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 develop transparency measurement scale on medicine use information in primary health care facilities. Methods The dimensions and items of scale were determined through literature review and expert consultation. The scale was used to investigate 100 primary health care facilities in eastern, central and western regions. Correlation coefficient analysis, Cronbach's coefficient, and exploratory factor analysis were used to select items. Five dimensions and 20 items were selected for the final scale. Cronbach's coefficient was used to evaluate the reliability of the formal scale. Factor analysis was used for construct validity evaluation. Results Dimensions were measured, including service processes, organization and regulation for pharmacy administration, patients informing and education, medicine catalogue, and the economic burden of patients. The scale had good reliability since overall Cronbach coefficient was 0. 844. Factor analysis extracted five common factors, total explaining 89. 69% of the cumulative variance, and the item distribution in five common factors was completely consistent with formal scale, indicating good construct validity. Conclusion This scale had good reliability and validity as a measurement tool to evaluate the transparency level of primary health care facilities in China.

Objective: To compare the effects of droperidol, midazolam sedation on implicit memory in patients under epidural anesthesia. Methods: Thirty- two patients with ASA I 一 II un-dergoing general surgery operations with epidural anesthesia were divided into 2 groups,group D received droperidol 2.5mg and fentanyl 0.05mg as sedative, group M received midazolam 3mg and fentanyl 0.05 mg as sedative. The explicit memory and implicit memory scores were gained by methods of process dissociation procedure, and the patients' assessment of alertness/sedation scales were graded simultaneouly. Results: The median of patient' assessment of alertness/ seda-tion scale in group D and group M were 4 and 3 respectively. Patients in group D had higher explicit memory scores and lower implicit memory scores than those in group M. But the implicit memory had not disappeared completely in group D. Respiratory depression emerged when increasing the dosage of droperidol. Conclustion: Droperidol can specifically depress the implicit memory, but it results in respiratory depression when the dosage of droperidol increased. It limits the usage of droperidol to eliminate the implicit memory completely.

Objective To explore the efficacy of tepronone and folic acid in the treatment of chronic atrophic gastritis (CAG) evaluated by the marking targeting biopsy (MTB).Methods A total of 224 H.pylori negative CAG patients were selected and divided into group A (n 96,tepronone 50 mg/time,folic acid 10 mg/time,three times/day),group B (n=23,tepronone 50 mg/time,three times/day),group C (n=74,unspecific treatment) and group D (n=31,no treatment).The treatment course lasted for one year.The clinical symptoms improvement of each group was observed before and after treatment.The pathological improvement of gastric mucosa by MTB was inspected before and after treatment.The chi square test was performed for the comparison between groups.Results The total efficacy rates of group A,B,C and D were 43.8％ (42/96),39.1％ (9/23),33.8％(25/74) and 32.3％ (10/31) respectively,there was no significant difference between groups (x2 =2.328,P =0.507).For the significant efficacy rate of gastric mucosa pathological improvement,group A was compared with group D,group A was compared with group C and group B was comparedwith group D,the differences were significant (x2 =14.520,14.628 and 8.995,all P＜0.01).In the total efficacy rate of gastric mucosa pathological improvement,group A (49.8％,131/263) was compared with group D (24.2％,16/66),group A was compared with group C (35.9％,66/184)and group B (44.7％,21/47) was compared with group D,the differences were significant (x2 =13.953,8.535 and 5.207,all P＜0.05).Conclusion Teprenone alone or teprenone and folic acid combination can obviously improve pathological changes of CAG patients.

Objective To investigate the effect of mechanical thrombectomy device Solitaire AB for the treatment of distal occlusion of the intracranial artery caused acute anterior circulation ischemic stroke. Methods The clinical data of 9 patients with arterial occlusion of distal anterior circulation treated by using the Solitaire AB thrombectomy device were analyzed retrospectively. Seven of them had M2 middle cerebral artery occlusion and 2 had A3 occlusion. Their clinical features,imaging data,treatment,and the results of 3-month clinical follow-up were analyzed. The effectiveness of treatment was evaluated through the recanalization rate after stent thrombectomy,the National Institutes of Health Stroke Scale (NIHSS)scores before and after treatment,the modified Rankin scale (mRS)scores,and the 3-month clinical follow-up results. The surgery-related complications and mortality of patients were used to evaluate the safety of the treatment. Results (1 )Of the 9 intracranial arterial occlusions,8 were recanalized successfully. The modified thrombolysis in cerebral infarction (mTICI)was 2b or grade 3. (2)The NIHSS score median 9. 5 (3. 0,15. 5)at discharge dropped significantly compared with 19. 0 (16. 0,22. 0)before procedure. (Z=2. 703,P=0. 007). No permanent complications related to operation occurred. Four of the recanalized patients had good prognosis (mRS 0-1)and 4 had disability (mRS 3-4 ). The mRS score of one non-recanalized patient was 4 at the 3 months follow-up. Conclusion The embolectomy effect is good whom using Solitaire AB device for acute occlusion occurred in the intracranial artery M2 or A3 segment. Its efficacy,safety,and indications still need to be further explored in a larger sample controlled trial.

AIM: To investigate the protective effect of Ginkgo biloba extract (GBE) on diabetic testis and explore its possible mechanism. METHODS: Testicular structure of streptozotocin-induced diabetic rats was observed under light microscopy (LM) and transmission electron microscopy (TEM). Content of malondialdehyde (MDA), NO_2~-/NO_3~- and activity of superoxide dismutase (SOD), nitric oxide synthase (NOS) were determined in testicular homogenate. RESULTS: In diabetic rats, it was manifestated as deformation of seminiferous tubule, atrophy and shedding of germinal epithelium under LM, while expansion of smooth endoplasmic reticulum, formation of fatty vacuoles and decrease of lysosome obviously in the cytoplasm of sertoli cell under TEM, the injury of testicular tissue was improved by GBE. Compared with diabetic rats, activity of SOD increased while activity of tNOS and iNOS, content of MDA and NO_2~-/NO_3~- decreased in GBE-treated rats. CONCLUSION: GBE could effectively prevent the development of diabetic testis and the effect may be partly achieved by resisting lipid peroxidation,restraining the activity of testicular tissue iNOS and reducing the pathological alterations of NO. [

Objective To investigate the feasibility and effectiveness of inducing rabbit common carotid fusiform aneurysms via the common carotid extravascular digestion method. Methods Sixteen New Zealand white rabbits were randomly assigned into either an experiment group ( n=12 ) or a control group (n=4). Porcine pancreatic elastase 80-400 U were used to incubate and digest 2 to 4 cm segment of artery distal to the origin of right common carotid artery. One week after modeling,intravenous angiography was performed and the length and width of fusiform dilatation of common carotid artery were measured. The fusiform dilated artery was examined with hematoxylin and eosin staining and the vascular morphological changes were observed with scanning electron microscope. Isotonic saline solution was used to incubate common carotid arteries of the 4 New Zealand white rabbits in the control group. After one week,the same method was used to observe the lumen of common carotid artery and intimal changes. Results After the digestion of common carotid artery adventitia,the angiography of 12 New Zealand white rabbits of the experimental group revealed fusiform dilatation of common carotid artery of the 10 model rabbits. The widest diameter of the fusiform artery was 3. 70 ± 0. 32 mm;two rabbits had common carotid artery occlusion. Compared with the control group,the right common carotid artery diameter enlarged significantly in the experimental group (1. 80 ± 0. 16 mm,P<0. 01). The HE staining showed that the lumen widened, adventitia and media reduced. Scanning electron microscope showed intimal inflammatory injury and thrombus attachment. Conclusion Using porcine pancreatic elastase to digest the adventitia of common carotid artery can make fusiform dilatation of common carotid artery in rabbits. Using this method may effectively induce a model of fusiform aneurysm,and it has certain feasibility.

AIM: To study the protective effects of the extract of Ginkgo biloba (EGB) on brain tissues in experimental diabetic rats and explore its possible mechanisms. METHODS: Thirty male Sprague-Dauley rats were divided into three groups: normal control group, diabetic group and EGB-treated group. Strepozotocin were injected intraperitoneally on the later two groups to induce diabetes, EGB-treated group was injected intraperitoneally with EGB, and the others were treated with normal saline at the same volume. After five weeks, the content of endothelin (ET), malondial dehyde (MDA), NO2~-/NO 3~-, and the activity of superoxide dismutase (SOD), nitric oxide synthase (NOS) were determined in brain homogenate, and the level of blood glucose, insulin and ET were measured respectively. In addition, the morphologic changes of the brain tissue were studied by light microscopy and electron microscopy, respectively. RESULTS: In diabetic group, there were degeneration of neuron, brain edema, softened focus and demyelination in the white matter of brain by light microcopy. There were expansion of mitochondria of neuron and gliocyte, the shortened of crista, demyelination of neurofiber and injury of blood-brain-barrier by the electron microscopy. After treated with EGB, the pathological changes decreased in brain under light microcopy and electron microcopy. Compared with diabetic rats, the activity of SOD and the level of serum insulin increased, while the level of blood ET, the activity of NOS, the content of ET, MDA, NO2~-/NO3~- decreased in EGB-treated rats (P

This research aims to construct a lentiviral expression vector carrying the extracelluar domain (ED) of human hepatocyte growth factor receptor (C-Met), and to express it in transfected 293T cells. The extracellular domain of C-Met was amplified by RT-PCR, ligated with lentiviral expression vector p RRL-CMV-ED, and then expressed in 293T cell line. The expressed protein was purified and identified by RT-PCR and Western blot. The enzyme digestion and sequence analysis showed that the lentiviral expression vector p RRL-CMV-ED was constructed correctly. The size of amplified genes was about 2 700 bp. The purified protein with Ni-affinity column was about 105 kD analyzed by SDS-PAGE. The Western blot and ELISA results showed that the expressed protein which could bind to HGF specifically was the extracelluar domain of human hepatocyte growth factor receptor. This research may lay a foundation for further study of anti-C-MET monoclonal antibody and neutralizing antibody.
Genetic Vectors ; HEK293 Cells ; Humans ; Lentivirus ; Proto-Oncogene Proteins c-met ; genetics ; metabolism ; Transfection

Objective:To evaluate the importance of 3D-CTA with volume rendering for the diagnosis of multiple intracranial aneurysms. Methods: Axial source images were obtained by helical CT scanning and reconstruction of 3D-CTA images was done by volume rendering technique in conjunction with multiplanar reformation. Results: In the past one year,there were 10 patients diagnosed as having multiple intracranial aneurysms by 3D-CTA and altogether 24 aneurysms were visualized,including 10 small aneurysms(≤5mm.Three dimensional CT angiography with volume rendering demonstrated aneurysms very well and provided useful information concerning the site,shape,size and spatial relationship with the surrounding vessels and bone anatomy. Conclusion: Three-dimensional CT angiography with volume rendering is a quick,reliable,and relatively noninvasive method for diagnosing multiple intracranial aneurysms.It delineates detailed aneurysmal morphology,and provides useful information for planning microsurgical approaches.