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About CF » Pathophysiology of CF » Pancreas

Pancreas

In a healthy pancreas, chloride and bicarbonate (HCO₃⁻) ions are normally secreted through CFTR channels into the lumen of pancreatic ducts. These alkaline secretions contain digestive enzymes essential to break down carbohydrates, proteins and lipids in the small intestine. In CF, abnormal CFTR leads to obstruction of pancreatic ducts, preventing the movement of digestive enzymes into the duodenum, causing:

  • Nutrient malabsorption and failure to thrive
  • Progressive damage and atrophy of the pancreas due to auto-digestion of pancreatic tissue by enzymes

Schematic illustration of the pancreas6
About 95% of people with CF suffer from pancreatic insufficiency from birth.1,2  If left untreated, pancreatic insufficiency can reduce fat absorption to just 50%3 and significantly impair protein uptake.4 As a consequence, many CF sufferers are reliant on enzyme replacement therapy.5

In addition, up to 30% of people with CF also suffer from diabetes mellitus (DM) by the age of 25.6 Cystic fibrosis-related diabetes (CFRD) has characteristics of both type I and type II DM.7

Did you know…

Did you know there is a correlation between CF disease severity and pancreatic sufficiency?

CF sufferers who are pancreatic insufficient have ‘severe’ class I, II or III CFTR mutations, whereas pancreatic-sufficient individuals have a milder form of the disease (e.g. class IV mutation).8

Molecular mechanisms

Find out more about the molecular mechanisms of pancreatic insufficiency.

References
  1. Davis PB et al. Am J Respir Crit Care Med 1996;154:1229–56
  2. Littlewood JM et al. Pediatr Pulmonol 2006;41:35–49
  3. Stern RC et al. Am J Gastroenterol 2000;95:1932–8
  4. Gow R et al. Lancet 1981;2:1071–4
  5. Wilschanski M et al. Gut 2007;56:1153–63
  6. Rosenecker J et al. J Pediatr 1995;127:441–3
  7. Wilschanski M et al. Gut 2007;56:1153–63
  8. Rowntree RK et al. Ann Hum Genet 2003;67:471–85