A 2-week old baby girl is presented to the emergency department (ED) by her parents on the advice of her Health Visitor (HV) who has noted that she still appears to be jaundiced. Her parents tell you that she has been breastfeeding less over the past 24-hours, but continues to have wet nappies as normal and last had her bowels open only few hours ago with a soft, yellow stool. Parents report she was born at 39 weeks gestation by normal vaginal delivery and began looking jaundiced at about 4 days old.
Following a full history and careful clinical examination, the suspected diagnosis of neonatal jaundice is made.
Neonatal jaundice (hyperbilirubinaemia) is a common and usually harmless condition in newborns that causes yellowing of the skin and the whites of the eyes (Wang, 2015). Approximately 60% of term and 80% of preterm babies develop jaundice in the first week of life and about 10% of breastfed babies are still jaundiced at 1 month. For most of these babies this early ‘physiological jaundice’ is harmless and not an indication of potential underlying disease. Prolonged jaundice (>14 days) is more common in breastfed babies, it is usually harmless but can be an indication of serious liver disease (NICE 2010).
Other symptoms can include:
- yellowing of the palms of the hands or soles of the feet
- dark, yellow urine – should be colourless
- pale-coloured stool – should be yellow or orange
Bilirubin, a yellow substance, is a breakdown product of heme segment of the haemaglobin molecule in red blood cells (RBC). This process starts in the spleen where the unconjugated bilirubin binds with albumin and is transported to the liver and conjugated with glucuronic acid. This is excreted into the bile ducts and then on into the intestines. When this process fails bilirubin builds up in the blood resulting in jaundice. Understanding the split of conjugated versus unconjugated bilirubin gives an idea where the presenting problem may lie.
Predominately unconjugated bilirubinaemia – is where it has not yet passed through the liver successfully which can be due to a number of reasons:
- RBC being broken down faster than the liver can process, as in blood group incompatibility, G6PD deficiency or infection.
- a problem with the conjugating capacity of the liver e.g. enzyme disorders.
- or (as with breast-milk-jaundice) where pregnanediol inhibits glucuronyl transferase activity.
Predominately conjugated bilirubinaemia – is where the liver has done its job but there is a problem with excretion into the bowel e.g. obstruction, hepatitis or metabolic disorders (Kitterman, 2004).
In newborns, because the liver is immature, it can sometimes initially be less effective at removing the bilirubin from the blood (NHS, 2017), with symptoms generally developing two to three days after birth. By the time a neonate is about two weeks old, their liver is more effective at processing bilirubin, so jaundice often corrects itself. In a small number of cases, prolonged jaundice can be the sign of an underlying health condition (NHS, 2017) and should be investigated in symptomatic newborns (NICE, 2010).
Differential Diagnosis for neonatal jaundice;
– immaturity of the liver
– internal bleeding (haemorrhage)
– infection (including sepsis)
– incompatibility between the mother’s blood and the baby’s blood
– liver duct malformation (perinatal biliary atresia)
– metabolic disorders
– breast milk jaundice
Kernicterus – a rare but serious complication of untreated neonatal jaundice caused by excess bilirubin damaging the brain or central nervous system resulting in bilirubin encephalopathy. Kernicterus is now extremely rare in the UK, affecting less than 1 in every 100,000 babies (NHS, 2017).
Investigation Pathway for Neonatal Jaundice (NICE, 2010, p.30)
– Bilirubin levels (total and split conjugated vs unconjugated) – this can be done by heelprick bloods
– FBC, LFT’s, clotting, TSH, blood group and Coomb’s test
– Urine culture
– Abdominal CT scan
– Liver biopsy
– Nuclear liver scan
- Phototherapy: whether continuous or intermittent (Khaliq, 2017). Sunlight is no longer recommended as a suitable form of ‘phototherapy’ for jaundiced infants (NICE 2010).
Figure 1. Bilirubin thresholds for phototherapy and exchange transfusion in babies with hyper-bilirubinaemia (NICE 2010)
- Exchange blood transfusion: in cases of extreme hyperbilirubinaemia and Kernicterus.
- Kasai procedure: surgical procedure used in infants with bilary atresia. It surgically bypasses the hepatic ducts within the liver enabling flow of bile into the intestine, resolving the previous backflow and build-up of bilirubin that can lead to irreparable liver damage (cirrhosis)(Wang, 2015).
- Liver transplant
Although most neonates will have an uneventful resolution of their jaundice, with a few requiring only phototherapy, consideration should be given to those attending the ED with the following red flags (Lillegard et al, 2017):
- jaundice >2-weeks
- pale coloured stools
- dark, yellow urine
- increased lethargy
- reduced feeding and/or poor weight gain
- signs of sepsis
& Ruth Carruthers (Paediatric ACP EM)
Biliapp newborn jaundice tool – based on the NICE 2010 guidelines. Enter babies gestation, date and time of birth, current bilirubin levels and it tells you what is the most appropriate treatment option and when to next test.
Driscoll, J. (2007) Practicing clinical supervision: a reflective approach for healthcare professionals (2nd edition). Elsevier. Edinburgh.
Kitterman, J.A. (Ed) (2004) Neonatal Jaundice [on-line] https://www.ucsfbenioffchildrens.org/pdf/manuals/41_jaundice.pdf (accessed 16/03/17).
Lillegard, J.B., Miller, A.C. and Flake, A.W. (2017) Biliary Atresia. Fundamentals of Pediatric Surgery 629-636.
National Health Service (NHS) Choices (2017) Newborn Jaundice [on-line] @http://www.nhs.uk/conditions/jaundice-newborn/pages/introduction.aspx (accessed 11/02/17).
National Institute for Clinical Excellence (NICE)(2010) Jaundice in newborns under 28-days – CG98 [on-line] https://www.nice.org.uk/guidance/cg98 (accessed 20/02/17).
Wang, K.S. (2015) Newborn Screening for Biliary Atresia. Pediatrics. Volume 136, number 6.
Khaliq, A. (2017) Comparison of continuous with intermittent phototherapy in the treatment of neonatal jaundice. [on-line] Journal of Postgraduate Medical Institute http://jpmi.org.pk/index.php/jpmi/article/view/1884 (accessed 21/02/17)