Due to the progressive damage caused by Biliary Atresia (or any other cause), the damage is often accompanied by fibrosis (scar tissue). As this progresses there is progressive scarring of the liver and distortion of its architecture - termed cirrhosis, which is irreversible.
In cirrhosis there is less viable liver tissue which can perform the liver's normal function, hence this can lead to liver failure, and there is also a build up of pressure in the veins that carry blood from the gut to the liver (the portal system) which leads to portal hypertension.
Click on the image below to see a video on Liver Cirrohsis:
Progression
Cirrhosis develops with progressive liver injury, with formation of fibrous septa and regeneration (forming nodules), thus distorting the architecture.
With continuing damage to the liver there is a gradual reduction in liver function - this can be seen as a falling albumin level, onset of coagulation abnormalities, as well as a rising bilirubin level. There is also progressive onset of portal hypertension which leads to splenomegally, ascites, and the formation of oesophageal varices. Having cirrhosis also puts the patient at risk for liver cancer.
Probable Outcomes
Cirrhosis is an irreversible process. In most cases there is no medical treatment that alters disease history.
How is Cirrhosis of the liver Diagnosed?
- Liver function tests - normal or increased bilirubin, AST,ALT, ALP, GGT. Later with decreased synthetic function there may be decreased albumin and increased PT/INR.
- Coagulation profile - may be abnormal with progressive liver failure.
- Full blood count - decreased white cell count and platelets indicate hypersplenism (an abnormally enlarged spleen often "chews up" blood cells)
- Urea and electrolytes - may be abnormal renal function. Low sodium is prognostically important.
How is Cirrhosis of the liver treated?
There is no treatment for cirrhosis itself short of liver transplantation but management involves treating (or avoiding further progression) of the cause, managing and avoiding further complications.General measures include nutritional supplements, a low protein diet (if encephalopathy), a low salt diet (if ascites). Abstain from alcohol. Avoid NSAIDs, sedatives and opioids.
Symptomatic treatment may be important. Cholestyramine may relieve pruritis (or ursodeoxycholic acid). Ascites is treated with bedrest, fluid restriction, diuretics, and a low salt diet. It can be drained - both therepeutically, but look for any infection in the fliud.
Source:
Virtual Medical Centre http://www.virtualgastrocentre.com
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