Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation

Search Page

Filters

My NCBI Filters

Text availability

Article attribute

Article type

Publication date

Search Results

17 results

Filters applied: . Clear all
Page 1
Rapid Improvement after Starting Elexacaftor-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease.
Burgel PR, Durieu I, Chiron R, Ramel S, Danner-Boucher I, Prevotat A, Grenet D, Marguet C, Reynaud-Gaubert M, Macey J, Mely L, Fanton A, Quetant S, Lemonnier L, Paillasseur JL, Da Silva J, Martin C; French Cystic Fibrosis Reference Network Study Group. Burgel PR, et al. Am J Respir Crit Care Med. 2021 Jul 1;204(1):64-73. doi: 10.1164/rccm.202011-4153OC. Am J Respir Crit Care Med. 2021. PMID: 33600738

The mean (95% confidence interval) absolute increase in the ppFEV(1) was +15.1 (+13.8 to +16.4; P < 0.0001), and the mean (95% confidence interval) in weight was +4.2 kg (+3.9 to +4.6; P < 0.0001). The number of patients requiring long-term oxygen, noninvasive ventil

…

The mean (95% confidence interval) absolute increase in the ppFEV(1) was +15.1 (+13.8 to +16.4; P < 0.0001), and the mean (95% confidence

…
Long-term safety and efficacy of tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study.
Flume PA, Biner RF, Downey DG, Brown C, Jain M, Fischer R, De Boeck K, Sawicki GS, Chang P, Paz-Diaz H, Rubin JL, Yang Y, Hu X, Pasta DJ, Millar SJ, Campbell D, Wang X, Ahluwalia N, Owen CA, Wainwright CE; VX14-661-110 study group. Flume PA, et al. Lancet Respir Med. 2021 Jul;9(7):733-746. doi: 10.1016/S2213-2600(20)30510-5. Epub 2021 Feb 10. Lancet Respir Med. 2021. PMID: 33581080
Here, we present results of study 661-110 (EXTEND), a 96-week open-label extension study that assessed long-term safety, tolerability, and efficacy of tezacaftor-ivacaftor in participants aged 12 years or older with cystic fibrosis who were homozygous or heterozygous for t …
Here, we present results of study 661-110 (EXTEND), a 96-week open-label extension study that assessed long-term safety, tolerability …
Pregnancy after lung and heart-lung transplantation: a French multicentre retrospective study of 39 pregnancies.
Bry C, Hubert D, Reynaud-Gaubert M, Dromer C, Mal H, Roux A, Boussaud V, Claustre J, Le Pavec J, Murris-Espin M, Danner-Boucher I. Bry C, et al. Among authors: hubert d. ERJ Open Res. 2019 Oct 30;5(4):00254-2018. doi: 10.1183/23120541.00254-2018. eCollection 2019 Oct. ERJ Open Res. 2019. PMID: 31687369 Free PMC article.
Meansd time between transplantation and pregnancy was 6344 months. 26 births occurred (67%) with a mean term of 36 weeks of amenorrhoea and a mean birthweight of 2409 g. ...
Meansd time between transplantation and pregnancy was 6344 months. 26 births occurred (67%) with a mean term of 36 weeks of amenorrho …
Retrospective observational study of French patients with cystic fibrosis and a Gly551Asp-CFTR mutation after 1 and 2years of treatment with ivacaftor in a real-world setting.
Hubert D, Dehillotte C, Munck A, David V, Baek J, Mely L, Dominique S, Ramel S, Danner Boucher I, Lefeuvre S, Reynaud Q, Colomb-Jung V, Bakouboula P, Lemonnier L. Hubert D, et al. J Cyst Fibros. 2018 Jan;17(1):89-95. doi: 10.1016/j.jcf.2017.07.001. Epub 2017 Jul 12. J Cyst Fibros. 2018. PMID: 28711222 Free article.
Real-world evaluation is warranted to examine its safety and effectiveness over the long term. METHODS: A retrospective observational multicentre study collected clinical data in the year before and the 2years after ivacaftor initiation in patients with CF and a Gly551Asp- …
Real-world evaluation is warranted to examine its safety and effectiveness over the long term. METHODS: A retrospective observational …
Real-life initiation of lumacaftor/ivacaftor combination in adults with cystic fibrosis homozygous for the Phe508del CFTR mutation and severe lung disease.
Hubert D, Chiron R, Camara B, Grenet D, Prévotat A, Bassinet L, Dominique S, Rault G, Macey J, Honoré I, Kanaan R, Leroy S, Desmazes Dufeu N, Burgel PR. Hubert D, et al. J Cyst Fibros. 2017 May;16(3):388-391. doi: 10.1016/j.jcf.2017.03.003. Epub 2017 Mar 18. J Cyst Fibros. 2017. PMID: 28325531 Free article.
OBJECTIVE: To investigate the short-term adverse events and effectiveness of lumacaftor/ivacaftor combination treatment in adults with cystic fibrosis (CF) and severe lung disease in a real life setting. ...
OBJECTIVE: To investigate the short-term adverse events and effectiveness of lumacaftor/ivacaftor combination treatment in adults wit …
DCTN4 as a modifier of chronic Pseudomonas aeruginosa infection in cystic fibrosis.
Viel M, Hubert D, Burgel PR, Génin E, Honoré I, Martinez B, Gaitch N, Chapron J, Kanaan R, Dusser D, Girodon E, Bienvenu T. Viel M, et al. Among authors: hubert d. Clin Respir J. 2016 Nov;10(6):777-783. doi: 10.1111/crj.12288. Epub 2015 Apr 15. Clin Respir J. 2016. PMID: 25763772
BACKGROUND AND AIMS: Pseudomonas aeruginosa (Pa) infection in cystic fibrosis (CF) patients is associated with worse long-term pulmonary disease and shorter survival, and chronic Pa infection (CPA) is associated with reduced lung function, faster rate of lung decline, incr …
BACKGROUND AND AIMS: Pseudomonas aeruginosa (Pa) infection in cystic fibrosis (CF) patients is associated with worse long-term pulmon …
Maternal and perinatal outcomes of pregnancies in women with cystic fibrosis--A single centre case-control study.
Girault A, Blanc J, Gayet V, Goffinet F, Hubert D. Girault A, et al. Among authors: hubert d. Respir Med. 2016 Apr;113:22-7. doi: 10.1016/j.rmed.2016.02.010. Epub 2016 Feb 23. Respir Med. 2016. PMID: 27021576 Free article.
METHODS: In this retrospective case-control study, we compared the maternal and perinatal outcomes of 33 pregnancies in CF women who delivered in our maternity ward from December 2000 to December 2013 and were matched to 66 controls. RESULTS: The median term of delivery wa …
METHODS: In this retrospective case-control study, we compared the maternal and perinatal outcomes of 33 pregnancies in CF women who deliver …
17 results