Fertility and pregnancy * Babies and children

Liver disease in children

Information about the liver and liver disease in children The liver
The liver is the largest internal organ in the body, lying in the upper right abdomen.

It has several functions:

• Regulation of chemicals that circulate in the bloodstream. These may be naturally produced by the body or ingested, such as medications
• The manufacture of several proteins blood proteins, such as albumin which affect the balance of water between blood and tissues, proteins for the immune system to help fight off infections and cholesterol and other special proteins needed to transport fats around the body
• The storage of glucose when it is not needed, and then releasing it back into the bloodstream when required
• The regulation of amino acids, used as the building 'bricks' of proteins, which when in excess, are broken down and excreted as urea in the urine
• The secretion of a green liquid bile, into the intestine (duodenum). Bile removes waste products from the liver, and also helps in the breakdown and absorption of fat

The liver has amazing regenerative powers, as nine tenths of it can be removed or destroyed, and the remaining one tenth can grow back to it's normal size.

Liver disease

• At least one baby a day is born with liver disease every day in the UK.
• Liver disease in babies is indiscriminate – there is no specific group at risk
• There are more than 100 liver diseases that can affect babies. For most, the cause is unknown
• Most liver diseases are life-threatening and mean a lifetime of care.
• Biliary atresia is a very serious liver disease which is unique to childhood and affects approximately 50 babies in England every year. If left untreated it is fatal with a mean age of death of two years. It is vital that biliary atresia is identified and surgical treatment given at an experienced centre before the baby is eight weeks of age

Spotting the signs of liver disease in a newborn

• Prolonged jaundice: Jaundice is the name given to the baby's yellow appearance of the skin and the whites of the eyes and is very common in newborn babies. It reaches its peak at about four days and then gradually disappears in most babies by the time they are two weeks old. Prolonged jaundice can be a sign of liver disease and is defined as jaundice persisting beyond two weeks of age in a term baby and three weeks in a pre-term baby
• Persistent yellow urine staining the nappy can be a sign of liver disease – urine in a healthy baby should be colourless
• Persistent pale coloured stools may indicate liver disease – healthy coloured stools can be described as English mustard yellow in bottle fed babies and daffodil yellow in breast fed babies

Further action in babies showing signs of liver disease

• A significant number of babies with prolonged jaundice will have breast-milk jaundice. This is harmless and disappears without the need for treatment. Breast-feeding can be continued safely
• Parents are frequently reassured that prolonged jaundice is breast-milk jaundice. In some cases the cause will be liver disease which is potentially life threatening. The only way to distinguish between breast-milk jaundice and liver disease jaundice is by doing a special blood test – a split bilirubin blood test
• Newly born babies with persistently pale stools and / or yellow urine should be referred to a paediatrician immediately for investigation
• All suspected babies should be referred promptly to a doctor

Testing

• A simple split bilirubin blood test should be conducted to rule out liver disease in a baby with prolonged jaundice and/or showing other signs. This can be carried out anywhere in the UK
• If the conjugated bilirubin level is greater that 20% of the total bilirubin (identified by the split bilirubin test) the baby should be referred immediately for investigation by a specialist

Treatment

• Further investigations will be needed to identify the liver disease
• Treatments vary according to the liver disease. Some are managed by diet, some medical intervention and surgery is required in others
• Approximately 50 babies will be diagnosed with biliary atresia every year in England. These babies will need an operation called Kasai portoenterostomy (named after the Japanese surgeon, Professor Morio Kasai who developed the procedure in 1959). Kasai portoenterostomy needs to be carried out before the liver becomes damaged and, ideally, needs to be performed before the baby is eight weeks old
• If surgery fails in babies with biliary atresia, the baby’s only other option is a liver transplant and can mean a long wait for a donor organ

Further Information
For further information on liver disease visit the Children’s Liver Disease Foundation, on www.childliverdisease.org or by calling 0121 212 3839.

For information specifically on identify liver disease in new born babies see the information at www.childliverdisease.org/education/yellowalert

This article was published on Mon 31 July 2006

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