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Items: 1 to 50 of 125

1.

The future of cystic fibrosis care: a global perspective.

Bell SC, Mall MA, Gutierrez H, Macek M, Madge S, Davies JC, Burgel PR, Tullis E, Castaños C, Castellani C, Byrnes CA, Cathcart F, Chotirmall SH, Cosgriff R, Eichler I, Fajac I, Goss CH, Drevinek P, Farrell PM, Gravelle AM, Havermans T, Mayer-Hamblett N, Kashirskaya N, Kerem E, Mathew JL, McKone EF, Naehrlich L, Nasr SZ, Oates GR, O'Neill C, Pypops U, Raraigh KS, Rowe SM, Southern KW, Sivam S, Stephenson AL, Zampoli M, Ratjen F.

Lancet Respir Med. 2019 Sep 27. pii: S2213-2600(19)30337-6. doi: 10.1016/S2213-2600(19)30337-6. [Epub ahead of print] Review.

PMID:
31570318
2.

Rethinking Strategies for Positive Newborn Screening Result (NBS+) Delivery (ReSPoND): a process evaluation of co-designing interventions to minimise impact on parental emotional well-being and stress.

Chudleigh J, Bonham J, Bryon M, Francis J, Moody L, Morris S, Simpson A, Ulph F, Southern K.

Pilot Feasibility Stud. 2019 Sep 4;5:108. doi: 10.1186/s40814-019-0487-5. eCollection 2019.

3.

Inhaled hypertonic saline for 3-6-year-olds with cystic fibrosis.

Southern KW, Sinha IP.

Lancet Respir Med. 2019 Sep;7(9):730-732. doi: 10.1016/S2213-2600(19)30183-3. Epub 2019 Jun 6. No abstract available.

PMID:
31178424
4.

A systematic Cochrane Review of correctors (specific therapies for class II CFTR mutations) for cystic fibrosis.

Southern KW, Patel S, Sinha IP, Nevitt SJ.

Paediatr Respir Rev. 2019 Apr;30:25-26. doi: 10.1016/j.prrv.2019.01.003. Epub 2019 Feb 7. Review. No abstract available.

PMID:
31128877
5.

An open-label extension study of ivacaftor in children with CF and a CFTR gating mutation initiating treatment at age 2-5 years (KLIMB).

Rosenfeld M, Cunningham S, Harris WT, Lapey A, Regelmann WE, Sawicki GS, Southern KW, Chilvers M, Higgins M, Tian S, Cooke J, Davies JC; KLIMB study group.

J Cyst Fibros. 2019 Apr 30. pii: S1569-1993(19)30061-X. doi: 10.1016/j.jcf.2019.03.009. [Epub ahead of print]

6.

International approaches for delivery of positive newborn bloodspot screening results for CF.

Chudleigh J, Ren CL, Barben J, Southern KW.

J Cyst Fibros. 2019 Sep;18(5):614-621. doi: 10.1016/j.jcf.2019.04.004. Epub 2019 Apr 30.

7.

Inconclusive diagnosis after a positive newborn bloodspot screening result for cystic fibrosis; clarification of the harmonised international definition.

Southern KW, Barben J, Gartner S, Munck A, Castellani C, Mayell SJ, Davies JC, Winters V, Murphy J, Salinas D, McColley SA, Ren CL, Farrell PM.

J Cyst Fibros. 2019 Apr 24. pii: S1569-1993(19)30070-0. doi: 10.1016/j.jcf.2019.04.010. [Epub ahead of print] No abstract available.

PMID:
31027826
8.

Aerosolized agents for airway clearance in cystic fibrosis.

Southern KW, Clancy JP, Ranganathan S.

Pediatr Pulmonol. 2019 Jun;54(6):858-864. doi: 10.1002/ppul.24306. Epub 2019 Mar 18.

PMID:
30884217
9.

Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis.

Skilton M, Krishan A, Patel S, Sinha IP, Southern KW.

Cochrane Database Syst Rev. 2019 Jan 7;1:CD009841. doi: 10.1002/14651858.CD009841.pub3.

PMID:
30616300
10.

The increasing challenge of genetic counseling for cystic fibrosis.

Foil KE, Powers A, Raraigh KS, Wallis K, Southern KW, Salinas D.

J Cyst Fibros. 2019 Mar;18(2):167-174. doi: 10.1016/j.jcf.2018.11.014. Epub 2018 Dec 7. Review.

PMID:
30527892
11.

Adherence to Aerosol Therapy in Young People With Cystic Fibrosis: Patient and Parent Perspectives Following Electronic Data Capture.

O'Toole DPH, Latchford GJ, Duff AJA, Ball R, McCormack P, McNamara PS, Brownlee KG, Southern KW.

Qual Health Res. 2019 May;29(6):846-856. doi: 10.1177/1049732318805754. Epub 2018 Oct 29.

PMID:
30371143
12.

A formative study exploring perceptions of physical activity and physical activity monitoring among children and young people with cystic fibrosis and health care professionals.

Shelley J, Fairclough SJ, Knowles ZR, Southern KW, McCormack P, Dawson EA, Graves LEF, Hanlon C.

BMC Pediatr. 2018 Oct 23;18(1):335. doi: 10.1186/s12887-018-1301-x.

13.

A systematic Cochrane Review of autogenic drainage (AD) for airway clearance in cystic fibrosis.

McCormack P, Burnham P, Southern KW.

Paediatr Respir Rev. 2019 Feb;29:23-24. doi: 10.1016/j.prrv.2018.06.002. Epub 2018 Jul 7. No abstract available.

PMID:
30145147
14.

Correctors (specific therapies for class II CFTR mutations) for cystic fibrosis.

Southern KW, Patel S, Sinha IP, Nevitt SJ.

Cochrane Database Syst Rev. 2018 Aug 2;8:CD010966. doi: 10.1002/14651858.CD010966.pub2. Review.

15.

Using Neural Networks with Routine Health Records to Identify Suicide Risk: Feasibility Study.

DelPozo-Banos M, John A, Petkov N, Berridge DM, Southern K, LLoyd K, Jones C, Spencer S, Travieso CM.

JMIR Ment Health. 2018 Jun 22;5(2):e10144. doi: 10.2196/10144.

16.

ECFS best practice guidelines: the 2018 revision.

Castellani C, Duff AJA, Bell SC, Heijerman HGM, Munck A, Ratjen F, Sermet-Gaudelus I, Southern KW, Barben J, Flume PA, Hodková P, Kashirskaya N, Kirszenbaum MN, Madge S, Oxley H, Plant B, Schwarzenberg SJ, Smyth AR, Taccetti G, Wagner TOF, Wolfe SP, Drevinek P.

J Cyst Fibros. 2018 Mar;17(2):153-178. doi: 10.1016/j.jcf.2018.02.006. Epub 2018 Mar 3. Review.

17.

Autogenic drainage for airway clearance in cystic fibrosis.

McCormack P, Burnham P, Southern KW.

Cochrane Database Syst Rev. 2017 Oct 6;10:CD009595. doi: 10.1002/14651858.CD009595.pub2. Review.

18.

Real life practice of sweat testing in Europe.

Cirilli N, Southern KW, Buzzetti R, Barben J, Nährlich L, Munck A, Wilschanski M, De Boeck K, Derichs N; of the ECFS Diagnostic Network Working Group.

J Cyst Fibros. 2017 Sep 27. pii: S1569-1993(17)30881-0. doi: 10.1016/j.jcf.2017.09.002. [Epub ahead of print]

PMID:
28964647
19.

Investigating the variation in the incidence of new Pseudomonas aeruginosa infection between paediatric cystic fibrosis centres.

Gilchrist FJ, Jones AM, Smyth AR, Southern KW, Webb AK, Lenney W.

J Cyst Fibros. 2017 Sep;16(5):e14-e16. doi: 10.1016/j.jcf.2017.06.006. Epub 2017 Jul 6. No abstract available.

20.

Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis.

Aslam A, Jahnke N, Remmington T, Southern KW.

Paediatr Respir Rev. 2017 Sep;24:32-34. doi: 10.1016/j.prrv.2017.04.001. Epub 2017 Apr 27. Review. No abstract available.

PMID:
28566196
21.

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.

J Pediatr. 2017 Feb;181S:S45-S51.e1. doi: 10.1016/j.jpeds.2016.09.066.

PMID:
28129812
22.

Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation.

Farrell PM, White TB, Ren CL, Hempstead SE, Accurso F, Derichs N, Howenstine M, McColley SA, Rock M, Rosenfeld M, Sermet-Gaudelus I, Southern KW, Marshall BC, Sosnay PR.

J Pediatr. 2017 Feb;181S:S4-S15.e1. doi: 10.1016/j.jpeds.2016.09.064. Erratum in: J Pediatr. 2017 May;184:243.

23.

Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis.

Aslam AA, Higgins C, Sinha IP, Southern KW.

Cochrane Database Syst Rev. 2017 Jan 19;1:CD012040. doi: 10.1002/14651858.CD012040.pub2. Review.

24.

The expansion and performance of national newborn screening programmes for cystic fibrosis in Europe.

Barben J, Castellani C, Dankert-Roelse J, Gartner S, Kashirskaya N, Linnane B, Mayell S, Munck A, Sands D, Sommerburg O, Pybus S, Winters V, Southern KW.

J Cyst Fibros. 2017 Mar;16(2):207-213. doi: 10.1016/j.jcf.2016.12.012. Epub 2016 Dec 30.

25.

Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease.

Perry LA, Penny-Dimri JC, Aslam AA, Lee TW, Southern KW.

Paediatr Respir Rev. 2017 Mar;22:47-49. doi: 10.1016/j.prrv.2016.10.005. Epub 2016 Nov 5. Review. No abstract available.

PMID:
27964951
26.

Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis.

Elphick HE, Southern KW.

Cochrane Database Syst Rev. 2016 Nov 8;11:CD002204. Review.

27.

Exhaled breath hydrogen cyanide as a marker of early Pseudomonas aeruginosa infection in children with cystic fibrosis.

Gilchrist FJ, Belcher J, Jones AM, Smith D, Smyth AR, Southern KW, Španěl P, Webb AK, Lenney W.

ERJ Open Res. 2015 Nov 16;1(2). pii: 00044-2015. eCollection 2015 Oct.

28.

Pneumococcal vaccines for cystic fibrosis.

Burgess L, Southern KW.

Cochrane Database Syst Rev. 2016 Sep 22;9:CD008865. doi: 10.1002/14651858.CD008865.pub4. Review.

29.

A mixed methods study of the administration of flucloxacillin oral liquid; identifying strategies to overcome administration issues of medicines with poor palatability.

Rouse C, Mistry P, Rayner O, Nickless J, Wan M, Southern KW, Batchelor HK.

Int J Pharm Pract. 2017 Oct;25(5):326-334. doi: 10.1111/ijpp.12308. Epub 2016 Sep 16.

PMID:
27633317
30.

Cystic fibrosis screen positive, inconclusive diagnosis.

Barben J, Southern KW.

Curr Opin Pulm Med. 2016 Nov;22(6):617-22. doi: 10.1097/MCP.0000000000000314. Review.

PMID:
27583671
31.

CHILDREN WITH CYSTIC FIBROSIS: UNDERSTANDING ISSUES RELATED TO ORAL ADMINISTRATION OF LIQUID FLUCLOXACILLIN.

Batchelor H, Rayner O, Nickless J, Wan M, Southern K, Rose C.

Arch Dis Child. 2016 Sep;101(9):e2. doi: 10.1136/archdischild-2016-311535.33.

PMID:
27540213
32.

Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease.

Lee TW, Southern KW, Perry LA, Penny-Dimri JC, Aslam AA.

Cochrane Database Syst Rev. 2016 Jun 17;(6):CD005599. doi: 10.1002/14651858.CD005599.pub5. Review.

PMID:
27314455
33.

Newborn screening for cystic fibrosis.

Castellani C, Massie J, Sontag M, Southern KW.

Lancet Respir Med. 2016 Aug;4(8):653-661. doi: 10.1016/S2213-2600(16)00053-9. Epub 2016 Apr 1. Review.

PMID:
27053341
34.

Safety, pharmacokinetics, and pharmacodynamics of ivacaftor in patients aged 2-5 years with cystic fibrosis and a CFTR gating mutation (KIWI): an open-label, single-arm study.

Davies JC, Cunningham S, Harris WT, Lapey A, Regelmann WE, Sawicki GS, Southern KW, Robertson S, Green Y, Cooke J, Rosenfeld M; KIWI Study Group.

Lancet Respir Med. 2016 Feb;4(2):107-15. doi: 10.1016/S2213-2600(15)00545-7. Epub 2016 Jan 21. Erratum in: Lancet Respir Med. 2016 Dec;4(12 ):e57.

35.

Social network analysis of Pseudomonas aeruginosa in cystic fibrosis.

Taylor-Robinson DC, Southern KW, Winstanley C.

Lancet Respir Med. 2015 Aug;3(8):595-6. doi: 10.1016/S2213-2600(15)00250-7. Epub 2015 Jul 22. No abstract available.

PMID:
26208995
36.

Topical nasal steroids for treating nasal polyposis in people with cystic fibrosis.

Beer H, Southern KW, Swift AC.

Cochrane Database Syst Rev. 2015 Jun 22;(6):CD008253. doi: 10.1002/14651858.CD008253.pub4. Review.

37.

Potentiators for cystic fibrosis - targeting the underlying molecular defect.

Patel S, Sinha IP, Dwan K, Echevarria C, Schechter M, Southern KW.

Paediatr Respir Rev. 2015 Jun;16(3):162-4. doi: 10.1016/j.prrv.2015.04.003. Epub 2015 Apr 20. Review. No abstract available.

PMID:
26002404
38.

Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis.

Patel S, Sinha IP, Dwan K, Echevarria C, Schechter M, Southern KW.

Cochrane Database Syst Rev. 2015 Mar 26;(3):CD009841. doi: 10.1002/14651858.CD009841.pub2. Review. Update in: Cochrane Database Syst Rev. 2019 Jan 07;1:CD009841.

PMID:
25811419
39.

Cystic Fibrosis Screen Positive, Inconclusive Diagnosis (CFSPID): A new designation and management recommendations for infants with an inconclusive diagnosis following newborn screening.

Munck A, Mayell SJ, Winters V, Shawcross A, Derichs N, Parad R, Barben J, Southern KW; ECFS Neonatal Screening Working Group.

J Cyst Fibros. 2015 Nov;14(6):706-13. doi: 10.1016/j.jcf.2015.01.001. Epub 2015 Jan 24.

40.

Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis.

Elphick HE, Southern KW.

Cochrane Database Syst Rev. 2014 Nov 28;(11):CD002204. doi: 10.1002/14651858.CD002204.pub3. Review. Update in: Cochrane Database Syst Rev. 2016 Nov 08;11:CD002204.

PMID:
25431975
41.

Turnover of strains and intraclonal variation amongst Pseudomonas aeruginosa isolates from paediatric CF patients.

Hall AJ, Fothergill JL, McNamara PS, Southern KW, Winstanley C.

Diagn Microbiol Infect Dis. 2014 Dec;80(4):324-6. doi: 10.1016/j.diagmicrobio.2014.09.007. Epub 2014 Sep 16.

PMID:
25284374
42.

Pneumococcal vaccines for cystic fibrosis.

Burgess L, Southern KW.

Cochrane Database Syst Rev. 2014 Aug 5;(8):CD008865. doi: 10.1002/14651858.CD008865.pub3. Review.

PMID:
25093421
43.

The challenge of improving outcomes for patients with CF sinonasal disease.

VanDevanter DR, Southern KW.

J Cyst Fibros. 2014 Jul;13(4):361-2. doi: 10.1016/j.jcf.2014.05.014. Epub 2014 Jun 2. No abstract available.

44.

European Cystic Fibrosis Society Standards of Care: Best Practice guidelines.

Smyth AR, Bell SC, Bojcin S, Bryon M, Duff A, Flume P, Kashirskaya N, Munck A, Ratjen F, Schwarzenberg SJ, Sermet-Gaudelus I, Southern KW, Taccetti G, Ullrich G, Wolfe S; European Cystic Fibrosis Society.

J Cyst Fibros. 2014 May;13 Suppl 1:S23-42. doi: 10.1016/j.jcf.2014.03.010. Review.

45.

The B lymphocyte differentiation factor (BAFF) is expressed in the airways of children with CF and in lungs of mice infected with Pseudomonas aeruginosa.

Neill DR, Saint GL, Bricio-Moreno L, Fothergill JL, Southern KW, Winstanley C, Christmas SE, Slupsky JR, McNamara PS, Kadioglu A, Flanagan BF.

PLoS One. 2014 May 21;9(5):e95892. doi: 10.1371/journal.pone.0095892. eCollection 2014.

46.

Sodium channel blockers for cystic fibrosis.

Burrows EF, Southern KW, Noone PG.

Cochrane Database Syst Rev. 2014 Apr 9;(4):CD005087. doi: 10.1002/14651858.CD005087.pub4. Review.

47.

Variations in inflammation-related genes may be associated with childhood febrile seizure susceptibility.

Emsley HC, Appleton RE, Whitmore CL, Jury F, Lamb JA, Martin JE, Ollier WE, de la Morandière KP, Southern KW, Allan SM.

Seizure. 2014 Jun;23(6):457-61. doi: 10.1016/j.seizure.2014.03.006. Epub 2014 Mar 18.

48.

Gastrointestinal surgery in cystic fibrosis: a 20-year review.

Farrelly PJ, Charlesworth C, Lee S, Southern KW, Baillie CT.

J Pediatr Surg. 2014 Feb;49(2):280-3. doi: 10.1016/j.jpedsurg.2013.11.038. Epub 2013 Nov 18.

PMID:
24528967
49.

Abnormal tracheal smooth muscle function in the CF mouse.

Wallace HL, Southern KW, Connell MG, Wray S, Burdyga T.

Physiol Rep. 2013 Nov;1(6):e00138. doi: 10.1002/phy2.138. Epub 2013 Nov 5.

50.

Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease.

Lee TW, Southern KW.

Cochrane Database Syst Rev. 2013 Nov 26;(11):CD005599. doi: 10.1002/14651858.CD005599.pub4. Review. Update in: Cochrane Database Syst Rev. 2016;(6):CD005599.

PMID:
24282073

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