Liver complications in Alagille syndrome
Alagille syndrome (ALGS) is a rare genetic condition. It mainly affects the liver, but can also affect other parts of the body, including the heart, kidneys, eyes, face, skeleton and blood vessels.
The information on this page is for:
- Parents of children with Alagille syndrome or suspected Alagille syndrome.
- Young people with Alagille syndrome.
- Adults with Alagille syndrome.
- Family, friends, carers, and healthcare professionals.
When bile gets trapped in the liver, it can damage the liver over time. It can lead to stiffness and scarring, known as fibrosis. It can also lead to severe scarring, known as cirrhosis. This damage can happen in childhood or later in adulthood.
The effects of severe scarring of the liver may include:
On this page:
Portal hypertension, varices and ascites
When the liver is damaged, it becomes stiff. This stiffness makes it harder for blood to flow through the liver. It causes high pressure in the vein which carries blood from organs in the tummy to the liver. This is called portal hypertension. Portal hypertension can cause several problems:
- the spleen can get bigger
- swollen blood vessels can form in the food pipe (called oesophageal varices)
- fluid may build up in the tummy (called ascites)
Liver failure
Liver failure happens when large parts of the liver become damaged and scarred. The liver can’t work properly and is said to be failing. This is a late stage of liver disease. Doctors find it hard to predict who will get liver failure and if this will happen during childhood or later in life.
Support
How Liver UK can help
A diagnosis of liver disease can be worrying, and you may have a lot of questions.
We're here for you and for your family and friends. Whether you have questions or just need someone to listen, we can help.
Your feedback
This content was last reviewed: July 2026
Our expert reviewers:
We would like to thank everyone who helped with creating and reviewing this page. Including Professor Alistair Baker, Consultant Paediatric Hepatologist, King’s College and Lindsay Hogg, Principal Specialist Nurse, Birmingham Women’s and Children’s Hospital. And all our parent and family reviewers.
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