PFIC complications
Progressive familial intrahepatic cholestasis (PFIC) is the name given to a group of conditions in which a digestive fluid, called bile, is not released properly from the liver into the gut (gastrointestinal tract).
The information on this page is for:
- Parents of children with PFIC or suspected PFIC.
- Young people with PFIC.
- Family, friends, carers, and healthcare professionals.
On this page:
What are the possible complications of PFIC?
In children with PFIC, there are some possible complications. Bile trapped in the liver may cause damage that gets worse over time. This can cause the liver to become stiff and scarred (fibrosis). It may also lead to severe scarring, known as cirrhosis.
The effects of severe scarring (cirrhosis) of the liver include:
Portal hypertension
When damage happens inside the liver, it stiffens. This makes it more difficult for blood to flow through it. This can cause high pressure in the portal vein. This is the blood vessel that carries blood from many organs in the tummy (abdomen) to the liver. This pressure is known as portal hypertension.
Portal hypertension can make the spleen get bigger. It can also cause swollen blood vessels in the food pipe (oesophageal varices). These blood vessels have thin walls and may bleed. This may cause your child to vomit blood or pass black tarry poo (stools). Both symptoms need urgent medical attention.
Portal hypertension can also cause fluid to build up in the tummy (abdomen). This is known as ascites.
Fluid in the tummy (ascites)
Ascites is the term for a build-up of fluid in the tummy (abdomen). It is caused by:
- increased pressure in some of the blood vessels.
- the reduced ability of the liver to make an important protein called albumin.
The main symptom of ascites is swelling of the tummy (abdomen).
Liver failure
Liver failure happens when large parts of the liver become damaged and scarred. The liver can no longer do its job and is said to be failing. This is a late stage of liver disease.
Increased risk of liver cancer
All types of liver disease increase the risk of liver cancer, but the risk is particularly raised in BSEP deficiency.
To help spot any problems at an early stage, your child will be offered screening for a type of liver cancer known as hepatocellular carcinoma.
How we can help
Support for you
We’re here to support anyone affected by liver disease, as well as their families and loved ones.
Your feedback
This content was last reviewed: March 2024
Our expert reviewers:
We would like to thank everyone who helped with creating and reviewing this page. This information has been produced with input from the three specialist paediatric liver centres in the UK. And with parents and families.
Find out how we make our patient information.
Everyone’s experience of liver disease will be different. Always talk to your specialist medical team for personal advice.
More from Liver UK
Check your risk
Find out more
Support us
Find out more
Keep up to date
Find out more