Paroxysmal nocturnal haemoglobinuria ( PNH) is a clonal hematopoietic stem cell disease characterized by intravascular hemolytic anemia , pancytopenia and thrombosis .Although PNH is an non-malignant disease , its complications have very negative impacts on patient's quality of life .The most common serious complication is thrombosis formation .

Objective To evaluate the effects of dexmedetomidine on postoperative cognitive function in the aged patients undergoing carotid endarterectomy.Methods Forty patients,aged 65-80 yr,of ASA physical status Ⅱ or Ⅲ,scheduled for elective carotid endarterectomy,were randomly divided into 2 groups (n =20 each):control group (group C) and dexmedetomidine group (group DEX).Anesthesia was induced with midazolam,etomidate,fentanyl and rocuronium.The patients were tracheally intubated.In group DEX,a loading dose of dexmedetomidine 0.03 μg· kg-1 ·min-1 was infused intravenously for 10 min starting from the time point before induction,and dexmedetomidine 0.30μg· kg-1 ·min-1 was infused until 30 min before the end of operation starting from the end of intubation.The equal volume of normal saline was given instead of dexmedetomidine in group C.At 1 day before operation (To) and 6 and 24 h after operation (T1.2),venous blood samples were collected for determination of serum brain-derived neurotrophic factor (BDNF) concentrations.The cognitive function of the patients was assessed using Mini-Mental State Examination (MMSE) at To,T2,and 48 h,72 h,7days and 1 month after operation (T3-6).Results Compared with the baseline value at T0,the serum BDNFconcentrations were significantly increased at T1 in the two groups,MMSE scores were decreased at T2,3 in groupC,and MMSE scores were decreased,and the serum BDNF concentrations were increased at T2 in group DEX.Compared with group C,the MMSE scores were significantly increased at T3.4,the serum BDNF concentrations were increased at T2,and no significant change was found in MMSE scores at T5.6 in group DEX.Conclusion Dexmedetomidine is helpful in improving postoperative cognitive function and in promoting the recovery of postoperative cognitive function,and the mechanism may be related to enhanced production of endogenous BDNF in the aged patients undergoing carotid endarterectomy.

To investigate the plasma thioredoxin-interacting protein ( TXNIP ) levels in different glucose tolerance groups and discuss the relationship between TXNIP and insulin resistance/β-cell dysfunction in diabetes and prediabetes, and to investigate the potential relationship between TXNIP and interleukin-1β( IL-1β) . According to oral glucose tolerance test, 93 participants were divided into 3 groups:diabetes mellitus group, prediabetes group, and normal glucose tolerance group. Plasma TXNIP, IL-1β, and other biochemical indices were measured. The relationship between TXNIP and glucose, IL-1β, homeostasis model assessment for insulin resistance ( HOMA-IR) , and homeostasis model assessment forβcell function ( HOMA-β) were analyzed by using multiple linear regression techniques and Pearson’s linear correlation analysis. Plasma TXNIP level was higher in prediabetes group compared with normal glucose tolerance group, but lower in prediabetes group compared with diabetes mellitus group[(355. 35±31. 88 vs 274. 36±33. 86, 426. 16±63. 15)pg/ml, P<0. 01 or P<0. 05]. TXNIP was positively correlated with IL-1βand HOMA-IR, but negatively correlated with HOMA-β. Multiple linear regression analysis indicated that IL-1βexerted significant influence on TXNIP ( P<0. 05 ). Plasma TXNIP level is affected by blood glucose concentration. There is a close relationship between TXNIP and IL-1β. In prediabetes patient, the TXNIP levels have already been raised.

Purpose To investigate the expression and clinical significance of Foxp3 ( cell surface marker of regulatroy T) mRNA and its protein in endometrial carcinomas and normal endometrial tissues. Methods Real-time fluorescence quantitative PCR and immuno-histochemical SP methods were used to detect the expressions of mRNA and protein in tumor tissue of 84 cases of endometrial carcino-mas and 40 cases of normal endometrial tissue, then to analyze the relationship between Foxp3 gene and clinical pathological character-istics of endometrial carcinoma specimens, such as differentiation, FIGO stage. Results Foxp3 mRNA and it′s protein expression of endometrial carcinomas were significantly higher than that of normal endometrial tissues. There were significantly relationships between Foxp3 mRNA expression and FIGO stage of endometrial cancer, Foxp3 mRNA expressions of III+IV stage was higher than that ofⅠ+Ⅱ stage endometrial carcinoma (P<0. 05). But the relationship between Foxp3 expression and differentiation degree reached differ-ent conclusions in the two detection methods. By immunohistochemistry the expression of Foxp3 protein was correlation with histological differentiation grade (rs =0. 72, P <0. 01). In poorly differentiated endometrial carcinoma Foxp3 + cell number was significantly higher than that in well differentiated endometrial carcinoma. By detection of real-time fluorescence quantitative PCR method, Foxp3 mRNA expression was not correlated with tumor grade (rs =0. 01, P=0. 35). Conclusion Foxp3 in endometrial carcinomas are high expressions. Immunohistochemical method has more clinical value than real-time fluorescence quantitative PCR test results. Foxp3 may be involved in the regulation of the development of endometrial cancers.

Objective:To investigate the role of p21 gene in the radiation-induced heart disease (RIHD) and to evaluate the effect on p21 gene knockout on RIHD phenotype in mouse models.Methods:p21 -/-mice were utilized in the experimental group, and p21 + /-mice were allocated in the control group. RIHD mouse models were established by exposure to 10 Gy whole heart irradiation by using a small animal radiation research platform. The heart samples were collected at 6 weeks after irradiation, the gross specimens were measured and subject to HE staining. The wall thickness and left ventricular ejection fraction of the mice were detected by the Vevo2100 ultrasound imaging system. The hypoxia in cardiac tissues was detected by the hypoxia probe method. The apoptosis of cardiac cells was determined by Tunel method. Results:Compared with the p21 + /-mice, the survival of p21 -/-mice was significantly shortened ( P=0.004), the interventricular septum was significantly thinned during the diastolic and systolic phases ( P=0.049, P=0.006), the left ventricular posterior wall was remarkably thickened ( P<0.001) and the left ventricular ejection fraction was significantly decreased ( P=0.004). The gross heart tissue was enlarged in the p21 -/-mice. HE staining showed the aggregation of inflammatory cells in cardiac tissues and disordered arrangement of myocardial cells. Significant hypoxia and apoptosis could be observed in the p21 -/-mouse heart tissues. Conclusions:p21 -/-mice are prone to more severe RIHD after irradiation, manifested with shortened cardiac survival, weakened cardiac function, abnormal cardiac structure, hypoxia and apoptosis of cardiac tissues. p21 plays an important role in the repair after cardiac irradiation.

OBJECTIVETo explore the high risk factors of thrombosis in paroxysmal nocturnal hemoglobinuria (PNH). It has been reported that in Chinese patients with venous thrombosis, the mutation frequency in PROC c.574_576 del (rs199469469), PROC c.565C>T (rs146922325) and THBD c.-151G>T (rs1698852) was higher than that of normal controls, indicating its importance in thrombophilia pathogenesis.

METHODS142 patients with PNH diagnosed between 2009 and 2015 were enrolled in the study. Clinical data were analyzed and thrombophilia risk factors, such as the level of protein C, protein S, antithrombin III, APC resistance, blood fat, phospholipid antibody, were evaluated. Samples from patients and 100 normal controls were detected for the mutations of PROC c.574_576 del (rs199469469), PROC c.565C>T (rs146922325) and THBD c.-151G>T (rs1698852) by Sanger sequence.

RESULTSOf the 142 PNH patients, 21 (14.8%) patients had at least 1 episode of thrombotic event. Only 2 patients had arterial thrombosis and 19 patients had venous thrombosis. The median age of patients with thrombosis was 35 years old, similar to those without episode (40 years old, P=0.687). The ratios of males and females were 1.33 in thrombosis group and 1.57 in non-thrombosis group (P=0.728) , respectively. Patients with thrombosis had the same disease pattern compared with those without episode. Although there was no difference in the level of hemoglobin, WBC and PLT count, and LDH level between patients with thrombosis and those without episode, patients with thrombosis showed higher RBC, higher percentage of CD59(-) granulocytes and RBC, and Flaer(-) granulocytes compared with those without episode. The routine thrombophilia screening tests did not show any difference either between PNH patients and normal controls, or between patients with or without thrombosis. There were two mutations in rs199469469 and rs16984852 sites in patients with PNH, but the mutated patients did not have any thrombosis. Mutation rs146922325 was found in PNH patients. The mutation rate was similar between PNH patients and normal controls, thrombotic PNH and non-thrombotic PNH (P>0.05).

CONCLUSIONSCompared with non-thrombotic patients, PNH thrombotic patients have bigger PNH clone and higher RBC count. There are no differences among the routine thrombophilia factors and the three known venous eligible genes either between PNH patients and normal controls or between thrombotic and non-thrombotic PNH patients.

Adult ; Antithrombin III ; metabolism ; Case-Control Studies ; Clone Cells ; cytology ; Female ; Granulocytes ; cytology ; Hemoglobinuria, Paroxysmal ; genetics ; physiopathology ; Humans ; Leukocyte Count ; Male ; Protein C ; metabolism ; Protein S ; metabolism ; Risk Factors ; Thrombosis ; genetics ; physiopathology

Objective To compare the dosimetric effects of micro-multileaf collimator (MLC)(2 mm leaf width) and conventional MLC (10 mm leaf width) on inverse intensity-modulated radiotherapy(IMRT) in intracranial stereotactic radiosurgery(SRS). Methods In view of the fact that the micro-MLC has a small open field,30 patients with intracranial tumor with a<10 cm diameter were enrolled in this study. Their inverse dynamic IMRT plans were established using conventional MLC (conventional group) and micro-MLC (micro group) with the same other conditions. The radiation doses to the target volume and the organs at risk (OAR) were compared between the two groups with t test. Results Compared with the conventional group, the micro group had a significantly better dose distribution in the target volume (P=0.019). However, there were no significant differences in D98,D95,D50,and D3between the two groups (P=0.774,0.650,0.170,0.080). The micro group had a 58.7% lower mean homogeneity index and a 20.1% higher mean conformity index than the conventional group (P=0.000). The micro group had significantly lower radiation doses to OAR than the conventional group (P=0.044). The mean Dmeanand Dmaxof the brain stem in the micro group were 10.0% and 8.2%,respectively,lower than those in the conventional group (P=0.768,0.753). The mean Dmeanand Dmax of the right eye and left eye in the micro group were 16.5%,19.3%,21.4%,and 13.4%,respectively,lower than those in the conventional group (P=0.572,0.775 and 0.734,0.630). The mean Dmaxof the left lens, right lens, left optic nerve, right optic nerve, and optic chiasm in the micro group were 50.4%, 24.1%, 38.5%, 27.8%, and 5.7%, respectively, lower than those in the conventional group (P=0.172,0.467, 0.521,0.740,0.899). The PRV100,PRV50,and PRV25of the normal tissue in the micro group were no more than those in the conventional group(P=0.839,0.832,0.972). Conclusions In inverse IMRT in intracranial SRS,micro-MLC is better than conventional MLC because it can improve CI of the target volume and reduce the radiation doses to OAR.

OBJECTIVETo explore the clinical characteristics of cataract surgery after radial keratotomy (RK) and appropriate calculation of intraocular lens (IOL) power.

METHODSEight patients with cataract (12 eyes) after RK were treated in our hospital from March, 2010 to June, 2013. The visual acuity, keratometric power and length of the ocular axis were examined before the operation. For each patient, 3 groups of corneal curvature values were measured using a automatic keratometer (TOPCON-KR8800) and the minimal K value was selected. Myopic or hyperopic posterior chamber IOL (-1.00 to -2.00 D) were selected based on automatic calculations with SRK-T. Phacoemulsification and IOL implantation were then performed, and the patients were followed up for visual acuity and refractive statuses at 3 months after the operation.

RESULTSAll the 12 operated eyes showed improved visual acuity after the operation. The uncorrected visual acuity reached 0.8 to 1.0 in 6 eyes and 0.4 to 0.6+ in the other 6, with a corrected visual acuity ranging from 0.6 to 1.0. The refractive status after operations was nearly emmetropic (+0.75 to -1.00 D) in 6 eyes and myopic in the other 6 (-1.00 to -2.50 D).

CONCLUSIONSPhacoemulsification and IOL implantation is feasible in cataract patients with previous RK. Selecting the minimal K values for central corneal curvature and calculation of the IOL power using the SRK T equation with a reservation of -1.00 to -2.00 D can better ensure the safety of the procedure and avoid the occurrence of hyperopia >+3.00D.

Cataract Extraction ; Cornea ; Humans ; Hyperopia ; Keratotomy, Radial ; Lens Implantation, Intraocular ; Lenses, Intraocular ; Myopia ; Phacoemulsification ; Visual Acuity

OBJECTIVE Congenital aural atresia repair is difficult owing to unpredictable anatomy. Benefits may be gained from image-guided surgery(IGS) . its exact role and surgery indication were def ined. METHODS From 2001 to 2004,36 ears with bony type C(Schuknecht classification) congenital atresia were performed. In the IGS group(n=18) ,repair surgery was performed with IGS,while in the control group(Non-IGS,n=18) ,similar intervention was applied without IGS. IGS group:aged from 12-29 years,follow-up from 6 months to 1 year. Non-IGS group:aged from 10-27 years,follow-up from 6 months to 3 years. Intra-and post-operative clinical and audiometric findings were compared. RESULTS All of the patients had congenital bony aural atresia,ossicles malformation,tympanic cavity hypoplasia and facial nerve malformation. IGS revealed a malformed horizontal semicircular canal hidden in the bony atresia plate during the operation while computed tomography(CT) did not show preoperatively. IGS computed tomography images correlated well with intra-operative findings,gave the surgeon more securityand reduced operative time(2 hours and 24 minutes) by 25 minutes. The prepare time increased 20 minutes(15-30 minutes) ,but total time decreased 5 minutes in IGS group. The registration accuracy was 0.6-1.3 mm,average 0.84 mm,which was suitable for the otologic surgery. There were 1 case in IGS group and 3 cases in Non-IGS group happened local aural restenosis after operation. But there were no facial nerve paralysis and hearing injury happened in both groups,and all of the patients got the satisfactory hearing after the hearing reconstruction(the air-bone gap with an average of IGS is 31.8dB,Non-IGS is 30.5dB) . CONCLUSION In our estimation,IGS is valuable for type C congenital aural atresia repair. It serves as an educational tool and a guide both for the experienced and inexperienced surgeons in critical situations where anatomical landmarks are distorted and approach is limited. There is no statistically significant between two groups on hearing improvement after operation.

Walking disorder is the most common sequela in patients with hemiplegia after stroke.More than half of stroke patients can not walk independently after discharge,and still present a gait asymmetry and slow pace,and other abnormalities.Then,the fall risk is increased.With security concerns,patients are more afraid to walk independently and can not complete the walk in community environment.Therefore,gait rehabilitation is considered as the main goal of stroke rehabilitation.Rhythmic auditory stimulation(RAS)is a new rehabilitation method to improve stroke gait.By elucidating the mechanism of RAS and their effects on stroke gait,the aim of this paper is to explore the effective RAS treatment method for hemiplegic patients,so that RAS is not only used in gait training,but also in real-time assessment of gait in any environment for chronic stroke patients.