Objective To improve the recognition for infantile spinal muscular atrophy (SMA-Ⅰ) and the level of early diagnosis,intervention and treatment for SMA-Ⅰ.Methods The clinical data of 39 patients with SMA-Ⅰ were analyzed retrospectively, including the clinical manifestations, neural electrophysiological characteristics, geno-type, diagnosis,treatment and prognosis of SMA-Ⅰ.Results Of the 39 cases with SMA-Ⅰ , 37 cases (94.9％) had onset in 6 months after birth.The paralyses of the limbs were symmetrical and flaccid.The lower was more severe than the upper , and the proximal was more severe than the distal, hypotonia and tendinous reflex disappears.Thirty-two cases (82.1％) had normal serum creatine kinase, and 7 cases (17.9％) increased slightly.Nerve electrophysiological examination showed that 169 (96.0％) had spontaneous potentials in 176 muscles.Of 160 limb muscles,35 (21.8％) released few motor unit potential (MUP) ,117 (73.1％) extended the duration of MUP and 104 (65.0％) increased the amplitude of M UP.Of 167 peripheral motor nerves, 160 (95.8％)decreased the amplitude of the compound muscle action potential and 162 (97.0％) had normal motor conduction velocity.Of 93 peripheral sensory nerves, 93 (100.0％) had normal range of the conduction.The gene detection showed that 38 cases had homozygous deletion of exon 7,8, and 1 case had homozygous deletion of exon 7 and heterozygous deletion of exon 8.In the effective follow-up of 30 cases,6 cases died in the 2-3 months after birth,4 cases died in 10 months after birth, 12 cases died in 12-18 months after birth.Six cases survived to 2 years old,2 cases survived to 3 years old,and all of them died of pulmonary infection.Conclusions There are typical clinical and nerve electrophysiological characteristics for SMA-I.Nerve electrophysiological examination can be used as an important method for diagnosis and differential diagnosis for SMA-I.Genetic testing can be used to identify the disease and make prenatal diagnosis.Through comprehensive intervention the quality of life can be improved in SMA-Ⅰ children.

Objective To study the preparation of nifedipine liposomes (NP) and evaluate their quality. Methods The liposomes were prepared by a pH steps degree-freeze thawing method, with the entrapment and stability as indices. The entrapment was studied by Sephadex G-50 minicolumn centrifugation UV-Vis spectroscopy method. Entrapment, and degree distribution before and after freezing were compared, the effect of freezing times on entrapment was studied. Results The entrapment and the average size of NP liposomes were 96.65% and 2.0 ?m, respectively. After freezing, both the entrapment and stability were increased. Conclusion The selected formulation and preparation of nifedipine liposomes are practicable.

Objective To study the clinical curative effect of Tongqiao Huoxue decoction combined with edaravone and hyperbaric oxygen for treatment of delayed encephalopathy after carbon monoxide poisoning (DEACMP). Methods A prospective study was conducted. Forty-six patients with DEACMP admitted into Yidu Central Hospital of Weifang Medical College from January 2012 to January 2014 were randomly divided into observation group (23 cases) and control group (23 cases). The basic treatments of two groups were identical. Based on the basic treatments including hyperbaric oxygen and citicoline sodium injection etc, the observation group was treated with our-self made Tongqiao Huoxue decoction which could be modified in accord to the individual differentiation of syndromes in traditional Chinese medicine (the composition of decoction included Paeoniae Radix Rubra 15 g, Chuanxiong Rhizoma 15 g, Persicae Semen 15 g, Carthami Flos 15 g, Zingiberis Rhizoma Recens 3 pieces, Jujubae Fructus 2 pieces, Moschus 0.5 g, Allium Fistulosum 1 segment). The decoction was administered orally or by nasogastric gavage, one dosage everyday for 1 month, and in the mean time, edaravone intravenously drip 30 mg was given to the observation group twice a day for 14 days. The control group was given hyperbaric oxygen and other conventional treatment for 30 days. The clinical therapeutic effect and adverse reaction were observed after treatment for 30 days. The changes of intelligent level were detected by Hasegawa dementia scale (HDS), and the changes of latency of P300 were measured by electromyologram/evoked potential instrument in two groups before and after treatment. Results The total effective rate in observation group was significantly higher than that in control group [91.3% (21/23) vs. 65.2% (15/23), P < 0.01]. Elevation of creatinine occurred in 1 case, moderate increase in alanine aminotransferase (ALT) appeared in 1 case, and both of them were reduced to normal after treatment in observation group; no adverse reaction occurred in control group. The HDS scores were significantly higher 30 days after treatment than those before treatment in the two groups [control group:13.4±2.8 vs. 6.8±2.3, observation group:20.8±3.4 vs. 6.6±2.5, both P<0.05]. The latency of P300 after treatment was significantly lower in two groups than that before treatment [control group (ms): 355.7±25.7 vs. 385.5±27.8, observation group (ms): 337.3±24.6 vs. 386.8±25.4, both P < 0.05], the change in observation group being more significant [the HDS score: 20.8±3.4 vs. 13.4±2.8, the latency of P300 (ms): 337.3±24.6 vs. 355.7±25.7, both P<0.05]. Conclusion Tongqiao Huoxue decoction combined with edaravone and hyperbaric oxygen has favorable cognitive effect on patients with DEACMP, thus, it can be used extensively in clinic.

Objective To investigate the effect of down-regulation of human lung adenocarcinoma metastasis-associated transcript 1 (MALAT1) on microglial activation and its possible mechanism.Methods BV2 microglial cells were cultured in vitro and divided into a normal control group (no treatment),a lipopolysaccharide (LPS) treatment group (cultured with 100 ng/mL LPS),a MALAT1 interference group (transfected with MALAT1 interference sequence + cultured with 100 ng/mL LPS) and a control sequence group (transfected with control sequence + cultured with 100 ng/mL LPS).Real-time fluorescence quantitative PCR was used to detect the expression ofMA LA T1 mRNA in the cells.Western blotting was used to detect the expression of NF-κB protein and IκB-α protein in the cells.Enzyme linked immunosorbent assay (ELISA) was used to detect the levels of IL-1β,IL-6,IL-10 and TNF-α in each group.Nitric acid reduction method (Griess method) was used to detect nitric oxide (NO) concentration in each group.Results The relative expression levels of MALA T1 mRNA and NF-κB protein were significantly increased,the relative expression level of IκB-α protein was significantly decreased,the levels ofIL-1β,IL-6,IL-10 and TNF-α and the release of NO in the cell supernatant were significantly increased in the LPS treatment,MALAT1 interference and control sequence groups than in the normal control group (P＜0.05).The relative expression levels of MALAT1 mRNA and NF-κB protein were significantly decreased,the expression level of IκB-α protein was significantly increased,the levels ofIL-1β,IL-6,IL-10 and TNF-α and the release of NO in the cell supematant were significantly decreased in the MALAT1 interference group than in the LPS treatment and control sequence groups (P＜0.05).Conclusions Down-regulation of MALA T1 gene expression may inhibit the inflammatory response induced by LPS-induced BV2 cell activation.The mechanism might be related to the inhibition of NF-κB signaling pathway.

Objective To study the clinical efficacy of transjugular intrahepatic portosystemic shunt (TIPS) combined with agitation thrombolysis in treatment of acute portal vein thrombosis (PVT) in patients with liver cirrhosis.Methods The clinical data of 37 cirrhotic patients with acute PVT treated from January 2014 to December 2017 at the First Affiliated Hospital of Henan University of Science and Technology was analyzed.There were 20 males and 17 females with age ranging from 29 to 71 years.The patients were divided into the combined group (n =15) and the anticoagulation group (n =22).The combined group was treated with TIPS combined with agitation thrombolysis anticoagulation.The anticoagulation group was treated with anticoagulation.The changes in liver function and hemodynamics of portal vein of the two group were compared.Follow-up studies included postoperative portal vein patency,bleeding and survival rates of patients.Results The postoperative portal vein pressure and maximum burden of PVT in the combined group were significantly lower than those before operation,and the portal vein maximum blood volume and flow velocity of portal vein were significantly higher than those before operation (all P ＜ 0.05).At 2 weeks,6 months and 12 months after operation,the maximum burden of PVT of the combined group was (13.9 ±5.4)％,(16.1 ±5.5)％ and (13.8 ±6.2)％,respectively,which was significantly lower than that of the anticoagulant group (84.1±31.3)％,(85.9±27.6)％ and (88.2±39.5)％ (all P＜0.05).At 2 weeks,6 months and 12 months after operation,the flow velocity of portal vein of the combined group was (21.6 ± 5.7) cm/s,(16.1 ± 6.3) crn/s and (17.6 ± 4.9) cm/s,respectively,which was significantly higher than that of the anticoagulation group (9.7 ± 4.6) cm/s,(8.1 ± 4.3) cm/s and (8.2 ± 3.5) cm/s (all P ＜ 0.05).After operation,3 patients in the combined group developed recurrence of portal vein thrombosis,while the remaining patients had smooth blood flow in the portal vein and shunt.In the anticoagulation group,only 3 patients had unobstructed portal vein blood flow.The cumulative rate of no gastrointes tinal bleeding in the combined group was significantly better than the anticoagulant group.The cumulative survival rate of the combined group was also significantly better than the anticoagulation group (P ＜ 0.05).Conclusion TIPS combined with agitation thrombolysis was more effective than the traditional anticoagulant therapy in treatment of acute portal vein thrombosis in cirrhotic patients.

BACKGROUND: Knee osteoarthritis (KOA) is a prevalent chronic joint disease caused by various factors. Mesenchymal stem cells (MSCs) therapy is an increasingly promising therapeutic option for osteoarthritis. However, the chronic inflammation of knee joint can severely impede the therapeutic effects of transplanted cells. Gelatin microspheres (GMs) are degradable biomaterial that have various porosities for cell adhesion and cell–cell interaction. Excellent elasticity and deformability of GMs make it an excellent injectable vehicle for cell delivery. METHODS: We created Wharton’s jelly derived mesenchymal stem cells (WJMSCs)-GMs complexes and assessed the effects of GMs on cell activity, proliferation and chondrogenesis. Then, WJMSCs loaded in GMs were transplanted in the joint of osteoarthritis mice. After four weeks, joint tissue was collected for histological analysis. Overexpressing-luciferase WJMSCs were performed to explore cell retention in mice. RESULTS: In vitro experiments demonstrated that WJMSCs loaded with GMs maintained cell viability and proliferative potential. Moreover, GMs enhanced the chondrogenesis differentiation of WJMSCs while alleviated cell hypertrophy. In KOA mice model, transplantation of WJMSCs-GMs complexes promoted cartilage regeneration and cartilage matrix formation, contributing to the treatment of KOA. Compared with other groups, in WJMSCs+GMs group, there were fewer cartilage defects and with a more integrated tibia structure. Tracking results of stable-overexpressing luciferase WJMSCs demonstrated that GMs significantly extended the retention time of WJMSCs in knee joint cavity. CONCLUSION Our results indicated that GMs facilitate WJMSCs mediated knee osteoarthritis healing in mice by promoting cartilage regeneration and prolonging cell retention. It might potentially provide an optimal strategy for the biomaterial-stem cell based therapy for knee osteoarthritis.

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.

【Objective】 To analyze the basic characteristics of whole blood donors from blood stations before and after the outbreak of COVID-19. 【Methods】 After excluding invalid data, data related to the basic characteristics of whole blood donors collected from 26 blood stations in China during 2018 to 2021 were statistically analyzed, including the trend of total whole blood donors, the number of repeated blood donors, the frequency of blood donation, the average age of donors and the recruitment of first-time blood donors. 【Results】 Affected by the epidemic, 8 out of 14 indicators were with large variations, accounting for 57%. The overall growth rate of total whole blood donors during the epidemic was higher than before the epidemic (P<0.05).The number of repeated blood donors has shown an increased trend, with a higher number during the epidemic than before (P<0.05). The frequency of blood donation was lower during the epidemic than before(P<0.05).Average ages of blood donors and female blood donors fluctuated widely during the epidemic, both higher than those before the epidemic(P<0.05).The donation rate of first-time blood donors <25 years old and ≥25 years old varied widely and irregularly during the epidemic (both P<0.05). The percentage of first-time blood donors fluctuated irregularly during the epidemic, with overall percentage lower than that before the epidemic(P<0.05). 【Conclusion】 Whole blood donors from 26 blood stations increased after the outbreak of COVID-19, and some indicators in certain areas showed significant fluctuations during the epidemic.

[摘 要] 目的：探究miR-218-5p靶向磷酸二酯酶7A（PDE7A）调节人非小细胞肺癌（NSCLC）A549细胞糖酵解过程的机制。方法：常规培养A549细胞，用Lipo3000将miR-218-5p mimic、mimic-NC、PDE7A过表达质粒（PDE7A-oe）和PDE7A对照质粒（PDE7A-NC）转染A549细胞，记为miR-218-5p mimic组、mimic-NC组、PDE7A-oe组和PDE7A-NC组。qPCR法验证转染效率，WB法检测糖酵解关键酶蛋白的表达，葡萄糖测定法和乳酸生成测定法检测各转染组A549细胞中2脱氧葡萄糖和乳酸含量，双萤光素酶报告基因实验验证miR-218-5p与PDE7A靶向结合关系，用TCGA数据库数据分析PDE7A mRNA在肺癌组织中的表达水平。结果：在A549细胞中成功地过表达了miR-218-5p（P<0.01）。过表达miR-218-5p均能显著抑制A549细胞中PDE7A、HK2、PKM2蛋白的表达（均P<0.01）、葡萄糖摄取量和乳酸生成量（均P<0.01）。过表达PDE7A均可显著促进A549细胞中PDE7A、HK2、PKM2蛋白的表达（均P<0.01），以及葡萄糖摄取量和乳酸生成量（均P<0.01）。A549细胞中miR-218-5p可与PDE7A mRNA的3´-UTR直接结合。数据库数据分析结果显示，PDE7A mRNA在肺鳞状细胞癌组织中呈高表达（P<0.01）。结论： miR-218-5p靶向PDE7A调控A549细胞中HK2和PKM2的表达水平，进而抑制糖酵解过程，miR-218-5p/PDE7A可能是NSCLC临床诊断和治疗的潜在靶点。