Congenital hepatic fibrosis
Congenital hepatic fibrosis Causes
Congenital hepatic fibrosis is categorized as an autosomal recessive disorder with no known causative agent or definite cause identified.
Congenital hepatic fibrosis Definition
Congenital hepatic fibrosis (CHF) is an extremely rare form of hereditary disorder that is characterized by the detection of a periportal fibrosis with some marked unevenly shaped producing bile ducts, as well as esophageal varices and intrahepatic portal hypertension. Congenital hepatic fibrosis is often associated with renal function disorder that is typically due to an autosomal recessive polycystic kidney disease.
Congenital hepatic fibrosis Diagnosis
Laboratory tests include examinations of the liver and the renal function. Ultrasonography, ultrasound examinations, CT scans, intravenous pyelography and splenoportography are among the possible imaging tools used to determine the extent of the disease. Liver biopsy and angiography may also be performed to derive a definitive diagnosis.
Congenital hepatic fibrosis Symptoms and Signs
Symptoms for congenital hepatic fibrosis hepatomegaly, evidence of some hypersplenism, experience of abdominal pain particularly on the upper right quadrant of the body. Abnormalities are usually diagnosed after the infant's birth.
Congenital hepatic fibrosis Treatment
The medical therapy provided for congenital hepatic fibrosis patients is mostly during the detected presence of cholangitis. In cases of uncontrolled hemorrhage, surgical shunting or ban litigation may be performed. Surgical care also includes sclerotherapy and portosystemic shunt surgery.