Intrahepatic cholestasis liver diseases can damage the liver and affect digestion and other important processes in the body. The team at Children's Health℠ includes board-certified hepatologists (liver experts) with experience in all kinds of liver disease.
We use the latest methods to diagnose and treat intrahepatic cholestasis so that children with this condition can manage their disease and thrive. If a transplant is necessary, we offer the only pediatric liver transplant program in North Texas, with success rates that meet or exceed national averages. We are also one of the sites for clinical trials exploring treatments for some intrahepatic cholestasis liver diseases in children.
Cholestasis is a condition that means bile is unable to flow out of the liver like it’s supposed to. This can happen for different reasons. With intrahepatic cholestasis, it happens because of problems inside the liver. (“Intrahepatic” means “inside the liver.”) Multiple diseases can cause these problems, and these are known as intrahepatic cholestasis liver diseases.
Bile is an important liquid in our bodies. It helps us get rid of toxins and digest fats. Normally, it is produced in the liver and then flows to the gallbladder and small intestine.
When bile stays in the liver instead of flowing, it can hurt our bodies in multiple ways. The liver can become damaged and lose function. Toxins can build up in the bloodstream. And the body isn’t able to absorb nutrients from fats we eat.
Symptoms of intrahepatic cholestasis liver diseases can resemble other conditions. Confirming a diagnosis may take multiple tests, such as:
Intrahepatic cholestasis liver diseases are caused by genetic changes. Each of these diseases is caused by a different changed gene. Both parents must have and pass on the changed version of one of these genes for their child to inherit one of these diseases.
Treatment for intrahepatic cholestasis depends on the severity of the disease and its effect on the body. Approaches include:
At Children’s Health, we care for children with pediatric intrahepatic cholestasis liver disease at our Pediatric Liver Disease Center. Our team includes board-certified hepatologists (liver experts) who work alongside dietitians and other specialists to care for all your child’s health needs. Our hepatologists also have special training in liver transplant, with success rates that are consistently better than national averages.
These diseases are genetic, so there is no way to prevent them. But if diagnosed and treated early, they can be managed effectively.
Not necessarily. Children in early stages of disease often do well by following the care and nutrition plans set by their healthcare providers. A liver transplant is necessary when the liver has become too damaged to function well. A new liver gives a child a fresh start and, in many cases, makes their symptoms go away.