Objective To investigate the trend of cytokines in patients with hemophagocytic lymphohistiocytosis (HLH) and analyze its significance.Methods 16 patients with HLH from January 2011 to May 2013 were selected.The patients were divided into remission group and death group by prognosis.Serums of the two groups were collected when they were hospitalized and at 7 th,14 th,21st,28th and 42nd day during chemotherapy,and they were fractionated HLH 1-6 groups and HLH a-d groups again,then the levels of IL-18,IL-10,IL-12,NF-κB,TNF-α and neopterin were tested by enzyme linked immuno sorbent assay (ELISA) to analysis their trend.Results The levels of all of cytokines in the remission group declined with chemotherapy,the difference between HLH1 group and another HLH groups was statistically significant (P ＜ 0.05).In the death group,the levels of NF-κB,IL-12 and neopterin had no downward trend with chemotherapy,and the difference between HLHa group and another HLH groups was not statistically significant (all P ＞ 0.05).The level of TNF-α declined with chemotherapy,and the differences between HLHa group and HLHc group,HLHa group and HLHd group were statistically significant (P =0.049,0.000).The level of IL-10 declined sharply in the first week of chemotherapy,and the difference between HLHa group and HLHb group was statistically significant (P =0.00).The level of IL-18 declined after the 2nd-weeks' chemotherapy,and the differences between HLHa group and HLHb group,HLHa group and HLHc group were statistically significant (P =0.03,0.02).Conclusions In the remission patients,the levels of serum IL-18,IL-10,IL-12,NF-κB,TNF-α and neopterin declined after chemotherapy.In the death patients,the downward trend is not obvious.It was preliminarily confirmed that the prognosis of HLH is related to the trend of cytokines during chemotherapy.

Objective:To analyze the predictive value of serum Nesfatin-1 combined with the Status Epilepticus Severity Scale (STESS) score on the short-term prognosis of children with status epilepticus (SE).Methods:A clinical data of 145 children with SE who were admitted to the Children′s Hospital Affiliated to Zhengzhou University, Henan Children′s Hospital, Zhengzhou Children′s Hospital, from January 2016 to January 2020 were analyzed retrospectively.After admission, the serum levels of Nesfatin-1 and the STESS score were measured.According to the Glasgow Outcome Scale (GOS) score at discharge, children with SE were divided into poor prognosis group (<5 scores) and good prognosis group (5 scores). Univariate and multivariate Logisitc regression analyses were performed to analyze influence of the serum Nesfatin-1 level and STESS score on the short-term prognosis of children with SE.Receiver operating characteristic (ROC) curve was depicted to evaluate the predictive value of serum Nesfatin-1 level combined with STESS score in the short-term prognosis of children with SE. Results:Twenty-five cases out of 145 (17.24%) children with SE were discharged with a GOS score of <5 (poor prognosis group), 120 cases were in the good prognosis group.In the poor prognosis group, the overall attack (88.00% vs.66.67%), attack time of SE > 1 h (76.00% vs.27.50%), admission to child intensive care unit(PICU) (76.00% vs.37.50%), implementation of endotracheal intubation (16.00% vs.5.00%), abnormal electroencephalogram(EEG) results (73.91% vs.41.03%), abnormal proportion of head imaging results (82.61% vs.29.49%), serum Nesfatin-1 level[(3.65±1.45) μg/L vs.(2.20±0.77) μg/L] and STESS score[(3.01±0.75) points vs.(1.80±0.60) points] were significantly higher than those in the good prognosis group (all P<0.05). Logistic regression analysis showed that the attack time of SE > 1 h, admission to PICU, abnormal EEG, abnormal proportion of head imaging results, serum Nesfatin-1 level and STESS score were independent risk factors for the poor short-term prognosis of children with SE ( OR=4.217, 3.456, 2.626, 4.109, 3.040 and 2.012, respectively, all P<0.001). The cut-off value of serum Nesfatin-1 level and STESS score was 3.01 μg/L and 2.38 points, respectively.The Youden index and AUC of the combination of serum Nesfatin-1 level and STESS scores were 0.736 and 0.921 (95% CI: 0.861-0.959), respectively, which were better than those of single detection of either serum Nesfatin-1 level [Youden index 0.447; AUC 0.795(95% CI: 0.720-0.858)] or STESS scores [Youden index 0.562; AUC 0.859(95% CI: 0.792-0.911)]. Conclusions:The abnormal increases in serum Nesfatin-1 level and STESS score are risk factors for poor prognosis of SE in children, and their combination has a high predictive value for the poor short-term prognosis.

Mitochondrial and peroxisome fission deficiency-related encephalopathy caused by DNM1L gene mutation is a rare and fatal epileptic encephalopathy, with clinical phenotype and genetic heterogeneity. The acute stage is drug-resistant epilepsy with poor prognosis and serious neurological sequelae. A case of genetically confirmed encephalopathy related to mitochondrial and peroxisome fission defects is reported, the clinical data, treatment process are summarized, and the previous literature is reviewed to improve the understanding of the rare disease.