Contents
Introduction
Neonatal Jaundice is very common, with 60% of babies becoming jaundiced within the first week of life. It is however, often very worrying for parents, and for clinicians, the challenge is distinguishing ‘harmless’ physiological/breastfeeding jaundice from jaundice resulting from a more worrying cause.
- It occurs in up to 80% of pre-term babies, and 60% of full-term babies
- Visual assessment is a poor clinical indicator of the bilirubin level
- Kernicterus is a serious but rare complication of unconjugated hyperbilirubinaemia that cause cause serious long-term neurological defects
Neonatal Jaundice can be split into three time periods
- Jaundice within the first 24 hours of life.
- Jaundice between days 2 and 14.
- Prolonged Jaundice occurring after 14 days.
Jaundice with onset BEFORE 48 hours, or AFTER 3 days is more likely to be pathological
As well as dividing cases by the time and duration of onset, they can be divided into unconjugated and conjugated.
- Unconjugated cases are more common. In the case of physiological and breast milk jaundices, often no treatment is required. In severe cases, or other causes, they can often be treated by phototherapy, or if required in serious cases, exchange transfusion
- Conjugated causes often have a more serious underlying cause, and the management depends on treating the underlying cause
Jaundice in the first day of life
- This is not normal!
- The commonest cause is due to haemolysis usually due to either ABO incompatibility, Rhesus disease of the newborn or Hereditary Spherocytosis.
- However can be caused by infection either from the mothers genital tract or in the amniotic fluid, therefore a TORCH screen should be used.
- Toxoplasmosis
- Other
- Rubella
- Cytomegalovirus (CMV)
- Hepatitis or Herpes
Jaundice in days 2- 14
- This is a tricky period, as this is the timeframe physiological or breastfeeding jaundice often manifests, however it is also the time more serious conditions also present with jaundice. Although other causes must be excluded, the most common causes in this time period are ;
- Physiological Jaundice is very common, usually appearing around day 2-4, peaking at 7 days and then regressing. It is caused by a combination of the following two factors:
- Breastfeeding Jaundice is thought to result from increased enterohepatic bilirubin. It has long been known that breastfed babies are often more jaundiced, and for longer than bottle fed babies. It is important to reassure the mother that this is a common occurrence; she should continue breastfeeding even though the jaundice may still be there.
Jaundice after 14 days
- This is known as prolonged jaundice.
- Breastfeeding jaundice can continue into the fourth or fifth weeks, however there are several more severe conditions that could cause the jaundice
- Congenital hypothyroidism can cause jaundice in this time period, however you would expect this condition to be picked up on the Guthrie heel prick test.
- Biliary atresia is a rare condition in which all or parts of the biliary tree fail to develop, preventing bile from flowing into the gut. This leads to a high concentration of conjugated bilirubin level in the blood. An important question to ask when taking a jaundice history is the colour of the stools. In biliary atresia the stools are often pale or ‘chalky white’. Ultrasound or radionuclide scans can identify the defect and surgery is needed to create a functional biliary tree.
Signs and symptoms
Jaundice gives a yellow/orange to the skin and sclera. In adults it often causes itching; however in neonates this is impossible to tell! There may be signs of the underlying cause, for example pale stools in biliary atresia, or in rhesus disease there is hepatosplenomegaly.
Red flags to look out for are signs of Kernicterus. This is a life threatening condition which happens when the bilirubin levels are very, very high (>360μmol/L). When bilirubin levels reach this high, they cross the blood brain barrier, and form deposits in the basal ganglia and the brainstem. This can cause neurological signs, such as lethargy, poor feeding, fits, coma and even result in death. Thankfully in the vast majority of cases neonatal jaundice is well monitored and treated, which means very few cases progress to Kernicterus.
Investigations of the Jaundiced Neonate
- As with everything, a thorough history and examination
- Bloods
- Serum Bilirubin
- Conjugated vs unconjugated
- FBC
- Blood Film
- Blood Group
- LFTs
- TFTs
- Blood cultures – TORCH Screen
- Coomb’s test
- Serum Bilirubin
- Urine dipstick and culture, to look for bilirubin, and a source of sepsis.
- If prolonged or the clinical picture is suggestive, then biliary US or other radiology.
Interpretation of bilirubin
Normal
- Total serum bilirubin (SBR) levels depend on gestation age. Quoted ranges online vary. An example for healthy term babies:
- Day 1 – SBR <200
- Day 2 – SBR <260
- Day 3 – SBR <320
- Day 4 – SBR <350
- Day 5 – SBR <360
If the level is raised, determine if conjugated or unconjugated is the cause
Unconjugated hyperbilirubinaemia
- Conjugated bilirubin level <15%
- Causes:
- Physiological jaundice
- Breast milk jaundice
- Sepsis
- Haemolysis
- Pyloric stenosis
- Prematurity
- Hypothyroidism
- Management:
- Treat sepsis
- If suspected haemolysis – discuss with haematologist
- Phototherapy if indicated by bilirubin levels – see below
Conjugated hyperbilirubinaemia
- Conjugated bilirubin level >15%
- Causes:
- Biliary atresia
- Neonatal hepatitis
- Metabolic disturbance
- Management:
- Is complex, and depends on the cause. Involve paediatric gastroenterologist early to discuss the management options
Management
Management of unconjugated huperbilirubinaemia is outlined below. The management of conjugated bilirubinaemia is more complex and dependent on the underlying cause.
- Use a bilirubin chart! Measures bilirubin against age. There are normally two lines on it
- LINE ONE = PHOTOTHERAPY TREATMENT LINE
- LINE TWO = EXCHANGE TRANSFUSION LINE
- If under the phototherapy line, and other investigations have come back as negative, then treatment is supportive. Make sure the baby is well hydrated and nourished, and repeat bilirubin levels.
- If above the phototherapy line then start phototherapy. This is when the baby is subjected to blue light of a specific wavelength (450nm). At this wavelength light converts non-conjugated bilirubin into a harmless substance and decreases bilirubin levels. The baby needs to be completely naked, apart from eye protection, and needs to be under the lights as much as possible, until bilirubin levels come down. Some mothers find this very frustrating as it reduces bonding time with the baby; however feeding must continue, which gives them some time together. Some babies also develop a rash as a result of the phototherapy. More recently special fibre optic blankets have been developed which can be used.
- Exchange transfusions are when the baby’s blood is replaced with carefully matched and screened donated blood. Two times the baby’s blood volume is exchanged, either through a UVC (umbilical venous catheter) or a peripheral vein paired with an arterial line.
Useful Resources
The NICE guidelines on neonatal jaundice, although very long, are superb for learning about neonatal Jaundice. There is also a very useful box (p105 I think) that gives a succinct guide to the treatment, well worth a look, but pick out the bits you need!
Alternatively NICE guideline 98 has a brief version which summarises the relevant information:
Flashcard
References
- Jandice in early infancy – RCH
- Murtagh’s General Practice. 6th Ed. (2015) John Murtagh, Jill Rosenblatt
- Oxford Handbook of General Practice. 3rd Ed. (2010) Simon, C., Everitt, H., van Drop, F.
- Beers, MH., Porter RS., Jones, TV., Kaplan JL., Berkwits, M. The Merck Manual of Diagnosis and Therapy