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Nutrition in Cystic Fibrosis

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Book cover Nutrition in Pediatric Pulmonary Disease

Part of the book series: Nutrition and Health ((NH))

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Abstract

Cystic fibrosis (CF) is a chronic multisystem disease and the most common recessive life-threatening illness in North America. In the United States, there is an estimated incidence of 1 in 2,906 Caucasian and 1 in 10,338 non-Caucasian live births [1]. Affected individuals demonstrate dysfunction of a multifunctional ion-channel protein, the cystic fibrosis transmembrane conductance regulator (CFTR) [1]. This protein acts as an important ion channel which regulates the hydration of airway surface liquid. In addition, mutations in CFTR give rise to a range of clinical manifestations extending from classic CF with airway obstruction and pancreatic insufficiency to single organ pathology. Mucosal obstruction of exocrine glands such as goblet cells in the respiratory and gastrointestinal tracts is the chief contributor to morbidity and mortality in patients with cystic fibrosis.

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Authors and Affiliations

  1. Pediatric Pulmonary Medicine, Lucile Packard Children’s Hospital at Stanford University, Stanford University School of Medicine, 770 Welch Road, Palo Alto, CA, 94304, USA

    Anne C. Coates M.D. & John D. Mark M.D.

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  1. Anne C. Coates M.D.
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  2. John D. Mark M.D.
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Correspondence to John D. Mark M.D. .

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Editors and Affiliations

  1. Raby Institute for Integrative Medicine, Chicago, Illinois, USA

    Robert Dumont

  2. Department of Pulmonary Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA

    Youngran Chung

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Coates, A.C., Mark, J.D. (2014). Nutrition in Cystic Fibrosis. In: Dumont, R., Chung, Y. (eds) Nutrition in Pediatric Pulmonary Disease. Nutrition and Health. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4614-8474-5_5

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  • DOI: https://doi.org/10.1007/978-1-4614-8474-5_5

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