Jaundice is the yellow colouring of skin and sclera caused by the accumulation of bilirubin in the skin and mucous membranes
Aetiology
- Neonatal jaundice occurs in 60% of term infants and 80% of preterm infants and is caused by hyperbilirubinaemia that is unconjugated (divided into physiological or pathological) or conjugated (always pathological)
- Around 10% of term breastfed babies are jaundiced at 1 month
Pathophysiology
Unconjugated
- Physiological - jaundice in a healthy baby, born at term, is normal and may result from increased RBC breakdown or the immature liver not being able to process high bilirubin concentrations
- In premature babies, the process of physiological jaundice is exaggerated due to the immature liver - increases risk of complications
- Breast milk jaundice - well baby, resolves between 1.5-4 months
- Haemolysis
- Infection - neonatal sepsis is a common cause of jaundice in the first 24 hours of life
- Inherited causes
- Intestinal obstruction
Conjugated
- Biliary atresia
- TPN
- Hypothyroidism
- Genetic conditions e.g. cystic fibrosis, trisomy 21
Prolonged jaundice
- Jaundice is 'prolonged' when it lasts longer than would be expected in physiological jaundice - over 14 days in a term baby or 21 days in a preterm baby
Clinical presentation
Kramer Zones Classification
Kramer Zone | Area of Jaundice Involvement | Approximate Serum Bilirubin (mg/dL) | Clinical Interpretation |
Zone 1 | Head and neck | 5–7 mg/dL | Mild jaundice |
Zone 2 | Upper trunk (chest, upper abdomen) | 8–10 mg/dL | Mild–moderate |
Zone 3 | Lower trunk and thighs | 11–13 mg/dL | Moderate |
Zone 4 | Arms and lower legs | 14–16 mg/dL | Moderate–severe |
Zone 5 | Palms and soles | ≥17–20 mg/dL | Severe jaundice |
Investigations
Bilirubin Assessment
Test | Purpose | Interpretation |
Total Serum Bilirubin (TSB) | Confirm jaundice severity | >5 mg/dL = clinically visible jaundice |
Direct (Conjugated) Bilirubin | Differentiate type | >1 mg/dL or >20% of TSB = pathological |
Transcutaneous bilirubin (TcB) | Screening | Correlates with TSB |
Clinical Scenario | Key Investigations |
Jaundice <24 hours | TSB, Coombs, Hb, reticulocytes |
Rapidly rising bilirubin | Repeat TSB, hemolysis workup |
Prolonged jaundice | Direct bilirubin, LFTs, TSH |
Pale stools + dark urine | LFTs, ultrasound, HIDA |
Poor feeding + lethargy | Sepsis screen |
Family history of jaundice | G6PD, RBC disorders |
Management
Phototherapy
Mechanism
- Converts unconjugated bilirubin into water-soluble isomers
Indications
Term Neonates (≥38 weeks, NO risk factors)
Age (hours) | Start Phototherapy if TSB ≥ |
<24 h | Pathological → urgent evaluation |
24–48 h | 15 mg/dL |
49–72 h | 18 mg/dL |
>72 h | 20 mg/dL |
Term Neonates with Risk Factors
(*hemolysis, sepsis, asphyxia, hypoalbuminemia, G6PD deficiency)
Age (hours) | Start Phototherapy if TSB ≥ |
24–48 h | 13 mg/dL |
49–72 h | 15 mg/dL |
>72 h | 17 mg/dL |
Exchange Transfusion
Indications
- Failure of intensive phototherapy
- Signs of acute bilirubin encephalopathy
- Very high bilirubin levels
Based on Serum Bilirubin (Term Neonates)
Age (hours) | Exchange Transfusion if TSB ≥ |
24–48 h | 20 mg/dL |
49–72 h | 25 mg/dL |
>72 h | 25–30 mg/dL |
Absolute Indications
Indication | Explanation |
Acute bilirubin encephalopathy | Neurological emergency |
Failure of intensive phototherapy | Persistent bilirubin rise |
Severe hemolysis (Rh disease) | Ongoing bilirubin production |
Rapid bilirubin rise (>1 mg/dL/hour) | Imminent kernicterus |