Peer-reviewed article
Pediatric Acute Liver Failure in Japan: Nationwide Survey Based on a National Classification Framework.
Uchida H, Inui A, Sakamoto S, Kasahara M, Nakayama N et al. ·
Summary
Written by AI, not yet AICS's view · claude-haiku-4-5-20251001This study examined 136 children in Japan with acute liver failure between 2016 and 2021 to understand their clinical features, causes, and outcomes. Children with non-comatose liver failure had much better survival rates (67%) than those in coma (28%). Younger infants had worse outcomes than older children. A scoring system was developed to help predict which children would survive without needing a liver transplant.
Key points
- Among 136 pediatric acute liver failure cases, 35% were non-comatose and 65% were comatose
- Transplant-free survival was 66.7% in non-comatose ALF versus 28.4% in comatose ALF (p < 0.001)
- Infants had significantly lower survival rates (31.6%) compared to older children (49.4%)
- Children with circulatory failure had a 55.2% mortality rate and did not receive transplants
- A prognostic scoring system showed good ability to predict transplant-free survival (AUC 0.729) with 92.9% specificity
Limitations
This is a retrospective registry study without a comparison group, so it cannot establish causal relationships or test interventions. The study does not explain what caused acute liver failure in different children or whether specific treatments improved outcomes beyond the prognostic scoring.
Abstract
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.