This Guidelines summary is based on guidance included within the Children’s Liver Disease Foundation Yellow Alert Pack, written to provide general guidelines on the early identification of liver disease in infants and their referral where appropriate. For further information, please refer to the full guideline.
Early identification of liver disease in infants
Whilst liver disease in infants is rare, early diagnosis is vital. The main indicators are:
- prolonged jaundice
- abnormal stool/urine colour.
You should be aware of the importance of stool and urine colour.
- a baby’s urine should be colourless
- persistently yellow urine which stains the nappy may indicate liver disease.
- stools should be green or yellow
- persistently pale stools may indicate liver disease
The interpretation of stool colour can be subjective. The following colour chart will help to overcome this problem.
Algorithm 1: Infant stool chart
On the first visit midwives and/or health visitors should:
- check all babies for jaundice by looking at the sclera of their eyes
- record the presence of jaundice when transferring care from midwife to health visitor. State on the record that a jaundice check has been carried out
- if baby is jaundiced, check the stools and urine, no matter how mild. Urine should be colourless and stool should be pigmented yellow or green (see Algorithm 1).
If stools and urine in a jaundiced baby are abnormal and/or a baby with prolonged jaundice (persists two weeks after birth in term babies and three weeks after birth in a pre-term baby) is unwell or not progressing normally, refer immediately to a paediatrician for further investigations.
Action in the event of prolonged jaundice
If a child has prolonged jaundice/abnormal stool or urine colour, carry out a general assessment. This includes:
- feeding history including whether breast or bottle-fed
- document stool and urine colour
- inform parents of the reason for blood tests
Request the following blood tests:
- serum bilirubin blood test—this test measures the total bilirubin in the blood
- split bilirubin test to measure conjugated bilirubin levels—this test measures the ratio of conjugated to unconjugated bilirubin in the blood and is the key test for diagnosing liver disease. It is recommended that all babies with prolonged jaundice be given this test.
A baby where the conjugated bilirubin is greater than 20% (25 micromoles/litre) of the total bilirubin should be referred directly to a paediatrician for investigation.
If the conjugated bilirubin is less than 20% of the total bilirubin and the total bilirubin is less than 200 micromoles/litre, the parent(s)/guardian(s) should be reassured and weekly serum bilirubin levels checked until it is within normal laboratory ranges.
Where the total bilirubin is high (greater than 200 micromoles/litre) and the conjugated fraction is less than 20%, healthcare professionals are advised to contact a paediatrician and refer to NICE neonatal jaundice clinical guideline (CG98) as well as any local guidelines.
The following tests should also be done:
- a full blood count
- a blood group determination (mother and baby) and DAT (Coombs’ test)
- a urine culture
- ensure that routine metabolic screening (including screening for congenital hypothyroidism) has been performed.
Algorithm 2: Early identification
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 greater than 20% (25 micromoles/litre) 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 colour.
Refer to a paediatrician for the following tests:
- repeat split bilirubin test
- liver function tests
- aspartate and alanine transaminases (AST, ALT)
- alkaline phosphatase (ALP)
- gamma-glutamyltransferase (GGT)
- coagulation tests
- prothrombin time (PT)
- partial thrombin time (PTT).
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 normalised ratio (INR) is normal, or by intravenous/intramuscular methods if abnormal.
- blood glucose.
Referral to specialist centres and advice
If any of the investigative tests are abnormal or liver disease is suspected, the infant may need referral to a specialist unit for further investigations or management. This may be at a regional paediatric gastroenterology unit with an interest in paediatric hepatology or a supra-regional paediatric liver unit.
There are three specialist paediatric liver centres in England; all offer medical and surgical management including transplantation. The Department of Health has designated the three specialist paediatric liver centres to carry out Kasai portoenterostomy for biliary atresia in England and Wales.
Birmingham Children’s Hospital
The Liver Unit, Birmingham Children’s Hospital, Steelhouse Lane, Birmingham, B4 6NH
Tel: 0121 333 9999 and ask to liaise with the on-call liver registrar
Secretarial team on 0121 333 8250 (office hours)
Nursing team on Liver Direct 0121 333 8989 or firstname.lastname@example.org
King’s College Hospital, London
Paediatric Liver, Gastroenterology and Nutrition Centre, King’s College Hospital,
London, SE5 9RS
Tel: 020 3299 9000 and ask to liaise with the on-call paediatric liver registrar
Leeds General Infirmary
Children’s Liver and GI Unit, Leeds General Infirmary, Great George Street, Leeds, West
Yorkshire, LS1 3EX
Tel: Admin office on 0113 3925640
Clinical Nurse Specialist Team on 0113 3926151/3926138
Full guideline: Children’s Liver Disease Foundation. Jaundice protocol. Early identification and referral of liver disease in infants. CLDF, 2018. Available at: childliverdisease.org/wp-content/uploads/2019/04/CLDF-Yellow-Alert-Prolonged-Jaundice-Protocol.pdf.
First published October 2007.
Last updated: November 2018.