What is cholestasis?

Cholestasis is a problem with the flow of bile that happens when the bile ducts are blocked or the liver has a problem producing bile. Children with cholestasis have trouble absorbing nutrients and gaining weight.

In a healthy system, bile flows through a tube called the common bile duct to the small intestine, where it helps to absorb fat. Bile also helps eliminate bilirubin, a waste product of the body’s continuous break down old red blood cells and creation of new ones.

What is bile?

Bile is liquid produced in the liver and excreted into the intestine. Bile consists of water, electrolytes, bile salts, bilirubin, cholesterol and other substances that our body wants to get rid of.

  • Bile salts play an important role in helping the body absorb fat.
  • Bilirubin, another component of bile, is formed by the breakdown of hemoglobin. It gives bile a yellowish-greenish color.

A child develops cholestasis when bile backs up in the blood stream. When bile and bilirubin stay in the body, a child will develop jaundice

Who is at risk for cholestasis?

Cholestasis is a rare condition that affects approximately 1 in every 2,500 newborn infants. Boys and girls have the condition in roughly equal numbers. In newborns, cholestasis can be an inherited condition. The condition can affect children of any age.

Some women develop cholestasis in pregnancy. This typically occurs in the third trimester and clears up after they give birth. Cholestasis in pregnancy does not increase a baby’s risk of being born with the condition, however, it does increase the risk of premature birth.

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:

Conditions and treatments that destroy the liver cell or change its function increase a child’s risk of hepatocellular cholestasis:

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 2 to 4 weeks

 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.