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Ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation.
Sermet-Gaudelus I. Sermet-Gaudelus I. Eur Respir Rev. 2013 Mar 1;22(127):66-71. doi: 10.1183/09059180.00008512. Eur Respir Rev. 2013. PMID: 23457167 Free PMC article. Review.
In the era of personalised medicine, ivacaftor offers an effective and well-tolerated treatment for the clinical management of CF patients with the G551D mutation. A long-term, open-label study will report the effects of ivacaftor over a further 48 weeks....
In the era of personalised medicine, ivacaftor offers an effective and well-tolerated treatment for the clinical management of CF patients w …
Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice.
da Cunha MF, Pranke I, Sassi A, Schreiweis C, Moriceau S, Vidovic D, Hatton A, Carlon MS, Creste G, Berhal F, Prestat G, Freund R, Odolczyk N, Jais JP, Gravier-Pelletier C, Zielenkiewicz P, Jullien V, Hinzpeter A, Oury F, Edelman A, Sermet-Gaudelus I. da Cunha MF, et al. Among authors: sermet gaudelus i. Sci Rep. 2022 Apr 12;12(1):6132. doi: 10.1038/s41598-022-09678-9. Sci Rep. 2022. PMID: 35413967 Free PMC article.
Using nasal potential difference measurement, we showed that in vivo administration of c407 by topical, short-term intraperitoneal and long-term subcutaneous route significantly increased the CFTR dependent chloride (Cl(-)) conductance in F508del Cftr(tm1Eur) mice. …
Using nasal potential difference measurement, we showed that in vivo administration of c407 by topical, short-term intraperitoneal an …
Involvement of CFTR in the pathogenesis of pulmonary arterial hypertension.
Le Ribeuz H, To L, Ghigna MR, Martin C, Nagaraj C, Dreano E, Rucker-Martin C, Girerd B, Bouligand J, Pechoux C, Lambert M, Boet A, Issard J, Mercier O, Hoetzenecker K, Manoury B, Becq F, Burgel PR, Cottart CH, Olschewski A, Sermet-Gaudelus I, Perros F, Humbert M, Montani D, Antigny F. Le Ribeuz H, et al. Among authors: sermet gaudelus i. Eur Respir J. 2021 Nov 25;58(5):2000653. doi: 10.1183/13993003.00653-2020. Print 2021 Nov. Eur Respir J. 2021. PMID: 33926975 Free article.
CFTR-mediated pulmonary artery relaxation was reduced in pulmonary arteries from iPAH patients and rats with monocrotaline- or chronic hypoxia-induced PH. Long-term in vivo CFTR inhibition in rats significantly increased right ventricular systolic pressure, which was relat …
CFTR-mediated pulmonary artery relaxation was reduced in pulmonary arteries from iPAH patients and rats with monocrotaline- or chronic hypox …
Genomic analysis of Staphylococcus aureus sequential isolates from lungs of patients with cystic fibrosis.
Ding X, Fu X, Euphrasie D, Ferroni A, Sermet-Gaudelus I, Charbit A, Coureuil M, Jamet A. Ding X, et al. Among authors: sermet gaudelus i. Microbes Infect. 2023 Jul-Aug;25(6):105124. doi: 10.1016/j.micinf.2023.105124. Epub 2023 Mar 5. Microbes Infect. 2023. PMID: 36871931 Free article.
Further explorations relying notably on proteomics will contribute to improve our understanding of the mechanisms at play in the striking long-term persistence ability of S. aureus....
Further explorations relying notably on proteomics will contribute to improve our understanding of the mechanisms at play in the striking lo …
Arterial abnormalities identified in kidneys transplanted into children during the COVID-19 pandemic.
Berteloot L, Berthaud R, Temmam S, Lozach C, Zanelli E, Blanc T, Heloury Y, Capito C, Chardot C, Sarnacki S, Garcelon N, Lacaille F, Charbit M, Pastural M, Rabant M, Boddaert N, Leruez-Ville M, Eloit M, Sermet-Gaudelus I, Dehoux L, Boyer O. Berteloot L, et al. Among authors: sermet gaudelus i. Am J Transplant. 2021 May;21(5):1937-1943. doi: 10.1111/ajt.16464. Epub 2021 Feb 18. Am J Transplant. 2021. PMID: 33346946 Free PMC article.
Graft artery stenosis can have a significant short- and long-term negative impact on renal graft function. From the beginning of the COVID-19 pandemic, we noticed an unusual number of graft arterial anomalies following kidney transplant (KTx) in children. ...
Graft artery stenosis can have a significant short- and long-term negative impact on renal graft function. From the beginning of the …
[Therapeutics in cystic fibrosis: Clinical revolution and new challenges].
Bardin E, Pranke I, Hinzpeter A, Sermet-Gaudelus I. Bardin E, et al. Among authors: sermet gaudelus i. Med Sci (Paris). 2024 Mar;40(3):258-267. doi: 10.1051/medsci/2024014. Epub 2024 Mar 22. Med Sci (Paris). 2024. PMID: 38520101 French.
The community of researchers, pharmaceutical industries, patient associations, and health authorities remains committed to monitor the long-term effects of these still poorly characterised treatments, and to explore new pharmacological approaches, such as gene therapies... …
The community of researchers, pharmaceutical industries, patient associations, and health authorities remains committed to monitor the long- …
A Phase 3, open-label, 96-week trial to study the safety, tolerability, and efficacy of tezacaftor/ivacaftor in children ≥ 6 years of age homozygous for F508del or heterozygous for F508del and a residual function CFTR variant.
Sawicki GS, Chilvers M, McNamara J, Naehrlich L, Saunders C, Sermet-Gaudelus I, Wainwright CE, Ahluwalia N, Campbell D, Harris RS, Paz-Diaz H, Shih JL, Davies JC. Sawicki GS, et al. Among authors: sermet gaudelus i. J Cyst Fibros. 2022 Jul;21(4):675-683. doi: 10.1016/j.jcf.2022.02.003. Epub 2022 Feb 18. J Cyst Fibros. 2022. PMID: 35190292 Free article. Clinical Trial.
CONCLUSIONS: Tezacaftor/ivacaftor is generally safe and well tolerated, and treatment effects are maintained for up to 120 weeks. These results support long-term use of tezacaftor/ivacaftor in children 6 years of age with CF and F/F or F/RF genotypes....
CONCLUSIONS: Tezacaftor/ivacaftor is generally safe and well tolerated, and treatment effects are maintained for up to 120 weeks. These resu …
Neuroimaging manifestations in children with SARS-CoV-2 infection: a multinational, multicentre collaborative study.
Lindan CE, Mankad K, Ram D, Kociolek LK, Silvera VM, Boddaert N, Stivaros SM, Palasis S; ASPNR PECOBIG Collaborator Group. Lindan CE, et al. Lancet Child Adolesc Health. 2021 Mar;5(3):167-177. doi: 10.1016/S2352-4642(20)30362-X. Epub 2020 Dec 16. Lancet Child Adolesc Health. 2021. PMID: 33338439 Free PMC article.
Studies of paediatric specific cohorts are needed to better understand the effects of SARS-CoV-2 infection on the CNS at presentation and on long-term follow-up in children. FUNDING: American Society of Pediatric Neuroradiology, University of Manchester (Manchester, UK). . …
Studies of paediatric specific cohorts are needed to better understand the effects of SARS-CoV-2 infection on the CNS at presentation and on …
ECFS standards of care on CFTR-related disorders: Towards a comprehensive program for affected individuals.
De Wachter E, De Boeck K, Sermet-Gaudelus I, Simmonds NJ, Munck A, Naehrlich L, Barben J, Boyd C, Veen SJ, Carr SB, Fajac I, Farrell PM, Girodon E, Gonska T, Grody WW, Jain M, Jung A, Kerem E, Raraigh KS, van Koningsbruggen-Rietschel S, Waller MD, Southern KW, Castellani C; ECFS Diagnostic Network Working Group. De Wachter E, et al. Among authors: sermet gaudelus i. J Cyst Fibros. 2024 Feb 21:S1569-1993(24)00011-0. doi: 10.1016/j.jcf.2024.01.012. Online ahead of print. J Cyst Fibros. 2024. PMID: 38388234
Topics included are: 1) benefits and obstacles to collect data from pwCFTR-RD are discussed, together with the opportunity to integrate them into established CF-registries; 2) the potential of infants designated CRMS/CFSPID to develop a CFTR-RD and how to communicate this informa …
Topics included are: 1) benefits and obstacles to collect data from pwCFTR-RD are discussed, together with the opportunity to integrate them …
Sweat Chloride Testing and Nasal Potential Difference (NPD) Are Primary Outcome Parameters in Treatment with Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators.
Sermet-Gaudelus I, Nguyen-Khoa T, Hatton A, Hayes K, Pranke I. Sermet-Gaudelus I, et al. J Pers Med. 2021 Jul 27;11(8):729. doi: 10.3390/jpm11080729. J Pers Med. 2021. PMID: 34442373 Free PMC article.
It has been used extensively as a biomarker of CFTR function in clinical trials of CFTR modulator therapies and provides evidence for change in the short term. The level of functional rescue in the NPD increases up to 40% of normal CFTR in patients with a Gly551Asp treated …
It has been used extensively as a biomarker of CFTR function in clinical trials of CFTR modulator therapies and provides evidence for change …
16 results