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Macrolide antibiotics (including azithromycin) for cystic fibrosis.
Southern KW, Solis-Moya A, Kurz D, Smith S. Southern KW, et al. Cochrane Database Syst Rev. 2024 Feb 27;2(2):CD002203. doi: 10.1002/14651858.CD002203.pub5. Cochrane Database Syst Rev. 2024. PMID: 38411248 Review.
Azithromycin appears to have a good safety profile (although a weekly dose was associated with more gastrointestinal side effects, which makes it less acceptable for long-term therapy), with a relatively minimal treatment burden for people with CF, and it is inexpensive. A …
Azithromycin appears to have a good safety profile (although a weekly dose was associated with more gastrointestinal side effects, which mak …
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: southern kw. 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 …
Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del).
Heneghan M, Southern KW, Murphy J, Sinha IP, Nevitt SJ. Heneghan M, et al. Among authors: southern kw. Cochrane Database Syst Rev. 2023 Nov 20;11(11):CD010966. doi: 10.1002/14651858.CD010966.pub4. Cochrane Database Syst Rev. 2023. PMID: 37983082 Review.
Lumacaftor-ivacaftor was associated with an increase in early transient shortness of breath and longer-term increases in blood pressure (not observed for tezacaftor-ivacaftor). ...In this population, lumacaftor-ivacaftor had an important impact on respiratory function with …
Lumacaftor-ivacaftor was associated with an increase in early transient shortness of breath and longer-term increases in blood pressu …
Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis.
Francis NZ, Southern KW. Francis NZ, et al. Among authors: southern kw. Cochrane Database Syst Rev. 2022 Sep 2;9(9):CD002204. doi: 10.1002/14651858.CD002204.pub5. Cochrane Database Syst Rev. 2022. PMID: 36053129 Free PMC article. Review.
High doses of corticosteroids are the main treatment for ABPA; although the long-term benefits are not clear, and their many side effects are well-documented. ...
High doses of corticosteroids are the main treatment for ABPA; although the long-term benefits are not clear, and their many side eff …
Exercise versus airway clearance techniques for people with cystic fibrosis.
Heinz KD, Walsh A, Southern KW, Johnstone Z, Regan KH. Heinz KD, et al. Among authors: southern kw. Cochrane Database Syst Rev. 2022 Jun 22;6(6):CD013285. doi: 10.1002/14651858.CD013285.pub2. Cochrane Database Syst Rev. 2022. PMID: 35731672 Free PMC article. Review.
BACKGROUND: There are many accepted airway clearance techniques (ACTs) for managing the respiratory health of people with cystic fibrosis (CF); none of which demonstrate superiority. Other Cochrane Reviews have reported short-term effects related to mucus transport, but no …
BACKGROUND: There are many accepted airway clearance techniques (ACTs) for managing the respiratory health of people with cystic fibrosis (C …
Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del).
Southern KW, Murphy J, Sinha IP, Nevitt SJ. Southern KW, et al. Cochrane Database Syst Rev. 2020 Dec 17;12(12):CD010966. doi: 10.1002/14651858.CD010966.pub3. Cochrane Database Syst Rev. 2020. PMID: 33331662 Free PMC article. Updated.
Lumacaftor-ivacaftor was associated with an increase in early transient shortness of breath and longer-term increases in blood pressure (not observed for tezacaftor-ivacaftor). ...In this population, lumacaftor-ivacaftor had an important impact on respiratory function with …
Lumacaftor-ivacaftor was associated with an increase in early transient shortness of breath and longer-term increases in blood pressu …
Correctors (specific therapies for class II CFTR mutations) for cystic fibrosis.
Southern KW, Patel S, Sinha IP, Nevitt SJ. Southern KW, et al. Cochrane Database Syst Rev. 2018 Aug 2;8(8):CD010966. doi: 10.1002/14651858.CD010966.pub2. Cochrane Database Syst Rev. 2018. PMID: 30070364 Free PMC article. Updated. Review.
Lumacaftor-ivacaftor is associated with an increase in early transient shortness of breath and longer-term increases in blood pressure (high-quality evidence). These adverse effects were not observed for tezacaftor-ivacaftor. ...In this age group, lumacaftor-ivacaftor had …
Lumacaftor-ivacaftor is associated with an increase in early transient shortness of breath and longer-term increases in blood pressur …
Cystic Fibrosis Transmembrane Conductance Regulator-Related Metabolic Syndrome and Cystic Fibrosis Screen Positive, Inconclusive Diagnosis.
Ren CL, Borowitz DS, Gonska T, Howenstine MS, Levy H, Massie J, Milla C, Munck A, Southern KW. Ren CL, et al. Among authors: southern kw. J Pediatr. 2017 Feb;181S:S45-S51.e1. doi: 10.1016/j.jpeds.2016.09.066. J Pediatr. 2017. PMID: 28129812
However, between 10% and 20% of asymptomatic infants can develop clinical features concerning for CF, such as a respiratory culture positive for Pseudomonas aeruginosa. Most studies have only reported short-term outcomes in the first 1-3 years of life; the long-term
However, between 10% and 20% of asymptomatic infants can develop clinical features concerning for CF, such as a respiratory culture positive …
Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis.
Elphick HE, Southern KW. Elphick HE, et al. Among authors: southern kw. Cochrane Database Syst Rev. 2016 Nov 8;11(11):CD002204. doi: 10.1002/14651858.CD002204.pub4. Cochrane Database Syst Rev. 2016. PMID: 27820955 Free PMC article. Updated. Review.
High doses of corticosteroids are the main treatment for ABPA; although the long-term benefits are not clear, their many side effects are well-documented. ...
High doses of corticosteroids are the main treatment for ABPA; although the long-term benefits are not clear, their many side effects …
Cystic fibrosis screen positive, inconclusive diagnosis.
Barben J, Southern KW. Barben J, et al. Among authors: southern kw. Curr Opin Pulm Med. 2016 Nov;22(6):617-22. doi: 10.1097/MCP.0000000000000314. Curr Opin Pulm Med. 2016. PMID: 27583671 Review.
According to recent studies, the majority of CRMS/CFSPID infants will remain well and have no long-term health implications. CRMS/CFSPID infants are at risk of developing CFTR-related disorder or atypical CF, with clinical features of CF but normal or intermediate sweat ch …
According to recent studies, the majority of CRMS/CFSPID infants will remain well and have no long-term health implications. CRMS/CFS …
27 results