eMedicine Specialties > Radiology > Chest

Cystic Fibrosis, Thoracic: Follow-up

Author: Eleni Giannouli, MD, Consulting Staff, Assistant Professor, Section of Respirology and Critical Care, Department of Critical Care,Winnipeg Regional Health Authority Health Sciences Center
Coauthor(s): Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St. Boniface General Hospital; Bruce Maycher, MD, Director of Pulmonary Radiology, St Boniface General Hospital; Associate Professor, Department of Radiology, University of Manitoba
Contributor Information and Disclosures

Updated: Apr 13, 2009

Intervention

Possible radiologic interventions in pulmonary CF include BAE, treatment with glycerin and amphotericin B, and bronchography.

BAE is a relatively safe and effective treatment of significant hemoptysis in patients with CF.96

Brinson et al reviewed a 10-year experience in the treatment of hemoptysis in 18 patients with CF.97 The treatment included 36 BAE procedures for the control of hemoptysis. Most patients had severe lung disease (FEV1 < 35%) with a high incidence of infection with multidrug-resistant bacteria. The overall effectiveness of BAE for the initial control of hemoptysis was 75% after 1 session, 89% after 2 sessions, and 93% after 3 sessions.

The patients were followed up for a mean of approximately 22 months after BAE. The overall recurrence rate per episode was 46%, with a mean time for recurrence of approximately 12 months. The incidence of bleeding from nonbronchial systemic collateral vessels was high among patients who had undergone previous BAE. Also reported are 2 deaths associated with massive hemoptysis despite BAE and 3 patients with transient neurologic deficits during BAE (transverse myelitis).98

The direct intracavitary percutaneous instillation of glycerin and amphotericin B with CT guidance has shown promise in the treatment of symptomatic patients with mycetoma (aspergilloma) as complication of a preexisting cavity with extensive bronchiectasis. In a study of 15 patients with bronchiectasis, the palliation of hemoptysis and sputum production occurred, with the diminishment or disappearance in serum precipitins.

As the criterion standard as a diagnostic tool for bronchiectasis, bronchography has been almost completely replaced by HRCT. Indications for bronchography may still exist in select surgical candidates in whom CT shows predominant segmental or unilateral involvement.99

Moran et al compared noninvasive ventilation (NIV) with no NIV in patients with cystic fibrosis. In a meta-analysis of randomized, controlled trials that compared a pressure preset or volume preset NIV with no NIV in cystic fibrosis patients with respiratory failure, the authors found that airway clearance may be easier with NIV and that patients with CF may have a preference for it. They concluded that NIV may be a useful adjunctive therapy to other airway-clearance approaches. In addition, in patients with moderate to severe disease, NIV used along with oxygen may improve gas exchange, as compared with oxygen therapy alone, while such patients are sleeping.100

Medicolegal Pitfalls

  • Failure to suspect CF in young children with either normal chest radiographic findings or hyperinflation and/or atelectasis and an asthmalike presentation
  • Failure to suspect CF in an adult with mild respiratory disease and/or obscure liver disease, occasionally with a lack of respiratory symptoms
  • Failure to consider and differentiate conditions related to CF that are superimposed on bronchiectasis, eg, allergic bronchopulmonary aspergillosis, a mycetoma formed in preexisting cavity, or an atypical mycobacterial infection
  • Failure to consider and differentiate any other causes of bronchiectasis associated with chronic sinopulmonary disease and azoospermia in a teenager or young adult (Examples include Young syndrome, or immotile cilia syndrome.)
  • Failure to consider the diagnosis, because the patient is not white, looks too healthy, or has normal pancreatic function.
 


More on Cystic Fibrosis, Thoracic

Overview: Cystic Fibrosis, Thoracic
Imaging: Cystic Fibrosis, Thoracic
Follow-up: Cystic Fibrosis, Thoracic
Multimedia: Cystic Fibrosis, Thoracic
References
Further Reading
[ CLOSE WINDOW ]

References

  1. Amodio JB, Berdon WE, Abramson S. Cystic fibrosis in childhood: pulmonary, paranasal sinus, and skeletal manifestations. Semin Roentgenol. Apr 1987;22(2):125-35. [Medline].

  2. Bye MR, Ewig JM, Quittell LM. Cystic fibrosis. Lung. 1994;172(5):251-70. [Medline].

  3. Ruzal-Shapiro C. Cystic fibrosis. An overview. Radiol Clin North Am. Jan 1998;36(1):143-61. [Medline].

  4. Tager AM, Wu J, Vermeulen MW. The effect of chloride concentration on human neutrophil functions: potential relevance to cystic fibrosis. Am J Respir Cell Mol Biol. Oct 1998;19(4):643-52. [Medline].

  5. Guggino WB. Cystic fibrosis and the salt controversy. Cell. Mar 5 1999;96(5):607-10. [Medline].

  6. Abramson SJ, Baker DH, Amodio JB. Gastrointestinal manifestations of cystic fibrosis. Semin Roentgenol. Apr 1987;22(2):97-113. [Medline].

  7. Amorosa JK, Laraya-Cuasay LR, Sohn L. Radiologic diagnosis of cystic fibrosis in adults and children. Acad Radiol. Mar 1995;2(3):222-5. [Medline].

  8. Stutts MJ, Canessa CM, Olsen JC. CFTR as a cAMP-dependent regulator of sodium channels. Science. Aug 11 1995;269(5225):847-50. [Medline].

  9. Johnson LG, Boyles SE, Wilson J. Normalization of raised sodium absorption and raised calcium-mediated chloride secretion by adenovirus-mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway epithelial cells. J Clin Invest. Mar 1995;95(3):1377-82. [Medline].

  10. Balistreri WF. Spectrum of liver disease in patients with CF. Pediatr Pulmonol. 1990;5:71-3.

  11. Pier GB, Grout M, Zaidi TS. Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections. Science. Jan 5 1996;271(5245):64-7. [Medline].

  12. Bals R, Weiner DJ, Wilson JM. The innate immune system in cystic fibrosis lung disease. J Clin Invest. Feb 1999;103(3):303-7. [Medline].

  13. Oliver A, Canton R, Campo P. High frequency of hypermutable Pseudomonas aeruginosa in cystic fibrosis lung infection. Science. May 19 2000;288(5469):1251-4. [Medline].

  14. DiMango E, Ratner AJ, Bryan R. Activation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells. J Clin Invest. Jun 1 1998;101(11):2598-605. [Medline].

  15. Heeckeren A, Walenga R, Konstan MW. Excessive inflammatory response of cystic fibrosis mice to bronchopulmonary infection with Pseudomonas aeruginosa. J Clin Invest. Dec 1 1997;100(11):2810-5. [Medline].

  16. McGuinness G, Naidich DP. CT of airways disease and bronchiectasis. Radiol Clin North Am. Jan 2002;40(1):1-19. [Medline].

  17. Population variation of common cystic fibrosis mutations. The Cystic Fibrosis Genetic Analysis Consortium. Hum Mutat. 1994;4(3):167-77. [Medline].

  18. Rommens JM, Iannuzzi MC, Kerem B. Identification of the cystic fibrosis gene: chromosome walking and jumping. Science. Sep 8 1989;245(4922):1059-65. [Medline].

  19. Ma J, Davis PB. What we know and what we do not know about cystic fibrosis transmembrane conductance regulator. Clin Chest Med. Sep 1998;19(3):459-71, v-vi. [Medline].

  20. Knowles MR, Paradiso AM, Boucher RC. In vivo nasal potential difference: techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis. Hum Gene Ther. Apr 1995;6(4):445-55. [Medline].

  21. LeGrys VA. Sweat testing for the diagnosis of cystic fibrosis: practical considerations. J Pediatr. Dec 1996;129(6):892-7. [Medline].

  22. Sharer N, Schwarz M, Malone G. Mutations of the cystic fibrosis gene in patients with chronic pancreatitis. N Engl J Med. Sep 3 1998;339(10):645-52. [Medline].

  23. Welsh MJ, Sui LC, Boat T. Cystic fibrosis. In: Scriver CR, Beaudet AL, Sly WS, et al, eds. The Metabolic and Molecular Basis of Inherited Disease. New York: McGraw-Hill;1995: 3799.

  24. Dodge JA. Male fertility in cystic fibrosis. Lancet. Sep 2 1995;346(8975):587-8. [Medline].

  25. McCallum TJ, Milunsky JM, Cunningham DL. Fertility in men with cystic fibrosis: an update on current surgical practices and outcomes. Chest. Oct 2000;118(4):1059-62. [Medline].

  26. Kerem E, Reisman J, Corey M. Prediction of mortality in patients with cystic fibrosis. N Engl J Med. Apr 30 1992;326(18):1187-91. [Medline].

  27. Shah RM, Friedman AC, Ostrum BJ. Pulmonary complications of cystic fibrosis in adults. Crit Rev Diagn Imaging. 1995;36(6):441-77. [Medline].

  28. Rosenstein BJ. What is a cystic fibrosis diagnosis?. Clin Chest Med. Sep 1998;19(3):423-41, v. [Medline].

  29. Rosenstein BJ, Cutting GR. The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel. J Pediatr. Apr 1998;132(4):589-95. [Medline].

  30. Donovan DS Jr, Papadopoulos A, Staron RB. Bone mass and vitamin D deficiency in adults with advanced cystic fibrosis lung disease. Am J Respir Crit Care Med. Jun 1998;157(6 Pt 1):1892-9. [Medline].

  31. Muller F, Dommergues M, Aubry MC. Hyperechogenic fetal bowel: an ultrasonographic marker for adverse fetal and neonatal outcome. Am J Obstet Gynecol. Aug 1995;173(2):508-13. [Medline].

  32. Farrell PM, Mischler EH. Newborn screening for cystic fibrosis. The Cystic Fibrosis Neonatal Screening Study Group. Adv Pediatr. 1992;39:35-70. [Medline].

  33. Cortese G, Malfitana V, Placido R, Ferrari A, Grosso B, De Rose V. Role of chest radiography in the diagnosis of allergic bronchopulmonary aspergillosis in adult patients with cystic fibrosis. Radiol Med (Torino). Aug 2007;112(5):626-36. [Medline].

  34. Conway S. Cystic fibrosis in teenagers and young adults. Arch Dis Child. Aug 1996;75(2):99-101. [Medline].

  35. Katz DS, Hines J, Math KR. Using CT to reveal fat-containing abnormalities of the pancreas. AJR Am J Roentgenol. Feb 1999;172(2):393-6. [Medline].

  36. Hubbard AM, Meyer JS, Mahboubi S. Diagnosis of liver disease in children: value of MR angiography. AJR Am J Roentgenol. Sep 1992;159(3):617-21. [Medline].

  37. Edenborough FP, Stableforth DE, Webb AK. Outcome of pregnancy in women with cystic fibrosis. Thorax. Feb 1995;50(2):170-4. [Medline].

  38. Laursen EM, Molgaard C, Michaelsen KF. Bone mineral status in 134 patients with cystic fibrosis. Arch Dis Child. Sep 1999;81(3):235-40. [Medline].

  39. Laursen EM, Molgaard C, Michaelsen KF. Bone mineral status in 134 patients with cystic fibrosis. Arch Dis Child. Sep 1999;81(3):235-40. [Medline].

  40. Cleveland RH, Zurakowski D, Slattery DM, Colin AA. Chest radiographs for outcome assessment in cystic fibrosis. Proc Am Thorac Soc. Aug 1 2007;4(4):302-5. [Medline].

  41. Tiddens HA. Chest computed tomography scans should be considered as a routine investigation in cystic fibrosis. Paediatr Respir Rev. Sep 2006;7(3):202-8. [Medline].

  42. Tiddens HA, de Jong PA. Update on the application of chest computed tomography scanning to cystic fibrosis. Curr Opin Pulm Med. Nov 2006;12(6):433-9. [Medline].

  43. Eggesbo HB, Sovik S, Dolvik S. CT characterization of developmental variations of the paranasal sinuses in cystic fibrosis. Acta Radiol. Sep 2001;42(5):482-93. [Medline].

  44. Eggesbo HB, Dolvik S, Stiris M. Complementary role of MR imaging of ethmomaxillary sinus disease depicted at CT in cystic fibrosis. Acta Radiol. Mar 2001;42(2):144-50. [Medline].

  45. Eggesbo HB, Ringertz S, Haanaes OC. CT and MR imaging of the paranasal sinuses in cystic fibrosis. Correlation with microbiological and histopathological results. Acta Radiol. Mar 1999;40(2):154-62. [Medline].

  46. Goss CH, Mayer-Hamblett N, Kronmal RA, Williams J, Ramsey BW. Laboratory parameter profiles among patients with cystic fibrosis. J Cyst Fibros. Apr 2007;6(2):117-23. [Medline].

  47. Drumm ML, Collins FS. Molecular biology of cystic fibrosis. Mol Genet Med. 1993;3:33-68. [Medline].

  48. Brock DJ. Prenatal screening for cystic fibrosis: 5 years'' experience reviewed. Lancet. Jan 20 1996;347(8995):148-50. [Medline].

  49. Gregg RG, Simantel A, Farrell PM, Koscik R, Kosorok MR, Laxova A. Newborn screening for cystic fibrosis in Wisconsin: comparison of biochemical and molecular methods. Pediatrics. Jun 1997;99(6):819-24. [Medline].

  50. Robinson TE. Imaging of the chest in cystic fibrosis. Clin Chest Med. Jun 2007;28(2):405-21. [Medline].

  51. Friedman PJ. Chest radiographic findings in the adult with cystic fibrosis. Semin Roentgenol. Apr 1987;22(2):114-24. [Medline].

  52. Friedman PJ, Harwood IR, Ellenbogen PH. Pulmonary cystic fibrosis in the adult: early and late radiologic findings with pathologic correlations. AJR Am J Roentgenol. Jun 1981;136(6):1131-44. [Medline].

  53. Reiff DB, Wells AU, Carr DH. CT findings in bronchiectasis: limited value in distinguishing between idiopathic and specific types. AJR Am J Roentgenol. Aug 1995;165(2):261-7. [Medline].

  54. Arakawa H, Webb WR. Air trapping on expiratory high-resolution CT scans in the absence of inspiratory scan abnormalities: correlation with pulmonary function tests and differential diagnosis. AJR Am J Roentgenol. May 1998;170(5):1349-53. [Medline].

  55. Robinson TE, Leung AN, Northway WH. Spirometer-triggered high-resolution computed tomography and pulmonary function measurements during an acute exacerbation in patients with cystic fibrosis. J Pediatr. Apr 2001;138(4):553-9. [Medline].

  56. Nasr SZ, Gordon D, Sakmar E, Yu X, Christodoulou E, Eckhardt BP. High resolution computerized tomography of the chest and pulmonary function testing in evaluating the effect of tobramycin solution for inhalation in cystic fibrosis patients. Pediatr Pulmonol. Dec 2006;41(12):1129-37. [Medline].

  57. Brody AS, Molina PL, Klein JS. High-resolution computed tomography of the chest in children with cystic fibrosis: support for use as an outcome surrogate. Pediatr Radiol. Oct 1999;29(10):731-5. [Medline].

  58. Cooper P, MacLean J. High-resolution computed tomography (HRCT) should not be considered as a routine assessment method in cystic fibrosis lung disease. Paediatr Respir Rev. Sep 2006;7(3):197-201. [Medline].

  59. Cartier Y, Kavanagh PV, Johkoh T. Bronchiectasis: accuracy of high-resolution CT in the differentiation of specific diseases. AJR Am J Roentgenol. Jul 1999;173(1):47-52. [Medline].

  60. Santamaria F, Grillo G, Guidi G. Cystic fibrosis: when should high-resolution computed tomography of the chest Be obtained?. Pediatrics. May 1998;101(5):908-13. [Medline].

  61. Nasr SZ, Kuhns LR, Brown RW. Use of computerized tomography and chest x-rays in evaluating efficacy of aerosolized recombinant human DNase in cystic fibrosis patients younger than age 5 years: a preliminary study. Pediatr Pulmonol. May 2001;31(5):377-82. [Medline].

  62. Maffessanti M, Candusso M, Brizzi F. Cystic fibrosis in children: HRCT findings and distribution of disease. J Thorac Imaging. Winter 1996;11(1):27-38. [Medline].

  63. Marchant JM, Masel JP, Dickinson FL. Application of chest high-resolution computer tomography in young children with cystic fibrosis. Pediatr Pulmonol. Jan 2001;31(1):24-9. [Medline].

  64. Logan PM, O''Laoide RM, Mulherin D. High resolution computed tomography in cystic fibrosis: correlation with pulmonary function and assessment of prognostic value. Ir J Med Sci. Jan-Mar 1996;165(1):27-31. [Medline].

  65. Helbich TH, Heinz-Peer G, Eichler I. Cystic fibrosis: CT assessment of lung involvement in children and adults. Radiology. Nov 1999;213(2):537-44. [Medline].

  66. Lugo-Olivieri CH, Soyer PA, Fishman EK. Cystic fibrosis: spectrum of thoracic and abdominal CT findings in the adult patient. Clin Imaging. Sep-Oct 1998;22(5):346-54. [Medline].

  67. Carr DH, Oades P, Trotman-Dickenson B. Magnetic resonance scanning in cystic fibrosis: comparison with computed tomography. Clin Radiol. Feb 1995;50(2):84-9. [Medline].

  68. Ciofetta G. Lung scintigraphy in the assessment of recurrent bronchitis and pneumonia in children. Q J Nucl Med. Dec 1997;41(4):292-301. [Medline].

  69. Haslam RH, Borovnicar DJ, Stroud DB. Correlates of prepubertal bone mineral density in cystic fibrosis. Arch Dis Child. Aug 2001;85(2):166-71. [Medline].

  70. Lipnick RN, Glass RB. Bone changes associated with cystic fibrosis. Skeletal Radiol. 1992;21(2):115-6. [Medline].

  71. Tonkin IL, Hanissian AS, Boulden TF. Bronchial arteriography and embolotherapy for hemoptysis in patients with cystic fibrosis. Cardiovasc Intervent Radiol. Jul-Aug 1991;14(4):241-6. [Medline].

  72. Greene KE, Takasugi JE, Godwin JD. Radiographic changes in acute exacerbations of cystic fibrosis in adults: a pilot study. AJR Am J Roentgenol. Sep 1994;163(3):557-62. [Medline].

  73. Grum CM, Lynch JP 3rd. Chest radiographic findings in cystic fibrosis. Semin Respir Infect. Sep 1992;7(3):193-209. [Medline].

  74. Don CJ, Dales RE, Desmarais RL. The radiographic prevalence of hilar and mediastinal adenopathy in adult cystic fibrosis. Can Assoc Radiol J. Aug 1997;48(4):265-9. [Medline].

  75. Nathanson I, Conboy K, Murphy S. Ultrafast computerized tomography of the chest in cystic fibrosis: a new scoring system. Pediatr Pulmonol. 1991;11(1):81-6. [Medline].

  76. Brasfield D, Hicks G, Soong S. The chest roentgenogram in cystic fibrosis: a new scoring system. Pediatrics. Jan 1979;63(1):24-9. [Medline].

  77. Brasfield D, Hicks G, Soong S. Evaluation of scoring system of the chest radiograph in cystic fibrosis: a collaborative study. AJR Am J Roentgenol. Jun 1980;134(6):1195-8. [Medline].

  78. FitzSimmons SC. The changing epidemiology of cystic fibrosis. J Pediatr. Jan 1993;122(1):1-9. [Medline].

  79. Elkin SL, Fairney A, Burnett S. Vertebral deformities and low bone mineral density in adults with cystic fibrosis: a cross-sectional study. Osteoporos Int. 2001;12(5):366-72. [Medline].

  80. Donadieu J, Roudier C, Saguintaah M, Maccia C, Chiron R. Estimation of the radiation dose from thoracic CT scans in a cystic fibrosis population. Chest. Oct 2007;132(4):1233-8. [Medline].

  81. Lynch DA, Newell J, Hale V, Dyer D, Corkery K, Fox NL. Correlation of CT findings with clinical evaluations in 261 patients with symptomatic bronchiectasis. AJR Am J Roentgenol. Jul 1999;173(1):53-8. [Medline].

  82. Helbich TH, Heinz-Peer G, Fleischmann D. Evolution of CT findings in patients with cystic fibrosis. AJR Am J Roentgenol. Jul 1999;173(1):81-8. [Medline].

  83. Pickhardt PJ, Yagan N, Siegel MJ. Cystic fibrosis: CT findings of colonic disease. Radiology. Mar 1998;206(3):725-30. [Medline].

  84. Bhalla M, Turcios N, Aponte V. Cystic fibrosis: scoring system with thin-section CT. Radiology. Jun 1991;179(3):783-8. [Medline].

  85. Kauczor HU, Chen XJ, van Beek EJ. Pulmonary ventilation imaged by magnetic resonance: at the doorstep of clinical application. Eur Respir J. May 2001;17(5):1008-23. [Medline].

  86. Perez-Aguilar F, Martinez-Sanjuan V, Montoliu G. [Usefulness of magnetic cholangioresonance in the study of hepatobiliary disease in patients adults with cystic fibrosis]. Gastroenterol Hepatol. Mar 2001;24(3):122-6. [Medline].

  87. Dietrich CF, Chichakli M, Bargon J, Wehrmann T, Wiewrodt R, Buhl R. Mediastinal lymph nodes demonstrated by mediastinal sonography: activity marker in patients with cystic fibrosis. J Clin Ultrasound. Jan 1999;27(1):9-14. [Medline].

  88. Munck A, Belbari N, de Lagausie P. Ultrasonography detects appendicular mucocele in cystic fibrosis patients suffering recurrent abdominal pain. Pediatrics. Apr 2000;105(4 Pt 1):921. [Medline].

  89. Ionescu AA, Ionescu AA, Payne N. Subclinical right ventricular dysfunction in cystic fibrosis. A study using tissue Doppler echocardiography. Am J Respir Crit Care Med. Apr 2001;163(5):1212-8. [Medline].

  90. Fridell JA, Wozniak TC, Reynolds JM, Powelson JA, Hollinger EF, Duncan MW. Bilateral sequential lung and simultaneous pancreas transplant: A new approach for the recipient with cystic fibrosis. J Cyst Fibros. Nov 22 2007;[Medline].

  91. Pérez-Enguix D, Morales P, Tomás JM, Vera F, Lloret RM. Computed tomographic screening of pulmonary arterial hypertension in candidates for lung transplantation. Transplant Proc. Sep 2007;39(7):2405-8. [Medline].

  92. Strocker AM, Snijders RJ, Carlson DE. Fetal echogenic bowel: parameters to be considered in differential diagnosis. Ultrasound Obstet Gynecol. Nov 2000;16(6):519-23. [Medline].

  93. Greenholz SK, Krishnadasan B, Marr C, Cannon R. Biliary obstruction in infants with cystic fibrosis requiring Kasai portoenterostomy. J Pediatr Surg. Feb 1997;32(2):175-9; discussion 179-80. [Medline].

  94. Morrison RT, Steuart RD, Chaun H. Multifocal uptake of Tc-99m mebrofenin in dilated liver ductules in a patient with cystic fibrosis. Clin Nucl Med. Jan 1995;20(1):25-6. [Medline].

  95. Cremaschi P, Nascimbene C, Vitulo P, Catanese C, Rota L, Barazzoni GC. Therapeutic embolization of bronchial artery: a successful treatment in 209 cases of relapse hemoptysis. Angiology. Apr 1993;44(4):295-9. [Medline].

  96. Cipolli M, Perini S, Valletta EA. Bronchial artery embolization in the management of hemoptysis in cystic fibrosis. Pediatr Pulmonol. Jun 1995;19(6):344-7. [Medline].

  97. Brinson GM, Noone PG, Mauro MA. Bronchial artery embolization for the treatment of hemoptysis in patients with cystic fibrosis. Am J Respir Crit Care Med. Jun 1998;157(6 Pt 1):1951-8. [Medline].

  98. Fraser KL, Grosman H, Hyland RH. Transverse myelitis: a reversible complication of bronchial artery embolisation in cystic fibrosis. Thorax. Jan 1997;52(1):99-101. [Medline].

  99. Lucas J, Connett GJ, Lea R. Lung resection in cystic fibrosis patients with localised pulmonary disease. Arch Dis Child. May 1996;74(5):449-51. [Medline].

  100. [Best Evidence] Moran F, Bradley JM, Piper AJ. Non-invasive ventilation for cystic fibrosis. Cochrane Database Syst Rev. Jan 21 2009;CD002769. [Medline].

[ CLOSE WINDOW ]

Further Reading

Related eMedicine topics

Cystic Fibrosis (Otolaryngology)

Cystic Fibrosis (Pediatrics)

Clinical guidelines

Newborn screening for cystic fibrosis: evaluation of benefits and risks and recommendations for state newborn screening programs. Centers for Disease Control and Prevention - Federal Government Agency [U.S.].  2004 Oct 15.  37 pages.  NGC:003919

Cystic fibrosis prenatal screening in genetic counseling practice: recommendations of the National Society of Genetic Counselors. National Society of Genetic Counselors - Medical Specialty Society.  2005 Feb.  15 pages.  NGC:004727

Infant/toddler pulmonary function tests-2008 revision & update. American Association for Respiratory Care - Professional Association.  1995 Jul (revised 2008 Jul).  17 pages.  NGC:006698

Pulmonary rehabilitation: joint ACCP/AACVPR evidence-based clinical practice guidelines. American Association of Cardiovascular and Pulmonary Rehabilitation - Medical Specialty Society
American College of Chest Physicians - Medical Specialty Society.  1997 (revised 2007 May).  39 pages.  NGC:005669

Clinical trials
Early Intervention in Pulmonary Exacerbation in Cystic Fibrosis

Can Quantitative Ultrasound be Used for the Evaluation of Bone Health in Adolescents and Adults With Cystic Fibrosis

The Effect of rhDNase on Ventilation Inhomogeneity in Patients With Cystic Fibrosis

FDG-PET Imaging in Young Cystic Fibrosis Patients

[ CLOSE WINDOW ]

Keywords

cystic fibrosis, CF, mucoviscidosis, hyperplastic airway epithelium, cystic fibrosis transmembrane conductance regulator, CFTR, chronic airway obstruction, distal intestinal obstruction syndrome, DIOS

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Eleni Giannouli, MD, Consulting Staff, Assistant Professor, Section of Respirology and Critical Care, Department of Critical Care,Winnipeg Regional Health Authority Health Sciences Center
Disclosure: Nothing to disclose.

Coauthor(s)

Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St. Boniface General Hospital
Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, and World Medical Association
Disclosure: Nothing to disclose.

Bruce Maycher, MD, Director of Pulmonary Radiology, St Boniface General Hospital; Associate Professor, Department of Radiology, University of Manitoba
Bruce Maycher, MD is a member of the following medical societies: American Roentgen Ray Society, Canadian Medical Association, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

Medical Editor

Judith K Amorosa, MD, FACR, Clinical Professor and Program Director, Department of Radiology, University of Medicine and Dentistry of New Jersey, Robert Wood Johnson Medical School; Consulting Staff, Department of Radiology, Robert Wood Johnson University Hospital
Judith K Amorosa, MD, FACR is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Association of University Radiologists, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Eric J Stern, MD, Professor of Radiology, Adjunct Professor of Medicine, Adjunct Professor of Medical Education and Biomedical Informatics, University of Washington School of Medicine; Director of Thoracic Imaging, Harborview Medical Center; Associate Medical Staff, Seattle Cancer Care Alliance
Eric J Stern, MD is a member of the following medical societies: American Roentgen Ray Society, Association of University Radiologists, European Society of Radiology, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Eugene C Lin, MD, Consulting Radiologist, Virginia Mason Medical Center; Clinical Assistant Professor of Radiology, University of Washington School of Medicine
Eugene C Lin, MD is a member of the following medical societies: American College of Nuclear Medicine, American College of Radiology, Radiological Society of North America, and Society of Nuclear Medicine
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.