Congenital hepatic fibrosis: A rare inherited birth disorder characterized by fibrosis (scarring) of the liver which affects its ability to function. The severity of the condition is variable with some patients being symptomatic during infancy while others may be asymptomatic for most of their life. More detailed information about the symptoms, causes, and treatments of Congenital hepatic fibrosis is available below.
Symptoms of Congenital hepatic fibrosis
- Kidney dysfunction
- Liver dysfunction
- Black loose stool
- Blood in vomit
- Gastrointestinal bleeding
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Congenital hepatic fi brosis is an inherited disease that affects both your liver and your kidneys. CHF causes scarring and hardening of the liver, which makes it more diffi cult for the blood to flow through it. This causes a condition called portal hypertension, where there is increased pressure in the veins that carry blood to the liver. Bleeding oesophageal varices – small, protruding veins that line your gullet (oesophagus) – are an early sign of this condition.
If you have CHF your kidney function may also be affected. Some scientists believe that CHF and another condition called autosomal recessive polycystic kidney disease (ARPKD) are part of the same disorder.
Both CHF and ARPKD are very rare, affecting around one person in 20,000 and symptoms occur more in newborns and young children. Both sexes are equally affected.
Symptoms of CHF may be apparent from early childhood. These may include abdominal pain. Your kidneys and spleen may also be enlarged. Complications from portal hypertension may cause bleeding in the stomach, vomiting blood and blood in your faeces (from oesophageal varices). This condition is usually diagnosed in childhood, either because you have an enlarged liver or because of bleeding varices. In some people the diagnosis is not made until adult life.
- An ultrasound scan of your liver and kidneys
- A CT scan of your abdomen
- An X-ray of blood vessels (angiography)
- An endoscopy to detect varices
- A liver biopsy.
CHF differs from cirrhosis because your liver cells remain able to function. There is no specific treatment for the condition but many people require re-routing of blood from the intestines. This is known as a ‘shunt’ operation and is carried out to prevent more intestinal bleeding. A metal or plastic tube (stent) is used to join two veins in order to change the direction of blood fl ow. It is usually done by a radiologist with the help of ultrasound or other imaging equipment to guide them. This procedure will mean at least a day or two in hospital.
Bleeding oesophageal varices can be treated during endoscopy. This is a procedure in which a long, flexible fibre optic tube with a tiny camera on the end (endoscope) is passed into your gullet after you have been sedated.
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