Congenital anomalies

Cystic fibrosis

Last author update: 1 August 2012
Last staff update: 30 August 2022

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PubMed Search: Cystic fibrosis pancreas

Deepali Jain, M.D.
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Cite this page: Jain D. Cystic fibrosis. website. Accessed February 6th, 2023.
Definition / general
  • Autosomal recessive disorder affecting most critically the lungs; also pancreas, liver, intestines; characterized by abnormal transport of chloride and sodium across an epithelium, leading to thick, viscous secretions (Wikipedia: Cystic Fibrosis [Accessed 11 December 2017])
  • Incidence: 1 in 20 in U.S. are carriers
  • Most common mutation is #708 (seen in 70% with disease)
  • Mutations cause reduced chloride ion in secretions, thicker respiratory secretions, upper respiratory infections
  • Complications: pancreatic insufficiency late in disease course in 90%, diabetes, malabsorption, pancreatitis
  • Mutations also cause defective cilia and infertility; may cause meconium ileus (5 - 10%), intussusception
  • Cysts form secondary to ductal obstruction due to thick, tenacious secretions, variable numbers of cysts range from 1 to 3 mm, very rarely entire pancreas replaced by multiple macroscopic cysts, termed "pancreatic cystosis"
Gross images

Images hosted on other servers:

At autopsy, pancreas
is mucoid and
slightly smaller
than normal

Microscopic (histologic) description
  • Grade I: accumulation of secretion
  • Grade II: exocrine atrophy
  • Grade III: atrophy with lipomatosis
  • Grade IV: fibrosis with total obliteration of the exocrine glands and ducts with scattered islets of Langerhans

  • Pancreatic ducts diffusely dilated and filled with numerous lamellated concretions
  • Associated with fibrosis
  • Nondiabetic patients have fibrocystic changes with normal islets, prominent nesidioblastosis, some persisting exocrine tissue
  • Young adult diabetic patients have total loss of exocrine pancreas with fat replacement, no nesidioblastosis, reduced islets (Hum Pathol 1984;15:278)
Positive stains

Histopathology pancreas: cystic fibrosis

Differential diagnosis
  • Kartagener syndrome:
    • Defective cilia syndrome
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