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Cystic Fibrosis and Liver Disease

Some children and young people with cystic fibrosis may have had prolonged jaundice as an infant. This usually disappears in a few months after birth. However, a number of children and young people with cystic fibrosis develop liver problems later in life regardless of whether they had prolonged jaundice in infancy.

It is not possible to predict who will be affected, but boys seem to be affected more than girls.The development of liver disease may be more likely in children who have had "meconium ileus" as a baby.

What happens to the liver in cystic fibrosis?

Damage to the liver starts in the small bile ducts. The bile produced by the liver cells into these ducts becomes stickier than normal and blockage of the ducts follows (this very occasionally causes jaundice). The surrounding liver tissue then becomes damaged and scarred, which may be referred to as biliary fibrosis. This is not unlike the underlying process occurring in the lungs. In time the fibrosis progresses and eventually spreads throughout the liver. This causes the liver to become hard and the blood flow through it more difficult.

How is a diagnosis made?

Most children and young people with cystic fibrosis have regular check-ups which include:

  • Physical examination
  • Liver function tests
  • Abdominal ultrasound

What are the main effects of liver disease in children with cystic fibrosis?

  • Portal hypertension
  • Enlarged spleen
  • Disturbance of the normal working of the liver

Treatments may include:

  • Medication
  • Nutrition

Diagnosis and treatment of varices, including:
  • Endoscopy
  • Sclerotherapy
  • Banding
  • TIPS
  • Medication

Want to know more?

For more information, download CLDF's leaflet.

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