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Modelling the association between fibrinogen concentration on admission and mortality in patients with massive transfusion after severe trauma: an analysis of a large regional database.

Bouzat P, Ageron FX, Charbit J, Bobbia X, Deras P, Nugues JBD, Escudier E, Marcotte G, Leone M, David JS.

Scand J Trauma Resusc Emerg Med. 2018 Jul 9;26(1):55. doi: 10.1186/s13049-018-0523-0.


Gas exchanges in children with cystic fibrosis or primary ciliary dyskinesia: A retrospective study.

Fuger M, Aupiais C, Thouvenin G, Taytard J, Tamalet A, Escudier E, Boizeau P, Corvol H, Beydon N.

Respir Physiol Neurobiol. 2018 May;251:1-7. doi: 10.1016/j.resp.2018.01.010. Epub 2018 Jan 31.


Homozygous missense mutation L673P in adenylate kinase 7 (AK7) leads to primary male infertility and multiple morphological anomalies of the flagella but not to primary ciliary dyskinesia.

Lorès P, Coutton C, El Khouri E, Stouvenel L, Givelet M, Thomas L, Rode B, Schmitt A, Louis B, Sakheli Z, Chaudhry M, Fernandez-Gonzales A, Mitsialis A, Dacheux D, Wolf JP, Papon JF, Gacon G, Escudier E, Arnoult C, Bonhivers M, Savinov SN, Amselem S, Ray PF, Dulioust E, Touré A.

Hum Mol Genet. 2018 Apr 1;27(7):1196-1211. doi: 10.1093/hmg/ddy034.


Infertility in an adult cohort with primary ciliary dyskinesia: phenotype-gene association.

Vanaken GJ, Bassinet L, Boon M, Mani R, Honoré I, Papon JF, Cuppens H, Jaspers M, Lorent N, Coste A, Escudier E, Amselem S, Maitre B, Legendre M, Christin-Maitre S.

Eur Respir J. 2017 Nov 9;50(5). pii: 1700314. doi: 10.1183/13993003.00314-2017. Print 2017 Nov. No abstract available. Erratum in: Eur Respir J. 2017 Dec 14;50(6):.


Functional characterization of tektin-1 in motile cilia and evidence for TEKT1 as a new candidate gene for motile ciliopathies.

Ryan R, Failler M, Reilly ML, Garfa-Traore M, Delous M, Filhol E, Reboul T, Bole-Feysot C, Nitschké P, Baudouin V, Amselem S, Escudier E, Legendre M, Benmerah A, Saunier S.

Hum Mol Genet. 2018 Jan 15;27(2):266-282. doi: 10.1093/hmg/ddx396.


A new index for characterizing micro-bead motion in a flow induced by ciliary beating: Part I, experimental analysis.

Bottier M, Blanchon S, Pelle G, Bequignon E, Isabey D, Coste A, Escudier E, Grotberg JB, Papon JF, Filoche M, Louis B.

PLoS Comput Biol. 2017 Jul 14;13(7):e1005605. doi: 10.1371/journal.pcbi.1005605. eCollection 2017 Jul.


Epidural analgesia in critically ill patients with acute pancreatitis: the multicentre randomised controlled EPIPAN study protocol.

Bulyez S, Pereira B, Caumon E, Imhoff E, Roszyk L, Bernard L, Bühler L, Heidegger C, Jaber S, Lefrant JY, Chabanne R, Bertrand PM, Laterre PF, Guerci P, Danin PE, Escudier E, Sossou A, Morand D, Sapin V, Constantin JM, Jabaudon M; EPIPAN Study Group; AzuRea network.

BMJ Open. 2017 May 29;7(5):e015280. doi: 10.1136/bmjopen-2016-015280. Erratum in: BMJ Open. 2018 Feb 10;8(2):e015280corr1.


The authors reply.

Tanaka S, Escudier E, Hamada S, Harrois A, Leblanc PE, Vicaut E, Duranteau J.

Crit Care Med. 2017 Apr;45(4):e459-e460. doi: 10.1097/CCM.0000000000002278. No abstract available.


X-linked primary ciliary dyskinesia due to mutations in the cytoplasmic axonemal dynein assembly factor PIH1D3.

Olcese C, Patel MP, Shoemark A, Kiviluoto S, Legendre M, Williams HJ, Vaughan CK, Hayward J, Goldenberg A, Emes RD, Munye MM, Dyer L, Cahill T, Bevillard J, Gehrig C, Guipponi M, Chantot S, Duquesnoy P, Thomas L, Jeanson L, Copin B, Tamalet A, Thauvin-Robinet C, Papon JF, Garin A, Pin I, Vera G, Aurora P, Fassad MR, Jenkins L, Boustred C, Cullup T, Dixon M, Onoufriadis A, Bush A, Chung EM, Antonarakis SE, Loebinger MR, Wilson R, Armengot M, Escudier E, Hogg C; UK10K Rare Group, Amselem S, Sun Z, Bartoloni L, Blouin JL, Mitchison HM.

Nat Commun. 2017 Feb 8;8:14279. doi: 10.1038/ncomms14279.


European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia.

Lucas JS, Barbato A, Collins SA, Goutaki M, Behan L, Caudri D, Dell S, Eber E, Escudier E, Hirst RA, Hogg C, Jorissen M, Latzin P, Legendre M, Leigh MW, Midulla F, Nielsen KG, Omran H, Papon JF, Pohunek P, Redfern B, Rigau D, Rindlisbacher B, Santamaria F, Shoemark A, Snijders D, Tonia T, Titieni A, Walker WT, Werner C, Bush A, Kuehni CE.

Eur Respir J. 2017 Jan 4;49(1). pii: 1601090. doi: 10.1183/13993003.01090-2016. Print 2017 Jan.


Effect of RBC Transfusion on Sublingual Microcirculation in Hemorrhagic Shock Patients: A Pilot Study.

Tanaka S, Escudier E, Hamada S, Harrois A, Leblanc PE, Vicaut E, Duranteau J.

Crit Care Med. 2017 Feb;45(2):e154-e160. doi: 10.1097/CCM.0000000000002064.


Mutations in DNAJB13, Encoding an HSP40 Family Member, Cause Primary Ciliary Dyskinesia and Male Infertility.

El Khouri E, Thomas L, Jeanson L, Bequignon E, Vallette B, Duquesnoy P, Montantin G, Copin B, Dastot-Le Moal F, Blanchon S, Papon JF, Lorès P, Yuan L, Collot N, Tissier S, Faucon C, Gacon G, Patrat C, Wolf JP, Dulioust E, Crestani B, Escudier E, Coste A, Legendre M, Touré A, Amselem S.

Am J Hum Genet. 2016 Aug 4;99(2):489-500. doi: 10.1016/j.ajhg.2016.06.022.


Clinical characteristics, functional respiratory decline and follow-up in adult patients with primary ciliary dyskinesia.

Frija-Masson J, Bassinet L, Honoré I, Dufeu N, Housset B, Coste A, Papon JF, Escudier E, Burgel PR, Maître B.

Thorax. 2017 Feb;72(2):154-160. doi: 10.1136/thoraxjnl-2015-207891. Epub 2016 Jul 5.


Mutations in GAS8, a Gene Encoding a Nexin-Dynein Regulatory Complex Subunit, Cause Primary Ciliary Dyskinesia with Axonemal Disorganization.

Jeanson L, Thomas L, Copin B, Coste A, Sermet-Gaudelus I, Dastot-Le Moal F, Duquesnoy P, Montantin G, Collot N, Tissier S, Papon JF, Clement A, Louis B, Escudier E, Amselem S, Legendre M.

Hum Mutat. 2016 Aug;37(8):776-85. doi: 10.1002/humu.23005. Epub 2016 May 12.


RSPH3 Mutations Cause Primary Ciliary Dyskinesia with Central-Complex Defects and a Near Absence of Radial Spokes.

Jeanson L, Copin B, Papon JF, Dastot-Le Moal F, Duquesnoy P, Montantin G, Cadranel J, Corvol H, Coste A, Désir J, Souayah A, Kott E, Collot N, Tissier S, Louis B, Tamalet A, de Blic J, Clement A, Escudier E, Amselem S, Legendre M.

Am J Hum Genet. 2015 Jul 2;97(1):153-62. doi: 10.1016/j.ajhg.2015.05.004. Epub 2015 Jun 11.


Technical and practical issues for tidal breathing measurements of nasal nitric oxide in children.

Beydon N, Chambellan A, Alberti C, de Blic J, Clément A, Escudier E, Le Bourgeois M.

Pediatr Pulmonol. 2015 Dec;50(12):1374-82. doi: 10.1002/ppul.23167. Epub 2015 Mar 2.


A new workflow for proteomic analysis of urinary exosomes and assessment in cystinuria patients.

Bourderioux M, Nguyen-Khoa T, Chhuon C, Jeanson L, Tondelier D, Walczak M, Ollero M, Bekri S, Knebelmann B, Escudier E, Escudier B, Edelman A, Guerrera IC.

J Proteome Res. 2015 Jan 2;14(1):567-77. doi: 10.1021/pr501003q. Epub 2014 Nov 12.


Proteomic analysis of nasal epithelial cells from cystic fibrosis patients.

Jeanson L, Guerrera IC, Papon JF, Chhuon C, Zadigue P, Prulière-Escabasse V, Amselem S, Escudier E, Coste A, Edelman A.

PLoS One. 2014 Sep 30;9(9):e108671. doi: 10.1371/journal.pone.0108671. eCollection 2014.


Airway cell involvement in intermittent hypoxia-induced airway inflammation.

Philippe C, Boussadia Y, Prulière-Escabasse V, Papon JF, Clérici C, Isabey D, Coste A, Escudier E, d'Ortho MP.

Sleep Breath. 2015 Mar;19(1):297-306. doi: 10.1007/s11325-014-1019-4. Epub 2014 Jul 4.


Loss-of-function mutations in RSPH1 cause primary ciliary dyskinesia with central-complex and radial-spoke defects.

Kott E, Legendre M, Copin B, Papon JF, Dastot-Le Moal F, Montantin G, Duquesnoy P, Piterboth W, Amram D, Bassinet L, Beucher J, Beydon N, Deneuville E, Houdouin V, Journel H, Just J, Nathan N, Tamalet A, Collot N, Jeanson L, Le Gouez M, Vallette B, Vojtek AM, Epaud R, Coste A, Clement A, Housset B, Louis B, Escudier E, Amselem S.

Am J Hum Genet. 2013 Sep 5;93(3):561-70. doi: 10.1016/j.ajhg.2013.07.013. Epub 2013 Aug 29.


Primary ciliary dyskinesia presentation in 60 children according to ciliary ultrastructure.

Vallet C, Escudier E, Roudot-Thoraval F, Blanchon S, Fauroux B, Beydon N, Boulé M, Vojtek AM, Amselem S, Clément A, Tamalet A.

Eur J Pediatr. 2013 Aug;172(8):1053-60. doi: 10.1007/s00431-013-1996-5. Epub 2013 Apr 10.


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.


Loss-of-function mutations in LRRC6, a gene essential for proper axonemal assembly of inner and outer dynein arms, cause primary ciliary dyskinesia.

Kott E, Duquesnoy P, Copin B, Legendre M, Dastot-Le Moal F, Montantin G, Jeanson L, Tamalet A, Papon JF, Siffroi JP, Rives N, Mitchell V, de Blic J, Coste A, Clement A, Escalier D, Touré A, Escudier E, Amselem S.

Am J Hum Genet. 2012 Nov 2;91(5):958-64. doi: 10.1016/j.ajhg.2012.10.003.


Quantitative analysis of ciliary beating in primary ciliary dyskinesia: a pilot study.

Papon JF, Bassinet L, Cariou-Patron G, Zerah-Lancner F, Vojtek AM, Blanchon S, Crestani B, Amselem S, Coste A, Housset B, Escudier E, Louis B.

Orphanet J Rare Dis. 2012 Oct 11;7:78. doi: 10.1186/1750-1172-7-78.


OFD1 mutations in males: phenotypic spectrum and ciliary basal body docking impairment.

Thauvin-Robinet C, Thomas S, Sinico M, Aral B, Burglen L, Gigot N, Dollfus H, Rossignol S, Raynaud M, Philippe C, Badens C, Touraine R, Gomes C, Franco B, Lopez E, Elkhartoufi N, Faivre L, Munnich A, Boddaert N, Van Maldergem L, Encha-Razavi F, Lyonnet S, Vekemans M, Escudier E, Attié-Bitach T.

Clin Genet. 2013 Jul;84(1):86-90. doi: 10.1111/cge.12013. Epub 2012 Oct 4. No abstract available.


Delineation of CCDC39/CCDC40 mutation spectrum and associated phenotypes in primary ciliary dyskinesia.

Blanchon S, Legendre M, Copin B, Duquesnoy P, Montantin G, Kott E, Dastot F, Jeanson L, Cachanado M, Rousseau A, Papon JF, Beydon N, Brouard J, Crestani B, Deschildre A, Désir J, Dollfus H, Leheup B, Tamalet A, Thumerelle C, Vojtek AM, Escalier D, Coste A, de Blic J, Clément A, Escudier E, Amselem S.

J Med Genet. 2012 Jun;49(6):410-6. doi: 10.1136/jmedgenet-2012-100867.


Longitudinal lung function and structural changes in children with primary ciliary dyskinesia.

Magnin ML, Cros P, Beydon N, Mahloul M, Tamalet A, Escudier E, Clément A, Le Pointe HD, Blanchon S.

Pediatr Pulmonol. 2012 Aug;47(8):816-25. doi: 10.1002/ppul.22577. Epub 2012 May 8.


Oxidative stress induces unfolding protein response and inflammation in nasal polyposis.

Jeanson L, Kelly M, Coste A, Guerrera IC, Fritsch J, Nguyen-Khoa T, Baudouin-Legros M, Papon JF, Zadigue P, Prulière-Escabasse V, Amselem S, Escudier E, Edelman A.

Allergy. 2012 Mar;67(3):403-12. doi: 10.1111/j.1398-9995.2011.02769.x. Epub 2011 Dec 22.


CCDC39 is required for assembly of inner dynein arms and the dynein regulatory complex and for normal ciliary motility in humans and dogs.

Merveille AC, Davis EE, Becker-Heck A, Legendre M, Amirav I, Bataille G, Belmont J, Beydon N, Billen F, Clément A, Clercx C, Coste A, Crosbie R, de Blic J, Deleuze S, Duquesnoy P, Escalier D, Escudier E, Fliegauf M, Horvath J, Hill K, Jorissen M, Just J, Kispert A, Lathrop M, Loges NT, Marthin JK, Momozawa Y, Montantin G, Nielsen KG, Olbrich H, Papon JF, Rayet I, Roger G, Schmidts M, Tenreiro H, Towbin JA, Zelenika D, Zentgraf H, Georges M, Lequarré AS, Katsanis N, Omran H, Amselem S.

Nat Genet. 2011 Jan;43(1):72-8. doi: 10.1038/ng.726. Epub 2010 Dec 5.


Otologic features in children with primary ciliary dyskinesia.

Prulière-Escabasse V, Coste A, Chauvin P, Fauroux B, Tamalet A, Garabedian EN, Escudier E, Roger G.

Arch Otolaryngol Head Neck Surg. 2010 Nov;136(11):1121-6. doi: 10.1001/archoto.2010.183.


Effect of neutrophil elastase and its inhibitor EPI-hNE4 on transepithelial sodium transport across normal and cystic fibrosis human nasal epithelial cells.

Prulière-Escabasse V, Clerici C, Vuagniaux G, Coste A, Escudier E, Planès C.

Respir Res. 2010 Oct 8;11:141. doi: 10.1186/1465-9921-11-141.


Abnormal respiratory cilia in non-syndromic Leber congenital amaurosis with CEP290 mutations.

Papon JF, Perrault I, Coste A, Louis B, Gérard X, Hanein S, Fares-Taie L, Gerber S, Defoort-Dhellemmes S, Vojtek AM, Kaplan J, Rozet JM, Escudier E.

J Med Genet. 2010 Dec;47(12):829-34. doi: 10.1136/jmg.2010.077883. Epub 2010 Aug 30.


Rhinitis in patients treated with a combination of an mTOR inhibitor and an EGFR inhibitor.

Prulière-Escabasse V, Gomez-Roca C, Escudier E, Coste A, Besse B, Massard C, Soria JC.

Onkologie. 2010;33(7):401-2. doi: 10.1159/000315757. Epub 2010 Jun 22. No abstract available.


Loss-of-function mutations in the human ortholog of Chlamydomonas reinhardtii ODA7 disrupt dynein arm assembly and cause primary ciliary dyskinesia.

Duquesnoy P, Escudier E, Vincensini L, Freshour J, Bridoux AM, Coste A, Deschildre A, de Blic J, Legendre M, Montantin G, Tenreiro H, Vojtek AM, Loussert C, Clément A, Escalier D, Bastin P, Mitchell DR, Amselem S.

Am J Hum Genet. 2009 Dec;85(6):890-6. doi: 10.1016/j.ajhg.2009.11.008.


A 20-year experience of electron microscopy in the diagnosis of primary ciliary dyskinesia.

Papon JF, Coste A, Roudot-Thoraval F, Boucherat M, Roger G, Tamalet A, Vojtek AM, Amselem S, Escudier E.

Eur Respir J. 2010 May;35(5):1057-63. doi: 10.1183/09031936.00046209. Epub 2009 Oct 19.


CC2D2A mutations in Meckel and Joubert syndromes indicate a genotype-phenotype correlation.

Mougou-Zerelli S, Thomas S, Szenker E, Audollent S, Elkhartoufi N, Babarit C, Romano S, Salomon R, Amiel J, Esculpavit C, Gonzales M, Escudier E, Leheup B, Loget P, Odent S, Roume J, Gérard M, Delezoide AL, Khung S, Patrier S, Cordier MP, Bouvier R, Martinovic J, Gubler MC, Boddaert N, Munnich A, Encha-Razavi F, Valente EM, Saad A, Saunier S, Vekemans M, Attié-Bitach T.

Hum Mutat. 2009 Nov;30(11):1574-82. doi: 10.1002/humu.21116.


Ciliary defects and genetics of primary ciliary dyskinesia.

Escudier E, Duquesnoy P, Papon JF, Amselem S.

Paediatr Respir Rev. 2009 Jun;10(2):51-4. doi: 10.1016/j.prrv.2009.02.001. Epub 2009 Apr 18. Review.


Muco-ciliary differentiation of nasal epithelial cells is decreased after wound healing in vitro.

Lazard DS, Moore A, Hupertan V, Martin C, Escabasse V, Dreyfus P, Burgel PR, Amselem S, Escudier E, Coste A.

Allergy. 2009 Aug;64(8):1136-43. doi: 10.1111/j.1398-9995.2009.02003.x. Epub 2009 Feb 24.


Rhinitis and epistaxis in patients treated by anti-angiogenic therapy.

Prulière-Escabasse V, Escudier E, Balheda R, Soria JC, Coste A, Massard C.

Invest New Drugs. 2009 Jun;27(3):285-6. doi: 10.1007/s10637-008-9168-6. Epub 2008 Aug 27.


Phagocytosis of Aspergillus fumigatus conidia by primary nasal epithelial cells in vitro.

Botterel F, Gross K, Ibrahim-Granet O, Khoufache K, Escabasse V, Coste A, Cordonnier C, Escudier E, Bretagne S.

BMC Microbiol. 2008 Jun 18;8:97. doi: 10.1186/1471-2180-8-97.


Modulation of epithelial sodium channel trafficking and function by sodium 4-phenylbutyrate in human nasal epithelial cells.

Prulière-Escabasse V, Planès C, Escudier E, Fanen P, Coste A, Clerici C.

J Biol Chem. 2007 Nov 23;282(47):34048-57. Epub 2007 Sep 21.


A heterozygous mutation disrupting the SPAG16 gene results in biochemical instability of central apparatus components of the human sperm axoneme.

Zhang Z, Zariwala MA, Mahadevan MM, Caballero-Campo P, Shen X, Escudier E, Duriez B, Bridoux AM, Leigh M, Gerton GL, Kennedy M, Amselem S, Knowles MR, Strauss JF 3rd.

Biol Reprod. 2007 Nov;77(5):864-71. Epub 2007 Aug 15.


A common variant in combination with a nonsense mutation in a member of the thioredoxin family causes primary ciliary dyskinesia.

Duriez B, Duquesnoy P, Escudier E, Bridoux AM, Escalier D, Rayet I, Marcos E, Vojtek AM, Bercher JF, Amselem S.

Proc Natl Acad Sci U S A. 2007 Feb 27;104(9):3336-41. Epub 2007 Feb 20. Erratum in: Proc Natl Acad Sci U S A. 2007 Apr 10;104(15):6490.


[Injury and epithelial wound healing: a pathophysiologic hypothesis for nasal and sinus polyposis].

Lazard DS, Prulière-Escabasse V, Papon JF, Escudier E, Coste A.

Presse Med. 2007 Jul-Aug;36(7-8):1104-8. Epub 2007 Feb 15. Review. French.


Verruculogen associated with Aspergillus fumigatus hyphae and conidia modifies the electrophysiological properties of human nasal epithelial cells.

Khoufache K, Puel O, Loiseau N, Delaforge M, Rivollet D, Coste A, Cordonnier C, Escudier E, Botterel F, Bretagne S.

BMC Microbiol. 2007 Jan 23;7:5.


Matrix metalloproteinase-2 and -9 expression in sinonasal inverted papilloma.

Papon JF, Lechapt-Zalcman E, Abina M, Abd-al-Sama I, Peynègre R, Escudier E, Coste A.

Rhinology. 2006 Sep;44(3):211-5.


Mutations of DNAI1 in primary ciliary dyskinesia: evidence of founder effect in a common mutation.

Zariwala MA, Leigh MW, Ceppa F, Kennedy MP, Noone PG, Carson JL, Hazucha MJ, Lori A, Horvath J, Olbrich H, Loges NT, Bridoux AM, Pennarun G, Duriez B, Escudier E, Mitchison HM, Chodhari R, Chung EM, Morgan LC, de Iongh RU, Rutland J, Pradal U, Omran H, Amselem S, Knowles MR.

Am J Respir Crit Care Med. 2006 Oct 15;174(8):858-66. Epub 2006 Jul 20.


Outcome of ICSI with ejaculated spermatozoa in a series of men with distinct ultrastructural flagellar abnormalities.

Mitchell V, Rives N, Albert M, Peers MC, Selva J, Clavier B, Escudier E, Escalier D.

Hum Reprod. 2006 Aug;21(8):2065-74. Epub 2006 Apr 27.


Transforming growth factor-beta1 increases airway wound repair via MMP-2 upregulation: a new pathway for epithelial wound repair?

Lechapt-Zalcman E, Prulière-Escabasse V, Advenier D, Galiacy S, Charrière-Bertrand C, Coste A, Harf A, d'Ortho MP, Escudier E.

Am J Physiol Lung Cell Mol Physiol. 2006 Jun;290(6):L1277-82. Epub 2006 Jan 13.


Impairment of nitric oxide output of conducting airways in primary ciliary dyskinesia.

Mahut B, Escudier E, de Blic J, Zerah-Lancner F, Coste A, Harf A, Delclaux C.

Pediatr Pulmonol. 2006 Feb;41(2):158-63.


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