Emergency Living Donor Transplantation for Pediatric Autoimmune Acute Liver Failure

A 14-year-old female presented with acute liver failure secondary to autoimmune hepatitis after four months of progressive jaundice and transaminitis. She had worsening coagulopathy and impaired synthetic function at referral.

Investigations revealed markedly elevated aminotransferases, hyperbilirubinemia, elevated INR, hypoalbuminemia, positive ANA and ASMA, and hypergammaglobulinemia. Viral, metabolic, Wilson’s disease, and drug-induced causes were excluded. Liver biopsy showed interface hepatitis with bridging necrosis, confirming severe autoimmune injury.

Despite initiation of high-dose corticosteroids and transient biochemical improvement, she developed Grade III hepatic encephalopathy with worsening coagulopathy, hypoglycemia, and lactic acidosis, consistent with fulminant ALF.

She received ICU care with therapeutic plasma exchange as a bridge to transplantation. Due to rapid deterioration, emergency living donor liver transplantation was performed, with her mother as donor. Explant showed extensive necro-inflammatory destruction. Post-transplant, she demonstrated rapid neurological and biochemical recovery. Both donor and recipient remain clinically stable with good graft function. 

Clinical Implications

This case highlights a few important lessons:

Autoimmune hepatitis in children can suddenly worsen, even if they initially improve with treatment.

Early diagnosis and close monitoring after discharge are crucial, as the condition can deteriorate quickly.

Plasma exchange can help temporarily support the patient until a transplant is arranged.

A timely liver transplant can be life-saving in rapidly worsening cases.

A strong team approach and the willingness of a living donor play a critical role in emergencies.

Overall, this case shows that quick action, regular reassessment, and timely liver transplantation can save lives in children with severe autoimmune liver failure.