ცისტური ფიბროზი (მუკოვისციდოზი)

წყაროები

ძირითადი სტატიები

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-S15.e1.სრული ტექსტი  აბსტრაქტი

Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al; Pulmonary Clinical Practice Guidelines Committee. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013 Apr 1;187(7):680-9.სრული ტექსტი  აბსტრაქტი

Flume PA, Robinson KA, O'Sullivan BP, et al. Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respir Care. 2009 Apr;54(4):522-37.სრული ტექსტი  აბსტრაქტი

Flume PA, O'Sullivan BP, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2007 Nov 15;176(10):957-69.სრული ტექსტი  აბსტრაქტი

Saiman L, Anstead M, Mayer-Hamblett N, et al. Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA. 2010 May 5;303(17):1707-15.სრული ტექსტი  აბსტრაქტი

გამოყენებული სტატიები

1. Cystic fibrosis mutation database. April 2011 [internet publication].სრული ტექსტი

2. Kerem E. Atypical CF and CF related diseases. Paediatr Respir Rev. 2006;7(suppl 1):S144-6. აბსტრაქტი

3. 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. აბსტრაქტი

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

5. Cystic Fibrosis Trust. What is cystic fibrosis? Accessed Jan 2018 [internet publication].სრული ტექსტი

6. The Cystic Fibrosis Foundation. About cystic fibrosis. Accessed Jan 2018 [internet publication].სრული ტექსტი

7. 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. აბსტრაქტი

8. 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. აბსტრაქტი

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;280:35751-35759.სრული ტექსტი  აბსტრაქტი

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;175:174-183.სრული ტექსტი  აბსტრაქტი

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;95:1005-1015. აბსტრაქტი

12. Knowles MR, Stutts MJ, Spock A, et al. Abnormal ion permeation through cystic fibrosis respiratory epithelium. Science. 1983;221:1067-1070. აბსტრაქტი

13. Knowles M, Gatzy J, Boucher R. Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N Engl J Med. 1981;305:1489-1495. აბსტრაქტი

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;281:7392-7398.სრული ტექსტი  აბსტრაქტი

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;581:271-278.სრული ტექსტი  აბსტრაქტი

16. Boucher RC. New concepts of the pathogenesis of cystic fibrosis lung disease. Eur Respir J. 2004;23:146-158.სრული ტექსტი  აბსტრაქტი

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;25:21-25.სრული ტექსტი  აბსტრაქტი

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;283:L329-L335.სრული ტექსტი  აბსტრაქტი

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-S15.e1.სრული ტექსტი  აბსტრაქტი

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. აბსტრაქტი

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. აბსტრაქტი

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. აბსტრაქტი

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. აბსტრაქტი

24. Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al; Pulmonary Clinical Practice Guidelines Committee. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013 Apr 1;187(7):680-9.სრული ტექსტი  აბსტრაქტი

25. Mckoy NA, Wilson LM, Saldanha IJ, et al. Active cycle of breathing technique for cystic fibrosis. Cochrane Database Syst Rev. 2016;(7):CD007862.სრული ტექსტი  აბსტრაქტი

26. Flume PA, Robinson KA, O'Sullivan BP, et al. Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respir Care. 2009 Apr;54(4):522-37.სრული ტექსტი  აბსტრაქტი

27. Flume PA, O'Sullivan BP, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2007 Nov 15;176(10):957-69.სრული ტექსტი  აბსტრაქტი

28. Elkins MR, Robinson M, Rose BR, et al. 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.სრული ტექსტი  აბსტრაქტი

29. Wark P, McDonald VM. Nebulised hypertonic saline for cystic fibrosis. Cochrane Database Syst Rev. 2009;(2):CD001506. აბსტრაქტი

30. Aitken ML, Bellon G, De Boeck K, et al; CF302 Investigators. Long-term inhaled dry powder mannitol in cystic fibrosis: an international randomized study. Am J Respir Crit Care Med. 2012 Mar 15;185(6):645-52. აბსტრაქტი

31. Balfour-Lynn IM, Welch K. Inhaled corticosteroids for cystic fibrosis. Cochrane Database Syst Rev. 2016;(8):CD001915.სრული ტექსტი  აბსტრაქტი

32. 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.სრული ტექსტი  აბსტრაქტი

33. 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. აბსტრაქტი

34. Saiman L, Marshall BC, Mayer-Hamblett N, et al. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA. 2003 Oct 1;290(13):1749-56.სრული ტექსტი  აბსტრაქტი

35. 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. აბსტრაქტი

36. Lands LC, Stanojevic S. Oral non-steroidal anti-inflammatory drug therapy for lung disease in cystic fibrosis. Cochrane Database Syst Rev. 2016;(4):CD001505.სრული ტექსტი  აბსტრაქტი

37. Ramsey BW, Davies J, McElvaney NG, et al; VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011 Nov 3;365(18):1663-72.სრული ტექსტი  აბსტრაქტი

38. Wainwright CE, Elborn JS, Ramsey BW, et al; TRAFFIC Study Group; TRANSPORT Study Group. Lumacaftor-Ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N Engl J Med. 2015 Jul 16;373(3):220-31. აბსტრაქტი

39. Donaldson SH, Pilewski JM, Griese M, et al. Tezacaftor/Ivacaftor in Subjects with Cystic Fibrosis and F508del/F508del-CFTR or F508del/G551D-CFTR. Am J Respir Crit Care Med. 2018 Jan 15;197(2):214-224.სრული ტექსტი  აბსტრაქტი

40. Taylor-Cousar JL, Munck A, McKone EF, et al. Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del. N Engl J Med. 2017 Nov 23;377(21):2013-2023.სრული ტექსტი  აბსტრაქტი

41. 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. აბსტრაქტი

42. Plummer A, Wildman M. Duration of intravenous antibiotic therapy in people with cystic fibrosis. Cochrane Database Syst Rev. 2016;(9):CD006682.სრული ტექსტი  აბსტრაქტი

43. Smyth AR, Bhatt J, Nevitt SJ. Once-daily versus multiple-daily dosing with intravenous aminoglycosides for cystic fibrosis. Cochrane Database Syst Rev. 2017;(3):CD002009.სრული ტექსტი  აბსტრაქტი

44. 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. აბსტრაქტი

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

46. 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.სრული ტექსტი  აბსტრაქტი

47. Holmes M, Murphy V, Taylor M, et al. Intussusception in cystic fibrosis. Arch Dis Child. 1991 Jun;66(6):726-7.სრული ტექსტი  აბსტრაქტი

48. Saiman L, Anstead M, Mayer-Hamblett N, et al. Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA. 2010 May 5;303(17):1707-15.სრული ტექსტი  აბსტრაქტი

49. Southern KW, Barker PM, Solis-Moya A, et al. Macrolide antibiotics for cystic fibrosis. Cochrane Database Syst Rev. 2012;(11):CD002203.სრული ტექსტი  აბსტრაქტი

50. Ratjen F, Saiman L, Mayer-Hamblett N, et al. Effect of azithromycin on systemic markers of inflammation in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa. Chest. 2012 Nov;142(5):1259-66.სრული ტექსტი  აბსტრაქტი

51. 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.სრული ტექსტი  აბსტრაქტი

52. 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. აბსტრაქტი

53. 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.სრული ტექსტი  აბსტრაქტი

54. 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.სრული ტექსტი  აბსტრაქტი

55. 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.სრული ტექსტი  აბსტრაქტი

56. Amin R, Noone PG, Ratjen F. Chemical pleurodesis versus surgical intervention for persistent and recurrent pneumothoraces in cystic fibrosis. Cochrane Database Syst Rev. 2012;(12):CD007481.სრული ტექსტი  აბსტრაქტი

57. 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.სრული ტექსტი  აბსტრაქტი

58. 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. აბსტრაქტი

59. Moran A, Pillay K, Becker DJ, et al; International Society for Pediatric and Adolescent Diabetes. ISPAD Clinical Practice Consensus Guidelines 2014. Management of cystic fibrosis-related diabetes in children and adolescents. Pediatr Diabetes. 2014 Sep;15 Suppl 20:65-76.სრული ტექსტი  აბსტრაქტი

60. Beer H, Southern KW, Swift AC. Topical nasal steroids for treating nasal polyposis in people with cystic fibrosis. Cochrane Database Syst Rev. 2015;(6):CD008253.სრული ტექსტი  აბსტრაქტი

61. 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. აბსტრაქტი

62. Conwell LS, Chang AB. Bisphosphonates for osteoporosis in people with cystic fibrosis. Cochrane Database Syst Rev. 2014;(3):CD002010.სრული ტექსტი  აბსტრაქტი

63. 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. აბსტრაქტი

64. 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.სრული ტექსტი  აბსტრაქტი

65. 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.სრული ტექსტი  აბსტრაქტი

66. Nixon PA, Orenstein DM, Kelsey SF, et al. The prognostic value of exercise testing in patients with cystic fibrosis. N Engl J Med. 1992;327:1785-1788. აბსტრაქტი

67. Orenstein DM, Hovell MF, Mulvihill M, et al. Strength vs aerobic training in children with cystic fibrosis: a randomized controlled trial. Chest. 2004;126:1204-1214.სრული ტექსტი  აბსტრაქტი

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