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Liver Disease Jaundice in Babies (hyperbilirubinaemia)

Jaundice is common so why is it a concern for my baby?

There are two types of bilirubin, unconjugated and conjugated, both of which can cause jaundice.

A raised level of unconjugated bilirubin occurs in the relatively common, physiological jaundice seen in many babies in the first two weeks of life especially if they are breast-fed and/or premature.  Find out more by downloading CLDF’s leaflet ‘Jaundice in the newborn baby’.  

If your baby’s health is currently being investigated, blood tests already done have shown your baby has a high level and also a significant percentage of conjugated bilirubin compared to the total bilirubin.  This indicates there is a problem in the liver.

In some cases the problem will clear itself but in other cases specific treatment is needed to prevent further problems.  It is necessary to do a number of special tests to find out which babies need treatment.

How is unconjugated bilirubin produced?

  • Unconjugated bilirubin is sometimes called indirect bilirubin
  • Unconjugated bilirubin is produced when red blood cells breakdown – it is yellow in colour.

How is unconjugated bilirubin changed to conjugated bilirubin?

When the unconjugated bilirubin reaches the liver it is ‘processed’ by having a sugar attached to it, this changes its form and it is now called ‘conjugated’ bilirubin.  It is still yellow in colour.

The liver is often not completely mature at the time of birth, even if the baby is full-term, so it may not be able to process the unconjugated bilirubin as quickly as it is made.  This can lead to a build up of unconjugated bilirubin in the blood which passes around the body and makes the baby appear jaundiced (yellow).  This explains why so many babies are jaundiced in the first two weeks of life but the jaundice usually clears fairly quickly.  This is called physiological jaundice.

An enzyme in breast milk can also slow the rate at which unconjugated bilirubin is converted into the conjugated form which explains why jaundice is more common in breast-fed babies and conjugated jaundice may not be considered.

How is jaundice due to raised levels of conjugated bilirubin different?

Conjugated bilirubin is not broken down by phototherapy (special light treatment which is often used to treat babies who have the common physiological unconjugated jaundice).

The different form of the conjugated bilirubin means it is unable to pass into the brain therefore it CANNOT cause brain irritation or damage which can be caused by very high levels of unconjugated bilirubin.

Conjugated bilirubin is soluble in water therefore when there are high levels in the blood some is passed out with water from the kidneys making the urine (wee) yellow in colour. The urine of a baby less than three months old should be completely colourless – like water.

What happens when there is a hold up of bile flow - called cholestasis?

  • The conjugated bilirubin level in the blood is raised; the total bilirubin should be less than 20mmol/l.
  • The baby is likely to be jaundiced; this is most noticeable as yellow colouring of the whites of the eyes. The yellowing of skin colour is more noticeable in babies who have naturally pale skin.
  • Some conjugated bilirubin is passed out in the urine making it more coloured. A baby’s urine (wee) should be completely colourless like water for the first few months.

  • The stools (poo) may appear more bulky and/or greasy because some of the fat passes through the intestines without being completely digested.
  • Stools may be paler in colour when there is less bile to colour them.
  • Loss of fat in the stool can cause your baby to be very hungry and want more milk than normal.
  • As the baby gets older they may find it difficult to gain the usual amount of weight.
  • The liver may be larger than usual due to inflammation and swelling, the trapped bile irritates the liver.
  • Vitamin levels may be low (especially the fat soluble vitamins A, D, E and K) – due to reduced absorption of these vitamins, babies quickly use up the supply stored in the liver before birth.

Vitamin K is especially important as low levels can reduce the ability of the blood to clot effectively. Signs of affected blood clotting may include bruising (usually very rare in the first few months), prolonged bleeding from the umbilical cord stump (belly button) and nose bleeds.  Blood tests including INR and/or Prothrombin time are used to measure blood clotting.

How will my baby’s jaundice be investigated?

Some babies will be seen at their local hospital, others may be referred to a specialist centre.
Investigations may include the following

  • An examination by the doctor
  • Blood tests
  • Urine tests
  • Stool examination/tests
  • Ultrasound scan
  • Other specialised scans
  • Liver biopsy
  • X-rays
  • Eye test
  • Heart (cardiac) test
  • Bone marrow test

In some liver related conditions, there may be significant signs in other parts of the body so it is common for the examination and tests to cover all parts of the body.

Find out more by downloading CLDF’s ‘routine investigations for liver disease’ leaflet.

How will my baby be fed?
While tests are being carried out you are likely to be advised about the best feeding regime for your baby.  One or more of the following changes may be advised:

Temporarily stopping breast feeding (if given) while some specific metabolic conditions are ruled out.

Giving a special milk formula. If your baby’s weight gain is not as good as it should be your baby will be given a formula containing a special type of fat called medium chain triglycerides, these are easier to digest when bile flow is poor.  You can download CLDF’s leaflet called ‘MCT Feeds, What are they?’ here.

Naso-gastric tube feeding - partial or complete feeding by a fine tube placed through your baby’s nostril and down into the stomach may be required if they are unable to take the amount or type of feed they need by mouth. CLDF has a leaflet on this called “An introduction - Tube feeding”. If a specific cause is found for the jaundice the feeds given may be changed again.

What medicines will my baby be given?

Your baby may be given medicines to help to reduce the effects of the poor bile flow, including:

  • Vitamins – these are less well absorbed from the milk when bile flow is poor. Vitamin K is especially important as the liver needs a good supply in order to make the proteins which control the ability of the blood to clot.
  • Ursodeoxycholic Acid (URSO) – a medicine that can improve bile flow in some cases.

If a specific cause is found for the jaundice additional medications may be needed.

What are the common causes of conjugated jaundice (hyperbilirubinaemia)?

There are many different reasons for this but they broadly fall into the following groups:

  1. Bile duct (drainage) problems – the bile ducts are abnormal in some way. This may affect the bile ducts inside the liver, outside the liver or both. One condition which it is important to consider quickly is biliary atresia. This condition is rare but does require early surgery to obtain the best outcome. In biliary atresia some unknown process affects the bile ducts inside and outside the liver and the bile ducts outside the liver collapse or disappear causing complete obstruction to bile flow.
  2. Metabolic conditions – an abnormality of one of the complex processes that occur within each liver cell. These include:
    1. abnormalities in the way the cells in the liver make the bile or pass it out into the bile ducts
    2. abnormalities in the way in which the liver cells metabolise (change) the nutrients in the milk into a form which the body can use.
  3. Unknown – despite the techniques available to modern medicine there are still many cases of high conjugated bilirubin (up to a third of all babies tested) where no clear explanation is found. This is more common in those babies who have had other medical problems soon after birth.

What is the treatment for conjugated jaundice?

  1. Special feeds and medication as above to assist with the effects of poor bile flow until this has completely resolved.
  2. Specific treatment relating to the cause if available, this may include
    1. Special diet
    2. Medication
    3. Operation

Want to know more?

Download CLDF’s leaflet ‘Conjugated Jaundice in Babies’ here.

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