Cystic Fibrosis Questions & Answers

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20. Cystic Fibrosis Foundation. Fibrosis Foundation Patient registry Annual Report 2008. Bethesda, MD: Cystic Fibrosis Foundation; 2009.

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27. Kelly A, Schall J, Stallings VA, Zemel BS. Trabecular and cortical bone deficits are present in children and adolescents with cystic fibrosis. Bone. 2016 Apr 29. [QxMD MEDLINE Link].

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29. Zielenski J, Patrizio P, Corey M, et al. CFTR gene variant for patients with congenital absence of vas deferens. Am J Hum Genet. 1995 Oct. 57(4):958-60. [QxMD MEDLINE Link]. [Full Text].

30. Vande Velde S, Van Biervliet S, Robberecht E. Cystic fibrosis presenting as diabetes insipidus unresponsive to desmopressin. Acta Gastroenterol Belg. 2007 Jul-Sep. 70(3):300-1. [QxMD MEDLINE Link].

31. [Guideline] 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. [QxMD MEDLINE Link]. [Full Text].

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33. Cystic Fibrosis Foundation., Borowitz D, Parad RB, Sharp JK, Sabadosa KA, Robinson KA, et al. Cystic Fibrosis Foundation practice guidelines for the management of infants with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome during the first two years of life and beyond. J Pediatr. 2009 Dec. 155 (6 Suppl):S106-16. [QxMD MEDLINE Link].

34. Farrell PM, Koscik RE. Sweat chloride concentrations in infants homozygous or heterozygous for F508 cystic fibrosis. Pediatrics. 1996 Apr. 97(4):524-8. [QxMD MEDLINE Link].

35. Farrell PM, White TB, Ren CL, Hempstead SE, Accurso F, Derichs N, et al. Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation. J Pediatr. 2017 Feb. 181S:S4-S15.e1. [QxMD MEDLINE Link].

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40. Savant AP, McColley SA. Cystic fibrosis year in review 2016. Pediatr Pulmonol. 2017 Aug. 52 (8):1092-1102. [QxMD MEDLINE Link].

41. Smith L, Marshall H, Aldag I, Horn F, Collier G, Hughes D, et al. Longitudinal Assessment of Children with Mild CF Using Hyperpolarised Gas Lung MRI and LCI. Am J Respir Crit Care Med. 2017 Jun 29. [QxMD MEDLINE Link].

42. Marshall H, Horsley A, Taylor CJ, Smith L, Hughes D, Horn FC, et al. Detection of early subclinical lung disease in children with cystic fibrosis by lung ventilation imaging with hyperpolarised gas MRI. Thorax. 2017 Aug. 72 (8):760-762. [QxMD MEDLINE Link].

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45. Rosenfeld M, Allen J, Arets BH, Aurora P, Beydon N, Calogero C, et al. An official American Thoracic Society workshop report: optimal lung function tests for monitoring cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheezing in children less than 6 years of age. Ann Am Thorac Soc. 2013 Apr. 10(2):S1-S11. [QxMD MEDLINE Link].

46. Filburn AG, Lumeng CN, Nasr SZ. Infant pulmonary function testing guides therapy in cystic fibrosis lung disease. Respiratory Medicine CME. 2011. 4:17-19.

47. Ren CL, Brucker JL, Rovitelli AK, Bordeaux KA. Changes in lung function measured by spirometry and the forced oscillation technique in cystic fibrosis patients undergoing treatment for respiratory tract exacerbation. Pediatr Pulmonol. 2006 Apr. 41(4):345-9. [QxMD MEDLINE Link].

48. Taylor-Robinson D, Whitehead M, Diderichsen F, Olesen HV, Pressler T, Smyth RL, et al. Understanding the natural progression in %FEV1 decline in patients with cystic fibrosis: a longitudinal study. Thorax. 2012 May 3. [QxMD MEDLINE Link].

49. Davies JC, Cunningham S, Alton EW, Innes JA. Lung clearance index in CF: a sensitive marker of lung disease severity. Thorax. 2008 Feb. 63(2):96-7. [QxMD MEDLINE Link].

50. Moran A, Pekow P, Grover P, et al. Insulin therapy to improve BMI in cystic fibrosis-related diabetes without fasting hyperglycemia: results of the cystic fibrosis related diabetes therapy trial. Diabetes Care. 2009 Oct. 32(10):1783-8. [QxMD MEDLINE Link]. [Full Text].

51. Lum S, Gustafsson P, Ljungberg H, Hülskamp G, Bush A, Carr SB. Early detection of cystic fibrosis lung disease: multiple-breath washout versus raised volume tests. Thorax. 2007 Apr. 62(4):341-7. [QxMD MEDLINE Link].

52. Owens CM, Aurora P, Stanojevic S, Bush A, Wade A, Oliver C. Lung Clearance Index and HRCT are complementary markers of lung abnormalities in young children with CF. Thorax. 2011 Jun. 66(6):481-8. [QxMD MEDLINE Link].

53. Steven LC, Gavel G, Young D, Carachi R. Immunoreactive trypsin levels in neonates with meconium ileus. Pediatr Surg Int. 2006 Mar. 22(3):236-9. [QxMD MEDLINE Link].

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56. Yang C, Montgomery M. Dornase alfa for cystic fibrosis. Cochrane Database Syst Rev. 2018 Sep 6. 9:CD001127. [QxMD MEDLINE Link].

57. Sagel SD, Khan U, Jain R, Graff G, Daines CL, Dunitz JM, et al. Effects of an Antioxidant-enriched Multivitamin in Cystic Fibrosis. A Randomized, Controlled, Multicenter Clinical Trial. Am J Respir Crit Care Med. 2018 Sep 1. 198 (5):639-647. [QxMD MEDLINE Link].

58. Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, et al. Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR. N Engl J Med. 2015 May 17. [QxMD MEDLINE Link]. [Full Text].

59. Taylor-Cousar JL, Munck A, McKone EF, van der Ent CK, Moeller A, Simard C, et al. Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del. N Engl J Med. 2017 Nov 23. 377 (21):2013-2023. [QxMD MEDLINE Link].

60. Rowe SM, Daines C, Ringshausen FC, Kerem E, Wilson J, Tullis E, et al. Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis. N Engl J Med. 2017 Nov 23. 377 (21):2024-2035. [QxMD MEDLINE Link].

61. Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, Boucher RC. Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N Engl J Med. 2006 Jan 19. 354(3):241-50. [QxMD MEDLINE Link].

62. Wark P, McDonald VM. Nebulised hypertonic saline for cystic fibrosis. Cochrane Database Syst Rev. 2018 Sep 27. 9:CD001506. [QxMD MEDLINE Link].

63. 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. [QxMD MEDLINE Link].

64. 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. [QxMD MEDLINE Link].

65. Yu H, Burton B, Huang CJ, Worley J, Cao D, Johnson JP Jr, et al. Ivacaftor potentiation of multiple CFTR channels with gating mutations. J Cyst Fibros. 2012 May. 11(3):237-45. [QxMD MEDLINE Link].

66. Rowe, Steven M., et al. Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis. New England Journal of Medicine. 2017 Nov 23. 377 (21):2024-2035. [QxMD MEDLINE Link]. [Full Text].

67. Taylor-Cousar JL, et al. Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del. N Engl J Med. 2017 Nov 23. 377 (21):2013-2023. [QxMD MEDLINE Link]. [Full Text].

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Girish D Sharma, MD, FCCP, FAAP Professor of Pediatrics, Rush Medical College; Director, Section of Pediatric Pulmonology and Rush Cystic Fibrosis Center, Rush Children's Hospital, Rush University Medical Center

Girish D Sharma, MD, FCCP, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society, Royal College of Physicians of Ireland

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Charles Callahan, DO Professor, Chief, Department of Pediatrics and Pediatric Pulmonology, Tripler Army Medical Center

Charles Callahan, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American College of Osteopathic Pediatricians, American Thoracic Society, Association of Military Surgeons of the US, Christian Medical and Dental Associations

Disclosure: Nothing to disclose.

Kenan Haver, MD Associate Professor of Pediatrics, Harvard Medical School; Director of Asthma Program, Director of Flexible Bronchoscopy Program, Director of Pulmonary Division Asthma Program, Co-Director of Primary Ciliary Dyskinesia, Co-Leader of Empyema Standardized Clinical Assessment and Management Plans (SCAMP), Boston Children’s Hospital

Kenan Haver, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society

Disclosure: Author for: UptoDate.

Susanna A McColley, MD Professor of Pediatrics, Northwestern University, The Feinberg School of Medicine; Director of Cystic Fibrosis Center, Head, Division of Pulmonary Medicine, Children's Memorial Medical Center of Chicago

Susanna A McColley, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Sleep Disorders Association, American Thoracic Society

Disclosure: Received honoraria from Genentech for speaking and teaching; Received honoraria from Genentech for consulting; Partner received consulting fee from Boston Scientific for consulting; Received honoraria from Gilead for speaking and teaching; Received consulting fee from Caremark for consulting; Received honoraria from Vertex Pharmaceuticals for speaking and teaching.

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