U.S. flag

An official website of the United States government

NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.

National Guideline Alliance (UK). Cystic Fibrosis: Diagnosis and management. London: National Institute for Health and Care Excellence (NICE); 2017 Oct 25. (NICE Guideline, No. 78.)

Cover of Cystic Fibrosis

Cystic Fibrosis: Diagnosis and management.

Show details

12References

  1. Aaron et al., 2012

    Aaron, S. D., Vandemheen, K. L., Freitag, A., Pedder, L., Cameron, W., Lavoie, A., Paterson, N., Wilcox, P., Rabin, H., Tullis, E., Morrison, N., Ratjen, F., Treatment of Aspergillus fumigatus in patients with cystic fibrosis: a randomized, placebo-controlled pilot study, PLoS ONE, 7, e36077, 2012 [PMC free article: PMC3340414] [PubMed: 22563440]

  2. Ahmed & Mukherjee, 2016

    Ahmed, M. I., Mukherjee, S., Treatment for chronic methicillin-sensitive S aureus pulmonary infection in people with cystic fibrosis, Cochrane Database of Systematic Reviews, 3, CD011581, 2016 [PubMed: 27003247]

  3. Aitken et al., 2012

    Aitken, M. L., Bellon, G., De Boeck, K., Flume, P. A., Fox, H. G., Geller, D. E., Haarman, E. G., Hebestreit, H. U., Lapey, A., Schou, I. M., Zuckerman, J. B., Charlton, B., C. F. Investigators, Long-term inhaled dry powder mannitol in cystic fibrosis: an international randomized study, American Journal of Respiratory & Critical Care Medicine, 185, 645–52, 2012 [PubMed: 22198974]

  4. Al-Yateem, 2012

    Al-Yateem, N., Child to adult: transitional care for young adults with cystic fibrosis, British Journal of Nursing, 21, 850–4, 2012 [PubMed: 23252167]

  5. Al-Yateem, 2013

    Al-Yateem, N., Guidelines for the transition from child to adult cystic fibrosis care, Nursing Children and Young People, 25, 29–34, 2013 [PubMed: 23988074]

  6. Amin et al., 2010

    Amin, R., Subbarao, P., Jabar, A., Balkovec, S., Jensen, R., Kerrigan, S., Gustafsson, P., Ratjen, F., Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function, Thorax, 65, 379–83, 2010 [PubMed: 20435858]

  7. Amin et al., 2011

    Amin, R., Subbarao, P., Lou, W., Jabar, A., Balkovec, S., Jensen, R., Kerrigan, S., Gustafsson, P., Ratjen, F., The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis, European Respiratory Journal, 37, 806–812, 2011 [PubMed: 20693248]

  8. Angst & Deatrick, 1996

    Angst, D. B., Deatrick, J. A., Involvement in health care decisions: parents and children with chronic illness, Journal of Family Nursing, 2, 174–194, 1996

  9. Anyanwu et al., 2001

    Anyanwu, A., McGuire, A., Rogers, C., Murday, A., Assessment of quality of life in lung transplantation using a simple generic tool, Thorax, 56, 218–222, 2001 [PMC free article: PMC1758767] [PubMed: 11182015]

  10. Anyanwu et al., 2002

    Anyanwu, A. C., McGuire, A., Rogers, C. A., Murday, A. J., An economic evaluation of lung transplantation, Journal of Thoracic and Cardiovascular Surgery, 123, 411–8; discussion 418–20, 2002 [PubMed: 11882810]

  11. Assael et al., 2013

    Assael, B. M., Pressler, T., Bilton, D., Fayon, M., Fischer, R., Chiron, R., LaRosa, M., Knoop, C., McElvaney, N., Lewis, S. A., Bresnik, M., Montgomery, A. B., Oermann, C. M., Azli, Active Comparator Study Group, Inhaled aztreonam lysine vs. inhaled tobramycin in cystic fibrosis: a comparative efficacy trial, Journal of Cystic Fibrosis, 12, 130–40, 2013 [PubMed: 22985692]

  12. Auerbach et al., 1985

    Auerbach, H. S., Williams, M., Kirkpatrick, J. A., Colten, H. R., Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis, Lancet, 2, 686–8, 1985 [PubMed: 2863676]

  13. Bagnasco et al., 2013

    Bagnasco, A., Petralia, P., Furnari, S., Ghio, S., Calza, S., Sasso, L., Paediatric nurses’ perception of the child-family dyad’s autonomy in managing a chronic disease situation: the experience of an Italian paediatric department, Journal of Preventive Medicine & Hygiene, 54, 124–9, 2013 [PMC free article: PMC4718381] [PubMed: 24396995]

  14. Baker et al., 2016

    Baker, J. F., Putman, M. S., Herlyn, K., Tillotson, A. P., Finkelstein, J. S., Merkel, P. A., Body composition, lung function, and prevalent and progressive bone deficits among adults with cystic fibrosis, Joint, Bone, Spine: Revue du Rhumatisme, 83, 207–11, 2016 [PubMed: 26677995]

  15. Balaguer & Gonzalez de Dios, 2015

    Balaguer, Albert, Gonzalez de Dios, Javier, Home versus hospital intravenous antibiotic therapy for cystic fibrosis, Cochrane Database of Systematic Reviews, 12, CD00191,7 2015 [PMC free article: PMC6481823] [PubMed: 26671062]

  16. Balfour-Lynn & Welch, 2016

    Balfour-Lynn, I. M., Welch, Karen, Inhaled corticosteroids for cystic fibrosis, Cochrane Database of Systematic Reviews, 8, CD001915, 2016 [PubMed: 27552284]

  17. Balfour-Lynn et al., 1997

    Balfour-Lynn, I. M., Klein, N. J., Dinwiddie, R., Randomised controlled trial of inhaled corticosteroids (fluticasone propionate) in cystic fibrosis, Archives of Disease in Childhood, 77, 124–30, 1997 [PMC free article: PMC1717269] [PubMed: 9301350]

  18. Balfour-Lynn et al., 2006

    Balfour-Lynn, I. M., Lees, B., Hall, P., Phillips, G., Khan, M., Flather, M., Elborn, J. S., Cf Wise Investigators, Multicenter randomized controlled trial of withdrawal of inhaled corticosteroids in cystic fibrosis, American Journal of Respiratory & Critical Care Medicine, 173, 1356–62, 2006 [PubMed: 16556691]

  19. Ballmann & von der Hardt, 1998

    Ballmann, M., von der Hardt, H., Hypertonic saline and recombinant human DNase: a randomised cross-over pilot study in patients with cystic fibrosis, 22nd European Cystic Fibrosis Conference, 1998, [Abstract] [PubMed: 15463808]

  20. Ballmann & von der Hardt, 2002

    Ballmann, M., von der Hardt, H., Hypertonic saline and recombinant human DNase: a randomised cross-over pilot study in patients with cystic fibrosis, Journal of Cystic Fibrosis, 1, 35–7, 2002 [PubMed: 15463808]

  21. Barker et al., 2012

    Barker, D., Driscoll, K., Modi, A., Light, M., Quittner, A., Supporting cystic fibrosis disease management during adolescence: The role of family and friends, Child: care, health and development, 38, 497–504, 2012 [PMC free article: PMC3479957] [PubMed: 21771002]

  22. Beardsmore et al., 1994

    Beardsmore, C.S., Thompson, J.R., Williams, A., McArdle, E.K., Gregory, G.A., Weaver, L.T., Simpson, H., Pulmonary function in infants with cystic fibrosis: the effect of antibiotic treatment, Archives of Disease in Childhood, 71, 133–137, 1994 [PMC free article: PMC1029944] [PubMed: 7944533]

  23. Beaudoin et al., 2016

    Beaudoin, N., Bouvet, G. F., Coriati, A., Rabasa-Lhoret, R., Berthiaume, Y., Combined Exercise Training Improves Glycemic Control in Adult With Cystic Fibrosis, Medicine and Science in Sports and Exercise, 2016 [PubMed: 27669451]

  24. Begley, 2013

    Begley, T., Transition to adult care for young people with long-term conditions, British Journal of Nursing, 22, 506, 508–11, 2013 [PubMed: 23752622]

  25. Beker et al., 1994

    Beker, L. T., Fink, R. J., Shamsa, F. H., Chaney, H. R., Kluft, J., Evans, E., Schidlow, D. V., Comparison of weight-based dosages of enteric-coated microtablet enzyme preparations in patients with cystic fibrosis, Journal of Pediatric Gastroenterology & Nutrition, 19, 191–7, 1994 [PubMed: 7815242]

  26. Bell et al., 2011

    Bell, S. C., Bye, P. T., Cooper, P. J., Martin, A. J., McKay, K. O., Robinson, P. J., Ryan, G. F., Sims, G. C., Cystic fibrosis in Australia, 2009: results from a data registry, Medical Journal of Australia, 195, 396–400, 2011 [PubMed: 21978347]

  27. Beresford & Sloper, 2003

    Beresford, B. A., Sloper, P., Chronically ill adolescents’ experiences of communicating with doctors: a qualitative study, Journal of Adolescent Health, 33, 172–9, 2003 [PubMed: 12944007]

  28. Bhudhikanok et al., 1998

    Bhudhikanok, G. S., Wang, M. C., Marcus, R., Harkins, A., Moss, R. B., Bachrach, L. K., Bone acquisition and loss in children and adults with cystic fibrosis: a longitudinal study, Journal of Pediatrics, 133, 18–27, 1998 [PubMed: 9672505]

  29. Bilton et al., 2011

    Bilton, D., Robinson, P., Cooper, P., Gallagher, C. G., Kolbe, J., Fox, H., Jaques, A., Charlton, B., C. F. Study Investigators, Inhaled dry powder mannitol in cystic fibrosis: an efficacy and safety study, European Respiratory Journal, 38, 1071–80, 2011 [PubMed: 21478216]

  30. Blumer et al., 2005

    Blumer, J. L., Saiman, L., Konstan, M. W., Melnick, D., The efficacy and safety of meropenem and tobramycin vs ceftazidime and tobramycin in the treatment of acute pulmonary exacerbations in patients with cystic fibrosis, Chest, 128, 2336–46, 2005 [PubMed: 16236892]

  31. Bond et al., 2007

    Bond, Mary, Mealing, Stuart, Anderson, Rob, Elston, Julian, Weiner, Graeme, Taylor, Rod S., Hoyle, Martin, Liu, Zulian, Price, Alison, Stein, Ken, Peninsula Technology Assessment Group (PenTAG), Peninsula Medical School, Universities of Exeter and Plymouth, The Effectiveness and Cost-Effectiveness of Cochlear Implants for Severe to Profound Deafness in Children and Adults: a Systematic Review and Economic Model, 2007, Plymouth: Peninsula Technology Assessment Group (PenTAG) [PubMed: 19799825]

  32. Bosworth & Nielson, 1997

    Bosworth, D. G., Nielson, D. W., Effectiveness of home versus hospital care in the routine treatment of cystic fibrosis, Pediatric Pulmonology, 24, 42–7, 1997 [PubMed: 9261852]

  33. Bradley et al., 2010

    Bradley, J., Blume, S., Stafford, M., Balp, M., Elborn, S., Quality of life and health utility in patients with cystic fibrosis, European Respiratory Journal, 36, 2652, 2010

  34. Bradley et al., 2012

    Bradley, G. M., Carson, K. A., Leonard, A. R., Mogayzel, P. J., Jr., Oliva-Hemker, M., Nutritional outcomes following gastrostomy in children with cystic fibrosis, Pediatric Pulmonology, 47, 743–8, 2012 [PMC free article: PMC3343175] [PubMed: 22298389]

  35. Brady et al., 1991

    Brady, M. S., Rickard, K., Yu, P. L., Eigen, H., Effectiveness and safety of small vs. large doses of enteric coated pancreatic enzymes in reducing steatorrhea in children with cystic fibrosis: a prospective randomized study, Pediatric Pulmonology, 10, 79–85, 1991 [PubMed: 2030925]

  36. Braggion et al., 1995

    Braggion, C., Cappelletti, L. M., Cornacchia, M., Zanolla, L., Mastella, G., Short-term effects of three chest physiotherapy regimens in patients hospitalized for pulmonary exacerbations of cystic fibrosis: a cross-over randomized study, Pediatric Pulmonology, 19, 16–22, 1995 [PubMed: 7675553]

  37. Braithwaite et al., 2011

    Braithwaite, M., Philip, J., Tranberg, H., Finlayson, F., Gold, M., Kotsimbos, T., Wilson, J., End of life care in CF: patients, families and staff experiences and unmet needs, Journal of Cystic Fibrosis, 10, 253–7, 2011 [PubMed: 21444251]

  38. Brenckmann et al., 2003

    Brenckmann, C., Papaioannou, A., Freitag, A., Hennessey, R., Hansen, S., Ioannidis, G., Webber, C., Adachi, J., Osteoporosis in Canadian adult cystic fibrosis patients: a descriptive study, BMC Musculoskeletal Disorders, 4, 13, 2003 [PMC free article: PMC198278] [PubMed: 12823863]

  39. Briesacher et al., 2011

    Briesacher, B. A., Quittner, A. L., Fouayzi, H., Zhang, J., Swensen, A., Nationwide trends in the medical care costs of privately insured patients with cystic fibrosis (CF), 2001–2007, Pediatric Pulmonology, 46, 770–6, 2011 [PubMed: 21465674]

  40. Brumfield & Lansbury, 2004

    Brumfield, K., Lansbury, G., Experiences of adolescents with cystic fibrosis during their transition from paediatric to adult health care: a qualitative study of young Australian adults, Disability & Rehabilitation, 26, 223–34, 2004 [PubMed: 15164956]

  41. Chatfield et al., 1991

    Chatfield, S., Owen, G., Ryley, H. C., Williams, J., Alfaham, M., Goodchild, M. C., Weller, P., Neonatal screening for cystic fibrosis in Wales and the West Midlands: clinical assessment after five years of screening, Archives of Disease in Childhood, 66, 29–33, 1991 [PMC free article: PMC1590363] [PubMed: 1996888]

  42. Chavasse et al., 2004

    Chavasse, R. J., Francis, J., Balfour-Lynn, I., Rosenthal, M., Bush, A., Serum vitamin D levels in children with cystic fibrosis, Pediatric Pulmonology, 38, 119–22, 2004 [PubMed: 15211694]

  43. Chen et al., 2001

    Chen, J. S., Witzmann, K. A., Spilker, T., Fink, R. J., LiPuma, J. J., Endemicity and inter-city spread of B cepacia genomovar III in cystic fibrosis, Journal of Pediatrics, 139, 643–9, 2001 [PubMed: 11713440]

  44. Cheng et al., 2014

    Cheng, Katharine, Ashby, Deborah, Smyth, Rosalind L., Ursodeoxycholic acid for cystic fibrosis-related liver disease, Cochrane Database of Systematic Reviews, 2014 [PubMed: 10796703]

  45. Cheng et al., 2015

    Cheng, K., Ashby, D., Smyth, R. L., Oral steroids for long-term use in cystic fibrosis, Cochrane Database of Systematic Reviews, 12, CD000407, 2015 [PMC free article: PMC7034414] [PubMed: 26649765]

  46. Chinuck et al., 2014

    Chinuck, R., Dewar, J., Baldwin, D. R., Hendron, E., Appetite stimulants for people with cystic fibrosis, Cochrane Database of Systematic Reviews, 7, CD008190, 2014 [PubMed: 25064192]

  47. Christopher et al., 1999

    Christopher, F., Chase, D., Stein, K., Milne, R., rhDNase therapy for the treatment of cystic fibrosis patients with mild to moderate lung disease, Journal of Clinical Pharmacy & Therapeutics, 24, 415–26, 1999 [PubMed: 10651974]

  48. Chuchalin et al., 2007

    Chuchalin, A., Csiszer, E., Gyurkovics, K., Bartnicka, M. T., Sands, D., Kapranov, N., Varoli, G., Monici Preti, P. A., Mazurek, H., A formulation of aerosolized tobramycin (Bramitob) in the treatment of patients with cystic fibrosis and P aeruginosa infection: a double-blind, placebo-controlled, multicenter study, Paediatric Drugs, 9 Suppl 1, 21–31, 2007 [PubMed: 17536872]

  49. Clement et al., 2006

    Clement, A., Tamalet, A., Leroux, E., Ravilly, S., Fauroux, B., Jais, J. P., Long term effects of azithromycin in patients with cystic fibrosis: A double blind, placebo controlled trial, Thorax, 61, 895–902, 2006 [PMC free article: PMC2104771] [PubMed: 16809416]

  50. Coates et al., 2007

    Coates, Nicola, Gregory, Maggie, Skirton, Heather, Gaff, Clara, Patch, Christine, Clarke, Angus, Parsons, Evelyn, Family communication about cystic fibrosis from the mother’s perspective: An exploratory study, Journal of Research in Nursing, 12, 619–634, 2007

  51. Colombo et al., 1996

    Colombo, C., Battezzati, P. M., Podda, M., Bettinardi, N., Giunta, A., Ursodeoxycholic acid for liver disease associated with cystic fibrosis: a double-blind multicenter trial. The Italian Group for the Study of Ursodeoxycholic Acid in Cystic Fibrosis, Hepatology, 23, 1484–90, 1996 [PubMed: 8675168]

  52. Conrad et al., 2015

    Conrad, C., Lymp, J., Thompson, V., Dunn, C., Davies, Z., Chatfield, B., Nichols, D., Clancy, J., Vender, R., Egan, M. E., Quittell, L., Michelson, P., Antony, V., Spahr, J., Rubenstein, R. C., Moss, R. B., Herzenberg, L. A., Goss, C. H., Tirouvanziam, R., Long-term treatment with oral N-acetylcysteine: affects lung function but not sputum inflammation in cystic fibrosis subjects. A phase II randomized placebo-controlled trial, Journal of Cystic Fibrosis, 14, 219–27, 2015 [PubMed: 25228446]

  53. Conway et al., 1997

    Conway, S. P., Pond, M. N., Watson, A., Etherington, C., Robey, H. L., Goldman, M. H., Intravenous colistin sulphomethate in acute respiratory exacerbations in adult patients with cystic fibrosis, Thorax, 52, 987–93, 1997 [PMC free article: PMC1758447] [PubMed: 9487348]

  54. Corry et al., 2008

    Corry, J., Poon, W., McPhee, N., Milner, A. D., Cruickshank, D., Porceddu, S. V., Rischin, D., Peters, L. J., Randomized study of percutaneous endoscopic gastrostomy versus nasogastric tubes for enteral feeding in head and neck cancer patients treated with (chemo)radiation, Journal of Medical Imaging and Radiation Oncology, 52, 503–510, 2008 [PubMed: 19032398]

  55. Cox et al., 2016

    Cox, N. S., Alison, J. A., Button, B. M., Wilson, J. W., Morton, J. M., Holland, A. E., Physical activity participation by adults with cystic fibrosis: An observational study, Respirology, 21, 511–8, 2016 [PubMed: 26715596]

  56. Curtis et al., 2016

    Curtis, L., Burns, A., Personal Social Services Research Unit, University of Kent, Unit costs of health and social care 2016, 2016, Canterbury: Personal Social Services Research Unit, University of Kent

  57. Cystic Fibrosis Trust, 2015

    Cystic Fibrosis Trust, UK CF Registry, Data on intestinal obstruction disaggregated by history of meconium ileus in relation to the UK, year 2015, [online; accessed 11 January 2017]

  58. Cystic Fibrosis Trust, 2016

    Cystic Fibrosis Trust, UK Cystic Fibrosis Registry 2015 Annual Data Report, London: Cystic Fibrosis Trust, 2016

  59. Cystic Fibrosis Trust, 2011

    Cystic Fibrosis Trust, Standards for the Clinical Care of Children and Adults with Cystic Fibrosis in the UK. London: Cystic Fibrosis Trust, 2011

  60. Cystic Fibrosis Trust, 2015a

    Cystic Fibrosis Trust, UK CF Registry, BMI and BMI percentile in relation to the UK, year 2015. [online; accessed 23 November 2016]

  61. Daniels et al., 2011

    Daniels, T., Goodacre, L., Sutton, C., Pollard, K., Conway, S., Peckham, D., Accurate assessment of adherence: self-report and clinician report vs electronic monitoring of nebulizers, Chest, 140, 425–32, 2011 [PubMed: 21330381]

  62. Darbee et al., 2005

    Darbee, J. C., Kanga, J. F., Ohtake, P. J., Physiologic evidence for high-frequency chest wall oscillation and positive expiratory pressure breathing in hospitalized subjects with cystic fibrosis, Physical Therapy, 85, 1278–89, 2005 [PubMed: 16305267]

  63. Darch et al., 2015

    Darch, S. E., McNally, Alan, Harrison, Freya, Corander, Jukka Barr, Helen L., Paszkiewicz, Konrad, Holden, Stephen, Fogarty, Andrew, Crusz, Shanika A., Diggle, Stephen P., Recombination is a key driver of genomic and phenotypic diversity in a P aeruginosa population during cystic fibrosis infection, Scientific Reports, 5, 7649, 2015 [PMC free article: PMC4289893] [PubMed: 25578031]

  64. D’Auria et al., 2000

    D’Auria, J. P., Christian, B. J., Henderson, Z. G., Haynes, B., The company they keep: the influence of peer relationships on adjustment to cystic fibrosis during adolescence, Journal of Pediatric Nursing, 15, 175–182, 2000 [PubMed: 10897555]

  65. DeBoeck et al., 1989

    De Boeck, K., Smet, M., Eggermont, E., Treatment of Pseudomonas lung infection in cystic fibrosis with piperacillin plus tobramycin versus ceftazidime monotherapy: preliminary communication, Pediatric Pulmonology, 7, 171–3, 1989 [PubMed: 2508049]

  66. DeBoeck et al., 2007

    De Boeck, K., De Baets, F., Malfroot, A., Desager, K., Mouchet, F., Proesmans, M., Do inhaled corticosteroids impair long-term growth in prepubertal cystic fibrosis patients?, European Journal of Pediatrics, 166, 23–8, 2007 [PubMed: 16799799]

  67. Debray et al., 2011

    Debray, D., Kelly, D.,Houwen, R., Strandvik, B., Colombo, C., Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease, Journal of Cystic Fibrosis, 10, Suppl 2, S29–36, 2011 [PubMed: 21658639]

  68. Dellon et al., 2012

    Dellon, E. P., Sawicki, G. S., Shores, M. D., Wolfe, J., Hanson, L. C., Physician practices for communicating with patients with cystic fibrosis about the use of noninvasive and invasive mechanical ventilation, Chest, 141, 1010–7, 2012 [PubMed: 21998257]

  69. Dentice et al., 2016

    Dentice, R. L., Elkins, M. R., Middleton, P. G., Bishop, J. R., Wark, P. A., Dorahy, D. J., Harmer, C. J., Hu, H., Bye, P. T., A randomised trial of hypertonic saline during hospitalisation for exacerbation of cystic fibrosis, Thorax, 71, 141–7, 2016 [PubMed: 26769016]

  70. Department of Health, 2010

    Department of Health, Healthcare Premises Cost Guides (HPCGs) 2010, 2010

  71. Department of Health, 2016

    Department of Health, NHS reference costs 2015 to 2016, 2016

  72. DerSimonian & Laird, 1986

    DerSimonian, R., Laird, N., Meta-analysis in clinical trials, Controlled Clinical Trials, 7, 177–88, 1986 [PubMed: 3802833]

  73. Diabetes​.co.uk, 2012

    Cost of diabetes, 2012

  74. Dias et al., 2016

    Dias, Sofia, Welton, Nicky J., Sutton, Alex J., Ades, A. E., NICE, NICE DSU Technical support document 2: a generalised linear modelling framework for pairwise and network meta-analysis of randomised controlled trials, 2016 [PubMed: 27466657]

  75. Dixon-Woods et al., 2005

    Dixon-Woods, M., Agarwal, S., Jones, D., Young, B., Sutton, A., Synthesising qualitative and quantitative evidence: a review of possible methods, Journal of Health Services & Research Policy, 10, 45–53, 2005 [PubMed: 15667704]

  76. Dolan, 1997

    Dolan, P., Modeling valuations for EuroQol health states, Medical care, 35, 1095–108, 1997 [PubMed: 9366889]

  77. Donati et al., 1987

    Donati, M. A., Guenette, G., Auerbach, H., Prospective controlled study of home and hospital therapy of cystic fibrosis pulmonary disease, Journal of Pediatrics, 111, 28–33, 1987 [PubMed: 3298594]

  78. Dupuis et al., 2011

    Dupuis, F., Duhamel, F., Gendron, S., Transitioning care of an adolescent with cystic fibrosis: development of systemic hypothesis between parents, adolescents, and health care professionals, Journal of Family Nursing, 17, 291–311, 2011 [PubMed: 21813812]

  79. Durie et al., 1980

    Durie, P. R., Bell, L., Linton, W., Corey, M. L., Forstner, G. G., Effect of cimetidine and sodium bicarbonate on pancreatic replacement therapy in cystic fibrosis, Gut, 21, 778–86, 1980 [PMC free article: PMC1419526] [PubMed: 7429342]

  80. Eigen et al., 1995

    Eigen, H., Rosenstein, B. J., FitzSimmons, S., Schidlow, D. V., A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group, Journal of Pediatrics, 126, 515–23, 1995 [PubMed: 7699528]

  81. Elborn et al., 1992

    Elborn, S., Colville, A., Cordon, S., Hiller, E. J., Shale, D., A comparison of intravenous ceftazidime and aztreonam in the treatment of respiratory exacerbations in cystic fibrosis, 11th International Cystic Fibrosis Congress, 1992, [Abstract]

  82. Elkins et al., 2006

    Elkins, M. R., Robinson, M., Rose, B. R., Harbour, C., Moriarty, C. P., Marks, G. B., Belousova, E. G., Xuan, W., Bye, P. T., National Hypertonic Saline in Cystic Fibrosis Study, Group, A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis, New England Journal of Medicine, 354, 229–40, 2006 [PubMed: 16421364]

  83. Elliott et al., 2005

    Elliott, R. A., Thornton, J., Webb, A. K., Dodd, M., Tully, M. P., Comparing costs of home- versus hospital-based treatment of infections in adults in a specialist cystic fibrosis center, International Journal of Technology Assessment in Health Care, 21, 506–10, 2005 [PubMed: 16262975]

  84. Elphick & Southern, 2014

    Elphick, H. E., Southern, K. W., Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis, Cochrane Database of Systematic Reviews, 11, CD002204, 2014 [PubMed: 25431975]

  85. Enright et al., 2004

    Enright, S., Chatham, K., Ionescu, A. A., Unnithan, V. B., Shale, D. J., Inspiratory muscle training improves lung function and exercise capacity in adults with cystic fibrosis, Chest, 126, 405–11, 2004 [PubMed: 15302725]

  86. Equi et al., 2002

    Equi, A., Balfour-Lynn, I.M., Bush, A., Rosenthal, M., Long term azithromycin in children with cystic fibrosis: A randomised, placebo-controlled crossover trial, Lancet, 360, 978–984, 2002 [PubMed: 12383667]

  87. Ericsson et al., 2013

    Ericsson, A., Pollock, R. F., Hunt, B., Valentine, W. J., Evaluation of the cost-utility of insulin degludec vs insulin glargine in Sweden, Journal of Medical Economics, 16, 1442–52, 2013 [PubMed: 24147661]

  88. Eriksen & Ingebretsen, 2006

    Eriksen, B. O., Ingebretsen, O. C., The progression of chronic kidney disease: a 10-year population-based study of the effects of gender and age, Kidney international, 69, 375–82, 2006 [PubMed: 16408129]

  89. Esmond et al., 2006

    Esmond, G., Butler, M., McCormack, A. M., Comparison of hospital and home intravenous antibiotic therapy in adults with cystic fibrosis, Journal of Clinical Nursing, 15, 52–60, 2006 [PubMed: 16390524]

  90. Etherington et al., 2008

    Etherington, C., Hall, M., Conway, S., Peckham, D., Denton, M., Clinical impact of reducing routine susceptibility testing in chronic P aeruginosa infections in cystic fibrosis, Journal of Antimicrobial Chemotherapy, 61, 425–7, 2008 [PubMed: 18156280]

  91. Eubanks et al., 2002

    Eubanks, V., Koppersmith, N., Wooldridge, N., Clancy, J. P., Lyrene, R., Arani, R. B., Lee, J., Moldawer, L., Atchison, J., Sorscher, E. J., Makris, C. M., Effects of megestrol acetate on weight gain, body composition, and pulmonary function in patients with cystic fibrosis, Journal of Pediatrics, 140, 439–44, 2002 [PubMed: 12006958]

  92. European Medicines Agency, 2012

    European Medicines Agency, Report of the workshop on endpoints for cystic fibrosis clinical trials, 2012, London: European Medicines Agency

  93. Fagundes et al., 2004

    Fagundes, E. D. T., Silva, R. A. P., Roquete, M. L. V., Penna, F. J., Reis, F. J. C., Goulart, E. M. A., Duque, C. G., Validation of the Williams ultrasound scoring system for the diagnosis of liver disease in cystic fibrosis, Jornal de Pediatria, 80, 380–386, 2004 [PubMed: 15505733]

  94. Fair et al., 2000

    Fair, A., Griffiths, K., Osman, L. M., Attitudes to fertility issues among adults with cystic fibrosis in Scotland. The Collaborative Group of Scottish Adult CF Centres, Thorax, 55, 672–7, 2000 [PMC free article: PMC1745836] [PubMed: 10899244]

  95. Farmer et al., 2009

    Farmer, A. J., Wade, A. N., French, D. P., Simon, J., Yudkin, P., Gray, A., Craven, A., Goyder, L., Holman, R. R., Mant, D., Kinmonth, A. L., Neil, H. A., Blood glucose self-monitoring in type 2 diabetes: a randomised controlled trial, Health Technology Assessment (Winchester, England), 13, iii–iv, ix–xi, 1–50, 2009 [PubMed: 19254484]

  96. Filigno et al., 2012

    Filigno, S. S., Brannon, E. E., Chamberlin, L. A., Sullivan, S. M., Barnett, K. A., Powers, S. W., Qualitative analysis of parent experiences with achieving cystic fibrosis nutrition recommendations, Journal of Cystic Fibrosis, 11, 125–30, 2012 [PMC free article: PMC3320219] [PubMed: 22112934]

  97. Finkelstein et al., 1992

    Finkelstein, S. M., Wielinski, C. L., Kujawa, S. J., Loewenson, R., Warwick, W. J., The impact of home monitoring and daily diary recording on patient status in cystic fibrosis, Pediatric Pulmonology, 12, 3–10, 1992 [PubMed: 1579374]

  98. Floto et al., 2016

    Floto, A., Olivier, K. N., Saiman, L., Daley, C. L., Herrmann, J., Nick, J. A., Noone, P. G., Bilton, D., Corris, P., Gibson, R. L., Hempstead, S. E., Koetz, K., Sabadosa, K. A., Sermet-Gaudelus, I., Smyth, A.R., van Ingen, J., Wallace, R. J., Winthrop, K. L., Marshall, B. C., Haworth, C. S., US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis, Thorax, 71, i1–i22, 2016 [PMC free article: PMC4717371] [PubMed: 26666259]

  99. Flume et al., 2016

    Flume, P. A., Clancy, J. P., Retsch-Bogart, G. Z., Tullis, D. E., Bresnik, M., Derchak, P. A., Lewis, S. A., Ramsey, B. W., Continuous alternating inhaled antibiotics for chronic pseudomonal infection in cystic fibrosis, Journal of Cystic Fibrosis, 15, 809–815, 2016 [PubMed: 27233377]

  100. Forest Laboratories UK, 2011

    Forest Laboratories UK, Colistimethate sodium powder for inhalation for the treatment of Pseudomonas lung infection in cystic fibrosis – Forest submission to NICE. COLO/DPI/02/05., 2011, Kent: Forest Laboratories UK

  101. Forest Laboratories UK, 2011a

    Forest Laboratories UK, A randomised, open label study to compare the efficacy and safety of a dry powder formulation of inhaled colistimethate sodium and nebulised TNSFI (tobramycin nebuliser solution for inhalation, TOBI®) in cystic fibrosis patients with P aeruginosa lung infection. Final protocol no: COLO/DPI/02/06., 2011, Kent: Forest Laboratories UK

  102. France et al., 2008

    France, M. W., Dodd, M. E., Govan, J. R., Doherty, C. J., Webb, A. K., Jones, A. M., The changing epidemiology of Burkholderia species infection at an adult cystic fibrosis centre, Journal of Cystic Fibrosis, 7, 368–72, 2008 [PubMed: 18276200]

  103. Francisco et al., 2002

    Francisco, M. P., Wagner, M. H., Sherman, J. M., Theriaque, D., Bowser, E., Novak, D. A., Ranitidine and omeprazole as adjuvant therapy to pancrelipase to improve fat absorption in patients with cystic fibrosis, Journal of Pediatric Gastroenterology & Nutrition, 35, 79–83, 2002 [PubMed: 12142815]

  104. Frederiksen et al., 1999

    Frederiksen, B., Koch, C., Hoiby, N., Changing epidemiology of P aeruginosa infection in Danish cystic fibrosis patients (1974–1995), Pediatric Pulmonology, 28, 159–66, 1999 [PubMed: 10495331]

  105. Fuchs et al., 1994

    Fuchs, H. J., Borowitz, D. S., Christiansen, D. H., Morris, E. M., Nash, M. L., Ramsey, B. W., Rosenstein, B. J., Smith, A. L., Wohl, M. E., Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis, New England Journal of Medicine, 331, 637–642, 1994 [PubMed: 7503821]

  106. Gabriel et al., 1991

    Gabriel, S. E., Jaakkimainen, L., Bombardier, C., Risk for serious gastrointestinal complications related to use of nonsteroidal anti-inflammatory drugs. A meta-analysis, Annals of Internal Medicine, 115, 787–96, 1991 [PubMed: 1834002]

  107. Galeva et al., 2013

    Galeva, I., Konstan, M.W., Higgins, M., Angyalosi, G., Brockhaus, F., Piggott, S., Thomas, K., Chuchalin, A.G., Tobramycin inhalation powder manufactured by improved process in cystic fibrosis: the randomized EDIT trial, Current Medical Research and Opinion, 29, 947–956, 2013 [PMC free article: PMC4086187] [PubMed: 23672633]

  108. Garcia Rodriguez & Jick, 1994

    Garcia Rodriguez, L. A., Jick, H., Risk of upper gastrointestinal bleeding and perforation associated with individual non-steroidal anti-inflammatory drugs, Lancet, 343, 769–72, 1994 [PubMed: 7907735]

  109. Gold et al., 1985

    Gold, R., Overmeyer, A., Knie, B., Fleming, P. C., Levison, H., Controlled trial of ceftazidime vs. ticarcillin and tobramycin in the treatment of acute respiratory exacerbations in patients with cystic fibrosis, Pediatric Infectious Disease, 4, 172–7, 1985 [PubMed: 3885181]

  110. Goldbeck et al., 2014

    Goldbeck, L., Fidika, A., Herle, M., Quittner, A. L., Psychological interventions for individuals with cystic fibrosis and their families, Cochrane Database of Systematic Reviews, 6, CD003148, 2014 [PMC free article: PMC7388585] [PubMed: 24941199]

  111. Greally et al., 1994

    Greally, P., Hussain, M. J., Vergani, D., Price, J. F., Interleukin-1 alpha, soluble interleukin-2 receptor, and IgG concentrations in cystic fibrosis treated with prednisolone, Archives of Disease in Childhood, 71, 35–9, 1994 [PMC free article: PMC1029909] [PubMed: 8067791]

  112. Grieve et al., 2003

    Grieve, R., Thompson, S., Normand, C., Suri, R., Bush, A., Wallis, C., A cost-effectiveness analysis of rhDNase in children with cystic fibrosis, International Journal of Technology Assessment in Health Care, 19, 71–9, 2003 [PubMed: 12701940]

  113. Griffiths et al., 2004

    Griffiths, A.L., Armstrong, D., Carzino, R., Robinson, P., Cystic fibrosis patients and families support cross-infection measures, European Respiratory Journal, 24, 449–452, 2004 [PubMed: 15358705]

  114. Griffiths et al., 2005

    Griffiths, A. L., Jamsen, K., Carlin, J. B., Grimwood, K., Carzino, R., Robinson, P. J., Massie, J., Armstrong, D. S., Effects of segregation on an epidemic P aeruginosa strain in a cystic fibrosis clinic, American Journal of Respiratory & Critical Care Medicine, 171, 1020–5, 2005 [PubMed: 15709051]

  115. Griffiths et al., 2012

    Griffiths, A. L., Wurzel, D. F., Robinson, P. J., Carzino, R., Massie, J., Australian epidemic strain pseudomonas (AES-1) declines further in a cohort segregated cystic fibrosis clinic, Journal of Cystic Fibrosis, 11, 49–52, 2012 [PubMed: 21907639]

  116. Grimaldi et al., 2015

    Grimaldi, C., Bremont, F., Berlioz-Baudoin, M., Brouard, J., Corvol, H., Couderc, L., Lezmi, G., Pin, I., Petit, I., Reix, P., Remus, N., Schweitzer, C., Thumerelle, C., Dubus, J. C., Sweat test practice in pediatric pulmonology after introduction of cystic fibrosis newborn screening, European Journal of Pediatrics, 174, 1613–20, 2015 [PubMed: 26074372]

  117. Grob, 2008

    Grob, R., Is my sick child healthy? Is my healthy child sick?: changing parental experiences of cystic fibrosis in the age of expanded newborn screening, Social Science & Medicine, 67, 1056–64, 2008 [PubMed: 18639968]

  118. Grossoehme et al., 2014

    Grossoehme, D. H., Filigno, S. S., Bishop, M., Parent routines for managing cystic fibrosis in children, Journal of Clinical Psychology in Medical Settings, 21, 125–135, 2014 [PMC free article: PMC4104356] [PubMed: 24838648]

  119. Gruber et al., 2014

    Gruber, W., Orenstein, D. M., Braumann, K. M., Beneke, R., Interval exercise training in cystic fibrosis - Effects on exercise capacity in severely affected adults, Journal of Cystic Fibrosis, 13, 86–91, 2014 [PubMed: 23863518]

  120. Grzincich et al., 2008

    Grzincich, G. L., Longo, F., Faverzani, S., Chetta, A., Spaggiari, C., Pisi, G., Short term effects of high frequency chest compression (HFCC) and positive expiratory pressure (PEP) in adults with cystic fibrosis, European Respiratory Society Annual Congress, Berlin, Germany, October, 2008, [Abstract]

  121. Gupta et al., 2012

    Gupta, S., Ahmed, F., Lodha, R., Gupta, Y. K., Kabra, S. K., Comparison of effects of 3 and 7% hypertonic saline nebulization on lung function in children with cystic fibrosis: a double-blind randomized, controlled trial, Journal of Tropical Pediatrics, 58, 375–81, 2012 [PubMed: 22374985]

  122. Gutthann et al., 1997

    Gutthann, S. P., Garcia Rodriguez, L. A., Raiford, D. S., Individual nonsteroidal antiinflammatory drugs and other risk factors for upper gastrointestinal bleeding and perforation, Epidemiology (Cambridge, Mass.), 8, 18–24, 1997 [PubMed: 9116088]

  123. Hanning et al., 1993

    Hanning, R. M., Blimkie, C. J., Bar-Or, O., Lands, L. C., Moss, L. A., Wilson, W. M., Relationships among nutritional status and skeletal and respiratory muscle function in cystic fibrosis: does early dietary supplementation make a difference?, American Journal of Clinical Nutrition, 57, 580–7, 1993 [PubMed: 8460615]

  124. Haworth et al., 2002

    Haworth, C. S., Selby, P. L., Horrocks, A. W., Mawer, E. B., Adams, J. E., Webb, A. K., A prospective study of change in bone mineral density over one year in adults with cystic fibrosis, Thorax, 57, 719–23, 2002 [PMC free article: PMC1746411] [PubMed: 12149534]

  125. Hayden et al., 2013

    Hayden, J. A., van der Windt, D. A., Cartwright, J. L., Cote, P., Bombardier, C., Assessing bias in studies of prognostic factors, Annals of Internal Medicine, 158, 280–6, 2013 [PubMed: 23420236]

  126. Hayes et al., 2010

    Hayes, D., Jr., West, S. E., Rock, M. J., Li, Z., Splaingard, M. L., Farrell, P. M., P aeruginosa in children with cystic fibrosis diagnosed through newborn screening: assessment of clinic exposures and microbial genotypes, Pediatric Pulmonology, 45, 708–16, 2010 [PMC free article: PMC2921980] [PubMed: 20575089]

  127. Health Technology Assessment, 2015

    Health Technology Assessment, Mannitol dry powder for inhalation for the treatment of cystic fibrosis (Project record), Health Technology Assessment (Winchester, England), 2015

  128. Hebestreit et al., 2010

    Hebestreit, H., Kieser, S., Junge, S., Ballmann, M., Hebestreit, A., Schindler, C., Schenk, T., Posselt, H. G., Kriemler, S., Long-term effects of a partially supervised conditioning programme in cystic fibrosis, European Respiratory Journal, 35, 578–83, 2010 [PubMed: 19643946]

  129. Heijerman et al., 1991

    Heijerman, H. G., Lamers, C. B., Bakker, W., Omeprazole enhances the efficacy of pancreatin (pancrease) in cystic fibrosis, Annals of Internal Medicine, 114, 200–1, 1991 [PubMed: 1984743]

  130. Heijerman et al., 1993

    Heijerman, H. G., Lamers, C. B., Bakker, W., Dijkman, J. H., Improvement of fecal fat excretion after addition of omeprazole to pancrease in cystic fibrosis is related to residual exocrine function of the pancreas, Digestive Diseases & Sciences, 38, 1–6, 1993 [PubMed: 8420740]

  131. Heltshe et al., 2014

    Heltshe, S. L., Borowitz, D. S., Leung, D. H., Ramsey, B., Mayer-Hamblett, N., Early attained weight and length predict growth faltering better than velocity measures in infants with CF, Journal of Cystic Fibrosis, 13, 723–9, 2014 [PMC free article: PMC4252713] [PubMed: 24917114]

  132. Henry et al., 1996

    Henry, D., Lim, L. L., Garcia Rodriguez, L. A., Perez Gutthann, S., Carson, J. L., Griffin, M., Savage, R., Logan, R., Moride, Y., Hawkey, C., Hill, S., Fries, J. T., Variability in risk of gastrointestinal complications with individual non-steroidal anti-inflammatory drugs: results of a collaborative meta-analysis, BMJ, 312, 1563–1566, 1996 [PMC free article: PMC2351326] [PubMed: 8664664]

  133. Hilliard et al., 2014

    Hilliard, M. E., Hahn, A., Ridge, A. K., Eakin, M. N., Riekert, K. A., User Preferences and Design Recommendations for an mHealth App to Promote Cystic Fibrosis Self-Management, JMIR MHealth and UHealth, 2, e44, 2014 [PMC free article: PMC4259916] [PubMed: 25344616]

  134. Hodgkinson & Lester, 2002

    Hodgkinson, R., Lester, H., Stresses and coping strategies of mothers living with a child with cystic fibrosis: implications for nursing professionals, Journal of Advanced Nursing, 39, 377–83, 2002 [PubMed: 12139650]

  135. Hodson et al., 2002

    Hodson, M. E., Gallagher, C. G., Govan, J. R., A randomised clinical trial of nebulised tobramycin or colistin in cystic fibrosis, European Respiratory Journal, 20, 658–64, 2002 [PubMed: 12358344]

  136. Hoiby & Pedersen, 1989

    Hoiby, N., Pedersen, S. S., Estimated risk of cross-infection with P aeruginosa in Danish cystic fibrosis patients, Acta Paediatrica Scandinavica, 78, 395–404, 1989 [PubMed: 2787093]

  137. Hommerding et al., 2015

    Hommerding, P. X., Baptista, R. R., Makarewicz, G. T., Schindel, C. S., Donadio, M. V. F., Pinto, L. A., Marostica, P. J. C., Effects of an educational intervention of physical activity for children and adolescents with cystic fibrosis: A randomized controlled trial, Respiratory Care, 60, 81–87, 2015 [PubMed: 25140032]

  138. Homnick et al., 1998

    Homnick, D. N., Anderson, K., Marks, J. H., Comparison of the flutter device to standard chest physiotherapy in hospitalized patients with cystic fibrosis: a pilot study, Chest, 114, 993–7, 1998 [PubMed: 9792567]

  139. Homnick et al., 2004

    Homnick, D. N., Homnick, B. D., Reeves, A. J., Marks, J. H., Pimentel, R. S., Bonnema, S. K., Cyproheptadine is an effective appetite stimulant in cystic fibrosis, Pediatric Pulmonology, 38, 129–34, 2004 [PubMed: 15211696]

  140. Horsley et al., 2016

    Horsley, A., Jones, A. M., Lord, R., Antibiotic treatment for B cepacia complex in people with cystic fibrosis experiencing a pulmonary exacerbation, Cochrane Database of Systematic Reviews, 1, CD009529, 2016 [PMC free article: PMC7100516] [PubMed: 26789750]

  141. Houston et al., 2013

    Houston, B. W., Mills, N., Solis-Moya, A., Inspiratory muscle training for cystic fibrosis, Cochrane Database of Systematic Reviews, 11, CD006112, 2013 [PubMed: 24258092]

  142. Hubert et al., 2004

    Hubert, D., Fajac, I., Bienvenu, T., Desmazes-Dufeu, N., Ellaffi, M., Dall’ava-Santucci, J., Dusser, D., Diagnosis of cystic fibrosis in adults with diffuse bronchiectasis, Journal of Cystic Fibrosis, 3, 15–22, 2004 [PubMed: 15463882]

  143. Hummelinck & Pollock, 2006

    Hummelinck, A., Pollock, K., Parents’ information needs about the treatment of their chronically ill child: A qualitative study, Patient Education and Counseling, 62, 228–234, 2006 [PubMed: 16139981]

  144. Hurley et al., 2015

    Hurley, M. N., Prayle, A. P., Flume, P., Intravenous antibiotics for pulmonary exacerbations in people with cystic fibrosis, Cochrane Database of Systematic Reviews, 7, CD009730, 2015 [PMC free article: PMC6481905] [PubMed: 26226131]

  145. Iles & Lowton, 2010

    Iles, N., Lowton, K., What is the perceived nature of parental care and support for young people with cystic fibrosis as they enter adult health services?, Health and Social Care in the Community, 18, 21–29, 2010 [PubMed: 19637994]

  146. Iles et al., 2003

    Iles, R., Legh-Smith, J., Drummond, M., Prevost, A., Vowler, S., Economic evaluation of Tobramycin nebuliser solution in cystic fibrosis, Journal of Cystic Fibrosis, 2, 120–8, 2003 [PubMed: 15463860]

  147. Jaques et al., 2008

    Jaques, A., Daviskas, E., Turton, J. A., McKay, K., Cooper, P., Stirling, R. G., Robertson, C. F., Bye, P. T., Lesouef, P. N., Shadbolt, B., Anderson, S. D., Charlton, B., Inhaled mannitol improves lung function in cystic fibrosis, Chest, 133, 1388–96, 2008 [PubMed: 18339790]

  148. Jensen et al., 1987

    Jensen, T., Pedersen, S. S., Garne, S., Heilmann, C., Hoiby, N., Koch, C., Colistin inhalation therapy in cystic fibrosis patients with chronic P aeruginosa lung infection, Journal of Antimicrobial Chemotherapy, 19, 831–8, 1987 [PubMed: 3301785]

  149. Jessup & Parkinson, 2010

    Jessup, M., Parkinson, C., “All at sea”: the experience of living with cystic fibrosis, Qualitative Health Research, 20, 352–64, 2010 [PubMed: 19955225]

  150. Jessup et al., 2016

    Jessup, M., Douglas, T., Priddis, L., Branch-Smith, C., Shields, L., Arest, C. F., Parental Experience of Information and Education Processes Following Diagnosis of Their Infant With Cystic Fibrosis Via Newborn Screening, Journal of Pediatric Nursing, 31, e233–41, 2016 [PubMed: 26699440]

  151. Johannesson et al., 1998

    Johannesson, M., Carlson, M., Brucefors, A. B., Hjelte, L., Cystic fibrosis through a female perspective: psychosocial issues and information concerning puberty and motherhood, Patient Education & Counseling, 34, 115–23, 1998 [PubMed: 9731171]

  152. Jones et al., 2005

    Jones, A. M., Dodd, M. E., Govan, J. R., Doherty, C. J., Smith, C. M., Isalska, B. J., Webb, A. K., Prospective surveillance for P aeruginosa cross-infection at a cystic fibrosis center, American Journal of Respiratory & Critical Care Medicine, 171, 257–60, 2005 [PubMed: 15542795]

  153. Kalnins et al., 2005

    Kalnins, D., Corey, M., Ellis, L., Pencharz, P. B., Tullis, E., Durie, P. R., Failure of conventional strategies to improve nutritional status in malnourished adolescents and adults with cystic fibrosis, Journal of Pediatrics, 147, 399–401, 2005 [PubMed: 16182685]

  154. Karlas et al., 2012

    Karlas, T., Neuschulz, M., Oltmanns, A., Guttler, A., Petroff, D., Wirtz, H., Mainz, J. G., Mossner, J., Berg, T., Troltzsch, M., Keim, V., Wiegand, J., Non-invasive evaluation of cystic fibrosis related liver disease in adults with ARFI, transient elastography and different fibrosis scores, PLoS ONE, 7, e42139, 2012 [PMC free article: PMC3405060] [PubMed: 22848732]

  155. Kazmerski et al., 2016

    Kazmerski, T. M., Borrero, S., Tuchman, L. K., Weiner, D. J., Pilewski, J. M., Orenstein, D. M., Miller, E., Provider and Patient Attitudes Regarding Sexual Health in Young Women With Cystic Fibrosis, Pediatrics, 137, 2016 [PubMed: 27244858]

  156. Kind et al., 1999

    Kind, P., Hardman, G., Macran, S., Centre for Health Economics, University of York, UK Population Norms for EQ-5D, 1999, York: University of York

  157. Kirk & Milnes, 2016

    Kirk, S., Milnes, L., An exploration of how young people and parents use online support in the context of living with cystic fibrosis, Health Expectations, 19, 309–21, 2016 [PMC free article: PMC5055278] [PubMed: 25691209]

  158. Kitson et al., 2013

    Kitson, M. T., Kemp, W. W., Iser, D. M., Paul, E., Wilson, J. W., Roberts, S. K., Utility of transient elastography in the non-invasive evaluation of cystic fibrosis liver disease, Liver International, 33, 698–705, 2013 [PubMed: 23432782]

  159. Klijn et al., 2004

    Klijn, P. H. C., Oudshoorn, A., Van Der Ent, C. K., Van Der Net, J., Kimpen, J. L., Helders, P. J. M., Effects of anaerobic training in children with cystic fibrosis: A randomized controlled study, Chest, 125, 1299–1305, 2004 [PubMed: 15078738]

  160. Konstan et al., 1995

    Konstan, M.W., Byard, P.J., Hoppel, C.L., Davis, P.B., Effect of high-dose ibuprofen in patients with cystic fibrosis, New England Journal of Medicine, 332, 848–854, 1995 [PubMed: 7503838]

  161. Konstan et al., 2011

    Konstan, M. W., Geller, D. E., Minic, P., Brockhaus, F., Zhang, J., Angyalosi, G., Tobramycin inhalation powder for P aeruginosa infection in cystic fibrosis: the EVOLVE trial, Pediatric Pulmonology, 46, 230–8, 2011 [PMC free article: PMC4112574] [PubMed: 20963831]

  162. Konstan et al., 2011a

    Konstan, M.W., Flume, P.A., Kappler, M., Chiron, R., Higgins, M., Brockhaus, F., Zhang, J., Angyalosi, G., He, E., Geller, D.E., Safety, efficacy and convenience of tobramycin inhalation powder in cystic fibrosis patients: The EAGER trial, Journal of Cystic Fibrosis, 10, 54–61, 2011 [PMC free article: PMC4086197] [PubMed: 21075062]

  163. Konstan et al., 2012

    Konstan, Michael W., Wagener, Jeffrey S., VanDevanter, Donald R., Pasta, David J., Yegin, Ashley, Rasouliyan, Lawrence, Morgan, Wayne J., Risk factors for rate of decline in FEV1 in adults with cystic fibrosis, Journal of Cystic Fibrosis, 11, 405–411, 2012 [PMC free article: PMC4086189] [PubMed: 22561369]

  164. Kriemler et al., 2013

    Kriemler, S., Kieser, S., Junge, S., Ballmann, M., Hebestreit, A., Schindler, C., Stussi, C., Hebestreit, H., Effect of supervised training on FEV1 in cystic fibrosis: a randomised controlled trial, Journal of Cystic Fibrosis, 12, 714–20, 2013 [PubMed: 23588193]

  165. Lai & Shoff, 2008

    Lai, H. J., Shoff, S. M., Classification of malnutrition in cystic fibrosis: implications for evaluating and benchmarking clinical practice performance, American Journal of Clinical Nutrition, 88, 161–6, 2008 [PMC free article: PMC2527817] [PubMed: 18614737]

  166. Lai et al., 2000

    Lai, H. C., FitzSimmons, S. C., Allen, D. B., Kosorok, M. R., Rosenstein, B. J., Campbell, P. W., Farrell, P. M., Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis, New England Journal of Medicine, 342, 851–9, 2000 [PubMed: 10727589]

  167. Lands & Stanojevic, 2016

    Lands, L. C., Stanojevic, S., Oral non-steroidal anti-inflammatory drug therapy for lung disease in cystic fibrosis, Cochrane Database of Systematic Reviews, 4, CD001505, 2016 [PubMed: 27055154]

  168. Lands et al., 2007

    Lands, L.C., Milner, R., Cantin, A.M., Manson, D., Corey, M., High-Dose Ibuprofen in Cystic Fibrosis: Canadian Safety and Effectiveness Trial, Journal of Pediatrics, 151, 249–254, 2007 [PubMed: 17719932]

  169. Lang et al., 2005

    Lang, L., Duff, A. J., Brownlee, K. G., Introducing the need for lung transplantation in children with cystic fibrosis: parental experiences, Journal of Cystic Fibrosis, 4, 259–62, 2005 [PubMed: 16257274]

  170. Langton Hewer & Smyth, 2014

    Langton Hewer, S. C., Smyth, A. R., Antibiotic strategies for eradicating P aeruginosa in people with cystic fibrosis, Cochrane Database of Systematic Reviews, 11, CD004197, 2014 [PubMed: 25383937]

  171. Laube et al., 1996

    Laube, B. L., Auci, R. M., Shields, D. E., Christiansen, D. H., Lucas, M. K., Fuchs, H. J., Rosenstein, B. J., Effect of rhDNase on airflow obstruction and mucociliary clearance in cystic fibrosis, American Journal of Respiratory & Critical Care Medicine, 153, 752–60, 1996 [PubMed: 8564129]

  172. Lee et al., 2004

    Lee, T. W., Brownlee, K. G., Denton, M., Littlewood, J. M., Conway, S. P., Reduction in prevalence of chronic P aeruginosa infection at a regional pediatric cystic fibrosis center, Pediatric Pulmonology, 37, 104–10, 2004 [PubMed: 14730654]

  173. Lee et al., 2011

    Lee, Joyce M., Rhee, Kirsten, O’Grady, Michael J., Basu, Anirban, Winn, Aaron, John, Priya, Meltzer, David O., Kollman, Craig, Laffel, Lori M., Lawrence, Jean M., Tamborlane, William V., Wysocki, Tim, Xing, Dongyuan, Huang, Elbert S., Health Utilities for Children and Adults with Type 1 Diabetes, Medical Care, 49, 924–931, 2011 [PMC free article: PMC3210197] [PubMed: 21544001]

  174. Lemaitre et al., 2016

    Lemaitre, C., Dominique, S., Billoud, E., Eliezer, M., Montialoux, H., Quillard, M., Riachi, G., Koning, E., Morisse-Pradier, H., Savoye, G., Savoye-Collet, C., Goria, O., Relevance of 3D Cholangiography and Transient Elastography to Assess Cystic Fibrosis-Associated Liver Disease?, Canadian Respiratory Journal, 2016, 4592702, 2016 [PMC free article: PMC4939173] [PubMed: 27445541]

  175. Lenoir et al., 2007

    Lenoir, G., Antypkin, Y. G., Miano, A., Moretti, P., Zanda, M., Varoli, G., Monici Preti, P. A., Aryayev, N. L., Efficacy, safety, and local pharmacokinetics of highly concentrated nebulized tobramycin in patients with cystic fibrosis colonized with P aeruginosa, Paediatric Drugs, 9 (Suppl 1), 11–20, 2007 [PubMed: 17536871]

  176. Lesko & Mitchell, 1995

    Lesko, S.M., Mitchell, A.A., An assessment of the safety of pediatric ibuprofen. A practitioner-based randomized clinical trial, JAMA, 273, 929–933, 1995 [PubMed: 7884951]

  177. Lewin et al., 2015

    Lewin, Simon, Glenton, Claire, Munthe-Kaas, Heather, Carlsen, Benedicte, Colvin, Christopher J., Gülmezoglu, Metin, Noyes, Jane, Booth, Andrew, Garside, Ruth, Rashidian, Arash, Using Qualitative Evidence in Decision Making for Health and Social Interventions: An Approach to Assess Confidence in Findings from Qualitative Evidence Syntheses (GRADE-CERQual), PLoS Medicine, 12, e1001895, 2015 [PMC free article: PMC4624425] [PubMed: 26506244]

  178. Lewindon et al., 2011

    Lewindon, P. J., Shepherd, R. W., Walsh, M. J., Greer, R. M., Williamson, R., Pereira, T. N., Frawley, K., Bell, S. C., Smith, J. L., Ramm, G. A., Importance of hepatic fibrosis in cystic fibrosis and the predictive value of liver biopsy, Hepatology, 53, 193–201, 2011 [PubMed: 21254170]

  179. Lewis et al., 2015

    Lewis, C., Blackman, S. M., Nelson, A., Oberdorfer, E., Wells, D., Dunitz, J., Thomas, W., Moran, A., Diabetes-related mortality in adults with cystic fibrosis. Role of genotype and sex, American Journal of Respiratory & Critical Care Medicine, 191, 194–200, 2015 [PMC free article: PMC4347432] [PubMed: 25479583]

  180. Lindblad et al., 1999

    Lindblad, A., Glaumann, H., Strandvik, B., Natural history of liver disease in cystic fibrosis, Hepatology, 30, 1151–8, 1999 [PubMed: 10534335]

  181. Lo et al., 2015

    Lo, D. K., Hurley, M. N., Muhlebach, M. S., Smyth, A. R., Interventions for the eradication of meticillin-resistant S aureus (MRSA) in people with cystic fibrosis, Cochrane Database of Systematic Reviews, 2, CD009650, 2015 [PubMed: 25927091]

  182. Lowson et al., 2011

    Lowson, K., Kelly, J., Bending, M., Whitehead, S., Wright, D., Lowson, P., Duffy, S., Trueman, P., Saxby, R., West, P., York Health Economics Consortium, University of York, Cost-Effectiveness of Hospital Design: Options to Improve Patient Safety and Wellbeing, 2011, York: University of York

  183. Lucidi et al., 2009

    Lucidi, V., Alghisi, F., Raia, V., Russo, B., Valmarana, L., Valmarana, R., Coruzzo, A., Beschi, S., Dester, S., Rinaldi, D., Maglieri, M., Guidotti, M. L., Ravaioli, E., Pesola, M., De Alessandri, A., Padoan, R., Grynzich, L., Ratclif, L., Repetto, T., Ambroni, M., Provenzano, E., Tozzi, A. E., Colombo, C., Growth assessment of paediatric patients with CF comparing different auxologic indicators: A multicentre Italian study, Journal of Pediatric Gastroenterology & Nutrition, 49, 335–42, 2009 [PubMed: 19543116]

  184. Lucidi et al., 2011

    Lucidi, V., Alghisi, F., Dall’Oglio, L., D’Apice, M. R., Monti, L., De Angelis, P., Gambardella, S., Angioni, A., Novelli, G., The etiology of acute recurrent pancreatitis in children: a challenge for pediatricians, Pancreas, 40, 517–21, 2011 [PubMed: 21499205]

  185. Maben et al., 2015

    Maben, J., Griffiths, P., Penfold, C., Simon, M., Pizzo, E., Anderson, J., Robert, G., Hughes, J., Murrells, T., Brearley, S., Barlow, J., Evaluating a major innovation in hospital design: workforce implications and impact on patient and staff experiences of all single room hospital accommodation, 2015, In: Health Services and Delivery Research, Vol. 3, No. 3 [PubMed: 25719188]

  186. Macdonald & Greggans, 2010

    Macdonald, K., Greggans, A., ‘Cool friends’: an evaluation of a community befriending programme for young people with cystic fibrosis, Journal of Clinical Nursing, 19, 2406–14, 2010 [PubMed: 20920068]

  187. Macfarlane et al., 1985

    Macfarlane, P. I., Hughes, D. M., Landau, L. I., Olinsky, A., The role of piperacillin therapy in pulmonary exacerbations of cystic fibrosis: a controlled study, Pediatric Pulmonology, 1, 249–55, 1985 [PubMed: 3906545]

  188. Maes et al., 2011

    Maes, I. H., Joore, M. A., Cima, R. F., Vlaeyen, J. W., Anteunis, L. J., Assessment of health state in patients with tinnitus: a comparison of the EQ-5D and HUI mark III, Ear and Hearing, 32, 428–35, 2011 [PubMed: 21221004]

  189. Mainz et al., 2016

    Mainz, J. G., Schumacher, U., Schadlich, K., Hentschel, J., Koitschev, C., Koitschev, A., Riethmuller, J., Prenzel, F., Sommerburg, O., Wiedemann, B., Staab, D., Gleiber, W., Fischer, R., Beck, J. F., Arnold, C., Cooperators,, Sino nasal inhalation of isotonic versus hypertonic saline (6.0%) in CF patients with chronic rhinosinusitis - Results of a multicenter, prospective, randomized, double-blind, controlled trial, Journal of Cystic Fibrosis, 4, 4, 2016 [PubMed: 27267518]

  190. Mantel & Haenszel, 1959

    Mantel, N., Haenszel, W., Statistical aspects of the analysis of data from retrospective studies of disease, Journal of the National Cancer Institute, 22, 719–48, 1959 [PubMed: 13655060]

  191. Marchand et al., 2000

    Marchand, V., Baker, S. S., Stark, T. J., Baker, R. D., Randomized, double-blind, placebo-controlled pilot trial of megestrol acetate in malnourished children with cystic fibrosis, Journal of Pediatric Gastroenterology & Nutrition, 31, 264–9, 2000 [PubMed: 10997370]

  192. Master et al., 2001

    Master, V., Roberts, G.W., Coulthard, K.P., Baghurst, P.A., Martin, A., Roberts, M.E., Onishko, C.R., Martin, A.J., Linke, R.J., Holmes, M., Jarvinen, A., Kennedy, D., Colebatch, K.A., Hansman, D., Parsons, D.W., Efficacy of once-daily tobramycin monotherapy for acute pulmonary exacerbations of cystic fibrosis: a preliminary study, Pediatric Pulmonology, 31, 367–376, 2001 [PubMed: 11340683]

  193. McCarty et al., 1988

    McCarty, J. M., Tilden, S. J., Black, P., Craft, J. C., Blumer, J., Waring, W., Halsey, N. A., Comparison of piperacillin alone versus piperacillin plus tobramycin for treatment of respiratory infections in children with cystic fibrosis, Pediatric Pulmonology, 4, 201–4, 1988 [PubMed: 3393383]

  194. McCoy et al., 1996

    McCoy, K., Hamilton, S., Johnson, C., Effects of 12-week administration of dornase alfa in patients with advanced cystic fibrosis lung disease. Pulmozyme Study Group, Chest, 110, 889–95, 1996 [PubMed: 8874241]

  195. McCoy et al., 2008

    McCoy, K. S., Quittner, A. L., Oermann, C. M., Gibson, R. L., Retsch-Bogart, G. Z., Montgomery, A. B., Inhaled aztreonam lysine for chronic airway P aeruginosa in cystic fibrosis, American Journal of Respiratory & Critical Care Medicine, 178, 921–8, 2008 [PMC free article: PMC2577727] [PubMed: 18658109]

  196. McIlwaine et al., 2001

    McIlwaine, P. M., Wong, L. T., Peacock, D., Davidson, A. G., Long-term comparative trial of positive expiratory pressure versus oscillating positive expiratory pressure (flutter) physiotherapy in the treatment of cystic fibrosis, Journal of Pediatrics, 138, 845–50, 2001 [PubMed: 11391327]

  197. McIlwaine et al., 2013

    McIlwaine, M. P., Alarie, N., Davidson, G. F., Lands, L. C., Ratjen, F., Milner, R., Owen, B., Agnew, J. L., Long-term multicentre randomised controlled study of high frequency chest wall oscillation versus positive expiratory pressure mask in cystic fibrosis, Thorax, 68, 746–51, 2013 [PubMed: 23407019]

  198. McIlwaine et al., 2014

    McIlwaine, M. P., Richmond, M., Agnew, J. L., Alarie, N., Lands, L., Chilvers, M., Ratjen, F., Cost-effectiveness of performing positive expiratory pressure versus high frequency chest wall oscillation, Journal of Cystic Fibrosis, 13, S11, 2014

  199. McIlwaine et al., 2015

    McIlwaine, M, Button, B, Dwan, K, Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis, Cochrane Database of Systematic Reviews, CD003147, 2015 [PubMed: 26083308]

  200. McIntyre, 1999

    McIntyre, A. M., Dornase alpha and survival of patients with cystic fibrosis, Hospital Medicine (London), 60, 736–9, 1999 [PubMed: 10656067]

  201. McKay et al., 2009

    McKay, K. O., Cooper, P. J., van Asperen, P. P., Segregation of children with CF diagnosed via newborn screening and acquisition of P aeruginosa, Journal of Cystic Fibrosis, 8, 400–4, 2009 [PubMed: 19716353]

  202. McNamara et al., 1997

    McNamara, R. L., Lima, J. A., Whelton, P. K., Powe, N. R., Echocardiographic identification of cardiovascular sources of emboli to guide clinical management of stroke: a cost-effectiveness analysis, Annals of Internal Medicine, 127, 775–87, 1997 [PubMed: 9382398]

  203. Medicines Complete, 2017

    BNF 72 and BNF for Children (September 2016 – March 2017), 2017, London: Pharmaceutical Press

  204. Menzin et al., 1996

    Menzin, J., Oster, G., Davies, L., Drummond, M. F., Greiner, W., Lucioni, C., Merot, J. L., Rossi, F., vd Schulenburg, J. G., Souetre, E., A multinational economic evaluation of rhDNase in the treatment of cystic fibrosis, International Journal of Technology Assessment in Health Care, 12, 52–61, 1996 [PubMed: 8690562]

  205. Merli et al., 1994

    Merli, M., Bertasi, S., Servi, R., Diamanti, S., Martino, F., De Santis, A., Goffredo, F., Quattrucci, S., Antonelli, M., Angelico, M., Effect of a medium dose of ursodeoxycholic acid with or without taurine supplementation on the nutritional status of patients with cystic fibrosis: a randomized, placebo-controlled, crossover trial, Journal of Pediatric Gastroenterology & Nutrition, 19, 198–203, 1994 [PubMed: 7815243]

  206. Miller et al., 2009

    Miller, A. R., Condin, C. J., McKellin, W. H., Shaw, N., Klassen, A. F., Sheps, S., Continuity of care for children with complex chronic health conditions: parents’ perspectives, BMC Health Services Research, 9, 242, 2009 [PMC free article: PMC2805629] [PubMed: 20025770]

  207. Minasian et al., 2010

    Minasian, C., Wallis, C., Metcalfe, C., Bush, A., Comparison of inhaled mannitol, daily rhDNase and a combination of both in children with cystic fibrosis: a randomised trial, Thorax, 65, 51–6, 2010 [PubMed: 19996349]

  208. Mitchell et al., 1982

    Mitchell, E. A., Quested, C., Marks, R. E., Pinnock, R. E., Elliott, R. B., Comparative trial of viokase, pancreatin and Pancrease pancrelipase (enteric coated beads) in the treatment of malabsorption in cystic fibrosis, Australian Paediatric Journal, 18, 114–7, 1982 [PubMed: 6923744]

  209. Moen et al., 2011

    Moen, I. E., Nilsson, K., Andersson, A., Fagerland, M. W., Fluge, G., Hollsing, A., Gilljam, M., Mared, L., Pressler, T., Santi, H., Storrosten, O. T., Hjelte, L., Dietary intake and nutritional status in a Scandinavian adult cystic fibrosis-population compared with recommendations, Food and Nutrition Research, 55 (no pagination), 2011 [PMC free article: PMC3220890] [PubMed: 22110412]

  210. Moodie et al., 2014

    Moodie, M., Lal, A., Vidmar, S., Armstrong, D. S., Byrnes, C. A., Carlin, J. B., Cheney, J., Cooper, P. J., Grimwood, K., Robertson, C. F., Tiddens, H. A., Wainwright, C. E., Australasian Cystic Fibrosis Bronchoalveolar Lavage Study, Investigators, Costs of bronchoalveolar lavage-directed therapy in the first 5 years of life for children with cystic fibrosis, Journal of Pediatrics, 165, 564–569.e5, 2014 [PubMed: 24996984]

  211. Moola & Norman, 2011

    Moola, F. J., Norman, M. E., ‘Down the rabbit hole’: enhancing the transition process for youth with cystic fibrosis and congenital heart disease by re-imagining the future and time, Child: Care, Health & Development, 37, 841–51, 2011 [PubMed: 22007984]

  212. Moorcroft et al., 2004

    Moorcroft, A. J., Dodd, M. E., Morris, J., Webb, A. K., Individualised unsupervised exercise training in adults with cystic fibrosis: a 1 year randomised controlled trial, Thorax, 59, 1074–80, 2004 [PMC free article: PMC1746905] [PubMed: 15563708]

  213. Moran et al., 2010

    Moran, A., Brunzell, Carol, Cohen, Richard C., Katz, Marcia, Marshall, Bruce C., Onady, Gary, Robinson, Karen A., Sabadosa, Kathryn A., Stecenko, Arlene, Slovis, Bonnie, The CFRD Guidelines committee, Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes: A position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society, Diabetes Care, 33, 2697–2708, 2010 [PMC free article: PMC2992215] [PubMed: 21115772]

  214. Moran et al., 2009

    Moran, A., Dunitz, J., Nathan, B., Saeed, A., Holme, B., Thomas, W., Cystic fibrosis-related diabetes: current trends in prevalence, incidence, and mortality, Diabetes Care, 32, 1626–31, 2009 [PMC free article: PMC2732133] [PubMed: 19542209]

  215. Moran et al., 2014

    Moran, Fidelma, Bradley, Judy M., Piper, Amanda J., Non-invasive ventilation for cystic fibrosis, Cochrane Database of Systematic Reviews, 4, CD00276, 2013 [PubMed: 23633308]

  216. Morrison & Agnew, 2014

    Morrison, L., Agnew, J., Oscillating devices for airway clearance in people with cystic fibrosis, Cochrane Database of Systematic Reviews, 7, CD006842, 2014 [PubMed: 25038719]

  217. Morton & Wolfe, 2015

    Morton, A., Wolfe, S., Enteral tube feeding for cystic fibrosis, Cochrane Database of Systematic Reviews, 4, CD001198, 2015 [PubMed: 25854213]

  218. Mueller-Abt et al., 2008

    Mueller-Abt, P. R., Frawley, K. J., Greer, R. M., Lewindon, P. J., Comparison of ultrasound and biopsy findings in children with cystic fibrosis related liver disease, Journal of Cystic Fibrosis, 7, 215–21, 2008 [PubMed: 17904429]

  219. Murphy et al., 2004

    Murphy, T. D., Anbar, R. D., Lester, L. A., Nasr, S. Z., Nickerson, B., VanDevanter, D. R., Colin, A. A., Treatment with tobramycin solution for inhalation reduces hospitalizations in young CF subjects with mild lung disease, Pediatric Pulmonology, 38, 314–20, 2004 [PubMed: 15334509]

  220. National Horizon Scanning Centre, 2008

    National Horizon Scanning Centre, National Institute for Health Research, Mannitol dry powder for inhalation (Bronchitol) for cystic fibrosis at risk for bronchiectasis, 2008, Southampton: NIHR

  221. National Horizon Scanning Centre, 2008a

    National Horizon Scanning Centre, National Institute for Health Research, Transient elastography (FibroScan) for evaluating liver fibrosis, 2008, Southampton: NIHR

  222. National Institute for Health and Care Excellence, 2013

    National Institute for Health and Care Excellence, Acute kidney injury: prevention, detection and management, 2013 [PubMed: 31909927]

  223. National Institute for Health and Clinical Excellence, 2011

    National Institute for Health and Clinical Excellence, Common mental health problems: identification and pathways to care, 2011

  224. National Institute for Health and Clinical Excellence, 2009

    National Institute for Health and Clinical Excellence, Depression in adults: recognition and management, 2009 [PubMed: 31990491]

  225. National Institute for Clinical Excellence, 2004

    National Institute for Clinical Excellence, Eating disorders in over 8s: management, 2004

  226. National Institute for Clinical Excellence, 2005

    National Institute for Clinical Excellence, Depression in children and young people: identification and management, 2005 [PubMed: 31577402]

  227. National Institute for Health and Care Excellence, 2013a

    National Institute for Health and Care Excellence, Generalised anxiety disorder and panic disorder in adults: management, 2013 [PubMed: 31961629]

  228. National Institute for Health and Clinical Excellence, 2012

    National Institute for Health and Clinical Excellence, Mannitol dry powder for inhalation for treating cystic fibrosis. Single technology appraisal, 2012

  229. National Institute for Health and Clinical Excellence, 2009a

    National Institute for Health and Clinical Excellence, Medicines adherence: involving patients in decisions about prescribed medicines and supporting adherence, 2009 [PubMed: 21834197]

  230. National Institute for Health and Clinical Excellence, 2012a

    National Institute for Health and Clinical Excellence, NICE guidelines manual, 2012 [PubMed: 27905714]

  231. National Institute for Health and Care Excellence, 2014

    National Institute for Health and Care Excellence, NICE guidelines manual, 2014 [PubMed: 27905714]

  232. National Institute for Health and Care Excellence, 2015

    National Institute for Health and Care Excellence, Diabetes in pregnancy: management from preconception to the postnatal period, 2015 [PubMed: 32212588]

  233. National Institute for Health and Care Excellence, 2015a

    National Institute for Health and Care Excellence, Type 1 diabetes in adults: diagnosis and management, 2015 [PubMed: 26334079]

  234. National Institute for Health and Care Excellence, 2016

    National Institute for Health and Care Excellence, Integrated sensor-augmented pump therapy systems for managing blood glucose levels in type 1 diabetes, 2016 [PMC free article: PMC4809467] [PubMed: 26933827]

  235. National Institute for Health and Care Excellence, 2016a

    National Institute for Health and Care Excellence, Cirrhosis in over 16s: assessment and management, 2016 [PubMed: 27441331]

  236. National Institute for Health and Care Excellence, 2016b

    National Institute for Health and Care Excellence, NICE TA submission by Vertex. (Lumacaftor and ivacaftor combination therapy for treating CF homozygous for the F508del mutation [ID786]), 2016

  237. National Institute for Health and Clinical Excellence, 2012b

    National Institute for Health and Clinical Excellence, Patient experience in adult NHS services: improving the experience of care for people using adult NHS services, 2012 [PubMed: 31909930]

  238. National Institute for Health and Care Excellence, 2016c

    National Institute for Health and Care Excellence, Transition from children’s to adults’ services for young people using health or social care services, 2016

  239. National Institute for Health and Clinical Excellence, 2009b

    National Institute for Health and Clinical Excellence, Type 2 diabetes: The management of type 2 diabetes, 2009

  240. Newbold et al., 2005

    Newbold, M. E., Tullis, E., Corey, M., Ross, B., Brooks, D., The Flutter Device versus the PEP Mask in the treatment of adults with cystic fibrosis, Physiotherapy Canada, 57, 199–207, 2005

  241. NHS, 2015

    NHS, NHS Supply Chain 2015, 2015, Leeds: NHS

  242. NHS, 2015a

    NHS, National Diabetes Audit (NDA) Mortality Analysis 2012–2013, 2015, Leeds: NHS Digital

  243. NHS Business Services Authority,

    NHS Business Services Authority, NHS Electronic Drug Tariff, Newcastle upon Tyne: NHS Business Services Authority

  244. NHS England,

    NHS England, Service Specifications: Cystic Fibrosis – Adults, Leeds: NHS

  245. NHS England,

    NHS England, Service Specifications: Cystic Fibrosis – Children, Leeds: NHS

  246. NHS Estates, 2005

    NHS Estates, Ward layouts with single rooms and space for flexibility, 2005, London: The Stationery Office

  247. Nolan, Sarah et al., 2015

    Nolan Sarah, J., Thornton, Judith, Murray Clare, S., Dwyer, Tiffany, Inhaled mannitol for cystic fibrosis, Cochrane Database of Systematic Reviews, 10, CD008649, 2015 [PubMed: 26451533]

  248. O’Brien et al., 1992

    O’Brien, S., Fitzgerald, M. X., Hegarty, J. E., A controlled trial of ursodeoxycholic acid treatment in cystic fibrosis-related liver disease, European Journal of Gastroenterology and Hepatology, 4, 857–863, 1992

  249. Oermann et al., 2001

    Oermann, C. M., Sockrider, M. M., Giles, D., Sontag, M. K., Accurso, F. J., Castile, R. G., Comparison of high-frequency chest wall oscillation and oscillating positive expiratory pressure in the home management of cystic fibrosis: a pilot study, Pediatric Pulmonology, 32, 372–7, 2001 [PubMed: 11596162]

  250. Ooi et al., 2012

    Ooi, C. Y., Dupuis, A., Ellis, L., Jarvi, K., Martin, S., Gonska, T., Dorfman, R., Kortan, P., Solomon, M., Tullis, E., Durie, P. R., Comparing the American and European diagnostic guidelines for cystic fibrosis: same disease, different language?, Thorax, 67, 618–24, 2012 [PubMed: 22504961]

  251. Orenstein et al., 2004

    Orenstein, D. M., Hovell, M. F., Mulvihill, M., Keating, K. K., Hofstetter, C. R., Kelsey, S., Morris, K., Nixon, P. A., Strength vs aerobic training in children with cystic fibrosis: a randomized controlled trial, Chest, 126, 1204–14, 2004 [PubMed: 15486384]

  252. Padman et al., 1999

    Padman, R., Geouque, D. M., Engelhardt, M. T., Effects of the flutter device on pulmonary function studies among pediatric cystic fibrosis patients, Delaware Medical Journal, 71, 13–8, 1999 [PubMed: 10024754]

  253. Papaioannou et al., 2008

    Papaioannou, A., Kennedy, C. C., Freitag, A., O’Neill, J., Pui, M., Ioannidis, G., Webber, C., Pathak, A., Hansen, S., Hennessey, R., Adachi, J. D., Longitudinal analysis of vertebral fracture and BMD in a Canadian cohort of adult cystic fibrosis patients, BMC Musculoskeletal Disorders, 9, 125, 2008 [PMC free article: PMC2567975] [PubMed: 18801200]

  254. Patriquin et al., 1999

    Patriquin, H., Lenaerts, C., Smith, L., Perreault, G., Grignon, A., Filiatrault, D., Boisvert, J., Roy, C. C., Rasquin-Weber, A., Liver disease in children with cystic fibrosis: US-biochemical comparison in 195 patients, Radiology, 211, 229–32, 1999 [PubMed: 10189476]

  255. Philips et al., 2004

    Philips, Z., Ginnelly, L., Sculpher, M., Claxton, K., Golder, S., Riemsma, R., Woolacoot, N., Glanville, J., Review of guidelines for good practice in decision-analytic modelling in Health Technology Assessment, Health Technology Assessment (Winchester, England), 8, iii–iv, ix–xi, 1–158, 2004 [PubMed: 15361314]

  256. Pignone et al., 2006

    Pignone, M., Earnshaw, S., Tice, J. A., Pletcher, M. J., Aspirin, statins, or both drugs for the primary prevention of coronary heart disease events in men: a cost-utility analysis, Annals of Internal Medicine, 144, 326–36, 2006 [PubMed: 16520473]

  257. Placidi et al., 2006

    Placidi, G., Cornacchia, M., Polese, G., Zanolla, L., Assael, B. M., Braggion, C., Chest physiotherapy with positive airway pressure: a pilot study of short-term effects on sputum clearance in patients with cystic fibrosis and severe airway obstruction, Respiratory Care, 51, 1145–53, 2006 [PubMed: 17005060]

  258. Pollock et al., 2010

    Pollock, R. F., Valentine, W. J., Goodall, G., Brandle, M., Evaluating the cost-effectiveness of self-monitoring of blood glucose in type 2 diabetes patients on oral anti-diabetic agents, Swiss Medical Weekly, 140, w13103, 2010 [PubMed: 21110238]

  259. Poustie et al., 2006

    Poustie, V. J., Russell, J. E., Watling, R. M., Ashby, D., Smyth, R. L., Calico Trial Collaborative Group, Oral protein energy supplements for children with cystic fibrosis: CALICO multicentre randomised controlled trial, BMJ, 332, 632–6, 2006 [PMC free article: PMC1403226] [PubMed: 16467348]

  260. Powers et al., 2003

    Powers, S. W., Byars, K. C., Mitchell, M. J., Patton, S. R., Schindler, T., Zeller, M. H., A randomized pilot study of behavioural treatment to increase calorie intake in toddlers with cystic fibrosis, Children’s Health Care, 32, 297–311, 2003

  261. Powers et al., 2015

    Powers, S. W., Stark, L. J., Chamberlin, L. A., Filigno, S. S., Sullivan, S. M., Lemanek, K. L., Butcher, J. L., Driscoll, K. A., Daines, C. L., Brody, A. S., Schindler, T., Konstan, M. W., McCoy, K. S., Nasr, S. Z., Castile, R. G., Acton, J. D., Wooldridge, J. L., Ksenich, R. A., Szczesniak, R. D., Rausch, J. R., Stallings, V. A., Zemel, B. S., Clancy, J. P., Behavioral and nutritional treatment for preschool-aged children with cystic fibrosis: a randomized clinical trial, JAMA Pediatrics, 169, e150636, 2015 [PubMed: 25938655]

  262. Proesmans et al., 2013

    Proesmans, M., Vermeulen, F., Boulanger, L., Verhaegen, J., De Boeck, K., Comparison of two treatment regimens for eradication of P aeruginosa infection in children with cystic fibrosis, Journal of Cystic Fibrosis, 12, 29–34, 2013 [PubMed: 22762867]

  263. Public Health England, 2012

    Public Health England, Newborn blood spot screening programme: supporting publications - Cystic fibrosis (CF), 2012 (URL: https://www​.gov.uk/government​/collections​/newborn-blood-spot-screening-programme-supporting-publications)

  264. Quan et al., 2001

    Quan, J. M., Tiddens, H. A., Sy, J. P., McKenzie, S. G., Montgomery, M. D., Robinson, P. J., Wohl, M. E., Konstan, M. W., Pulmozyme Early Intervention Trial Study, Group, A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities, Journal of Pediatrics, 139, 813–20, 2001 [PubMed: 11743506]

  265. Quon et al., 2011

    Quon, B.S., Mayer-Hamblett, N., Aitken, M.L., Smyth, A.R., Goss, C.H., Risk factors for chronic kidney disease in adults with cystic fibrosis, American Journal of Respiratory and Critical Care Medicine, 184, 1147–1152, 2011 [PMC free article: PMC3262023] [PubMed: 21799076]

  266. Qvist et al., 2016

    Qvist, Tavs, Taylor-Robinson, David, Waldmann, Elisabeth, Olesen, Hanne Vebert, Hansen, Christine Rønne, Mathiesen, Inger Hee, Høiby, Niels, Katzenstein, Terese L., Smyth, Rosalind L., Diggle, Peter J., Pressler, Tania, Comparing the harmful effects of nontuberculous mycobacteria and Gram negative bacteria on lung function in patients with cystic fibrosis, Journal of Cystic Fibrosis, 15, 380–385, 2016 [PMC free article: PMC4893021] [PubMed: 26482717]

  267. Radtke et al., 2015

    Radtke, T., Nolan, S. J., Hebestreit, H., Kriemler, S., Physical exercise training for cystic fibrosis [Update of Cochrane Database Systematic Reviews. 2008; (1): CD002768], Cochrane Database of Systematic Reviews, 6, CD002768, 2015 [PubMed: 26116828]

  268. Ramsey et al., 1993

    Ramsey, B. W., Dorkin, H. L., Eisenberg, J. D., Gibson, R. L., Harwood, I. R., Kravitz, R. M., Schidlow, D. V., Wilmott, R. W., Astley, S. J., McBurnie, M. A., et al, Efficacy of aerosolized tobramycin in patients with cystic fibrosis, New England Journal of Medicine, 328, 1740–6, 1993 [PubMed: 8497284]

  269. Ramsey et al., 1993a

    Ramsey, B. W., Astley, S. J., Aitken, M. L., Burke, W., Colin, A. A., Dorkin, H. L., Eisenberg, J. D., Gibson, R. L., Harwood, I. R., Schidlow, D. V., et al, Efficacy and safety of short-term administration of aerosolized recombinant human deoxyribonuclease in patients with cystic fibrosis, American Review of Respiratory Disease, 148, 145–51, 1993 [PubMed: 8317790]

  270. Ramsey et al., 1999

    Ramsey, B. W., Pepe, M. S., Quan, J. M., Otto, K. L., Montgomery, A. B., Williams-Warren, J., Vasiljev, K. M., Borowitz, D., Bowman, C. M., Marshall, B. C., Marshall, S., Smith, A. L., Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. Cystic Fibrosis Inhaled Tobramycin Study Group, New England Journal of Medicine, 340, 23–30, 1999 [PubMed: 9878641]

  271. Rana et al., 2014

    Rana, M., Wong-See, D., Katz, T., Gaskin, K., Whitehead, B., Jaffe, A., Coakley, J., Lochhead, A., Fat-soluble vitamin deficiency in children and adolescents with cystic fibrosis, Journal of Clinical Pathology, 67, 605–8, 2014 [PubMed: 24711511]

  272. Ranasinha et al., 1993

    Ranasinha, C., Assoufi, B., Shak, S., Christiansen, D., Fuchs, H., Empey, D., Geddes, D., Hodson, M., Efficacy and safety of short-term administration of aerosolised recombinant human DNase I in adults with stable stage cystic fibrosis, Lancet, 342, 199–202, 1993 [PubMed: 8100928]

  273. Rath et al., 2012

    Rath, T., Menendez, K. M., Kugler, M., Hage, L., Wenzel, C., Schulz, R., Graf, J., Nahrlich, L., Roeb, E., Roderfeld, M., TIMP-1/-2 and transient elastography allow non invasive diagnosis of cystic fibrosis associated liver disease, Digestive & Liver Disease, 44, 780–7, 2012 [PubMed: 22652148]

  274. Rath et al., 2013

    Rath, T., Hage, L., Kugler, M., Menendez Menendez, K., Zachoval, R., Naehrlich, L., Schulz, R., Roderfeld, M., Roeb, E., Serum Proteome Profiling Identifies Novel and Powerful Markers of Cystic Fibrosis Liver Disease, PLoS ONE, 8, 2013 [PMC free article: PMC3597583] [PubMed: 23516586]

  275. Ratjen et al., 1985

    Ratjen, F., Wonne, R., Posselt, H. G., Stover, B., Hofmann, D., Bender, S. W., A double-blind placebo controlled trial with oral ambroxol and N-acetylcysteine for mucolytic treatment in cystic fibrosis, European Journal of Pediatrics, 144, 374–8, 1985 [PubMed: 3908111]

  276. Regan & Bhatt, 2016

    Regan, K. H., Bhatt, J., Eradication therapy for B cepacia complex in people with cystic fibrosis, Cochrane Database of Systematic Reviews, 11, CD009876, 2016 [PubMed: 27804115]

  277. Remmington et al., 2016

    Remmington, T., Jahnke, N., Harkensee, C., Oral anti-pseudomonal antibiotics for cystic fibrosis, Cochrane Database of Systematic ReviewsCochrane Database Syst Rev, 7, CD005405, 2016 [PMC free article: PMC6457863] [PubMed: 27412131]

  278. Retsch-Bogart et al., 2009

    Retsch-Bogart, G. Z., Quittner, A. L., Gibson, R. L., Oermann, C. M., McCoy, K. S., Montgomery, A. B., Cooper, P. J., Efficacy and safety of inhaled aztreonam lysine for airway pseudomonas in cystic fibrosis, Chest, 135, 1223–32, 2009 [PMC free article: PMC2818415] [PubMed: 19420195]

  279. Richard et al., 1997

    Richard, D. A., Nousia-Arvanitakis, S., Sollich, V., Hampel, B. J., Sommerauer, B., Schaad, U. B., Oral ciprofloxacin vs. intravenous ceftazidime plus tobramycin in pediatric cystic fibrosis patients: comparison of antipseudomonas efficacy and assessment of safety with ultrasonography and magnetic resonance imaging. Cystic Fibrosis Study Group, Pediatric Infectious Disease Journal, 16, 572–8, 1997 [PubMed: 9194107]

  280. Riemsma et al., 2011

    Riemsma, R., Maiwenn, J. Al, Armstrong, Nigel, Misso, Kate, Allen, Alexander, Manning, Nathan, Tusabe, Doreen Allen, Severans, Johan L., Kleijnen, Jos, Kleijnen Systematic Reviews Ltd., Mannitol dry powder for inhalation for the treatment of cystic fibrosis: A Single Technology Appraisal, 2011, York: Kleijnen Systematic Reviews Ltd

  281. Riethmueller et al., 2002

    Riethmueller, J., Busch, A., Damm, V., Ziebach, R., Stern, M., Home and hospital antibiotic treatment prove similarly effective in cystic fibrosis, Infection, 30, 387–91, 2002 [PubMed: 12478330]

  282. Robinson et al., 2012

    Robinson, P., Schechter, M. S., Sly, P. D., Winfield, K., Smith, J., Brennan, S., Shinkai, M., Henke, M. O., Rubin, B. K., Clarithromycin therapy for patients with Cystic Fibrosis: A randomized controlled trial, Pediatric Pulmonology, 47, 551–557, 2012 [PubMed: 22266895]

  283. Roehrer et al., 2013

    Roehrer, E., Cummings, E., Beggs, S., Turner, P., Hauser, J., Micallef, N., Ellis, L., Reid, D., Pilot evaluation of web enabled symptom monitoring in cystic fibrosis, Informatics for Health & Social Care, 38, 354–65, 2013 [PubMed: 23957685]

  284. Rogers et al., 2012

    Rogers, Chris, Thomas, Helen, Taylor, Rhiannon, Cromwell, David, Parameshwar, Jayan, Banner, Nicholas R., UKCTA Steering Group, Royal College of Surgeons of England, UK Cardiothoracic Transplant Audit in patients who received a transplant between 1st July 1995 and 31st March 2012. Annual Report, 2012, London: Royal College of Surgeons of England

  285. Rosenfeld et al., 2012

    Rosenfeld, M., Ratjen, F., Brumback, L., Daniel, S., Rowbotham, R., McNamara, S., Johnson, R., Kronmal, R., Davis, S. D., Isis Study Group, Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: the ISIS randomized controlled trial, JAMA, 307, 2269–77, 2012 [PMC free article: PMC3586815] [PubMed: 22610452]

  286. Rovedder et al., 2014

    Rovedder, P. M., Flores, J., Ziegler, B., Casarotto, F., Jaques, P., Barreto, S. S., et al, Exercise programme in patients with cystic fibrosis: a randomized controlled trial, Respiratory Medicine, 108, 1134–40, 2014 [PubMed: 25002195]

  287. Royal College of Paediatrics and Child Health, 2014

    Royal College of Paediatrics and Child Health, Guidelines for the Performance of the Sweat Test for the Investigation of Cystic Fibrosis in the UK, 2nd Version, 2014, London: Royal College of Paediatrics and Child Health

  288. Royal College of Paediatrics and Child Health, 2017

    Royal College of Paediatrics and Child Health, School age charts and resources 2017, 2017, London: Royal College of Paediatrics and Child Health

  289. Russell et al., 1996

    Russell, M. T., Reinbold, J., Maltby, H. J., Transferring to adult health care: experiences of adolescents with cystic fibrosis, Journal of Pediatric Nursing, 11, 262–268, 1996 [PubMed: 8772045]

  290. Russo et al., 2006

    Russo, K., Donnelly, M., Reid, A. J., Segregation--the perspectives of young patients and their parents, Journal of Cystic Fibrosis, 5, 93–9, 2006 [PubMed: 16448864]

  291. Ryan et al., 2011

    Ryan, G., Singh, M., Dwan, K., Inhaled antibiotics for long-term therapy in cystic fibrosis, Cochrane Database of Systematic Reviews, 3, CD001021, 2011 [PubMed: 21412868]

  292. Ryan et al., 2012

    Ryan, G., Jahnke, N., Remmington, T., Inhaled antibiotics for pulmonary exacerbations in cystic fibrosis, Cochrane Database of Systematic Reviews, 12, CD008319, 2012 [PubMed: 23235659]

  293. Sadler et al., 2015

    Sadler, M. D., Crotty, P., Fatovich, L., Wilson, S., Rabin, H. R., Myers, R. P., Noninvasive methods, including transient elastography, for the detection of liver disease in adults with cystic fibrosis, Canadian Journal of Gastroenterology & Hepatology, 29, 139–44, 2015 [PMC free article: PMC4399373] [PubMed: 25855877]

  294. Saiman et al., 2003

    Saiman, L., Marshall, B. C., Mayer-Hamblett, N., Burns, J. L., Quittner, A. L., Cibene, D. A., Coquillette, S., Fieberg, A. Y., Accurso, F. J., Campbell, P. W., 3rd, Macrolide Study, Group, Azithromycin in patients with cystic fibrosis chronically infected with P aeruginosa: a randomized controlled trial, JAMA, 290, 1749–56, 2003 [PubMed: 14519709]

  295. Saiman et al., 2010

    Saiman, L., Anstead, M., Mayer-Hamblett, N., Lands, L. C., Kloster, M., Hocevar-Trnka, J., Goss, C. H., Rose, L. M., Burns, J. L., Marshall, B. C., Ratjen, F., A. Z. Azithromycin Study Group, Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with P aeruginosa: a randomized controlled trial, JAMA, 303, 1707–15, 2010 [PubMed: 20442386]

  296. Salh et al., 1992

    Salh, B., Bilton, D., Dodd, M., Abbot, J., Webb, K., A comparison of aztreonam and ceftazidime in the treatment of respiratory infections in adults with cystic fibrosis, Scandinavian Journal of Infectious Diseases, 24, 215–8, 1992 [PubMed: 1641599]

  297. Sanders et al., 2015

    Sanders, D. B., Li, Z., Brody, A. S., Chest computed tomography predicts the frequency of pulmonary exacerbations in children with cystic fibrosis, Annals of the American Thoracic Society, 12, 64–69, 2015 [PMC free article: PMC5466157] [PubMed: 25474182]

  298. Santana-Sosa et al., 2012

    Santana-Sosa, E., Groeneveld, I. F., Gonzalez-Saiz, L., Lopez-Mojares, L. M., Villa-Asensi, J. R., Barrio Gonzalez, M. I., Fleck, S. J., Perez, M., Lucia, A., Intrahospital weight and aerobic training in children with cystic fibrosis: a randomized controlled trial, Medicine & Science in Sports & Exercise, 44, 2–11, 2012 [PubMed: 21685814]

  299. Santana-Sosa et al., 2014

    Santana-Sosa, E., Gonzalez-Saiz, L., Groeneveld, I. F., Villa-Asensi, J. R., Barrio Gomez de Aguero, M. I., Fleck, S. J., Lopez-Mojares, L. M., Perez, M., Lucia, A., Benefits of combining inspiratory muscle with ‘whole muscle’ training in children with cystic fibrosis: a randomised controlled trial, British Journal of Sports Medicine, 48, 1513–7, 2014 [PubMed: 23681502]

  300. Savage et al., 2014

    Savage, E., Beirne, P. V., Ni Chroinin, M., Duff, A., Fitzgerald, T., Farrell, D., Self-management education for cystic fibrosis, Cochrane Database of Systematic Reviews, 9, CD007641, 2014 [PMC free article: PMC6481678] [PubMed: 25198249]

  301. Savant et al., 2014

    Savant, A. P., O’Malley, C., Bichl, S., McColley, S. A., Improved patient safety through reduced airway infection rates in a paediatric cystic fibrosis programme after a quality improvement effort to enhance infection prevention and control measures, BMJ Quality & Safety, 23, i73–i80, 2014 [PubMed: 24608553]

  302. Schaad et al., 1987

    Schaad, U. B., Wedgwood-Krucko, J., Suter, S., Kraemer, R., Efficacy of inhaled amikacin as adjunct to intravenous combination therapy (ceftazidime and amikacin) in cystic fibrosis, Journal of Pediatrics, 111, 599–605, 1987 [PubMed: 3309236]

  303. Schaad et al., 1989

    Schaad, U. B., Wedgwood-Krucko, J., Guenin, K., Buehlmann, U., Kraemer, R., Antipseudomonal therapy in cystic fibrosis: aztreonam and amikacin versus ceftazidime and amikacin administered intravenously followed by oral ciprofloxacin, European Journal of Clinical Microbiology & Infectious Diseases, 8, 858–65, 1989 [PubMed: 2512129]

  304. Schechter et al., 2015

    Schechter, M. S., Trueman, D., Farquharson, R., Higuchi, K., Daines, C. L., Inhaled Aztreonam Lysine versus Inhaled Tobramycin in Cystic Fibrosis. An Economic Evaluation, Annals of the American Thoracic Society, 12, 1030–8, 2015 [PubMed: 26053185]

  305. Schindel et al., 2015

    Schindel, C. S., Hommerding, P. X., Melo, D. A. S., Baptista, R. R., Marostica, P. J. C., Donadio, M. V. F., Physical exercise recommendations improve postural changes found in children and adolescents with cystic fibrosis: A randomized controlled trial, Journal of Pediatrics, 166, 710–716, 2015 [PubMed: 25596097]

  306. Schneider et al., 2006

    Schneider, V., Levesque, L. E., Zhang, B., Hutchinson, T., Brophy, J. M., Association of selective and conventional nonsteroidal antiinflammatory drugs with acute renal failure: A population-based, nested case-control analysis, American Journal of Epidemiology, 164, 881–9, 2006 [PubMed: 17005625]

  307. Schneiderman-Walker et al., 2000

    Schneiderman-Walker, J., Pollock, S. L., Corey, M., Wilkes, D. D., Canny, G. J., Pedder, L., Reisman, J. J., A randomized controlled trial of a 3-year home exercise program in cystic fibrosis, Journal of Pediatrics, 136, 304–10, 2000 [PubMed: 10700685]

  308. Schulze et al., 2006

    Schulze, K. J., Cutchins, C., Rosenstein, B. J., Germain-Lee, E. L., O’Brien, K. O., Calcium acquisition rates do not support age-appropriate gains in total body bone mineral content in prepuberty and late puberty in girls with cystic fibrosis, Osteoporosis International, 17, 731–40, 2006 [PubMed: 16505982]

  309. Schuster et al., 2013

    Schuster, A., Haliburn, C., Doring, G., Goldman, M. H., Freedom Study, Group, Safety, efficacy and convenience of colistimethate sodium dry powder for inhalation (Colobreathe DPI) in patients with cystic fibrosis: a randomised study, Thorax, 68, 344–50, 2013 [PMC free article: PMC3595146] [PubMed: 23135343]

  310. Seear & Wensley, 1997

    Seear, M., Wensley, D., Chronic cough and wheeze in children: do they all have asthma?, European Respiratory Journal, 10, 342–345, 1997 [PubMed: 9042630]

  311. Selvadurai et al., 2002

    Selvadurai, H. C., Blimkie, C. J., Meyers, N., Mellis, C. M., Cooper, P. J., Van Asperen, P. P., Randomized controlled study of in-hospital exercise training programs in children with cystic fibrosis, Pediatric Pulmonology, 33, 194–200, 2002 [PubMed: 11836799]

  312. Sermet-Gaudelus et al., 2011

    Sermet-Gaudelus, I., Bianchi, M. L., Garabedian, M., Aris, R. M., Morton, A., Hardin, D. S., Elkin, S. L., Compston, J. E., Conway, S. P., Castanet, M., Wolfe, S., Haworth, C. S., European cystic fibrosis bone mineralisation guidelines, Journal of Cystic Fibrosis, 10, Suppl 2, S16–23, 2011 [PubMed: 21658635]

  313. Shah et al., 1995

    Shah, P. I., Bush, A., Canny, G. J., Colin, A. A., Fuchs, H. J., Geddes, D. M., Johnson, C. A., Light, M. C., Scott, S. F., Tullis, D. E., et al, Recombinant human DNase I in cystic fibrosis patients with severe pulmonary disease: a short-term, double-blind study followed by six months open-label treatment, European Respiratory Journal, 8, 954–8, 1995 [PubMed: 7589382]

  314. Shah et al., 1996

    Shah, P. L., Scott, S. F., Knight, R. A., Marriott, C., Ranasinha, C., Hodson, M. E., In vivo effects of recombinant human DNase I on sputum in patients with cystic fibrosis, Thorax, 51, 119–25, 1996 [PMC free article: PMC473012] [PubMed: 8711640]

  315. Sharples et al., 2001

    Sharples, L. D., Taylor, G. J., Karnon, J., Caine, N., Buxton, M., McNeil, K., et al A model for analyzing the cost of the main clinical events after lung transplantation, Journal of Heart and Lung Transplant, 20, 474–82, 2001 [PubMed: 11295586]

  316. Shearer & Bryon, 2004

    Shearer, J. E., Bryon, M., The nature and prevalence of eating disorders and eating disturbance in adolescents with cystic fibrosis, Journal of the Royal Society of Medicine, 97 (Suppl 44), 36–42, 2004 [PMC free article: PMC1308797] [PubMed: 15239292]

  317. Sheldon et al., 1993

    Sheldon, C. D., Assoufi, B. K., Hodson, M. E., Regular three monthly oral ciprofloxacin in adult cystic fibrosis patients infected with P aeruginosa, Respiratory Medicine, 87, 587–93, 1993 [PubMed: 8290742]

  318. Silverstein et al., 2000

    Silverstein, F. E., Faich, G., Goldstein, J. L., Simon, L. S., Pincus, T., Whelton, A., Makuch, R., Eisen, G., Agrawal, N. M., Stenson, W. F., Burr, A. M., Zhao, W. W., Kent, J. D., Lefkowith, J. B., Verburg, K. M., Geis, G. S., Gastrointestinal toxicity with celecoxib vs nonsteroidal anti-inflammatory drugs for osteoarthritis and rheumatoid arthritis: the CLASS study: A randomized controlled trial. Celecoxib Long-term Arthritis Safety Study, JAMA, 284, 1247–55, 2000 [PubMed: 10979111]

  319. Siracusa et al., 2014

    Siracusa, C., Clancy, J. P., Drotar, D., Electronic monitoring reveals highly variable adherence patterns in patients prescribed ivacaftor, Pediatric Pulmonology, 49, 440, 2014 [PMC free article: PMC4567928] [PubMed: 26074007]

  320. Smyth et al., 2014

    Smyth, A. R., Bell, Scott C., Bojcin, Snezana, Bryon, Mandy, Duff, Alistair, Flume, Patrick, Kashirskaya, Nataliya, Munck, Anne, Ratjen, Felix, Schwarzenberg, Sarah Jane, Sermet-Gaudelus, Isabelle, Southern, Kevin W., Taccetti, Giovanni, Ullrich, Gerald, Wolfe, Sue, European Cystic Fibrosis Society Standards of Care: Best Practice guidelinesmoran, Journal of Cystic Fibrosis, 13, Suppl 1, S23–S42, 2014 [PubMed: 24856775]

  321. Smyth & Rayner, 2014a

    Smyth, R. L., Rayner, O., Oral calorie supplements for cystic fibrosis, Cochrane Database of Systematic Reviews, 11, CD000406, 2014 [PubMed: 25363148]

  322. Smyth & Walters, 2014

    Smyth, A. R., Walters, S., Prophylactic anti-staphylococcal antibiotics for cystic fibrosis, Cochrane Database of Systematic Reviews, 11, CD001912, 2014 [PubMed: 25419599]

  323. Solem et al., 2014

    Solem, C. T., Vera Llonch, M., Liu, S., Botteman, M. F., Lasch, K., Rodriguez, S., Castiglione, B., Responsiveness Of The Eq-5d Index And Visual Analog Scale To Changes In Lung Function In Patients With Cystic Fibrosis, Value in Health, 17, A178, 2014

  324. Solem et al., 2014a

    Solem, C. T., Vera-Llonch, M., Liu, S., Botteman, M., Lin, F. J., Castiglione, B., Impact of pulmonary exacerbations on EQ-5D measures in patients with cystic fibrosis, Value in Health, 17 (7), A535, 2014 [PubMed: 27201711]

  325. Somerville et al., 2013

    Somerville, R., Lackson, A., Zhou, S., Fletcher, C., Fitzpatrick, P., Non-pulmonary chronic diseases in adults with cystic fibrosis: analysis of data from the Cystic Fibrosis Registry, Irish Medical Journal, 106, 166–8, 2013 [PubMed: 23909150]

  326. Sordelli et al., 1994

    Sordelli, D.O., Macri, C.N., Maillie, A.J., Cerquetti, M.C., A preliminary study on the effect of anti-inflammatory treatment in cystic fibrosis patients with P aeruginosa lung infection, International Journal of Immunopathology and Pharmacology, 7, 109–117, 1994

  327. South Devon Healthcare NHS Foundation Trust, 2015

    South Devon Healthcare NHS Foundation Trust, Private patient and overseas visitor price list, 2015, Torquay: South Devon Healthcare NHS Foundation Trust

  328. Southampton Children’s Hospital, 2014

    Southampton Children’s Hospital, Transition to adult care: ready steady go, from http://www​.uhs.nhs.uk​/Ourservices/Childhealth​/TransitiontoadultcareReadySteadyGo​/Transitiontoadultcare.aspx, 2014

  329. Southern et al., 2012

    Southern, Kevin W., Barker, Pierre M., SolisMoya, Arturo, Patel, Latifa, Macrolide antibiotics for cystic fibrosis, Cochrane Database of Systematic Reviews, 11, CD002203, 2012 [PMC free article: PMC7098459] [PubMed: 23152214]

  330. Stark et al., 1996

    Stark, L. J., Mulvihill, M. M., Powers, S. W., Jelalian, E., Keating, K., Creveling, S., Byrnes-Collins, B., Harwood, I., Passero, M. A., Light, M., Miller, D. L., Hovell, M. F., Behavioral intervention to improve calorie intake of children with cystic fibrosis: treatment versus wait list control, Journal of Pediatric Gastroenterology & Nutrition, 22, 240–53, 1996 [PubMed: 8708877]

  331. Stark et al., 2009

    Stark, L. J., Quittner, A. L., Powers, S. W., Opipari-Arrigan, L., Bean, J. A., Duggan, C., Stallings, V. A., Randomized clinical trial of behavioral intervention and nutrition education to improve caloric intake and weight in children with cystic fibrosis, Archives of Pediatrics & Adolescent Medicine, 163, 915–21, 2009 [PMC free article: PMC2811078] [PubMed: 19805710]

  332. Stephenson et al., 2013

    Stephenson, A. L., Mannik, L. A., Walsh, S., Brotherwood, M., Robert, R., Darling, P. B., Nisenbaum, R., Moerman, J., Stanojevic, S., Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: a population-based cohort study, American Journal of Clinical Nutrition, 97, 872–7, 2013 [PubMed: 23388659]

  333. Stevens, 2011

    Stevens, J. W., A note on dealing with missing standard errors in meta-analyses of continuous outcome measures in WinBUGS, Pharmaceutical Statistics, 10, 374–8, 2011 [PubMed: 21394888]

  334. Stutman et al., 2002

    Stutman, H. R., Lieberman, J. M., Nussbaum, E., Marks, M. I., Antibiotic prophylaxis in infants and young children with cystic fibrosis: a randomized controlled trial, Journal of Pediatrics, 140, 299–305, 2002 [PubMed: 11953726]

  335. Suri et al., 2001

    Suri, R., Metcalfe, C., Lees, B., Grieve, R., Flather, M., Normand, C., Thompson, S., Bush, A., Wallis, C., Comparison of hypertonic saline and alternate-day or daily recombinant human deoxyribonuclease in children with cystic fibrosis: a randomised trial, Lancet, 358, 1316–21, 2001 [PubMed: 11684212]

  336. Suri et al., 2002

    Suri, R., Grieve, R., Normand, C., Metcalfe, C., Thompson, S., Wallis, C., Bush, A., Effects of hypertonic saline, alternate day and daily rhDNase on healthcare use, costs and outcomes in children with cystic fibrosis, Thorax, 57, 841–6, 2002 [PMC free article: PMC1746194] [PubMed: 12324668]

  337. Suri et al., 2002a

    Suri, R., Wallis, C., Bush, A., Thompson, S., Normand, C., Flather, M., Grieve, R., Metcalfe, C., Lees, B., A comparative study of hypertonic saline, daily and alternate-day rhDNase in children with cystic fibrosis, Health Technology Assessment (Winchester, England), 6, 2002 [PubMed: 12583821]

  338. Suri et al., 2002b

    Suri, R., Marshall, L.J., Wallis, C., Metcalfe, C., Bush, A., Shute, J.K., Effects of recombinant human DNase and hypertonic saline on airway inflammation in children with cystic fibrosis, American Journal of Respiratory and Critical Care Medicine, 166, 352–355, 2002 [PubMed: 12153969]

  339. Taccetti et al., 2012

    Taccetti, G., Bianchini, E., Cariani, L., Buzzetti, R., Costantini, D., Trevisan, F., Zavataro, L., Campana, S., Italian Group for, P aeruginosa Eradication in Cystic Fibrosis, Early antibiotic treatment for P aeruginosa eradication in patients with cystic fibrosis: a randomised multicentre study comparing two different protocols, Thorax, 67, 853–9, 2012 [PubMed: 22379071]

  340. Tajima et al., 2010

    Tajima, R., Kondo, M., Kai, H., Saito, C., Okada, M., Takahashi, H., Doi, M., Tsuruoka, S., Yamagata, K., Measurement of health-related quality of life in patients with chronic kidney disease in Japan with EuroQol (EQ-5D), Clinical & Experimental Nephrology, 14, 340–8, 2010 [PubMed: 20567874]

  341. Tannenbaum et al., 2005

    Tannenbaum, E, Prasad, SA, Main, E, Scrase, E, 374 Long term effects of positive expiratory pressure (PEP) or oscillatory positive pressure (RC cornet®) on FEV1 and perceived health in children with CF, Journal of Cystic Fibrosis, 4, S100, 2005

  342. Tappenden et al., 2013

    Tappenden, P., Harnan, S., Uttley, L., Mildred, M., Carroll, C., Cantrell, A., Colistimethate sodium powder and tobramycin powder for inhalation for the treatment of chronic P aeruginosa lung infection in cystic fibrosis: systematic review and economic model, Health Technology Assessment (Winchester, England), 17, v–xvii, 2013 [PMC free article: PMC4781138] [PubMed: 24290164]

  343. Tappenden et al., 2014

    Tappenden, P., Harnan, S., Uttley, L., Mildred, M., Walshaw, M., Taylor, C., Brownlee, K., The cost effectiveness of dry powder antibiotics for the treatment of P aeruginosa in patients with cystic fibrosis, Pharmacoeconomics, 32, 159–72, 2014 [PubMed: 24338264]

  344. The Cochrane Collaboration, 2011

    The Cochrane Collaboration, The Cochrane Collaboration’s tool for assessing risk of bias, 2011

  345. Thomas et al., 2006

    Thomas, C., Mitchell, P., O’Rourke, P., Wainwright, C., Quality-of-life in children and adolescents with cystic fibrosis managed in both regional outreach and cystic fibrosis center settings in Queensland, Journal of Pediatrics, 148, 508–516, 2006 [PubMed: 16647415]

  346. Thomas et al., 2008

    Thomas, C. L., O’Rourke, P. K., Wainwright, C. E., Clinical outcomes of Queensland children with cystic fibrosis: a comparison between tertiary centre and outreach services, Medical Journal of Australia, 188, 135–9, 2008 [PubMed: 18241167]

  347. Thomassen et al., 1986

    Thomassen, M. J., Demko, C. A., Doershuk, C. F., Stern, R. C., Klinger, J. D., Pseudomonas cepacia: decrease in colonization in patients with cystic fibrosis, American Review of Respiratory Disease, 134, 669–71, 1986 [PubMed: 3767122]

  348. Thornton et al., 2005

    Thornton, J., Elliott, R. A., Tully, M. P., Dodd, M., Webb, A. K., Clinical and economic choices in the treatment of respiratory infections in cystic fibrosis: comparing hospital and home care, Journal of Cystic Fibrosis, 4, 239–47, 2005 [PubMed: 16242385]

  349. Tierney et al., 2013

    Tierney, S., Deaton, C., Jones, A., Oxley, H., Biesty, J., Kirk, S., Liminality and transfer to adult services: a qualitative investigation involving young people with cystic fibrosis, International Journal of Nursing Studies, 50, 738–46, 2013 [PubMed: 22624956]

  350. Tipping et al., 2010

    Tipping, C. J., Scholes, R. L., Cox, N. S., A qualitative study of physiotherapy education for parents of toddlers with cystic fibrosis, Journal of Cystic Fibrosis, 9, 205–11, 2010 [PubMed: 20359964]

  351. Tluczek et al., 2006

    Tluczek, A., Koscik, R. L., Modaff, P., Pfeil, D., Rock, M. J., Farrell, P. M., Lifchez, C., Freeman, M. E., Gershan, W., Zaleski, C., Sullivan, B., Newborn screening for cystic fibrosis: parents’ preferences regarding counseling at the time of infants’ sweat test, Journal of Genetic Counseling, 15, 277–91, 2006 [PubMed: 16865559]

  352. Tluczek et al., 2009

    Tluczek, A., Orland, K. M., Nick, S. W., Brown, R. L., Newborn screening: an appeal for improved parent education, Journal of Perinatal & Neonatal Nursing, 23, 326–34, 2009 [PMC free article: PMC2947955] [PubMed: 19915416]

  353. Trapnell et al., 2012

    Trapnell, B. C., McColley, S. A., Kissner, D. G., Rolfe, M. W., Rosen, J. M., McKevitt, M., Moorehead, L., Montgomery, A. B., Geller, D. E., Phase, F. T. I. Study Group, Fosfomycin/tobramycin for inhalation in patients with cystic fibrosis with pseudomonas airway infection, American Journal of Respiratory & Critical Care Medicine, 185, 171–8, 2012 [PMC free article: PMC3361752] [PubMed: 22095545]

  354. Tuchman et al., 2008

    Tuchman, L. K., Slap, G. B., Britto, M. T., Transition to adult care: experiences and expectations of adolescents with a chronic illness, Child: Care, Health & Development, 34, 557–63, 2008 [PubMed: 18796047]

  355. Tunis & Minshall, 2010

    Tunis, S. L., Minshall, M. E., Self-monitoring of blood glucose (SMBG) for type 2 diabetes patients treated with oral anti-diabetes drugs and with a recent history of monitoring: cost-effectiveness in the US, Current Medical Research and Opinion, 26, 151–62, 2010 [PubMed: 19919376]

  356. Tunis et al., 2010

    Tunis, S. L., Sauriol, L., Minshall, M. E., Cost effectiveness of insulin glargine plus oral antidiabetes drugs compared with premixed insulin alone in patients with type 2 diabetes mellitus in Canada, Applied Health Economics and Health Policy, 8, 267–80, 2010 [PubMed: 20578781]

  357. Turner et al., 2012

    Turner, Rebecca M., Davey, Jonathan, Clarke, Mike J., Thompson, Simon G., Higgins, Julian P. T., Predicting the extent of heterogeneity in meta-analysis, using empirical data from the Cochrane Database of Systematic Reviews, International Journal of Epidemiology, 41, 818–827, 2012 [PMC free article: PMC3396310] [PubMed: 22461129]

  358. Van Gestel et al., 2010

    Van Gestel, Aukje, Severens, Johan L., Webers, Carroll A. B., Beckers, Henny J. M., Jansonius, Nomdo M., Schouten, Jan S. A. G., Modeling Complex Treatment Strategies: Construction and Validation of a Discrete Event Simulation Model for Glaucoma, Value in Health, 13, 358–367, 2010 [PubMed: 20659272]

  359. Van Koolwijk et al., 2002

    Van Koolwijk, L. M. E., Uiterwaal, C. S. P. M., Van der Laag, J., Hoekstra, J. H., Gulmans, V. A. M., Van der Ent, C. K., Treatment of children with cystic fibrosis: Central, local or both?, Acta Paediatrica, 91, 972–977, 2002 [PubMed: 12412875]

  360. Van Staa et al., 2011

    Van Staa, A. L., Jedeloo, S., van Meeteren, J., Latour, J. M., Crossing the transition chasm: experiences and recommendations for improving transitional care of young adults, parents and providers, Child: Care, Health & Development, 37, 821–32, 2011 [PubMed: 22007982]

  361. Van Valkenhoef et al., 2016

    Van Valkenhoef, Gert, Dias, Sofia, Ades, A. E., Welton, Nicky J., Automated generation of node-splitting models for assessment of inconsistency in network meta-analysis, Research Synthesis Methods, 7, 80–93, 2016 [PMC free article: PMC5057346] [PubMed: 26461181]

  362. Van Winden et al., 1998

    Van Winden, C. M., Visser, A., Hop, W., Sterk, P. J., Beckers, S., de Jongste, J. C., Effects of flutter and PEP mask physiotherapy on symptoms and lung function in children with cystic fibrosis, European Respiratory Journal, 12, 143–7, 1998 [PubMed: 9701429]

  363. Vieni et al., 2013

    Vieni, G., Faraci, S., Collura, M., Lombardo, M., Traverso, G., Cristadoro, S., Termini, L., Lucanto, M. C., Furnari, M. L., Trimarchi, G., Triglia, M. R., Costa, S., Pellegrino, S., Magazzu, G., Stunting is an independent predictor of mortality in patients with cystic fibrosis, Clinical Nutrition, 32, 382–5, 2013 [PubMed: 22974535]

  364. Waine et al., 2007

    Waine, D. J., Whitehouse, J., Honeybourne, D., Cross-infection in cystic fibrosis: the knowledge and behaviour of adult patients, Journal of Cystic Fibrosis, 6, 262–6, 2007 [PubMed: 17141578]

  365. Wainwright et al., 2011

    Wainwright, C. E., Vidmar, S., Armstrong, D. S., Byrnes, C. A., Carlin, J. B., Cheney, J., Cooper, P. J., Grimwood, K., Moodie, M., Robertson, C. F., Tiddens, H. A., Acfbal Study Investigators, Effect of bronchoalveolar lavage-directed therapy on P aeruginosa infection and structural lung injury in children with cystic fibrosis: a randomized trial, JAMA, 306, 163–71, 2011 [PubMed: 21750293]

  366. Wainwright et al., 2011a

    Wainwright, C.E., Quittner, A.L., Geller, D.E., Nakamura, C., Wooldridge, J.L., Gibson, R.L., Lewis, S., Montgomery, A.B., Aztreonam for inhalation solution (AZLI) in patients with cystic fibrosis, mild lung impairment, and P aeruginosa, Journal of Cystic Fibrosis, 10, 234–242, 2011 [PubMed: 21441078]

  367. Walters et al., 1994

    Walters, S., Britton, J., Hodson, M. E., Hospital care for adults with cystic fibrosis: an overview and comparison between special cystic fibrosis clinics and general clinics using a patient questionnaire, Thorax, 49, 300–6, 1994 [PMC free article: PMC475360] [PubMed: 8202897]

  368. Wark & McDonald, 2009

    Wark, Peter, McDonald, Vanessa M., Nebulised hypertonic saline for cystic fibrosis, Cochrane Database of Systematic Reviews, 2, CD001506, 2009 [PubMed: 19370568]

  369. Wark & McDonald, 2010

    Wark, Peter, McDonald, Vanessa M., Nebulised hypertonic saline for cystic fibrosis, Cochrane Database of Systematic Reviews, 6, CD001506, 2010 [PubMed: 12535409]

  370. Warnock et al., 2013

    Warnock, L., Gates, A., van der Schans, C. P., Chest physiotherapy compared to no chest physiotherapy for cystic fibrosis, Cochrane Database of Systematic Reviews, 9, CD001401, 2013 [PubMed: 24006212]

  371. Warwick et al., 2004

    Warwick, W. J., Wielinski, C. L., Hansen, L. G., Comparison of expectorated sputum after manual chest physical therapy and high-frequency chest compression, Biomedical Instrumentation & Technology, 38, 470–5, 2004 [PubMed: 15635998]

  372. Waters & Ratjen, 2014

    Waters, V., Ratjen, F., Antibiotic treatment for nontuberculous mycobacteria lung infection in people with cystic fibrosis, Cochrane Database of Systematic Reviews, 12, CD010004, 2014 [PubMed: 25470304]

  373. Waters & Ratjen, 2016

    Waters, Valerie, Ratjen, Felix, Antibiotic treatment for nontuberculous mycobacteria lung infection in people with cystic fibrosis, Cochrane Database of Systematic Reviews, 12, CD010004, 2016 [PMC free article: PMC6464024] [PubMed: 28000919]

  374. Watson et al., 2008

    Watson, H., Bilton, D., Truby, H., A randomized controlled trial of a new behavioral home-based nutrition education program, “Eat Well with CF,” in adults with cystic fibrosis, Journal of the American Dietetic Association, 108, 847–52, 2008 [PubMed: 18442509]

  375. Watts et al., 2009

    Watts, K. D., Seshadri, R., Sullivan, C., McColley, S. A., Increased prevalence of risk factors for morbidity and mortality in the US Hispanic CF population, Pediatric Pulmonology, 44, 594–601, 2009 [PubMed: 19437506]

  376. Waugh et al., 2012

    Waugh, N., Royle, P., Craigie, I., Ho, V., Pandit, L., Ewings, P., Adler, A., Helms, P., Sheldon, C., Screening for cystic fibrosis-related diabetes: a systematic review, Health Technology Assessment (Winchester, England), 16, 24, iii–iv, 1–179, 2012 [PMC free article: PMC4781640] [PubMed: 22572153]

  377. Weaver et al., 1994

    Weaver, L.T., Green, M.R., Nicholson, K., Mills, J., Heeley, M.E., Kuzemko, J.A., Austin, S., Gregory, G.A., Dux, A.E., Davis, J.A., Prognosis in cystic fibrosis treated with continuous flucloxacillin from the neonatal period, Archives of Disease in Childhood, 70, 84–89, 1994 [PMC free article: PMC1029705] [PubMed: 8129449]

  378. Wells et al., 2008

    Wells, G. A., Shea, B., O’Connell, D., Peterson, J., Welch, V., Losos, M., Tugwell, P., Ottawa Hospital Research Institute, The Newcastle-Ottawa Scale (NOS) for assessing the quality of nonrandomised studies in meta-analyses, 2008, Ottawa: Ottawa Hospital Research Institute

  379. Wesley et al., 1988

    Wesley, A. W., Quested, C., Edgar, B. W., Lennon, D. R., A double-blind comparison of ceftazidime with tobramycin and ticarcillin in the treatment of exacerbations of pseudomonas chest infection in children with cystic fibrosis, 10th International Cystic Fibrosis Congress, 1988, [Abstract]

  380. White et al., 2013

    White, H., Morton, A. M., Conway, S. P., Peckham, D. G., Enteral tube feeding in adults with cystic fibrosis; patient choice and impact on long term outcomes, Journal of Cystic Fibrosis, 12, 616–22, 2013 [PubMed: 23770301]

  381. White et al., 2014

    White, H., Denman, S., Shaw, N., Pollard, K., Peckham, D., Do longitudinal measures of clinical variation correlate with adherence in cystic fibrosis, Pediatric Pulmonology, 49, 437, 2014

  382. Whiteford et al., 1995

    Whiteford, M. L., Wilkinson, J. D., McColl, J. H., Conlon, F. M., Michie, J. R., Evans, T. J., Paton, J. Y., Outcome of Burkholderia (Pseudomonas) cepacia colonisation in children with cystic fibrosis following a hospital outbreak, Thorax, 50, 1194–8, 1995 [PMC free article: PMC475093] [PubMed: 8553277]

  383. Whiting et al., 2014

    Whiting, P., Al, M., Burgers, L., Westwood, M., Ryder, S., Hoogendoorn, M., Armstrong, N., Allen, A., Severens, H., Kleijnen, J., Ivacaftor for the treatment of patients with cystic fibrosis and the G551D mutation: a systematic review and cost-effectiveness analysis, Health Technology Assessment (Winchester, England), 18, 1–106, 2014 [PMC free article: PMC4780965] [PubMed: 24656117]

  384. Whyte et al., 1995

    Whyte, D. A., Baggaley, S., Rutter, C., Chronic illness in childhood: A comparative study of family support across four diagnostic groups, Physiotherapy, 81, 515–520, 1995

  385. Widerman, 2002

    Widerman, E., Communicating a diagnosis of cystic fibrosis to an adult: what physicians need to know, Behavioral Medicine, 28, 45–52, 2002 [PubMed: 12613285]

  386. Widerman, 2003

    Widerman, E., Knowledge, interests and educational needs of adults diagnosed with cystic fibrosis after age 18, Journal of Cystic Fibrosis, 2, 97–104, 2003 [PubMed: 15463857]

  387. Widerman, 2004

    Widerman, E., The experience of receiving a diagnosis of cystic fibrosis after age 20: Implications for social work, Social Work in Health Care, 39, 415–433, 2004 [PubMed: 15774404]

  388. Wiedemann et al., 2001

    Wiedemann, B., Steinkamp, G., Sens, B., Stern, M., German Cystic Fibrosis Quality Assurance, Group, The German cystic fibrosis quality assurance project: clinical features in children and adults, European Respiratory Journal, 17, 1187–94, 2001 [PubMed: 11491163]

  389. Wilcock et al., 2015

    Wilcock, M. J., Ruddick, A., Gyi, K. M., Hodson, M. E., Renal diseases in adults with cystic fibrosis: a 40 year single centre experience, Journal of Nephrology, 28, 585–91, 2015 [PubMed: 25712235]

  390. Wilkinson et al., 2008

    Wilkinson, O. M., Duncan-Skingle, F., Pryor, J. A., Hodson, M. E., A feasibility study of home telemedicine for patients with cystic fibrosis awaiting transplantation, Journal of Telemedicine & Telecare, 14, 182–5, 2008 [PubMed: 18534951]

  391. Wilmott et al., 1996

    Wilmott, R. W., Amin, R. S., Colin, A. A., DeVault, A., Dozor, A. J., Eigen, H., Johnson, C., Lester, L. A., McCoy, K., McKean, L. P., Moss, R., Nash, M. L., Jue, C. P., Regelmann, W., Stokes, D. C., Fuchs, H. J., Aerosolized recombinant human DNase in hospitalized cystic fibrosis patients with acute pulmonary exacerbations, American Journal of Respiratory & Critical Care Medicine, 153, 1914–7, 1996 [PubMed: 8665055]

  392. Witters et al., 2009

    Witters, P., De Boeck, K., Dupont, L., Proesmans, M., Vermeulen, F., Servaes, R., Verslype, C., Laleman, W., Nevens, F., Hoffman, I., Cassiman, D., Non-invasive liver elastography (Fibroscan) for detection of cystic fibrosis-associated liver disease, Journal of Cystic Fibrosis, 8, 392–9, 2009 [PubMed: 19733131]

  393. Woestenbenk et al., 2013

    Woestenenk, J. W., Castelijns, S. J. A. M., van der Ent, C. K., Houwen, R. H. J., Nutritional intervention in patients with Cystic Fibrosis: A systematic review., Journal of Cystic Fibrosis, 12, 102–115, 2013 [PubMed: 23232324]

  394. Wolter et al., 1997

    Wolter, J. M., Bowler, S. D., Nolan, P. J., McCormack, J. G., Home intravenous therapy in cystic fibrosis: a prospective randomized trial examining clinical, quality of life and cost aspects, European Respiratory Journal, 10, 896–900, 1997 [PubMed: 9150331]

  395. Wolter et al., 2002

    Wolter, J., Seeney, S., Bell, S., Bowler, S., Masel, P., McCormack, J., Effect of long term treatment with azithromycin on disease parameters in cystic fibrosis: a randomised trial, Thorax, 57, 212–6, 2002 [PMC free article: PMC1746273] [PubMed: 11867823]

  396. Woodruff et al., 2016

    Woodruff, S. A., Sontag, M. K., Accurso, F. J., Sokol, R. J., Narkewicz, M. R., Prevalence of elevated liver enzymes in children with cystic fibrosis diagnosed by newborn screen, Journal of Cystic Fibrosis, no pagination, 2016 [PubMed: 27555301]

  397. Yang et al., 2016

    Yang, C., Chilvers, M., Montgomery, M., Nolan, S. J., Dornase alfa for cystic fibrosis, Cochrane Database of Systematic Reviews, 4, CD001127, 2016 [PubMed: 27043279]

  398. Yi et al., 2003

    Yi, M. S., Britto, M. T., Wilmott, R. W., Kotagal, U. R., Eckman, M. H., Nielson, D. W., Kociela, V. L., Tsevat, J., Health values of adolescents with cystic fibrosis, Journal of Pediatrics, 142, 133–40, 2003 [PubMed: 12584533]

  399. Young et al., 2008

    Young, A. C., Wilson, J. W., Kotsimbos, T. C., Naughton, M. T., Randomised placebo controlled trial of non-invasive ventilation for hypercapnia in cystic fibrosis, Thorax, 63, 72–7, 2008 [PubMed: 17675317]

  400. Zhang et al., 2010

    Zhang, Z., Shoff, S. M., Lai, H. J., Incorporating genetic potential when evaluating stature in children with cystic fibrosis, Journal of Cystic Fibrosis, 9, 135–42, 2010 [PMC free article: PMC2834199] [PubMed: 20138592]

  401. Zhang et al., 2013

    Zhang, Z., Lindstrom, M. J., Lai, H. J., Pubertal height velocity and associations with prepubertal and adult heights in cystic fibrosis, Journal of Pediatrics, 163, 376–82, 2013 [PMC free article: PMC3700583] [PubMed: 23535012]
Copyright © NICE 2017.
Bookshelf ID: NBK535667
Contents

Views

Other titles in this collection

Related information

  • PMC
    PubMed Central citations
  • PubMed
    Links to PubMed

Recent Activity

ClearTurn OffTurn On

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...