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Cystic fibrosis: abdominal manifestations

Abdominal manifestations in cystic fibrosis are common, varied and nearly all organ systems can be affected.

Only 39% of patients with cystic fibrosis (CF) have pulmonary symptoms as their sole complaint 1. 7% of CF patients do not present until adulthood.

For a general discussion of cystic fibrosis, and a discussion of its other manifestations, please refer to:

Hepatobiliary manifestations

Liver parenchymal disease
  • 40% of CF patients will develop liver disease
  • 1-8% progress to portal hypertension
  • up to 40% progress to focal cirrhosis and up to 12% progress to multilobular cirrhosis 5, but development of HCC is unusual
  • pathophysiology is multifactorial
  • fatty infiltration seen in 30% of CF patients at biopsy, 30-50% at imaging and 60% at autopsy 2
Biliary tree
  • gallstones: 12-24% of CF patients
  • sclerosing cholangitis
  • intrahepatic ductal strictures seen in 100% of patients with CF and liver disease and may correspond histologically to focal biliary fibrosis seen in 78% of CF patients >24 years old
  • microgallbladder: seen in up to 30% of CF patients at autopsy 3

Pancreatic manifestations

Exocrine gland insuffciency affects 85-90% of all CF patients and results from inspissated secretions leading to proximal duct obstruction with subsequent acinar disruption and replacement by fibrous tissue and fat.

Endocrine dysfunction occurs in 30-50% of CF patients

  • fatty replacement: most common manifestation of the pancreas, which can progress to complete pancreatic lipomatosis
  • acute pancreatitis: occurs in patients with residual pancreatic exocrine function
  • pancreatic calcifications occur in 7% of patients
  • pancreatic cysts and cystosis: typically microscopic 3mm diameter
  • pancreatic duct strictures

Gastrointestinal manifestations

  • gastro-oesophageal reflux and associated complications such as Barrett oesophagus: thought to be secondary to chronic cough, hyperinflation and diaphragmatic depression
  • gastric and duodenal ulcerations: likely related to impaired bicarbonate secretion
  • distal intestinal obstruction syndrome (DIOS)
  • intussusception: typically ileocolic and may result from adherent fecal residue or enlarged lymphoid follicles 4; a chronically distended appendix may be the lead point
  • despite the common distention of the appendix by inspissated secretions, the reported prevalence of acute appendicitis in CF patients is lower than that in the general population
  • colon: often abnormal in patients with CF, with proximal colonic wall thickening, pericolonic fat proliferation and mesenteric fat infiltration
  • pneumatosis intestinalis: confined to the colon and typically coincides with the development of obstructive lung disease
  • rectal mucosal prolapse: typically in young children in whom CF diagnosis not yet made or young adults that are non compliant with treatment
  • gastrointestinal tract malignancies: of which colorectal carcinoma is most common

Renal manifestations

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