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Liver Disease in Childhood

Author: Dr. Simon Horslen, Seattle Children's Hospital

Dr. Simon Horslen, MB ChB, is a member of the Pacific Northwest Division Medical Advisory Committee. He is Director of the Hepatobiliary and Intestinal Failure programs and Medical Director for Liver and Intestine Transplantation at Seattle Children’s Hospital as well as Professor of Pediatrics at the University of Washington School of Medicine. Dr. Horslen earned his medical degree from the University of Bristol, England. He has written this month's article about Liver Disease in Childhood.

Liver Disease in Childhood

1. How common is liver disease in children? Liver disease in children is thankfully very uncommon. Biliary atresia which is the most common cause of an infant with liver disease occurs in only one in 15 -20,000 infants. In older children and adolescents the most common form of liver disease is now fatty liver disease related to the national epidemic of obesity.

2. Who looks after children with liver disease? Most cases of acute hepatitis are cared for by primary pediatricians and family medicine doctors but in more complicated disease the child is referred onto the local Pediatric Gastroenterologist. In larger centers there are also Pediatric Hepatologists, pediatric doctors who specialize in children with liver diseases. Your PMD will know the most appropriate resources and facilities within your community.

3. Do children get the same liver diseases as adults? The spectrum of liver diseases is generally very different in children compared to adults. Most adults with liver disease have diseases that have developed in adulthood after having had a healthy childhood; these may be due to infection, environmental exposures or changes in their immune system. Although some children, and particularly teenagers, can get similar conditions as they get older a great deal of the liver diseases seen in childhood are conditions that are present at or shortly after birth. Such congenital conditions are often, although not always, genetic in origin.

4. What types of liver disease do children suffer from? Infants may be born with genetic defects of the chemical functioning of the liver cell, called metabolic defects, which interfere with essential functions of the liver and can lead to damage to the infants liver and sometimes injury to other body organs such as the heart, kidneys and brain. Another group of diseases affect the bile ducts such as biliary atresia, choledochal cyst and Alagille’s syndrome. Infants can also be seen with congenital tumors in their liver, ranging from a small benign hemangioma (an abnormal mass of blood vessels) to highly malignant hepatoblastoma. Children of all ages can have infectious causes of hepatitis although these are much less frequent in the US than in less developed parts of the world. In older children and teenagers diseases such as autoimmune hepatitis, primary sclerosing cholangitis and Wilson disease become apparent and as mentioned previously liver disease due to excess fat deposition in the liver is becoming ever more common, just like in adults. Chronic viral hepatitis, particularly hepatitis C, is not as great a problem in children as in adults. Prior to the recognition of the cause of hepatitis C many children did become infected from blood products but since universal screening of blood products was introduced 20 years ago new cases do not occur from these source.

5. What are the signs and symptoms that suggest liver disease in a child? The most common sign of liver disease is jaundice, usually first revealed in yellowing of the whites of the eyes. Jaundice occurs because the liver usually excretes the yellow pigment called bilirubin into the gut from where it is removed from the body in the stool, but when the liver is inflamed or damaged this excretion fails and the bilirubin level builds up in the blood and is deposited in the body tissues including the skin.

The liver is also responsible for making proteins that help the blood clot and so liver disease can be manifest in children by excessive bruising of bleeding.
Another way liver disease might be detected in infants and children is by a doctor feeling a mass in the abdomen which may represent either an enlarged liver or spleen (the spleen often enlarges as a result of liver disease).
Commonly the first indication is the presence of abnormalities on blood tests done for other illness or routine childhood screening. Of course once the possibility of liver disease arises blood tests are used to confirm these suspicions.

6. Are treatments available for liver disease in children? Yes. Many milder forms of hepatitis resolve on there own without specific treatment over a matter of a few weeks. There are an increasing range of options for the treatment of chronic viral hepatitis and although the newer treatments are first used in adults, those found safe and beneficial are being increasingly made available for the treatment of affected children. Immune modulation is effective in most cases of autoimmune liver disease in childhood and there are ever expanding treatments for liver-based metabolic diseases. Nutritional support in general and notably fat-soluble vitamin supplementation is essential in many chronic liver diseases. Surgical treatments for tumors, biliary atresia and choledochal cysts have seen huge improvements in outcomes over time. In cases of liver failure, whether from severe acute hepatitis or as the end result of chronic liver damage, liver transplantation is also available and nowadays highly successful. Between 500 & 600 liver transplants are carried out each year in children some even tiny infants only a few days old.

Although liver disease is very rare in childhood it can be very serious. Advances in medicine have reach the position where treatment effective treatment is available for the vast majority of conditions and any child with liver disease beyond acute self-limiting hepatitis should be referred promptly to an appropriate specialist.

For more information about Dr. Simon Horslen or Seattle Children's Hospital please click here.

Page updated: October 3rd, 2012

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