Fulminant hepatic failure (FHF), also called acute liver failure, is defined as severe liver failure (including encephalopathy or decreased brain function) occurring within six weeks of onset of jaundice (yellowing of the skin and eyes). The term is used to describe sudden liver failure in someone who was previously healthy.

Causes

FHF may be due to a virus but because of a child's young age (infant) or other health problems, his or her immune system can't fight the virus and the liver becomes damaged. Other children may experience acute liver failure because of an overactive immune system (autoimmune; the body attacks the liver), a drug reaction or other unspecified causes. Often, the cause of FHF cannot be determined.

Symptoms

• Jaundice (yellowing of skin and eyes), followed by itching
• Abdominal fluid build-up and swelling (ascites)
• Abdominal pain
• Pale stool
• Dark urine
• Nausea
• Loss of appetite
• Tendency to bruise or bleed easily
• Fatigue
• Fever

As liver failure progresses:

• toxins build-up in the body and may cause brain swelling; child may become confused and display erratic behavior; child may become comatose; death due to brain swelling can occur;
• the liver cannot make clotting factors, resulting in bleeding that can be life threatening;
• the liver cannot excrete (get rid of) bilirubin; child becomes deeply jaundiced
• the kidneys may fail

Exams and Tests

A pediatric GI specialist will need to confirm the liver failure diagnosis and determine the cause. Common tests include:

• Blood tests
• Urine tests
• Ultrasound
• Liver biopsy

Liver Biopsy

Your child will receive sedation medications or a general anesthetic before the biopsy. These medications will prevent your child from feeling pain or anxiety or remembering the procedure. If the child's blood clotting is very poor, a special biopsy through a neck vein may be needed (trans-jugular liver biopsy). Your child's pediatric gastroenterologist will insert a needle through the skin and into the liver to obtain a very small piece of liver tissue. The sample will be sent to the lab to be analyzed. The doctor will apply a small pressure dressing (bandage) to the site.

After the biopsy your child will stay in or return to his/her room. Being in bed will reduce the chance of bleeding after the biopsy. Once your child is awake and alert, he/she will be allowed to drink fluids. If fluids are tolerated, a regular diet can be restarted. The results of the liver biopsy are usually available 2-3 days after the procedure.

Treatment

Children with FHF require intensive care unit (ICU) support in the hospital. They receive intravenous (IV) fluids to balance electrolytes and glucose levels. They may need blood transfusions or hemodialysis (if the kidneys fail). Children who are comatose may need a tube placed in the throat (intubation) to help with breathing. In some cases, the best chance for recovery is liver transplantation.