Objective To explore the importance of gene diagnosis and prenatal diagnosis of spinal muscular atrophy(SMA),and improve the clinical diagnosis of SMA by analyzing the electrophysiological and gene characteristics of SMA.Methods Fifteen cases with SMA including 9 male and 6 female were enrolled in this study.The age was 5 months to 12 years.The 15 cases were subdivided into 3 clinical types,5 cases of type Ⅰ including 3 male and 2 female aging 5-18 months;7 cases of type Ⅱ including 4 males and 3 females aging 5 months-3 years;3 cases of type Ⅲ including 2 male and 1 female aging 3-12 years.They were all characterized by symmetric muscle weakness(more proximal than distal)associated with atrophy,absence or marked decrease of deep tendon reflexes,loss of voluntary movement and inability to sit or stand.The clinical characteristics and changes of electromyography(EMG)and nerve conduction velocity were assessed in all cases by using Danish Medoc Keypoint myoelectricity and evoked potentials inducer.The survival of motor neuron(SMN)gene was detected by PCR and restriction endonuclease spectrum analysis in 10 cases.Results EMG analysis found 94% patients had spontaneous potential,90% patients had increased duration of motor unit,and amplitude increased in 89% patients.Motor nerve conduction velocity was determined in 78 nerves.Motor nerve compound action potential wave amplitude decreased in 52 nerves,among them,distal latent period prolonged and motor conduction velocity reduced slightly in 36 nerves.Sensory nerve conduction velocity was determined in 45 nerves and remained normal.The SMN gene detection revealed deletion of exon 7 and 8 in 9 cases,deletion exon 7 in 1 case.The SMN gene detection in 10 patients and their parents didn't find any deletion of exon 7 and 8.Conclusions The definite diagnosis of SMA will rely on the typical clinical characteristics,changes of EMG and gene deletion analysis.Gene diagnosis of SMA lays a basis for prenatal diagnosis.

Objective To investigate the effect of Nd: YAG laser on microtensile bond strength of single bond adhesive system for non-carious sclerotic dentin. Methods Ten human molars with occlusal wearing were cut into equal halves ( nearly 12 mm2 ), and randomly divided into experimental group and control group. The teeth in experimental group were processed with Nd: YAG laser( 1 W,10 Hz) ,and then applied with Scotchbond and filled with Z350 resin. In control group, the teeth were processed with single bond and filled with Z350 resin. The specimens were sectioned, and the microtensile bond strengths of each sample was tested by a universal testing machine. Results The bond strength of the experimental group [ (26. 11 ±1.62) MPa] was significantly higher than that of the control group[ (22. 27 ±2. 16) MPa],P＜0. 05. Stero-microscope examination indicated that most of the fractures occurred in dentin-resin interface. Conclusions Nd: YAG laser can increase the rnicrotensile bond strength of single bond adhesive system in non-carious sclerotic dentin.

Objective To construct PPAR?and PPAR?response element (PPRE)-controlled luciferase expression vectors,and to determine whether the traditional Chinese medicine emodin activates PPAR?and improves the glucose uptake by HepG2 hepatocytes.Methods (1) PPAR?and PPRE luciferase expression vectors were constructed and were applied to screen more than 20 ingredients of the traditional Chinese medicine. (2) HepG2 cells were incubated with emodin which can activate PPAR?and PPRE luciferase activity,and the PPAR?mRNA expression level was evaluated by RT-PCR/Southern blot.(3) PPAR?and glucose transporter 2 (Glut2) proteins were determined by Western blot analysis in HepG2 cells treated with emodin.(4) The glucose uptake rate was measured using 2-deoxy-[~3H]-D-glucose in HepG2 cells after treatment with emodin.Results (1) Emodin stimulated luciferase activity controlled by PPRE in dose-dependent manner at concentrations of 0.04 to 180?mol/L in COS-7 cells.The highest value was about 4 folds of control in the cells treated with 90?mol/L emodin (P

Objective To investigate the dietary pattern in college freshmen students and to analyze the influencing factors on their dietary patterns. Methods A questionnaire survey on situation of dietary pattern and influencing factors was conducted among 1319 freshmen students.Results Four major dietary patterns were noticed and they were: Ⅰ , high consumption in hamburger,fried food, nuts, biscuit, chocolate, cola, coffee, sugars, Ⅱ, high consumption in pork, mutton, beef,poultry meat, animal liver, Ⅲ, high consumption in fresh fruits, eggs, fish and shrimps, kelp laver and sea fish, milk and dairy products, beans and bean products, Ⅳ, high consumption in rice and grain,fresh fruits, fresh vegetables, pork. Risk factors on dietary pattern were presented as follows: ( 1 )boys: having the food pattern Ⅰ and Ⅱ showed a strong positive association with the place where they live (OR= 1.67, 95％CI: 0.87-3.19; OR= 1.51,95％CI: 0.79-2.88), eating place (OR=1.63,95％CI: 1.O3-2.59; OR= 1.83, 95％CI: 1.04-3.23), level of mother' s education (OR=2.52,95％CI: 1.07-5.95; OR=3.38, 95％CI: 1.50-7.63), family income (OR=2.24, 95％CI: 1.30-3.88;OR=3.06, 95％ CI: 1.77-5.29) and the status of passive smoking (OR= 1.80, 95％CI: 0.70-4.59;OR=1.83, 95％CI: 0.75-4.45). Inverse correlations was found on the level of mother's education (OR=0.56,95％CI: 0.17-1.79). The food pattern Ⅳ showed a strong positive association with place of eating(OR= 1.83,95％CI: 1.04-3.23) but having an inverse correlation with the level of mother's education (OR=0.56, 95％CI: 0.17-1.79). (2)girls when compared with boys, the food pattern Ⅰ showed minor association with the places of living and eating; while the food pattern Ⅱ and Ⅲ had minor association with the status of passive smoking. Conclusion Socio-demographic factors and lifestyle had influenced on the dietary patterns among college freshmen students who should be guided to have a reasonable, balanced diet in the college.

The aim of the present study was to investigate the effect of removal of the adventitia on vascular remodeling and vasoconstriction of the carotid artery in New Zealand rabbit. Adventitia of carotid artery was removed mechanically. The histology, morphology and reactivity of the carotid artery was observed by immunohistochemistry and measurement of carotid ring tension immediately, 1 week and 2 weeks after removal of the adventitia. No damage of intima and media was observed after removing the adventitia. Removal of the adventitia caused a remarkable proliferation of the vascular media and formed the neointima. Compared with the control ring, norepinephrine (NE)-induced vasocontraction in adventitia-denuded carotid artery was significantly reduced immediately and 1 week after the operation (P<0.05). Adventitia removal promoted the neointima formation and decreased vasoconstriction of the carotid artery, indicating that the adventitia is involved in the regulation of vascular remodeling and vasoconstriction.
Animals ; Carotid Arteries ; pathology ; physiology ; surgery ; Connective Tissue ; physiology ; surgery ; Muscle, Smooth, Vascular ; pathology ; Rabbits ; Tunica Intima ; pathology ; Vasoconstriction ; physiology

A total of 126 patients with type 2 diabetes mellitus were randomized into two groups:one received glimepiride 1 mg twice daily and the other 2 mg once daily.Fasing blood glucose(BG),BG 2 h after meals(breakfast,lunch and dinner)and HbA_(IC)were tested,△and standard deviation of the 4 point BG were calculated.It was found that two kinds of administration of glimepiride were equally effective in decreasing BG and once daily aministration could ease better the fluctuation of BG.

Objective To provide a scientific basis for hospitalized pa-tients receiving warfarin anticoagulant therapy.Methods Collecting 136 medication records of inpatients that have used warfarin in cardiology wards of our hospital from January 2012 to June 2013 , and analyzing the correlation of drug interactions and pathological factors with warfarin effi-cacy and adverse reaction.Results and Conclusion Moxifloxacin , acarbose , metformin and methylprednisolone are probably effect on warfa-rin treatment -related indicators.Infection and bleeding adverse effect prolonged hospitalization days.Warfarin therapy were influenced by drug interactions and pathological factors.

Objective To observe the mechanism of perindopril on basic fibroblast growth factor(bFGF) in the conduction pathway of cardiac hypertrophy rats.Methods A total of 90 male healthy SD rats were randomly divided into model group,control group and experimental group.The model group were subcutaneously injected with 3 mg · kg-1 · d-1 of isoproterenol,and intragastric administration of saline 2 mL.Control group was injected with saline by hypodermic injection,and intragastric administration of saline 2 mL.Experimental group were subcutaneous injection with 3 mL · kg-1 · d-1 of isoproterenol,and 2 mL intragastric administration of 1 mg · kg-1 · d-1perindopril in saline once daily,each group was treated for 10 days.After treatment,the left ventricular mass and heart mass index were measured and calculated,and the morphology and arrangement of left ventricular myocardium were observed by hematoxylin-eosin(HE) staining.The expression of bFGF mRNA in left ventricular myocardium was detected by fluorescent quantitative PCR (RT-PCR).Results The left ventricular mass indexes in the experimental group,the model group and the control group were (1.95±0.11),(2.47±0.09) (1.45±0.11) g·m-2;the heart mass index were(2.69 ±0.36),(3.81 ±0.10),(1.92±0.12) g · m-2;the relative mRNA expression levels of bFGF in cardiomyocytes were 0.64 ± 0.11,0.74 ± 0.11,0.38 ± 0.08 respectively.There were significant differences between the experimental group and the control group as well as the model group (P ＜ 0.05).Conclusion Perindopril can reduce the expression of bFGF and thus inhibit ventricular remodeling.

OBJECTIVETo investigate the in vitro effect of iron overload on the generation of reactive oxygen species (ROS) and of bone marrow (BM) cell function.

METHODSBM mononuclear cells (BMMNCs) were cultured with ferric citrate (FAC) at different concentrations and for different time to create iron overload and confirmed by the detection of cellular labile iron pool (LIP). The changes of ROS, apoptosis, hematopoietic colony formation (CFU-E, BFU-E, CFU-GM and CFU-mix) and the percentage of the CD34 + cells percentage were analyzed. The differences of these index were tested after the iron overload treated with deferasirox (DFO) or antioxidants (N-acetyl-L-cysteine, NAC).

RESULTS1) When BMMNCs were cultured with FAC, the LIP was found to increase in a time and concentration dependent manner. The intracellular LIP reached maximum at 400 micromol/L of FAC for 24 hours. 2) The ROS of total cells, leukocytes and erythrocytes increased to 1.77, 1.75 and 2.12 fold respectively compared with that of normal control when cells were cultured at 400 micromol/L of FAC for 24 hours . DFO and NAC could reduce the ROS efficiently (P<0.05). 3) The apoptotic rates of the FAC treated cells [(24.80 +/- 2.99)%] increased significantly compared with that of normal control [(8.90 +/- 0.96)%]. The capacity of hematopoietic colony formation in FAC treated cells decreased markedly compared with that of normal control (P<0.05). The percentage of CD34+ cells of FAC treated cells [(0.39 +/- 0.07)%] also decreased significantly compared with that of normal control [(0.91 +/- 0.12)%]. And these changes could be recovered by addition of NAC or DFO.

CONCLUSIONIron overload can affect the hematopoiesis by inducing the generation of ROS and this damage could be corrected by removing the excess iron and ROS of the BM cells. These findings might improve the treatment of dyshematopoiesis in patients with iron overload.

Bone Marrow Cells ; physiology ; Cells, Cultured ; Culture Media ; chemistry ; Erythrocytes ; Ferric Compounds ; pharmacology ; Hematopoiesis ; Humans ; Iron Overload ; Reactive Oxygen Species ; metabolism

OBJECTIVESTo study the clinical characteristics of hepatic failure with aspergillosis.

METHODSThe data of hepatic failure patients with fungal infection hospitalized in our hospital form January 1985 to June 2006 were collected. This research mainly focused on the clinical characteristics of the patients co-infected with aspergillosis.

RESULTSThe occurrence of aspergillosis was 20.5% (104 cases) among 507 hepatic failure patients with fungal infection. Compared with other fungal infection in hepatic failure patients, the effective rate of antifungal therapy and the improvement rate of underlying disease were worse in patients with aspergillus infection (36.5% vs 57.8%, P = 0.000; 26.0% vs 36.7%, P = 0.049). Aspergillus fumigatus was the most common species among 108 fungal species. The species next to Aspergillus fumigatus were Aspergillus niger and Aspergillus flavus. The mainly infected organ was lung and its clinical manifestation was atypical. Liver function could be improved with effective anti-fungus therapy.

CONCLUSIONSDiagnosis and treatment of aspergillosis is difficult in hepatic failure patients co-infected with aspergillosis. Early and effective antifungal therapy is helpful to the recovery of liver function in the hepatic failure patients suspected with aspergillosis co-infection.

Antifungal Agents ; therapeutic use ; Aspergillosis ; diagnosis ; drug therapy ; Aspergillus ; isolation & purification ; Humans ; Liver Failure ; diagnosis ; drug therapy ; microbiology