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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 …
Intrapulmonary percussive ventilation improves lung function in cystic fibrosis patients chronically colonized with Pseudomonas aeruginosa: a pilot cross-over study.
Dingemans J, Eyns H, Willekens J, Monsieurs P, Van Houdt R, Cornelis P, Malfroot A, Crabbé A. Dingemans J, et al. Among authors: malfroot a. Eur J Clin Microbiol Infect Dis. 2018 Jun;37(6):1143-1151. doi: 10.1007/s10096-018-3232-8. Epub 2018 Mar 20. Eur J Clin Microbiol Infect Dis. 2018. PMID: 29560543 Clinical Trial.
We performed a first-of-its-kind pilot clinical study to evaluate short-term IPV therapy at medium (200 bursts per minute, bpm) and high frequency (400 bpm) as compared to autogenic drainage (AD) on lung function and the behavior of P. aeruginosa in the CF lung in four pat …
We performed a first-of-its-kind pilot clinical study to evaluate short-term IPV therapy at medium (200 bursts per minute, bpm) and h …
The ciliopathy-associated CPLANE proteins direct basal body recruitment of intraflagellar transport machinery.
Toriyama M, Lee C, Taylor SP, Duran I, Cohn DH, Bruel AL, Tabler JM, Drew K, Kelly MR, Kim S, Park TJ, Braun DA, Pierquin G, Biver A, Wagner K, Malfroot A, Panigrahi I, Franco B, Al-Lami HA, Yeung Y, Choi YJ; University of Washington Center for Mendelian Genomics; Duffourd Y, Faivre L, Rivière JB, Chen J, Liu KJ, Marcotte EM, Hildebrandt F, Thauvin-Robinet C, Krakow D, Jackson PK, Wallingford JB. Toriyama M, et al. Among authors: malfroot a. Nat Genet. 2016 Jun;48(6):648-56. doi: 10.1038/ng.3558. Epub 2016 May 9. Nat Genet. 2016. PMID: 27158779 Free PMC article.
Combining proteomics, in vivo imaging and genetic analysis of proteins linked to planar cell polarity (Inturned, Fuzzy and Wdpcp), we identified and characterized a new genetic module, which we term CPLANE (ciliogenesis and planar polarity effector), and an extensive assoc …
Combining proteomics, in vivo imaging and genetic analysis of proteins linked to planar cell polarity (Inturned, Fuzzy and Wdpcp), we identi …
The clinical benefits of long-term supplementation with omega-3 fatty acids in cystic fibrosis patients - A pilot study.
Hanssens L, Thiébaut I, Lefèvre N, Malfroot A, Knoop C, Duchateau J, Casimir G. Hanssens L, et al. Among authors: malfroot a. Prostaglandins Leukot Essent Fatty Acids. 2016 May;108:45-50. doi: 10.1016/j.plefa.2016.03.014. Epub 2016 Mar 28. Prostaglandins Leukot Essent Fatty Acids. 2016. PMID: 27154364 Clinical Trial.
Supplementation significantly increased the levels of eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) as early as <3 months of administration, with concomitant decreases in arachidonic acid (AA) levels. This pilot study suggests that long-term omega-3 supplem …
Supplementation significantly increased the levels of eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) as early as <3 months of …
Long-term inhaled dry powder mannitol in cystic fibrosis: an international randomized study.
Aitken ML, Bellon G, De Boeck K, Flume PA, Fox HG, Geller DE, Haarman EG, Hebestreit HU, Lapey A, Schou IM, Zuckerman JB, Charlton B; CF302 Investigators. Aitken ML, et al. Am J Respir Crit Care Med. 2012 Mar 15;185(6):645-52. doi: 10.1164/rccm.201109-1666OC. Epub 2011 Dec 28. Am J Respir Crit Care Med. 2012. PMID: 22198974 Clinical Trial.
RATIONALE: New treatment strategies are needed to improve airway clearance and reduce the morbidity and the time burden associated with cystic fibrosis (CF). OBJECTIVES: To determine whether long-term treatment with inhaled mannitol, an osmotic agent, improves lung functio …
RATIONALE: New treatment strategies are needed to improve airway clearance and reduce the morbidity and the time burden associated with cyst …
Chest physiotherapy in cystic fibrosis: short-term effects of autogenic drainage preceded by wet inhalation of saline versus autogenic drainage preceded by intrapulmonary percussive ventilation with saline.
Van Ginderdeuren F, Verbanck S, Van Cauwelaert K, Vanlaethem S, Schuermans D, Vincken W, Malfroot A. Van Ginderdeuren F, et al. Among authors: malfroot a. Respiration. 2008;76(2):175-80. doi: 10.1159/000111818. Epub 2007 Nov 28. Respiration. 2008. PMID: 18042976 Clinical Trial.
BACKGROUND: Chest physiotherapy has been used for many years to assist in the removal of abnormal viscid bronchial secretions in cystic fibrosis (CF) patients. OBJECTIVES: This study compared the short-term effects of two physiotherapy regimens in patients with CF: autogen …
BACKGROUND: Chest physiotherapy has been used for many years to assist in the removal of abnormal viscid bronchial secretions in cystic fibr …