Moyamoya disease is a chronic progressive cerebrovascular disease. It got its nickname, the moyamoya disease, because the image of abnormal blood vascular net at the skull base in the encephaloangiography of such patients is very similar to smog. The moyamoya disease can be divided into two types: ischemic and hemorrhagic according to its clinical manifestations and imaging characteristics. In the ischemic type of moyamoya disease, when the blood vascular lumens are not narrow enough to completely block the blood flow, the chief manifestation is the cerebral blood circulation insufficiency; when the salvage is not in time, the disease will further progress to develop vascular obstruction or thrombosis, resulting in cerebral infarction, finally hemiplegia, aphasia, etc. irreversible symptoms and signs occur. In the primary hospital, the first choice for treatment of acute cerebral infarction (ACI) is drug thrombolysis. However, the main treatment for moyamoya disease with ACI is chiefly revascularization. This article reported a patient with moyamoya disease and ACI successfully treated by intravenous reteplase for thrombolysis in People's Hospital of Pucheng County.

Objective To explore the value of energy spectrum CT in the diagnosis of breast cancer.Methods Thirty-two hospitalized patients whose American Colledge of Radiology (ACR) breast imaging reporting and data system (BI-RADS) scores were 4-5 by mammography received non-enhanced spectral CT scans.The spectrum images,spectrum curve,lesion's size,morphology were observed,and also the pectoralis major muscle and axillary lymph node metastasis were evaluated and compared with mammography.Results Thirty-two patients were confirmed by pathology,including 16 cases of invasive duct carcinomas,1 case of medullary carcinoma,15 cases of lobular carcinomas,and 11 cases of the pectoralis major muscle invaded,9 cases of the axillary lymph nodes metastasis.ACR BI-RADS scores 4 were 23 cases,5 were 9 cases.Axillary lymph node metastasis and primary tumor spectrum curves were basically the same.Energy spectrum CT showed the lesion's shape,size,the relationship with the pectoralis major muscle and axillary lymph node metastasis.In 40-70 keV spectrum curve breast cancer displayed a downward trend.There were no significant differences between energy spectrum CT and mammography for the lesion's shape,edge,internal calcification and thickening of adjacent skin (P ＞ 0.05).While energy spectrum CT exhibited obvious advantages in demonstrating the pectoralis major muscle invaded and axillary lymph node metastasis (P ＜ 0.05).Conclusion Energy spectrum CT imaging displays greater clinical value for diagnosing breast cancer,and it can provide multi-parameter image for supporting clinical practice.

ObjectiveToinvestigatetheeffectofserumuricacid(SUA)levelonshort-term outcomes of recombinant tissue plasminogen activator (rtPA) for intravenous thrombolysis in patients w ith ischemic stroke. Methods The patients w ith acute ischemic stroke treated w ith intravenous rtPA thrombolysis w ere enrol ed. The demographic data, clinical data, and laboratory parameters w ere compared and analyzed according to the modified Rankin scale (mRS) scores at discharge. A good outcome was defined as a 3-month mRS score of 0 in patients w ith a baseline National Institute of Health Stroke Scale (NIHSS) score≤7, a score of 0–1 in those w ith a baseline NIHSS score of 8-14, and a score of 0–2 in those w ith a baseline NIHSS score ≥ 15. Results A total of 108 patients w ith acute ischemic stroke treated w ith intravenous rtPA thrombolysis w ere enrol ed. There w ere 66 patients (61.11%) in the good outcome group and 42 (38.89%) in the poor outcome group. The constituent ratios of age (62.21 ±10.25 years vs. 57.83 ±10.457 years; t=2.138, P=0.035), the baseline NIHSS scores (median and interquartile range, 10 [8-12] vs.4 [3-7]; Z=5.537, P<0.001), type 2 diabetes mel itus (40.48%vs.12.12%; χ2 =11.600, P=0.001), and previous history of stroke (9.52%vs.9.09%;χ2 =4.366, P=0.037) of the poor outcome group w ere significantly higher than those of the good outcome group, w hile the SUA level (323.119 ±87.869 mmol/L vs.385.961 ±76.166 mmol/L; t=3.936, P<0.001) w as significantly low er than that of the good outcome group. Multivariate logistic regression analysis show ed that the previous history of diabetes melitus type 2 (odds ratio [OR] 5.471, 95%confidence interval [CI] 1.472-20.334;P=0.011) and higher baseline NIHSS score (OR 1.306, 95%CI 1.147-1.486; P<0.001) were the independent risk factor for short-term clinical outcomes, w hile higher SUV level ( OR 0.992, 95%CI 0.986-0.998; P=0.015) w as an independent protective factor for poor short-term outcome. Conclusions The increased SUA level is an independent protective factor for good short-term outcome in patients treated w ith intravenous rtPA.

Matrix metalloproteinases (MMPs) are endocellular proteolytic enzymes. They are so named because they need Ca2+, Zn2+ and other metal ions as their cofactors. MMPs play an important biological role in regulating the formation, remodeling and degradation of extracellular matrix and participate in various physiological and pathological processes of cells. Bone marrow-derived mesenchymal stem cells (BMSCs) are a kind of pluripotent stem cell which has the ability to self-renew and differentiate into functional cells. Meanwhile, they can respond to the damage signals and migrate to injured site for tissue repair and regeneration. MMPs and their inhibitors TIMPs affect the differentiation and migration of BMSCs. This article reviews the roles of MMPs in differentiation and migration of BMSCs.
Bone Marrow Cells ; cytology ; Cell Differentiation ; physiology ; Cell Movement ; physiology ; Humans ; Matrix Metalloproteinases ; physiology ; Mesenchymal Stromal Cells ; cytology

Aim To investigate the effect of oxymatrine on lipid metabolism regulated genes in liver in fat-in-duced insulin resistance in ApoE -/- mice.Methods Seventeen C57BL/6J male mice were selected in normal control group.Sixty-eight ApoE -/- mice with high fat diet for 1 6 weeks,were randomly divided into model group,oxymatrine low,middle and high dose groups.Then they were gavaged for 8 weeks.Body weight and general biochemical indicators were deter-mined in mice.The mRNA and protein expression lev-els of LPL,FAT/CD36,CPT1 ,UCP2,SREBP-1 c,FAS and ACC were examined by real-time PCR and West-ern blot in the liver.Results Compared with model group,oxymatrine reduced body weight(BW),fasting
blood glucose (FBG),cholesterol (TC ),triglyceride (TG),free fatty acids(FFA),fasting plasma insulin (FINS)and insulin resistance index(HOMA-IR)(P <0.05),while improved glucose infusion rate (GIR). Oxymatrine down-regulated the mRNA and protein ex-pression of LPL,FAT/CD36,UCP2,SREBP-1 c,FAS and ACC(P <0.05),and up-regulated the mRNA and protein expression of CPT1 in varying degrees (P <0.05).Conclusion Oxymatrine can regulate the ex-pression of lipid metabolism regulated genes in liver and improve insulin resistance in ApoE -/- mice in-duced by high fat diet.

Objective To prepare a kind of Gelsolin-targeted ultrasound contrast agent (GSN-PLGA) and to explore its targeting and imaging effection in vitro.Methods The high molecular PLGA-COOH ultrasound contrast agents were prepared by a modified double emulsion technique and then conjugated with Gelsolin monoclonal antibody by carbodiimide technique.The physical property of contrast agent was determined.And the connectivity condition of ultrasound contrast agent with Gelsolin monoclonal antibody was estimated.The targeting ability and the effect of enhancing ultrasound imaging in vitro were explored.Results The average diameter of GSN-PLGA was (575.67 ± 4.71) nm.The potential was (-11.46±1.19) mV.And the binding rate of Gelsolin monoclonal antibody was 96.93％.In vitro experimentshowed more GSN-PLGA could be intaked by Hca-F cells and the ultrasound imaging cloud be enhanced greatly.Conclusion The GSN-PLGA nanoparticle can bind to Hca-F cells specifically and can enhance the ultrasound imaging greatly.

Objective To investigate the effects of Gli2 on the proliferation,growth,migration,and invasion of oral cancer cells(Tca8113)at the cellular level,and to clarify the molecular mechanism of how Gli2 regulation affects the migration and invasion of oral cancer cells.Methods Small interfering(si)RNA was used to inhibit Gli2 expression in Tca8113 cells.The effects of Gli2 on the proliferation,growth,migration,and invasion of Tca8113 cells were examined by CCK-8,platb cloning,and transwell chamber assay.Further qRT-PCR and Western blot assays were used to explore the mechanism of how Gli2 regulation effects the malignant proliferation and metastasis of Tca8113 cells.Results The mRNA and protein expression of Gli2 in oral cancer cells(Tca8113)increased.Interference of Gli2 expression inhibited the proliferation,growth,migration,and invasion of Tca8113 cells.Further experiments showed that interfering with Gli2 expression inhibited the mRNA and protein expression of key factors in the Hedgehog(Hh)pathway.In addition,interference of Gli2 expression significantly affected the mRNA and protein expression of key factors in epithelial mesenchymal transformation(EMT)pathways.Conclusions Gli2 is abnormally activated during oral cancer,and interference of Gli2 expression significantly inhibits the proliferation,growth,migration,and invasion of oral cancer cells.Gli2 influences the migration and invasion of oral cancer cells by regulating the Hh and EMT pathways.This study has provided a new way to elucidate the pathogenesis of oral cancer and new perspectives on the clinical treatment of oral cancer.

OBJECTIVETo summarize the clinical features of those Duchenne and Becker muscular dystrophy (DMD and BMD) patients who are complicated with epilepsy, and try to analyze the genotype- phenotype correlation.

METHODBy a retrospective analysis of 307 patients with DMD and BMD who attended Peking University First Hospital from February 2006 to September 2014,7 patients complicated with epilepsy were identified and their clinical data were collected. The possible mechanism of epilepsy in DMD and BMD patients was proposed after analyzing the genotype-phenotype correlation.

RESULT(1) Among 307 DMD and BMD patients, 7 cases had epilepsy, the prevalence was 2. 28%. (2) The age of onset of epilepsy ranged from 8 months to 11 years. Focal seizure was the most common seizure type (6 cases) , while other seizure types were also involved, such as generalized tonic-clonic seizure. As to epilepsy syndromes, 1 boy was diagnosed as benign childhood epilepsy with centrotemporal spikes (BECT). Six patients were treated with 1 or 2 types of antiepileptic drugs and seizures were controlled well. On follow-up, 6 of the 7 children had normal mental development, while the remaining 1 patient was diagnosed as mild mental retardation. (3) DMD gene mutations of all 7 patients were analyzed. Exons deletions were found in 6 cases while point mutation was found in 1 case.

CONCLUSIONThe prevalence of epilepsy in DMD and BMD patients was higher than the prevalence in normal population. The age of onset of epilepsy varies, and focal seizure may be the most common seizure type. Some patients may also present as some kind of epilepsy syndrome, such as BECT. In most patients, seizures can be controlled well by 1 or 2 types of antiepiletic drugs. No clear correlation was found between genotype and phenotype in DMD and BMD patients who were complicated with epilepsy, probably due to limited number of cases.

Anticonvulsants ; therapeutic use ; Child ; Epilepsy ; complications ; drug therapy ; epidemiology ; Exons ; Genotype ; Humans ; Intellectual Disability ; etiology ; Male ; Muscular Dystrophy, Duchenne ; complications ; genetics ; Mutation ; Phenotype ; Prevalence ; Retrospective Studies ; Seizures ; Sequence Deletion

Objective To summarize the clinical features of 22 probands diagnosed with congenital muscular dystrophy (CMD),and to provide genetic counseling and prenatal diagnosis for 23 fetuses of these pedigrees.Methods Data of 22 CMD patients who were treated in the Pediatric Department of Peking University First Hospital during October 2006 to March 2016 were analyzed.Informed written consents for participation in this study were obtained from the parents or guardians.Prenatal diagnosis was performed using DNA samples extracted from fetal villus cells of 12 cases at 11-13 gestational weeks and amniotic fluid of 11 cases at 18-22 gestational weeks.Direct DNA sequencing by polymerase chain reaction (PCR) and multiplex ligation-dependent probe amplification (MLPA) were used to detect CMD-related gene mutations.Linkage analysis of short tandem repeats (STRs) was used to identify maternal blood contamination and biological parents.Results Thirteen out of the 22 probands with CMD were diagnosed with congenital muscular dystrophy type 1 A (MDC1A),and all of them carried compound heterozygous mutations in LAMA2 gene.Prenatal diagnosis of 13 fetuses from these pedigrees found that four fetuses were wild-type,seven were heterozygotes and two carried the same mutations as their proband.Three probands with LMNA-related congenital muscular dystrophy (L-CMD) carried de novo mutations in LMNA gene.In these pedigrees,two fetuses were wild-type and one whose mother was mosaicism carried the same mutations as the proband.One proband with Ullrich congenital muscular dystrophy carried compound heterozygous mutations in COL6A2 gene and the fetus of the same pedigree was wild-type.Five probands were diagnosed with α-dystroglycanopathies.And among them,two cases of muscle-eye-brain disease (MEB) carried compound heterozygous mutations in POMGnT1 gene and the fetuses of the two peidgrees were heterozygotes;one case of congenital muscular dystrophy type 1C (MDC1C) had compound heterozygous mutations in FKRP gene and the fetus carried the same mutations;one patient diagnosed with POMGnT1-related congenital muscular dystrophy with mental retardation (CMD-MR) carried compound heterozygous mutations in POMGnT1 gene,and the fetus was positive for the same mutations;one proband with POMT1-related CMD-MR was positive for compound heterozygous mutations in POMT1 gene and the results of prenatal diagnosis for two fetuses of this pedigree showed that the first fetus had the same mutations as the proband,while the second was heterozygote.Conclusions No effective therapeutic method is available for CMD.Therefore,accurate genetic counseling and prenatal diagnosis are necessary to prevent CMD child from birth.

Objective:To overcome the disadvantages of bismuth removal by bismuth sulfide precipitation method recommended by existing analytical standards and improve the quality of analytical result.Methods:Based on 201×7 anion exchange resin, the experimental process of bismuth removal was designed, and verified by using spiked samples and IAEA test samples.Results:Bismuth was removed by anion exchange resin. In the removal experiments of strontium, yttrium and bismuth, the chemical recovery rate of strontium and yttrium could reach (98.6 ± 0.8)% and (98.5 ± 0.7)%, respectively, with no Bi 2S 3 precipitation found. The relative standard deviation between analytical result and theoretical values was -2.97% to 5.94%, better than 3.96%-17.8% by the standard bismuth removal method. Through validation using IAEA test samples, the relative standard deviation between the reported value and the target value for 90Sr was between 3.40%-7.09%, and all the results were acceptable. Conclusions:Bismuth could be quantitatively removed using anion exchange resin without adsorption of strontium and yttrium. In addition, the bismuth removal solution system of anion exchange resin was the same as the elution system in 90Sr analysis, and the purpose of rapid bismuth removal could be achieved without conversion system. Compared with the current standard analytical method, it was feasible and better to quantitatively remove bismuth based on anion exchange resin, which could meet the needs of routine analysis of 90Sr.