Pediatric Acute Liver Failure in Japan: Nationwide Survey Based on a National Classification Framework.

OBJECTIVES

Acute pediatric liver failure (ALF) is a rare but life-threatening condition. This nationwide retrospective study aimed to characterize the clinical features, etiologies, management strategies, and outcomes of pediatric noncomatose and comatose ALF in Japan and to evaluate the utility of an early liver transplantation (LT) indication scoring system in this population.

METHODS

We reviewed pediatric cases of noncomatose and comatose ALF reported in a national registry between 2016 and 2021. Based on the Japanese criteria, patients were classified into noncomatose and comatose ALF groups. Subgroup analyses were carried out based on age, etiology, and outcomes.

RESULTS

A total of 136 patients (median age, 15.8 months) were included. Among the 136 patients, 48 (35.3%) were classified as noncomatose ALF and 88 (64.7%) as comatose ALF. Transplant-free survival was significantly higher in noncomatose ALF (66.7%) than in comatose ALF (28.4%) (p < 0.001). Patients with circulatory failure had a markedly worse prognosis, a high mortality rate (55.2%), and did not undergo LT. Infants had significantly lower transplant-free survival rates than older children (31.6% vs. 49.4% and p = 0.052). Survivors (n = 57) had higher platelet counts, lower bilirubin levels, and less liver atrophy patients who died or underwent LT (n = 79). The prognostic score correlated with outcomes and demonstrated a strong discriminative ability for predicting transplant-free survival (AUC 0.729; sensitivity 32.5% and specificity 92.9%).

CONCLUSION

Age, etiology, and early prognostic scoring may appropriately guide clinical decisions. These findings underscore the need for pediatric-specific prognostic tools and support the refinement of pediatric LT selection criteria.