Cholestasis | Symptoms & Causes
What are the symptoms of cholestasis liver disease?
Cholestasis symptoms include:
- jaundice, yellowish eyes and skin
- dark yellow or brown urine
- grey or white stools
- swollen or extended abdomen
- itchy skin
- nausea or vomiting
- slow weight gain
What causes cholestasis?
There are two types of cholestasis that affect infants and children:
- obstructive cholestasis, blockage of bile flow
- hepatocellular cholestasis, a problem with the production of bile at the liver (hepatic) cell level
The following conditions increase a child’s risk of obstructive cholestasis:
- biliary atresia, caused by blocked or damaged bile ducts
- choledochal cysts, an inherited malformation of the bile ducts
- Alagille syndrome, an inherited condition in which a child has underdeveloped/ too few bile ducts
- cystic fibrosis that causes thick bile and secretions that block the bile ducts
Conditions and treatments that destroy the liver cell or change its function increase a child’s risk of hepatocellular cholestasis:
- hepatitis A, hepatitis B, or hepatitis C infection
- certain medications and total parenteral nutrition
Are some children at greater risk for cholestasis?
Certain things can increase a child’s risk for developing cholestasis, including:
- premature birth, being born before 37 weeks of pregnancy
- short bowel syndrome, loss of a functioning small intestine
- receiving parenteral nutrition, food delivered through a vein, for more than two to four weeks
Cholestasis | Diagnosis & Treatments
How is cholestasis diagnosed?
Your child’s doctor may order blood tests to measure the level of liver enzymes and bilirubin in your child’s blood. A high bilirubin level could indicate that not enough bile is present to carry this waste product out of the body.
Other tests to help identify the exact cause of cholestasis may include:
- blood testing for infections and inherited genetic conditions
- imaging tests such as a CT scan, MRI, or ultrasound
- liver biopsy
How is cholestasis treated?
Treatment depends on the cause. If your child has cholestasis due to medication or parenteral nutrition, the condition will clear up when those treatments are discontinued.
If cholestasis is caused by biliary atresia, your child will need surgery called Kasai portoenterostomy. This procedure restores the drainage of bile from the bile ducts directly into the intestines.
If your child has a chronic liver condition, a liver doctor will work with your family to manage the condition and keep your child’s liver as healthy as possible.
Other treatments may include:
- high-calorie diet with special type of fat and extra fat soluble-vitamins to help your child gain weight
- medications for itchy skin
In the most severe cases, a child with cholestasis may develop cirrhosis or liver failure and need a liver transplant.
How we care for cholestasis
The Center for Childhood Liver Disease at Boston Children’s Hospital takes a multidisciplinary approach to diagnosing and treating cholestasis. Our multidisciplinary team specializes in helping infants, children, adolescents, and young adults who have a wide variety of liver, gallbladder, and bile duct disorders. At every step, our specialists endeavor to provide compassionate care that respects the values of each family and addresses their hopes and concerns for their child’s present and future health. Doctors refer children with liver disease to our program from all over the world.
