Pediatric Intrahepatic Cholestasis Liver Diseases
What is intrahepatic cholestasis?
Intrahepatic cholestasis is a problem that affects the release of bile from the liver and can eventually lead to liver disease.
Bile is a liquid produced in the liver that helps remove toxins from the body. It also helps break down the fat in food so it can be processed by the intestines. Any condition that completely blocks the flow of bile from the liver or causes the bile flow to be slower than normal is called cholestasis. Intrahepatic cholestasis means that the cause of the problem started inside the liver.
If your child has intrahepatic cholestasis, three things happen:
- Bile is not released properly, so it builds up in the liver and damages the organ.
- Bile is not released into the bloodstream to help remove toxins, so waste builds up there.
- Bile is not available to help break down fats, so the body cannot correctly absorb necessary nutrients.
Eventually, intrahepatic cholestasis can lead to liver disease.
What causes intrahepatic cholestasis?
Intrahepatic cholestasis is caused by genetic defects that may lead to:
- Progressive familial intrahepatic cholestasis — an inherited gene mutation that affects the normal bile production and bile excretion
- Metabolic and mitochondrial disorders — occur during fetal development
- Idiopathic neonatal hepatitis syndrome — a rare inflammatory condition of the liver that occurs during fetal development
What are the symptoms of intrahepatic cholestasis?
Babies with intrahepatic cholestasis will show symptoms within two weeks of birth, including: