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Pediatric intrahepatic cholestasis liver disease
Intrahepatic cholestasis liver diseases (in·tra·he·pat·ic - cho·le·sta·sis - liv·er - dis·ease) 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.
What is pediatric intrahepatic cholestasis liver disease?
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.
What are the signs and symptoms of pediatric intrahepatic cholestasis liver disease?
- Dark urine
- Jaundice
- Poor feeding
- Poor weight gain and growth
- Severe itching (pruritus)
- Vomiting
How is pediatric intrahepatic cholestasis liver disease?
Symptoms of intrahepatic cholestasis liver diseases can resemble other conditions. Confirming a diagnosis may take multiple tests, such as:
- Blood test - We measure certain elements in the blood to see how well the liver is functioning and whether bile is removing toxins or not.
- Imaging - These are the different ways of getting an image of the liver so we can look for scarring and structural abnormalities:
- Liver biopsy - We get a sample of the liver and look for evidence of bile buildup.
- Genetic testing - This can tell us exactly which intrahepatic cholestasis liver disease a child has by identifying the gene that is causing it.
What causes pediatric intrahepatic cholestasis liver disease?
Intrahepatic cholestasis liver disease is 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.
How are Pediatric Intrahepatic Cholestasis Liver Diseases treated?
Treatment for intrahepatic cholestasis depends on the severity of the disease and its effect on the body.
- Medication - We may prescribe medicine to protect the liver from damage and help reduce itching or other symptoms.
- Vitamins and diet - Our team includes a registered dietitian who may recommend vitamins and a special diet to help children get the nutrients they need.
- Liver transplant - Sometimes the buildup of bile harms the liver so much that it needs to be replaced. With a new liver, bile is often able to flow properly and symptoms go away.
Pediatric intrahepatic cholestasis liver disease doctors and providers
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.
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Amal Aqul, MD Pediatric Hepatologist -
Jorge Bezerra, MD Pediatric Hepatologist -
Megha Mehta, MD Pediatric Gastroenterologist -
Charina Ramirez, MD Pediatric Gastroenterologist -
Norberto Rodriguez-Baez, MD Pediatric Hepatologist -
Isabel Rojas Santamaria, MD Pediatric Gastroenterologist -
Shabina Walji-Virani, APRN, PNP-PC Nurse Practitioner - Gastroenterology
Frequently Asked Questions
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Can intrahepatic cholestasis liver diseases be prevented?
These diseases are genetic, so there is no way to prevent them. But if diagnosed and treated early, they can be managed effectively.
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Does having intrahepatic cholestasis liver disease mean my child will need a liver transplant?
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.