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Items: 1 to 50 of 155

1.

Genetic diagnosis in practice: From cystic fibrosis to CFTR-related disorders.

Pagin A, Sermet-Gaudelus I, Burgel PR.

Arch Pediatr. 2020 Feb;27 Suppl 1:eS25-eS29. doi: 10.1016/S0929-693X(20)30047-6.

PMID:
32172933
2.

[Focus on cystic fibrosis].

Sermet-Gaudelus I, Pierre-Joseph MC, Lustre A, Voltine A, Roze H.

Rev Infirm. 2020 Jan;69(257):16-18. doi: 10.1016/j.revinf.2019.12.007. Epub 2019 Dec 26. French.

PMID:
32146956
3.

Efficacy and safety of ataluren in patients with nonsense-mutation cystic fibrosis not receiving chronic inhaled aminoglycosides: The international, randomized, double-blind, placebo-controlled Ataluren Confirmatory Trial in Cystic Fibrosis (ACT CF).

Konstan MW, VanDevanter DR, Rowe SM, Wilschanski M, Kerem E, Sermet-Gaudelus I, DiMango E, Melotti P, McIntosh J, De Boeck K; ACT CF Study Group.

J Cyst Fibros. 2020 Jan 23. pii: S1569-1993(20)30030-8. doi: 10.1016/j.jcf.2020.01.007. [Epub ahead of print]

PMID:
31983658
4.

Characterization of two rat models of cystic fibrosis-KO and F508del CFTR-Generated by Crispr-Cas9.

Dreano E, Bacchetta M, Simonin J, Galmiche L, Usal C, Slimani L, Sadoine J, Tesson L, Anegon I, Concordet JP, Hatton A, Vignaud L, Tondelier D, Sermet-Gaudelus I, Chanson M, Cottart CH.

Animal Model Exp Med. 2019 Nov 25;2(4):297-311. doi: 10.1002/ame2.12091. eCollection 2019 Dec.

5.

Suppressing 'nonsense' in cystic fibrosis.

Hinzpeter A, Sermet-Gaudelus I, Sheppard DN.

J Physiol. 2020 Feb;598(3):429-430. doi: 10.1113/JP279267. Epub 2020 Jan 16. No abstract available.

PMID:
31869855
6.

Unsolved severe chronic rhinosinusitis elucidated by extensive CFTR genotyping.

Degrugillier F, Simon S, Aissat A, Remus N, Mekki C, Decrouy X, Hatton A, Hinzpeter A, Hoffmann B, Sermet-Gaudelus I, Callebaut I, Fanen P, Prulière-Escabasse V.

Clin Case Rep. 2019 Sep 27;7(11):2128-2134. doi: 10.1002/ccr3.2443. eCollection 2019 Nov.

7.

CFTR: New insights into structure and function and implications for modulation by small molecules.

Kleizen B, Hunt JF, Callebaut I, Hwang TC, Sermet-Gaudelus I, Hafkemeyer S, Sheppard DN.

J Cyst Fibros. 2020 Mar;19 Suppl 1:S19-S24. doi: 10.1016/j.jcf.2019.10.021. Epub 2019 Nov 21.

PMID:
31759907
8.

Author Correction: Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis.

Simonin J, Bille E, Crambert G, Noel S, Dreano E, Edwards A, Hatton A, Pranke I, Villeret B, Cottart CH, Vrel JP, Urbach V, Baatallah N, Hinzpeter A, Golec A, Touqui L, Nassif X, Galietta LJV, Planelles G, Sallenave JM, Edelman A, Sermet-Gaudelus I.

Sci Rep. 2019 Nov 21;9(1):17535. doi: 10.1038/s41598-019-54253-4.

9.

Insights into the variability of nasal potential difference, a biomarker of CFTR activity.

Kyrilli S, Henry T, Wilschanski M, Fajac I, Davies JC, Jais JP, Sermet-Gaudelus I.

J Cyst Fibros. 2019 Nov 4. pii: S1569-1993(19)30899-9. doi: 10.1016/j.jcf.2019.09.015. [Epub ahead of print]

PMID:
31699569
10.

Cystic fibrosis bone disease treatment: Current knowledge and future directions.

Putman MS, Anabtawi A, Le T, Tangpricha V, Sermet-Gaudelus I.

J Cyst Fibros. 2019 Oct;18 Suppl 2:S56-S65. doi: 10.1016/j.jcf.2019.08.017.

11.

Cystic Fibrosis Diagnosis in Newborns, Children, and Adults.

Castellani C, Linnane B, Pranke I, Cresta F, Sermet-Gaudelus I, Peckham D.

Semin Respir Crit Care Med. 2019 Dec;40(6):701-714. doi: 10.1055/s-0039-1697961. Epub 2019 Nov 3.

PMID:
31679154
12.

Real-Life Safety and Effectiveness of Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis.

Burgel PR, Munck A, Durieu I, Chiron R, Mely L, Prevotat A, Murris-Espin M, Porzio M, Abely M, Reix P, Marguet C, Macey J, Sermet-Gaudelus I, Corvol H, Bui S, Lemonnier L, Dehillotte C, Da Silva J, Paillasseur JL, Hubert D; French Cystic Fibrosis Reference Network Study Group.

Am J Respir Crit Care Med. 2020 Jan 15;201(2):188-197. doi: 10.1164/rccm.201906-1227OC.

PMID:
31601120
13.

Prevention of osteoporosis in cystic fibrosis.

Chedevergne F, Sermet-Gaudelus I.

Curr Opin Pulm Med. 2019 Nov;25(6):660-665. doi: 10.1097/MCP.0000000000000624.

PMID:
31567515
14.

A critical review of definitions used to describe Pseudomonas aeruginosa microbiological status in patients with cystic fibrosis for application in clinical trials.

Taccetti G, Denton M, Hayes K; ECFS-CTN Microbiology Group, Drevinek P, Sermet-Gaudelus I.

J Cyst Fibros. 2020 Jan;19(1):52-67. doi: 10.1016/j.jcf.2019.08.014. Epub 2019 Sep 13.

PMID:
31526710
15.

Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis.

Simonin J, Bille E, Crambert G, Noel S, Dreano E, Edwards A, Hatton A, Pranke I, Villeret B, Cottart CH, Vrel JP, Urbach V, Baatallah N, Hinzpeter A, Golec A, Touqui L, Nassif X, Galietta LJV, Planelles G, Sallenave JM, Edelman A, Sermet-Gaudelus I.

Sci Rep. 2019 Apr 24;9(1):6516. doi: 10.1038/s41598-019-42751-4. Erratum in: Sci Rep. 2019 Nov 21;9(1):17535.

16.

Mycobacterium bolletii Lung Disease in Cystic Fibrosis.

Rollet-Cohen V, Roux AL, Le Bourgeois M, Sapriel G, El Bahri M, Jais JP, Heym B, Mougari F, Raskine L, Véziris N, Gaillard JL, Sermet-Gaudelus I.

Chest. 2019 Aug;156(2):247-254. doi: 10.1016/j.chest.2019.03.019. Epub 2019 Mar 29.

PMID:
30935892
17.

Emerging Therapeutic Approaches for Cystic Fibrosis. From Gene Editing to Personalized Medicine.

Pranke I, Golec A, Hinzpeter A, Edelman A, Sermet-Gaudelus I.

Front Pharmacol. 2019 Feb 27;10:121. doi: 10.3389/fphar.2019.00121. eCollection 2019. Review.

18.

Chronic Staphylococcus aureus Lung Infection Correlates With Proteogenomic and Metabolic Adaptations Leading to an Increased Intracellular Persistence.

Tan X, Coureuil M, Ramond E, Euphrasie D, Dupuis M, Tros F, Meyer J, Nemazanyy I, Chhuon C, Guerrera IC, Ferroni A, Sermet-Gaudelus I, Nassif X, Charbit A, Jamet A.

Clin Infect Dis. 2019 Nov 13;69(11):1937-1945. doi: 10.1093/cid/ciz106.

PMID:
30753350
19.

Predictive factors for lumacaftor/ivacaftor clinical response.

Masson A, Schneider-Futschik EK, Baatallah N, Nguyen-Khoa T, Girodon E, Hatton A, Flament T, Le Bourgeois M, Chedevergne F, Bailly C, Kyrilli S, Achimastos D, Hinzpeter A, Edelman A, Sermet-Gaudelus I.

J Cyst Fibros. 2019 May;18(3):368-374. doi: 10.1016/j.jcf.2018.12.011. Epub 2018 Dec 28.

PMID:
30595473
20.

Antisense oligonucleotide eluforsen improves CFTR function in F508del cystic fibrosis.

Sermet-Gaudelus I, Clancy JP, Nichols DP, Nick JA, De Boeck K, Solomon GM, Mall MA, Bolognese J, Bouisset F, den Hollander W, Paquette-Lamontagne N, Tomkinson N, Henig N, Elborn JS, Rowe SM.

J Cyst Fibros. 2019 Jul;18(4):536-542. doi: 10.1016/j.jcf.2018.10.015. Epub 2018 Nov 19.

21.

Increased expression of ATP12A proton pump in cystic fibrosis airways.

Scudieri P, Musante I, Caci E, Venturini A, Morelli P, Walter C, Tosi D, Palleschi A, Martin-Vasallo P, Sermet-Gaudelus I, Planelles G, Crambert G, Galietta LJ.

JCI Insight. 2018 Oct 18;3(20). pii: 123616. doi: 10.1172/jci.insight.123616.

22.

Might Brushed Nasal Cells Be a Surrogate for CFTR Modulator Clinical Response?

Pranke I, Hatton A, Masson A, Flament T, Le Bourgeois M, Chedevergne F, Bailly C, Urbach V, Hinzpeter A, Edelman A, Sermet-Gaudelus I.

Am J Respir Crit Care Med. 2019 Jan 1;199(1):123-126. doi: 10.1164/rccm.201808-1436LE. No abstract available.

PMID:
30326728
23.

Structure-guided combination therapy to potently improve the function of mutant CFTRs.

Veit G, Xu H, Dreano E, Avramescu RG, Bagdany M, Beitel LK, Roldan A, Hancock MA, Lay C, Li W, Morin K, Gao S, Mak PA, Ainscow E, Orth AP, McNamara P, Edelman A, Frenkiel S, Matouk E, Sermet-Gaudelus I, Barnes WG, Lukacs GL.

Nat Med. 2018 Nov;24(11):1732-1742. doi: 10.1038/s41591-018-0200-x. Epub 2018 Oct 8.

24.

Standardized Measurement of Nasal Membrane Transepithelial Potential Difference (NPD).

Solomon GM, Bronsveld I, Hayes K, Wilschanski M, Melotti P, Rowe SM, Sermet-Gaudelus I.

J Vis Exp. 2018 Sep 13;(139). doi: 10.3791/57006.

25.

Correction to: In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study.

Sermet-Gaudelus I, Renouil M, Fajac A, Bidou L, Parbaille B, Pierrot S, Davy N, Bismuth E, Reinert P, Lenoir G, Lesure JF, Rousset JP, Edelman A.

BMC Med. 2018 Aug 25;16(1):159. doi: 10.1186/s12916-018-1138-z.

26.

N1303K: Leaving no stone unturned in the search for transformational therapeutics.

Noel S, Sermet-Gaudelus I, Sheppard DN.

J Cyst Fibros. 2018 Sep;17(5):555-557. doi: 10.1016/j.jcf.2018.07.009. Epub 2018 Aug 17. No abstract available.

PMID:
30126793
27.

Comparative proteomics of respiratory exosomes in cystic fibrosis, primary ciliary dyskinesia and asthma.

Rollet-Cohen V, Bourderioux M, Lipecka J, Chhuon C, Jung VA, Mesbahi M, Nguyen-Khoa T, Guérin-Pfyffer S, Schmitt A, Edelman A, Sermet-Gaudelus I, Guerrera IC.

J Proteomics. 2018 Aug 15;185:1-7. doi: 10.1016/j.jprot.2018.07.001. Epub 2018 Jul 3.

PMID:
30032860
28.

ECFS best practice guidelines: the 2018 revision.

Castellani C, Duff AJA, Bell SC, Heijerman HGM, Munck A, Ratjen F, Sermet-Gaudelus I, Southern KW, Barben J, Flume PA, Hodková P, Kashirskaya N, Kirszenbaum MN, Madge S, Oxley H, Plant B, Schwarzenberg SJ, Smyth AR, Taccetti G, Wagner TOF, Wolfe SP, Drevinek P.

J Cyst Fibros. 2018 Mar;17(2):153-178. doi: 10.1016/j.jcf.2018.02.006. Epub 2018 Mar 3. Review.

29.

Factors influencing readthrough therapy for frequent cystic fibrosis premature termination codons.

Pranke I, Bidou L, Martin N, Blanchet S, Hatton A, Karri S, Cornu D, Costes B, Chevalier B, Tondelier D, Girodon E, Coupet M, Edelman A, Fanen P, Namy O, Sermet-Gaudelus I, Hinzpeter A.

ERJ Open Res. 2018 Feb 23;4(1). pii: 00080-2017. doi: 10.1183/23120541.00080-2017. eCollection 2018 Jan. Erratum in: ERJ Open Res. 2018 Jul 13;4(3):.

30.

Cis variants identified in F508del complex alleles modulate CFTR channel rescue by small molecules.

Baatallah N, Bitam S, Martin N, Servel N, Costes B, Mekki C, Chevalier B, Pranke I, Simonin J, Girodon E, Hoffmann B, Mornon JP, Callebaut I, Sermet-Gaudelus I, Fanen P, Edelman A, Hinzpeter A.

Hum Mutat. 2018 Apr;39(4):506-514. doi: 10.1002/humu.23389. Epub 2018 Jan 16.

PMID:
29271547
31.

Resolvin D1 regulates epithelial ion transport and inflammation in cystic fibrosis airways.

Ringholz FC, Higgins G, Hatton A, Sassi A, Moukachar A, Fustero-Torre C, Hollenhorst M, Sermet-Gaudelus I, Harvey BJ, McNally P, Urbach V.

J Cyst Fibros. 2018 Sep;17(5):607-615. doi: 10.1016/j.jcf.2017.11.017. Epub 2017 Dec 8.

PMID:
29233471
32.

Guidelines for the clinical management and follow-up of infants with inconclusive cystic fibrosis diagnosis through newborn screening.

Sermet-Gaudelus I, Brouard J, Audrézet MP, Couderc Kohen L, Weiss L, Wizla N, Vrielynck S, LLerena K, Le Bourgeois M, Deneuville E, Remus N, Nguyen-Khoa T, Raynal C, Roussey M, Girodon E.

Arch Pediatr. 2017 Dec;24(12):e1-e14. doi: 10.1016/j.arcped.2017.07.015. Epub 2017 Nov 22.

PMID:
29174009
33.

Omalizumab treatment for allergic bronchopulmonary aspergillosis in young patients with cystic fibrosis.

Perisson C, Destruys L, Grenet D, Bassinet L, Derelle J, Sermet-Gaudelus I, Thumerelle C, Prevotat A, Rosner V, Clement A, Corvol H.

Respir Med. 2017 Dec;133:12-15. doi: 10.1016/j.rmed.2017.11.007. Epub 2017 Nov 10.

34.

The suppression of premature termination codons and the repair of splicing mutations in CFTR.

Oren YS, Pranke IM, Kerem B, Sermet-Gaudelus I.

Curr Opin Pharmacol. 2017 Jun;34:125-131. doi: 10.1016/j.coph.2017.09.017. Epub 2017 Nov 10. Review.

PMID:
29128743
35.

Multifocal fixed drug eruption to ceftazidime in a child with cystic fibrosis.

Ben Mansour A, Bellon N, Frassati-Biaggi A, Sermet-Gaudelus I, Ponvert C, de Blic J, Lezmi G.

Pediatr Allergy Immunol. 2018 Feb;29(1):115-117. doi: 10.1111/pai.12829. Epub 2017 Nov 28. No abstract available.

PMID:
29047164
36.

Pain is an underestimated symptom in cystic fibrosis.

Masson A, Kirszenbaum M, Sermet-Gaudelus I.

Curr Opin Pulm Med. 2017 Nov;23(6):570-573. doi: 10.1097/MCP.0000000000000427. Review.

PMID:
28806187
37.

Pseudomonas aeruginosa LasB protease impairs innate immunity in mice and humans by targeting a lung epithelial cystic fibrosis transmembrane regulator-IL-6-antimicrobial-repair pathway.

Saint-Criq V, Villeret B, Bastaert F, Kheir S, Hatton A, Cazes A, Xing Z, Sermet-Gaudelus I, Garcia-Verdugo I, Edelman A, Sallenave JM.

Thorax. 2018 Jan;73(1):49-61. doi: 10.1136/thoraxjnl-2017-210298. Epub 2017 Aug 8.

38.

Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators.

Pranke IM, Hatton A, Simonin J, Jais JP, Le Pimpec-Barthes F, Carsin A, Bonnette P, Fayon M, Stremler-Le Bel N, Grenet D, Thumerel M, Mazenq J, Urbach V, Mesbahi M, Girodon-Boulandet E, Hinzpeter A, Edelman A, Sermet-Gaudelus I.

Sci Rep. 2017 Aug 7;7(1):7375. doi: 10.1038/s41598-017-07504-1.

39.

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.

40.

[Management of infants whose diagnosis is inconclusive at neonatal screening for cystic fibrosis].

Sermet-Gaudelus I, Brouard J, Audrézet MP, Couderc Kohen L, Weiss L, Wizla N, Vrielynck S, LLerena K, Le Bourgeois M, Deneuville E, Remus N, Nguyen-Khoa T, Raynal C, Roussey M, Girodon E.

Arch Pediatr. 2017 Apr;24(4):401-414. doi: 10.1016/j.arcped.2017.01.019. Epub 2017 Mar 1. French.

PMID:
28258861
41.

[New therapeutic developments in cystic fibrosis].

Bui S, Macey J, Fayon M; « nouvelles thérapeutiques de la Société Française pour la Mucoviscidose », Bihouée T, Burgel PR, Colomb V, Corvol H, Durieu I, Hubert D, Marguet C, Mas E, Munck A, Murris-Espin M, Reix P, Sermet-Gaudelus I.

Arch Pediatr. 2016 Dec;23(12S):12S47-12S53. doi: 10.1016/S0929-693X(17)30062-3. Review. French.

PMID:
28231894
42.

Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation.

Farrell PM, White TB, Ren CL, Hempstead SE, Accurso F, Derichs N, Howenstine M, McColley SA, Rock M, Rosenfeld M, Sermet-Gaudelus I, Southern KW, Marshall BC, Sosnay PR.

J Pediatr. 2017 Feb;181S:S4-S15.e1. doi: 10.1016/j.jpeds.2016.09.064. Erratum in: J Pediatr. 2017 May;184:243.

43.

Diagnosis of Cystic Fibrosis in Screened Populations.

Farrell PM, White TB, Howenstine MS, Munck A, Parad RB, Rosenfeld M, Sommerburg O, Accurso FJ, Davies JC, Rock MJ, Sanders DB, Wilschanski M, Sermet-Gaudelus I, Blau H, Gartner S, McColley SA.

J Pediatr. 2017 Feb;181S:S33-S44.e2. doi: 10.1016/j.jpeds.2016.09.065.

44.

French law: what about a reasoned reimbursement of serum vitamin D assays?

Souberbielle JC, Benhamou CL, Cortet B, Rousière M, Roux C, Abitbol V, Annweiler C, Audran M, Bacchetta J, Bataille P, Beauchet O, Bardet R, Benachi A, Berenbaum F, Blain H, Borson-Chazot F, Breuil V, Briot K, Brunet P, Carel JC, Caron P, Chabre O, Chanson P, Chapurlat R, Cochat P, Coutant R, Christin-Maitre S, Cohen-Solal M, Combe C, Cormier C, Courbebaisse M, Debrus G, Delemer B, Deschenes G, Duquenne M, Duval G, Fardellone P, Fouque D, Friedlander G, Gauvain JB, Groussin L, Guggenbuhl P, Houillier P, Hannedouche T, Jacot W, Javier RM, Jean G, Jeandel C, Joly D, Kamenicky P, Knebelmann B, Lafage-Proust MH, LeBouc Y, Legrand E, Levy-Weil F, Linglart A, Machet L, Maheu E, Mallet E, Marcelli C, Marès P, Mariat C, Maruani G, Maugars Y, Montagnon F, Moulin B, Orcel P, Partouche H, Personne V, Pierrot-Deseilligny C, Polak M, Pouteil-Noble C, Prié D, Raynaud-Simon A, Rolland Y, Sadoul JL, Salle B, Sault C, Schott AM, Sermet-Gaudelus I, Soubrier M, Tack I, Thervet E, Tostivint I, Touraine P, Tremollières F, Urena-Torres P, Viard JP, Wemeau JL, Weryha G, Winer N, Young J, Thomas T.

Geriatr Psychol Neuropsychiatr Vieil. 2016 Dec 1;14(4):377-382. Review.

PMID:
27818369
45.

New Pharmacological Approaches to Treat Patients with Cystic Fibrosis with Nonsense Mutations.

Sermet-Gaudelus I, Namy O.

Am J Respir Crit Care Med. 2016 Nov 1;194(9):1042-1044. No abstract available.

PMID:
27797609
46.

Analysis of nasal potential in murine cystic fibrosis models.

da Cunha MF, Simonin J, Sassi A, Freund R, Hatton A, Cottart CH, Elganfoud N, Zoubairi R, Dragu C, Jais JP, Hinzpeter A, Edelman A, Sermet-Gaudelus I.

Int J Biochem Cell Biol. 2016 Nov;80:87-97. doi: 10.1016/j.biocel.2016.10.001. Epub 2016 Oct 4. Review.

PMID:
27717840
47.

Changes of CFTR functional measurements and clinical improvements in cystic fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor.

Mesbahi M, Shteinberg M, Wilschanski M, Hatton A, Nguyen-Khoa T, Friedman H, Cohen M, Escabasse V, Le Bourgeois M, Lucidi V, Sermet-Gaudelus I, Bassinet L, Livnat G.

J Cyst Fibros. 2017 Jan;16(1):45-48. doi: 10.1016/j.jcf.2016.08.006. Epub 2016 Sep 20.

48.

Bone demineralization is improved by ivacaftor in patients with cystic fibrosis carrying the p.Gly551Asp mutation.

Sermet-Gaudelus I, Delion M, Durieu I, Jacquot J, Hubert D.

J Cyst Fibros. 2016 Nov;15(6):e67-e69. doi: 10.1016/j.jcf.2016.09.003. Epub 2016 Oct 10.

49.

Infections caused by Mycobacterium abscessus: epidemiology, diagnostic tools and treatment.

Mougari F, Guglielmetti L, Raskine L, Sermet-Gaudelus I, Veziris N, Cambau E.

Expert Rev Anti Infect Ther. 2016 Dec;14(12):1139-1154. Epub 2016 Oct 3. Review.

PMID:
27690688
50.

Long-Term Rasamsonia argillacea Complex Species Colonization Revealed by PCR Amplification of Repetitive DNA Sequences in Cystic Fibrosis Patients.

Mouhajir A, Matray O, Giraud S, Mély L, Marguet C, Sermet-Gaudelus I, Le Gal S, Labbé F, Person C, Troussier F, Ballet JJ, Gargala G, Zouhair R, Bougnoux ME, Bouchara JP, Favennec L.

J Clin Microbiol. 2016 Nov;54(11):2804-2812. Epub 2016 Sep 7.

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