Acute Liver Failure in Children: Optimal Care and Accessing Transplant

Definition and Overview

Acute liver failure (ALF) in children is a rare, rapidly progressive condition characterized by severe hepatic dysfunction in a previously healthy child. It is marked by coagulopathy (INR >1.5) and hepatic encephalopathy within weeks of the onset of symptoms. ALF demands urgent medical attention and, in severe cases, liver transplantation is often the only lifesaving option.

Common Causes in Paediatrics

1. Viral Hepatitis: Hepatitis A, B, E, or rarely non-A-E hepatitis.
2. Drug-Induced Hepatotoxicity: Overdose or idiosyncratic reactions to medications like paracetamol or traditional/herbal remedies.
3. Metabolic Disorders: Wilson’s disease, urea cycle defects and mitochondrial disorders are only a few examples. 
4. Autoimmune Hepatitis: Sudden severe autoimmune liver injury.
5. Unknown Causes: Up to 30% of cases remain idiopathic.

Signs and Symptoms to Watch For
Early recognition of ALF is crucial. Key signs include:

• Persistent Jaundice: Sudden and progressive.
• Encephalopathy: Behavioural changes, confusion, irritability, or altered consciousness.
• Coagulopathy: Prolonged bleeding or bruising due to clotting factor deficiency.
• Severe Fatigue: Often associated with nausea, vomiting, and poor oral intake.

Initial Management Prior to Referral

1. Stabilization:
   • Correct hypoglycaemia with intravenous glucose.
   • Address coagulopathy with fresh frozen plasma only if actively bleeding. The INR is used as a measure of progression and FFP can mask the severity.
   • Manage encephalopathy with appropriate measures including ventilation for neuroprotection if indicated.
2. Investigations:
   • Liver function tests (ALT, AST, bilirubin, INR).
   • Viral hepatitis panel.
   • Ammonia and Factor 5 
3. Early Communication:
   • Contact a paediatric liver transplant centre immediately when ALF is suspected.

When to Refer to a Transplant Centre
Early referral is vital, as delays can compromise transplant eligibility. Refer immediately if the patient demonstrates:

• Encephalopathy of any degree including irritability, somnolence or mild behavioural changes.
• INR >2.0 or refractory coagulopathy.
• Worsening jaundice or signs of systemic instability.
• Unknown aetiology with no response to supportive treatment.

Accessing Liver Transplant Centres in South Africa
In South Africa, paediatric liver transplants are centralized in urban centres such as the Wits Donald Gordon Medical Centre in Johannesburg. To facilitate access:

1. Initiate Contact: Discuss the case with transplant specialists for advice on stabilization and transfer.
2. Streamline Transport: Arrange for urgent transfer with appropriate medical support.
3. Collaborate on Assessment: The centre will assess for transplant eligibility and plan the next steps.

Challenges and Importance of Early Action

1. Resource Limitations: Organ availability is scarce, making timely listing critical.
2. Geographic Barriers: Rural practitioners must act quickly to ensure timely referrals.
3. Parental Counselling: Educate families on the gravity of ALF and the potential need for transplantation.

Conclusion
Paediatric ALF is a critical emergency requiring swift diagnosis, stabilization, and referral to a liver transplant centre. Doctors play a key role in recognizing early warning signs, initiating appropriate care, and facilitating rapid access to specialized services. Optimal outcomes depend on timely intervention, multidisciplinary collaboration, and community awareness of this life-threatening condition.