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Cystic fibrosis 

Cystic fibrosis
Chapter:
Cystic fibrosis
Source:
Paediatric Gastroenterology, Hepatology and Nutrition (Oxford Specialist Handbooks in Paediatrics) (1 ed.)
Author(s):

R. Mark Beattie

, Anil Dhawan

, and John W.L. Puntis

DOI:
10.1093/med/9780198569862.003.0021

Gastrointestinal manifestations 156

Management of gastrointestinal symptoms in children with CF 158

Nutrition in CF 158

Nutritional management 159

Vitamins 160

The incidence of cystic fibrosis (CF) is around 1 in 2500. Cases are diagnosed as a consequence of population screening or high-risk screening, or following presentation with clinical symptoms typical of the disorder. The basic defect is in the CFTR (cystic fibrosis transmembrane conductance regulator) protein which codes for a cyclic adenosine monophosphate-regulated chloride transporter in epithelial cells of exocrine organs. This is involved in salt and water balance across epithelial surfaces. The gene is on chromosome 7. There are multiple known mutations, the most common being ...

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