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What Is Cystic Fibrosis? The Relationship Between Nutrition and Outcomes in Cystic Fibrosis

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Nutrition in Cystic Fibrosis

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

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Abstract

Cystic fibrosis (CF) is the most common life-shortening inherited disease in the USA, affecting approximately 30,000 children and adults. This autosomal recessive disorder is caused by mutations in the gene encoding the cystic fibrosis transmembrane regulator (CFTR) (Riordan et al., Science 245:1066–1073, 1989). CFTR is an ion channel that is present in multiple organs throughout the body. Mutations in this gene are divided into five major categories. Class I, II, and III mutations generally result in more severe pulmonary disease and pancreatic insufficiency; CF patients with these mutations generally have symptomatic disease in infancy or childhood. Class IV and V mutations typically result in milder disease, which is often diagnosed later in life (O’Sullivan and Freedman, Lancet 373:1891–1904, 2009). Neonatal screening catches most patients in the USA with diagnosis confirmed by sweat or genetic testing. Diagnosis in the neonatal period leads to opportunities to optimize nutrition earlier in life.

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Author information

Authors and Affiliations

  1. Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, UCSF Benioff Children’s Hospital, San Francisco, Mail Code 0136, 550 16th Street, 5th Floor, San Francisco, CA, 94143, USA

    Danielle Usatin M.D.

  2. Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, University of California, San Francisco, CA, USA

    Elizabeth H. Yen M.D.

  3. Benioff Children’s Hospital, San Francisco, CA, USA

    Elizabeth H. Yen M.D.

Authors
  1. Danielle Usatin M.D.
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  2. Elizabeth H. Yen M.D.
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Correspondence to Danielle Usatin M.D. .

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Editors and Affiliations

  1. Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, University of California, San Francisco, San Francisco, California, USA

    Elizabeth H. Yen

  2. Gastorenterology and Nutrition, The Johns Hopkins Children’s Center, Baltimore, Maryland, USA

    Amanda Radmer Leonard

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Usatin, D., Yen, E.H. (2015). What Is Cystic Fibrosis? The Relationship Between Nutrition and Outcomes in Cystic Fibrosis. In: Yen, E., Leonard, A. (eds) Nutrition in Cystic Fibrosis. Nutrition and Health. Humana Press, Cham. https://doi.org/10.1007/978-3-319-16387-1_1

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  • DOI: https://doi.org/10.1007/978-3-319-16387-1_1

  • Publisher Name: Humana Press, Cham

  • Print ISBN: 978-3-319-16386-4

  • Online ISBN: 978-3-319-16387-1

  • eBook Packages: MedicineMedicine (R0)

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