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Early identification and referral of liver disease in infants

General awareness and training

Jaundice

  • Community healthcare professionals should be aware that there are many causes for jaundice in infants and know how to tell them apart:
    • physiological jaundice
    • breast milk jaundice
    • jaundice caused by liver disease
    • jaundice from other causes, e.g. haemolysis
    • jaundice caused by infection
    • jaundice caused by hypothyroidism

Prolonged jaundice is defined as jaundice persisting beyond two weeks of age in term babies and three weeks in pre-term babies

Urine and stool colour

  • Community healthcare professionals should be aware of the importance of urine and stool colour:
    • normally a baby’s urine is colourless
    • persistently yellow urine which stains the nappy can be a sign of liver disease
    • normally a baby’s stools are green or yellow
    • persistently pale coloured stools may indicate liver disease
  • A jaundiced baby with pale stools and yellow urine can appear completely healthy, however, the baby may have a potentially lethal liver disease

The antenatal period

  • Children’s Liver Disease Foundation experience is that when baby jaundice is explained in the antenatal period, parents are less anxious if their baby becomes jaundiced. In addition, they are knowledgeable about the course of action to be taken in the event of prolonged jaundice

First visit of midwife and/or health visitor

  • Every baby should be checked for jaundice by looking at the sclera of the eyes
  • The presence of jaundice in an infant should always be recorded when transferring a baby from the midwife to the health visitor
  • On transferring a baby from the midwife to the health visitor the record should state that a jaundice check has been carried out
  • If the baby is jaundiced, however mild, stools and urine should be checked and seen by either the health visitor and/or midwife
  • A baby’s urine should be colourless. If yellow, this should be investigated, see below
  • Stools should be pigmented yellow or green. If pale or clay-coloured this should be investigated, see below
  • If the stools and urine in a jaundiced baby are abnormal in colour, the baby should be referred to a paediatrician immediately

Prolonged jaundice

  • Definition of prolonged jaundice
    • jaundice which persists in the sclera of the eyes two weeks after birth in term babies and three weeks after birth in a pre-term baby, whether or not the baby has pale stools
  • Action in the event of prolonged jaundice:
    • if the baby is unwell and/or not progressing normally then the infant should be referred to a paediatrician for investigation
  • Carry out general assessment:
    • feeding history including whether breast or bottle-fed
    • weight
    • document stool and urine colour
    • inform parents of reason for blood tests
  • Request the following blood tests:
    • serum bilirubin blood test. This test measures the total bilirubin in the blood
    • split bilirubin blood test. This test measures the conjugated (direct) bilirubin level and the unconjugated (indirect) bilirubin levels
      • it is recommended that all babies with prolonged jaundice be given a split bilirubin test
      • in breast-fed babies it is vital that a split bilirubin test is carried out so that the conjugated and unconjugated levels are known. This is because breast milk jaundice is unconjugated hyperbilirubinaemia
  • Causes of unconjugated hyperbilirubinaemia are:
    • prolonged physiological jaundice
    • breast milk jaundice
    • Crigler Najjar Syndrome
    • haemolysis (red cell breakdown)
  • If the conjugated bilirubin is >20% of the total bilirubin, the baby should be referred for immediate investigation by a paediatrician
  • If the conjugated bilirubin is <20% of the total and the total bilirubin is less than 200micromoles/l, the parent(s)/guardian(s) should be reassured and weekly serum bilirubin levels checked until it returns to normal
  • Where the total bilirubin is very high (>200 micromoles/l) and the conjugated fraction is <20%, healthcare professionals are advised to contact a paediatrician. There may be regional/local variations for the upper limit of the total bilirubin after which advice should be sought

Early identification algorithm

Early identification algorithm

Investigation

  • The following should be referred to a paediatrician:
    • a baby who is unwell and/or not progressing normally
    • a baby with abnormal colour of stools and/or urine at any age
    • a baby where the conjugated bilirubin is > 20% of the total bilirubin
    • any infant with prolonged jaundice that has not been investigated
  • Breast-fed babies may also have liver disease; be extra careful to check stools and urine
  • The tests should include:
    • repeat split bilirubin test
    • liver function tests. These include the following:
      • albumin
      • aspartate and alanine transaminases (AST, ALT)
      • alkaline phosphatase (ALP)
      • gamma glutamyltransferase (GGT)
    • blood glucose
    • coagulation tests
      • prothrombin time (PT)
      • partial thrombin time (PPT)
  • Coagulation may be prolonged secondary to vitamin K deficiency, particularly in breast-fed babies not given vitamin K at birth. All babies with suspected liver disease must be given vitamin K orally if the international normalized ratio is normal or intravenous/intra-muscular if abnormal
  • If any of the investigative tests are abnormal or liver disease is suspected, the infant may need referral to a specialist unit for further diagnosis or management. This may be a regional gastroenterology unit or a supra-regional liver unit, as appropriate
  • The British Society of Paediatric Gastroenterology, Hepatology and Nutrition (BSPGHAN) has further advice for paediatricians on tests (www.bspghan.org.uk)
  • There are 3 supra-regional units for the treatment of children with liver disease. GPs or paediatricians can make referrals directly to the units and are welcome to seek advice:
    • Paediatric Liver Service
      King’s College Hospital
      Denmark Hill
      London
      SE5 9RS
      Tel: 020 3299 3214
      Fax: 020 3299 4228
    • The Liver Unit
      Birmingham Children’s Hospital
      NHS Trust
      Steelhouse Lane
      Birmingham
      B4 6NH
      Tel: 0121 333 8256
      Fax: 0121 333 8251
    • Children’s Liver and GI Unit
      Leeds General Infirmary
      Great George Street
      Leeds
      West Yorkshire
      LS1 3EX
      Tel: 0113 392 7112
      For up to date contact information go to http://www.leedsth.nhs.uk
  • Please note the Department of Health has designated these three centres in England to perform Kasai-portoenterostomy for biliary atresia

full guidelines available from…
Children’s Liver Disease Foundation, 36 Great Charles Street, Birmingham B3 3JY (Tel – 0121 212 3839)
www.childliverdisease.org

Jaundice protocol. Early identification and referral of liver disease in infants. October 2007
First included: February 2008.