Maggie Simpson’s Second Audition – Prolonged Jaundice

Author: Meriel Tolhurst-Cleaver / Reviewer: Katy Vose / Codes: A3 / Published: 14/08/2017 / Reviewed: 26/08/2025

Prolonged jaundice is different from early jaundice, which we discussed in our previous blog, as it is jaundice which persists for 14 days in a baby who was born at term (37 weeks or more gestation), or to 21 days in a pre-term baby born before 37 weeks gestation.

Usually, these patients are referred to a paediatric clinic but jaundiced neonates will still rock-up to the Emergency Department (ED), so it is good for us to know how to manage them.

Up to 15% of babies have prolonged jaundice, and many will have no underlying pathology. The most common cause of prolonged jaundice is exclusive breastfeeding (breast milk jaundice), and immature neonatal mechanisms and prematurity can also cause benign physiological jaundice after 14 days.

However, these are both diagnoses of exclusion and in prolonged jaundice we have to do a bit more digging before we can reassure the parents (and ourselves) that all is well. Most importantly, we have to exclude liver disease and conjugated hyperbilirubinaemia. The most common surgical cause of neonatal liver disease is biliary atresia and the management of this is time critical, ideally before 8 weeks of age. As such it is vital not to miss this diagnosis and any neonate with a history of pale stools should be investigated thoroughly.

*diagnosis of biliary atresia is time critical as the treatment needs to be performed within 8 weeks of life for optimal outcome.

To get our brains thinking about how we will approach the baby with prolonged jaundice it is worth thinking about the possible causes. These can be broken down into unconjugated and conjugated causes. To determine which is present you will have to perform a split bilirubin (SBR), this will give you a value for the total bilirubin and a value for the fraction that represents conjugated bilirubin. Anything where the conjugated fraction is >25 or >25% of the total bilirubin should be treated as conjugated jaundice and the appropriate next line investigations be undertaken, we will discuss what these are later on. As you will see from the table above there is a wide range of causes and it is not important to be expert in all of these, but it is helpful as it provides the background to the long list of investigations that are sometimes required in babies with prolonged jaundice.

Where to start

Congratulations on your beautiful little boy/girl/ bundle of joy you must be exhausted, how are you doing?, or similar congratulatory and empathetic phrase is a great way to break the ice with these tired, stressed parents before you launch into the detailed history you need. Then down to the nitty gritty:

• Presenting complaint might sound obvious but is the jaundice the only problem? Are they unwell at all with poor feeding, vomiting, difficulty breathing. Has there been any bleeding or bruising?
• Pregnancy history – any anti-D given? Any maternal drugs? Normal scans?
• Delivery type (thinking about potential for bleeding or bruising), did they have vitamin K at birth, risk factors for sepsis (prematurity, low birth weight, prolonged rupture of membranes, Group B streptococcus, maternal infection at the time of birth)?
• Weight note their birth weight and any recent/current weights (you need to weigh again today if not already done), plot them in the red book and note the centile they are on. Note that most babies have regained their birth weight by day 14
• Feeding – breast, bottle, mixed? Are they feeding well or are they vomiting excessively, uninterested in feeding, sleepy. Has this changed or has feeding been an ongoing issue?
• Jaundice – when was it noticed, has any treatment already been given?
• Stools and urine – careful questioning about stools, specifically asking parents whether stools are pale or chalky and whether the urine is very dark and stains the nappy (stools in newborns who are breastfed can range in colour from yellow, orange, brown through to green but should NOT be white/chalky or bloody. Urine should be nearly colourless). Have there been plenty of wet and dirty nappies? Did they pass meconium in the first 24 hours of life?
• Drugs/PMH are they on any medications or have any known medical conditions. Have the Guthrie card (heelprick blood tests) been done on day 5 and are the family aware of the results?
• Family history ask about jaundice in the family and, specifically about any blood or liver disorders, cystic fibrosis or metabolic disease.

Examination

A set of observations should have been performed at triage, but if not, make sure you get them. If the baby appears obviously unwell, perform an ABCD assessment, get help and resuscitate as appropriate. Otherwise perform a neonatal top-to-toe examination.

A set of observations should have been performed at triage, but if not make sure you get them. If the baby appears obviously unwell perform an ABCD assessment, get help and resuscitate as appropriate. Otherwise perform a neonatal top-to-toe examination.

Look for dysmorphic features, jaundice, pallor and signs of bleeding (petechiae or purpura). Does the baby have normal tone, alertness and handling? Note if the baby is floppy or appears encephalopathic (even a newborn baby should have a flat back if held in ventral suspension, they shouldnt droop over your hand, check out this video if you are unsure what is normal tone in a newborn). Assess their hydration status (capillary refill, anterior fontanelle, mucous membranes). Quickly scan the eyes for a present red reflex to exclude cataracts. Examine the chest and pulses for features of congenital heart disease and examine the abdomen for hepatosplenomegaly.

ALWAYS check the nappy to see if you can see any evidence of stool or urine colour for yourself. Let this part of the examination be a reminder for you to ask about stool and urine colour if you havent already.

After history, observations and thorough examination including weight (and the trend in this by comparison with red book) you will hopefully be able to decide if this baby is well or unwell.

Investigations and Management

What investigations are required and the resultant management will be based on two things in the first instance, one whether the baby is well or unwell and two whether this is simple (unconjugated) prolonged jaundice or the potentially more serious conjugated jaundice.

In a well baby you need only perform a split bilirubin (SBR). Guidelines differ from hospital to hospital and in some centres they include FBC and DAT as first line also. I think this is a practical point, if the total bilirubin is raised the baby will require those investigations and you will need to perform a second heel prick. If you do it all together you have saved the baby a second prick but requested two investigations you could have avoided. See what your guidelines suggest.

Unconjugated hyperbilirubinaemia

Mild SBR <250

• Discharge back to community

Moderate SBR >250

• Perform FBC and DAT if not already done
• Refer to medical team as baby will require repeat bloods in a week
• LFT/SBR (plus FBC if DAT positive)
• Ensure Guthrie results are normal
  • At the review if trend not improving then further investigations may be required (G6PD screen, TFTs, urine culture, consideration of rarer diagnoses e.g. Crigler-Najjar)
  • If haemolysis is ongoing then advise from haematology may be required

Occasionally an FBC will detect a low neutrophil count, this is almost always nothing to worry about and will resolve spontaneously, however please follow local guidelines regarding monitoring. It often involves a repeat FBC 2-4 weeks later.

Conjugated Hyperbilirubinemia

Conjugated fraction >25 or >25% of total bilirubin:

• Refer to medical team as patient requires further assessment and investigations
• For information they will likely perform the following to identify the underlying cause;
• Clotting screen (if PT prolonged give IV Vitamin K)
• LFTs and TFTs
• Biochemistry (U&E, Calcium profile, blood sugar)
• USS abdomen looking for biliary atresia
• Metabolic investigations (urine organic acids, urine & plasma amino acids, GAL-1-PUT, alpha-1-antitrypsin)
• Clean catch urine for culture
• Congenital infection screen (CMV urine, TORCH screen)

If you have an unwell infant, then your priority will be ABCDE. Gain IV access, take blood and urine cultures, commence broad spectrum antibiotics. FBC, Group and DAT, SBR, LFTs, Blood gas which will include a glucose, ammonia and clotting will be essential, of note babies who have acute liver failure will almost certainly have deranged clotting. If PT/INR are prolonged this needs correcting with IV Vitamin K. It is important to prevent the complications of intracranial haemorrhage secondary to vit K malabsorption by correcting this. It is also important to monitor glucose levels in any sick neonate but particularly in one where metabolic disease is a differential and if this is suspected stopping feeds and commencing a 10% Dextrose infusion is important.

Infection/sepsis with bacterial or viral aetiology can be the primary cause of deranged LFTs but it can also be the precipitant to a decompensation due to underlying metabolic disease. This patient will need referral to the medical team and depending on the clinical picture discussion with tertiary specialists.

Whether the family you are looking after is being admitted or discharged, a patient information leaflet is useful to explain what is going on. Most trusts will have one, but the excellent www.yellowalert.org website which has generic leaflets to download on all aspects of neonatal jaundice, including a very useful and comprehensive overview advice leaflet.

References & Further reading

1. National Institute for Health and Care Excellence (NICE). Jaundice in Newborn Babies Under 28 Days. [CG98], May 2010. Last updated:
   31 October 2023.
2. Gupte G. Conjugated Hyperbilirubinaemia. Paediatrics and Child Health.2008:18(10):474476.
3. Gilmour SM. Prolonged neonatal jaundice: When to worry and what to do. Paediatr Child Health. 2004 Dec;9(10):700-704.
4. British Society of Paediatric Gastroenterology, Hepatology and Nutrition. Proposed Prolonged Jaundice Pathway following national audit 2022- 2023. Jan 23, 2024.
5. LRI Childrens Hospital, Prolonged Jaundice Assessment & Investigations, Jan 2025: M Joshi & N Muhammad.
6. NI PEAR Regional Guideline for the Management of Prolonged Jaundice, Sep 2021: Drs Kathy Wilson, Colin Higgins, Maura Scott, Diarmuid McLaughlin, Caoimhe Glancy and Stephen Mullen.