What is the management approach for a patient with hyperbilirubinemia?

Management of Hyperbilirubinemia

The management of hyperbilirubinemia should be guided by the patient's age, bilirubin levels, and underlying cause, with phototherapy initiated when total serum bilirubin (TSB) reaches thresholds based on age, gestational age, and risk factors. 1

Diagnostic Approach

Initial Assessment

• Determine if hyperbilirubinemia is direct (conjugated) or indirect (unconjugated)
• Direct bilirubin >1.0 mg/dL when total bilirubin is ≤5 mg/dL is considered abnormal 1

Laboratory Evaluation

1. First-line tests:

   • Total and direct bilirubin levels
   • Complete blood count with peripheral smear
   • Blood type and Rh status (mother and infant)
   • Direct antiglobulin test (Coombs)

2. Additional tests based on clinical presentation:

   • For elevated indirect bilirubin:

     • Reticulocyte count
     • G6PD screening
     • Albumin level
     • End-tidal carbon monoxide (ETCOc) if available 2

   • For elevated direct bilirubin:

     • Urinalysis and urine culture
     • Liver function tests
     • Evaluation for sepsis if indicated 2
     • Consider imaging studies (ultrasound, MR cholangiography) for suspected biliary obstruction 1

Management of Neonatal Hyperbilirubinemia

Risk Assessment

• Major risk factors for severe hyperbilirubinemia in infants ≥35 weeks gestation 2:
  • TSB in high-risk zone
  • Jaundice in first 24 hours
  • Blood group incompatibility with positive direct antiglobulin test
  • G6PD deficiency
  • Gestational age 35-36 weeks
  • Previous sibling requiring phototherapy
  • Cephalohematoma or significant bruising
  • Exclusive breastfeeding with poor feeding or excessive weight loss
  • East Asian race

Phototherapy

• Indications: Based on TSB thresholds according to AAP nomogram, considering:

  • Age in hours
  • Gestational age
  • Presence of risk factors 1

• Effective phototherapy delivery:

  • Use special blue fluorescent tubes or LED light sources (425-475 nm wavelength)
  • Ensure irradiance level >30 mW/cm² per nm
  • Maximize skin exposure (except eyes and genitalia) 1
  • Continue until bilirubin levels are clearly declining 1

Exchange Transfusion

• Indications:

  • TSB ≥25 mg/dL (428 μmol/L)
  • TSB reaches exchange level per AAP guidelines
  • Signs of acute bilirubin encephalopathy
  • Failure to respond to intensive phototherapy 1

• Bilirubin/Albumin (B/A) ratio:

  • Can be used as an additional factor in determining need for exchange transfusion
  • Should not replace TSB-based decisions 2

Management of Hyperbilirubinemia in Older Infants and Adults

Approach Based on Etiology

1. Unconjugated hyperbilirubinemia:

   • Hemolytic disorders: Treat underlying cause
   • Gilbert syndrome: Usually no treatment required
   • Drug-induced: Discontinue offending agent

2. Conjugated hyperbilirubinemia:

   • Biliary obstruction: Consider endoscopic or surgical intervention
   • Hepatocellular disease: Treat underlying condition
   • Regular monitoring of bilirubin levels and liver function tests until resolution 1

Supportive Care

• Maintain adequate hydration
• Nutritional support
• Monitor for complications of underlying disease

Special Considerations

Breastfeeding and Hyperbilirubinemia

• Continue breastfeeding during phototherapy
• Advise mothers to nurse 8-12 times per day
• Avoid routine supplementation with water in nondehydrated breastfed infants 1
• Breastfed infants may have higher bilirubin levels than formula-fed infants 1

Follow-up

• Schedule follow-up at 2-4 weeks to check hemoglobin levels with rare antibodies
• Monitor for late-onset anemia 1
• Frequency of monitoring depends on severity of elevation and underlying cause

Complications and Prognosis

• Untreated severe hyperbilirubinemia can lead to:
  • Acute bilirubin encephalopathy
  • Kernicterus (chronic bilirubin encephalopathy)
  • Long-term neurodevelopmental impairments 3
• Incidence of chronic bilirubin encephalopathy is estimated at 0.9 per 100,000 live births 1
• Exchange transfusion has complications in about 5% of treated infants 4

The management of hyperbilirubinemia requires prompt identification of the underlying cause and appropriate intervention based on bilirubin levels and risk factors to prevent potentially devastating neurological complications.