Cirrhosis of the Liver

Overview

Cirrhosis (pronounced sur-o-sis) of the liver occurs when cells in the liver are damaged and scar tissue forms. This scar tissue causes blood flow to be blocked and waste products to build up in the body. In cirrhosis, normal areas of liver are surrounded by scarred areas that do not function properly.

People often think of cirrhosis as a disease caused by long-term alcohol abuse. While this is sometimes a factor in adults, cirrhosis in children often stems from liver disorders. Cirrhosis of the liver has many causes, including:

• Hepatitis viruses B, C, and D
• Autoimmune hepatitis
• Inherited diseases:
  • Glycogen storage disease
  • Galactosemia
  • Fructose intolerance
  • Tyrosinemia
  • Hemochromatosis
  • Wilson's disease
  • Alpha1-antitrypsin deficiency
  • Cystic fibrosis
• Bile duct blockages (e.g. biliary atresia) associated with:
  • Congenital defects
  • Scarred ducts
  • Gallbladder surgery
• Drugs and toxins:
  • Arsenic
  • Isoniazid
  • Methotrexate
  • Excess vitamin A
  • Excess iron
• Infections:
  • Schistosomiasis
  • Brucellosis
  • Echinococcosis
  • Advanced or congenital syphilis

Symptoms

Cirrhosis of the liver itself often causes no symptoms early in the disease process. Symptoms start when the liver begins to fail, as scar tissue replaces healthy cells. Symptom severity depends on the extent of liver damage.

A person in the early stages of cirrhosis may feel fatigued, weak, nauseated. Sometimes he or she will experience abdominal swelling that feels tender or painful. Family may notice the person has a poor appetite or is losing weight.

As the disease progresses, bile flow is blocked or stopped, and jaundice (yellow skin or eyes) appears. The same bile pigment, bilirubin, which is responsible for the yellow skin tones of jaundice can turn urine dark. Bleeding and bruising can occur more easily and take longer to heal. Other later symptoms, some due to complications, include:

• Reddened palms
• Loss of body hair
• Enlarged liver
• Enlarged spleen
• Appearance of thin, purplish-red, spidery looking blood vessels on the skin, especially around the navel
• Water retention and swelling in the legs and abdomen
• Vomiting blood
• Itching
• Abdominal infections
• Forgetfulness or confusion
• Tremors
• Inability to fully process drugs
• Enlarged, twisted, thin-walled blood vessels called varices that bleed easily
• Liver cancer

Diagnosis

If your child's doctor suspects that your child has cirrhosis, he or she will perform tests to confirm or rule out the diagnosis. Tests may include:

• Blood Tests – to assess how well the liver is working and determine a cause
• CT Scan, Ultrasound or Liver/Spleen Scan to identify changes in the liver
• Liver Biopsy – analyzing a sample of liver tissue removed via a thin needle inserted through the abdomen and into the liver
• Laparoscopy – looking at the liver via a thin tube with a lighted tip inserted through a small incision near the belly button
• Other tests include:
  • Measuring the amount of caffeine in the saliva
  • Measuring the pressure within the liver vein
  • Removing fluid from the abdomen and examining it
  • If the cause of your child's liver disease and cirrhosis is not already known, doctors will perform other tests to help find out.

Treatment

Though cirrhosis of the liver cannot be cured or reversed, doctors treat it with the following goals:

• Controlling the cause of the liver damage
• Preventing additional damage
• Treating symptoms and complications
• Treating underlying medical conditions

Your child's doctor may prescribe drugs to control symptoms or fight infections. Some medications are prescribed to get rid of excess fluid in the body or reduce the risk of a blood vessel breaking. Others help your child's body cut down on its absorption of harmful waste products or toxins.

If the complications of cirrhosis can no longer be controlled, or if the liver is in danger of no longer functioning, a liver transplant is often the best option.

Many of the liver disorders that cause childhood cirrhosis are not preventable, but there are precautions you can take. Make sure your child receives hepatitis vaccines at the times your pediatrician recommends. If your child needs to take medications that may damage the liver, follow your doctor's recommendations about blood tests.

People who already have cirrhosis of the liver can prevent or slow further liver damage by following their doctor's instructions regarding diet. Your child may need extra calories and a generous amount of protein to help his or her liver regenerate. If the cirrhosis is more advanced and compromises the liver's ability to process protein properly, the doctor may instead recommend limiting protein. The doctor may also recommend limiting salt in your child's diet, because salt tends to make the body retain water. They may also advise avoiding raw seafood. Make sure your child takes any vitamin supplements prescribed. Due to increased risk of infections, doctors recommend vaccines against flu, pneumonia, and hepatitis for people with cirrhosis of the liver.