Neonatal Jaundice
Summary
Neonatal jaundice is yellowing of the skin and sclera caused by elevated bilirubin levels. It is extremely common, affecting 60% of term and 80% of preterm infants. Most cases are physiological and benign. However, pathological jaundice (early onset, rapidly rising, or prolonged) requires urgent investigation. Severe unconjugated hyperbilirubinaemia can cause kernicterus — irreversible bilirubin encephalopathy with permanent neurological damage.
Key Facts
- Incidence: 60% term infants, 80% preterm infants visible jaundice
- Physiological: Appears after 24 hours, peaks day 3-5, resolves by 2 weeks (term)
- Pathological: Before 24 hours, rapidly rising, over 14 days (term), over 21 days (preterm)
- Kernicterus: Irreversible brain damage from very high unconjugated bilirubin — PREVENTABLE
- Treatment: Phototherapy (vast majority), exchange transfusion (severe cases)
- Conjugated hyperbilirubinaemia: URGENT — biliary atresia is time-critical surgical emergency
Clinical Pearls
Jaundice in the first 24 hours of life is ALWAYS pathological — investigate and treat urgently
Pale stools + dark urine = conjugated hyperbilirubinaemia → urgent referral to exclude biliary atresia (Kasai procedure has best outcomes if performed before 6 weeks)
Always plot bilirubin on the gestation-appropriate NICE treatment threshold chart — don't rely on clinical inspection alone
Why This Matters Clinically
Neonatal jaundice is one of the most common conditions encountered in paediatrics. While usually benign, delayed recognition or treatment of severe hyperbilirubinaemia causes preventable brain damage. Every clinician working with newborns must be able to identify red flags, use treatment threshold charts correctly, and recognise conjugated hyperbilirubinaemia requiring urgent referral.
Visual assets to be added:
- Bilirubin metabolism pathway diagram
- NICE treatment threshold chart
- Phototherapy setup image
- Stool colour chart for biliary atresia screening
Incidence & Prevalence
- Visible jaundice: 60% of term infants, 80% of preterm infants
- Clinically significant jaundice (requiring treatment): 5-10% of newborns
- Exchange transfusion: 1 in 100,000 (rare with good care)
- Kernicterus incidence: 0.4-2.7 per 100,000 (higher in preterm)
- Biliary atresia: 1 in 10,000-15,000 live births
Demographics
- Gestation: Preterm infants at higher risk (immature liver enzymes)
- Ethnicity: Higher in East Asian populations
- Breastfeeding: More common but generally benign
- Birth weight: Low birth weight infants at higher risk
Risk Factors for Severe Hyperbilirubinaemia
| Risk Factor | Mechanism |
|---|---|
| Prematurity | Immature hepatic conjugation |
| ABO/Rh incompatibility | Haemolysis |
| G6PD deficiency | Haemolysis |
| Bruising from birth trauma | Increased bilirubin load |
| Previous sibling with jaundice | Familial factors |
| Exclusive breastfeeding | Breast milk jaundice |
| East Asian ethnicity | Higher incidence |
| Albumin under 30 g/L | Reduced bilirubin binding |
| Sepsis | Multiple mechanisms |
Normal Bilirubin Metabolism
- Haem breakdown: RBCs → haemoglobin → haem → biliverdin → unconjugated bilirubin
- Hepatic uptake: Unconjugated bilirubin bound to albumin → transported to liver
- Conjugation: UGT1A1 enzyme conjugates bilirubin with glucuronic acid
- Excretion: Conjugated bilirubin excreted in bile → gut → stercobilin (stool colour)
Why Neonates Are Susceptible
- High bilirubin production: Fetal RBCs have shorter lifespan (70-90 days vs 120 days)
- Immature conjugation: UGT1A1 activity only 1% of adult at birth, reaches adult levels by 14 weeks
- Enterohepatic recirculation: Increased reabsorption of unconjugated bilirubin from gut
Unconjugated vs Conjugated
| Feature | Unconjugated | Conjugated |
|---|---|---|
| Cause | Physiological, haemolysis | Biliary atresia, hepatitis |
| Stool colour | Normal (yellow/green) | Pale/acholic |
| Urine | Normal | Dark |
| Kernicterus risk | HIGH | Low (doesn't cross BBB) |
| Urgency | Depends on level | ALWAYS URGENT |
Kernicterus Pathophysiology
- Unconjugated bilirubin is lipid-soluble and crosses blood-brain barrier
- Deposits in basal ganglia, hippocampus, brainstem nuclei
- Causes neuronal necrosis → permanent damage
- Acute bilirubin encephalopathy: Lethargy, hypotonia, poor feeding → opisthotonus, seizures → death
- Chronic bilirubin encephalopathy (kernicterus): Choreoathetoid cerebral palsy, sensorineural deafness, upward gaze palsy, dental enamel dysplasia
Typical Presentation
Atypical/Concerning Presentations
Red Flags for Acute Bilirubin Encephalopathy
| Phase | Signs |
|---|---|
| Early | Lethargy, hypotonia, poor suck |
| Intermediate | Irritability, hypertonia, fever, high-pitched cry |
| Advanced | Opisthotonus, seizures, apnoea |
Any neurological signs + jaundice = immediate escalation and consideration of exchange transfusion
General Approach
- Assess jaundice extent (cephalocaudal progression)
- Check vital signs and growth parameters
- Look for signs of underlying cause
- Assess hydration and feeding
- Neurological assessment
Specific Examination Findings
| Finding | Suggests |
|---|---|
| Pallor | Haemolysis |
| Hepatosplenomegaly | Haemolysis, infection, metabolic |
| Cephalhaematoma/bruising | Increased bilirubin load |
| Petechiae | Infection (TORCH) |
| Pale stools | Conjugated hyperbilirubinaemia |
| Hypotonia, poor suck | Bilirubin encephalopathy |
| Opisthotonus, seizures | Advanced encephalopathy |
Clinical Assessment of Jaundice
- Visual inspection unreliable, especially in dark-skinned infants
- Always measure bilirubin — do not rely on clinical judgement alone
- Use transcutaneous bilirubinometer (TcB) as screening
- Confirm with serum bilirubin (SBR) if above threshold or TcB unavailable
First-Line
- Serum bilirubin (or transcutaneous if lower risk)
- Plot on NICE treatment threshold chart (gestation-appropriate)
- Repeat in 6-12 hours if concerning trend
If Pathological Features Present
| Test | Indication |
|---|---|
| FBC + blood film | Haemolysis (spherocytes, reticulocytes) |
| Blood group (mother & baby) | ABO/Rh incompatibility |
| Direct Coombs test (DAT) | Immune haemolysis |
| G6PD screen | If high-risk ethnicity or unexplained haemolysis |
| Infection screen | Sepsis suspected |
| TFTs | Prolonged jaundice |
| LFTs | Conjugated hyperbilirubinaemia |
| Conjugated bilirubin | If prolonged jaundice, pale stools, dark urine |
Prolonged Jaundice Work-Up (NICE)
For jaundice persisting over 14 days (term) or 21 days (preterm):
- Total and conjugated bilirubin
- LFTs
- TFTs
- FBC, blood group, DAT (if not done)
- Urine for reducing substances (galactosaemia)
- Stool colour chart (biliary atresia screening)
Conjugated bilirubin over 25 μmol/L = URGENT referral to exclude biliary atresia
Causes by Timing
| Timing | Causes |
|---|---|
| Under 24 hours | Haemolysis (Rh/ABO incompatibility, G6PD), sepsis — ALWAYS PATHOLOGICAL |
| 24h-14 days | Physiological, breast milk jaundice, haemolysis, infection, dehydration |
| Over 14 days (prolonged) | Breast milk, biliary atresia, hypothyroidism, metabolic, infection |
Unconjugated vs Conjugated
| Type | Common Causes | Key Features |
|---|---|---|
| Unconjugated | Physiological, haemolysis, breast milk, Crigler-Najjar | Normal stools, kernicterus risk |
| Conjugated | Biliary atresia, neonatal hepatitis, sepsis, TPN cholestasis, metabolic | Pale stools, dark urine, NO kernicterus risk but serious pathology |
NICE Treatment Thresholds
- Use gestation-appropriate treatment threshold graphs
- Different thresholds for phototherapy vs exchange transfusion
- Lower thresholds in preterm infants
- Plot actual bilirubin on chart at baby's age in hours
Management Algorithm
- Measure bilirubin (TcB or SBR)
- Plot on NICE chart for gestational age
- If below threshold: Reassure, check feeds, repeat if clinically indicated
- If above phototherapy threshold: Start phototherapy
- If approaching exchange threshold: Intensive phototherapy + urgent senior review
- If above exchange threshold: Exchange transfusion
Phototherapy
- Mechanism: Blue light (450-490nm) converts unconjugated bilirubin to water-soluble photoisomers excreted without conjugation
- Types: Conventional, intensive (multiple lights/LED blanket)
- Monitoring: Check bilirubin 4-6 hourly during phototherapy
- Side effects: Temperature instability, increased fluid requirements, bronze baby (conjugated)
- Can stop when: Bilirubin at least 50 μmol/L below treatment threshold
Exchange Transfusion
- Indication: Bilirubin above exchange threshold OR signs of acute bilirubin encephalopathy
- Mechanism: Double volume exchange (2 × blood volume ~160 ml/kg) removes bilirubin and antibodies
- Risks: Infection, electrolyte disturbance, NEC, thrombocytopenia
- Location: NICU with continuous monitoring
Intravenous Immunoglobulin (IVIG)
- Consider in Rh or ABO haemolytic disease
- May reduce need for exchange transfusion
Supportive Care
- Adequate feeding: Prevents dehydration, promotes gut motility
- Maintain temperature: Phototherapy increases heat loss
- Eye protection: During phototherapy
- Skin-to-skin breaks: For bonding during phototherapy
Acute Complications
- Acute bilirubin encephalopathy: Lethargy → hypertonia → seizures → death
- Bronze baby syndrome: Conjugated bilirubin + phototherapy → grey-brown discoloration (benign)
- Dehydration: Increased insensible losses during phototherapy
- Hypocalcaemia: During exchange transfusion
Chronic Complications (Kernicterus)
- Choreoathetoid cerebral palsy
- Sensorineural hearing loss
- Upward gaze palsy
- Dental enamel dysplasia
- Intellectual disability
Complications of Missed Conjugated Hyperbilirubinaemia
- Biliary atresia: Progressive liver failure, death if Kasai procedure not performed early
- Metabolic disease: Galactosaemia, tyrosinaemia — progressive liver failure
Natural History
- Physiological jaundice: Resolves by 2 weeks, no sequelae
- Breast milk jaundice: May persist 3-4 weeks, benign, no treatment needed
- Haemolytic disease: May have prolonged anaemia requiring monitoring
With Treatment
- Phototherapy: Highly effective; most infants respond within 24-48 hours
- Exchange transfusion: Effective at reducing bilirubin rapidly; rare complications
Prognostic Factors
| Factor | Impact |
|---|---|
| Duration of severe hyperbilirubinaemia | Longer = higher kernicterus risk |
| Prematurity | Lower threshold for kernicterus |
| Albumin level | Low albumin increases free bilirubin |
| Concurrent illness | Sepsis, acidosis increase kernicterus risk |
| Speed of treatment | Early phototherapy prevents sequelae |
Biliary Atresia Prognosis
- Kasai procedure before 60 days: 60-80% successful bile drainage
- After 90 days: Success rate drops significantly
- Without treatment: Death from liver failure by 2 years
Key Guidelines
- NICE CG98: Neonatal Jaundice (2010, updated 2023) — UK guideline for screening and management
- AAP Clinical Practice Guideline: Management of Hyperbilirubinaemia (2022) — US guideline with updated thresholds
- BAPM Framework for Newborn Jaundice — UK practical guidance
Key Evidence
Phototherapy Efficacy
- Multiple RCTs show phototherapy effectively reduces bilirubin
- LED phototherapy as effective as conventional
- Intensive phototherapy reduces need for exchange transfusion
Exchange Transfusion
- Historically standard; now rare due to effective phototherapy and screening
- Still indicated for severe cases and acute encephalopathy
Biliary Atresia Screening
- Stool colour cards in Taiwan reduced delayed diagnosis
- UK considering national screening programme
What is Neonatal Jaundice?
Jaundice is yellowing of the skin and eyes caused by a substance called bilirubin. It is very common in newborn babies — more than half of all babies develop some jaundice in the first week of life.
Most jaundice is normal and goes away on its own. However, if bilirubin levels get too high, it can sometimes be harmful. That's why we may need to measure your baby's bilirubin level with a blood test or special device on the skin.
When to Seek Help
Contact your midwife, health visitor, or GP urgently if:
- Your baby is jaundiced in the first 24 hours of life
- Jaundice is getting worse or spreading down the body
- Your baby is not feeding well or seems very sleepy
- Your baby has pale poo (chalky white/grey) or dark wee
- Jaundice is still present after 14 days (term baby) or 21 days (premature baby)
What Does Treatment Involve?
Phototherapy (light treatment):
- Your baby lies under special blue lights
- This helps break down bilirubin in the skin
- Your baby will wear eye protection
- You can still feed and cuddle your baby
How Can I Help My Baby?
- Feed frequently (at least 8-12 times in 24 hours)
- Wake your baby for feeds if they're very sleepy
- Keep them warm during phototherapy
- Watch for warning signs listed above
Resources
- NHS: Jaundice in Newborns
- NICE Patient Information on Neonatal Jaundice
- CLDF (Children's Liver Disease Foundation)
Primary Guidelines
- NICE. Jaundice in newborn babies under 28 days (CG98). 2010, updated 2023. nice.org.uk/guidance/cg98
- Kemper AR, et al. Clinical Practice Guideline Revision: Management of Hyperbilirubinemia in the Newborn Infant 35 or More Weeks of Gestation. Pediatrics. 2022;150(3):e2022058859. PMID: 35927462
Key Studies
- Maisels MJ, McDonagh AF. Phototherapy for neonatal jaundice. N Engl J Med. 2008;358(9):920-928. PMID: 18305267
- Lain SJ, et al. Association between jaundice at 24 to 72 hours of age and neonatal readmission. Pediatrics. 2015;135(2):314-320. PMID: 25624382
- Bhutani VK, et al. Kernicterus: epidemiological strategies for its prevention through systems-based approaches. J Perinatol. 2004;24(10):650-662. PMID: 15254556
Further Resources
- UK Newborn Stool Colour Card: childliverdisease.org
- UpToDate: Unconjugated Hyperbilirubinemia in the Newborn
- BMJ Best Practice: Neonatal Jaundice