Objective: To investigate the risk factors for death in children with acute 
necrotizing encephalopathy (ANE) in pediatric intensive care unit (PICU). 
Methods: This was a multicenter retrospective study. Thirty-nine children with 
ANE were from PICUs in 4 centers from December 1, 2014 to December 1, 2020. The 
4 participating centers were Beijing Children's Hospital, Shengjing Hospital of 
China Medical University, Hebei Children's Hospital, and Bao'an Maternity & 
Child Health Hospital. Patients were divided into survival and non-survival 
groups by the outcome at discharge, and the differences in clinical data between 
the two groups were compared. Risk factors for death in children with ANE and 
the odds ratios (OR) were analyzed by univariable Logistic regression. Results: 
Thirty-nine children with ANE were included. There were 18 males and 21 females. 
The median onset age was 30 months. The mortality at discharge was 41% (16/39). 
The onset age of most patients (74%, 29/39) was younger than 4 years old. 
Influenza virus was the most common precursor infection (80%, 20/25). Patients 
with shock at PICU admission were more common in the non-survival group (12/16 
vs. 17% (4/23), P=0.001). Glasgow coma score (GCS) at PICU admission was 
significantly lower in the non-survival group than survival group (3 (3, 6) vs. 
6 (5, 7), Z=-2.598, P=0.009). The optimal cut-off value was 4. The proportion of 
patients with GCS ≤ 4 at PICU admission was higher in the non-survival group 
(10/16 vs. 22% (5/23), P=0.018). ANE severity score (ANE-SS) at PICU admission 
was significantly higher in the non-survival group (5 (2, 6) vs. 2 (1, 4), 
Z=-2.436, P=0.015). The proportion of patients with high risk ANE-SS was higher 
in non-survival group than the survival group (9/16 vs. 22% (5/23), P=0.043). 
The proportion of application of high-dose methylprednisolone (20 mg/(kg·d)) was 
significantly higher in survival group than non-survival group (43% (10/23) vs. 
1/13, P=0.031). Univariable Logistic regression indicated that risk factors for 
death in children with ANE were shock (OR=14.250, 95%CI 2.985-68.018, P=0.001), 
GCS≤4 (OR=6.000, 95%CI 1.456-24.733, P=0.013) and high risk ANE-SS (OR=4.629, 
95%CI 1.142-18.752, P=0.032) at PICU admission. Conclusions: ANE usually occurs 
in children under 4 years old after influenza infection. Shock, GCS≤4 and high 
risk ANE-SS at PICU admission were risk factors for death in children with ANE. 
High-dose methylprednisolone may improve the prognosis of children with ANE.