Pediatric neurology · 2026
A Comparative Study of Clinical Features and Outcomes in Children With Acute Fulminant Cerebral Edema and Acute Necrotizing Encephalopathy.
СтатьяСравнительное исследованиеНазвание и аннотация: Перевод в очереди
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Lin Sufang, Su Qiru, Su Guoyun, Long Weiwei, Ma Weike, Fan Qimeng, Liao Jianxiang, Yang Weiguo
Аннотация
BACKGROUND: To compare clinical characteristics, laboratory parameters, and prognosis between children with acute fulminant cerebral edema (AFCE) and acute necrotizing encephalopathy (ANE).
METHODS: A single-center retrospective study was conducted between June 2021 and June 2023. Demographics, etiology, complications, laboratory markers, and outcomes were compared. Survival was estimated by Kaplan-Meier analysis. Receiver operating characteristic curves assessed biomarkers for differentiating AFCE from ANE.
RESULTS: A total of 36 children (13 AFCE, 23 ANE) were included. Both conditions were associated with influenza and severe acute respiratory syndrome coronavirus 2, with no age/sex differences. AFCE patients had significantly higher incidences of multiple organ dysfunction syndrome (92.3% vs 34.8%, P = 0.001), disseminated intravascular coagulation (92.3% vs 30.4%, P < 0.001), and brain herniation (92.3% vs 34.8%, P = 0.001). Procalcitonin and interleukin-6 were significantly elevated in AFCE patients. Prognosis was substantially worse in AFCE: 7-day mortality (76.9% vs 30.4%, P = 0.014), 30-day mortality (84.6% vs 47.8%, P = 0.039), 2-year mortality (92.3% vs 52.2%, P = 0.025), and worse modified Rankin Scale distribution (P = 0.034). In this exploratory analysis, procalcitonin showed the highest discriminative ability, with an area under the curve of 0.818 (95% confidence interval: 0.659-0.978). A procalcitonin cutoff of 38.89 ng/mL yielded a sensitivity of 76.9% and a specificity of 81.8% for identifying AFCE.
CONCLUSIONS: These exploratory findings suggest that AFCE may represent a more severe phenotypic presentation than ANE within the infection-triggered encephalopathy syndromes spectrum, characterized by hyperinflammation, multi-organ injury, and poor outcomes. However, given the small sample size and lack of adjustment for multiple comparisons, the results should be interpreted as hypothesis-generating and require prospective validation.
METHODS: A single-center retrospective study was conducted between June 2021 and June 2023. Demographics, etiology, complications, laboratory markers, and outcomes were compared. Survival was estimated by Kaplan-Meier analysis. Receiver operating characteristic curves assessed biomarkers for differentiating AFCE from ANE.
RESULTS: A total of 36 children (13 AFCE, 23 ANE) were included. Both conditions were associated with influenza and severe acute respiratory syndrome coronavirus 2, with no age/sex differences. AFCE patients had significantly higher incidences of multiple organ dysfunction syndrome (92.3% vs 34.8%, P = 0.001), disseminated intravascular coagulation (92.3% vs 30.4%, P < 0.001), and brain herniation (92.3% vs 34.8%, P = 0.001). Procalcitonin and interleukin-6 were significantly elevated in AFCE patients. Prognosis was substantially worse in AFCE: 7-day mortality (76.9% vs 30.4%, P = 0.014), 30-day mortality (84.6% vs 47.8%, P = 0.039), 2-year mortality (92.3% vs 52.2%, P = 0.025), and worse modified Rankin Scale distribution (P = 0.034). In this exploratory analysis, procalcitonin showed the highest discriminative ability, with an area under the curve of 0.818 (95% confidence interval: 0.659-0.978). A procalcitonin cutoff of 38.89 ng/mL yielded a sensitivity of 76.9% and a specificity of 81.8% for identifying AFCE.
CONCLUSIONS: These exploratory findings suggest that AFCE may represent a more severe phenotypic presentation than ANE within the infection-triggered encephalopathy syndromes spectrum, characterized by hyperinflammation, multi-organ injury, and poor outcomes. However, given the small sample size and lack of adjustment for multiple comparisons, the results should be interpreted as hypothesis-generating and require prospective validation.