Congenital Hepatic Fibrosis
Also known as: CHF.
What is congenital hepatic fibrosis?
Congenital hepatic fibrosis is a disease that affects the liver. Specifically, it’s known for causing structural problems with the bile ducts and blood vessels of the liver that are present at birth. Congenital hepatic fibrosis is often associated with other conditions, such as ones in which cysts are noted within the kidneys.
What causes congenital hepatic fibrosis?
Congenital hepatic fibrosis appears to be related to a number of possible genetic mutations. It is a hereditary disorder that can be passed down from parents to their children.
What are the symptoms of congenital hepatic fibrosis?
Congenital hepatic fibrosis may lead to enlargement of the spleen and the development of varicose veins at the base of the food pipe that may lead to vomiting of blood. Additionally, abnormal liver function may be seen, leading to suboptimal absorption of nutrients from the gastrointestinal tract. In persons with congenital hepatic fibrosis that occurs as part of a syndrome, other symptoms and signs may be seen that arise secondary to abnormalities in affected organs such as the kidneys.
What are congenital hepatic fibrosis care options?
There is no cure for congenital hepatic fibrosis. Treatment is focused on treating the symptoms of the disease, such as gastrointestinal bleeding and nutritional complications.
Reviewed by: Shifra A Koyfman, MD
This page was last updated on: 2/3/2018 4:09:02 PM
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The Section of Pediatric Gastroenterology at Nicklaus Children's is growing to better meet the needs of our community, we have opened a new office on the hospital's main campus!