<b>Objective:</b> A case-control study of Influenza-Associated Necrotizing Encephalopathy (IANE) in children was conducted to explore the risk factors for the diagnosis of IANE, and to provide a predictive...<b>Objective:</b> A case-control study of Influenza-Associated Necrotizing Encephalopathy (IANE) in children was conducted to explore the risk factors for the diagnosis of IANE, and to provide a predictive reference for the diagnosis of IANE. <b>Methods:</b> The children with IANE who received treatment in our hospital from January 2016 to December 2020 were selected as the study group, and the children with Influenza-Associated Encephalopathy (IAE) group who received treatment in the same period were selected as the control group. The blood biochemical, coagulation function and cerebrospinal fluid test results of the two groups were analyzed by univariate analysis. Receiver Operating Characteristic curve (ROC) analysis was used to determine the optimal threshold point of each index for the indicators with statistically significant differences in univariate analysis results, and multivariate Logistic stepwise regression analysis was performed according to the optimal threshold points. <b>Results:</b> In the IANE group, there were 32 children, including 20 males and 12 females, aged 60 (35, 84) months. There were 40 children in IAE group, including 26 males and 14 females, aged 58 (23, 97) months. Univariate results showed that serum Lactate Dehydrogenase (LDH), Cerebrospinal Fluid Lactate Dehydrogenase (CSF LDH) and Cerebrospinal Fluid Protein (CSF PRO) in the IANE group were significantly higher than those in the IAE group, and the difference between the two groups was statistically significant (P < 0.001). The optimal threshold points of blood LDH, CSF LDH and CSF PRO by ROC curve analysis were 535 U/L, 67 U/L and 0.49 g/L, respectively. Further Multivariate Logistic stepwise regression analysis showed that LDH > 535 U/L (OR = 31.264, 95% CI: 5.892 - 165.878, P < 0.001) and CSF PRO > 0.49 g/L (OR = 7.695, 95% CI: 1.052 - 56.305, P = 0.044) were independent risk factors for IANE. <b>Conclusion:</b> For children with influenza whose neurological symptoms appear rapidly and persist in the early stages of the disease, blood LDH > 535 U/L and CSF PRO > 0.49 g/L are independent risk factors for IANE.展开更多
Importance Acute necrotizing encephalopathy(ANE)is a rare disease with high mortality.Plasma exchange(PLEX)has recently been reported to treat ANE of childhood(ANEC),but its efficacy is uncertain.Objective This study ...Importance Acute necrotizing encephalopathy(ANE)is a rare disease with high mortality.Plasma exchange(PLEX)has recently been reported to treat ANE of childhood(ANEC),but its efficacy is uncertain.Objective This study aimed to investigate the effectiveness of PLEX on ANEC.Methods A retrospective study was conducted in four pediatric intensive care units from December 2014 to December 2020.All patients who were diagnosed with ANEC were included;however,these patients were excluded if their length of stay was less than 24 h.Participants were classified into PLEX and non-PLEX groups.Results Twenty-nine patients with ANEC were identified,10 in the PLEX group and 19 in the non-PLEX group.In the PLEX group,C-reactive protein,procalcitonin,alanine aminotransferase,and aspartate aminotransaminase levels were significantly lower after 3 days of treatment than before treatment(13.1 vs.8.0,P=0.043;9.8 vs.1.5,P=0.028;133.4 vs.31.9,P=0.028;282.4 vs.50.5,P=0.046,respectively).Nine patients(31.0%,9/29)died at discharge,and a significantly difference was found between the PLEX group and non-PLEX group[0 vs.47.4%(9/19),P=0.011].The median follow-up period was 27 months,and three patients were lost to follow-up.Thirteen patients(50.0%,13/26)died at the last follow-up,comprising three(33.3%,3/9)in the PLEX group and ten(58.8%,10/17)in the non-PLEX group,but there was no significant difference between the two groups(P=0.411).Three patients(10.3%,3/29)fully recovered.Interpretation PLEX may reduce serum C-reactive protein and procalcitonin levels and improve liver function in the short term.PLEX may improve the prognosis of ANEC,and further studies are needed.展开更多
Introduction:Acute necrotizing encephalopathy(ANE),a fatal subtype of infection-triggered encephalopathy syndrome(ITES),can be triggered by many systemic infections.RANBP2 gene mutations were associated with recurrent...Introduction:Acute necrotizing encephalopathy(ANE),a fatal subtype of infection-triggered encephalopathy syndrome(ITES),can be triggered by many systemic infections.RANBP2 gene mutations were associated with recurrent ANE.Case presentation:Here we report a 1-year-old girl with recurrent ITES and RANBP2 mutation.She was diagnosed with influenza-associated encephalopathy and made a full recovery on the first episode.After severe acute respiratory syndrome coronavirus 2 infection,the patient presented with seizures and deteriorating mental status.Brain magnetic resonance imaging revealed necrotic lesions in bilateral thalami and pons.Methylprednisolone,immunoglobulin,and interleukin 6 inhibitors were administered.Her consciousness level was improved at discharge.Nineteen cases of 2019 coronavirus disease-related ANE have been reported,of which 22.2%of patients died and 61.1%had neurologic disabilities.RANBP2 gene mutation was found in five patients,two of whom developed recurrent ITES.Conclusion:Patients with RANBP2 mutations are at risk for recurrent ITES,may develop ANE,and have a poor prognosis after relapse.展开更多
文摘<b>Objective:</b> A case-control study of Influenza-Associated Necrotizing Encephalopathy (IANE) in children was conducted to explore the risk factors for the diagnosis of IANE, and to provide a predictive reference for the diagnosis of IANE. <b>Methods:</b> The children with IANE who received treatment in our hospital from January 2016 to December 2020 were selected as the study group, and the children with Influenza-Associated Encephalopathy (IAE) group who received treatment in the same period were selected as the control group. The blood biochemical, coagulation function and cerebrospinal fluid test results of the two groups were analyzed by univariate analysis. Receiver Operating Characteristic curve (ROC) analysis was used to determine the optimal threshold point of each index for the indicators with statistically significant differences in univariate analysis results, and multivariate Logistic stepwise regression analysis was performed according to the optimal threshold points. <b>Results:</b> In the IANE group, there were 32 children, including 20 males and 12 females, aged 60 (35, 84) months. There were 40 children in IAE group, including 26 males and 14 females, aged 58 (23, 97) months. Univariate results showed that serum Lactate Dehydrogenase (LDH), Cerebrospinal Fluid Lactate Dehydrogenase (CSF LDH) and Cerebrospinal Fluid Protein (CSF PRO) in the IANE group were significantly higher than those in the IAE group, and the difference between the two groups was statistically significant (P < 0.001). The optimal threshold points of blood LDH, CSF LDH and CSF PRO by ROC curve analysis were 535 U/L, 67 U/L and 0.49 g/L, respectively. Further Multivariate Logistic stepwise regression analysis showed that LDH > 535 U/L (OR = 31.264, 95% CI: 5.892 - 165.878, P < 0.001) and CSF PRO > 0.49 g/L (OR = 7.695, 95% CI: 1.052 - 56.305, P = 0.044) were independent risk factors for IANE. <b>Conclusion:</b> For children with influenza whose neurological symptoms appear rapidly and persist in the early stages of the disease, blood LDH > 535 U/L and CSF PRO > 0.49 g/L are independent risk factors for IANE.
基金This study was supported by Capital’s Funds for Health Improvement and Research of Beijing Children’s Hospital,Capital Medical University(No.2020-2-2094)the CAMS Innovation Fund for Medical Sciences(No.2019-I2M-5-026)We would like to thank all the families and individuals who participated in this study,and appreciate all the staffs in the PICU from the four centers.
文摘Importance Acute necrotizing encephalopathy(ANE)is a rare disease with high mortality.Plasma exchange(PLEX)has recently been reported to treat ANE of childhood(ANEC),but its efficacy is uncertain.Objective This study aimed to investigate the effectiveness of PLEX on ANEC.Methods A retrospective study was conducted in four pediatric intensive care units from December 2014 to December 2020.All patients who were diagnosed with ANEC were included;however,these patients were excluded if their length of stay was less than 24 h.Participants were classified into PLEX and non-PLEX groups.Results Twenty-nine patients with ANEC were identified,10 in the PLEX group and 19 in the non-PLEX group.In the PLEX group,C-reactive protein,procalcitonin,alanine aminotransferase,and aspartate aminotransaminase levels were significantly lower after 3 days of treatment than before treatment(13.1 vs.8.0,P=0.043;9.8 vs.1.5,P=0.028;133.4 vs.31.9,P=0.028;282.4 vs.50.5,P=0.046,respectively).Nine patients(31.0%,9/29)died at discharge,and a significantly difference was found between the PLEX group and non-PLEX group[0 vs.47.4%(9/19),P=0.011].The median follow-up period was 27 months,and three patients were lost to follow-up.Thirteen patients(50.0%,13/26)died at the last follow-up,comprising three(33.3%,3/9)in the PLEX group and ten(58.8%,10/17)in the non-PLEX group,but there was no significant difference between the two groups(P=0.411).Three patients(10.3%,3/29)fully recovered.Interpretation PLEX may reduce serum C-reactive protein and procalcitonin levels and improve liver function in the short term.PLEX may improve the prognosis of ANEC,and further studies are needed.
文摘Introduction:Acute necrotizing encephalopathy(ANE),a fatal subtype of infection-triggered encephalopathy syndrome(ITES),can be triggered by many systemic infections.RANBP2 gene mutations were associated with recurrent ANE.Case presentation:Here we report a 1-year-old girl with recurrent ITES and RANBP2 mutation.She was diagnosed with influenza-associated encephalopathy and made a full recovery on the first episode.After severe acute respiratory syndrome coronavirus 2 infection,the patient presented with seizures and deteriorating mental status.Brain magnetic resonance imaging revealed necrotic lesions in bilateral thalami and pons.Methylprednisolone,immunoglobulin,and interleukin 6 inhibitors were administered.Her consciousness level was improved at discharge.Nineteen cases of 2019 coronavirus disease-related ANE have been reported,of which 22.2%of patients died and 61.1%had neurologic disabilities.RANBP2 gene mutation was found in five patients,two of whom developed recurrent ITES.Conclusion:Patients with RANBP2 mutations are at risk for recurrent ITES,may develop ANE,and have a poor prognosis after relapse.
