Congenital hepatic fibrosis

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Congenital hepatic fibrosis (pronunciation: /kənˈjenɪtəl hɪˈpatɪk faɪˈbroʊsɪs/) is a rare, genetic, liver disease that is present at birth. It is characterized by the presence of scar tissue in the liver and abnormalities in the bile ducts and blood vessels.

Etymology

The term "congenital hepatic fibrosis" is derived from the Latin congenitus meaning "born with", hepaticus meaning "of the liver", and fibrosis referring to the formation of excess fibrous connective tissue in an organ.

Symptoms

Symptoms of congenital hepatic fibrosis may include jaundice, portal hypertension, splenomegaly, and gastrointestinal bleeding.

Diagnosis

Diagnosis of congenital hepatic fibrosis is often made through a combination of medical history, physical examination, and imaging studies such as ultrasound, computed tomography (CT) scan, and magnetic resonance imaging (MRI). A liver biopsy may also be performed to confirm the diagnosis.

Treatment

Treatment for congenital hepatic fibrosis is primarily aimed at managing the symptoms and complications of the disease. This may include medications to control portal hypertension, procedures to stop gastrointestinal bleeding, and in severe cases, liver transplantation.

Prognosis

The prognosis for individuals with congenital hepatic fibrosis varies and depends on the severity of the disease and the presence of other health conditions. Some individuals may live a normal lifespan with few complications, while others may experience serious health problems.

See also

References


External links

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