Objective To analyze the clinical features and efficacy of different treatments on 5 cases with Landau-Kleffner syndrome (LKS) with poor prognosis.Methods Five patients with LKS who had poor prognosis were enrolled in the Children's Hospital of Fudan University from September of 2008 to December of 2012.The clinical features and findings of video electroencephalograph (VEEG) of these patients were analyzed.In addition,the outcomes of treatments (antiepileptic drug,corticosteroids and ketogenic diet) on LKS who had poor prognosis were studied.The outcome of epilepsy was classified by using the Engel classification scheme and the outcome of electrical status epilepticus during sleep (ESES) was classified by the decrease in spike wave index (SWI).Results All of 5 patients (2 male and 3 female) had auditory agnosia and epileptic seizure.All patients had normal background activity and epileptiform discharges,and the location of epileptiform discharge was mainly from bilateral temporal;3 patients had ESES.Four out of the 5 patients had refractory epilepsy and the other one got seizure free with antiepileptic drug.Four patients received corticosteroids for 6 months.The aphasia disappeared in 1 patient,improved in 1 patient and sustained in the other 2 patients.Three patients who had ESES did not response to corticosteroids.Among 3 patients receiving ketogenic diet,1 patient was seizure free and the ESES disappeared;in another SWI was reduced;the rest one had a significant reduction in seizure.All the patients had improvement in their aphasia and cognition,however they still had cognitive disorder of various degrees.Conclusions The patients who had multiple seizure type or special seizure type may have poor outcome.Most of these patients have refractory epilepsy or persistent ESES.Corticosteroids can improve aphasia and cognition,but the persistence of ESES can make aphasia and cognition worse.Ketogenic diet can be used as an alternative treatment for refractory epilepsy or persistent ESES in LKS.

14-3-3 protein is a highly conserved soluble acid family. Recent researches suggest that they play critical regulatory roles in cellular processes such as cellular mitosis,growth,differentiation,proliferation and death,etc.14-3-3sigma protein,one member of the family,expresses mainly in epithelial cells and has histological specificity.14-3-3sigma protein interacts with several signal transduction proteins,induces cell cycle arrest in G2 phase after DNA damage,and regulates cellular biological functions.Abnormal expression of 14-3-3? sigma protein is common during tumor transformation,which may be induced by p53 mutation or CpG island hypermethylation of promoter. It is closely related with the transformation,progress and prognosis of human malignant tumors.

Objective To set up a method for the determination of ginsenoside Rg1 in Changyu Granules. Methods The content of ginsenoside Rg1 was determined by RP-HPLC on C18 column (4.6 mm?250 mm, 5 ?m). The mobile phase consisted of ethane nitrile and water (80:20). The flow rate was 1.0 mL/min. The detection wavelength was at 210 nm. Results Ginsenoside Rg1 showed a good linear relationship at the range of 0.125～4 ?g, r =0.999 7. The average recovery rate was 97.30%, RSD=1.31%. Conclusion The method is simple with good reproducibility, and can be used for the quality control of Changyu Granules.

AIM: To elevate the stability of the volatile oil in Jiufen Cataplasm (Semen strychni,Herba ephedrae,Oilbanum and Myrrha) with the help of inclusion with ?-Cyclodextrin. METHODS: The quality of the volatile oil in Jiufen Cataplasm before and after stability test was examined by thin layer chromatography and differential thermal analysis and thermogravimetric analysis. RESULTS: The combination of volatile oil and ?-cyclodextrin could form stable inclusion compound that helped constant in before and after inclusion. CONCLUSION: The process of enclosure with ?-CD can keep the active components of the volatile oil in Jiufen Cataplasm.

Objective To assess the concurrent management for portal hypertension (PHT) in gastric cancer patients. Methods Retrospective analysis was made on 60 cases of gastric cancer occurring on the background of cirrhotic PHT, of which 30 cases were of Child A grade, and 30 patients were Child B class. Ten cases were on gastric cancer stage Ⅰ , 38 cases on stage Ⅱ and 12 cases were on stage Ⅲ.Radical gastrectomy plus splenectomy and devascularization were performed in 26 cases, while radical gastrectomy only was done in 34 cases. Results Postoperative Child-Pugh degrading developed in 100% cases undergoing combined procedures; while in the 34 cases undergoing radical gastrectomy only,postoperative degradation from the preoperative Child-Pugh classification occurred in 7 cases ( 20% ), P ＜0. 001. In this series the peri-operative mortality was 3%, all were patients at stage Ⅲ gastric cancer combined with Child-Pugh class B. Conclusions The principle of treatment for gastric cancer with cirrhotic portal hypertension is restrictive surgery. The main purpose is to cure cancer and not to damage liver function.

Objective To study the efficacy of the orthotopie liver transplantation for hilar cholangiocarcinoma and analyse relevant factors.Methods A retrospective study was conducted in 10 cases of hilar cholangiocarcinoma treated with orthotopic liver transplantation from 2002 to 2010.Results The male:fe5 and 8 years were 70.0%and 60.4%.50.9%and 50.9%,respectively.No tumor survival rates for 1,3,5 and 8 year were 65.3%,58.7%,50.9%and 50.9%,respectively.Operative time was(306.7±38.1)min.The average blood loss was(659.4±300.6)mL.Multiple regression analysis showed that positive lymph nodes(P=0.0092)positive surgical margin(P=0.0028)and neural invasion(P=0.021)were the most important factor affecting hilar cholangiocarcinoma recurrence after liver transplantation.Conclusions Orthotopic liver transplantation for hilar cholangiocarcinoma is an effective means.Intraoperative radical resection will receive a good effect.Resection margin and lymph node-positive are the most important factors affecting long-term survival.

Objective To explore the relationship between age-related cerebral white matter changes (ARWMC) and cognitive function, observe the features of cognitive impairment in ARWMC patients, and provide evidences for preventing and treating cognitive impairment in elderly people.Methods Fifty patients with ARWMC were divided into mild-to-moderate group (n= 26, ARWMC score 1-10) and severe group (n= 24, ARWMC score＞ 10) based on the ARWMC scale of CT/MRI scans. Twenty healthy elderly people, who underwent physical examination in hospital, were selected as control group. The cognitive function was evaluated using Montreal Cognitive Assessment (MoCA,Beijing version). Cognitive functions were compared between patients with ARWMC at different extents and the controls. The correlation between severity of ARWMC and cognitive function, the characteristics of cognitive impairment in patients with ARWMC were analyzed. Results The cognitive function in patients with ARWMC was impaired significantly (the MoCA scores were 26.60±1.23 in control group, 23. 19±2. 62 in mild-to-moderate group and 19.83±3. 09 in sever group, F =39. 930, P = 0. 000). The severity of ARWMC was negatively related with MoCA score (r =-0. 476, P=0. 000). The patients showed cognitive impairment especially in the visuoconstructional and executive functions (F= 13. 189, P＜0.05), delayed recall (F=32.340, P＜0.05) and orientation (F= 15.813, P＜0. 05). Conclusions ARWMC is related with cognitive impairment.The severity of white matter changes is correlated with worse cognitive function.

The antimalarial effects of 24 chemical compounds on Plasmodium yoelii in mice and on Plasmodium falciparum in culture were observed . The results from both tests for most compounds are parallel or comparable, except few compounds such as Hydroxyurea and Lincomycin.What suitable methods may be used for preliminary screening of antimalarials are discussed.

Objective To observe the clinical effect of herbal decoction Sishen Jian on acute osteoarthritis of knee joint.Methods The 60 osteoarthritis cases in the attack stage were randomized into treatment group(30 cases)and control group(30 cases).The treatment group was given the decoction Sishen Jian and Diclofenac Sodium Sustained Release Capsules and the control group was given the capsules only.Two weeks made one course of treatment.Before and after treatment,the symptoms such as pain and morning stiff- ness,the signs including joint swelling and tenderness,and the erythrocyte sedimentation rate(ESR)and C reactive protein were ob- served for comparison.Results The total effective rate of the treatment group and control group was 96.66% and 53.26% respec- tively.The difference of two groups was significant(P

Objective To develop a new methodology of prenatal sonography scoring to assess and dignose clubfoot.Methods Thirty-one fetuses suspected clubfoot(50 foots) and 62 normal fetuses(124 foots) were scanned with both 2D and 3D sonography,and were divided into normal feet group and clubfeet group.Our scoring system was made according to the Pirani-scoring for children,while considering the morphology of mid-foot and hind-foot of infants.The doctors would give final diagnosis for all fetuses after delivery or induced labor,and analyse the correlations among the abnormal signs,sonographic score and the clinical outcomes.Results The curve lat border,the medial crease,the empty heel and the post crease can be observed in the 3D reconstruction images.These four abnormal signs had close correlations with the clinical results.Conclusions The new prenatal clubfeet scoring system by 3D sonography is a promising methodology with clinical values.