The treatment of cerebral palsy is still a big medical problem.The pathogenesis of spasticity,as result of a variety of lesions of the cerebral cortex,brain stem,spinal cord,is caused by involvement of the inhibitory pyramidal and parapyramidal descending tracts terminating on the spinal facilitatory myotatic reflex.A lesion of the descending tracts disturbs this equilibrium leading to spasticity,which is cha-racterized by muscle resistance at rest that is velocity dependent and associated with an increase in tonic stretch reflexes resulting from hype-rexcitability of the stretch reflex.Spasticity caused abnomal posture,caused special movement,and higher multilation,which affect children's life severely.There are so many ways to lower hypermyotonia,such as:drugs,rehabilitation care,acupuncture and so on.Bioelectric stimulation therapy is a new ways in the zone.Its curative effect and the mechanism are still in explore,this article just to give an overview about bioelectric stimulation therapy in curing spastic cerebral palsy.

Botulinum Toxins, Type A ; administration & dosage ; adverse effects ; therapeutic use ; Cerebral Palsy ; drug therapy ; Child ; Child, Preschool ; Dose-Response Relationship, Drug ; Female ; Humans ; Male

Objective To observe the curative effect of botulinum toxin A(BTX-A)repeated intramuscular injection for treatment of serious spastic cerebral palsy.Methods Thirty cases with serious spastic cerebral palsy received local repeated intramuscular injection. The interval time was 3 months. The dose of BTX-A was 4 U/kg.The muscle tone was assessed with the modified ashworth scale and range of motion with physical rating scale(PRS).The indexes were evaluated before injection and 3 months after the second injection. Paired-samples t-test was used in the statistic analysis.Result The muscle tone and PRS had remarkable improvement after second therapy with BTX-A(P

ObjectiveTo evaluate the efficacy of rehabilitation associated with local intramuscular injection of botulinum toxic A (BTX-A) on spastic cerebral palsy (CP).Methods60 children with spastic CP were divided into experimental group and control group with 30 cases in each group. Cases of experimental group were treated with rehabilitation associated with local intramuscular injection of BTX-A. Cases of control group were treated only with rehabilitation treatment. The therapeutic efficacies of two groups were evaluated with physician rating scale (PRS) and activities of daily living (ADL) evaluation systems.ResultsImprovement of clinical evaluations index-PRS and ADL in experimental group was much more significant than that of control group (P<0.05). ConclusionRehabilitation associated with local intramuscular injection of BTX-A can improve the efficacy of spastic CP.

Niemann-Pick disease type C (NPC) is an autosomal recessive lysosomal lipid storage disease associated with impaired intracellular cholesterol trafficking. A wide spectrum of clinical phenotype has been described, with a possible onset at all ages of life from the neonatal period to adulthood, more often in childhood. Typically, hepatosplenomegaly, dystaxia, dysphagia, dysarthria and dementia are presented in NPC patients. Neurologic symptoms vary according to the onset age, but prolonged neonatal cholestasis, splenomegaly, cataplexy and vertical supranuclear gaze palsy are more specific signs to the diagnosis of the disease. Impaired cholesterol trafficking and unesterified cholesterol accumulation in the late endosomes and lysosomals, as a results of mutations in NPC1 or NPC2 genes, are initial for the disease, and defective cellular autophagy, defective lysosomal calcium homeostasis and oxidative stress may all play roles in the physiological processes. The definite diagnosis requires demonstration of unesterified cholesterol accumulated in fibroblasts cultured from skin biopsies or of pathogenic mutation of NPC1/NPC2 genes. Miglustat, the only available treatment approved to date, can alleviate neurological symptoms and slow disease progression when administered earlier.
Diagnosis, Differential ; Humans ; Niemann-Pick Disease, Type C ; diagnosis ; etiology ; genetics ; therapy

Background The proliferation and migration of vascular endothelial cells is a primary link during angiogenesis.Studies showed that heat shock protein B6 (HspB6) promotes the secretion of multiple angiogenesis-related factors and therefore leads to neovascularization.Understanding the effects of neutralizing HspB6 antibody on the biological behavior of human choroidal vascular endothelial cells has an important significance in the target treatment of choroidal neovacularization diseases.Objective This study was to address the role and mechanism of neutralizing HspB6 antibody in tube formation of human choroidal vascular endothelial cells.Methods Human choroidal vascular endothelial cell line was normally cultured and harvested for total RNA extraction.Expressions of HspB6 mRNA and protein in human choroidal vascular endothelial cells were detected by reverse transcription PCR (RT-PCR) and flow cytometry (FCM).The cells were seeded on 96-well plate covered with matrigel at the density of 2×104/hole.Then the neutralizing HspB6 antibody at the concentration of 100 μg/Land 500 μg/L was added into the medium respectively,and the control cells were set without the addition of HspB6 antibody.The number of capillary tubes was calculated 12 hours after culture by three-dimensional matrigel assay.In addition,0,50,100,500 μg/L of neutralizing HspB6 antibody were added into the cell medium separately for 24hours,cell counting kit-8 (CCK-8) method was employed to assay the inhibitory rate(IR) of the cells.Transwell test was used to count the cell number across chamber membrane for the evaluation of migration ability of the cells.The apoptosis of the cells was assayed by FCM.Results Both HspB6 mRNA and protein were expressed on human choroidal vascular endothelial cells.The number of capillary tube formation of human choroidal vascular endothelial cells was (67.25±5.75),(60.39±6.41) and (39.76±10.73) /field in the 0,100 and 500 μg/L neutralizing HspB6 antibody groups,with significant difference among them (F =10.210,P =0.012),and the tube number was significantly less in the 500 μg/L neutralizing HspB6 antibody group compared with 0 μg/L neutralizing HspB6 group (P =0.005).The IR of neutralizing HspB6 antibody to the cellular proliferation and migration was enhanced with the increases of concentration and time lapse(Fconcentration =7.485,P =0.002 ; Ftime =16.684,P =0.001).The number of the cells through Transwell chamber membrane was 14.0 ± 2.5,11.1 ± 0.8,6.6 ± 0.1,6.7 ± 0.2 in the 0,50,100,500 μg/L neutralizing HspB6 antibody group respectively,and that in the 100 μg/L and 500 μg/L neutralizing HspB6 antibody group was lessened in comparison with the 0 μg/L neutralizing HspB6 antibody group(both at P=0.000).The apoptosis rate of the cells was (22.73 ± 2.53)％ in the neutralizing HspB6 antibody group,which was significantly lower than (13.33±2.08) ％ of the control group (t=4.967,P=0.008).Conclusions Neutralizing HspB6 antibody inhibits capillary tube formation of human choroidal endothelial cells in vitro in dose-and timedependent manner,probably through suppressing the proliferation and migration and promoting the apoptosis of choroidal endothelial cells.

Objective To explore influencing factors of peripherally inserted central catheter(PICC)infection among premature infants and low birth weight infants,and to implement nursing intervention and reduce infection.Methods 128 subjects that underwent the PICC phlebitis,positive blood culture and positive catheter culture in our department were retrospectively reviewed.Results 12 cases had phlebitis:5 cases of the great saphenous vein,2 cases of the cephalic vein,4 cases of the median cubital vein and 1 case of the basilic vein,the difference was significant(x2 =11.351,P ＜ 0.01).1 case had 1-7 d catheter retention,of which neither positive blood culture nor positive catheter culture was observed.10 cases had 8-21 d catheter retention,and positive rate of blood culture and catheter culture was 17.14％,11.32％,respectively.1 case had 22-47 d catheter retention,and positive rate of blood culture and catheter culture was 20.75％,17.14％,respectively.Conclusions Phlebitis can be effectively prevented and decreased by establishing PICC group,producing standard operating card,improving puncture skills,selecting appropriate retention site and strengthening maintenance measures.

In order to study the distribution of virulence genes of Listeria monocytogenes (Lm) in Hebei Province,29 virulence genes of Lm,including Listeria monocytogenes pathogenicity islands Ⅰ (LIPI-Ⅰ:prfA,plcA,plcB,hlyA,mpl and actA),10 internalins genes (inlA,inlB,inlC,inlD,inlE,inlF,inlG,inlH /C2,inlI and inlJ) and the other 13 virulence-associated genes (bsh,srtA,iap,sigB,virR,mprF,dltA,dltB,dltC,dltD,srtB,fbpA and hpt) were detected by PCR.Results showed that in the 91 Lm strains,the detection rate of 23 virulence genes were 100％.The 29 virulence genes of 26 Lm strains were all detected,and 65 Lm strains had different deletion of 6 virulence genes inlD,inlF,inlG,inlH /C2,inlJ and mpl.The deletion rate of inlG and inlF were 60.44％ and 54.95％,respectively,following by mpl gene,with a deletion rate of 19.78％.According to the absence of virulence genes,91 strains could be divided into 10 subtypes,and the dominant virulence subtypes was type Ⅰ with all 23 virulence genes.The deletion rate of virulent genes in Shijiazhuang was higher than that in northern Hebei.It is suggested that the rate of virulence gene of food-borne Lm in Hebei Province is high,and the virulence gene deletion patterns has diversity and regional differences.

Objective To identify risk factors for a human orf disease outbreak in a village in Chongqing city. Methods Standardized case-definition was set and a case-finding program was conducted among all the residents of the village. All the patients were interviewed using a standardized questionnaire and collected fluids in the skin rash for laboratory testing. A retrospective cohort investigation was conducted among all the village residents who introduced the black goats to analyze the risk of orf infection, in relation to the mode and frequency of contacts to the infected goats. Results We found 18 cases (including 16 suspected cases and 2 confirmed cases) among the members of 10 families that introduced the black goats. Village residents who had ever used their legs to grip the goats were nearly five times as likely to develop orf disease as those who did not (RR=4.98, 95％CI: 1.34-75.27). Village residents who had ever washed and wiped the goats were three times as likely to develop orf disease as those who had not (RR = 3.09,95％CI: 0.98-45.38). The frequency of contacts with the infected goats was associated with the risk of developing orf disease in a dose-response fashion (x2 test for trends: P= 0.006).Frequently wearing long trousers when dealing with the goats appeared as a protective factor (RR=0.30,95％CI: 0.15-0.78). Conclusion This outbreak was caused by the introduced black goats which carried and infected by the orf virus. Direct physical contact with infected goats but without wearing protective clothing were risk factors for the development of human orf disease.