Pancreatic exocrine function in patients with cystic fibrosis

Curr Gastroenterol Rep. 2005 Jun;7(3):227-33. doi: 10.1007/s11894-005-0039-4.

Abstract

Pancreatic insufficiency in cystic fibrosis (CF) is associated with more severe disease and requires replacement therapy. Outcome measures such as growth and number of stools, frequency of abdominal pain, and flatulence have often been used to identify pancreatic-insufficient patients and to adjust the dose of replacement enzymes. Unfortunately, some patients with CF are misclassified, and approximately 9% do not receive therapy appropriate for their pancreatic exocrine functional status. Growth, number of stools, frequency of abdominal pain, and flatulence cannot be used to adjust enzyme doses.

Publication types

  • Review

MeSH terms

  • Cystic Fibrosis / metabolism*
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism
  • Disease Progression
  • Humans
  • Ion Transport / physiology
  • Pancreas, Exocrine / metabolism*
  • Pancreatic Function Tests
  • Severity of Illness Index

Substances

  • CFTR protein, human
  • Cystic Fibrosis Transmembrane Conductance Regulator