Objective To evaluate exertional overheating and endurance during and after physical exercise on individuals with hypohidrotic ectodermal dysplasia(HED) ,to assess protective effects of skin cooling device ,and to provide theoretical basis for ex-ternal cooling devices .Methods 12 HED patients and 12 age-matched healthy controls were studied during standardized exercise on a treadmill at ambient temperatures of 25 ℃ and 30 ℃ .Body core temperature ,performance ,heart rate ,respiratory rate ,blood pres-sure and serum lactate were investigated during and after exercise .Results HED subjects experienced a significantly greater rise in body temperature after cycling than healthy controls ,and their body temperature remained elevated longer(P<0 .05) .HED subjects had a lower endurance time(F=9 .985 ,P=0 .005) and increasing speed value(F=7 .158 ,P=0 .014) .However ,serum lactate value of the HED subjects found to be higher than the controls (F=5 .204 ,P=0 .033) .Maximum heart rates ,respiratory rate and blood pressure did not differ significantly between HED and the control groups .However ,compared with controls ,body temperature and endurance time of HED patients equipped with skin cooling device had no statistical significance .Conclusion HED subjects showed a significantly greater rise of body temperature during exercise than the control groups ,and their body temperature remained elevat-ed longer than in healthy subjects ,and had a lower performance .External evaporative skin cooling attenuates exertional overheating in HED patients and may facilitate their participation in athletic activities and professional life .

To retrospectively analyze the clinical data of 42 patients at our department with bulbar myasthenia gravis.They underwent video-assisted thoracoscopic extended thymectomy (VATET) from May 2003 to August 2010.Another 42 patients treated with medications alone were selected as controls.Compared with the control group (4.32 ± 0.23),postoperative dysphagia scores improved significantly in patients undergoing VATET(9.21 ±0.41).And so did the dysphagia assessment classification (P ＜0.05).Thus VATET could effectively and definitely relieve dysphagia in patients with bulbar myasthenia gravis.

Objective To explore the patterns of motor and linguistic activation in cortical and its correlations with abnormal gray matter in patients with malformations of cortical development(MCD)and epilepsy.Methods Seven MCD patients with epilepsy(2 patients with focal cortical dysplasia,2 heterotopia,2 schizencephaly,and 1 polymicrogyria)underwent blood-oxygen-level-dependent(BOLD)functional MRI(fMRI)in a 3 T MR scanner when practicing bilateral fingers tapping,toes twisting,verb generation,and picture naming.Functional images were post-processed by using SPM 5 software based on a general linear model(GLM)to generate activations above a uniform threshold with the cluster size (≥30 voxels,P ＜0.001 corrected).The activations were recognized and classified by two experienced neuroradiologists,and then compared with that in abnormal gray matter.Results The clusters and intensities of motor activations were mainly located in the sensormotor cortex(SMC)and premotor area (PMA).In linguistic tasks,activations produced by verb generation were found in language-associated cortical regions and PMA with higher activation in Wemicke area,picture naming significantly in the visual cortex,and language in Broca area.Combination of the two linguistic tasks produced significant clusters and intensities in language cortex.For MCD patients with abnormal cortical abnormalities,motor and language task could produce neuronal activities within normal as well as abnormal cortex regions.In 6 patients who underwent resective surgery,epileptic seizures decreased significantly,and the follow-up images demonstrated no new neurological dysfunctions and cognitive impairments.Conclusions fMRI can visualize neuronal activities in patients with MCD and epilepsy and demonstrate the motor and linguistic activations occurring in normal and abnormal gray matter.It should be cautious for surgery in patient with MCD and epilepsy.

Objective To investigate the effect of ectodysplasin A (EDA‐A1) gene of hypohidrotic ectodermal dysplasia on pro‐liferation and cell cycle of human umbilical vein endothelial cell (ECV304). Methods Recombinant eukaryotic expression vectors pcDNA3. 1(‐)‐EDA‐A1‐M /W (mutant, M and wild, W) containing the coding sequence were transected into ECV304 cells. EDA‐A1 gene was amplified by reverse transcription polymerase chain reaction (RT‐PCR), and the protein was detected by Western blot. Cell viability and cycle distribution were invested by MTT and Flow cytometry (FCM ). Results The EDA‐A1 gene and pro‐tein were detected respectively by RT‐PCR and Western blot in ECV cells transfected with pcDNA3. 1(‐)‐EDA‐A1‐M /W, but not in ECV cells transfected with plasmid pcDNA3. 1(‐) and cells without transection. And also, compared with control groups, EDA‐A1 gene mutant significantly decreased proliferation of ECV cells and its inhibition rate was 45. 70% ( P < 0. 01), whereas wild EDA‐A1 gene did not cause such growth inhibition (P> 0. 05). A significant increase of the G0 /G1 and S fraction was seen in the ECV cells of mutant group, compared with wild group with an accumulation in S phase and a concomitant decrease in G2 /M phase population (P< 0. 05). Conclusion Mutant and wild EDA‐A1 gene may have distinct biological functions on proliferation and cell cycle distribution of cultured human umbilical vein endothelial cell.

The expressions of estrogen receptor (ER) α,ERβ1,and ERβ2 in thyroid carcinoma,thyroid adenoma,and adjacent normal thyroid tissue were determined by RT-PCR and immunohistochemistry,and the relationship of expression levels of three ERs and the main clinical pathologic parameters were evaluated.The result showed that ERα mRNA expression in papillary thyroid carcinoma(PTC) tissues was significantly higher than adjacent normal tissue,while,ERβ2 mRNA expression in PTC tissues was significantly lower than normal tissue ( P＜0.05 ).The positive expression of ERβ1 and ERβ2 in the thyroid carcinoma was related with the histological variant of carcinomas; the ERβ2 expressions were related with the lymph node metastasis and TNM staging of the carcinomas.These results suggest that the decreased ERβ2 expression may induce proliferation and carcinogenesis of thyroid follicular epithelial cells as well as progression of the carcinoma,and is a powerful prognostic indicator.

Objective To evaluate the value of dual echo technique in combination with apparent diffusion coefficient(ADC) in differentiating acute benign vertebral fracture from metastasis.Methods 43 patients (mean age, 68.2 years) with 79 metastatic vertebrae and 59 patients(mean age,60) with 68 acute vertebral fractures were included in our study.The slices of largest size of lesion on T1WI were selected to measure and calculate the signal intensity ratio(SIR) and ADC of lesions.The difference of ADC values and SIR between benign acute vertebral fractures and vertebral metastasis were compared.Parallel test,serial test and receiver operating characteristic curve(ROC) analysis of logistic model were done.Results ADC values of metastasis and acute benign fracture were (1.32±0.5)×10~（-3）mm~2/s and (2.39±0.4)×10~(-3)mm~~2/s respectively,and SIR of them were 1.00±0.01 and 0.85±0.01 respectively,there were significantly different between these two entities with the two tests(P<0.05).The area under the curve(AUC) for SIR was 0.897 and for ADC was 0.960,there is significantly different between these two tests in AUC(P=0.02).When 0.97 as cut off value for SIR,the sensitivity and specificity were 84.81% and 76.47%% respectively,when 1.87×10~(-3)mm~2/s as cutoff value for ADC , the sensitivity and specificity were 93.67% and 91.18% respectively . The sensitivity and specificity were 99.04% and 69.73% respectively in the parallel tests and 79.44%,97.92% in the serial test.A combining predictor established with logistic regression model showed a high AUC:0.985,which was higher than that of ADC and SIR alone.The AUC under ROC for ADC was significantly larger than that of SIR . When 0.55 as cut off value for this combining predictor , the sensitivity and specificity were 98.73% and 95.59% respectively.Multiple comparisons were performed with LSD-t test :combining predictor>ADC>SIR,it showed that there was significantly different between indexes(P<0.05). Conclusion ADC in combination with SIR can improve the effects in differentiating benign acute vertebral fractures and vertebral metastasis.

Objective To analyze and evaluate changes of the brain gray matter in patients with Parkinson's disease (PD).Methods 46 patients with PD and 19 normal control(NC) subjects(matched to the patients in age and gender) were selected in this study.46 cases of PD were divided into early PD group(ePD,25 cases) and middle-advanced PD group(maPD,21 cases) by improved Hoehn-Yahr(H-Y) stages.All the subjects underwent 3.0T MR scanning,and data of high resolution T1-weighted imageing (T1WI) were acquired.Gray matter volume differences between PD group and NC group,or ePD group and maPD group were assessed by voxel-based morphometry (VBM) combined diffeomorphic anatomical registration through exponentiated lie (DARTEL) method.Results The gray matter volume of the bilateral frontal lobes, temporal lobes, insular lobes, cingulum gyrus, hippocampus, fusiform gyrus, cerebellum and right cuneus lobe and precuneus lobe in the PD group was smaller than that in the NC group.The gray matter volume of the bilateral frontal lobes, temporal lobes,insular lobes,cingulum gyrus,rectus gyrus,lingualis gyrus,fusiform gyrus,hippocampus,amygdaloid and cerebellum in the maPD group was smaller than that in the ePD group.Conclusion VBM reveals a widespread volume reduction of the gray matter in PD patients,and detectes a correlation with disease duration and severity.These changes located in special distribution may be in line with the pathology of PD.

Objective To investigate the effect of Wnt1 on the expression of Cyclin D1 in human corneal epithelial cells and its related molecular mechanisms. Methods 12 T25 cell culture flasks were cultured after hu-man corneal epithelial cells anabiosis ,culture and continuous passage for 2 times. Culture flasks were divided into 3 groups with 4 culture flasks in each group. Twenty-five ng/mL and 50 ng/mL recombinant human Wnt1 protein were added in two of the groups,and one group without T-cell culture medium(Wnt1)was used as control. Cells cultured in T25 flask were taken from three groups at different time(6 h,24 h,48 h and 72 h). The total number of corneal epithelial cells in each group was calculated. Expression of Cyclin D1 in corneal epithelial cells was de-tected by Western blot. Results The expression of Cyclin D1 protein in the control group decreased gradually from 0 h to 48 h,and reached the lowest level at 48 h and increased at 72 h. Cyclin D1 protein expression in 25 ng/mL group at 6 h after Wnt1 was added was not detected,and Cyclin D1 protein expression in 50 ng/mL group in-creased. The expression of Cyclin D1 protein in 25 ng/mL group and 50 ng/mL group was significantly higher than that in control group at 24 h,48 h and 72 h,reaching the peak at 48 h and decreased at 72 h. Compared with the control group,the growth rate of corneal epithelial cells in 25ng/ml group and 50ng/ml group increased after Wnt1 was added. There was significant difference in 72 h,but no significant difference in 6h,24h and 48h. Conclu-sions The stimulation of Wnt1 protein can enhance the expression of Cyclin D1 in a certain time range,and has a positive correlation with Wnt1 protein. As one of the target genes of Wnt1 signaling pathway,Cyclin D1 may play an important role in the repair of corneal epithelial injury and its cell proliferation and differentiation.

mRNAs of alpha-adrenoceptor (α-AR) subtypes are found in neurons in dorsal root ganglion (DRG) and change after peripheral nerve injury. In this study, the distribution of α-AR subtype proteins was studied in L5 DRG of normal rats and rats with chronic constriction injury of sciatic nerve (CCI). Using immunofluorescence technique, it was found that α1A-, α1B-, and α2A-AR proteins were expressed in large, medium, and small size neurons in normal DRG, and significantly increased in all size neurons 14 days after CCI. α1D- and α2C-AR was also expressed in all size neurons in normal DRG. However, α1D-AR was significantly increased and α2C-AR was decreased in small size neurons 14 days post CCI. α2B-AR neurons were not detectable in normal and CCI DRG. Co-expression of α1A- and α2A-AR in the same neuron was observed in normal DRG and increased post CCI. Collectively, these results indicated that there is distinct distribution of α-AR subtypes in DRG neurons, and the distribution and levels of expression of α-AR subtypes change differently after CCI. The up-regulation of α-AR subtypes in DRG neurons may play an important role in the process of generating and transmitting neuropathic pain.

Background The deficiency of limbus stem cells will cause various kinds of the disorder of eye surface,in serious condition,it will lead to corneal conjunctivalization,chronic inflammation,lasting deficiency of corneal epithelium and corneal opacity accompanying long-term visual loss.Objective The aim of this study was to investigate how to establish and evaluate the successful model of total limbal stem cell deficiency (LSCD).Methods Twenty Japanese white rabbits aged 3-5 months were selected.The limbus corneal epithelium was removed from 1 mm inner to 2 mm outer of the limbus 100-150 μm in deep,and the total central epithelium and shallow stroma were simultaneously removed in the left eyes using scalpel.The experimental eyes were examined by slitlamp 2,3,4,5 weeks after operation,and the inflammatory score was performed according to corneal opacity,corneal neovascularization and corneal flurescein dye using international universal rating criteria of LSCD model.Cornea tissue was obtained in various time points mentioned above to examine the structural change and goblet cells.The expression of cytokeratin 3(CK3) in the cornea was detected using indirect immuno-fluorescence staining.Fisher Exact Probability method was used to compare the successful rate of models in different time points.Results The successful rate of models rate was 12.5％,62.5％,81.3％ and 87.5％,respectively 2,3,4,5 weeks after operation,showing a significant difference between the 3-week group and 2-week group (P =0.009).However,no significant differences were found in the model successful rate between the 3-week group and the 4-week group,the 4-week group and the 5-week group (P =0.465,0.200).There was a little difference between the 3-week group and the 5-week group(P=0.049).The mean time of achieving successful model was(3.21 ±0.80) weeks.The result of hematoxylin & eosin staining showed the edema of cornea stroma,a mass of inflammatory cells infiltration and new vessels.Goblet cells could be found by Periodic acid-Schiff staining 5 weeks after operation.Expression of CK3 was absent in the corneas of successful model eyes.Conclusions The LSCD model can be successfully established by cutting off the epithelium of limbus and central cornea,and the average time of model formation is about 3.2 weeks.