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Monitoring and Evaluation of Patients with Cystic Fibrosis

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Handbook of Cystic Fibrosis

Abstract

The monitoring of individuals with cystic fibrosis (CF) has become increasingly complex, as the benefits of screening for complications and proactive early intervention have been realized. The era of newborn screening (NBS) has provided a wealth of information that has demonstrated the importance of optimal nutrition early in life. The prognosis of an individual patient is closely tied to his or her nutritional status and, therefore necessitates close attention to growth parameters. Lung disease is an overwhelming cause of morbidity and mortality, and monitoring of lung disease is an essential component of CF care.

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References

  1. Stallings VA, Stark LJ, Robinson KA, et al. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008;108:832–9.

    Article  PubMed  Google Scholar 

  2. Cystic Fibrosis Foundation, Borowitz D, Robinson KA, et al. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009;155:S73–93.

    Article  Google Scholar 

  3. Simpson RM, Lloyd DJ, Gvozdanovic D, Russell G. Vitamin B12 deficiency in cystic fibrosis. Acta Paediatr Scand. 1985;74:794–6.

    Article  CAS  PubMed  Google Scholar 

  4. Littlewood JM, Wolfe SP, Conway SP. Diagnosis and treatment of intestinal malabsorption in cystic fibrosis. Pediatr Pulmonol. 2006;41:35–49.

    Article  PubMed  Google Scholar 

  5. Colombo C, Ellemunter H, Houwen R, et al. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients. J Cyst Fibros. 2011;10 Suppl 2:S24–8.

    Article  PubMed  Google Scholar 

  6. Kelly T, Buxbaum J. Gastrointestinal manifestations of cystic fibrosis. Dig Dis Sci. 2015;60:1903–13.

    Article  PubMed  Google Scholar 

  7. Borowitz D, Gelfond D. Intestinal complications of cystic fibrosis. Curr Opin Pulm Med. 2013;19:676–80.

    Article  CAS  PubMed  Google Scholar 

  8. Sokol RJ, Durie PR. Recommendations for management of liver and biliary tract disease in cystic fibrosis. Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group. J Pediatr Gastroenterol Nutr. 1999;28 Suppl 1:S1–13.

    Article  PubMed  Google Scholar 

  9. Gory I, Brown G, Wilson J, Kemp W, Paul E, Roberts SK. Increased risk of colorectal neoplasia in adult patients with cystic fibrosis: a matched case–control study. Scand J Gastroenterol. 2014;49:1230–6.

    Article  PubMed  Google Scholar 

  10. Brody AS, Tiddens HA, Castile RG, et al. Computed tomography in the evaluation of cystic fibrosis lung disease. Am J Respir Crit Care Med. 2005;172:1246–52.

    Article  PubMed  Google Scholar 

  11. Dournes G, Menut F, Macey J et al. Lung morphology assessment of cystic fibrosis using MRI with ultra-short echo time at submillimeter spatial resolution. Eur Radiol. 2016. [Epub ahead of print].

    Google Scholar 

  12. Altes TA, de Lange EE. Applications of hyperpolarized helium-3 gas magnetic resonance imaging in pediatric lung disease. Top Magn Reson Imaging. 2003;14:231–6.

    Article  PubMed  Google Scholar 

  13. Rosenfeld M, Allen J, Arets BH, 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;10:S1–11.

    Article  PubMed  Google Scholar 

  14. Aurora P, Stanojevic S, Wade A, et al. Lung clearance index at 4 years predicts subsequent lung function in children with cystic fibrosis. Am J Respir Crit Care Med. 2011;183:752–8.

    Article  PubMed  Google Scholar 

  15. Subbarao P, Milla C, Aurora P, et al. Multiple-breath washout as a lung function test in cystic fibrosis. A Cystic Fibrosis Foundation workshop report. Ann Am Thorac Soc. 2015;12:932–9.

    Article  PubMed  Google Scholar 

  16. Cystic Fibrosis Foundation Patient Registry. 2014 annual data report. 2015. https://www.cff.org/Our-Research/CF-Patient-Registry/. Accessed 16 Mar 2016.

  17. Brody AS, Klein JS, Molina PL, Quan J, Bean JA, Wilmott RW. High-resolution computed tomography in young patients with cystic fibrosis: distribution of abnormalities and correlation with pulmonary function tests. J Pediatr. 2004;145:32–8.

    Article  PubMed  Google Scholar 

  18. Hauser AR, Jain M, Bar-Meir M, McColley SA. Clinical significance of microbial infection and adaptation in cystic fibrosis. Clin Microbiol Rev. 2011;24:29–70.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Brennan S, Gangell C, Wainwright C, Sly PD. Disease surveillance using bronchoalveolar lavage. Paediatr Respir Rev. 2008;9:151–9.

    Article  PubMed  Google Scholar 

  20. Stevens DA, Moss RB, Kurup VP, et al. Allergic bronchopulmonary aspergillosis in cystic fibrosis--state of the art: Cystic Fibrosis Foundation Consensus Conference. Clin Infect Dis. 2003;37 Suppl 3:S225–64.

    Article  PubMed  Google Scholar 

  21. Moran A, Pillay K, Becker DJ, Acerini CL, 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;15 Suppl 20:65–76.

    Article  CAS  PubMed  Google Scholar 

  22. Moran A, Brunzell C, Cohen RC, et al. 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. 2010;33:2697–708.

    Article  PubMed  PubMed Central  Google Scholar 

  23. Aris RM, Merkel PA, Bachrach LK, et al. Guide to bone health and disease in cystic fibrosis. J Clin Endocrinol Metab. 2005;90:1888–96.

    Article  CAS  PubMed  Google Scholar 

  24. Mainz JG, Koitschev A. Pathogenesis and management of nasal polyposis in cystic fibrosis. Curr Allergy Asthma Rep. 2012;12:163–74.

    Article  CAS  Google Scholar 

  25. Frayman KB, Sawyer SM. Sexual and reproductive health in cystic fibrosis: a life-course perspective. Lancet Respir Med. 2015;3:70–86.

    Article  PubMed  Google Scholar 

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Filbrun, A.G., Lahiri, T., Ren, C.L. (2016). Monitoring and Evaluation of Patients with Cystic Fibrosis. In: Handbook of Cystic Fibrosis. Adis, Cham. https://doi.org/10.1007/978-3-319-32504-0_5

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  • DOI: https://doi.org/10.1007/978-3-319-32504-0_5

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  • Publisher Name: Adis, Cham

  • Print ISBN: 978-3-319-32502-6

  • Online ISBN: 978-3-319-32504-0

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