The effects of tetrandrine on the aggregation and morphological changes of platelets were studied by using the rabbit platelet-rich plasma prepared with centri-fugation.It was found that tetrandrine could markedly inhibit the platelet aggregation induced by ADP,collagen,and arachidonic acid with an IC50(50% inhibitory concentration) of 38.9,30.3 and 44.3?g/ml respectively.The platelets pretreated with tetrandrine could not be stimulated to initiate morphological changes and granule release,and their normal structures were well preserved.In addition,di-pyridamole could enhance but excessive amount of extracellular calcium could weaken the inhibition of tetrandrine on platelet aggregation.

Objective To evaluate the localization of adrenal functionless tumor by ultrasonographv(US).Methods One hundred and six patients with adrenal functionless tumor were examined by US and subsequentlv proved by surgery and pathology.Results In 106 cases,true positive was 103,true negative,false positive and false negative were 1 each.The sensitivity,specificity and accuracy was 99.0%,50.0%and 98.1%,respectively.The adrenal tunctionless tumor tended increasing year by year.Tumors detected at very first by US were 77.9%.Tumor size distribution was gradually increasing as following: adenoma,pheochromocytoma(PHEO), neurilemmoma,myelolipoma,gangliocytoma and adenocarcinoma,in which there is significant difference.The myelolipoma presented charactersitically hyperecho feature to be easily distinguished from other tumors.The echo featLlres in the rest tumors chaotic echo.Conclusions US is a reliable examination of the first chdce for localizing diagnosis of adrenal functionless tumor and also helpful in differentiating histopathologically.

Objective To explore the solution to the secure problem of EMR based upon No.1 Military Medical Project.Methods The existing EMR system provides medical records service and the EMR are stored and managed in ordinary or established file format in the database server.Based upon above database characters related to EMR and the requirement of EMR security,the MRBACKUP system was developed to resolve EMR backup,recovery and dump.Results The MRBACKUP system safeguarded the security of EMR in the lowest cost,the least system resources and the highest efficiency.Conclusion The new secure solution to EMR can better resolve the security problems in the widely-used EMR in hospitals.

Background Laser in situ keratomileusis (LASIK) for myopia and/or myopic astigmatism still is one of primary surgeries.Femtosecond laser (FS)-LASIK offers several advantages over microkeratomes for flap creation including better safety,reproducibility,and predictability.However,the visual performance after FS-LASIK is of high clinical concern.Objective This study was to compare the visual performance between Intralase FS60 femtosecond laser flap and mechanical microkeratome Moria M2 flap in LASIK.Methods A prospective nonrandomized controlled study was performed.Two hundred and four myopic eyes of 102 patients were enrolled in this study and divided into two groups according to patients’ opinion,with matched demography between the two groups.LASIK with FS60 femtosecond laser flap was performed on 100 eyes of 50 myopia and/or myopic astigmatism in the FS group,and Moria M2 flap LASIK was performed on 104 eyes of 52 patients in the microkeratomes group.Zernike coefficients and the root-mean-square (RMS) of higher order aberrations were measured by Wavescan wavefront aberrometer before and 3,6 and 12 months after surgery,and the contrast sensitivity (CS) in different spatial frequencies under the scotopia and scotopia + glare background was detected using Optec 6500 visual function instrument before and 3,6,12 months after surgery.This study protocol was approved by Ethic Committec of Beijing Tongren Hospital,and written informed consent was obtained from each patient prior to surgery.Results The uncorrected visual acuity (UCVA) levels after surgery reached or was superior to the preoperative best corrected visual acuity (BCVA) in 95.1％,94.2％ and 93.9％ patients in the FS group and in 94.2％,93.8％ and 93.2％ patients in the microkeratomes group 3,6,12 months after surgery,respectively.The mean spherical equivalent (SE) was (-6.37 ±2.06)D,(-0.26±0.45)D and (-0.45± 0.51)) in the FS group,(-6.25± 2.43) D,(-0.44± 0.64) D and (-0.35±0.59)D in the microkeratomes group before surgery and 3,12 months after surgery,respectively.There were no significant differences in the SE in various time points between the two groups (u =1.194,1.429;both at P＞ 0.05).The root means square high order (RMSh) and RMS3-6 in the FS group were significantly lower than those in the microkeratomes group 3,6 and 12 months after surgery (all at P＜0.05).Under the background of scotopia or scotopia+glare,the LogCS values under different spatial frequencies were significantly higher in the FS group than those in the microkeratomes group 3 and 6 months after surgery (all at P＜0.05).In 12 months after surgery,no significant difference was found in the LogCS under the 1.5 c/d in the background of scotopia between FS group and microkeratomes group (P＞0.05),and the LogCS values under the 12.0 c/d and 18.0 c/d in the background of scotopia or scotopia+glare in the FS group were better than those in the microkeratomes group (all at P＜0.05).Conclusions Femtosecond laser assistant LASIK has better visual performance and lower higher-order aberrations than microkeratomes assistant LASIK.

Background Keratectasia after laser in situ keratomileusis (LASIK) is a rare but severe complication,which threatens the visual acuity and corneal strength.Corneal collagen crosslinking (CXL) is a new therapy that increases the security and decreases the risk of complication.However,the effectiveness and safety of LASIK-CXL is still need to be concerned.Objective This study was to evaluate the safety of LASIK-CXL for myopia and astigmatism with thin cornea.Methods A prospective cohort study was designed.A total of 128 eyes of 64 patients with thin corneal and myopic astigmatism enrolled in Beijing Tongren Eye Center from January 2014 to January 2015.The patients were assigned to LASIK group (74 eyes of 37 patients) and LASIK-CXL group (54 eyes of 27 patients).Refractive surgery was performed by Visumax femtosecond lasrer and VISX S4 excimer laser.Eyes of LASIK-CXL group applied accelerated CXL immediately after LASIK.The follow-up was 6 months.Manifest refraction,uncorrected (UDVA) and corrected distance visual acuity (CDVA),average keratometry values (AveK),anterior segment OCT (AS-OCT),corneal hysteresis (CH) and corneal resistance factor (CRF) were examined before and after operation.This research passed through Ethics Committee of Beijing Tongren Hospital.Results The spherical equivalent (SE) of the LASIK group and LASIK-CXL group were (-6.49 ±2.41)D and (-6.97 ±2.41) D before operation and decreased to (-0.68 ±0.88) D and (-0.75 ±0.94) D 6 months after operation.The UDVA (LogMAR) was 1.18±0.28 and 1.05±0.38 before operation and elevated to-0.06±0.09 and-0.03±0.186 months after operation in the LASIK group and LASIK-CXL group.The preoperative AveK values were (44.37 ±1.46) D and (44.47± 1.50)D in the LASIK group and LASIK-CXL group and reduced to postoperative (39.30±2.06) D and (38.66± 1.80) D.The preoperative SRI of LASIK group and LASIK-CXL group were 0.25 ±0.21 and 0.24±0.22,which increased to 0.29±0.24 and 0.28±0.24.The SAI values were 0.36±0.16 and 0.39±0.15 before operation,which increased to 0.57 ±0.31 and 0.75 ±0.376 months after operation,and the SAI value of the LASIK-CXL group was significantly higher than that of LASIK (F =10.220,P--0.002).CRF values of LASIK and LASIK-CXL were (8.44±1.44)mmHg and (8.63±1.35) mmHg in preoperation,which decreased to (5.74±1.31) mmHg and (6.25± 1.24) mmHg in postoperation.The result of LASIK-CXL was higher than that of LASIK (F=8.650,P =0.040).CH values were 8.78 ± 1.51 and 8.69 ± 1.62 in preoperation,which decreased to (7.23 ± 1.08) mmHg and (6.50±1.32)mmHg.The value of LASIK-CXL was lower than that of LASIK (F =5.860,P =0.017).The mean depth of demarcation line was (228.45±28.24) μm (range 165 to 310 μm) on OCT,which was presented in 45 eyes (81.82％) at 1 month in postoperation.Conclusions Accelerated CXL with FS-LASIK is effective and safe in improving visual acuity in myopic astigmatism patients with thin cornea,which also can increase the rigidity of the cornea.

Objective To explore the genetic etiologies in 2 siblings with different epileptic encephalopathies (EEs) diagnosed as Ohtahara syndrome(OS) and atypical benign partial epilepsy(ABPE) from one family.Methods The 2 brothers were diagnosed at the Pediatric Neurological Clinic of Peking University First Hospital in September 2013,whose clinical data were collected.The coding region of the syntaxin-binding protein 1 gene (STXBP1) and glutamate receptor subunit gene (GRIN2A) were detected by using Sanger sequencing in the 2 siblings.For the elder brother,targeted next-generation sequencing was further performed to detect the genes associated with epilepsy.Results The younger brother manifested focal motor seizures and tonic spasms in cluster at the age of 1 month.Interictal electroencephalogram (EEG) showed suppression-burst pattern.He was diagnosed as OS.The elder brother had seizure onset at age of 6 years old.Focal motor seizure during sleep was his seizure type.His EEG showed interictal discharges in Rolandic area primarily.Electrical status epilepticus during sleep,epileptic negative myoclonus and intellectual disabilities occurred during the course.He was diagnosed as ABPE.Brain magnetic resonance imagines for both of them were normal.Screening of STXBP1 mutations for the younger brother found a de novo heterozygous mutation:c.1672C ＞ T (p.Q558X).Gene detection for the elder brother and the parents showed negative results.Conclusions Coexistence of distinct EEs was reported in 2 brother siblings:the younger brother had OS associated with a novel nonsense mutation in STXBP1,and the elder brother had ABPE without genetic evidence.This study indicated that different pathological mechanisms might exist underlying the two different EEs in a family.

Objective To evaluate the predictive effect of Braden scale for the risk of development of pressure ulcers (PU) in the department of neurology bedridden patients and to explore subgroup preventive measures. Methods 400 cases newly hospitalized bedridden patients in the department of neurology were collected with no pressure ulcers at the first evaluation and pressure ulcer risk was continuously predicted by a Braden scale skin assessment. The high-risk, middle-risk and low-risk groups were randomized into the experimental group and the control group respectively. Routine preventive measures were taken for the control group while the air fluidized bed for the high-risk group, the sponge mattress for the middle-riskgroup, and turning the body over every 4 hours for low-risk group. Other preventive procedures were undertaken simultaneously in beth the experimental and the control groups. Results The area under the ROC curve (AUC)was 0.771 and 0.828 at the first and last time Braden scale scores respectively. Such vMues as sensitiveness, specificity, positive predictive value, negative predictive value were found in higher level,when the diagnosis value was 17. There was no significant difference of incidence rate of the subgroup pressure ulcers between the high-risk, middle-risk, low-risk groups compared to the control group. Conclusions The effect of predicting pressure ulcer risk for bedridden patients in the department of neurology with Braden scale was fairly good, while the score 17 as the diagnosis value was ideal. The air fluidized bed for the high-risk group and the sponge mattress for the middle-risk group resulted in no significant decrease of incidence rate of the pressure ulcer, while taming over the patients' body every 4 hours for low-risk groups showed acceptable and therefore saving medical resources.

The clinical and genetic data were retrospectively analyzed in a pedigree with pseudohypoparathyroidism type Ⅰ a.Clinically typical Albright hereditary osteodystrophy (AHO),hypocalcemia,hyperphosphatemia,and PTH- and TSH-resistance were manifested in the proband,but not in his brother and parents.The proband's symptom of epilepsy was alleviated by treatment with calcium and vitamin D,which was of no avail in regard to AHO.After GNAS1 genes were sequenced and compared with the GenBank data among the family members,a deletion of c.1107_1108 ( p.Glu370ArgfsX11 ) in exon l3 of GNAS1 gene leading to a frameshift mutation was found in the proband and his mother.It suggested that the GNAS1 gene mutation might be related to the pathogenesis of the disease.

Objective: To investigate the electroclinical findings in epilepsy children with epileptic negative myoclonus (ENM) restricted to the lower limb as the first seizure type. Methods: Each retrieved electroencephalogram record performed between March 2011 and March 2018 at the Department of Pediatrics of Peking University First Hospital was searched with "midline" . There were 302 records of 175 patients with "benign" or "functional" midline spikes. A retrospective review of each patient′s hospital record was performed. Thirteen patients had ENM restricted to the lower limb as the first seizure type. The clinical and electroencephalogram characteristics of them were analyzed. Results: Thirteen patients manifested ENM restricted to the lower limb as the first seizure type, diagnosed as benign childhood focal epilepsy with vertex spikes (BEVS). Six patients had ENM as the first and only seizure type during the short-time follow-up. Among them, there were 1 male and 5 females. The age at seizure onset was (2.5±0.7) years. One of them had electrical status epilepticus during sleep (ESES) identified on electroencephalogram at theage of 4 years and 8 months. The last follow-up age was (3.8±1.5) years. The remaining 7 patients developed nocturnal focal motor seizures. Among them, there were 4 males and 3 females. The age at seizure onset was (3.5±0.7) years. Two of them were diagnosed as BEVS evolving into benign childhood epilepsy with centrotemporal spikes (BECTS) and 5 were diagnosed as BEVS concurring with BECTS. The age at focal seizures was (4.1±0.6) years. The interval ranged from 1 month to 1 years. Six of 7 patients had electrical ESES with the age of (5.2±1.0) years. All had developmental regression, further diagnosed as atypical benign partial epilepsy (ABPE). The median age at last follow-up was 5.9 years. Five of 13 patients had repeated electroencephalogram records at our apartment, showing that epileptiform discharges in midline regions were significantly reduced either in frequency or amplitude with the improvement of ENM restricted to the lower limb and that independent epileptiform discharges in Rolandic regions from midline regions were noticed with the onset of nocturnal focal seizures. Conclusions ENM restricted to the lower limb has a close association with vertex (midline) epileptiform discharges. ENM restricted to the lower limb as the first seizure type is a peculiar phenomenon of BEVS. Some patients could evolve into BECTS or overlap with BECTS, and further into ABPE. The age of seizure onset in BEVS with ENM restricted to the lower limb as the first symptom is a little earlier than in BECTS. Ignorance of the close association between midline spikes and ENM restricted to the lower limb may lead to misdiagnosis of these patients.

OBJECTIVETo detect potential mutations of the glutamate receptor subunit (GRIN2A) gene and delineate the clinical-genetic characteristics of patients with epilepsy-aphasia spectrum (EAS) disorders.

METHODSOne hundred twenty two patients with Landau-Kleffner syndrome (LKS), epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS), benign childhood epilepsy with centrotemporal spikes (BECT) and BECT variants were recruited. Potential mutations of the GRIN2A gene were screened with Sanger sequencing. And clinical-genetic characteristics for all patients were analyzed.

RESULTSThe patients have included 9 LKS, 26 CSWS, 42 BECT variants and 45 BECT. The mean age of onset of seizure or aphasia was 5 years old (10 months to 11 years). Mutation screening has detected 4 possible pathogenic missense mutations including c.2278G>A (p.G760S), c.4153G>T (p.D1385Y), c.1364G>A (p.C455Y) and c.691T>C (p.C231R) in four unrelated probands, which comprised one case with LKS and three with BECT variants. The mutation rate was 11.1% (1/9) for LKS and 7.2% (3/42) for BECT variants. No GRIN2A mutation was found in the 26 patients with CSWS and 45 patients with BECT. Among the 122 probands, 25 (20.5%) patients without a GRIN2A mutation had a positive family history of febrile seizures or epilepsy.

CONCLUSIONGRIN2A mutation do exist in EAS patients, but with a relatively low rate. A proportion of EAS patients without a GRIN2A mutation have a positive family history, which suggested a complex mechanism for EAS.