Serum phosphorylated neurofilament heavy-chain levels reflect phenotypic heterogeneity and are an independent predictor of survival in motor neuron disease

Journal of Neurology - To investigate the prognostic role and the major determinants of serum phosphorylated neurofilament heavy -chain (pNfH) concentration across a large cohort of motor neuron...     

Independent walking after neonatal arterial ischemic stroke and sinovenous thrombosis

Few studies have examined walking after neonatal arterial ischemic stroke and sinovenous thrombosis. We looked at the development of walking in a retrospective and consecutive cohort study of 88 term and near-term neonates. We used Kaplan-Meier survival curves and Cox proportional hazards models to assess (1) sex, (2) stroke type (arterial ischemic stroke or sinovenous thrombosis), (3) number of cerebral hemispheres with infarction, and (4) presence of neonatal comorbidity as predictors of the probability over time of starting to walk independently. These variables were assessed as predictors of parent-reported gait normality using the chi-square test on 2 x 2 contingency tables. Seventy-five of 83 survivors (90.4%, 95% confidence interval = 81.9-95.7) walked with a median time of first steps at 13 months of age (95% confidence interval = 12-14). Only bilateral strokes were associated with a lower probability over time of initiating independent walking (hazard ratio = 0.41, P = .04). Parents reported normal gait for 58 of 75 walkers (77.3%, 95% confidence interval = 67.8-86.8). No variables predicted parent-reported gait normality. Our findings suggest that most survivors of neonatal arterial ischemic stroke and sinovenous thrombosis walk with a gait that appears normal to parents, but bilateral infarctions decrease the probability over time of starting to walk independently.     

胶原海绵复合新生大鼠原代心肌细胞构建工程化心肌组织

目的:探索以胶原海绵为支架、新生大鼠原代心肌细胞为种子细胞,于体外构建工程化心肌组织的方法。方法:实验于2005-12/2006-11在解放军第四军医大学西京医院心内科实验室完成。Ⅰ型胶原海绵剪切成方形片状(2.0cm×1.4cm×0.2cm),经60Co照射消毒,于DMEM培养液中水化1h左右。另取1d龄SD大鼠心脏,剪成小碎块,然后用2.5g/L胰蛋白酶于37℃中消化,吸取上清至含胎牛血清的DMEM中,重复消化四五次,用差速贴壁法除去大部分成纤维细胞,将细胞沉淀用DMEM培养液以2×109L-1的密度悬浮备用。将上述的心肌细胞悬液1mL缓慢滴注于玻璃模型中的胶原海绵上,然后置于细胞培养中培养。肉眼及显微镜主要观察工程化心肌组织在培养期间的自发收缩情况,包括收缩的部位、强度、频率、一致性以及收缩随时间变化的情况。苏木精-伊红染色观察工程化心肌组织内胶原纤维的变化,细胞形态,胞核的形状及细胞之间的连接。免疫组织化学染色和透射电镜观察工程化心肌组织片的形态和功能。结果:①细胞接种于胶原海绵上1d后,细胞/胶原复合物的凝胶化过程基本完毕,体积保持恒定,维持至培养结束,第3天细胞/胶原复合物局部出现点片状自发收缩,第5天整个细胞/胶原复合物出现同步化自发收缩,收缩频率61～199次/min。2周后37.5%的工程化心肌组织的自发收缩活动减弱,但75%的工程化心肌组织的自发收缩活动持续至培养结束。②苏木精-伊红染色、免疫组织化学染色和透射电子显微镜显示,工程化心肌组织内细胞间连接广泛存在,细胞多呈纵向分布,胞核呈长圆形,胞浆内α-肌节肌动蛋白阳性,胞内肌原纤维排列整齐,可见到心肌特异性的肌小节结构和Z线,多数细胞具有分化的心肌细胞表型。结论:用新生大鼠原代心肌细胞为种子细胞、以Ⅰ型胶原海绵为支架材料,构建出的工程化心肌组织,于体外可长时间持续自发收缩,该细胞/胶原复合物的形态结构与生理功能均类似于成熟大鼠心肌组织。     

High expression levels of IKK alpha and IKK beta are necessary for the malignant properties of liver cancer

IKK-NF-kappa B signaling is regarded as an important factor in hepatocarcinogenesis and a potential target for liver cancer therapy.Therefore,in this study,we analyzed the expression of mRNAs encoding components and targets of NF-kappa B signaling including IKK alpha,IKK beta,RANK,RANKL,OPG,CyclinD3,mammary serine protease inhibitor(Maspin),CyclinD1,c-FLIP,Bcl-x1,Stat3,Cip1 and Cip2 by real-time PCR in 40 patients with liver cancer.After statistical analysis,7 indices including IKK alpha,IKK beta,RANK,Maspi...     

人工神经元网络用于复方氯丙嗪的含量测定

将误差反向传播(BP)的人工神经元网络(ANN)模型，用于复方氯丙嗪紫外重叠光谱分 析。对ANN模型的参数进行了优化。采用f(x)二1/(1+e^-x)作为网络节点的输入输出转换函数的三 层神经网络具有较佳性能，当取隐含节点数为15时，该网络预测结果的最小平均相对误差为1.22％， 将研制的ANN模型用于复方氯丙嗪含量测定，其结果与药品标准法和偏最小二乘法(PLs)一致。     

Clinical score of 62 Italian patients with Cornelia de Lange syndrome and correlations with the presence and type of NIPBL mutation

Cornelia de Lange syndrome (CdLS) is a rare multisystem disorder characterized by facial dysmorphisms, upper limb abnormalities, growth and cognitive retardation. About half of all patients with CdLS carry mutations in the NIPBL gene. The first Italian CdLS cohort involving 62 patients (including 4 related members) was screened for NIPBL mutations after a clinical evaluation using a quantitative score that integrates auxological, malformation and neurodevelopmental parameters. The patients were classified as having an overall 'severe', 'moderate' or 'mild' phenotype. NIPBL screening showed 26 mutations so classified: truncating (13), splice-site (8), missense (3), in-frame deletion (1) and regulatory (1). The truncating mutations were most frequently found in the patients with a high clinical score, whereas most of the splice-site and all missense mutations clustered in the low-medium score groups. The NIPBL-negative group included patients covering the entire clinical spectrum. The prevalence of a severe phenotype in the mutated group and a mild phenotype in the non-mutated group was statistically significant. In terms of the isolated clinical signs, the statistically significant differences between the mutation-positive and mutation-negative individuals were pre- and post-natal growth deficits, limb reduction, and delayed speech development. The proposed score seems to be a valuable means of prioritizing the patients with CdLS to undergo an NIPBL mutation test.     

Elevation of cerebrospinal fluid soluble CD27 levels in patients with meningeal localization of lymphoid malignancies [published erratum appears in Blood 1996 Oct 1;88(7):2818]

Diagnosis of meningeal localization of lymphoid malignancies by means of cytologic examination of the cerebrospinal fluid (CSF) can be difficult. Thus far no reliable CSF tumor markers have been identified. CD27 is a transmembrane disulfide-linked 55-kD homodimer present on most peripheral blood T cells and on a subset of B cells. CD27 is also expressed on human malignant B cells and high levels of soluble CD27 can be present in the serum of patients with B-cell malignancies. The aim of this study is to determine prospectively the diagnostic value of CSF sCD27 as a tumor marker in patients with meningeal localization of lymphoid malignancies. CSF sCD27 levels were determined by sandwich enzyme-linked immunosorbent assay. The optimal cut-off value using receiver operator characteristics curves was found to be 10 U/mL. sCD27 levels were normal in all 50 control patients (lumbar disc protrusion) and in 39 of 40 samples obtained from patients with either solid tumors or acute myeloid leukemia. Of 104 CSF samples from 70 children with acute lymphoblastic leukemia (ALL) or non-Hodgkin's lymphoma (NHL) undergoing routine central nervous system (CNS) staging, sCD27 was false positive and false negative in only one sample each. In 70 samples from 45 patients suspected of meningeal localization of ALL or NHL, the sCD27 test had an excellent sensitivity (100%) and specificity (82%). In 7 patients with positive CSF studied longitudinally, sCD27 levels correlated very well with remission and relapse. sCD27 levels were not nonspecifically increased by the administration of cytostatic drugs. Finally, sCD27 was also elevated in the 4 patients studied with primary central nervous system lymphoma (PCNSL). CSF sCD27 is a promising tumor marker in patients with either meningeal localization of lymphoid malignancies or PCNSL, and can be useful in the differential diagnosis of CNS involvement by either lymphoid malignancies or solid tumors.     

卡尔曼滤波滴定分析法:苯扎溴铵的测定

研究了卡尔曼滤波及平滑在滴定分析中的应用,该法对热力学平衡及非平衡(动力学)体系均能适应;特别是对非平衡滴定,当许多其它方法误差较大或无法使用时,卡尔曼滤波法仍可获得正确的结果。已用于苯扎溴铵的硝酸银电位滴定分析与快速测定。