What is the management approach for elevated bilirubin (hyperbilirubinemia)?

Management of Hyperbilirubinemia

The management of hyperbilirubinemia requires prompt identification of the cause and implementation of appropriate treatment based on bilirubin levels, with phototherapy being the primary intervention for severe neonatal cases and specific targeted therapies for adults based on underlying etiology. 1

Neonatal Hyperbilirubinemia Management

Assessment and Risk Stratification

1. Measure total serum bilirubin (TSB) or transcutaneous bilirubin (TcB) levels in all jaundiced infants 1

   • Visual assessment alone is unreliable, especially in darkly pigmented infants
   • Plot results on hour-specific nomogram to assess risk (high, intermediate, or low risk zones)

2. Risk factors for severe hyperbilirubinemia in neonates 1:

   • Major risk factors:
     • Predischarge TSB/TcB in high-risk zone
     • Jaundice in first 24 hours
     • Blood group incompatibility with positive direct antiglobulin test
     • Gestational age 35-36 weeks
     • Previous sibling received phototherapy
     • Cephalohematoma or significant bruising
     • Exclusive breastfeeding with poor feeding or excessive weight loss
     • East Asian race

Treatment Thresholds and Interventions

1. Phototherapy 1:

   • Initiate based on TSB level, gestational age, and risk factors
   • "Intensive phototherapy" requires:
     • Blue-green spectrum light (430-490 nm wavelength)
     • Irradiance of at least 30 mW/cm² per nm
     • Maximum skin surface exposure
   • Line bassinet with aluminum foil or white material to increase efficacy if TSB approaches exchange transfusion levels

2. Exchange transfusion 1:

   • Medical emergency when TSB ≥25 mg/dL (428 μmol/L)
   • Admit directly to pediatric service, not emergency department
   • Should only be performed by trained personnel in NICU with full monitoring capabilities
   • Consider bilirubin/albumin ratio as additional factor for exchange transfusion decision

3. Intravenous immunoglobulin (IVIG) 1:

   • Recommended for isoimmune hemolytic disease (0.5-1 g/kg over 2 hours)
   • Use when TSB rising despite intensive phototherapy or within 2-3 mg/dL of exchange level

4. Monitoring during treatment 1:

   • If TSB ≥25 mg/dL: repeat TSB within 2-3 hours
   • If TSB 20-25 mg/dL: repeat within 3-4 hours
   • If TSB <20 mg/dL: repeat in 4-6 hours
   • Continue until TSB <13-14 mg/dL

Follow-up Care

1. Post-discharge follow-up timing 1:

   • Discharge before 24h: see by 72h
   • Discharge between 24-47.9h: see by 96h
   • Discharge between 48-72h: see by 120h

2. Earlier follow-up for infants with risk factors 1

Adult Hyperbilirubinemia Management

Diagnostic Approach

1. Determine pattern of hyperbilirubinemia:

   • Unconjugated (indirect) hyperbilirubinemia
   • Conjugated (direct) hyperbilirubinemia
   • Mixed pattern

2. Laboratory evaluation:

   • Total and direct bilirubin levels
   • Complete blood count with peripheral smear
   • Liver function tests
   • Appropriate imaging based on suspected etiology

Management Based on Etiology

1. Unconjugated hyperbilirubinemia:

   • Gilbert's syndrome: usually benign, no specific treatment needed 2
   • Hemolytic causes: treat underlying hemolytic process
   • Drug-induced: consider medication adjustment or discontinuation 3

2. Conjugated hyperbilirubinemia:

   • Hepatocellular injury: treat underlying liver disease
   • Biliary obstruction: may require endoscopic or surgical intervention
   • Drug-induced: modify antiviral drug choice or dose in cases of liver injury 3

Special Considerations

Neonatal Jaundice Types

1. Physiological jaundice 4:

   • Most common type
   • Self-limiting, typically resolves within 1-2 weeks

2. Pathological jaundice 4:

   • Requires prompt evaluation and treatment
   • Causes include hemolytic disease, infections, metabolic disorders

3. Breast milk jaundice 4:

   • May persist longer than physiological jaundice
   • Typically benign but requires monitoring

Pitfalls and Caveats

1. Direct bilirubin measurement is imprecise; values >1.0 mg/dL are considered abnormal when TSB ≤5 mg/dL 1

2. Do not subtract direct bilirubin from total when using guidelines for phototherapy and exchange transfusion 1

3. If direct bilirubin is ≥50% of total bilirubin, consult with an expert 1

4. Capillary versus venous samples: obtaining a venous sample to "confirm" an elevated capillary TSB is not recommended as it delays treatment 1

5. G6PD deficiency screening is important in significant hyperbilirubinemia, especially in at-risk populations (African Americans, Mediterranean, Asian) 1

6. Mild indirect hyperbilirubinemia associated with impaired conjugation (as in Gilbert's syndrome or some antiviral medications) is generally well-tolerated 3, 2

7. TSB that doesn't decrease or continues to rise despite intensive phototherapy strongly suggests hemolysis 1