Cystic fibrosis

Farrell PM, White TB, Ren CL, et al. Diagnosis of cystic fibrosis: consensus guidelines from the Cystic Fibrosis Foundation. J Pediatr. 2017 Feb;181S:S4-15.e1.Full text  Abstract

Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013 Apr 1;187(7):680-9.Full text  Abstract

Flume PA, Robinson KA, O'Sullivan BP, et al; Clinical Practice Guidelines for Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respir Care. 2009 Apr;54(4):522-37.Full text  Abstract

Kapnadak SG, Dimango E, Hadjiliadis D, et al. Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease. J Cyst Fibros. 2020 May;19(3):344-54.Full text  Abstract

Ramos KJ, Smith PJ, McKone EF, et al. Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines. J Cyst Fibros. 2019 May;18(3):321-33.Full text  Abstract

Borowitz D, Robinson KA, Rosenfeld M, et al; Cystic Fibrosis Foundation. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009 Dec;155(suppl 6):S73-93.Full text  Abstract

1. Cystic fibrosis mutation database. Apr 2011 [internet publication].Full text

2. Southern KW, Murphy J, Sinha IP, et al. Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del). Cochrane Database Syst Rev. 2020 Dec 17;(12):CD010966.Full text  Abstract

3. Orenstein DM, Rosenstein BJ, Stern RC. Cystic fibrosis: medical care. Philadelphia, PA: Lippincott Williams & Wilkins; 2000:365.

4. The Cystic Fibrosis Foundation. About cystic fibrosis. 2022 [internet publication].Full text

5. Eigen H, Rosenstein BJ, FitzSimmons S, et al. A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group. J Pediatr. 1995 Apr;126(4):515-23. Abstract

6. Hale JE, Parad RB, Comeau AM. Newborn screening showing decreasing incidence of cystic fibrosis. N Engl J Med. 2008 Feb 28;358(9):973-4. Abstract

7. Scotet V, L'Hostis C, Férec C. The changing epidemiology of cystic fibrosis: incidence, survival and impact of the CFTR gene discovery. Genes (Basel). 2020 May 26;11(6):589.Full text  Abstract

8. Cystic Fibrosis Foundation. 2020 patient registry annual data report. Bethesda, MD: Cystic Fibrosis Foundation; 2021.Full text

9. Tarran R, Button B, Picher M, et al. Normal and cystic fibrosis airway surface liquid homeostasis. The effects of phasic shear stress and viral infections. J Biol Chem. 2005 Oct 21;280(42):35751-9.Full text  Abstract

10. Moskwa P, Lorentzen D, Excoffon KJ, et al. A novel host defense system of airways is defective in cystic fibrosis. Am J Respir Crit Care Med. 2007 Jan 15;175(2):174-83.Full text  Abstract

11. Matsui H, Grubb BR, Tarran R, et al. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell. 1998 Dec 23;95(7):1005-15. Abstract

12. Knowles MR, Stutts MJ, Spock A, et al. Abnormal ion permeation through cystic fibrosis respiratory epithelium. Science. 1983 Sep 9;221(4615):1067-70. Abstract

13. Knowles M, Gatzy J, Boucher R. Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N Engl J Med. 1981 Dec 17;305(25):1489-95. Abstract

14. Joo NS, Irokawa T, Robbins RC, et al. Hyposecretion, not hyperabsorption, is the basic defect of cystic fibrosis airway glands. J Biol Chem. 2006 Mar 17;281(11):7392-8.Full text  Abstract

15. Conner GE, Wijkstrom-Frei C, Randell SH, et al. The lactoperoxidase system links anion transport to host defense in cystic fibrosis. FEBS Lett. 2007 Jan 23;581(2):271-8.Full text  Abstract

16. Boucher RC. New concepts of the pathogenesis of cystic fibrosis lung disease. Eur Respir J. 2004 Jan;23(1):146-58.Full text  Abstract

17. Bals R, Weiner DJ, Meegalla RL, et al. Salt-independent abnormality of antimicrobial activity in cystic fibrosis airway surface fluid. Am J Respir Cell Mol Biol. 2001 Jul;25(1):21-5.Full text  Abstract

18. Ballard ST, Trout L, Mehta A, et al. Liquid secretion inhibitors reduce mucociliary transport in glandular airways. Am J Physiol Lung Cell Mol Physiol. 2002 Aug;283(2):L329-35.Full text  Abstract

19. Farrell PM, White TB, Ren CL, et al. Diagnosis of cystic fibrosis: consensus guidelines from the Cystic Fibrosis Foundation. J Pediatr. 2017 Feb;181S:S4-15.e1.Full text  Abstract

20. Comeau AM, Accurso FJ, White TB, et al. Guidelines for implementation of cystic fibrosis newborn screening programs: Cystic Fibrosis Foundation workshop report. Pediatrics. 2007 Feb;119(2):e495-518. Abstract

21. Castellani C, Southern KW, Brownlee K, et al. European best practice guidelines for cystic fibrosis neonatal screening. J Cyst Fibros. 2009 May;8(3):153-73. Abstract

22. Sermet-Gaudelus I, Munck A, Rota M, et al. French guidelines for sweat test practice and interpretation for cystic fibrosis neonatal screening [in French]. Arch Pediatr. 2010 Sep;17(9):1349-58. Abstract

23. Sermet-Gaudelus I, Mayell SJ, Southern KW, et al; European Cystic Fibrosis Society, Neonatal Screening Working Group. Guidelines on the early management of infants diagnosed with cystic fibrosis following newborn screening. J Cyst Fibros. 2010 Sep;9(5):323-9. Abstract

24. Castellani C, Duff AJA, Bell SC, et al. ECFS best practice guidelines: the 2018 revision. J Cyst Fibros. 2018 Mar;17(2):153-78.Full text  Abstract

25. National Institute for Health and Care Excellence. Cystic fibrosis: diagnosis and management. Oct 2017 [internet publication].Full text

26. Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013 Apr 1;187(7):680-9.Full text  Abstract

27. Wilson LM, Morrison L, Robinson KA. Airway clearance techniques for cystic fibrosis: an overview of Cochrane systematic reviews. Cochrane Database Syst Rev. 2019 Jan 24;(1):CD011231.Full text  Abstract

28. Mckoy NA, Wilson LM, Saldanha IJ, et al. Active cycle of breathing technique for cystic fibrosis. Cochrane Database Syst Rev. 2016 Jul 5;(7):CD007862.Full text  Abstract

29. Flume PA, Robinson KA, O'Sullivan BP, et al; Clinical Practice Guidelines for Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respir Care. 2009 Apr;54(4):522-37.Full text  Abstract

30. Morrison L, Milroy S. Oscillating devices for airway clearance in people with cystic fibrosis. Cochrane Database Syst Rev. 2020 Apr 30;(4):CD006842.Full text  Abstract

31. Cystic Fibrosis Trust (UK). Standards of care and good clinical practice for the physiotherapy management of cystic fibrosis. Fourth ed. Nov 2020 [internet publication].Full text

32. McIlwaine M, Button B, Nevitt SJ. Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis. Cochrane Database Syst Rev. 2019 Nov 27;(11):CD003147.Full text  Abstract

33. Hill AT, Barker AF, Bolser DC, et al. Treating cough due to non-CF and CF bronchiectasis with nonpharmacological airway clearance: CHEST Expert Panel report. Chest. 2018 Apr;153(4):986-93.Full text  Abstract

34. Moran F, Bradley JM, Piper AJ. Non-invasive ventilation for cystic fibrosis. Cochrane Database Syst Rev. 2017 Feb 20;(2):CD002769.Full text  Abstract

35. Yang C, Montgomery M. Dornase alfa for cystic fibrosis. Cochrane Database Syst Rev. 2021 Mar 18;(3):CD001127.Full text  Abstract

36. Elkins MR, Robinson M, Rose BR, et al; National Hypertonic Saline in Cystic Fibrosis (NHSCF) Study Group. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med. 2006 Jan 19;354(3):229-40.Full text  Abstract

37. Wark P, McDonald VM. Nebulised hypertonic saline for cystic fibrosis. Cochrane Database Syst Rev. 2018 Sep 27;(9):CD001506.Full text  Abstract

38. Ratjen F, Davis SD, Stanojevic S, et al. Inhaled hypertonic saline in preschool children with cystic fibrosis (SHIP): a multicentre, randomised, double-blind, placebo-controlled trial. Lancet Respir Med. 2019 Sep;7(9):802-9. Abstract

39. Langton Hewer SC, Smyth AR. Antibiotic strategies for eradicating Pseudomonas aeruginosa in people with cystic fibrosis. Cochrane Database Syst Rev. 2017 Apr 25;(4):CD004197.Full text  Abstract

40. Palser S, Smith S, Nash EF, et al. Treatments for preventing recurrence of infection with Pseudomonas aeruginosa in people with cystic fibrosis. Cochrane Database Syst Rev. 2019 Dec 17;(12):CD012300.Full text  Abstract

41. Rosenfeld M, Rayner O, Smyth AR. Prophylactic anti-staphylococcal antibiotics for cystic fibrosis. Cochrane Database Syst Rev. 2020 Sep 30;(9):CD001912.Full text  Abstract

42. Lo DK, Muhlebach MS, Smyth AR. Interventions for the eradication of meticillin-resistant Staphylococcus aureus (MRSA) in people with cystic fibrosis. Cochrane Database Syst Rev. 2018 Jul 21;(7):CD009650.Full text  Abstract

43. Balfour-Lynn IM, Welch K, Smith S. Inhaled corticosteroids for cystic fibrosis. Cochrane Database Syst Rev. 2019 Jul 4;(7):CD001915.Full text  Abstract

44. Dovey M, Aitken ML, Emerson J, et al. Oral corticosteroid therapy in cystic fibrosis patients hospitalized for pulmonary exacerbation: a pilot study. Chest. 2007 Oct;132(4):1212-8. Abstract

45. Auerbach HS, Williams M, Kirkpatrick JA, et al. Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis. Lancet. 1985 Sep 28;2(8457):686-8. Abstract

46. Saiman L, Marshall BC, Mayer-Hamblett N, et al; Macrolide Study Group. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA. 2003 Oct 1;290(13):1749-56.Full text  Abstract

47. Fennell PB, Quante J, Wilson K, et al. Use of high-dose ibuprofen in a pediatric cystic fibrosis center. J Cyst Fibros. 2007 Apr;6(2):153-8. Abstract

48. Lands LC, Stanojevic S. Oral non-steroidal anti-inflammatory drug therapy for lung disease in cystic fibrosis. Cochrane Database Syst Rev. 2019 Sep 9;(9):CD001505.Full text  Abstract

49. Ren CL, Morgan RL, Oermann C, et al. Cystic Fibrosis Foundation pulmonary guidelines. Use of cystic fibrosis transmembrane conductance regulator modulator therapy in patients with cystic fibrosis. Ann Am Thorac Soc. 2018 Mar;15(3):271-80.Full text  Abstract

50. Rosenfeld M, Wainwright CE, Higgins M, et al; ARRIVAL study group. Ivacaftor treatment of cystic fibrosis in children aged 12 to <24 months and with a CFTR gating mutation (ARRIVAL): a phase 3 single-arm study. Lancet Respir Med. 2018 Jul;6(7):545-53.Full text  Abstract

51. Davies J, Wang LT, Panorchan P, et al. WS06-4 ivacaftor (IVA) treatment in patients 6 to <12 months old with cystic fibrosis with a CFTR gating mutation: results of a 2-part, single-arm, phase 3 study. Paper presented at: 42nd European Cystic Fibrosis Conference. 5-8 Jun 2019. Liverpool, UK. J Cyst Fibros. 2019 Jun 1;18(suppl 1):S11.Full text

52. Davies JC, Wainwright CE, Sawicki GS, et al. Ivacaftor in infants aged 4 to &lt;12 months with cystic fibrosis and a gating mutation. Results of a two-part phase 3 clinical trial. Am J Respir Crit Care Med. 2021 Mar 1;203(5):585-93.Full text  Abstract

53. Skilton M, Krishan A, Patel S, et al. Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis. Cochrane Database Syst Rev. 2019 Jan 7;(1):CD009841.Full text  Abstract

54. Mitchell RM, Jones AM, Stocking K, et al. Longitudinal effects of ivacaftor and medicine possession ratio in people with the Gly551Asp mutation: a 5-year study. Thorax. 2021 Sep;76(9):874-9. Abstract

55. Hoppe JE, Chilvers M, Ratjen F, et al. Long-term safety of lumacaftor-ivacaftor in children aged 2-5 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a multicentre, phase 3, open-label, extension study. Lancet Respir Med. 2021 Sep;9(9):977-88. Abstract

56. Chilvers MA, Davies JC, Milla C, et al. Long-term safety and efficacy of lumacaftor-ivacaftor therapy in children aged 6-11 years with cystic fibrosis homozygous for the F508del-CFTR mutation: a phase 3, open-label, extension study. Lancet Respir Med. 2021 Jul;9(7):721-32. Abstract

57. Walker S, Flume P, McNamara J, et al. A phase 3 study of tezacaftor in combination with ivacaftor in children aged 6 through 11 years with cystic fibrosis. J Cyst Fibros. 2019 Sep;18(5):708-13. Abstract

58. Flume PA, Biner RF, Downey DG, et al; VX14-661-110 study group. Long-term safety and efficacy of tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study. Lancet Respir Med. 2021 Jul;9(7):733-46. Abstract

59. Heijerman HGM, McKone EF, Downey DG, et al. Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial. Lancet. 2019 Nov 23;394(10212):1940-8. Abstract

60. Zemanick ET, Taylor-Cousar JL, Davies J, et al. A phase 3 open-label study of elexacaftor/tezacaftor/ivacaftor in children 6 through 11 years of age with cystic fibrosis and at least one F508del allele. Am J Respir Crit Care Med. 2021 Jun 15;203(12):1522-32.Full text  Abstract

61. Kapnadak SG, Dimango E, Hadjiliadis D, et al. Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease. J Cyst Fibros. 2020 May;19(3):344-54.Full text  Abstract

62. Marmor M, Jonas A, Mirza A, et al. Opportunities to improve utilization of palliative care among adults with cystic fibrosis: a systematic review. J Pain Symptom Manage. 2019 Dec;58(6):1100-12.e1.Full text  Abstract

63. Ramos KJ, Smith PJ, McKone EF, et al. Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines. J Cyst Fibros. 2019 May;18(3):321-33.Full text  Abstract

64. Koutsokera A, Varughese RA, Sykes J, et al. Pre-transplant factors associated with mortality after lung transplantation in cystic fibrosis: a systematic review and meta-analysis. J Cyst Fibros. 2019 May;18(3):407-15.Full text  Abstract

65. Douglas TA, Brennan S, Gard S, et al. Acquisition and eradication of P. aeruginosa in young children with cystic fibrosis. Eur Respir J. 2009 Feb;33(2):305-11. Abstract

66. Abbott L, Plummer A, Hoo ZH, et al. Duration of intravenous antibiotic therapy in people with cystic fibrosis. Cochrane Database Syst Rev. 2019 Sep 5;(9):CD006682.Full text  Abstract

67. Bhatt J, Jahnke N, Smyth AR. Once-daily versus multiple-daily dosing with intravenous aminoglycosides for cystic fibrosis. Cochrane Database Syst Rev. 2019 Sep 4;(9):CD002009.Full text  Abstract

68. Vonk SEM, Weersink EJM, Majoor CJ, et al; Amsterdam Mucociliary Clearance Disease (AMCD) Research Group. Tobramycin and vestibulotoxicity: retrospective analysis of four cases. Eur J Hosp Pharm. 2022 Mar;29(e1):e88-90.Full text  Abstract

69. Aaron SD, Vandemheen KL, Ferris W, et al. Combination antibiotic susceptibility testing to treat exacerbations of cystic fibrosis associated with multiresistant bacteria: a randomised, double-blind, controlled clinical trial. Lancet. 2005 Aug 6-12;366(9484):463-71. Abstract

70. Somayaji R, Parkins MD, Shah A, et al; Antimicrobial Resistance in Cystic Fibrosis International Working Group. Antimicrobial susceptibility testing (AST) and associated clinical outcomes in individuals with cystic fibrosis: a systematic review. J Cyst Fibros. 2019 Mar;18(2):236-43.Full text  Abstract

71. Rowbotham NJ, Palser SC, Smith SJ, et al. Infection prevention and control in cystic fibrosis: a systematic review of interventions. Expert Rev Respir Med. 2019 May;13(5):425-34.Full text  Abstract

72. Colombo C. Liver disease in cystic fibrosis. Curr Opin Pulm Med. 2007 Nov;13(6):529-36. Abstract

73. Shidrawi RG, Murugan N, Westaby D, et al. Emergency colonoscopy for distal intestinal obstruction syndrome in cystic fibrosis patients. Gut. 2002 Aug;51(2):285-6.Full text  Abstract

74. Holmes M, Murphy V, Taylor M, et al. Intussusception in cystic fibrosis. Arch Dis Child. 1991 Jun;66(6):726-7.Full text  Abstract

75. Lee TW, Southern KW, Perry LA, et al. Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease. Cochrane Database Syst Rev. 2016;(6):CD005599.Full text  Abstract

76. Stalvey MS, Anbar RD, Konstan MW, et al. A multi-center controlled trial of growth hormone treatment in children with cystic fibrosis. Pediatr Pulmonol. 2012 Mar;47(3):252-63. Abstract

77. Phung OJ, Coleman CI, Baker EL, et al. Recombinant human growth hormone in the treatment of patients with cystic fibrosis. Pediatrics. 2010 Nov;126(5):e1211-26. Abstract

78. Thaker V, Carter B, Putman M. Recombinant growth hormone therapy for cystic fibrosis in children and young adults. Cochrane Database Syst Rev. 2021 Aug 23;(8):CD008901.Full text  Abstract

79. Schuster A, Haliburn C, Döring G, et al; 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. 2013 Apr;68(4):344-50.Full text  Abstract

80. Geller DE, Flume PA, Staab D, et al; Mpex 204 Study Group. Levofloxacin inhalation solution (MP-376) in patients with cystic fibrosis with Pseudomonas aeruginosa. Am J Respir Crit Care Med. 2011 Jun 1;183(11):1510-6.Full text  Abstract

81. Devereux G, Wrolstad D, Bourke SJ, et al. Oral cysteamine as an adjunct treatment in cystic fibrosis pulmonary exacerbations: an exploratory randomized clinical trial. PLoS One. 2020;15(12):e0242945.Full text  Abstract

82. ClinicalTrials.gov. SAD and MAD of inhaled AR-501 in health adults and P. aeruginosa infected cystic fibrosis subjects. NCT03669614. Apr 2022 [internet publication].Full text

83. ClinicalTrials.gov. Study to evaluate the safety and tolerability of MRT5005 administered by nebulization in adults with cystic fibrosis (RESTORE-CF). NCT03375047 Nov 2020 [internet publication].Full text

84. Santi M, Milani GP, Simonetti GD, et al. Magnesium in cystic fibrosis - systematic review of the literature. Pediatr Pulmonol. 2016 Feb;51(2):196-202. Abstract

85. Ciofu O, Smith S, Lykkesfeldt J. Antioxidant supplementation for lung disease in cystic fibrosis. Cochrane Database Syst Rev. 2019 Oct 3;(10):CD007020.Full text  Abstract

86. Nevitt SJ, Thornton J, Murray CS, et al. Inhaled mannitol for cystic fibrosis. Cochrane Database Syst Rev. 2020 May 1;(5):CD008649.Full text  Abstract

87. Flume PA, Amelina E, Daines CL, et al. Efficacy and safety of inhaled dry-powder mannitol in adults with cystic fibrosis: an international, randomized controlled study. J Cyst Fibros. 2021 Nov;20(6):1003-9.Full text  Abstract

88. De Boeck K, Haarman E, Hull J, et al; DPM-CF-204 Study Group. Inhaled dry powder mannitol in children with cystic fibrosis: a randomised efficacy and safety trial. J Cyst Fibros. 2017 May;16(3):380-7.Full text  Abstract

89. Schwarzenberg SJ, Hempstead SE, McDonald CM, et al. Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines. J Cyst Fibros. 2016 Nov;15(6):724-35.Full text  Abstract

90. Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al; Cystic Fibrosis Foundation Pulmonary Clinical Practice Guidelines Committee. Cystic Fibrosis Foundation pulmonary guideline. Pharmacologic approaches to prevention and eradication of initial Pseudomonas aeruginosa infection. Ann Am Thorac Soc. 2014 Dec;11(10):1640-50.Full text  Abstract

91. Borowitz D, Robinson KA, Rosenfeld M, et al; Cystic Fibrosis Foundation. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009 Dec;155(suppl 6):S73-93.Full text  Abstract

92. Flume PA, Mogayzel PJ Jr, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations. Am J Respir Crit Care Med. 2009 Nov 1;180(9):802-8.Full text  Abstract

93. Turck D, Braegger CP, Colombo C, et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr. 2016 Jun;35(3):557-77.Full text  Abstract

94. Amin R, Noone PG, Ratjen F. Chemical pleurodesis versus surgical intervention for persistent and recurrent pneumothoraces in cystic fibrosis. Cochrane Database Syst Rev. 2012 Dec 12;(12):CD007481.Full text  Abstract

95. Lord RW, Jones AM, Webb AK, et al. Pneumothorax in cystic fibrosis: beyond the guidelines. Paediatr Respir Rev. 2016 Aug;20 (suppl):30-3. Abstract

96. Milla CE, Warwick WJ, Moran A. Trends in pulmonary function in patients with cystic fibrosis correlate with the degree of glucose intolerance at baseline. Am J Respir Crit Care Med. 2000 Sep;162(3 Pt 1):891-5.Full text  Abstract

97. Peterson ML, Jacobs DR Jr, Milla CE. Longitudinal changes in growth parameters are correlated with changes in pulmonary function in children with cystic fibrosis. Pediatrics. 2003 Sep;112(3 Pt 1):588-92. Abstract

98. Moran A, Pillay K, Becker D, et al. ISPAD clinical practice consensus guidelines 2018: management of cystic fibrosis-related diabetes in children and adolescents. Pediatr Diabetes. 2018 Oct;19(suppl 27):64-74. Abstract

99. Ballmann M, Hubert D, Assael BM, et al. Repaglinide versus insulin for newly diagnosed diabetes in patients with cystic fibrosis: a multicentre, open-label, randomised trial. Lancet Diabetes Endocrinol. 2018 Feb;6(2):114-21. Abstract

100. Onady GM, Stolfi A. Drug treatments for managing cystic fibrosis-related diabetes. Cochrane Database Syst Rev. 2020 Oct 19;(10):CD004730.Full text  Abstract

101. Harun SN, Holford NHG, Grimwood K, et al; Australasian Cystic Fibrosis Bronchoalveolar Lavage (ACFBAL) study group. Pseudomonas aeruginosa eradication therapy and risk of acquiring Aspergillus in young children with cystic fibrosis. Thorax. 2019 Aug;74(8):740-8.Full text  Abstract

102. Beer H, Southern KW, Swift AC. Topical nasal steroids for treating nasal polyposis in people with cystic fibrosis. Cochrane Database Syst Rev. 2015 Jun 22;(6):CD008253.Full text  Abstract

103. Tangpricha V, Kelly A, Stephenson A, et al; Cystic Fibrosis Foundation Vitamin D Evidence-Based Review Committee. An update on the screening, diagnosis, management, and treatment of vitamin D deficiency in individuals with cystic fibrosis: evidence-based recommendations from the Cystic Fibrosis Foundation. J Clin Endocrinol Metab. 2012 Apr;97(4):1082-93.Full text  Abstract

104. Conwell LS, Chang AB. Bisphosphonates for osteoporosis in people with cystic fibrosis. Cochrane Database Syst Rev. 2014 Mar 14;(3):CD002010.Full text  Abstract

105. Konstan MW, Butler SM, Wohl ME, et al. Investigators and coordinators of the epidemiologic study of cystic fibrosis: growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis. J Pediatr. 2003 Jun;142(6):624-30. Abstract

106. Shah GB, De Keyzer L, Russell JA, et al. Treatment of chronic rhinosinusitis with dornase alfa in patients with cystic fibrosis: a systematic review. Int Forum Allergy Rhinol. 2018 Jun;8(6):729-36. Abstract

107. Stefano MA, Poderoso RE, Mainz JG, et al. Prevalence of constipation in cystic fibrosis patients: a systematic review of observational studies. J Pediatr (Rio J). 2020 Nov - Dec;96(6):686-92.Full text  Abstract

108. Flume PA, Mogayzel PJ Jr, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: pulmonary complications: hemoptysis and pneumothorax. Am J Respir Crit Care Med. 2010 Aug 1;182(3):298-306.Full text  Abstract

109. Quittner AL, Abbott J, Georgiopoulos AM, et al. International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety. Thorax. 2016 Jan;71(1):26-34.Full text  Abstract

110. Yamada A, Komaki Y, Komaki F, et al. Risk of gastrointestinal cancers in patients with cystic fibrosis: a systematic review and meta-analysis. Lancet Oncol. 2018 Jun;19(6):758-67. Abstract

111. Hadjiliadis D, Khoruts A, Zauber AG, et al; Cystic Fibrosis Colorectal Cancer Screening Task Force. Cystic fibrosis colorectal cancer screening consensus recommendations. Gastroenterology. 2018 Feb;154(3):736-45.e14.Full text  Abstract

112. Breuer O, Caudri D, Stick S, et al. Predicting disease progression in cystic fibrosis. Expert Rev Respir Med. 2018 Nov;12(11):905-17. Abstract

113. Nixon PA, Orenstein DM, Kelsey SF, et al. The prognostic value of exercise testing in patients with cystic fibrosis. N Engl J Med. 1992 Dec 17;327(25):1785-8.Full text  Abstract

114. Orenstein DM, Hovell MF, Mulvihill M, et al. Strength vs aerobic training in children with cystic fibrosis: a randomized controlled trial. Chest. 2004 Oct;126(4):1204-14. Abstract