Abstract
BACKGROUND: Acute necrotizing encephalopathy (ANE) is a para-infectious neurological disorder with high mortality in children. There are limited studies evaluating predictive factors for mortality in ANE patients. This study aims to investigate the predictive value of independent risk factors and the ANE Severity Score (ANE-SS), combined with ferritin levels and Disseminated Intravascular Coagulation (DIC) scores, in assessing mortality risk in children with ANE. METHODS: We conducted a retrospective study of children with ANE in the pediatric intensive care unit of Beijing Children's Hospital from January 2016 to May 2024. The patients were divided into survival and non-survival groups, and clinical data were collected within 12 hours after admission. The predictive value of clinical indicators and a joint model for mortality in ANE patients was evaluated using receiver operating characteristic (ROC) curve analysis. RESULTS: Of 56 patients enrolled, the overall mortality rate was 51.8%. In the non-survival group, the levels of ANE-SS, DIC scores, ferritin, procalcitonin, interleukin-6, activated partial thromboplastin time, prothrombin time, D-dimer, and cerebrospinal fluid protein at admission were significantly higher than those in the survival group. Multivariate analysis identified ferritin, ANE-SS, and DIC scores as independent risk factors for mortality. Ferritin exhibited an area under the curve (AUC) of 0.827, with sensitivity of 84.6% and specificity of 87.5%, which were superior to those of ANE-SS (AUC 0.782, sensitivity 75.9%, specificity 66.7%) and the DIC scores (AUC 0.773, sensitivity 60.7%, specificity 81.5%). In combined analysis, ANE-SS, ferritin, and DIC scores demonstrated the strongest predictive performance, with an AUC of 0.99 (95% CI: 0.965-1.000), sensitivity and specificity of 92.3% and 100%, respectively. CONCLUSIONS: This study indicates that the combination of ANE-SS with ferritin and DIC scores provides a superior predictive value for 28-day mortality in ANE patients.