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Prolonged jaundice in infants: health visitors have a vital role in recognising prolonged jaundice.

At least two children are diagnosed with a liver disease every day in the UK, more than with childhood leukaemia. (1) Jaundice occurs in up to 90% of babies in the first few days of life. However, prolonged jaundice is a significant indicator of paediatric liver disease, and can occur in up to 15% of babies. (2,3) The role of health visitors and midwives is vital for early referral and management of these infants. This update uses one case to raise questions about recognising and responding to prolonged jaundice (see Box 1).

What is prolonged jaundice?

Prolonged jaundice is defined as jaundice persisting beyond two weeks of age in a term baby and three weeks in a pre-term baby. Although it is more common in breastfed infants, the definition does not change with feeding type. All infants with prolonged jaundice, whether bottle- or breastfed, must be investigated for liver disease. In the case discussed here, Kyle's mother should not have been reassured that he simply had 'breastmilk jaundice'.
Box 1: A case of prolonged jaundice (based on a real case)

Kyle is a clinically well three-week old infant and his health
visitor suspects that he is jaundiced. He is breastfeeding well and
so his mother is reassured that it is likely to be due to
'breastmilk jaundice'. A week later, the community midwife sees
Kyle and agrees with the health visitor. At the six-week check at
his local GP practice, the GP reassures Kyle's mother that jaundice
is quite common in babies. A week later, Kyle's grandmother is
concerned that he is still jaundiced and requests to be seen by a
hospital paediatrician.

A blood test is performed and Kyle is found to have a high
conjugated bilirubin level. Later that week he is diagnosed as
having biliary atresia, and he has major corrective surgery the
following day. Unfortunately, the surgery is not successful, and
Kyle has a liver transplant 12 months later. A number of questions
may be raised by this situation:

* What is prolonged jaundice?

* Why is prolonged jaundice so important?

* What should the health visitor have done initially in this case?

* What if the health visitor is reassured by a midwife or GP?

* Surely not all infants with prolonged jaundice have liver
disease?

* Where else can help be sought?


Why is prolonged jaundice so important?

Prolonged jaundice can be a sign of a significant paediatric liver disease and should never be ignored. There are two kinds of jaundice--unconjugated and conjugated hyperbilirubinaemia (4) (see Table 1), and in this case Kyle would need a blood test to ascertain which is present. This blood test is often called a 'split bilirubin' level, though it is worth specifically asking for a total and conjugated bilirubin level.

Jaundice is caused by the yellow-brown pigment bilirubin. Unconjugated bilirubin is produced by the breakdown of haem, which is found in red blood cells. The liver then acts on unconjugated bilirubin to make it soluble (conjugated) in water. This conjugated bilirubin is then excreted by the liver via the biliary system (gallbladder and ducts) into the gut. It is the presence of bilirubin metabolites in the stools which give them their characteristic colour.

Kernicterus

In the newborn period, immaturity of the liver and the increased breakdown of red blood cells mean that most healthy babies have a raised unconjugated bilirubin level. This is termed physiological jaundice and has cleared by two weeks of age in over 90% of babies. Very high levels of unconjugated bilirubin--more than 350 micromoles per litre (micromol/l)--can occur early in a baby's life in conditions such as rhesus incompatibility. This can lead to brain damage in the baby, called kernicterus. Kernicterus is irreversible damage caused by bilirubin being deposited in the brain. Referral to a paediatrician is therefore recommended for any infant with unconjugated bilirubinaemia when the total bilirubin is greater than 200micromol/l.

Biliary atresia

A high blood-level of conjugated bilirubin (more than 20% of total bilirubin) is indicative of liver disease. The most common cause of paediatric liver disease is biliary atresia, which affects one in 16 700 infants. (5) In this, the bile ducts outside the liver become obliterated by scar tissue, and bile (containing conjugated bilirubin) cannot be excreted into the gut. The treatment of biliary atresia involves major surgery (Kasai portoenterostomy) to relieve this obstruction. Without surgery, these infants will die by one to two years of life. Sadly, surgery is not always successful and many of these infants go on to require a liver transplant. If the Kasai portoenterostomy is performed before 60 days of age, there is a 60% chance of success. However, the success rate falls to below 30% if surgery is delayed beyond three months of age. Thus, early referral of babies with prolonged jaundice for investigation is vitally important to improve the outcome of babies with biliary atresia.

Other complications and causes

Early referral of children with liver disease also allows identification of complications such as malabsorption of vitamin K. Vitamin K is vital for forming blood clots, and its malabsorption can result in intracranial bleeds, causing permanent brain damage. This complication is easily avoided by giving parenteral vitamin K.

As well as biliary atresia, there are many other causes of conjugated hyperbilirubinaemia. These include congenital or acquired infections, metabolic conditions (such as galactosaemia), endocrine disease (such as hypothyroidism), toxins, birth asphyxia, and biliary hypoplasia (such as Alagille syndrome).

[FIGURE 1 OMITTED]

What should the health visitor have done initially in this case?

The health visitor in this case correctly identified that Kyle was jaundiced at over two weeks of age. The algorithm from the Children's Liver Disease Foundation (CLDF) (see Figure 1) gives an easy protocol to follow when worried about a baby with prolonged jaundice. At this point, the first step would have been to carry out a general assessment. This involves ensuring that the baby is clinically well, how the baby is feeding, and documenting the baby's weight along with the colour of the stool and urine. Persistently pale-coloured stools may indicate liver disease.

As Kyle was clinically well, then a split bilirubin test in the community would have been appropriate. A split bilirubin test is a simple blood test that can usually be taken from the infant's heel. If the health visitor is unable to do this, then a community midwife or GP should be contacted to perform the test. Kyle had a conjugated jaundice--conjugated bilirubin greater than 20% of the total fraction. For this reason, he should have been referred to a paediatrician in order to investigate for the cause of the liver disease.

[ILLUSTRATION OMITTED]

A baby's urine should be colourless--urine that is anything other than colourless could be a sign of liver disease. Infants with pale stools or urine that is anything other than colourless should therefore also have a split bilirubin test.

What if the health visitor is reassured by a midwife or GP?

Late referral of an infant with liver disease can be catastrophic for that baby. If any healthcare professional is reassured that jaundice after two weeks of life in a term baby is normal (without a split bilirubin test), then they should seek a second opinion, such as from a paediatrician at a local paediatric department.

Surely not all infants with prolonged jaundice have liver disease?

Most infants who are jaundiced at over two weeks of age will not have liver disease. If the conjugated fraction of bilirubin is less than 20% of total bilirubin and the total bilirubin is less than 200 micromol/l, then these infants can be followed up in the community (see Figure 1).

Where else can help be sought?

The CLDF is a charitable organisation that provides emotional support to children and families affected by liver disease, and is also involved in research and education. It provides invaluable online 'Yellow alert' resources, including the protocol, a leaflet for parents and a stool colour chart. (6)

References

(1) Children's Liver Disease Foundation. Children's Liver Disease Foundation. Birmingham: Children's Liver Disease Foundation, 2008. Available at: www.childliverdisease.org (accessed 26 March 2009).

(2) Winfield CR, MacFaul R. Clinical study of jaundice in breast and bottle fed babies. Archives of Disease in Childhood, 1978; 53(6): 506-7.

(3) Kelly DA, Stanton A. Jaundice in babies: implications for community screening for biliary atresia. British Medical Journal, 1995; 310(6988): 1172-3.

(4) McClean P. Recognising liver disease in jaundiced infants. British Journal of Midwifery, 2008: 12(2): 106-9.

(5) McKiernan PJ, Baker AJ, Kelly DA. The frequency and outcome of biliary atresia in the UK and Ireland. The Lancet, 2000; 355(9197): 25-9.

(6) Children's Liver Disease Foundation. Yellow alert. Birmingham: Children's Liver Disease Foundation, 2008. Available at: www.childliverdisease.org/ education/yellowalert (accessed 1 April 2009).

Dr Peter Cartledge

Specialist trainee in paediatrics,

Hull Royal Infirmary

Dr Patricia McClean

Consultant paediatric hepatologist,

St James's University Hospital, Leeds
Table 1. Differentiation between hyperbilirubinaemias (4)

 Unconjugated

Split bilirubin test Conjugated bilirubin less than
 20% of total bilirubin

Urine colour Colourless

Stool colour Yellow, brown, green

Cause Can be physiological or
 pathological

Implications High levels can cause brain
 damage (kernicterus)--refer to
 paediatrician if total bilirubin is
 greater than 200micromol/l

 Conjugated

Split bilirubin test Conjugated bilirubin greater than
 20% of total bilirubin

Urine colour Yellow

Stool colour Pale lemon, white, grey

Cause Always pathological and indicates
 liver disease

Implications Requires prompt referral to a
 paediatrician
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Title Annotation:CLINICAL UPDATE
Author:Cartledge, Peter; McClean, Patricia
Publication:Community Practitioner
Geographic Code:4EUUK
Date:May 1, 2009
Words:1568
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