Objective To study the clinical and imaging features of Marinesco Sjgren syndrome.Methods The clinical,laboratorial and imaging information of a patient with Marinesco Sjgren syndrome (28 years old, female) was reported.Results The clinical findings included besides the major features of the syndrome, e.g. ataxia, cataracts, growth deficiency and mental retardation,chronic renal deficiency, hypoparathyroidism and hypogonadism. Karyotype of chromosome was 46,XX,1q +,22s +. CT scan showed stripped path like high density in both lenticular nucleus, cerebellar atrophy; MRI showed cerebellar vermis and hemispheres of small volume. Other abnormal superatentorial findings were showed in MRI, including enlarged third, fourth and lateral ventricles,and expanded cistern;pachygyria in left temporal lobe and a thin corpus callosum lap.Conclusion The major features of Marinesco Sjgren syndrome were growth deficiency and mental retardation,congenital cataracts, cerebellar ataxia, etc.Skull MRI might be helpful in the early diagnosis of the disorders.

Objective To investigate the effect of dexmedetomidine on the stress responses during wakeup test in patients undergoing cerebral functional area operation performed under propofol combined with remifentanil anesthesia.Methods Thirty-six ASA physical status Ⅰ or Ⅱ patients,undergoing cerebral functional area operation requiring wake-up test,aged 18-60 yr,weighing 50-70kg,were randomly divided into control group (group C) or dexmedetomidine group (group D) with 18 cases in each group.Dexmedetomidine 0.8 μg/kg was infused over 10 min before induction of anesthesia followed by infusion at 0.4 μg·kg-1 · h-1 in group D,while the equal volume of normal saline was infused in group C.Anesthesia was induced with target-controlled infusion of propofol and remifentanil and iv injection of cisatracurium.At 30 min prior to wake-up test,target-controlled infusion of propofol and application of mulscle relaxants were stopped,the target plasma concentration of remifentanil was decreased to 1 ng/ml,and in group D the infusion rate of dexmedetomidine was decreased to 0.1 μg·kg 1· h-1.Anesthesia time and consumption of anesthetics before wake-up test,wake-up time,and development of complications and intraoperative awareness during wake-up test were recorded.At 30 min prior to wake-up test (T1),immediately after wake-up (T2),at 5 min after wake-up (T3),and at 10 min after the anesthetic depth was deepened (T4),HR,mean arterial pressure and BIS value were recorded and blood samples were taken for determination of plasma concentrations of epinephrine (E) and norepinephrine (NE).Results Compared with group C,the consumption of propofol and remifentanil was significantly reduced before wake-up,the incidence of hypertension was decreased during wake-up test,and HR and plasma E and NE concentrations were decreased at each time point (P ＜ 0.05),and no significant difference in mean arterial pressure and BIS value was found in group D (P ＞ 0.05).Tachycardia,restlessness,bucking and awareness were not observed during wake-up test in group D.Conclusion Dexmedetomidine can inhibit the stress responses during wake-up test and raise the quality of wake-up test in patients undergoing cerebral functional area operation performed under propofol combined with remifentanil anesthesia.

Objective To investigate the effects of trimetazidine on renal function in patients with shock.Methods A prospective randomized controlled double-blind study was conducted.128 patients with shock admitted to intensive care unit (ICU) of Guangzhou Red Cross Hospital from April 2011 to April 2013 were enrolled and randomly divided into control group and trimetazidine treatment group,each n＝64.All patients received anti-shock treatment,while the patients in trimetazidine group received trimetazidine treatment (20 mg orally,tid) for 7 days,and patients in control group received placebo (10 mL of sterile water for injection,tid) for 7 days.The urinary output,serum creatinine (SCr),blood urea nitrogen (BUN),cystatin C,and creatinine clearance (CCr) reflecting renal function were recorded in both groups,and the values were compared before treatment,48 hours after treatment,and 1 week after the treatment.At the same time,dynamic mean arterial pressure (MAP) was monitored,and 48-hour and 1-week mortality rates were recorded.Results There was no significant difference in results in all the renal function parameters before the treatment between two groups.The levels of SCr,BUN,cystatin C were gradually decreased after treatment in both groups,but CCr and MAP were gradually increased.Compared with the control group,cystatin C at 48 hours after treatment was significantly decreased,while CCr was significantly increased in treatment group [cystatin C (mg/L):0.85 ± 0.81 vs.1.01 ± 0.91,t=2.562,P=0.017; CCr (mL/s):0.93 ± 0.64 vs.0.69 ± 0.40,t=2.155,P=0.033].SCr and BUN at 1 week after treatment were significantly decreased in treatment group [SCr (lμmol/L):94.23 ± 88.31 vs.104.99 ± 98.37,t=2.921,P=0.003 ; BUN (mmol/L):9.46 ± 8.24 vs.11.87 ± 8.65,t=2.611,P=0.010].Urine output per hour and MAP was improved after treatment in both groups,and no significant difference was found between treatment group and control group [urine output (mL):48 hours after treatment 55.67 ± 31.43 vs.45.34 ± 11.79,t =0.934,P=0.323 ; 1 week after treatment 71.67 ± 37.23 vs.75.35 ± 22.88,t=1.280,P=0.210; MAP (mmHg,1 mmHg=0.133 kPa):48 hours after treatment 72.13 ± 33.24 vs.69.28 ± 39.98,t=1.408,P=0.179; 1 week after treatment 71.44 ± 21.98 vs.72.32 ± 31.11,t =1.184,P =0.252].Mortality rate in treatment group was lowered compared with control group,however no statistical significance was found [48 hours after treatment:31.2％ (20/64) vs.32.8％ (21/64),x2=0.084,P=0.785; 1 week after treatment:32.8％ (21/64) vs.35.9％ (23/64),x2=2.084,P=0.173].Conclusions Trimetazidine can improve renal function in patients with shock.

Objective To investigate the effect of theanine pretreatment on DNA repair function in neurons during brain ischemia-reperfusion (I/R) injury in rats.Methods One hundred and eight male Sprague-Dawley rats,weighing 290-310 g,aged 15 weeks,were randomly divided into 3 groups (n =36 each) using a random number table:sham operation group (S group),I/R group and theanine pretreatment group (T group).Global cerebral I/R was produced by 4-vessel occlusion method.Bilateral vertebral arteries were electrically cauterized,and bilateral common carotid arteries were clamped for 6 min.Theanine 1 g/kg was injected intravenously at 4 h before clamping bilateral common carotid arteries in T group,and the equal volume of normal saline was given in the other two groups.At 2,6,12,24,48 and 72 h of reperfusion,6 rats were selected in each group and sacrificed,the brains were removed,and the hippocampus was isolated for determination of the number of viable neurons in the hippocampal CA1 region (with a light microscope),apoptosis in neurons in the hippocampal CA1 region (by TUNEL),and expression of DNA repair protein X-ray repair cross-complementing group 1 (XRCC1) and Ku70 (by immunohistochemistry).The apoptotic index was calculated.Results Compared with S group,the number of viable neurons was significantly decreased,and the apoptotic index was significantly increased at 6,12,24,48 and 72 h of reperfusion,and the expression of XRCC1 and Ku70 was significantly down-regulated at 2,6,12,24,48 and 72 h of reperfusion in I/R group (P＜0.01).Compared with I/R group,the number of viable neurons was significantly increased at 12,24,48 and 72 h of reperfusion,the apoptotic index was significantly decreased at 6,12,24,48 and 72 h of reperfusion,and the expression of XRCC1 and Ku70 was significantly up-regulated at 2,6,12,24,48 and 72 h of reperfusion in T group (P ＜ 0.01).Conclusion The mechanism by which theanine pretreatment attenuates brain I/R injury is related to enhancement of DNA repair function and reduction of neuronal apoptosis in rats.

Objective Analysis of MRI image of lateral ventricular subependymomas to improve our recognition of radiology manifestation of the disease. Methods Clinical data and MRI findings of 6 patients with subependymomas proved by surgical pathology, observed from January 2007 to June 2013, were analyzed retrospectively. Results Six patients (4 males and 2 females) presented with headache and dizziness. Among these 6 patients, tumor developed in right lateral ventri-cle in three patients, in left lateral ventricle in 2 patients and in frontal of dual lateral ventricle in one patient. In MRI image, tumors showed iso-intense to hypointense to normal white matter on T1-weighted and DWI images, hyperintense on T2-weighted and FLAIR. Mild or no enhancement was noted in most cases. MRS showed findings consistent with low-grade tu-mor, with a normal choline peak and depressed N-acetyl-aspartate peak. Conclusion Lateral ventricular subependymo-mas have characteristic MRI features and multi-direction images are helpful for diagnosis.

Objective: To explore therapeutic effect of modified constraint-induced movement therapy on rehabilitation in patients with stroke hemiplegia.Methods: A total of 136 patients with stroke hemiplegia were selected and divided into routine rehabilitation group and modified rehabilitation group.After 12 weeks, scores of Fugal-Meyer motor assessment (FMA), modified Barthel index (MBI) and Berg balance scale (BBS), 6min walking distance (6MWD), psychological state and quality of life (QOL) were compared between two groups.Results: After 12 weeks, there were significant rise in scores of FMA, MBI and BBS and 6MWD, and significant reductions in scores of self-rating anxiety scale (SAS) and self-rating depression scale (SDS) in two groups, P<0.05 all.Compared with routine rehabilitation group, there were significant rise in scores of FMA [(53.23±5.13) scores vs.(60.67±5.67) scores], MBI [(57.83±6.11) scores vs.(69.93±6.74) scores] and BBS [(41.45±4.12) scores vs.(50.53±4.56) scores] and 6MWD [(179.46±24.62)m vs.(211.34±26.71)m], and significant reductions in scores of SAS [(50.75±2.56) scores vs.(41.11±1.53) scores] and SDS [(51.34±3.35) scores vs.(40.78±2.11) scores] in modified rehabilitation group, P<0.05 all.Compared with before rehabilitation, there were significant rise in physiological, psychological, social and setting dimension scores in both groups after rehabilitation, and those of modified rehabilitation group were significantly higher than those of routine rehabilitation group, P<0.05 all.Conclusion:Modified constraint-induced movement therapy can significantly improve movement, balance function, and anxiety, depression and daily living capacities in patients with stroke hemiplegia.

Objective To observe the effect of aspirin on phtelets activation markers in patients with coronary heart disease and set up a diagnostic criteria of aspirin resistance.To preliminarily predict the incidence of aspirin resistance in hospital patients.Methods The subjects were divided into 3 groups:aspirin group(103 cases),control group(24 eases),and healthy control group(23 cases).Using whole blood samples,we detected the ratio of CD62P,PAC-1 expression by flow cytometry(FCM)before and after 7-day treatment and compared the changes of CD62P and PAC-1 expression ratio,then calculated the inhibition ratio of platelets glycoprotein,set up the diagnostic criteria of aspirin resistance with receiver operator characteristic curve(SOC)and calculate the incidenee of aspirin resistance in hospital patients.Results The statisticsl reaults are listed as below:in asptirin group,before treatment CD62P(10.16±6.80)%,PAC-1(14.66±10.56)%,and after treatment CD62P(5.70±4.28)%,PAC-1(8.93±7.08)%,P＜0.01.In control group,before treatment CD62P(9.14±6.52)%,PAC-1(17.67±11.53)%,and after treatment CD62P(7.81±5.72)%,PAG-1(14.97±8.05)%,P＜0.05.According to ROC,the inhibition ratio of CD62P＜21.5% or PAG-1＜17.7% was individually set up asdiagnostic criteria of AR.Our study indicate that the incidence of aspirin resistance in hospital CHD patients is 17.5%.Conclusion There exists platelet activation in CHD patients.CD62P and PAC-1 could be considered as the sensitive index of platelet activation and used in the evaluation of anti-platelet therapy.Aspirin can decrease the expression of CD62P and PAC-1,and inhibit the activation of platelet.According to this study,aspirin resistance really exists in CHD patients.By FCM,the diagnostic criteria of aspirin resistance in CHD is the inhibition ratio of CD62P＜21.5% or PAC-1 ＜17,7% due to aspirin.The incidence of aspirin resistance in hospital CHD patients is 17.5%.

Objective To study the over-expression and clinical implications of the oncogene MDM2 in acute leukemia (AL). Methods The expression of MDM2 gene in 100 patients with newly diagnosed and relapse or refractory AL and 20 healthy as control was measured by relative quantitative reverse transcriptase polymerase chain reaction (RT-PCR),then the results was measured by χ2-test,t-test and one-way ANOVA to compare expession positive rate and intensity of MDM2. Results Among 100 patients,fifty-eight had the high expression of MDM2 gene (58 %). The expression level of MDM2 gene in patients was higher than that of health controls(P ＜0.05). The expression positive rate of MDM2 is higher in poor outcome group (67.9 %,19/28)than that in general outcome group (33.9%,19/56) (P＜0.05). Conclusion Our results suggest that the expression of MDM2 gene plays an important role in the pathogenesis and poor outcome of AL.

Objective To explore crossed cerebellar diaschisis (CCD) in cerebral gliomas with three dimensional arterialspin-labeling (3D ASL) perfusion MRI.Methods The images of 31 patients with cerebral gliomas and 31 normal subjects were retrospectively analyzed.The cerebral blood flow (CBF) were measured with 3D ASL technology,and the asymmetry index (AI) of cerebellar hemispheric CBF value changes were calculated and compared in patients.And the relationship between the AI values of cerebellar hemispheric and the AI values of tumor region in cerebral hemispheric,tumor histological grade and size were investigated.Results Compared with tumor ipsilateral cerebellar hemisphere of supratentorial glioma,the CBF value of contralateral cerebellar hemisphere significantly reduced (t=5.04,P＜0.01),and the AI values of cerebellar hemispheric in patients obviously increased compared to normal subjects (t=4.13,P＜0.01).But there was no significant difference in the AI values of cerebellum between high grade and low grade gliomas (t=1.31,P＞0.05).In cerebral gliomas patients,there was no significant correlation between the AI values of cerebellar hemispheres and the AI values of cerebral hemisphere (tumor:r=-0.28;tumor parenchyma:r=-0.24;tumor plus edema:r=-0.19,all P＞0.05),and tumor size (r=0.18,P＞0.05).Conclusion Cerebral glioma can cause CCD phenomenon,and 3D ASL is able to quantitatively assess the degree of cerebellar hypoperfusion noninvasively.This phenomenon may not be associated with tumor histological grade,size and the AI values of cerebral hemispheric.

Objective To evaluate the protective effect and mechanism of luomaishutong(LMST)on acute myocardial ischemia-reperfusion injury in rabbits.Method Twenty-four New Zealand white rabbits were randomly divided into 3 groups:the LMST group,the control group and the sham-operated group.The AMI reperfusion model was established by removing the blockade after the occlusion of coronary artery for 2 hours.The changes of hemodynamics,oxygen free radical and clearance system were measured in all rabbits.Results (1)Compared with the control group,left ventricular systolic pressure(LVSP)and maximal changing rate of left ventricular innner pressure(?dp/dt_(max))increased remarkably in the LMST group after reperfusion,meanwhile, LVEDP decreased significantly.(2)In the control group,MDA level of cardiocyte was noticeably higher,while SOD and NOS levels were lower than in the sham-operated group.Compared with the control group,MDA level in the LMST group was significantly lower.Furthermore,SOD and NOS levels were higher in LMST group,and the infarcted area was smaller in the LMST group as well.Conclusions LMSF can protect myocardium after ischemia-repcrfusioninjury and improve cardiac function through inhibiting induced by oxygen free radicals.