF, Coste A, Escudier E, Dusser DJ, Fajac I, Burgel PR.

Eur Respir J. 2013 Dec;42(6):1553-62. doi: 10.1183/09031936.00164212. Epub 2013 Mar 21.

20.

CFTR biomarkers: time for promotion to surrogate end-point.

De Boeck K, Kent L, Davies J, Derichs N, Amaral M, Rowe SM, Middleton P, de Jonge H, Bronsveld I, Wilschanski M, Melotti P, Danner-Boucher I, Boerner S, Fajac I, Southern K, de Nooijer RA, Bot A, de Rijke Y, de Wachter E, Leal T, Vermeulen F, Hug MJ, Rault G, Nguyen-Khoa T, Barreto C, Proesmans M, Sermet-Gaudelus I; European Cystic Fibrosis Society Clinical Trial Network Standardisation Committee.

Eur Respir J. 2013 Jan;41(1):203-16. doi: 10.1183/09031936.00057512. Epub 2012 Aug 9. Review.

21.

Should bronchoscopy be advocated to study airway remodelling and inflammation in adults with cystic fibrosis?

Burgel PR, Martin C, Fajac I, Dusser DJ.

Thorax. 2012 Feb;67(2):177; author reply 177. doi: 10.1136/thoraxjnl-2011-200824. Epub 2011 Oct 11. No abstract available.

PMID:
21989735
22.

Guideline on the design and conduct of cystic fibrosis clinical trials: the European Cystic Fibrosis Society-Clinical Trials Network (ECFS-CTN).

De Boeck K, Bulteel V, Tiddens H, Wagner T, Fajac I, Conway S, Dufour F, Smyth AR, Lee T, Sermet I, Kassai B, Elborn S; ECFS-CTN network partners.

J Cyst Fibros. 2011 Jun;10 Suppl 2:S67-74. doi: 10.1016/S1569-1993(11)60010-6.

23.

New clinical diagnostic procedures for cystic fibrosis in Europe.

De Boeck K, Derichs N, Fajac I, de Jonge HR, Bronsveld I, Sermet I, Vermeulen F, Sheppard DN, Cuppens H, Hug M, Melotti P, Middleton PG, Wilschanski M; ECFS Diagnostic Network Working Group; EuroCareCF WP3 Group on CF diagnosis.

J Cyst Fibros. 2011 Jun;10 Suppl 2:S53-66. doi: 10.1016/S1569-1993(11)60009-X. Review.

24.

Pseudomonas aeruginosa induces vascular endothelial growth factor synthesis in airway epithelium in vitro and in vivo.

Martin C, Thévenot G, Danel S, Chapron J, Tazi A, Macey J, Dusser DJ, Fajac I, Burgel PR.

Eur Respir J. 2011 Oct;38(4):939-46. doi: 10.1183/09031936.00134910. Epub 2011 Mar 24.

25.

Abnormal electrochemical skin conductance in cystic fibrosis.

Hubert D, Brunswick P, Calvet JH, Dusser D, Fajac I.

J Cyst Fibros. 2011 Jan;10(1):15-20. doi: 10.1016/j.jcf.2010.09.002.

26.

Clinical phenotype and genotype of children with borderline sweat test and abnormal nasal epithelial chloride transport.

Sermet-Gaudelus I, Girodon E, Sands D, Stremmler N, Vavrova V, Deneuville E, Reix P, Bui S, Huet F, Lebourgeois M, Munck A, Iron A, Skalicka V, Bienvenu T, Roussel D, Lenoir G, Bellon G, Sarles J, Macek M, Roussey M, Fajac I, Edelman A.

Am J Respir Crit Care Med. 2010 Oct 1;182(7):929-36. doi: 10.1164/rccm.201003-0382OC. Epub 2010 Jun 10.

PMID:
20538955
27.

Measurement of nasal potential difference in young children with an equivocal sweat test following newborn screening for cystic fibrosis.

Sermet-Gaudelus I, Girodon E, Roussel D, Deneuville E, Bui S, Huet F, Guillot M, Aboutaam R, Renouil M, Munck A, des Georges M, Iron A, Thauvin-Robinet C, Fajac I, Lenoir G, Roussey M, Edelman A.

Thorax. 2010 Jun;65(6):539-44. doi: 10.1136/thx.2009.123422.

PMID:
20522854
28.

Cystic fibrosis transmembrane conductance regulator channel dysfunction in non-cystic fibrosis bronchiectasis.

Bienvenu T, Sermet-Gaudelus I, Burgel PR, Hubert D, Crestani B, Bassinet L, Dusser D, Fajac I.

Am J Respir Crit Care Med. 2010 May 15;181(10):1078-84. doi: 10.1164/rccm.200909-1434OC. Epub 2010 Feb 18.

PMID:
20167849
29.

Non-classic cystic fibrosis associated with D1152H CFTR mutation.

Burgel PR, Fajac I, Hubert D, Grenet D, Stremler N, Roussey M, Siret D, Languepin J, Mely L, Fanton A, Labbé A, Domblides P, Vic P, Dagorne M, Reynaud-Gaubert M, Counil F, Varaigne F, Bienvenu T, Bellis G, Dusser D.

Clin Genet. 2010 Apr;77(4):355-64. doi: 10.1111/j.1399-0004.2009.01294.x. Epub 2009 Oct 15.

PMID:
19843100
30.

Combination of ENaC and CFTR mutations may predispose to cystic fibrosis-like disease.

Fajac I, Viel M, Gaitch N, Hubert D, Bienvenu T.

Eur Respir J. 2009 Sep;34(3):772-3. doi: 10.1183/09031936.00057309. No abstract available.

31.

[The diagnosis of cystic fibrosis in adults: lessons from a family story].

Coman T, Fajac I, Bienvenu T, Desmazes-Dufeu N, Hubert D, Kanaan R, Dusser D, Burgel PR.

Rev Mal Respir. 2009 Jan;26(1):67-73. French.

PMID:
19212293
32.

In vivo gene delivery in the mouse lung with lactosylated polyethylenimine, questioning the relevance of in vitro experiments.

Grosse S, Thévenot G, Aron Y, Duverger E, Abdelkarim M, Roche AC, Monsigny M, Fajac I.

J Control Release. 2008 Dec 8;132(2):105-12. doi: 10.1016/j.jconrel.2008.08.018. Epub 2008 Sep 4.

PMID:
18801395
33.

[Potential nasal transepithelial difference].

Fajac I, Sermet I.

Rev Pneumol Clin. 2008 Feb;64(1):34-7. Review. French. No abstract available.

PMID:
18613347
34.

Could a defective epithelial sodium channel lead to bronchiectasis.

Fajac I, Viel M, Sublemontier S, Hubert D, Bienvenu T.

Respir Res. 2008 May 28;9:46. doi: 10.1186/1465-9921-9-46.

35.

Airway ion transport impacts on disease presentation and severity in cystic fibrosis.

Leal T, Fajac I, Wallace HL, Lebecque P, Lebacq J, Hubert D, Dall'Ava J, Dusser D, Ganesan AP, Knoop C, Cumps J, Wallemacq P, Southern KW.

Clin Biochem. 2008 Jul;41(10-11):764-72. doi: 10.1016/j.clinbiochem.2008.03.013. Epub 2008 Apr 8.

PMID:
18424267
36.

[Respiratory function tests for older children and adults with cystic fibrosis].

Fajac I, Counil F, Reynaud-Gaubert M.

Rev Pneumol Clin. 2007 Dec;63(6):367-72. Review. French. No abstract available.

PMID:
18166942
37.

Cytoskeletal involvement in the cellular trafficking of plasmid/PEI derivative complexes.

Grosse S, Aron Y, Thévenot G, Monsigny M, Fajac I.

J Control Release. 2007 Sep 11;122(1):111-7. Epub 2007 Jun 23.

PMID:
17658650
38.

[Cystic fibrosis: how to use pulmonary function tests].

Counil FP, Karila C, Le Bourgeois M, Matecki S, Lebras MN, Couderc L, Fajac I, Reynaud-Gaubert M, Bellet M, Gauthier R, Denjean A; Groupe de travail Explorations Fonctionnelles Respiratoires de la Société Française de la Mucoviscidose.

Rev Mal Respir. 2007 Jun;24(6):691-701. Review. French.

PMID:
17632430
39.

Association of pancreas divisum and recurrent acute pancreatitis with the IVS8-5T-12TG allele of the CFTR gene and CFTR dysfunction.

Dray X, Fajac I, Bienvenu T, Chryssostalis A, Sogni P, Hubert D.

Pancreas. 2007 Jul;35(1):90-3.

PMID:
17575549
40.

ENaCbeta and gamma genes as modifier genes in cystic fibrosis.

Viel M, Leroy C, Hubert D, Fajac I, Bienvenu T.

J Cyst Fibros. 2008 Jan;7(1):23-9. Epub 2007 Jun 7.

41.

Severity of scleroderma lung disease is related to alveolar concentration of nitric oxide.

Tiev KP, Cabane J, Aubourg F, Kettaneh A, Ziani M, Mouthon L, Duong-Quy S, Fajac I, Guillevin L, Dinh-Xuan AT.

Eur Respir J. 2007 Jul;30(1):26-30. Epub 2007 Apr 11.

42.

Which mechanism for nuclear import of plasmid DNA complexed with polyethylenimine derivatives?

Grosse S, Thévenot G, Monsigny M, Fajac I.

J Gene Med. 2006 Jul;8(7):845-51.

PMID:
16685744
43.

Reduced exhaled NO is related to impaired nasal potential difference in patients with cystic fibrosis.

Texereau J, Fajac I, Hubert D, Coste J, Dusser DJ, Bienvenu T, Dall'Ava-Santucci J, Dinh-Xuan AT.

Vascul Pharmacol. 2005 Dec;43(6):385-9. Epub 2005 Sep 22.

PMID:
16182611
44.

Transcription of plasmid DNA: influence of plasmid DNA/polyethylenimine complex formation.

Honoré I, Grosse S, Frison N, Favatier F, Monsigny M, Fajac I.

J Control Release. 2005 Oct 20;107(3):537-46.

PMID:
16087268
45.

Potocytosis and cellular exit of complexes as cellular pathways for gene delivery by polycations.

Grosse S, Aron Y, Thévenot G, François D, Monsigny M, Fajac I.

J Gene Med. 2005 Oct;7(10):1275-86.

PMID:
15906406
46.

Nasal airway ion transport is linked to the cystic fibrosis phenotype in adult patients.

Fajac I, Hubert D, Guillemot D, Honoré I, Bienvenu T, Volter F, Dall'Ava-Santucci J, Dusser DJ.

Thorax. 2004 Nov;59(11):971-6.

47.

Diagnosis of cystic fibrosis in adults with diffuse bronchiectasis.

Hubert D, Fajac I, Bienvenu T, Desmazes-Dufeu N, Ellaffi M, Dall'Ava-Santucci J, Dusser D.

J Cyst Fibros. 2004 Aug;3(3):203. No abstract available.

48.

Diagnosis of cystic fibrosis in adults with diffuse bronchiectasis.

Hubert D, Fajac I, Bienvenu T, Desmazes-Dufeu N, Ellaffi M, Dall'ava-Santucci J, Dusser D.

J Cyst Fibros. 2004 Mar;3(1):15-22.

49.

Glyco-dependent nuclear import of glycoproteins, glycoplexes and glycosylated plasmids.

Monsigny M, Rondanino C, Duverger E, Fajac I, Roche AC.

Biochim Biophys Acta. 2004 Jul 6;1673(1-2):94-103. Review.

PMID:
15238252
50.

Recombinant Escherichia coli as a gene delivery vector into airway epithelial cells.

Fajac I, Grosse S, Collombet JM, Thevenot G, Goussard S, Danel C, Grillot-Courvalin C.

J Control Release. 2004 Jun 18;97(2):371-81.

PMID:
15196763

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