Liver Failure

Liver failure is the severe decline of liver function. It is an uncommon but serious condition that occurs when the liver is impaired and can no longer perform important tasks like clearing toxins from the blood or producing bile, a substance that helps digest food.

Liver failure can affect infants, toddlers, older children, and adolescents.

There are two kinds of liver failure: acute liver failure and chronic liver failure.

Acute liver failure, also called fulminant hepatic failure, is the rapid loss of liver function over the course of days or weeks in a child who has no previous history of liver disease. Acute liver failure is a medical emergency that requires immediate medical attention.

The most common cause of acute liver failure in children is a toxin or virus that damages the liver.

Chronic liver failure, also known as end-stage liver disease, develops over a period of months or years as the result of damage caused by long-term liver disease such as biliary atresia, metabolic liver disease, hepatitis B or hepatitis C, or cirrhosis.

Without medical attention, a child with liver failure will become confused and disoriented, a condition known as encephalopathy.

Most children with acute or chronic liver failure will need a liver transplant. In a small number of cases of liver failure, 15 percent to 20 percent, the liver repairs itself spontaneously.

The liver is the second largest organ in the body. Located in the abdominal cavity, the liver helps the body in many ways.

The liver:

• Produces proteins that allow blood to clot normally, transport oxygen, and support the immune system
• Produces bile, a substance that helps digest food
• Stores extra nutrients
• Helps clean the bloodstream of harmful substances
• Helps control blood sugar and cholesterol levels

In many cases, doctors are unable to determine the cause of acute liver failure.

When the cause is known, the most common cause is taking too much acetaminophen, the active ingredient in Tylenol and other cold medications. Acetaminophen can damage the liver after one large dose or after a child takes more than the recommended dose for several days in a row.

Other prescription medications and even some herbal supplements, such as kava, ephedra, skullcap, and pennyroyal, can also cause acute liver failure.

In a small number of cases, acute liver failure is caused by a viral infection such as herpes simplex or Epstein-Barr.

Chronic liver failure occurs when a long-term illness damages the liver. The following liver conditions can lead to chronic liver failure:

• Biliary atresia, when a child is born with blocked or damaged bile duct, the tube that bile, fluid produced by the liver, flows through on its way to the intestines
• Metabolic liver diseases like Wilson disease, in which the body is unable to process copper from food and the copper builds up in the liver.
• Chronic viral hepatitis C, which causes the liver to swell and encourages scar tissue to form

Jaundice, a yellow discoloration of the skin and eyes, is usually the first and sometimes the only sign of liver disease. Other symptoms include:

• Confusion or disorientation
• Pain or tenderness in the upper right abdomen
• Weakness
• Fatigue
• Nausea
• Vomiting
• Dark urine
• Easy bleeding
• Itching
• Small, spider-like vessels visible in the skin
• Chills

Liver failure may cause:

• Liver encephalopathy: a drop in brain function caused by the buildup of toxic substances in the blood
• Jaundice: a yellow discoloration of the skin and whites of your child's eyes due to an abnormally high level of bilirubin (bile pigment) in the bloodstream
• Coagulopathy: a bleeding disorder that happens when blood does not clot and can result in excessing bleeding from even a small cut
• Portal hypertension: abnormally high blood pressure in the vein that brings blood from the intestine to the liver (the portal vein), that can cause a distended abdominal cavity (ascites), prominence of abdominal wall veins, or bleeding of the varicose veins at the lower end of the esophagus or the stomach lining
• Ascites: the build up of fluid in the abdominal cavity caused by fluid leaks from the vessels on the surface of the liver and intestine that can make a child feel short of breath
• Hepatomegaly: an enlarged liver that may cause abdominal pain or a feeling of fullness

To diagnose liver disease, clinicians typically consider the child’s symptoms and conduct a physical examination. The following tests are often part of the diagnostic process.

Blood tests

A sample of the child's blood may be tested for the following signs of liver disease:

• Albumin, a protein made by the liver and found in the bloodstream & lower-than-normal levels are associated with many liver disorders.
• Bilirubin, a substance produced by the liver and excreted in the bile. Elevated levels of bilirubin indicate a problem with the liver.
• Liver enzymes, elevated levels of liver enzymes can alert physicians to liver damage or injury.
• Prothrombin time (PT) test, measures the time it takes for blood to clot. Since blood clotting requires vitamin K and a protein made by the liver, liver cell damage and bile flow obstruction can interfere with proper blood clotting.

Abdominal ultrasound (also called sonography) enables clinicians to view a child's liver function and assess blood flow through various vessels.

Liver biopsy, a procedure that takes a small sample of liver tissue to be examined under a microscope. Besides helping diagnose liver disease, a biopsy can give doctors a better idea of the extent of damage and make decisions about the best treatment for the child.

Computerized tomography scan (CT or CAT scan), a diagnostic imaging procedure, uses a combination of X-rays and computer technology to show a detailed image of internal organs such as the liver. Images from a CT scan is more detailed than an X-ray alone.

Treatment depends on many factors, including the stage of liver failure and underlying cause. While a healthy liver can recover from damage or illness, acute or chronic liver failure are both severe conditions that often cannot be reversed. In many cases, the only option for a child with liver failure is a liver transplant. Doctors will work with the family to stabilize the child’s condition while they wait for the donation of a healthy liver.

Treatment for a child on a liver transplant wait list may include:

• Treating the underlying condition that caused the initial damage
• Treating the side effects of liver failure

The Center for Childhood Liver Disease at Boston Children’s Hospital specializes in helping infants, children, adolescents, and young adults with a wide variety of liver, gallbladder, and bile duct disorders. Our services include the most up-to-date technology to make an accurate diagnosis and comprehensive treatment plan. Doctors refer children with liver disease to Boston Children’s from all over the world.

Since 1984, specialists in our Liver Transplant Program have performed more than 200 liver transplants. We offer expertise in whole-organ transplants, reduced-size liver transplants, split liver transplants, and related living-donor transplants.

Avoiding liver transplant for patients with metabolic disorders and liver tumors

One of our most promising research projects involves autologous hepatocyte transplantation — transplanting not the liver but only the liver cells, and doing so using cells from the patient to avoid the need for immunosuppression. Although the project is in its early stages, the potential implications are promising.

Eliminating waitlist mortality for children

Infants and young children have the highest risk of death on the liver transplant waiting list, mainly due to the shortage of appropriately sized organs. But Heung Bae Kim, MD, director of Boston Children’s Pediatric Transplant Center, has been researching ways to change that statistic.

Kim led a team of Boston Children’s researchers who examined pediatric data mortality and graft survival rates of patients under the age of 2 who received liver transplants. Published online in Liver Transplantation, a journal of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society, the data suggested that both split and whole organs had similarly low rates of both graft failure and mortality, suggesting that their use could be increased to meet the demand for smaller grafts.

Promoting liver cell regeneration to reduce the need for liver transplant

In addition, researcher Fernando Camargo, PhD, is investigating the different processes that promote liver regeneration and healing. This research could someday help reduce the number of children with liver failure who need a liver transplant.