The incidence, frequency of late liver-related complications, and transplant likelihood in pediatric acute liver failure in Californian children.

BACKGROUND

Early studies of pediatric acute liver failure (PALF) estimate that 60%-70% of patients survive with their native liver (SNL), 20%-25% require an emergent liver transplant (LT), and 10%-15% are mortalities. These studies were largely limited to academic centers and focused on short-term outcomes.

METHODS

Herein, we analyzed California Department of Healthcare Access and Information data, which is demographically representative of the US population. Between June 1990 and December 2018, 2270 children were admitted to California hospitals with PALF.

RESULTS

The overall incidence of PALF was 16.75 per 100,000 live births, with no evidence of seasonality. LT was performed in 188 (8.3%) patients and 425 (18.7%) patients died. Mortality was associated with contraindications to transplant, such as concomitant hematologic malignancy or cardiac disease. With a median follow-up of 4.5 years, nearly all deaths occurred within 4 months of presentation, with no long-term liver-related comorbidities in patients with SNL. Implementation of pediatric end-stage liver disease/MELD-based organ allocation in 2002 resulted in a more than 2-fold increase in the likelihood of LT in children <10 years (subdistribution hazard ratio: 3.35). Patients receiving care at transplant centers after this change were younger (10 vs. 12 y), more often from urban areas (98.2% vs. 94%), and more likely to receive LT for metabolic, genetic, or "other" causes of PALF (up to subdistribution hazard ratio 6.4 [1.84-22.26], p<0.01). While mortality was higher at transplant centers (26.5% vs. 14.6%), the time to mortality was also increased (31 vs. 7 d), likely reflecting disease complexity requiring management in a specialized children's hospital.

CONCLUSION

In conclusion, this study supports an estimate of incidence and long-term outcomes of PALF across a large population and highlights the role of specialized pediatric transplant centers for the optimized care of PALF.