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.

Objective To study the the changes of serum tumor necrosis factor-?(TNF-?)and epidermal growth factor (EGF) in rats with experimental liver fibrosis and its significance. Methods All the SD rats were randomly divided into the normal group and the model group. The rats in the model group were subcutaneously injected with 40%CCl4 according to 0.3ml/100g body weight twice one week for the first four weeks, the initial dose was double. In the following five weeks, the rats were injected 40% CCl4 of 0.2ml/100g body weight twice one week.The rats in the normal group were subcutaneously injected with equal dosage of saline,mentioned aboveo.At the end of the ninth week,the serum was obtained,the levels of serum TNF-?, EGF were detected with radioimmunity, with HE stains, the severity of inflammation and fibrosis in all liver tissuses were measured by semiquantitative scoring. Results The fibrosis scorings, the levels of serum TNF-?, EGF in the model group were higher than that in the normal group, the difference between the two group was significance(P

ve To understand the indoor air quality of hotels after the openning for business and its health impacts on employees. Methods The hygienic investigation on indoor air quality was carried out in 4 newly built and decorated 2~4-star-grade hotels during the 1st week to the 4th week after openning for businesses well as the health impacts on employees. Results The levels of room temperature, relative humidity, carbon monoxide, carbon dioxide and total count of bacteria in indoor air of hotels showed no significant differences, but the concentrations of formaldehyde(HCHO) and inhalable paniculate (IP) of hotels showed significant differences compared with those of outdoor control(P

Objective To observation dynamic changes and gender difference of heart rate variability and blood pressure variability in unbonded spontaneously hypertensive rats using Telemetry technology .Method Taking sixteen spontaneously hypertensive rats of 3-month-old in SPF grade, there were implanted TL11M2-C50-PXT device intraperitoneally.Seven days later, respectively monitoring chainless blood pressure and electrocardiogram for 24 h continuously time using telemetry system when 3, 5, 7 months of age in spontaneously hypertensive rats .Analysing 24 h systolic pressure , mean blood pressure , diastolic blood pressure , heart rate variability and blood pressure variability . Result Systolic pressure , diastolic pressure and mean pressure , blood pressure variability increased with age of the moon increased in spontaneously hypertensive rats , moreover female SHR rats of blood pressure variability was significantly lower than that of the male rats .Correlated indexes of heart rate variability had no obvious change Between three to seven month -old in male spontaneously hypertensive rats .But RR interval , SDNN, TP, VLF and HF increased significantly with age of the moon increased in female spontaneously hypertensive rats , moreover the female SHR rats of TP , VLF and HF index of heart rate variability were lower than the male rats .Conclusion With the SHR rats age and blood pressure , blood pressure variability increased , and the female SHR rat variability in blood pressure and heart rate was significantly lower than that of the male .

Objective To study the application of implantable telemetry and whole-body plethysmography to observe the changes of circadian rhythm in conscious rats and evaluate the pharmacological safety of doxofylline, and to provide a basis for the future application of this technological system for drug safety evaluation.Methods Eight healthy SPF Sprague-Dawley rats were used in this study, 4 males and 4 females.The rats were implanted with telemetry transmitters by surgery to establish a telemetry system combined with plethysmography to observe the changes of 24 h physiological parameters and circadian rhythm in conscious rats at 14 d after operation, including heart rate (HR), blood pressure, the time interval from the Q wave to point A in the ECG of the aortic pressure wave (QA interval), respiration, activity, body temperature and pulmonary function parameters.The rats were divided into 3 groups: normal control group, doxofylline 40 mg/kg and 80 mg/kg groups, and the performance was validated by aerosolizing saline, doxofylline 40 mg/kg and 80 mg/kg inhalation, respectively, to observe the changes in physiological parameters after the drug administration.Results The physiological parameters of rats showed obvious changes in circadian rhythms at 14 d after operation.Compared with the normal control group, the doxofylline 40 mg/kg-treated group showed significantly increased changes of HR, tidal volume (TV), minute ventilation (MV), 50% expiratory flow (EF50), peak inspiratory flow (PIF) and peak expiratory flow (PEF) (P<0.01), significantly decreased respiratory frequency, QA interval and enhance pause (Penh) (P<0.05, P<0.01), but no significant differences in the blood pressure, activity and body temperature (P>0.05).Compared with the normal control group, the group treated with doxofylline 80 mg/kg had significantly increased HR, blood pressure, TV, MV, EF50, PIF and PEF (P<0.01), significantly decreased respiratory frequency, QA interval and Penh not (P<0.01), but not significantly changed activity and body temperature (P>0.05).Conclusions The application of implantable telemetry and whole-body plethysmography in this study does not obviously affect the circadian rhythm, and can sensitively monitor the relevant cardiovascular and respiratory parameters in conscious rats.It can be used in drug safety pharmacological research of cardiovascular and respiratory systems in conscious rats.

Objective: To summarize the gene mutation of early onset epileptic spasm with unknown reason. Method: In this prospective study, data of patients with early onset epileptic spasm with unknown reason were collected from neurological department of Children's Hospital of Fudan University between March 2016 and December 2016. Patients with known disorders such as infection, metabolic, structural, immunological problems and known genetic mutations were excluded. Patients with genetic disease that can be diagnosed by clinical manifestations and phenotypic characteristics were also excluded. Genetic research methods included nervous system panel containing 1 427 epilepsy genes, whole exome sequencing (WES), analysis of copy number variation (CNV) and karyotype analysis of chromosome. The basic information, phenotypes, genetic results and the antiepileptic treatment of patients were analyzed. Result: Nine of the 17 cases with early onset epileptic spasm were boys and eight were girls. Patients' age at first seizure onset ranged from 1 day after birth to 8 months (median age of 3 months). The first hospital visit age ranged from 1 month to 2 years (median age of 4.5 months). The time of following-up ranged from 8 months to 3 years and 10 months. All the 17 patients had early onset epileptic spasm. Video electroencephalogram was used to monitor the spasm seizure. Five patients had Ohtahara syndrome, 10 had West syndrome, two had unclear classification. In 17 cases, 10 of them had detected pathogenic genes. Nine cases had point mutations, involving SCN2A, ARX, UNC80, KCNQ2, and GABRB3. Except one case of mutations in GABRB3 gene have been reported, all the other cases had new mutations. One patient had deletion mutation in CDKL5 gene. One CNV case had 6q 22.31 5.5MB repeats. Ten cases out of 17 were using 2-3 antiepileptic drugs (AEDs) and the drugs had no effect. Seven cases used adrenocorticotropic hormone (ACTH) and prednisone besides AEDs (a total course for 8 weeks). Among them, five cases had no effect and two cases were seizure free recently. A case with GABRB3 (C.905A>G) had seizure controlled for 3 mouths. A case with ARX (C.700G>A) had seizure controlled for 6 mouths. Conclusion The early onset epileptic spasm with unknown reason is highly related to genetic disorders. A variety of genetic mutations, especially new mutations were found. Genetic heterogeneity of epileptic spasm is obvious.

Objective To evaluate the role of dynamic contrast-enhanced magnetic resonance imaging (MRI) in the diagnose of prostatic cancer and benign prostatic hyperplasia (BPH), and to determine the correlation between dynamic MRI findings with angiogenesis.Methods Thirty-two cases of prostatic cancer and 40 cases of BPH underwent dynamic contrast-enhanced MRI.All the patients in this study were diagnosed by histopathology.The results of dynamic contrast-enhanced MRI were evaluated by early-phase enhancement parameters and time-signal intensity curves (SI-T curves), and the curves were classified according to their shapes as type Ⅰ, which had steady enhancement; type Ⅱ, plateau of signal intensity; and type Ⅲ, washout of signal intensity.The pathologic specimens of region of interest (ROI) were obtained, and HE staining, immunohistochemical vascular endothelial growth factor (VEGF), and microvessel density (MVD) measurements were performed.The relationships among dynamic contrast-enhanced MRI features, VEGF, and MVD expression were analyzed.Results In the early-phase enhancement parameters of dynamic contrast-enhanced MRI, onset time,maximum signal intensity, and early-phase enhancement rate differed between prostatic cancer and BPH(P