Children's Hospital Colorado
Pediatric Liver Center

Congenital Hepatic Fibrosis

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What is congenital hepatic fibrosis?

Congenital hepatic fibrosis is a rare condition that some children are born with. Congenital fibrosis can affect the liver and the kidneys, causing polycystic kidney disease and hepatic fibrosis (scarring on the liver).

Serious scarring can make the liver become enlarged and cause portal hypertension, which is increased pressure in the veins that carry blood through the liver and spleen.

What causes congenital hepatic fibrosis?

Congenital hepatic fibrosis is an inherited disorder that causes the bile ducts and blood vessels to form improperly. It is an autosomal recessive condition. Bile ducts excrete bile from the liver. Bile is a fluid that helps to digest fats. Bile ducts carry bile from the liver to the gallbladder and small intestine.

The hepatic portal system is a network of veins that carry blood to the liver to be cleaned. This inherited fibrocystic liver disease can also affect the formation of these blood vessels.

Who gets congenital hepatic fibrosis?

Children inherit congenital hepatic fibrosis from their parents, who must both carry a faulty gene to pass on this condition. The parents themselves may not have the disease.

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