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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: simmonds nj. 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 …
Cystic fibrosis modulator therapy can reverse cystic bronchiectasis.
Middleton PG, Simmonds NJ. Middleton PG, et al. Among authors: simmonds nj. Respirol Case Rep. 2023 Jun 12;11(7):e01172. doi: 10.1002/rcr2.1172. eCollection 2023 Jul. Respirol Case Rep. 2023. PMID: 37323158 Free PMC article.
Dramatic improvements in lung function, sputum production, daytime functioning, and quality of life are seen within weeks. However, the effect of long-term exposure to elexacaftor + tezacaftor + ivacaftor (ETI) on the structural abnormalities is at present unknown. ...
Dramatic improvements in lung function, sputum production, daytime functioning, and quality of life are seen within weeks. However, the effe …
VOCAL: An observational study of ivacaftor for people with cystic fibrosis and selected non-G551D-CFTR gating mutations.
Simmonds NJ, van der Ent CK, Colombo C, Kinnman N, DeSouza C, Thorat T, Chew ML, Chandarana K, Castellani C. Simmonds NJ, et al. J Cyst Fibros. 2023 Jan;22(1):124-131. doi: 10.1016/j.jcf.2022.05.007. Epub 2022 May 23. J Cyst Fibros. 2023. PMID: 35613999 Free article.
BACKGROUND: VOCAL was an observational study of the effect of long-term ivacaftor on real-world clinical outcomes and healthcare resource utilization (HCRU) in people with cystic fibrosis (pwCF) in Italy, the Netherlands, and the UK. ...Reported safety results were consist …
BACKGROUND: VOCAL was an observational study of the effect of long-term ivacaftor on real-world clinical outcomes and healthcare reso …
Entering the era of highly effective modulator therapies.
Dave K, Dobra R, Scott S, Saunders C, Matthews J, Simmonds NJ, Davies JC. Dave K, et al. Among authors: simmonds nj. Pediatr Pulmonol. 2021 Feb;56 Suppl 1:S79-S89. doi: 10.1002/ppul.24968. Pediatr Pulmonol. 2021. PMID: 33434412 Review.
It is highly effective with impressive clinical impact in the lungs and gastrointestinal tract; longer-term data from patient registries show fewer exacerbations, a slower rate of lung function loss and reduced need for transplantation in patients receiving ivacaftor. ...T …
It is highly effective with impressive clinical impact in the lungs and gastrointestinal tract; longer-term data from patient registr …
Ageing in cystic fibrosis and long-term survival.
Simmonds NJ. Simmonds NJ. Paediatr Respir Rev. 2013 May;14 Suppl 1:6-9. doi: 10.1016/j.prrv.2013.01.007. Epub 2013 Feb 24. Paediatr Respir Rev. 2013. PMID: 23497942 Review.
Cystic fibrosis: management of haemoptysis.
Hurt K, Simmonds NJ. Hurt K, et al. Among authors: simmonds nj. Paediatr Respir Rev. 2012 Dec;13(4):200-5. doi: 10.1016/j.prrv.2012.01.003. Epub 2012 Feb 28. Paediatr Respir Rev. 2012. PMID: 23069116 Review.
This will control the bleeding in the majority of cases but recurrence rates are high and there are little data to support long-term improved outcomes. Surgery is a last resort in patients with CF....
This will control the bleeding in the majority of cases but recurrence rates are high and there are little data to support long-term …
Improved survival at low lung function in cystic fibrosis: cohort study from 1990 to 2007.
George PM, Banya W, Pareek N, Bilton D, Cullinan P, Hodson ME, Simmonds NJ. George PM, et al. Among authors: simmonds nj. BMJ. 2011 Feb 28;342:d1008. doi: 10.1136/bmj.d1008. BMJ. 2011. PMID: 21357627 Free PMC article.
Significantly increased risks were associated with a body mass index under 19 (hazard ratio 1.52, 1.10 to 2.10), long term oxygen therapy (3.52, 2.49 to 4.99), and nebulised antibiotics (1.84, 1.05 to 3.22). ...
Significantly increased risks were associated with a body mass index under 19 (hazard ratio 1.52, 1.10 to 2.10), long term oxygen the …
Cystic fibrosis and survival to 40 years: a study of cystic fibrosis transmembrane conductance regulator function.
Simmonds NJ, D'Souza L, Roughton M, Alton EW, Davies JC, Hodson ME. Simmonds NJ, et al. Eur Respir J. 2011 May;37(5):1076-82. doi: 10.1183/09031936.00079010. Epub 2010 Sep 16. Eur Respir J. 2011. PMID: 20847077 Free article.
Our aim was to determine whether residual function of the cystic fibrosis transmembrane conductance regulator (CFTR) is present in long-term survivors with severe mutations. Nasal potential difference (PD) and sweat chloride were measured in 34 long-term survivors ( …
Our aim was to determine whether residual function of the cystic fibrosis transmembrane conductance regulator (CFTR) is present in long-t …
13 results