What is the treatment for hepatitis with encephalopathy in children?

Treatment of Hepatitis with Encephalopathy in Children

The first-line treatment for hepatitis with encephalopathy in children is non-absorbable disaccharides (lactulose or lactitol) combined with identification and treatment of precipitating factors. 1

Initial Management

Assessment and Stabilization

• Evaluate for signs of acute liver failure and cerebral edema
• Monitor vital signs, neurological status, and level of consciousness
• Assess for precipitating factors:
  • Infections
  • Gastrointestinal bleeding
  • Electrolyte disturbances
  • Dehydration
  • Medication side effects

First-Line Treatment

1. Non-absorbable disaccharides

   • Lactulose is FDA-approved for treatment of hepatic encephalopathy 2
   • Dosing:
     • Oral: 1-2 mL/kg/dose every 4-6 hours
     • Rectal: 10 mL/kg as retention enema for severe cases
   • Titrate to achieve 2-3 soft bowel movements daily
   • Mechanism: Reduces ammonia absorption by acidifying colonic contents and trapping ammonia as non-absorbable NH4+ 3
   • Evidence: Studies show lactulose therapy leads to complete recovery from hepatic encephalopathy in 73% of pediatric patients with cirrhosis 4

2. Identify and treat precipitating factors

   • This should be done simultaneously with lactulose initiation 1
   • Common precipitating factors in children include:
     • Infections (treat with appropriate antibiotics)
     • Dehydration (correct fluid status)
     • Electrolyte imbalances (correct sodium, potassium)
     • Gastrointestinal bleeding (control bleeding source)

Viral Encephalitis Considerations

If viral etiology is suspected:

• Acyclovir should be started immediately if HSV or VZV encephalitis is suspected 1
  • Children 3 months-12 years: 500 mg/m² IV every 8 hours
  • Children >12 years: 10 mg/kg IV every 8 hours
  • Duration: 14-21 days for HSV encephalitis
  • Consider repeat lumbar puncture at treatment completion to confirm CSF is negative for HSV by PCR 1

Second-Line Treatment

If inadequate response to lactulose after 24-48 hours:

1. Rifaximin (for children ≥12 years) 5

   • Dosing: 550 mg orally twice daily
   • Used as add-on therapy to lactulose when lactulose alone fails 1
   • Not FDA-approved for children <12 years

2. Alternative antibiotics (if rifaximin unavailable or contraindicated)

   • Neomycin or metronidazole may be considered in combination with lactulose 6
   • However, these have more side effects than rifaximin

Management of Specific Hepatitis Etiologies

Autoimmune Hepatitis with Encephalopathy

For children with autoimmune hepatitis presenting with encephalopathy:

• Corticosteroids 1
  • Prednisone/prednisolone: 1-2 mg/kg/day (up to 60 mg/day)
  • Taper over 6-8 weeks to 0.1-0.2 mg/kg/day or 5 mg daily
  • Consider adding azathioprine (1-2 mg/kg/day) early to minimize steroid side effects 1

Viral Hepatitis with Encephalopathy

For chronic viral hepatitis (e.g., HBV) with encephalopathy:

• Treat underlying viral infection according to specific guidelines 1
• Continue management of encephalopathy with lactulose

Acute Severe Hepatitis or Acute Liver Failure

For children with acute severe hepatitis or acute liver failure with encephalopathy:

• Immediate evaluation for liver transplantation 1
• Trial of corticosteroids (prednisone/prednisolone 0.5-1 mg/kg/day) may be considered in select cases 1
• Abandon ineffective treatment quickly (within 1-2 weeks) and proceed to liver transplantation if no improvement 1

Monitoring and Follow-up

• Daily neurological assessment using age-appropriate encephalopathy scales
• Monitor serum ammonia levels, though treatment decisions should be based on clinical status rather than ammonia levels alone
• Ensure adequate hydration and monitor renal function, especially with acyclovir treatment 1
• Monitor for lactulose side effects: diarrhea, abdominal cramping, flatulence, electrolyte disturbances

Special Considerations

• Cerebral edema management: For severe encephalopathy with cerebral edema, consider:

  • Head elevation to 30 degrees
  • Fluid restriction
  • Mannitol or hypertonic saline for increased intracranial pressure 6
  • Mechanical ventilation for airway protection if needed

• Nutritional support:

  • Maintain adequate caloric intake
  • Avoid excessive protein restriction in children
  • Consider branched-chain amino acid supplementation in severe cases

Pitfalls and Caveats

1. Delaying treatment of encephalopathy can lead to irreversible neurological damage
2. Excessive lactulose administration can cause dehydration and electrolyte imbalances
3. Failure to identify and treat precipitating factors will result in poor response to therapy
4. Overreliance on ammonia levels rather than clinical assessment may lead to inappropriate management
5. Inadequate duration of acyclovir treatment (if viral etiology) can lead to relapse, especially in children who require a minimum of 21 days of treatment 1