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Liver Failure

  • Liver failure is severe deterioration of your child’s liver function.

    • Liver failure occurs when a large portion of your child’s liver is damaged due to any type of liver disorder.
    • For a description of normal liver functions

    Pioneering research

    Faculty members of the Boston Children's Hospital Liver Transplant Program are conducting clinical studies to better understand why children’s livers fail and how we can improve liver transplants. We’re constantly striving to translate our research into treatments that can help sick children.

    Treatment at an early stage of liver disease can be managed by specialists in our Center for Childhood Liver Disease and can be quite successful.

    Boston Children's Hospital
    300 Longwood Avenue
    Fegan 5
    Boston MA 02115

     617-355-5837
     fax: 617-730-0716

    Late-stage liver disease, resulting in liver failure, requires a liver transplantation and would be performed by our pediatric transplant specialists.

    Boston Children's Hospital
    300 Longwood Avenue
    Fegan 3
    Boston MA 02115

     617-35-LIVER

     

  • What causes liver failure?

    A number of liver diseases, such as biliary atresia, metabolic liver disease and hepatitis, can lead to liver failure as they progress into late stages.

    Liver diseases that can lead to liver failure share the following characteristics:

    • 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 his bloodstream.
      • High levels of bilirubin may be attributed to inflammation or other abnormalities of the liver cells or blockage of the bile ducts.

      • Sometimes jaundice is caused by the breakdown of a large number of red blood cells, which can occur in newborns.

      • Jaundice is usually the first sign, and sometimes the only sign, of liver disease.

    • Cholestasis - Reduced or stopped bile flow.

      • Bile flow may be blocked inside your child’s liver, outside the liver or in both places.

      • Cholestasis can be caused by hepatitis, metabolic liver diseases, drug effects, a stone in the bile duct, bile duct narrowing, biliary atresia or inflammation of the pancreas.

      • Symptoms of cholestasis may include the following:

        • dark urine
        • jaundice
        • pale stool
        • easy bleeding
        • itching
        • small, spider-like vessels visible in the skin
        • enlarged spleen
        • fluid in the abdominal cavity
        • chills
        • abdominal pain
    • Liver enlargement (hepatomegaly) is usually an indicator of liver disease.
      • There are usually no symptoms associated with a slightly enlarged liver.
      • Symptoms of a grossly enlarged liver include abdominal discomfort or "feeling full."
    • Portal hypertension — Abnormally high blood pressure in your child’s portal vein, which brings blood from the intestine to the liver.
      • Portal hypertension may be due to increased blood pressure in the portal blood vessels or resistance to blood flow through the liver.

      • Portal hypertension can lead to the growth of new blood vessels that bypass the liver.

      • When this occurs, substances that are normally removed by the liver pass into the general circulation.

      • Symptoms of portal hypertension may include:

        • a distended abdominal cavity (ascites)

        • prominence of abdominal wall veins

        • bleeding of the varicose veins at the lower end of the esophagus or the stomach lining

    • Ascites — Fluid build-up in your child’s abdominal cavity caused by fluid leaks from the vessels on the surface of the liver and intestine.
      • Ascites due to liver disease usually accompanies other liver disease characteristics such as portal hypertension.

      • Symptoms of ascites may include a distended abdomen, which causes discomfort and shortness of breath.

    • Liver encephalopathy — The deterioration of your child’s brain function due to toxic substances building up in the blood which are normally removed by the liver.

      • Liver encephalopathy is also called portal-systemic encephalopathy, hepatic encephalopathy, or hepatic coma.

      • Symptoms of liver encephalopathy may include:

        • changes in logical thinking, personality and behavior
        • mood changes
        • impaired judgment
        • drowsiness
        • confusion
        • sluggish speech and movement
        • disorientation
        • loss of consciousness and coma

    What are the symptoms of liver failure?

    • jaundice
    • tendency to bruise or bleed easily
    • ascites
    • impaired brain function (encephalopathy)
    • poor weight gain and growth
    • fatigue
    • weakness
    • nausea
    • loss of appetite
  • How is liver failure diagnosed?

    When diagnosing liver disease, your child’s physician looks at your child’s symptoms and conducts a physical examination. In addition, the physician may request a combination of the following tests:

    Blood tests

    Albumin level
    A sample of blood is obtained from your child's vein. Below-normal levels of albumin — a protein made by the liver and found in the bloodstream — are associated with many chronic liver disorders.

    Bilirubin level
    A sample of blood is taken from your child's vein. Bilirubin is produced by the liver and is excreted in the bile. Elevated levels of bilirubin may indicate an obstruction of bile flow or a defect in the processing of bile by the liver.

    Liver enzymes
    A sample of blood is taken from your child's vein, and the amounts of enzymes that the liver normally makes are measured. Elevated levels of liver enzymes can alert physicians to liver damage or injury, since the enzymes leak from the liver into the bloodstream under these circumstances.

    Prothrombin time (PT) test
    This test measures the time it takes for blood to clot. Blood clotting requires vitamin K and a protein made by the liver. Liver cell damage and bile flow obstruction can both interfere with proper blood clotting.

    Abdominal ultrasound (also called sonography)

    Ultrasounds are used to view your child’s liver as it functions to and assess blood flow through various vessels

    Liver biopsy

    A procedure that takes a small tissue sample for examination.

    Computerized tomography scan (CT or CAT scan)

    A diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called “slices”), both horizontally and vertically, of your child’s liver.

    • A CTscan shows a detailed image of your child’s liver. CT scans are more detailed than general x-rays.

    • Your child will lie on a bed that moves into a doughnut shaped machine that takes many pictures of different areas of the body.

    • Because the machine is noisy, and because your child may need to lie still for a while with his arms over his head, a sedative might be given to help your child rest during the procedure.

  • How is liver failure treated?

    It depends on whether the liver disease is diagnosed and treated before your child's liver fails.

    • Treatment at an early stage of liver disease can be quite successful.
    • Late-stage liver disease, resulting in liver failure, requires a liver transplantation.
    • A liver transplant is an operation performed to replace a diseased liver with a healthy one from another person.
  • Omegaven saves livers

    Many children who have complex intestinal surgery can’t eat normally for a long time. These children are placed on an intravenous method of feeding called pareneteral nutrition (PN).

    PN provides the necessary nutrition for children until their digestive systems adapt and they can eat on their own. It has revolutionized treatment, but its prolonged use often damages the liver, potentially leading to the need for transplant. And unfortunately, infants are at the greatest risk due to the small size of their livers.

    Back in 2001, surgeon Mark Puder, MD, surgical resident Jenna Garza, MD, and pharmacist Kathy Gura, PharmD, decided to conduct studies in mice to see why PN was causing liver disease. They found evidence that the fat used in standard PN solutions, called Intralipid was contributing to liver disease by causing fat to accumulate in the liver.

    They then tested Omegaven, an IV fat mixture made from fish oil. Fish oil contains omega-3 fatty acids, which have been shown to prevent fat accumulation and have anti-inflammatory properties.

    As they hoped, PN using Omegaven as the fat prevented liver injury in the mice.

    Surgeon Rusty Jennings, MD, who directs Children's Advanced Fetal Care Center, had heard of Puder's research and wanted to try Omegaven in one of his patient. Since Omegaven isn't approved for use in the United States, Puder had to receive special permission from the FDA to use Omegaven rather than Intralipid in his PN solution. Within 8 weeks, the baby's liver function improved so much that he was removed from the liver transplant list.

    Puder later treated a second child, a premature baby whose bowel had ruptured; he too had complete resolution of liver disease. Now, more than 100 children at Children’s have received Omegaven.

    Puder and colleagues are now conducting a formal clinical trial, and have received funding from the March of Dimes aimed at preventing liver disease in PN recipients.

    Their work has caused a worldwide shift in treatment.

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