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Pediatric Acute Liver Failure in Japan: Nationwide Survey Based on a National Classification Framework
Hajime Uchida1, Ayano Inui2, Seisuke Sakamoto1
1Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan.
Insights
Pediatric acute liver failure (ALF) outcomes differ significantly between noncomatose and comatose patients. Early prognostic scoring aids in guiding clinical decisions for pediatric ALF, highlighting the need for specialized tools.
Area of Science:
- Pediatric Hepatology
- Clinical Toxicology
- Transplantation Medicine
Background:
- Acute pediatric liver failure (ALF) is a critical condition with significant mortality.
- Differentiating between noncomatose and comatose ALF is crucial for prognosis and management.
- Existing prognostic tools may not be optimized for the pediatric population.
Purpose of the Study:
- To characterize clinical features, etiologies, management, and outcomes of pediatric noncomatose and comatose ALF in Japan.
- To evaluate an early liver transplantation (LT) indication scoring system in pediatric ALF.
- To identify factors influencing transplant-free survival in pediatric ALF.
Main Methods:
- Nationwide retrospective study of pediatric ALF cases (2016-2021) from a national registry.
- Classification of patients into noncomatose and comatose ALF groups based on Japanese criteria.
- Subgroup analyses by age, etiology, and outcomes; evaluation of a prognostic scoring system.
Main Results:
- 136 pediatric ALF cases analyzed; 35.3% noncomatose, 64.7% comatose.
- Transplant-free survival was significantly higher in noncomatose (66.7%) versus comatose ALF (28.4%).
- Circulatory failure indicated poor prognosis; infants had lower survival rates. Prognostic score showed good discriminative ability (AUC 0.729).
Conclusions:
- Age, etiology, and early prognostic scoring are vital for clinical decision-making in pediatric ALF.
- Pediatric-specific prognostic tools are necessary for improved management.
- Refinement of pediatric LT selection criteria is supported by these findings.
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.
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