Publications
2024
ECFS standards of care on CFTR-related disorders: Identification and care of the disorders.
Simmonds, N. J., Southern, K. W., De Wachter, E., De Boeck, K., Bodewes, F., Mainz, J. G., . . . ECFS Diagnostic Network Working Group. (2024). ECFS standards of care on CFTR-related disorders: Identification and care of the disorders.. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 23(4), 590-602. doi:10.1016/j.jcf.2024.03.008
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, N. J., Munck, A., Naehrlich, L., . . . ECFS Diagnostic Network Working Group. (2024). ECFS standards of care on CFTR-related disorders: Towards a comprehensive program for affected individuals.. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 23(3), 388-397. doi:10.1016/j.jcf.2024.01.012
Standards for the care of people with cystic fibrosis (CF); Planning for a longer life.
Gramegna, A., Addy, C., Allen, L., Bakkeheim, E., Brown, C., Daniels, T., . . . Southern, K. W. (2024). Standards for the care of people with cystic fibrosis (CF); Planning for a longer life.. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 23(3), 375-387. doi:10.1016/j.jcf.2024.05.007
Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues.
Burgel, P. -R., Southern, K. W., Addy, C., Battezzati, A., Berry, C., Bouchara, J. -P., . . . Middleton, P. G. (2024). Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues.. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, S1569-1993(24)00005-5. doi:10.1016/j.jcf.2024.01.005
Letter to the editor: Risk of false newborn screening after intra-uterine exposure to ETI.
De Wachter, E., Davies, J. C., Simmonds, N. J., Castellani, C., de Winter-de Groot, K. M., Munck, A., . . . Barben, J. (2024). Letter to the editor: Risk of false newborn screening after intra-uterine exposure to ETI.. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 23(1), 176-177. doi:10.1016/j.jcf.2023.07.003
2023
Standards for the care of people with cystic fibrosis; establishing and maintaining health.
Southern, K., Addy, C., Bell, S., Bevan, A., Borawska, U., Brown, C., . . . van Koningsbruggen-Rietschel, S. (2023). Standards for the care of people with cystic fibrosis; establishing and maintaining health.. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, S1569-1993(23)01727-7. doi:10.1016/j.jcf.2023.12.002
CORRECTOR THERAPIES (WITH OR WITHOUT POTENTIATORS) FOR PEOPLE WITH CYSTIC FIBROSIS WITH CLASS II CFTR GENE VARIANTS (MOST COMMONLY F508DEL)
Heneohan, M., Southern, K. W., Murphy, J., Sinha, I. P., & Nevitt, S. J. (2022). CORRECTOR THERAPIES (WITH OR WITHOUT POTENTIATORS) FOR PEOPLE WITH CYSTIC FIBROSIS WITH CLASS II CFTR GENE VARIANTS (MOST COMMONLY F508DEL). In THORAX Vol. 77 (pp. A118-A119). Retrieved from https://www.webofscience.com/
Standards for the care of people with cystic fibrosis (CF).
Southern, K. W., Burgel, P. -R., Castellani, C., De Boeck, K., Davies, J. C., Dunlevy, F., . . . van Koningsbruggen-Rietschel, S. (2023). Standards for the care of people with cystic fibrosis (CF).. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 22(6), 961-962. doi:10.1016/j.jcf.2023.09.009
Standards for the care of people with cystic fibrosis (CF): A timely and accurate diagnosis.
Castellani, C., Simmonds, N. J., Barben, J., Addy, C., Bevan, A., Burgel, P. -R., . . . Southern, K. W. (2023). Standards for the care of people with cystic fibrosis (CF): A timely and accurate diagnosis.. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 22(6), 963-968. doi:10.1016/j.jcf.2023.09.008
Development and preliminary validation of the challenges of living with cystic fibrosis (CLCF) questionnaire: a 46-item measure of treatment burden for parent/carers of children with CF
Glasscoe, C., Hope, H. F., Lancaster, G. A., McCray, G., West, K., Patel, L., . . . Southern, K. W. (2023). Development and preliminary validation of the challenges of living with cystic fibrosis (CLCF) questionnaire: a 46-item measure of treatment burden for parent/carers of children with CF. PSYCHOLOGY & HEALTH, 38(10), 1309-1344. doi:10.1080/08870446.2021.2013483
Development and validation of a short form psychometric tool assessing the caregiving Challenge of Living with Cystic Fibrosis (CLCF-SF) in a child
McCray, G., Hope, H. F. F., Glasscoe, C., Hill, J., Quittner, A., Southern, K. W. W., & Lancaster, G. A. A. (2023). Development and validation of a short form psychometric tool assessing the caregiving Challenge of Living with Cystic Fibrosis (CLCF-SF) in a child. PSYCHOLOGY & HEALTH. doi:10.1080/08870446.2023.2231489
Raised Intracranial Pressure in Three Children with Cystic Fibrosis Receiving Elexacaftor-Tezacaftor-Ivacaftor Modulator Therapy.
Southern, K. W., Barben, J., Goldring, S., Kneen, R., Southward, S., Rajeev, Y., . . . Bush, A. (2023). Raised Intracranial Pressure in Three Children with Cystic Fibrosis Receiving Elexacaftor-Tezacaftor-Ivacaftor Modulator Therapy.. American journal of respiratory and critical care medicine. doi:10.1164/rccm.202303-0380le
European survey of newborn bloodspot screening for CF: opportunity to address challenges and improve performance
Munck, A., Berger, D., Southern, K. W., Carducci, C., Groot, K. M. D. W. -D., Gartner, S., . . . Barben, J. (2023). European survey of newborn bloodspot screening for CF: opportunity to address challenges and improve performance. JOURNAL OF CYSTIC FIBROSIS, 22(3), 484-495. doi:10.1016/j.jcf.2022.09.012
Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis.
Aslam, A. A., Sinha, I. P., & Southern, K. W. (2023). Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis.. The Cochrane database of systematic reviews, 3, CD012040. doi:10.1002/14651858.cd012040.pub3
Future therapies for cystic fibrosis.
Allen, L., Allen, L., Carr, S. B., Davies, G., Downey, D., Egan, M., . . . Davies, J. C. (2023). Future therapies for cystic fibrosis.. Nature communications, 14(1), 693. doi:10.1038/s41467-023-36244-2
Standards of care for <i>CFTR</i> variant-specific therapy (including modulators) for people with cystic fibrosi
Southern, K. W., Castellani, C., Lammertyn, E., Smyth, A., VanDevanter, D., van Koningsbruggen-Rietschel, S., . . . Duff, A. (2023). Standards of care for <i>CFTR</i> variant-specific therapy (including modulators) for people with cystic fibrosi. JOURNAL OF CYSTIC FIBROSIS, 22(1), 17-30. doi:10.1016/j.jcf.2022.10.002
2022
ECFS standards of care on CFTR-related disorders: Updated diagnostic criteria
Castellani, C., De Boeck, K., De Wachter, E., Sermet-Gaudelus, I., Simmonds, N. J., & Southern, K. W. (2022). ECFS standards of care on CFTR-related disorders: Updated diagnostic criteria. JOURNAL OF CYSTIC FIBROSIS, 21(6), 908-921. doi:10.1016/j.jcf.2022.09.011
Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis
Francis, N. Z., & Southern, K. W. (2022). Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis. COCHRANE DATABASE OF SYSTEMATIC REVIEWS, (9). doi:10.1002/14651858.CD002204.pub5
Co-designing Improved Communication of Newborn Bloodspot Screening Results to Parents: Mixed Methods Study.
Chudleigh, J., Shakespeare, L., Holder, P., Chinnery, H., Hack, G., Gill, T., . . . Moody, L. (2022). Co-designing Improved Communication of Newborn Bloodspot Screening Results to Parents: Mixed Methods Study.. Journal of participatory medicine, 14(1), e33485. doi:10.2196/33485
Co-designed strategies for delivery of positive newborn bloodspot screening results to parents: the ReSPoND mixed-methods study
Chudleigh, J., Holder, P., Fusco, F., Bonham, J. R., Bryon, M., Moody, L., . . . Southern, K. W. (2022). Co-designed strategies for delivery of positive newborn bloodspot screening results to parents: the ReSPoND mixed-methods study. Health and Social Care Delivery Research.
Exercise versus airway clearance techniques for people with cystic fibrosis
Heinz, K. D., Walsh, A., Southern, K. W., Johnstone, Z., & Regan, K. H. (2022). Exercise versus airway clearance techniques for people with cystic fibrosis. COCHRANE DATABASE OF SYSTEMATIC REVIEWS, (6). doi:10.1002/14651858.CD013285.pub2
Receiving results of uncertain clinical relevance from population genetic screening: systematic review & meta-synthesis of qualitative research
Johnson, F., Ulph, F., MacLeod, R., & Southern, K. W. (2022). Receiving results of uncertain clinical relevance from population genetic screening: systematic review & meta-synthesis of qualitative research. EUROPEAN JOURNAL OF HUMAN GENETICS, 30(5), 520-531. doi:10.1038/s41431-022-01054-5
Delivering Positive Newborn Screening Results: Cost Analysis of Existing Practice versus Innovative, Co-Designed Strategies from the ReSPoND Study
Fusco, F., Chudleigh, J., Holder, P., Bonham, J. R., Southern, K. W., Simpson, A., . . . Morris, S. (2022). Delivering Positive Newborn Screening Results: Cost Analysis of Existing Practice versus Innovative, Co-Designed Strategies from the ReSPoND Study. INTERNATIONAL JOURNAL OF NEONATAL SCREENING, 8(1). doi:10.3390/ijns8010019
Standards of care guidance for sweat testing; phase two of the ECFS quality improvement programme
Cirilli, N., Southern, K. W., Barben, J., Vermeulen, F., Munck, A., Wilschanski, M., . . . De Wachter, E. (2022). Standards of care guidance for sweat testing; phase two of the ECFS quality improvement programme. Journal of Cystic Fibrosis. doi:10.1016/j.jcf.2022.01.004
International Approaches to Management of CFTR-Related Metabolic Syndrome/Cystic Fibrosis Screen Positive, Inconclusive Diagnosis
Chudleigh, J., Barben, J., Ren, C. L., & Southern, K. W. (2022). International Approaches to Management of CFTR-Related Metabolic Syndrome/Cystic Fibrosis Screen Positive, Inconclusive Diagnosis. INTERNATIONAL JOURNAL OF NEONATAL SCREENING, 8(1). doi:10.3390/ijns8010005
2021
Achieving respiratory excellence in pre-school children with cystic fibrosis
Southern, K. W. (2021). Achieving respiratory excellence in pre-school children with cystic fibrosis. JOURNAL OF CYSTIC FIBROSIS, 20(6), 904-905. doi:10.1016/j.jcf.2021.09.016
Co-designing interventions for communicating positive newborn bloodspot screening results: use of online Experience-based Co-design (Preprint)
Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID)
Barben, J., Castellani, C., Munck, A., Davies, J. C., De Winter-de Groot, K. M., Gartner, S., . . . Southern, K. W. (2021). Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID). JOURNAL OF CYSTIC FIBROSIS, 20(5), 810-819. doi:10.1016/j.jcf.2020.11.006
COVID-19 and the mental health of children with respiratory illness
Sinha, I. P., Murphy, R., Biffin, H., Gait, L., Abrams, E. M., Lee, A. R., . . . Brown, A. (2021). COVID-19 and the mental health of children with respiratory illness. LANCET RESPIRATORY MEDICINE, 9(9), 952-954. doi:10.1016/S2213-2600(21)00319-2
Cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID)
Sinha, A., & Southern, K. W. (2021). Cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID). BREATHE, 17(3). doi:10.1183/20734735.0088-2021
Defining key outcomes to evaluate performance of newborn screening programmes for cystic fibrosis
Munck, A., Southern, K. W., Castellani, C., De Winter-de Groot, K. M., Gartner, S., Kashirskaya, N., . . . Barben, J. (2021). Defining key outcomes to evaluate performance of newborn screening programmes for cystic fibrosis. JOURNAL OF CYSTIC FIBROSIS, 20(5), 820-823. doi:10.1016/j.jcf.2021.02.006
Process evaluation of co-designed interventions to improve communication of positive newborn bloodspot screening results
Chudleigh, J., Holder, P., Moody, L., Simpson, A., Southern, K., Morris, S., . . . Olander, E. (2021). Process evaluation of co-designed interventions to improve communication of positive newborn bloodspot screening results. BMJ OPEN, 11(8). doi:10.1136/bmjopen-2021-050773
A systematic cochrane review of corrector therapies (with or without potentiators) for people with cystic fibrosis with class II gene variants (most commonly F508DEL).
Southern, K. W., Murphy, J., Sinha, I. P., & Nevitt, S. J. (n.d.). A systematic cochrane review of corrector therapies (with or without potentiators) for people with cystic fibrosis with class II gene variants (most commonly F508DEL).. Paediatric respiratory reviews. doi:10.1016/j.prrv.2021.03.001
A survey of the prevalence, management and outcome of infants with an inconclusive diagnosis following newborn bloodspot screening for cystic fibrosis (CRMS/CFSPID) in six Italian centres
Terlizzi, V., Claut, L., Tosco, A., Colombo, C., Raia, V., Fabrizzi, B., . . . Padoan, R. (2021). A survey of the prevalence, management and outcome of infants with an inconclusive diagnosis following newborn bloodspot screening for cystic fibrosis (CRMS/CFSPID) in six Italian centres. JOURNAL OF CYSTIC FIBROSIS, 20(5), 828-834. doi:10.1016/j.jcf.2021.03.015
Cystic fibrosis newborn screening: the importance of bloodspot sample quality
Doull, I., Course, C. W., Hanks, R. E., Southern, K. W., Forton, J. T., Thia, L. P., & Moat, S. J. (2021). Cystic fibrosis newborn screening: the importance of bloodspot sample quality. ARCHIVES OF DISEASE IN CHILDHOOD, 106(3), 253-257. doi:10.1136/archdischild-2020-318999
Transmission, adaptation and geographical spread of the<i> Pseudomonas</i><i> aeruginosa</i> Liverpool epidemic strain
Moore, M. P., Lamont, I. L., Williams, D., Paterson, S., Kukavica-Ibrulj, I., Tucker, N. P., . . . Winstanley, C. (2021). Transmission, adaptation and geographical spread of the<i> Pseudomonas</i><i> aeruginosa</i> Liverpool epidemic strain. MICROBIAL GENOMICS, 7(3). doi:10.1099/mgen.0.000511
2020
Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del)
Southern, K. W., Murphy, J., Sinha, I. P., & Nevitt, S. J. (2020). Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del). COCHRANE DATABASE OF SYSTEMATIC REVIEWS, (12). doi:10.1002/14651858.CD010966.pub3
Processing of positive newborn screening results: a qualitative exploration of current practice in England
Chudleigh, J., Chinnery, H., Holder, P., Carling, R. S., Southern, K., Olander, E., . . . Simpson, A. (2020). Processing of positive newborn screening results: a qualitative exploration of current practice in England. BMJ OPEN, 10(12). doi:10.1136/bmjopen-2020-044755
Cystic fibrosis newborn screening for R117H in England
Dafydd, C., Moat, S., Southern, K. W., & Doull, I. J. M. (2020). Cystic fibrosis newborn screening for R117H in England. In EUROPEAN RESPIRATORY JOURNAL Vol. 56. doi:10.1183/13993003.congress-2020.2762
Should the D1152H variant be included in cystic fibrosis newborn screening programmes?
Dafydd, C., Southern, K. W., Moat, S., & Doull, I. J. M. (2020). Should the D1152H variant be included in cystic fibrosis newborn screening programmes?. In EUROPEAN RESPIRATORY JOURNAL Vol. 56. doi:10.1183/13993003.congress-2020.2761
Why Do We Screen Newborn Infants for Cystic Fibrosis?
Barben, J., & Southern, K. W. (2020). Why Do We Screen Newborn Infants for Cystic Fibrosis?. International journal of neonatal screening, 6(3). doi:10.3390/ijns6030056
Constructing a Bioethical Framework to Evaluate and Optimise Newborn Bloodspot Screening for Cystic Fibrosis
Armstrong, R. E., Frith, L., Ulph, F. M., & Southern, K. W. (2020). Constructing a Bioethical Framework to Evaluate and Optimise Newborn Bloodspot Screening for Cystic Fibrosis. International Journal of Neonatal Screening, 6(2). doi:10.3390/ijns6020040
COVID-19 infection in children
Sinha, I. P., Harwood, R., Semple, M. G., Hawcutt, D. B., Thursfield, R., Narayan, O., . . . Southern, K. W. (2020). COVID-19 infection in children. LANCET RESPIRATORY MEDICINE, 8(5), 446-447. Retrieved from https://www.webofscience.com/
Impact of COVID-19 on people with cystic fibrosis
Colombo, C., Burgel, P. -R., Gartner, S., van Koningsbruggen-Rietschel, S., Naehrlich, L., Sermet-Gaudelus, I., & Southern, K. W. (2020). Impact of COVID-19 on people with cystic fibrosis. LANCET RESPIRATORY MEDICINE, 8(5), E35-E36. doi:10.1016/S2213-2600(20)30177-6
The future of cystic fibrosis care: a global perspective
Bell, S. C., Mall, M. A., Gutierrez, H., Macek, M., Madge, S., Davies, J. C., . . . Ratjen, F. (2020). The future of cystic fibrosis care: a global perspective. LANCET RESPIRATORY MEDICINE, 8(1), 65-124. doi:10.1016/S2213-2600(19)30337-6
2019
Impact of newborn screening on outcomes and social inequalities in cystic fibrosis: a UK CF registry-based study
Schlüter, D. K., Southern, K. W., Dryden, C., Diggle, P., & Taylor-Robinson, D. (2020). Impact of newborn screening on outcomes and social inequalities in cystic fibrosis: a UK CF registry-based study. Thorax, 75(02), 123-131. doi:10.1136/thoraxjnl-2019-213179
An open-label extension study of ivacaftor in children with CF and a <i>CFTR</i> gating mutation initiating treatment at age 2-5 years (KLIMB)
Rosenfeld, M., Cunningham, S., Harris, W. T., Lapey, A., Regelmann, W. E., Sawicki, G. S., . . . Davies, J. C. (2019). An open-label extension study of ivacaftor in children with CF and a <i>CFTR</i> gating mutation initiating treatment at age 2-5 years (KLIMB). JOURNAL OF CYSTIC FIBROSIS, 18(6), 838-843. doi:10.1016/j.jcf.2019.03.009
Inconclusive diagnosis after a positive newborn bloodspot screening result for cystic fibrosis; clarification of the harmonised international definition.
Southern, K. W., Barben, J., Gartner, S., Munck, A., Castellani, C., Mayell, S. J., . . . Farrell, P. M. (2019). Inconclusive diagnosis after a positive newborn bloodspot screening result for cystic fibrosis; clarification of the harmonised international definition. JOURNAL OF CYSTIC FIBROSIS, 18(6), 778-780. doi:10.1016/j.jcf.2019.04.010
Inhaled hypertonic saline for 3-6-year-olds with cystic fibrosis
Southern, K. W., & Sinha, I. P. (2019). Inhaled hypertonic saline for 3-6-year-olds with cystic fibrosis. LANCET RESPIRATORY MEDICINE, 7(9), 730-732. doi:10.1016/S2213-2600(19)30183-3
International approaches for delivery of positive newborn bloodspot screening results for CF
Chudleigh, J., Ren, C. L., Barben, J., & Southern, K. W. (2019). International approaches for delivery of positive newborn bloodspot screening results for CF. JOURNAL OF CYSTIC FIBROSIS, 18(5), 614-621. doi:10.1016/j.jcf.2019.04.004
Psychological Impact on Parents of an Inconclusive Diagnosis Following Newborn Bloodspot Screening for Cystic Fibrosis: A Qualitative Study
Johnson, F., Southern, K. W., & Ulph, F. (2019). Psychological Impact on Parents of an Inconclusive Diagnosis Following Newborn Bloodspot Screening for Cystic Fibrosis: A Qualitative Study. INTERNATIONAL JOURNAL OF NEONATAL SCREENING, 5(2). doi:10.3390/ijns5020023
Adherence to Aerosol Therapy in Young People With Cystic Fibrosis: Patient and Parent Perspectives Following Electronic Data Capture
O'Toole, D. P. H., Latchford, G. J., Duff, A. J. A., Ball, R., McCormack, P., McNamara, P. S., . . . Southern, K. W. (2019). Adherence to Aerosol Therapy in Young People With Cystic Fibrosis: Patient and Parent Perspectives Following Electronic Data Capture. QUALITATIVE HEALTH RESEARCH, 29(6), 846-856. doi:10.1177/1049732318805754
A systematic Cochrane Review of correctors (specific therapies for class II CFTR mutations) for cystic fibrosis [1]
Southern, K. W., Patel, S., Sinha, I. P., & Nevitt, S. J. (2019). A systematic Cochrane Review of correctors (specific therapies for class II CFTR mutations) for cystic fibrosis [1]. PAEDIATRIC RESPIRATORY REVIEWS, 30, 25-26. doi:10.1016/j.prrv.2019.01.003
Exercise versus airway clearance techniques for people with cystic fibrosis
Patterson, K. D., Walsh, A., McCormack, P., & Southern, K. W. (n.d.). Exercise versus airway clearance techniques for people with cystic fibrosis. Cochrane Database of Systematic Reviews. doi:10.1002/14651858.cd013285
The increasing challenge of genetic counseling for cystic fibrosis.
Foil, K. E., Powers, A., Raraigh, K. S., Wallis, K., Southern, K. W., & Salinas, D. (2019). The increasing challenge of genetic counseling for cystic fibrosis. JOURNAL OF CYSTIC FIBROSIS, 18(2), 167-174. doi:10.1016/j.jcf.2018.11.014
A systematic Cochrane Review of autogenic drainage (AD) for airway clearance in cystic fibrosis
McCormack, P., Burnham, P., & Southern, K. W. (2019). A systematic Cochrane Review of autogenic drainage (AD) for airway clearance in cystic fibrosis. In PAEDIATRIC RESPIRATORY REVIEWS Vol. 29 (pp. 23-24). doi:10.1016/j.prrv.2018.06.002
Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis
Skilton, M., Krishan, A., Patel, S., Sinha, I. P., & Southern, K. W. (2019). Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis. COCHRANE DATABASE OF SYSTEMATIC REVIEWS, 1-120. doi:10.1002/14651858.CD009841.pub3
Rethinking Strategies for Positive Newborn Screening Result (NBS plus ) 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., . . . Southern, K. (2019). Rethinking Strategies for Positive Newborn Screening Result (NBS plus ) Delivery (ReSPoND): a process evaluation of co-designing interventions to minimise impact on parental emotional well-being and stress. PILOT AND FEASIBILITY STUDIES, 5(1). doi:10.1186/s40814-019-0487-5
2018
SEROLOGICAL CLASSIFICATION OF ASPERGILLUS DISEASE IN CHILDHOOD
Akinyemi, H. A., Mangat, S., Southern, K. W., Bedford, C., & McNamara, P. S. (2018). SEROLOGICAL CLASSIFICATION OF ASPERGILLUS DISEASE IN CHILDHOOD. In THORAX Vol. 73 (pp. A86-A87). doi:10.1136/thorax-2018-212555.145
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, S. J., Knowles, Z. R., Southern, K. W., McCormack, P., Dawson, E. A., . . . Hanlon, C. (2018). A formative study exploring perceptions of physical activity and physical activity monitoring among children and young people with cystic fibrosis and health care professionals. BMC PEDIATRICS, 18. doi:10.1186/s12887-018-1301-x
THE IMPACT OF NEWBORN SCREENING ON OUTCOMES AND INEQUALITIES IN CYSTIC FIBROSIS: A UK REGISTRY BASED STUDY
Schluter, D. K., Dryden, C., Southern, K. W., Diggle, P. J., & Taylor-Robinson, D. (2018). THE IMPACT OF NEWBORN SCREENING ON OUTCOMES AND INEQUALITIES IN CYSTIC FIBROSIS: A UK REGISTRY BASED STUDY. In JOURNAL OF EPIDEMIOLOGY AND COMMUNITY HEALTH Vol. 72 (pp. A27). doi:10.1136/jech-2018-SSMabstracts.55
Correctors (specific therapies for class II CFTR mutations) for cystic fibrosis
Southern, K. W., Patel, S., Sinha, I. P., & Nevitt, S. J. (2018). Correctors (specific therapies for class II CFTR mutations) for cystic fibrosis. Cochrane Database of Systematic Reviews. doi:10.1002/14651858.CD010966.pub2
ECFS best practice guidelines: the 2018 revision
Castellani, C., Duff, A. J. A., Bell, S. C., Heijerman, H. G. M., Munck, A., Ratjen, F., . . . Drevinek, P. (2018). ECFS best practice guidelines: the 2018 revision. JOURNAL OF CYSTIC FIBROSIS, 17(2), 153-178. doi:10.1016/j.jcf.2018.02.006
2017
Autogenic drainage for airway clearance in cystic fibrosis
McCormack, P., Burnham, P., & Southern, K. W. (2017). Autogenic drainage for airway clearance in cystic fibrosis. COCHRANE DATABASE OF SYSTEMATIC REVIEWS, (10). doi:10.1002/14651858.CD009595.pub2
Real life practice of sweat testing in Europe
Cirilli, N., Southern, K. W., Buzzetti, R., Barben, J., Nahrlich, L., Munck, A., . . . Working, E. C. F. S. D. N. (2018). Real life practice of sweat testing in Europe. JOURNAL OF CYSTIC FIBROSIS, 17(3), 325-332. doi:10.1016/j.jcf.2017.09.002
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, K. W., & Batchelor, H. K. (2017). A mixed methods study of the administration of flucloxacillin oral liquid; identifying strategies to overcome administration issues of medicines with poor palatability. INTERNATIONAL JOURNAL OF PHARMACY PRACTICE, 25(5), 326-334. doi:10.1111/ijpp.12308
Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis
Aslam, A., Jahnke, N., Remmington, T., & Southern, K. W. (2017). Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis. PAEDIATRIC RESPIRATORY REVIEWS, 24, 32-34. doi:10.1016/j.prrv.2017.04.001
Investigating the variation in the incidence of new Pseudomonas aeruginosa infection between paediatric cystic fibrosis centres.
Gilchrist, F. J., Jones, A. M., Smyth, A. R., Southern, K. W., Webb, A. K., & Lenney, W. (2017). Investigating the variation in the incidence of new Pseudomonas aeruginosa infection between paediatric cystic fibrosis centres.. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 16(5), e14-e16. doi:10.1016/j.jcf.2017.06.006
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., . . . Southern, K. W. (2017). The expansion and performance of national newborn screening programmes for cystic fibrosis in Europe. JOURNAL OF CYSTIC FIBROSIS, 16(2), 207-213. doi:10.1016/j.jcf.2016.12.012
Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease.
Perry, L. A., Penny-Dimri, J. C., Aslam, A. A., Lee, T. W., & Southern, K. W. (2017). Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease.. Paediatric respiratory reviews, 22, 47-49. doi:10.1016/j.prrv.2016.10.005
Cystic Fibrosis Transmembrane Conductance Regulator-Related Metabolic Syndrome and Cystic Fibrosis Screen Positive, Inconclusive Diagnosis.
Ren, C. L., Borowitz, D. S., Gonska, T., Howenstine, M. S., Levy, H., Massie, J., . . . Southern, K. W. (2017). Cystic Fibrosis Transmembrane Conductance Regulator-Related Metabolic Syndrome and Cystic Fibrosis Screen Positive, Inconclusive Diagnosis. JOURNAL OF PEDIATRICS, 181, S45-+. doi:10.1016/j.jpeds.2016.09.066
Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation.
Farrell, P. M., White, T. B., Ren, C. L., Hempstead, S. E., Accurso, F., Derichs, N., . . . Sosnay, P. R. (2017). Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation. JOURNAL OF PEDIATRICS, 181, S4-S15. doi:10.1016/j.jpeds.2016.09.064
Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis
Aslam, A. A., Higgins, C., Sinha, I. P., & Southern, K. W. (2017). Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis. Cochrane Database of Systematic Reviews. doi:10.1002/14651858.cd012040
Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis.
Aslam, A. A., Higgins, C., Sinha, I. P., & Southern, K. W. (2017). Ataluren and similar compounds (specific therapies for premature termination codon class I mutations) for cystic fibrosis.. The Cochrane database of systematic reviews, (1), 1-20. doi:10.1002/14651858.cd012040.pub2
Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease
Perry, L. A., Penny-Dimri, J. C., Aslam, A. A., Lee, T. W., & Southern, K. W. (2017). Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease. PAEDIATRIC RESPIRATORY REVIEWS, 22, 47-49. doi:10.1016/j.prrv.2016.10.005
2016
Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis
Elphick, H. E., & Southern, K. W. (2016). Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis. COCHRANE DATABASE OF SYSTEMATIC REVIEWS, (11). doi:10.1002/14651858.CD002204.pub4
Cystic fibrosis screen positive, inconclusive diagnosis
Barben, J., & Southern, K. W. (2016). Cystic fibrosis screen positive, inconclusive diagnosis. CURRENT OPINION IN PULMONARY MEDICINE, 22(6), 617-622. doi:10.1097/MCP.0000000000000314
Pneumococcal vaccines for cystic fibrosis
Burgess, L., & Southern, K. W. (2016). Pneumococcal vaccines for cystic fibrosis. COCHRANE DATABASE OF SYSTEMATIC REVIEWS, (9). doi:10.1002/14651858.CD008865.pub4
Newborn screening for cystic fibrosis
Castellani, C., Massie, J., Sontag, M., & Southern, K. W. (2016). Newborn screening for cystic fibrosis. The Lancet Respiratory Medicine, 4(8), 653-661. doi:10.1016/S2213-2600(16)00053-9
Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease
Lee, T. W. R., Southern, K. W., Perry, L. A., Penny-Dimri, J. C., & Aslam, A. A. (2016). Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease. COCHRANE DATABASE OF SYSTEMATIC REVIEWS, (6). doi:10.1002/14651858.CD005599.pub5
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, J. C., Cunningham, S., Harris, W. T., Lapey, A., Regelmann, W. E., Sawicki, G. S., . . . Grp, K. I. W. I. S. (2016). 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. LANCET RESPIRATORY MEDICINE, 4(2), 107-115. doi:10.1016/S2213-2600(15)00545-7
2015
IVACAFTOR TREATMENT IN PRESCHOOL CHILDREN WITH CYSTIC FIBROSIS AND A CFTR GATING MUTATION: EXTENDED EVALUATION
Davies, J. C., Cunningham, S., Southern, K. W., Robertson, S., Green, Y., Cooke, J., . . . Rosenfeld, M. (2015). IVACAFTOR TREATMENT IN PRESCHOOL CHILDREN WITH CYSTIC FIBROSIS AND A CFTR GATING MUTATION: EXTENDED EVALUATION. In THORAX Vol. 70 (pp. A14). doi:10.1136/thoraxjnl-2015-207770.24
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, S. J., Winters, V., Shawcross, A., Derichs, N., Parad, R., . . . Southern, K. W. (2015). Cystic Fibrosis Screen Positive, Inconclusive Diagnosis (CFSPID): A new designation and management recommendations for infants with an inconclusive diagnosis following newborn screening. Journal of Cystic Fibrosis, 14(6), 706-713. doi:10.1016/j.jcf.2015.01.001
Exhaled breath hydrogen cyanide as a marker of early <i>Pseudomonas aeruginosa</i> infection in children with cystic fibrosis.
Gilchrist, F. J., Belcher, J., Jones, A. M., Smith, D., Smyth, A. R., Southern, K. W., . . . Lenney, W. (2015). Exhaled breath hydrogen cyanide as a marker of early <i>Pseudomonas aeruginosa</i> infection in children with cystic fibrosis.. ERJ open research, 1(2), 44-2015. doi:10.1183/23120541.00044-2015
Social network analysis of <i>Pseudomonas aeruginosa</i> in cystic fibrosis
Taylor-Robinson, D. C., Southern, K. W., & Winstanley, C. (2015). Social network analysis of <i>Pseudomonas aeruginosa</i> in cystic fibrosis. LANCET RESPIRATORY MEDICINE, 3(8), 595-596. doi:10.1016/S2213-2600(15)00250-7
Topical nasal steroids for treating nasal polyposis in people with cystic fibrosis
Beer, H., Southern, K. W., & Swift, A. C. (2015). Topical nasal steroids for treating nasal polyposis in people with cystic fibrosis. COCHRANE DATABASE OF SYSTEMATIC REVIEWS, (6). doi:10.1002/14651858.CD008253.pub4
Potentiators for cystic fibrosis - targeting the underlying molecular defect
Patel, S., Sinha, I. P., Dwan, K., Echevarria, C., Schechter, M., & Southern, K. W. (2015). Potentiators for cystic fibrosis - targeting the underlying molecular defect. PAEDIATRIC RESPIRATORY REVIEWS, 16(3), 162-164. doi:10.1016/j.prrv.2015.04.003
Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis
Patel, S., Sinha, I. P., Dwan, K., Echevarria, C., Schechter, M., & Southern, K. W. (2015). Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis. COCHRANE DATABASE OF SYSTEMATIC REVIEWS, (03), 1-91. doi:10.1002/14651858.CD009841.pub2
PERFORMANCE OF THE UK NEWBORN SCREENING PROGRAMME FOR CF, WHICH INCORPORATES STRATEGIES TO IMPROVE POSITIVE PREDICTIVE VALUE AND REDUCE CARRIER RECOGNITION
Southern, K. W., Morgan, T., Kour, S., Scott, E., Coppinger, C., Cavanagh, C., . . . Price, J. (2015). PERFORMANCE OF THE UK NEWBORN SCREENING PROGRAMME FOR CF, WHICH INCORPORATES STRATEGIES TO IMPROVE POSITIVE PREDICTIVE VALUE AND REDUCE CARRIER RECOGNITION. PEDIATRIC PULMONOLOGY, 50, 382-383. Retrieved from https://www.webofscience.com/
2014
THE INCIDENCE OF NEW PSEUDOMONAS AERUGINOSA INFECTION IN CHILDREN WITH CYSTIC FIBROSIS
Gilchrist, F. J., Belcher, J., Jones, A. M., Smith, D., Smyth, A., Southern, K. W., . . . Lenney, W. (2014). THE INCIDENCE OF NEW PSEUDOMONAS AERUGINOSA INFECTION IN CHILDREN WITH CYSTIC FIBROSIS. In THORAX Vol. 69 (pp. A162-A163). doi:10.1136/thoraxjnl-2014-206260.326
Turnover of strains and intraclonal variation amongst <i>Pseudomonas aeruginosa</i> isolates from paediatric CF patients
Hall, A. J., Fothergill, J. L., McNamara, P. S., Southern, K. W., & Winstanley, C. (2014). Turnover of strains and intraclonal variation amongst <i>Pseudomonas aeruginosa</i> isolates from paediatric CF patients. DIAGNOSTIC MICROBIOLOGY AND INFECTIOUS DISEASE, 80(4), 324-326. doi:10.1016/j.diagmicrobio.2014.09.007
Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis
Elphick, H. E., & Southern, K. W. (2014). Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis. COCHRANE DATABASE OF SYSTEMATIC REVIEWS, (11). doi:10.1002/14651858.CD002204.pub3
Pneumococcal vaccines for cystic fibrosis
Burgess, L., & Southern, K. W. (2014). Pneumococcal vaccines for cystic fibrosis. COCHRANE DATABASE OF SYSTEMATIC REVIEWS, (8). doi:10.1002/14651858.CD008865.pub3
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, D. R., Saint, G. L., Bricio-Moreno, L., Fothergill, J., Southern, K. W., Winstanley, C., . . . Flanagan, B. F. (2014). THE B LYMPHOCYTE DIFFERENTIATION FACTOR (BAFF) IS EXPRESSED IN THE AIRWAYS OF CHILDREN WITH CF AND IN LUNGS OF MICE INFECTED WITH PSEUDOMONAS AERUGINOSA. PLoS One, 9(5). doi:10.1371/journal.pone.0095892.
European Cystic Fibrosis Society Standards of Care: Best Practice guidelines
Smyth, A. R., Bell, S. C., Bojcin, S., Bryon, M., Duff, A., Flume, P., . . . Wolfe, S. (2014). European Cystic Fibrosis Society Standards of Care: Best Practice guidelines. JOURNAL OF CYSTIC FIBROSIS, 13(Supplement 1), S23-S42. doi:10.1016/j.jcf.2014.03.010
Sodium channel blockers for cystic fibrosis.
Burrows, E. F., Southern, K. W., & Noone, P. G. (2014). Sodium channel blockers for cystic fibrosis.. The Cochrane database of systematic reviews, (4), CD005087. doi:10.1002/14651858.cd005087.pub4
Considering consent: a structural equation modelling analysis of factors influencing decisional quality when accepting newborn screening
Nicholls, S. G., & Southern, K. W. (2014). Considering consent: a structural equation modelling analysis of factors influencing decisional quality when accepting newborn screening. JOURNAL OF INHERITED METABOLIC DISEASE, 37(2), 197-205. doi:10.1007/s10545-013-9651-x
Correctors (specific therapies for class II CFTR mutations) for cystic fibrosis
Sinha, I. P., Southern, K. W., Dwan, K., & Patel, S. (n.d.). Correctors (specific therapies for class II CFTR mutations) for cystic fibrosis. Cochrane Database of Systematic Reviews. doi:10.1002/14651858.cd010966
Gastrointestinal surgery in cystic fibrosis: A 20-year review
Farrelly, P. J., Charlesworth, C., Lee, S., Southern, K. W., & Baillie, C. T. (2014). Gastrointestinal surgery in cystic fibrosis: A 20-year review. JOURNAL OF PEDIATRIC SURGERY, 49(2), 280-283. doi:10.1016/j.jpedsurg.2013.11.038
Newborn screening for cystic fibrosis: opportunities and remaining challenges
Mehta, A., Sommerburg, O., & Southern, K. W. (2014). Newborn screening for cystic fibrosis: opportunities and remaining challenges. CYSTIC FIBROSIS, (64), 65-76. doi:10.1183/1025448x.10008913
The challenge of improving outcomes for patients with CF sinonasal disease
VanDevanter, D. R., & Southern, K. W. (2014). The challenge of improving outcomes for patients with CF sinonasal disease. JOURNAL OF CYSTIC FIBROSIS, 13(4), 361-362. doi:10.1016/j.jcf.2014.05.014
Variations in inflammation-related genes may be associated with childhood febrile seizure susceptibility
Emsley, H. C. A., Appleton, R. E., Whitmore, C. L., Jury, F., Lamb, J. A., Martin, J. E., . . . Allan, S. M. (2014). Variations in inflammation-related genes may be associated with childhood febrile seizure susceptibility. SEIZURE-EUROPEAN JOURNAL OF EPILEPSY, 23(6), 457-461. doi:10.1016/j.seizure.2014.03.006
2013
Comparison of real time diagnostic chemistries to detect <i>Pseudomonas aeruginosa</i> in respiratory samples from cystic fibrosis patients
Fothergill, J. L., Ledson, M. J., Walshaw, M. J., McNamara, P. S., Southern, K. W., & Winstanley, C. (2013). Comparison of real time diagnostic chemistries to detect <i>Pseudomonas aeruginosa</i> in respiratory samples from cystic fibrosis patients. JOURNAL OF CYSTIC FIBROSIS, 12(6), 675-681. doi:10.1016/j.jcf.2013.04.007
Abnormal tracheal smooth muscle function in the CF mouse.
Wallace, H. L., Southern, K. W., Connell, M. G., Wray, S., & Burdyga, T. (2013). Abnormal tracheal smooth muscle function in the CF mouse.. Physiological reports, 1(6), e00138. doi:10.1002/phy2.138
Adherence to nebulised therapies in adolescents with cystic fibrosis is best on week-days during school term-time
Ball, R., Southern, K. W., McCormack, P., Duff, A. J. A., Brownlee, K. G., & McNamara, P. S. (2013). Adherence to nebulised therapies in adolescents with cystic fibrosis is best on week-days during school term-time. JOURNAL OF CYSTIC FIBROSIS, 12(5), 440-444. doi:10.1016/j.jcf.2012.12.012
Intraclonal genetic diversity amongst cystic fibrosis and keratitis isolates of Pseudomonas aeruginosa.
Hall, A. J., Fothergill, J. L., Kaye, S. B., Neal, T. J., McNamara, P. S., Southern, K. W., & Winstanley, C. (2013). Intraclonal genetic diversity amongst cystic fibrosis and keratitis isolates of Pseudomonas aeruginosa.. Journal of medical microbiology, 62(Pt 2), 208-216. doi:10.1099/jmm.0.048272-0
After a long wait, two arrive, one after the other!
Southern, K. W., McCormack, P., & McNamara, P. S. (2013). After a long wait, two arrive, one after the other!. THORAX, 68(4), 311-312. doi:10.1136/thoraxjnl-2012-202891
CARERS COPE BETTER WITH THE DEMANDS OF CYSTIC FIBROSIS DURING TIMES OF ACUTE CARE; RESULTS FROM THE HOME INTRAVENOUS ANTIBIOTIC STUDY (H-IVAT)
Hope, H. F., Glasscoe, C., Nunn, T., Dale, C., Hill, J., & Southern, K. W. (2013). CARERS COPE BETTER WITH THE DEMANDS OF CYSTIC FIBROSIS DURING TIMES OF ACUTE CARE; RESULTS FROM THE HOME INTRAVENOUS ANTIBIOTIC STUDY (H-IVAT). In PEDIATRIC PULMONOLOGY Vol. 48 (pp. 438). Retrieved from https://www.webofscience.com/
Intraclonal genetic diversity amongst cystic fibrosis and keratitis isolates of Pseudomonas aeruginosa
Hall, A. J., Fothergill, J. L., Kaye, S. B., Neal, T. J., McNamara, P. S., Southern, K. W., & Winstanley, C. (2013). Intraclonal genetic diversity amongst cystic fibrosis and keratitis isolates of Pseudomonas aeruginosa. Journal of Medical Microbiology, 62, 208-216.
Parental Decision-Making and Acceptance of Newborn Bloodspot Screening: An Exploratory Study
Nicholls, S. G., & Southern, K. W. (2013). Parental Decision-Making and Acceptance of Newborn Bloodspot Screening: An Exploratory Study. PLOS ONE, 8(11). doi:10.1371/journal.pone.0079441
2012
New Nebulizer Technology to Monitor Adherence and Nebulizer Performance in Cystic Fibrosis
McCormack, P., Southern, K. W., & McNamara, P. S. (2012). New Nebulizer Technology to Monitor Adherence and Nebulizer Performance in Cystic Fibrosis. JOURNAL OF AEROSOL MEDICINE AND PULMONARY DRUG DELIVERY, 25(6), 307-309. doi:10.1089/jamp.2011.0934
Assessing the Liverpool Respiratory Symptom Questionnaire in children with cystic fibrosis
Trinick, R., Southern, K. W., & McNamara, P. S. (2012). Assessing the Liverpool Respiratory Symptom Questionnaire in children with cystic fibrosis. EUROPEAN RESPIRATORY JOURNAL, 39(4), 899-905. doi:10.1183/09031936.00070311
Mutation-specific therapies that potentiate cystic fibrosis transmembrane conductance regulator (CFTR) function in cystic fibrosis
Sinha, I. P., Southern, K. W., Dwan, K., Echevarria, C., & Schechter, M. (n.d.). Mutation-specific therapies that potentiate cystic fibrosis transmembrane conductance regulator (CFTR) function in cystic fibrosis. doi:10.1002/14651858.cd009841
2011
Poster Session Abstracts
Poster Session Abstracts (2011). Pediatric Pulmonology, 46(S34), 212-428. doi:10.1002/ppul.21583
A randomised controlled trial of breathing modes for adaptive aerosol delivery in children with cystic fibrosis
McCormack, P., McNamara, P. S., & Southern, K. W. (2011). A randomised controlled trial of breathing modes for adaptive aerosol delivery in children with cystic fibrosis. JOURNAL OF CYSTIC FIBROSIS, 10(5), 343-349. doi:10.1016/j.jcf.2011.04.006
Array tube genotyping of Pseudomonas aeruginosa isolates as a strategy for identifying potential emerging transmissible strains.
Hall, A. J., Fothergill, J. L., Southern, K. W., McNamara, P. S., Ledson, M. J., Walshaw, M. J., & Winstanley, C. (2011). Array tube genotyping of Pseudomonas aeruginosa isolates as a strategy for identifying potential emerging transmissible strains.. Journal of Cystic Fibrosis, 10 (S1, S32.
Comparison of real time diagnostic chemistries to detect early and chronic Pseudomonas aeruginosa colonisation in cystic fibrosis patients
Fothergill, J. L., Walshaw, M. J., Ledson, M. J., Southern, K. W., McNamara, P. S., & Winstanley, C. (2011). Comparison of real time diagnostic chemistries to detect early and chronic Pseudomonas aeruginosa colonisation in cystic fibrosis patients. Journal of Cystic Fibrosis, 10 (S1, S32.
2010
Guidelines on the early management of infants diagnosed with cystic fibrosis following newborn screening.
Sermet-Gaudelus, I., Mayell, S. J., Southern, K. W., & European Cystic Finrosis Society (ECFS), Neonatal Screening Working Group. (2010). Guidelines on the early management of infants diagnosed with cystic fibrosis following newborn screening.. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 9(5), 323-329. doi:10.1016/j.jcf.2010.04.008
Components of risk associated with home intravenous antibiotic therapy (H-IVAT) in the UK
Glasscoe, C., Quittner, A. L., Patel, L., Dale, C., Burrows, E. F., Hope, H. F., . . . Nunn, A. J. (2010). Components of risk associated with home intravenous antibiotic therapy (H-IVAT) in the UK. Journal of Cystic Fibrosis, 9, S105. doi:10.1016/s1569-1993(10)60408-0
Microarray genotyping of Pseudomonas aeruginosa isolates from CF units
Hall, A. J., Fothergill, J. L., Southern, K. W., McNamara, P. S., Ledson, M. J., Walshaw, M. J., & Winstanley, C. (2010). Microarray genotyping of Pseudomonas aeruginosa isolates from CF units. Journal of Cystic Fibrosis, 9, S30. doi:10.1016/s1569-1993(10)60115-4
2009
Phenotypic characterisation of patients with intermediate sweat chloride values: towards validation of the European diagnostic algorithm for cystic fibrosis.
Goubau, C., Wilschanski, M., Skalická, V., Lebecque, P., Southern, K. W., Sermet, I., . . . De Boeck, K. (2009). Phenotypic characterisation of patients with intermediate sweat chloride values: towards validation of the European diagnostic algorithm for cystic fibrosis.. Thorax, 64(8), 683-691. doi:10.1136/thx.2008.104752
Open adherence monitoring using routine data download from an adaptive aerosol delivery nebuliser in children with cystic fibrosis
McNamara, P. S., McCormack, P., McDonald, A. J., Heaf, L., & Southern, K. W. (2009). Open adherence monitoring using routine data download from an adaptive aerosol delivery nebuliser in children with cystic fibrosis. JOURNAL OF CYSTIC FIBROSIS, 8(4), 258-263. doi:10.1016/j.jcf.2009.04.006
Estimating risk associated with home-based intravenous antibiotic therapy (IVAT)
Glasscoe, C., Southern, K. W., Lancaster, G. A., Quittner, A. L., Burrows, E. F., Heaf, L. J., . . . Nunn, A. J. (2009). Estimating risk associated with home-based intravenous antibiotic therapy (IVAT). Journal of Cystic Fibrosis, 8, S95. doi:10.1016/s1569-1993(09)60368-4
European best practice guidelines for cystic fibrosis neonatal screening.
Castellani, C., Southern, K. W., Brownlee, K., Dankert Roelse, J., Duff, A., Farrell, M., . . . Elborn, S. (2009). European best practice guidelines for cystic fibrosis neonatal screening.. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 8(3), 153-173. doi:10.1016/j.jcf.2009.01.004
Newborn screening for cystic fibrosis.
Southern, K. W., Mérelle, M. M. E., Dankert-Roelse, J. E., & Nagelkerke, A. D. (2009). Newborn screening for cystic fibrosis.. The Cochrane database of systematic reviews, (1), CD001402. doi:10.1002/14651858.cd001402.pub2
A European consensus for the evaluation and management of infants with an equivocal diagnosis following newborn screening for cystic fibrosis.
Mayell, S. J., Munck, A., Craig, J. V., Sermet, I., Brownlee, K. G., Schwarz, M. J., . . . European Cystic Fibrosis Society Neonatal Screening Working Group. (2009). A European consensus for the evaluation and management of infants with an equivocal diagnosis following newborn screening for cystic fibrosis.. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 8(1), 71-78. doi:10.1016/j.jcf.2008.09.005
A reducing prevalence of the Liverpool Epidemic Strain of Pseudomonas aeruginosa in children attending the index paediatric clinic.
Morris, D., Fallon, L., Heaf, L., Burrows, E. F., Wallace, H., McNamara, P. S., . . . Southern, K. W. (2009). A reducing prevalence of the Liverpool Epidemic Strain of Pseudomonas aeruginosa in children attending the index paediatric clinic.. Ped Pulmonol, 32, 325.
A reducing prevalence of the Liverpool Epidemic Strain of Pseudomonas aeruginosa in children attending the index paediatric clinic.
Morris, D., Fallon, L., Heaf, L., Burrows, E. F., Wallace, H., McNamara, P. N., . . . Southern, K. W. (2009). A reducing prevalence of the Liverpool Epidemic Strain of Pseudomonas aeruginosa in children attending the index paediatric clinic.. Pediatric Pulmonology, S32, 325.
A reference interval for sweat chloride in infants aged between five and six weeks of age.
Jayaraj, R., Barton, P. V., Newland, P., Mountford, R., Shaw, N. J., McCarthy, E., . . . Southern, K. W. (2009). A reference interval for sweat chloride in infants aged between five and six weeks of age.. Annals of clinical biochemistry, 46(Pt 1), 73-78. doi:10.1258/acb.2008.008081
2008
Embryonic lung growth is normal in a cftr-knockout mouse model.
Wallace, H. L., Connell, M. G., Losty, P. D., Jesudason, E. C., & Southern, K. W. (2008). Embryonic lung growth is normal in a cftr-knockout mouse model.. Experimental lung research, 34(10), 717-727. doi:10.1080/01902140802389719
Airway ion transport impacts on disease presentation and severity in cystic fibrosis.
Leal, T., Fajac, I., Wallace, H. L., Lebecque, P., Lebacq, J., Hubert, D., . . . Southern, K. W. (2008). Airway ion transport impacts on disease presentation and severity in cystic fibrosis.. Clinical biochemistry, 41(10-11), 764-772. doi:10.1016/j.clinbiochem.2008.03.013
Challenge of Living with Cystic Fibrosis (CLCF): Psychometric evaluation
Glasscoe, C., Southern, K. W., Hope, H. F., Burrows, E., Heaf, L., Brownlee, K., . . . Quittner, A. L. (2008). Challenge of Living with Cystic Fibrosis (CLCF): Psychometric evaluation. In European CF Conference (pp. S96). Prague: Journal of Cystic Fibrosis.
2007
Less is best? The impact of urokinase as the first line management of empyema thoracis.
Khalil, B. A., Corbett, P. A., Jones, M. O., Baillie, C. T., Southern, K., Losty, P. D., & Kenny, S. E. (2007). Less is best? The impact of urokinase as the first line management of empyema thoracis.. Pediatric surgery international, 23(2), 129-133. doi:10.1007/s00383-006-1806-5
A survey of newborn screening for cystic fibrosis in Europe.
Southern, K. W., Munck, A., Pollitt, R., Travert, G., Zanolla, L., Dankert-Roelse, J., . . . ECFS CF Neonatal Screening Working Group. (2007). A survey of newborn screening for cystic fibrosis in Europe.. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 6(1), 57-65. doi:10.1016/j.jcf.2006.05.008
Achieving sustained compliance with nebulised therapies in a paediatric CF population
McCormack, P., MacDonald, A., Southern, K. S., & McNamara, P. S. (2007). Achieving sustained compliance with nebulised therapies in a paediatric CF population. Journal of Cystic Fibrosis, 6(June), S67.
Challenges of Living with Cystic Fibosis (CLCF): Psychometric evaluation of a new measure
Glasscoe, C., Southern, K. W., Smith, J. A., Lancaster, G. A., Hill, J., Burrows, E. F., . . . Quittner, A. L. (2007). Challenges of Living with Cystic Fibosis (CLCF): Psychometric evaluation of a new measure. Pediatric Pulmonology, 30(Supp), 403.
Electrical potential difference across the nasal epithelium is reduced in premature infants with chronic lung disease but is not associated with lower airway inflammation.
Gaillard, E. A., Shaw, N. J., Wallace, H. L., Vince, G., & Southern, K. W. (2007). Electrical potential difference across the nasal epithelium is reduced in premature infants with chronic lung disease but is not associated with lower airway inflammation.. Pediatric research, 61(1), 77-82. doi:10.1203/01.pdr.0000250035.10339.ce
Living with Cystic Fibrosis Questionnaire (CFLC-Q): Development and preliminary validation of a new measure of the challenge experienced by caregivers
Glasscoe, C., Quittner, A. L., Smith, J. A., Burrows, E. F., Heaf, L., Cottrell, J., . . . Lancaster, G. A. (2007). Living with Cystic Fibrosis Questionnaire (CFLC-Q): Development and preliminary validation of a new measure of the challenge experienced by caregivers. Journal of Cystic Fibrosis, 6(Supp 1), S76.
Significant findings on cranial CT scan after a first unprovoked seizure in children from North India.
Mathur, S., Southern, K., & Sharma, M. (2007). Significant findings on cranial CT scan after a first unprovoked seizure in children from North India.. Journal of Tropical Pediatrics, 56(6), 428-430.
2006
Pulmonary edema in meningococcal septicemia associated with reduced epithelial chloride transport.
Eisenhut, M., Wallace, H., Barton, P., Gaillard, E., Newland, P., Diver, M., & Southern, K. W. (2006). Pulmonary edema in meningococcal septicemia associated with reduced epithelial chloride transport.. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies, 7(2), 119-124. doi:10.1097/01.pcc.0000200944.98424.e0
A national survey of palivizumab for infants with cystic fibrosis
McCormick, J., & Southern, K. W. (2006). A national survey of palivizumab for infants with cystic fibrosis. Paediatric Pulmonology, Supp., 431.
A systematic (Cochrane) review of topical sodium channel blockers for cystic fibrosis lung disease
Burrows, E. F., Noone, P. G., & Southern, K. W. (2006). A systematic (Cochrane) review of topical sodium channel blockers for cystic fibrosis lung disease. Paediatric Pulmonology, Supp., 355.
Developing a tool to assess the impact on a family of caring for a child with cystic fibrosis
Glasscoe, C., Quittner, A. L., Evans, J., Burrows, E. F., Cottrell, J. J., Heaf, L., . . . Southern, K. W. (2006). Developing a tool to assess the impact on a family of caring for a child with cystic fibrosis. Pediatric Pulmonology, 29, 405-406.
Establishing paediatric patients on an Adaptive Aerosol Delivery (AAD) device
McCormick, P., McDonald, A., & Southern, K. W. (2006). Establishing paediatric patients on an Adaptive Aerosol Delivery (AAD) device. Journal of Cystic Fibrosis, 5 (Sup, S76 (348).
Reference range for sweat chloride in infants at 5-6 weeks of age
Jayaraj, R., Newland, P., Barton, P., Shaw, N. J., Isherwood, D., & Southern, K. W. (2006). Reference range for sweat chloride in infants at 5-6 weeks of age. Paediatric Pulmonology, Supp., 547.
The challenge of living with cystic fibrosis: A collective response from parents
Glasscoe, C., Smith, J. A., Hope, H. F., Jones, S., Cottrell, J. J., Burrows, E. F., . . . Southern, K. W. (2006). The challenge of living with cystic fibrosis: A collective response from parents. Pediatric Pulmonology, 29, 406.
Transition to adulthood for young people with CF
Hogan, J., Horgan, M., Southern, K., Cotrell, J., & Burrows, E. (2006). Transition to adulthood for young people with CF. Journal of Cystic Fibrosis, 5 (Sup, S87 (393.
Use of exacerbation score to determine intravenous antibiotic use for acute respiratory exacerbations in Cystic Fibrosis
Das, D., Bako, O. L., Javadpour, S., Heaf, L. J., Southern, K. W., & Heaf, D. P. (2006). Use of exacerbation score to determine intravenous antibiotic use for acute respiratory exacerbations in Cystic Fibrosis. Journal of Cystic Fibrosis, 5 (Sup, S20 (91).
Working in partnership with families to openly monitor aerosol therapy with an in-device data logger
McCormack, P., McDonald, A., Couriel, J. M., & Southern, K. W. (2006). Working in partnership with families to openly monitor aerosol therapy with an in-device data logger. Journal of Cystic Fibrosis, 5 (sup, S77 (352).
2005
Nasal potential difference increases with gestation in moderately preterm neonates on the first postnatal day.
Gaillard, E. A., Shaw, N. J., Wallace, H. L., Subhedar, N. V., & Southern, K. W. (2005). Nasal potential difference increases with gestation in moderately preterm neonates on the first postnatal day.. Archives of disease in childhood. Fetal and neonatal edition, 90(2), F172-F173. doi:10.1136/adc.2004.054494
THYROID FUNCTION DURING SEVERE MENINGOCOCCAL SEPTICAEMIA
Eisenhut, M., Newland, P., & Southern, K. W. (2005). THYROID FUNCTION DURING SEVERE MENINGOCOCCAL SEPTICAEMIA. Pediatric Critical Care Medicine, 6(2), 239. doi:10.1097/00130478-200503000-00051
A Survey on CF neonatal screening practice around Europe
Castellani, C., Dankert-Roelse, J., Munck, A., Pollitt, R., Southern, K. W., & Travert, G. (2005). A Survey on CF neonatal screening practice around Europe. Journal of Cystic Fibrosis, 4, S130.
Chloride channel dysfunction associated with pulmonary oedema in meningococcal septicaemia
Eisenhut, M., Wallace, H. L., Barton, P., Gaillard, E., Newland, P., & Southern, K. W. (2005). Chloride channel dysfunction associated with pulmonary oedema in meningococcal septicaemia. Current Paediatrics, 15, 190-191.
Does sputum induction (SI) increase recognition of an epidemic strain of Pseudomonas aeruginosa (PA)?
Wallace, H. L., McCormack, P., Jeffers, G., Heaf, D. P., Winstanley, C., Hart, C. A., & Southern, K. W. (2005). Does sputum induction (SI) increase recognition of an epidemic strain of Pseudomonas aeruginosa (PA)?. Journal of Cystic Fibrosis, 4, S42.
Once versus three-times daily regimens of tobramycin treatment for pulmonary exacerbations of cystic fibrosis--the TOPIC study: a randomised controlled trial.
A, S., & et.al., F. T. T. S. G. (2005). Once versus three-times daily regimens of tobramycin treatment for pulmonary exacerbations of cystic fibrosis--the TOPIC study: a randomised controlled trial.. Lancet, 365(9459), 573-578. Retrieved from http://dx.doi.org/10.1016/S0140-6736(05)17906-9
Sputum induction (SI) with 3% hypertonic saline in children
McCormack, P., Wallace, H. L., Heaf, D. P., & Southern, K. W. (2005). Sputum induction (SI) with 3% hypertonic saline in children. Journal of Cystic Fibrosis, 4, S100.
Suitability of the upper airway models obtained from MRI studies in simulating drug lung deposition from inhalers.
Ehtezazi, T., Southern, K. W., Allanson, D., Jenkinson, I., & O'Callaghan, C. (2005). Suitability of the upper airway models obtained from MRI studies in simulating drug lung deposition from inhalers.. Pharmaceutical research, 22(1), 166-170. doi:10.1007/s11095-004-9023-1
2004
Azithromycin for cystic fibrosis.
Southern, K. W., & Barker, P. M. (2004). Azithromycin for cystic fibrosis.. The European respiratory journal, 24(5), 834-838. doi:10.1183/09031936.04.00084304
Elevated sweat sodium associated with pulmonary oedema in meningococcal sepsis
Eisenhut, M., Sidaras, D., Barton, P., Newland, P., & Southern, K. W. (2004). Elevated sweat sodium associated with pulmonary oedema in meningococcal sepsis. Eur J Clin Invest, 34, 834-838.
2003
Nasal airway ion transport and lung function in young people with cystic fibrosis.
Wallace, H. L., Barker, P. M., & Southern, K. W. (2003). Nasal airway ion transport and lung function in young people with cystic fibrosis.. American journal of respiratory and critical care medicine, 168(5), 594-600. doi:10.1164/rccm.200211-1302oc
Airway ion transport on the first postnatal day in infants delivered vaginally or by elective cesarean section.
Gaillard, E. A., Shaw, N. J., Wallace, H. L., Subhedar, N. V., & Southern, K. W. (2003). Airway ion transport on the first postnatal day in infants delivered vaginally or by elective cesarean section.. Pediatric research, 54(1), 58-63. doi:10.1203/01.pdr.0000069842.09976.cb
Regulation of liquid secretion and absorption by the fetal and neonatal lung: basic mechanisms and role in neonatal lung disease
Barker, P. M., & Southern, K. W. (2003). Regulation of liquid secretion and absorption by the fetal and neonatal lung: basic mechanisms and role in neonatal lung disease. In R. A. Polin, W. W. Fox, & S. H. Abman (Eds.), Fetal and Neonatal Physiology (pp. 822-834). Penn: Saunders.
2002
Airway surface liquid recovered by lavage with perfluorocarbon liquid in cats.
Southern, K. W., Funkhouser, W. K., Kazachkova, I., Godfrey, V. L., Fordham, L. A., Gatzy, J. T., & Barker, P. M. (2002). Airway surface liquid recovered by lavage with perfluorocarbon liquid in cats.. European journal of clinical investigation, 32(12), 956-961. doi:10.1046/j.1365-2362.2002.01100.x
2001
A modified technique for measurement of nasal transepithelial potential difference in infants.
Southern, K. W., Noone, P. G., Bosworth, D. G., Legrys, V. A., Knowles, M. R., & Barker, P. M. (2001). A modified technique for measurement of nasal transepithelial potential difference in infants.. The Journal of pediatrics, 139(3), 353-358. doi:10.1067/mpd.2001.116278
2000
Macrolide antibiotics for cystic fibrosis.
Southern, K. W., Barker, P. M., & Solis, A. (2000). Macrolide antibiotics for cystic fibrosis.. The Cochrane database of systematic reviews, (3), CD002203. doi:10.1002/14651858.cd002203
1995
Generation and characterization of a delta F508 cystic fibrosis mouse model.
Colledge, W. H., Abella, B. S., Southern, K. W., Ratcliff, R., Jiang, C., Cheng, S. H., . . . Evans, M. J. (1995). Generation and characterization of a delta F508 cystic fibrosis mouse model.. Nature genetics, 10(4), 445-452. doi:10.1038/ng0895-445