Objective:To validate and compare the value of the Status Epilepticus in Pediatric Severity Score (STEPSS) versus PEDSS in assessing the short-term prognosis of children with status epilepticus (SE).Methods:Clinical data of 152 children with SE hospitalized at the First Affiliated Hospital of Zhengzhou University from January 2020 to December 2022 were retrospectively analyzed.According to the STEPSS and PEDSS scores, children with SE were scored and their prognosis was predicted.Receiver operating characteristic (ROC) curves of the 2 scales in assessing the short-term prognosis of SE in children were plotted, and the area under the curve (AUC), optimal cut-off, sensitivity and specificity were calculated, thus validating and comparing the value of the STEPSS versus PEDSS in assessing the short-term prognosis of children with SE.Results:Of the 152 children with SE, 90 were male and 62 were female, with the age of (5.8±3.9) years (1 month to 15 years). There were 112 cases with good prognosis and 40 cases with poor prognosis, involving 13 deaths.The AUC of STEPSS and PEDSS scores in predicting the death in children with SE were 0.908(95% CI: 0.848-0.967) and 0.887(95% CI: 0.831-0.942), respectively, both with the optimal cut-off value of 4.The sensitivity of STEPSS and PEDSS scores in predicting the death in children with SE were 0.740 and 0.846, respectively, and the specificity were 0.745 and 0.835, respectively.There was no significant difference in predicting the death in children with SE between the 2 scales ( P>0.05). In predicting adverse outcomes, the AUC of the STEPSS and PEDSS scores were 0.869(95% CI: 0.800-0.937) and 0.926(95% CI: 0.873-0.979), respectively, both with the optimal cut-off value of 3.The sensitivity of STEPSS and PEDSS scores in predicting adverse outcomes in children with SE were 0.827 and 0.900, respectively, and the specificity were 0.732 and 0.866, respectively.There was significant difference in predicting the adverse outcomes in children with SE between the 2 scales ( P<0.05). Conclusions:Compared with the STEPSS, the PEDSS has a higher application in predicting the short-term treatment outcome of children with SE, which can be used as a routine method to assess the prognosis of children with SE.

Chloromethyl ether and diclomethyl ether are statutory substances that cause occupational lung cancer. From 2021 to 2022, the Department of Occupational Diseases of the Eighth People's Hospital of Hebei Province successively received 4 cases of lung cancer from a chemical company that required occupational disease diagnosis. All four patients had a clear occupational history of chloromethyl ether and diclomethyl ether for more than 1 year, diagnosis of primary small cell lung cancer supported by relevant histopathology, immunohistochemistry, and tumor markers. All the 4 patients were diagnosed as occupational lung cancer (chloromethyl ether, diclomethyl ether) .

Chloromethyl ether and diclomethyl ether are statutory substances that cause occupational lung cancer. From 2021 to 2022, the Department of Occupational Diseases of the Eighth People's Hospital of Hebei Province successively received 4 cases of lung cancer from a chemical company that required occupational disease diagnosis. All four patients had a clear occupational history of chloromethyl ether and diclomethyl ether for more than 1 year, diagnosis of primary small cell lung cancer supported by relevant histopathology, immunohistochemistry, and tumor markers. All the 4 patients were diagnosed as occupational lung cancer (chloromethyl ether, diclomethyl ether) .

Febrile infection-related epilepsy syndrome (FIRES) is primarily characterized by frequent focal epileptic seizures followed by generalized seizures and status epilepticus.Multiple anti-seizure drugs are ineffective in its treatment, and there′s a high mortality rate during the acute phase.Survivors often suffer from drug-resistant epilepsy and neurological dysfunctions.Anakinra is an interleukin-1 receptor antagonist.Recent studies have shown that it has potential therapeutic effects on FIRES.This article analyzes the mechanism, efficacy, safety, and future challenges of Anakinra in the treatment of FIRES, aiming to provide a basis for its application in FIRES.

Objective:To explore the clinical characteristics and genetic etiology for a Chinese pedigree affected with Dyschromatosis symmetrica hereditaria (DSH) in conjunct with developmental delay.Methods:A child who had presented at the First Affiliated Hospital of Zhengzhou University on May 28 2021 for abnormal skin pigmentation of the extremities and growth retardation for over 2 years was selected as the study subject. Clinical data of the child and his pedigree (11 individuals from three generations) was collected. The child was subjected to whole exome sequencing, and candidate variant was verified by Sanger sequencing.Results:The child, a two-year-and-seven-month-old male, had hyper- and hypopigmentation on his hands, feet and face, in addition with delayed development. All members of his pedigree had typical presentation of DSH. A heterozygous c. 2657G>A variant was found in exon 8 of the ADAR gene in the child, his mother, and elder sister. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the variant was predicted as likely pathogenic (PM1+ PM2_Supporting+ PP1+ PP3). Conclusion:The c. 2657G>A variant of the ADAR gene probably underlay the DSH in this pedigree.

Objective:To explore the classification and treatment strategy of femoral neck fracture combined with ipsilateral intertrochanteric fracture.Methods:A retrospective analysis was performed of the 44 patients who had been admitted to Department of Orthopedics, Liangxiang Teaching Hospital, Capital Medical University from March 2003 to March 2019 for femoral neck fracture combined with ipsilateral intertrochanteric fracture. They were 19 males and 25 females, aged from 37 to 93 years (average, 77.9 years). According to the anatomical location and displacement severity, the femoral neck fractures were divided into 3 types while the intertrochanteric fractures were classified as stable or unstable ones. There were 3 cases of type Ⅰ which were completely extracapsular ones, 31 cases of type Ⅱ which were intracapsular stable ones, and 10 cases of type Ⅲ which were intracapsular unstable ones. Types Ⅰ and Ⅱ fractures were treated with intramedullary fixation, and type Ⅲ fractures with cemented hip hemi-replacement+reduction and fixation of the intertrochanteric fracture with Kirschner wires and steel cables. Recorded were fracture healing time, function of the affected hip and complications.Results:The 44 patients were followed up for at least 2 years. The fracture healing time for the 3 patients with type I fracture averaged 5.6 months (from 4.4 to 6.8 months); their hip function at the last follow-up, evaluated by the Harris scoring system, was excellent in 2 cases and good in one case. For 30 of the 31 patients with type Ⅱ fracture, the fracture healing time averaged 7.2 months (from 5.1 to 9.3 months); their hip function at the last follow-up, evaluated by the Harris scoring system, was excellent in 18 cases, good in 6 cases, fair in 5 cases, and poor in 2 cases, giving an excellent and good rate of 77.4% (24/31). As for complications, withdrawal or cutting-out of the head screw happened in 6 cases, infection in one case and nonunion in one case. In 10 patients with type Ⅲ fracture, the hip function at the last follow-up was excellent in 7 cases, good in 2 cases and poor in one case.Conclusions:For type Ⅰ and type Ⅱ femoral neck fractures combined with ipsilateral intertrochanteric fracture, intramedullary fixation with angulation stability may be a proper choice. For type Ⅲ ones, hip joint replacement should be the first choice.

Objective To explore the safety and feasibility of laparoscopic hepatectomy combined with radiofrequency ablation for primary liver cancer difficult to manage.Methods A retrospective study was conducted to analyze the clinical data of 16 patients who underwent laparoscopic hepatectomy combined with radiofrequency ablation in the Zhejiang Provincial People's Hospital from Apr 2015 to Dec 2017.Results 2 more tumors were found by intraoperative laparoscopic ultrasound.All patients underwent laparoscopic hepatectomy combined with radiofrequency ablation successfully.There were 2 laparoscopic left hepatectomy combined with radiofrequency ablation,2 laparoscopic right hepatectomy combined with radiofrequency ablation,6 laparoscopic left lateral lobectomy combined with radiofrequency ablation,4 laparoscopic right postrior lobectomy combined with radiofrequency ablation and 2 irregular laparoscopic hepatectomy combined with radiofrequency ablation.The intraoperative blood loss ranged from 100-800 ml.The average operative time was (283 ± 112) min.The length of postoperative hospital stay ranged from 5 to 12 days.The tumor-free survival rate after operation is 100％ (16/16) on 6 to 38 months follow up.Conclusion It is safe and feasible to carry out laparoscopic hepatectomy combined with radiofrequency ablation in difficult to manage primary liver cancer.

Objective To investigate the role of neuropeptide trefoil factor 3 (TFF3) in cocaine reward responses and the underlying mechanism.Methods Fifty SD rats were divided into 6 groups including Control group,Cocaine group,Cocaine + TFF3 (0.01 mg/kg) group,cocaine + TFF3 (0.1 mg/kg) group,cocaine + TFF3 (0.5 mg/kg) group and TFF3 (0.1 mg/kg) group,ip,n=7 ～ 9),and were treated with TFF3 / saline (ip),30 min later,rats were injected with cocaine (10 mg/kg ip) followed by 1 h hypedocomotion test.Immediately after behavioral test,rats (n=4～6) were decapitated and tissues of nucleus accumbens (NAc) were isolated and prepared for neurotransmitters analysis by HPLC; Another twenty one rats were divided into control and TFF3-treatment group,and the rats were trained with a modified 2-day cocaine CPP conditioning procedure.During cocaine CPP conditioning process,saline or TFF3 (0.1 mg/kg ip) was injected 30 min prior to cocaine injection (5 mg/kg ip).Results Systemic administration of TFF3 (0.1 mg/kg,ip) significantly increased the cocaine-induced locomotor activity (Total distance in 1 hour were (180±41) cm,(359±53) cm,(590±75) cm,(153±27) cm for Control,Vehicle + Cocaine,TFF3+Cocaine and TFF3+Vehicle groups respectively) and augmented cocaine rewarding effects in CPP(Post-training CPP score were (98± 18) s,(187±24) s for Vehicle + Cocaine,TFF3+Cocaine groups respectively).TFF3 (0.1 mg/kg ip) administration increased the dopamine concentration in the NAc induced by cocaine injection ((0.65±0.1) ng/ml,(1.24±0.14) ng/ml,(1.75±0.23) ng/ml,(0.74±0.21) ng/ml for Control,Cocaine,TFF3 + Cocaine and TFF3 + Vehicle groups respectively).Conclusion TFF3 is involved in regulation of behavioral response to cocaine,which is associated with the increasing of dopamine in the NAc.

The aim of the study is to obtain an efficient expression of recombinant ubiquitin-like specific protease 1 (Ulp1) by gene engineering. We cloned the Ulp1p, active fragment (403 aa-621 aa) of Ulp1, from Saccharomyces cerevisia, and subcloned into pGEX/Rosetta (DE3) to form an expression plasmid, pGEX-Ulp1p-His6. In order to enhance the solubility of GST-Ulp1p-His6, we purified the fusion protein GST-Ulp1p-His6 by either glutathione S-transferase agarose or Ni-NTA resin chromatography, the purity was up to 98%. We utilized the protein to cleave the SUMO fusions, and the specific activity of GST-Ulp1p-His6 was 1.375 x 10(4) U/mg. This study showed that the recombinant protein GST-Ulp1p-His6 displayed high specificity and activity.
Cloning, Molecular ; Cysteine Endopeptidases ; biosynthesis ; genetics ; Escherichia coli ; genetics ; metabolism ; Fungal Proteins ; biosynthesis ; genetics ; Glutathione Transferase ; biosynthesis ; genetics ; Recombinant Fusion Proteins ; biosynthesis ; genetics ; Saccharomyces cerevisiae ; enzymology ; Solubility

Objective To clone the full length staphylococcal protein A(SPA) gene from Staphylococcus aureus (ATCC6538), and subsequently study the gene structure and antibody binding ability.Methods The full length and the functional region of the SPA gene were cloned into pHisSUMO vector respectively, and expressed in E. coli. The full length and the functional fragment of the SPA protein were detected for antibody binding ability and stability. The functional fragment of the SPA protein fused with SUMO was coupled to the CNBr-activated agarose for antibody purification from rabbit serum. Results A variant of the full length SPA gene was cloned, which has been submitted to GenBank (the accession number is EU695225). Two fusion proteins had the same antibody binding ability as the untagged SPA protein. However, the formers was more stable than the latter at the tested conditions. SUMO-SPA conjugated-agarose kept high efficiency for antibody binding. Conclusion To our knowledge, the full length SPA gene of S.aureus(ATCC6538) is a novel variant. The SUMO tag can improve the stability of the functional region of the SPA protein without damaging the antibody binding ability. This fusion protein has been used for antibody purification successfully.