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Cystic Fibrosis
Book chapter

Cystic Fibrosis

Michael Wilschanski and Aliye Uc
Textbook of Pediatric Gastroenterology, Hepatology and Nutrition, pp.471-485
Springer International Publishing
10/01/2015
DOI: 10.1007/978-3-319-17169-2_41

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Abstract

The cystic fibrosis transmembrane regulator protein (CFTR) is a chloride and bicarbonate channel situated at the apical surface of secretory membranes. Cystic fibrosis (CF) results from a dysfunction of this ion channel. CFTR dysfunction affects the pancreas, hepatobiliary system, and the intestinal tract. This chapter describes the gastrointestinal and nutritional manifestations of CF with current medical management.
Pancreatic sufficiency Fat-soluble vitamins Pancreatic enzyme replacement therapy Cystic fibrosis Pancreatic insufficiency

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