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digestive cystic fibrosis

Wednesday 9 February 2005

Cystic fibrosis (CF), the most common lethal autosomal recessive disease in white populations, is characterized by dysfunctional chloride ion transport across epithelial surfaces.

Although recurrent pulmonary infections and pulmonary insufficiency are the principal causes of morbidity and death, gastrointestinal symptoms commonly precede the pulmonary findings and may suggest the diagnosis in infants and young children.

The protean gastrointestinal manifestations of CF result primarily from abnormally viscous luminal secretions within hollow viscera and the ducts of solid organs.

Synopsis

- meconium peritonitis

- neonatal digestive obstruction

  • meconial calcifications (meconial calcifications)
  • meconium peritonitis (meconial peritonitis)
  • meconial ileus (meconium ileus)
    • neonatal intestinal volvulus
    • neonatal intestinal atresia
    • neonatal intestinal perforation
      • meconial peritonitis with abdominal calcifications
  • colonic meconial plug syndrome
    • colonic perforation
      • right colon perforation (7175631)
      • junction left colon-sigmoid colon perforation
      • meconial peritonitis with abdominal calcifications

- in older children with CF

  • intestinal obstruction
    • distal intestinal obstruction syndrome
      • colonic stricture
      • tenacious intestinal residue
      • intestinal intussusception
      • recurrent rectal prolapse
  • rare necrotizing enterocolitis
  • peptic esophageal stricture due to gastroesophageal reflux
  • duodenal ulcer
  • enteritis (10799435)
  • appendicitis due to inspissated secretions
  • neonatal acute appendicitis
  • Clostridium difficile-associated colitis (or antibiotic-associated colitis or pseudomembranous colitis (9930816, 9470027, 8014775)
  • Crohn-like colitis (indeterminate colitis) (9587500)
  • fibrosing colonopathy

- pancreatic anomalies

  • obstruction of ducts and ductules
  • exocrine pancreatic insufficiency
  • pancreatitis
  • small pancreas
  • largely replaced by fat
  • calcifications
  • macrocysts

- hepatic anomalies

  • cholestasis
  • cholelithiasis
  • biliary cirrhosis with portal hypertension
  • steatosis
  • gallstones
  • shrunken nodular liver
  • portal hypertension
    • splenomegaly
    • portosystemic collateral vessels

- increased risk for developing gastrointestinal carcinomas, as early as the 3rd decade.

Localization

- colonic cystic fibrosis

  • cystic fibrosis colonopathy

- appendiceal cystic fibrosis

Videos

- Meconium obstruction with necrosis (by Washington Deceit)

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References

- Agrons GA, Corse WR, Markowitz RI, Suarez ES, Perry DR. Gastrointestinal manifestations of cystic fibrosis: radiologic-pathologic correlation. Radiographics. 1996 Jul;16(4):871-93. PMID: 8835977

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