Hereditary motor and sensory neuropathy is the most common peripheral neuropathy.It is slowly progressive,proximal limb weakness and muscular dystrophy and severe foot deformity can cause function disability.For most patients were diagnosed in early children,so function disability evaluation and effective treatment from children is significant to their prognosis.

Objective To investigate any correlation between diffusion tensor imaging (DTI) results and Montreal cognitive assessment (MoCA) scores after severe traumatic brain injury (TBI).Methods Eight male pa-tients with chronic severe TBI were given the MoCA (including memory,attention,speech and executive function).DTI was used to quantify the fractional anisotropy (FA) of white matter fiber tracts in the radial and longitudinal fasciculus,under longitudinal fasciculus,internal capsule,corpus callosum genu and body,and the cingulate cortex.Pearson correlation coefficients were calculated to quantify the correlation between the FA values and MoCA scores.Results There was a positive correlation between FA in the corpus callosum body,corpus callosum genu and the superior longitudinal fasciculus and MoCA total scores.Conclusion The MoCA scores of patients with chronic severe TBI are related to white matter damage in the corpus callosum body,corpus callosum genu and the superior longitudinal fasciculus.

Ohjective To study the effects of emphysema on peripheral skeletal muscle biomechanics,pathomorphology and oxidative metabolism in rats.Methods Twenty Sprague-Dawley rats were used and randomized equally into a control group and an emphysema group.A dose of 40 U/100 g body weight of porcine pancreatic elastase was instilled into the trachea of the animals of the emphysema group to model emphysema,while the same volume of saline was instilled into those of the control group.Twenty weeks after instillation,in situ mechanical properties of gastrocnemius were evaluated.Gastrocnemius fiber type composition and capillary density (CD) were assessed by using ATPase staining.Lipofuscin accumulation (LI/F) was determined with the ferric-ferricyanide reduction test technique.Immunohistochemistry was used for the detection of nitric oxide synthases (NOS) in gastrocnemius.The muscle biopsy homogenate was used to measure the activity of superoxide dismutase (SOD),catalase (CAT),NOS,total antioxidant capacity (T-AOC) and the content of nitric oxide (NO).Results Emphysema increased fatigability and decreased the recovery rate of gastrocnemius muscle [(145.0 ± 55.4) s vs (55.2 ± 29.3)s,P ＜ 0.05].Compared to control,the gastrocnemius muscle in rats with emphysema had a lower CD [(513.9±71.1)n/mm2 vs (578.6 ±59.9)n/mm2,P ＜0.05] and a decreased proportion of type I fibers [(16.0 ±5.0)％ vs (30.7 ±4.1) ％,P ＜0.05],with a reciprocal increase in type II b/x fibers [(27.3 ±4.8)％vs (11.0±3.2)％,P＜0.05].LI/F was higher (3.3±0.5 vsl.7±0.4,P＜0.05) and the activity of SOD,CAT and T-AOC was increased in emphysema group.Compared with control,rats with emphysema demonstrated a lower expression of endothelial NOS (eNOS) (1.9 ± 0.5 vs 3.4 ± 0.6,P ＜ 0.05),and an equivalent expression of neuronal NOS (nNOS) (4.7 ± 1.0 vs 5.1 ± 0.8,P ＞ 0.05) in the gastrocnemius muscle.The inducible NOS (iNOS) was not found in both groups.Conclusions Emphysema could induce biomechanical,pathomorphological and oxidative metabolic changes in peripheral skeletal muscle.

Objective To investigate the effect and safety of self-made blenderized diet used for nutritional support treatment in the ICU critically ill patients with diabetes. Methods 74 ICU critically ill patients with diabetes were randomly divided into two groups:study group and control group. The self-made blenderized diet were given to the patients in study group and enteral nutrition to the patients in control group for nutritional support treatment for four weeks. Then glucose metabolism, various nutrition indicators and gastrointestinal dysfunction were observed in these two groups. Results The fasting blood glucose and fructosamine levels were decreased significantly,while the nutritional indicators was significantly higher after treatment than at baseline in all patients. There was no significant difference in the levels of fasting blood glucose, postprandial glucose, fructosamine and nutritional indicators between two groups after treatment,so were the incidence of the hypoglycemia and gastrointestinal complications. Conclusion The self-made blenderized diet was a good enteral nutrition support therapy for ICU critically ill patients with diabetes which had a considerable effect, but more cheaper,and practical compared to the enteral nutrition emulsions.

Objective To compare the effects of no-rinse skin cleanser and warm water with paraffin oil in first brush bath of the new-born. Methods About 42 newborns who were born from January to March 2016 were set as control group and used warm water with paraffin oil in first brush bath. About 43 newborns who were born from April to June 2016 were set as observation group and used no-rinse skin cleanser in first brush bath. The cleaning time and effect ,the rectal temperature before and after sponge and dermatitis were compared. Results Both groups did not develop dermatitis. But the observation group had better cleaning satisfaction , less cleaning time, faster rewarming than those of the control group with statistical significance (all p<0.05). Conclusion No-rinse skin cleanser used in the first brush for the new-born can reduce cleaning time and difference of temperature, which is safe for clinical application.

Objective To investigate the effect of celecoxib on the cell cycle and cell autophagy of HepG2 cell line and its possible molecular mechanism. Methods HepG2 cells were treated with celecoxib at different concentrations (0, 50, 100, 200, 500μM), then MTT was used to detect the cell proliferation, cell cycle and apoptosis were detected by flow cytometry, the cell autophagy was observed by transmission electron microscopy, and the expressions of cyclin and autophagy protein were determined using Western blot. Results Celecoxib inhibited the proliferation of HepG2 cells in a concentration dependent manner (0, 50, 100, 200 and 500 μM) and time-dependent manner (24,48h)(P<0.05). There was no significant change in necrocytosis and apoptosis after ttreated by different concentrations of (0, 50, 100, 200 and 500 μM). The celecoxib inhibited cell cycle arrest at G1 phase, the cell rate of G1 phase increased, while the cell rate of S phase decreased in a concentration dependent manner (0, 50, 100, 200 and 500 μM) and time-dependent manner (0, 8, 16, 24h)(P<0.05). The protein expressions of cyclin D1,cyclin D3, cyclin E2, CDK2 and CDK4 significantly decreased by 500 μM celecoxib (P<0.05). The celecoxib induced autophagy in HepG2 cells, and transformed autophagy protein LC3-Ⅰto LC3-Ⅱ in a concentration dependent manner. Conclusion Celecoxib can effectively inhibit the proliferation of human malignant hepatocellular carcinoma cell line HepG2, which provides a new idea for the clinical application of celecoxib.

Objective To investigate the characteristics of brain MRI in the patients with maple syrup urine disease(MSUD). Methods Nine patients with MSUD were diagnosed by gas chromatography mass spectrometry,tandem mass spectrometry and gene test.The MRI,clinical featuresand laboratory tests were analyzed.Results The 9 patients onset age were 3 days to 6 years old.The symptoms were varied such as poor response,lethargy,seizures and learning difficulties.Remarkable elevations of blood levels of leucine and valine were found in all patients.All patients had MRI examination,2 of them also took the examination of proton magnetic resonance spectroscopy (1 H-MRS).Involving cerebellar hemisphere,cerebellar peduncle,cerebral peduncle,brain stem,globus pallidus in 5 cases respectively,thalamus and posterior limb of internal capsule in 4 cases,centrum semiovale in 3 cases.2 cases also showed abnormal signal in corpus callosum,occipital,deep temporal lobe,and frontal lobe,parietal lobe.All lesions showed mild low signal intensity in T1 ,mild high signal intensity in T2 and obvious high signal intensity in DWI,except one case.1 H-MRS did not show a methyl resonance peak at 0.9 ppm in the two patients.Conclusion The MSUD lacked of specific clinical features.The MRI characteristics are the myelinated white matter such as the cerebellar hemispheres,posterior limb of the internal capsule,the brainstem,cerebellar peduncle,cerebral peduncle showing high signal in DWI.

Aim Toinvestigatetheinducementeffect of isatin on apoptosis of breast cancer cell line MCF-7 , andexploreitsdetailedmechanism.Methods MCF-7 cell lines were exposed to isatin at different concentra-tions(0,50,100,200 μmol·L-1 )for 48 h.Apop-totic features were demonstrated by nuclei staining with Hoechst 33258.Bcl-2,Bax and p53 mRNA were ana-lyzed by RT-PCR.Caspase-9 activation and mitochon-drial depolarization were assayed by flow cytometry. Bcl-2,Bax,p53 and cytochrome c proteins were ana-lyzedbyWesternblot.Results Isatininducesapopto-sis of MCF-7 cells.Furthermore,Bcl-2 expression was decreased and the ratio of Bcl-2 to Bax was significant-ly decreased by isatin.While,p53 expression relative-ly decreased.The mitochondrial transmembrane poten-tial was markedly reduced and the release of cyto-chrome c into the cytosol was increased after treatment with isatin.Simultaneously,caspase-9 was activated. Conclusions Isatinsignificantlyinducedtheapopto-sis of MCF-7 cells in vitro.These results strongly sug-gest that the p53 dependent mitochondrial pathway is involved in apoptosis.

Background The identification of mesenchymal stem cells(MSCs) have provided exciting prospects for cell-based regeneration after myocardic infraction.However cell therapy have inherent limitations such as low survival rate of transplanted cells and insufficient cell number.It is known that cell-matrix adhesion plays a key role in cell proliferation,differentiation and survival,and chemokine CXCL12 may involved in these prcesses.Transfected mesenchymalstem cells with CXCL12 for local secretion of CXCL12 and then explored CXCL12 triggered adhesion of mesenchymal stem cells to extracellular matrix proteins.Mesenchymal stem cells was transfected with CXCL12.?V and ?3 integrins content was evaluated by Western blot analysis.Cell adhesion to extracellular matrix was examined in vitro and cell prolife-ration after transplantation in vivo.Transfection of CXCL12 resulted increased CXCL12 in situ.Increased CXCL12 induced elevated adhesion to fibronectin in vitro and higher survival in vivo.CXCL12 mediated adhesion and proliferation was established by ?V and ?3 integrin subunits.Chemoattractive mechanisms are involved in adhesion processes of mesenchymal stem cells.Increased CXCL12 leads to enhanced expression of ?V and ?3 integrins,which may augment cell survival,proliferation and differrentiation.

Objective To discuss the application of transanal placement of ileus tube in treatment of colonic obstruction in left colorectal cancer.Methods Thirty-two patients with left malignant colorectal obstruction were divided into ordinary colonoscope group (16 cases) and superslim endoscope group (16 cases) by random digits table.The catheter success rate,catheter operating time and exposure to X-ray time was compared between two groups.Results Fifteen cases were successful and 1 case was failed in ordinary colonoscope group.Thirteen cases were successful and 3 cases were failed in superslim endoscope group.There was no significant difference in the catheter success rate between two groups (x2 =1.143,P =0.285).The catheter operating time was (42 + 15),(20 +6) min in ordinary colonoscope group and superslim endoscope group,and there was significant difference between two groups (t =3.895,P =0.005).The exposure to X-ray time was (20 + 12),(5 + 2) min,and there was significant difference between two groups (t =-3.596,P =0.007).Conclusion Transanal placement of ileus tube is more successful and convenient by superslim endoscope combined with anesthesia than by ordinary colonoscope.