July 02, 2007

Liver Diseases - Cirrhosis of the liver


The liver is an organ that can normally withstand a lot of damage because of its ability to regenerate. However, because of progressive damage due to whatever cause, this 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.

Statistics Summary of Cirrhosis of the liver
It is difficult to estimate the incidence as cirrhosis can often be silent until the patient develops liver failure - 30-40% of cases discovered at autopsy were clinically silent. Its incidence is around 2/100,000. It also depends on the prevalence of the various causes - for example the Hepatitis B infection is very prevalent in South-East Asia.

Predisposing Factors of Cirrhosis of the liver
Chronic alcohol abuse and hepatitis (due to Hepatitis B and Hepatitis C most commonly) are the most important causes of cirrhosis.

Other causes include:
- Biliary cirrhosis (primary or secondary).
- Metabolic causes: haemochromatosis, Wilson's disease, Alpha-1 antitrypsin deficiency.
- Auto-immune hepatitis.
- Primary sclerosing cholangitis.
- Budd-Chiari syndrome.
- Drugs (amiodarone, methyldopa, methotrexate).
- The cause is unknown in 30% of cases (cryptogenic cirrhosis).

Progression of Cirrhosis of the liver
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.

An acute illness may tip some patients over the edge - causing liver failure i.e. decompensated cirrhosis. Decompensated cirrhosis may be precipitated by: infection, gastrointestinal bleeds, alcohol, electrolyte imbalances, progression of underlying diseases or liver cancer.

Probable Outcomes of Cirrhosis of the liver
Cirrhosis is an irreversible process. In most cases there is no medical treatment that alters disease history. However, stopping
alcohol does stop progression of alcoholic cirrhosis. Patients with well compensated cirrhosis who abstain may have a survival similar to normal.

Diagnosis and Testing for Cirrhosis of the liver
- 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.

Treatment Overview of Cirrhosis of the liver
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.

An ultrasound and alpha fetoprotein every 3 months to screen for liver cancer.

Liver transplantation can be performed in many patients provided the patient is compliant (eg. abstains from alcohol), of reasonable health (no active malignancy or infection, no HIV) and is psychologically committed.

Symptoms of this disease
Variceal bleeding

Treatments used in this disease
Liver transplants (see next post)

Adapted from: Virtual Medical Centre