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The pancreas in cystic fibrosis

2002; Elsevier BV; Volume: 3; Issue: 1 Linguagem: Inglês

10.1053/prrv.2002.0183

1526-0550

Chris Taylor, N Aswani,

Pediatric Hepatobiliary Diseases and Treatments

The pancreas secretes a bicarbonate-rich fluid containing digestive enzymes via the ampulla of Vater into the duodenum. Defective secretion leads to maldigestion of fat and protein with increased faecal losses. Cystic fibrosis (CF) is the major cause of pancreatic exocrine failure in childhood, whereas pancreatic insufficiency in adults is commonly associated with chronic pancreatitis and alcohol ingestion. In cystic fibrosis, pancreatic function correlates with genotype; pancreatic-sufficient (PS) patients have a milder course of respiratory disease, improved survival and lower mean sweat chloride concentrations than those with pancreatic insufficiency. Recent observations suggest that mutant CF alleles are over-represented in patients with chronic pancreatitis. Few show evidence of sino-pulmonary disease or high sweat electrolyte concentrations.