Pancreas and cystic fibrosis: the implications of increased survival in cystic fibrosis

Pancreatology. 2007;7(5-6):447-50. doi: 10.1159/000108961. Epub 2007 Oct 1.

Abstract

Pancreatitis affects 0.5% people with cystic fibrosis (CF) in the UK and 0.01% of the normal population. Why do some with CF get pancreatitis and some not? And does pancreatitis in neonates result in pancreatic failure with no further inflammation or risk of pancreatic cancer? Review of the literature would suggest that 85% of those with CF have pancreatic destruction as children with minimal risk of further inflammatory or neoplastic changes. Those with a functioning pancreas are at risk of developing pancreatitis. There are several case series of pancreatic cancer reported in CF patients, but overall the risk is unknown. As patients with CF and pancreatic sufficiency are living longer, further studies to assess the risk of developing pancreatic cancer in this subgroup should be considered.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Child
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / epidemiology
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Humans
  • Incidence
  • Infant
  • Pancreas / physiology*
  • Pancreatic Diseases / etiology*
  • Pancreatic Neoplasms / etiology
  • Pancreatitis / epidemiology
  • Pancreatitis / etiology

Substances

  • CFTR protein, human
  • Cystic Fibrosis Transmembrane Conductance Regulator