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Background and purpose: The aim was to investigate whether abnormal TTTTA and TTTCA repeat expansions in introns of SAMD12,TNRC6A and RAPGEF2 are involved in the pathogenesis of familial cortical myoclonic tremor with epilepsy (FCMTE). Methods: Five families diagnosed with FCMTE were included in the current genetic analysis. Whole‐exome sequencing was performed in selected patients of each family. TTTTA and TTTCA expansions were examined by repeat‐primed polymerase chain reaction. The clinical features of FCMTE were elicited as defined by the common genetic mechanism of 14 patients. Results: Abnormal TTTCA expansion was identified and co‐segregated in all five FCMTE families, four inserted in SAMD12 and one in RAPGEF2. The insertion of expanded TTTCA was not found in 116 control alleles. TTTTA expansion in SAMD12 was detected in 90.9% (10/11) of patients or mutation carriers; TTTTA expansion in RAPGEF2 was not found. The onset age of myoclonic tremor was 27.4 ± 5.9 (19–37) and epilepsy usually presented around age 34. Focal and generalized seizures were witnessed with various origins recorded by electroencephalogram. Cognitive deficits were not common within the first 3 years after epilepsy onset. Emotional instability was reported by most patients. No patients showed any cerebellar deficits. Valproate added with clonazepam is effective in controlling seizures but cannot guarantee a complete remission of tremor. Repeat length showed intergenerational instability and was inversely correlated with age at onset of myoclonic tremor and epilepsy. Conclusions: TTTCA expansion insertion is associated with FCMTE in Chinese families. The homogenous genetic mechanism allowed for a higher precision of FCMTE description. [ABSTRACT FROM AUTHOR]

The dependence of reduced remanence (Mr/Ms) and coercivity of [Sm.sub.22][Co.sub.78]/[Fe.sub.65][Co.sub.78]/[Sm.sub.22][Co.sub.78] triple-layer films on [Fe.sub.65][Co.sub.35] soft-magnetic interlayer thickness, d, was systematically investigated. All the hysteresis loops of the triple-layer films exhibit a single-phase behavior, reflecting a strong exchange coupling between soft- and hard-magnetic phases. Mr/Ms shows a monotonic increase with increasing the [Fe.sub.65][Co.sub.35] thickness, d, and the coercivity exists a peak with increasing d. From the plot of the interaction-based deviation parameter AM (H) of two principle remanence curves (isothermal remanence magnetization and dc-demagnetization remanence) versus applied field, H, a negative interaction profile was observed, indicating a negligible inter-granular exchange coupling within hard-magnetic grains. Index Terms--Coercivity, enhanced remanence, SmCo/FeCo/ SmCo triple-layer films, tow-phase magnet.

Nutrients in animal manure are valuable inputs in agronomic crop production. Timely and reliable information on animal manure nutrient content will facilitate the utilization of manure as organic fertilizer and reduce any associated potential environmental problems. The objective of this study was to investigate the feasibility of using multiple linear regression (MLR), polynomial regression, and artificial neural network (ANN) models to determine nutrient content in dairy manure. Fresh manure samples (n = 86) from Holstein dairy cattle were collected from 34 dairy farms located in Beijing city, China. All samples were analyzed for nutrient content (ammonium nitrogen, total potassium, total nitrogen, and total phosphorus) by standard laboratory methods. The physicochemical properties (specific gravity, electrical conductivity, and pH) of dairy manure samples were measured. Relationships between nutrient content and physicochemical properties were explored by MLR, polynomial regression, and ANN models. Several parameters (R², modeling efficiency statistic, mean squared error of prediction, mean bias, linear bias, and maximum bias) were calculated to evaluate model performance. The residual analysis results indicated that all MLR models for the testing data set had significant mean and linear bias. When compared with MLR and polynomial regression models, the ANN model for all nutrient contents had better performance with higher R² and modeling efficiency statistics and lower mean squared error of prediction, mean bias, linear bias, and maximum bias. These findings demonstrated that the ANN model may be an appropriate tool to predict dairy manure nutrient content. [ABSTRACT FROM AUTHOR]

Studies have demonstrated that obesity and osteoporosis are linked disorders in humans. This study examined the hypothesis that excessive lipid consumption affects bone metabolism in laying hens. A total of one hundred 63-wk-old laying hens were randomly divided into 2 treatments and fed either a regular layer diet (control) or a high energy and low protein diet (HE-LP; experimental treatment) for 80 d. Egg production, feed intake, and BW were recorded at various days during the treatment. At d 80, ten randomly chosen birds per treatment group were killed. Abdominal fat weight, liver weight, and liver fat content were determined. Serum levels of total calcium, inorganic phosphate, and alkaline phosphatase were measured using a biochemical analyzer. Serum concentrations of osteocalcin, leptin-like protein, and estrogen were measured by enzyme-linked immunosorbent assay. Tibia length and width were measured using a vernier caliper; density of the right tibias was determined using an x-ray scanner; and mechanical properties of the left tibias were analyzed using a material testing machine. The expression of osteocalcin and osteoprotegerin mRNA in the keel bone was analyzed by real-time PCR. The concentration of osteocalcin protein in the keels was measured using western blot. Compared with control hens, hens fed the HE-LP diet had lower egg production, lower feed intake, greater liver fat content, and greater abdominal fat pad mass (P < 0.05). Feeding the HE-LP diet increased serum alkaline phosphatase activity, osteocalcin, leptin-like protein, and estrogen concentrations (P < 0.05), and decreased the keel osteocalcin concentrations (P < 0.05). There were significant positive correlations between the serum concentrations of leptin-like protein, estrogen, and osteocalcin regardless of treatment (P < 0.05). The results indicated that HE-LP diet induced a fatty liver disorder in laying hens with an upregulation in bone turnover and exacerbated skeletal damage. The data supported a role for lipid metabolism in skeletal heath of laying hens. [ABSTRACT FROM AUTHOR]

Allergic diseases are immune disorders caused by allergens, leading to inflammation or organ dysfunction. In the past decade, the prevalence of allergic diseases in China has increased dramatically, imposing a heavy economic burden on the health care system and society. In vitro diagnosis of allergic diseases plays a crucial role in the diagnosis, treatment, and prevention of such diseases. This article enumerates the in vitro tests and diagnostic techniques of allergic diseases, gives an advocacy for quality management of IgE-related tests, summarizes the clinical interpretation and relevant research progress, aiming to provide a reference for improving laboratory diagnosis of allergic diseases.

Amyotrophic lateral sclerosis(ALS) is a progressive and fatal neurodegenerative disease that mainly involves upper and lower motor neurons. It lacks clear biomarkers and can be clearly diagnosed only one and a half years after the onset. Gene test is of great significance for diagnosis, prognosis and genetic counseling. In recent years, several gene therapy studies have entered the clinical trial stage of ALS, among which the antisense oligonucleotide therapy targeting the pathogenic variation of the superoxide dismutase 1 (SOD1) gene has been launched, and it is urgent to carry out routine gene test in clinical practice. On the basis of progress of ALS gene research in recent years, family history, age of onset and typical clinical manifestations of patients are no longer considered as the basis for genetic testing. However, the target genes of clinical gene testing needs to be further clarified according to the diagnostic purpose, the testing method and scheme need to be standardized, and the genetic consultation before testing should be paid attention to, and the informed consent should be fully achieved.

Antiphospholipid syndrome (APS) is characterized by arterial and venous thrombosis and(or) morbid pregnancy, accompanied by persistent antiphospholipid antibody (aPL) positivity. However, due to the complex pathogenesis of APS and the large individual differences in the expression of aPL profiles of patients, the problem of APS diagnosis, prognosis judgment and risk assessment may not be solved only from antibody level. It is necessary to use new technologies and multiple dimensions to explore novel APS biomarkers. The application of next generation sequencing (NGS) technology in diseases with high incidence of somatic mutations, such as genetic diseases and tumors, has been very mature. Thus, gradually understanding the research and application progress of APS by NGS technology from genome, transcriptome, epigenome and other aspects is meaningful. This article reviews the related research of NGS technology in APS, and provide more reference for the deep understanding of the APS-related screening markers and disease pathogenesis.

Objective: To identify the predisposing factors, clinical characteristics, and risk factors of disease progression to establish a novel predictive survival model and evaluate its application value for hepatitis B virus-related acute-on-chronic liver failure. Methods: 153 cases of HBV-ACLF were selected according to the guidelines for the diagnosis and treatment of liver failure (2018 edition) of the Chinese Medical Association Hepatology Branch. Predisposing factors, the basic liver disease stage, therapeutic drugs, clinical characteristics, and factors affecting survival status were analyzed. Cox proportional hazards regression analysis was used to screen prognostic factors and establish a novel predictive survival model. The receiver operating characteristic curve (ROC) was used to evaluate predictive value with the Model for End-Stage Liver Disease (MELD) and the Chronic Liver Failure Consortium Acute-on-Chronic Liver Failure score (CLIF-C ACLF). Results: 80.39% (123/153) based on hepatitis B cirrhosis had developed ACLF. HBV-ACLF's main inducing factors were the discontinuation of nucleos(t)ide analogues (NAs) and the application of hepatotoxic drugs, including Chinese patent medicine/Chinese herbal medicine, non-steroidal anti-inflammatory drugs, anti-tuberculosis drugs, central nervous system drugs, anti-tumor drugs, etc. 34.64% of cases had an unknown inducement. The most common clinical symptoms at onset were progressive jaundice, poor appetite, and fatigue. The short-term mortality rate was significantly higher in patients complicated with hepatic encephalopathy, upper gastrointestinal hemorrhage, hepatorenal syndrome, and infection ( P < 0.05). Lactate dehydrogenase, albumin, the international normalized ratio, the neutrophil-to-lymphocyte ratio, hepatic encephalopathy, and upper gastrointestinal bleeding were the independent predictors for the survival status of patients. The LAINeu model was established. The area under the curve for evaluating the survival of HBV-ACLF was 0.886, which was significantly higher than the MELD and CLIF-C ACLF scores ( P < 0.05), and the prognosis was worse when the LAINeu score ≥ -3.75. Conclusion: Discontinuation of NAs and the application of hepatotoxic drugs are common predisposing factors for HBV-ACLF. Hepatic decompensation-related complications and infection accelerate the disease's progression. The LAINeu model can predict patient survival conditions more accurately.

Objective: To investigate the blood pressure change in patients with acute ischemic stroke (AIS) and hypertension treated with cinepazide maleate injection. Methods: This was a subgroup analysis of post-marketing clinical confirmation study of cinepazide maleate injection for acute ischemic stroke: a randomized, double-blinded, multicenter, placebo-parallel controlled trial, which conducted in China from August 2016 to February 2019. Eligible patients fulfilled the inclusive criteria of acute anterior circulation ischemic stroke with National Institutes of Health Stroke Scale (NIHSS) scores of 7-25. The primary endpoints were mean blood pressure of AIS patients treated with cinepazide maleate or control, which were assessed during the treatment period (14 days), and the proportion of the patients with normal blood pressure was analyzed after the treatment period. Furthermore, a subgroup analysis was performed to investigate a possible effect of the history of hypertension on outcomes. Results: This analysis included 809 patients with hypertension. There was no significant difference in patients blood pressure and the proportion of patients with normal blood pressure (60.5% vs. 59.0%, P >0.05) between cinepazide maleate group and control group. Conclusion: Administration of cinepazide maleate injection does not affect the management of clinical blood pressure in patients with AIS.

Objective: To explore the relationship between white matter lesions and clinical features and response of cerebral spinal fluid (CSF) tap test in patients with idiopathic normal pressure hydrocephalus(iNPH). Methods: Possible iNPH patients were enrolled from outpatients and inpatients in Peking Union Medical College Hospital between 2014 and 2019. All patients underwent detailed neuropsychological and walking assessments, CSF tap test, as well as head magnetic resonance imaging. The Fazekas score of white matter lesions, the fractional anisotropy (FA)and mean diffusivity (MD) values of regions of interest by means ofdiffusion tensor imaging (DTI) were compared between CSF tap test positive and negative response groups. The correlation between DTI parameters and clinical characteristics was analyzed. Results: Forty-three patients (29 male and 14 female, age range: 52-79 years] wererecruited.Compared with the negative group, patients in the positive group tended to have higher Fazekas score of periventricular white matter( U =108.00, P =0.03), higher MD value of the region near anterior horn of left lateral ventricles[(1.14±0.27)×10 -9 mm 2 /s vs (0.85±0.08) ×10 -9 mm 2 /s, P =0.003], lower FA value of the region near anterior horn of the right lateral ventricles[(0.20±0.07)vs(0.27±0.09), P =0.058], and higher MD value near the posterior horn of right lateral ventricle [(1.17±0.34)×10 -9 mm 2 /s vs (0.95±0.01)×10 -9 mm 2 /s, P =0.003]. FA and MD were significantly correlated with motor function, cognitive and functional scores, and iNPH grading scale (iNPHGS) scores(all P <0.05). Conclusions: The white matter lesions might be one of the pathogeneses of lNPH and apathological changewhich can be reversed by CSF drainage. More white matter lesions should not be the contraindication of CSF drainage surgery.

Objective: To investigate the relationship between plasma Golgi protein 73 (GP73) levels and the occurrence and development of non-alcoholic fatty liver disease (NAFLD), and to establish a diagnostic model based on this combination with lipid metabolism indicators to clarify its diagnostic efficacy and clinical application value for NAFLD. Methods: 225 cases with NAFLD [diagnosed by ultrasound, transient elastography (FibroScan502) and liver biopsy (some patients)] and 108 healthy controls were selected from the Department of Hepatology and Physical Examination Center of Integrated Traditional Chinese and Western Medicine, The Third Hospital of Hebei Medical University. Clinical data, routine peripheral blood and serum biochemical test results were collected. The plasma GP73 level was detected by enzyme-linked immunosorbent assay. SPSS 21.0 statistical software was used for statistical analysis. Binary logistic regression model was used to calculate the NAFLD diagnostic model. Receiver operating characteristic curve was used to evaluate the NAFLD constructed model diagnostic efficacy. Results: NAFLD incidence was significantly reduced in younger age group, mostly in young and middle-aged male. However, the NAFLD incidence was increased with increasing age in female. The analysis of age ratio composition showed that the average age for NAFLD onset was 20 ~ 50 years old, and the incidence rate was as high as 47% in among 30 ~ 39 years old, but the incidence rate was significantly decreased in over 60 years old (4.00%). GP73 was an independent risk factor for the occurrence and development of NAFLD. The diagnostic models of GBT, GB and GT were established by GP73 (G) combined with body mass index (BMI, B) and serum triglyceride (TG, T), and the results showed that the areas under the curves of GBT, GB and GT models were 0.969, 0.937 and 0.909, respectively. The sensitivity and the specificity were 84.90%, 77.80% and 84.00%, and 95.40%, 95.40% and 82.40%, respectively, P < 0.05. The GBT model had efficacy of best diagnostic performance. Conclusion: NAFLD is more common in young and middle-aged male, but with advanced age, the incidence of female patients gradually increases. Plasma GP73 levels are related to the occurrence and development of NAFLD. The GBT model can be used as a new model for non-invasive diagnosis and one of the indicators for clinical evaluation of diagnostic efficacy of NAFLD.

Objective: The results of syphilis antibody screening in Peking University Third Hospital from 2016 to 2020 were analyzed,to explore the characteristics of sex, age and distribution of patients with positive syphilis antibody. Methods: A retrospective study was conducted to collect the results of syphilis antibody in outpatients and inpatients of Peking University Third Hospital from 2016 to 2020. Syphilis antibodies were screened in 626 528 patients aged 1-98 years, 4 232 were retested positive by TPPA test, including 2 132 males (50.4%) and 2 100 females (49.6%). Chemiluminescence immunoassay (CMIA) was used for syphilis antibody screening, and Treponema pallidum particle agglutination (TPPA) test was used for reexamination. SPSS20.0 data statistical analysis software was used to analyze the detection rate, age, sex, department and clinical diagnosis of patients with positive syphilis antibody by χ² test. Results: Among 626 528 patients who were positive for treponema pallidum antibody screening, 4 232 were retested positive by TPPA test, accounting for 0.68% of the total number of patients tested. The number of syphilis tests increased year by year, however the positive detection rate decreased. The positive detection rate of syphilis antibody in 2020 decreased by 18.9% compared with 2016. The positive rate of syphilis antibody in male patients was higher than that in female patients, accounting for 0.80% and 0.59% of the total number of patients tested respectively. The positive rate of syphilis antibody of different genders increased with age, the total positive rate of 0-20, 21-40, 41-60, 61-80,>80 years old were 0.15%, 0.45%, 0.95%, 1.07% and 1.41%, respectively. While the increase rate of males was higher than that of females. The positive rate of male over 80 years old was 18.13 times of the group of 0-20 years old, and 5.54 times in women. The top 6 departments with positive syphilis antibody detection rate were emergency department, oncology department, respiratory department, geriatrics department, endocrinology department and neurology department, and the positive rates were 1.79% (104/5 810),1.46% (55/3 767),1.20% (74/6 167),1.20% (22/1 833),1.10% (32/2 909),1.09% (94/8 624), respectively. From the analysis of clinical diagnosis, the proportion of positive syphilis antibody in infertile patients (0.64%, 672/104 911) was higher than that in naturally conceived patients (0.10%, 24/23 969). Conclusions: From 2016 to 2020, the positive detection rate of syphilis antibody in Peking University Third Hospital decreased year by year. However, the positive detection rate increased with the age of patients. The positive rate of syphilis antibody in male was higher than that in female. The positive rate of syphilis antibody in pregnant women was lower than that in infertile patients.

Objective: To determine the correlation between the titer of anti-muscle-specific tyrosine kinase (anti-MuSK) antibodies (Abs) and the severity and prognosis of Musk-associated myasthenia gravis (Musk-MG). Methods: A total of 33 MuSK-MG patients diagnosed at Department of Neurology, Peking Union Medical College Hospital from May 2018 to June 2020 were prospectively included. Patients were divided into different groups according to immune state, and the immune naive patients were further divided by the Myasthenia Gravis Foundation of America (MGFA) classification. There were 28 Musk-MG patients who completed the follow-up and subdivided into different groups according to post-intervention status (PIS). Twenty-five patients who received immunotherapy were divided into corticosteroid monotherapy group ( n =17) and corticosteroid combined with immunosuppressant group ( n =8). The comparison of Ab titers between different MGFA groups and PIS groups was determined by Kruskal-Wallis method, and the comparison of Ab titers between different time points was analyzed by Mann-Whitney U method. Results: There were 11 males and 22 females included in the study, with an onset age of 48 (18, 73) years, of which 16 cases were immune naive and 17 cases were treated with corticosteroids or immunosuppressant at least once. In immune naive population, a significant difference of Ab titers among different MGFA phenotypes was detected ( P =0.04). Ab titers were reduced by immunosuppression therapy (the median value decreased from 1.20 to 0.87, P =0.01). Twenty-four (85.7%) MuSK-MG patients achieved a good prognosis (PIS-PR/MM), 1 (3.6%) case achieved improvement (PIS-I), and 3 (10.7%) patients' condition worsened (PIS-W), there was no significant difference of Ab titers among the three groups ( P =0.21). Meanwhile, there was no significant difference of Ab titers between different treatment groups ( P =0.95). Conclusions: In the immune naive state, the concentration of MuSK-Ab is consistent with the severity of the disease, and the Ab titers decrease after immunotherapy. Change of Ab titers is related to the daily dosage of corticosteroid and is not consistent with PIS grades.

Objective: Longitudinally extensive transverse myelitis (LETM) could be seen in patients with connective tissue disease (CTD), especially systemic lupus erythematosus (SLE) or primary Sjögren's syndrome (pSS). Some patients are combined with neuromyelitis optica spectrum disorders (NMOSD)(termed CTD-LETM-NMOSD) while others without (termed CTD-LETM-non-NMOSD). The aim of this study is to compare the clinical characteristics of CTD-LETM-NMOSD patients to CTD-LETM-non-NMOSD patients. Methods: We retrospectively collected data from 40 CTD patients with LETM who were admitted to the Department of Neurology or Rheumatology at Peking Union Medical College Hospital from Jan, 2006 to Dec, 2016. They were divided into CTD-LETM-NMOSD and CTD-LETM-non-NMOSD two groups. Demographic characteristics, clinical and laboratory features were obtained from the database. Relapse rates and clinical outcome were analyzed by Kaplan-Meier method. Results: Among 40 patients with CTD, 28 (70.0%) were NMOSD while 12 (30.0%) were not. The positivity rates of anti-SSA, antibodies to aquaporin-4 (anti-AQP4) were significantly higher in patients with NMOSD than those in patients with non-NMOSD ( P <0.05). Age, gender, clinical features, disease duration, anti-double-stranded DNA antibody, anti-ribosomal P antibody, antiphospholipid antibodies, expanded disability status scale (EDSS) scores, and magnetic resonance imaging (MRI) features were all comparable between two groups. CTD-NMOSD patients had significantly higher disease relapse rate (75.0% vs. 3/12, P <0.01). Conclusion: Anti-SSA and anti-AQP4 positivity is associated with NMOSD and higher relapse rates, which suggests that NMOSD in CTD-LETM patients may represent distinct characteristics and pathogenesis from patients with CTD-LETM-non NMOSD.

Objective: To study the bacterial pathogen, the optimal plan of antibacterial drugs and the prognostic factors in patients with liver cirrhosis combined with bacterial pneumonia. Methods: Data of 324 cases with liver cirrhosis from the Department of Traditional and Western Medical Hepatology, the Third Hospital of Hebei Medical University were collected, including 217 cases of bacterial pneumonia. Baseline characteristics of the patients, factors affecting the efficacy of antibacterial treatment and prognosis were compared and analyzed. Logistic regression analysis was used to screen and predict the antibacterial efficacy indicators and a prediction model was established. Receiver operating characteristic curve was used to evaluate the value of the established model and Child-Turcortte-Pugh, model for end-stage liver disease, and model for end-stage liver disease combined with serum sodium concentration predict the therapeutic efficacy. Results: Chronic HBV and HCV infections were the main causes of cirrhosis, followed by cryptogenic cirrhosis and alcoholic cirrhosis. Diabetes, cardio-cerebrovascular and chronic obstructive pulmonary disease were susceptible factors for bacterial pneumonia. As infection occurred, the ratio of neutrophils to lymphocytes, serum C-reactive protein, procalcitonin, alanine aminotransferase, and total bilirubin levels had increased significantly. The results of pathogenic analysis showed that the top three pathogenic bacteria were Klebsiella pneumoniae, Acinetobacter baumannii, Pseudomonas aeruginosa and Staphylococcus aureus. The resistance rate of Klebsiella pneumoniae to ceftriaxone was 50.0%, and that of ceftazidime, cefepime, and cefoperazone sulbactam were 27.8%. Imipenem and piperacillin tazobactam containing β-lactamase inhibitors were the most effective antibacterial therapies. Regression analysis showed that age, procalcitonin, and albumin was significantly correlated with antibacterial effects. The PAA model was established and had predicted the efficacy of Child-Turcortte-Pugh, model for end-stage liver disease, and model for end-stage liver disease combined with serum sodium. The specificity and sensitivity of the PAA was confirmed to be 94.12% and 93.62%, which was significantly higher than other models. Conclusion: The main common pathogenic bacterium of cirrhosis combined with bacterial pneumonia is Klebsiella pneumonia (G-bacilli). In addition, gram positive cocci (Staphylococcus aureus) and other are also visible. The elderly, diabetics and patients using hormones are prone to secondary fungal infections. Age, procalcitonin and serum albumin can accurately predict the antibacterial effect, guide clinical treatment and judge the prognosis of the established PAA model.

Objective: To explore the clinical features of post-viral-encephalitis autoimmune encephalitis (PVEAE). Methods: Ten cases of PVEAE, who were hospitalized in the Neurology Department of Peking Union Medical College Hospital (PUMCH) between November 2014 and October 2019, were retrospectively reviewed. Clinical manifestation, immunology, neuroradiology, treatment and outcomes were analyzed. Results: There were 5 males and 5 females, with a median age of 44 (18, 66) years. Of 9 cases, the median interval between the two onsets of encephalitis was 37 (24, 60) days, and there was no obvious interval in case 7. In viral encephalitis phase, the peak modified Rankin scale (mRS) was 4.5 (4.0, 5.0) and the remission mRS was 2.0 (1.0, 3.0). In autoimmune encephalitis (AE) phase, the peak mRS was 4.0 (3.0, 5.0). Symptoms of AE included mental and behavioral abnormalities (10/10), amnesia (10/10), motor disorders (5/10), autonomic dysfunction (5/10), speech disorders (4/10), seizures (2/10) and consciousness disturbance (2/10). On average, each case presented with 4 (2, 6) symptoms. In AE phase, the positive rate of anti-N-methyl-D-aspartate (anti-NMDA) receptor antibody in cerebrospinal fluid (CSF) was 80% (8/10), while in serum it was only 20% (2/10). Neuroimaging showed that in AE phase, the lesions expanded in 8 cases, remained unchanged in 1 case and shrank in 1 case. In AE phase, 10 cases received first line treatments, and 2 cases accepted long-course immunotherapy. After treatment, symptoms of 9 cases were obviously relieved. The mRS for short-term and long-term outcomes was 2.0 (1.0, 4.0) and 1.0 (0, 2.0), respectively. Conclusions: PVEAE might present with either typical biphasic course or monophasic/pseudo-monophasic course. In AE phase, anti-NMDA receptor antibody turned positive in most cases. Much importance should be attached to the recognition and diagnosis of PVEAE and treat it actively thereafter.

Objective: To clarify the clinical efficacy of Yiqi Huoxue recipe in the treatment of liver fibrosis of chronic viral hepatitis. Methods: An open, positive-drug, parallel-controlled study method was applied. A total of 207 cases of liver fibrosis with chronic hepatitis B and C diagnosed with liver biopsy and transient elastography were selected. According to the principle of syndrome differentiation in traditional Chinese medicine, self-made Yiqi Huoxue recipe ( n = 127) and Fuzheng Huayu capsule ( n = 80) were used for the treatment course of 24-48 weeks. Change score of TCM symptom, liver biochemistry, liver stiffness measurement (LSM), and noninvasive liver fibrosis index [aspartate transaminase to platelet ratio index (APRI), and fibrosis-4 score (FIB-4)] were compared between the two groups to evaluate the therapeutic effect of Yiqi Huoxue recipe on liver fibrosis. Results: Yiqi Huoxue recipe group and Fuzheng Huayu capsule group baseline LSM, APRI and FIB-4 was compared, and there was no statistically significant difference between them ( P > 0.05). Yiqi Huoxue recipe and Fuzheng Huayu capsule received patients had improved symptom scores to a certain extent. Hepatic facies, discomfort over liver area, and soreness and weakness of waist and knees ( P < 0.05) was significantly improved in Yiqi Huoxue recipe than Fuzheng Huayu capsule. Liver biochemical indicators (ALT, AST, GGT, ALP) had gradually relapsed with the extension of treatment duration and the normalization rate between the two groups after 24 to 48 weeks had reached 100% vs. 100%, 100% vs. 93.8%, 96.8% vs. 92.3% and 87.5% vs. 81.8%. After 12 weeks of treatment, APRI values ​​of both groups had significantly reduced, and after 48 weeks of treatment, LSM values of both groups had significantly improved. Moreover, Yiqi Huoxue recipe FIB-4 score was significantly improved after 48 weeks of treatment, and the difference was statistically significant compared to Fuzheng Huayu capsule group ( P < 0.05). After treatment, LSM, APRI, and FIB-4 total effectiveness in the two groups were 80.0% vs. 63.6%, P = 0.046; 68.4% vs. 52.0%, P = 0.052; 68.4% vs. 62.0%, P = 0.437, respectively. LSM total effectiveness was significantly higher in Yiqi Huoxue recipe treated group than Fuzheng Huayu capsule group. Conclusion: Traditional Chinese medicine Yiqi Huoxue decoction can be used as an optimal treatment for liver fibrosis of chronic viral hepatitis.

Diarrhea is a common digestive symptom. Here, we reported a case of young patient admitted with diarrhea caused by lead poisoning and cytomegalovirus infection. Through informative medical history and multi-disciplinary team discussion, Satoyoshi syndrome was finally diagnosed.

Direct-acting antiviral agents (DAAs) are the main antiviral therapeutics for hepatitis C virus-related decompensated stage cirrhosis. DAAs of NS3/4A protease inhibitors use is not recommended for patients with decompensated cirrhosis due to characteristics of DAAs metabolism in liver. The recent guidelines have recommended sofosbuvir (SOF)-based plan including pan-genotype plan of sofosbuvir(SOF)/velpatasvir (VEL), sofosbuvir combined with daclatasvir (DCV), genotype 1,4,5,6 specific plan of sofosbuvir (SOF) / ledipasvir (LDV) for 24 weeks or above in combination with ribavirin for 12 weeks because NS5B and NS5A inhibitors has no obvious effect on CYP450 enzyme system and achievement of sustained virological response (SVR) rates at 12/24 weeks is achievable in 88% ~ 100%, and liver reserve function improves in 42% ~ 53% of patients. Furthermore, approximately 15.5% ~ 49% of patients waiting for liver transplantation after treatment with DAAs do not require liver transplantation for short-term and 10.3% ~19.2% of patients receiving SOF/LDV, and SOF combined with DCV not needed liver transplantation. Thus, the clinical application of DAAs provides a safe and reliable antiviral treatment plan for hepatitis C virus-related decompensated stage cirrhosis.

Objective: To summarize the neurological manifestations in patients with primary Sjögren's syndrome (pSS). Methods: A total of 68 patients were diagnosed as pSS in neurology department of Peking Union Medical College Hospital from March 2014 to February 2018, among whom sixteen cases were excluded due to modified final diagnoses of primary neurological diseases. Therefore 52 pSS patients with neurological involvement were enrolled and retrospectively analyzed. They were divided into two groups as extensive group in which both central and peripheral nervous system were involved, non-extensive group in which either central or peripheral nervous system was involved. Results: Neurological manifestations were presented as primary symptoms in 98.1%(51/52) patients, while 35 had neurological involvement as their only extraglandular manifestations. Thirteen cases were in extensive group. The other 39 in non-extensive group including 22 cases with only peripheral nervous system involved and 17 cases with only single central nervous system involved. Compared to non-extensive group, the proportion of woman patients [13/13 vs.71.8% (28/39), P =0.047], serum IgG level [17.73(11.11,22.41)g/L vs. 11.49(9.58,13.40)g/L, P =0.017] and positive rates of oligoclonal band (OB) in cerebral spinal fluid (CSF) [7/13 vs. 22.6%(7/31), P =0.042)] were significantly higher in extensive involvement group. Conclusions: Neurological manifestations in pSS patients could be extensive, both central and peripheral nervous system might be associated. Female patients, high serum IgG level and positive OB in CSF are risk factors of extensive neurological involvement, suggesting that the immune system may be generally over-stimulated.

Objective: To explore the clinical value of plasma heme oxygenase 1(HO-1) in the development of non-alcoholic fatty liver disease(NAFLD). Methods: Patients with NAFLD were selected from the Physical examination center and the Department of Traditional and Western Medical Hepatology of Third Hospital of Hebei Medical University. A combination of ultrasound and liver elastography was used to screen NAFLD patients and healthy persons. General clinical characteristics, peripheral blood cell count and liver biochemical test results were collected synchronously, plasma samples were retained, and plasma HO-1 level was detected by enzyme-linked immunosorbent assay. SPSS21.0 statistical software was used for statistical analysis, multivariate logistic regression analyses was used to analyse the independent risk factors affecting the incidence and progression of NAFLD. The diagnostic efficacy of indicators related to development of NAFLD was assessed by the receiver operating characteristic curve(ROC). Results: A total of 328 patients with NAFLD and 113 healthy controls were included. According to the liver biochemical results, the NAFLD group was divided into 148 patients with normal liver enzymes and 180 patients with abnormal liver enzymes. The level of HO-1 in the three groups was 9.09 ± 2.19, 14.38 ± 2.63, 17.00 ± 3.30 ng/ml, and was increased respectively of healthy controls, patients with normal liver enzymes and patients with abnormal liver enzymes. Analyzing plasma HO-1 levels of components associated with metabolic disorders suggests that components without metabolic syndrome(9.83 ± 3.21) < components with 1 metabolic syndrome(13.59 ± 3.72) < components with 2 or more metabolic syndrome(16.09 ± 3.41), P < 0.001. The results of HO-1 level stratification analysis showed that WBC, ALT, AST, GGT, TG increased as HO-1 level increased, and the pairwise difference was statistically significant ( P < 0.001). The WBC count of NAFLD is significantly higher than healthy group(6.79 ± 1.62 vs 5.68 ± 1.36, P < 0.001). The univariate and multivariate regression analyses of all the subjects showed that HO-1, TG and BMI were prognostic factors for the occurrence of NAFLD and HO-1, TC, GLU were prognostic factors for the progression of NAFLD, P < 0.05. The ROC analysis showed that HO-1 was reliable markers for predicting the occurrence and progression of NALFD, the sensitivity and specificity were respectively 85.10%, 92.90% and 38.33%, 95.27%. Conclusion: Plasma HO-1 can predict the occurrence and progression of NAFLD and is expected to be a novel molecular diagnostic marker for NAFLD and NASH.

Objective: To investigate the prevalence on imaging, topographic distribution and risk factors of perivascular space (PVS) in community population. Methods: This study was based on the population of Shunyi Study in China from June 2013 to September 2016. One thousand two hundred and thirty-two stroke free participants older than 35 years, who completed cerebral MRI, were included. Cardiovascular risk factors were assessed by interview, physical examination and blood sample tests. PVS were evaluated on high-resolution 3-dimensional-T1WI, T2WI and FLAIR sequences. On T1WI, PVS were detected according to 3D imaging criteria. The number and anatomic location of large PVS (≥3 mm) were recorded. And severity of PVS was then semiquantitatively graded in both white matter (WM) and basal ganglia (BG). Associations between risk factors and PVS were analyzed by multinominal Logistic regression models and adjusted for age, gender and relevant confounders. Results: Perivascular spaces were observed in the BG or WM in every subject, aged (55±10) years and 37.3% male. Large PVS were detected in 19.0% (460/1 232) of participants. Severity of PVS was found to be strongly associated with age in both basal ganglia (severe versus mild: OR= 1.81; 95% CI 1.45-2.27, P< 0.01) and white matter ( OR= 1.39; 95% CI 1.13-1.71, P< 0.01), and the proportion of subjects with hypercholesterolemia decreased with the severity of PVS in BG ( OR= 0.58, P= 0.01) . The present of large PVS was found to be associated with age(present versus absent: OR= 1.03, 95 %CI 1.02-1.05, P< 0.01) and ApoE ε4 carrier genotype( OR= 1.53, 95 %CI 1.01-2.23, P= 0.04). Conclusions: Perivascular spaces are always detected in the BG or WM in elderly people, and large PVS are also prevalent in community-based population. The risk factors are different due to distribution and size, and may depend on anatomic or pathologic characteristics.

Objective: To investigate a diffusion weighted imaging (DWI)-based morphological classification for acute isolated pontine infarcts (AIPI) and to assess differences in clinical characteristics, risk factors and functional outcomes among the different morphologies. Methods: All participants from SMART cohort with DWI-proven AIPI were included and categorized into 3 groups: basilar artery branch disease (BABD) group, small artery disease (SAD) group and large-artery-occlusive disease (LAOD) group, according to lesion extent of the transverse axial plane and basilar artery atherosclerosis severity. The clinical characteristics, vascular risk factors and 6-month's functional outcome was analyzed among 3 groups. Results: Of the 1 129 patients enrolled, 175 had AIPI. BABD was the most frequent subtype of AIPI (46.3%), followed by SAD (36.0%) and LAOD (17.7%). Neurological impairment on admission was more severe in the LAOD and BABD group than SAD group ( P <0.001). In terms of risk factors, the percentage of hypertension was significant different among three groups ( P <0.05). The average National Institute of Health stroke scale was 3.49 for SAD group, 5.93 for LAOD group, 5.97 for BABD group, and the differences were significant ( P <0.001). The Poor outcome (mRS>2) was found in only 13.7% of patients at 6-month post-stroke and there was no difference among 3 groups. Conclusions: According to the morphological classification, BABD is the most frequent subtype of AIPI. The differences of the clinical characteristics and risk factors among three groups indicate that differences observed in morphology might have distinct pathophysiologic mechanisms. AIPI can be reliably classified based on morphology using clinical magnetic resonance images.