Objective:Through a typical case of corticobasal degeneration (CBD) with primary progressive aphasia (PPA) to analyze the clinical characteristics of CBD and the special manifestations of aphasia with that disease.Methods:Retrospective analysis was performed on a patient with PPA based CBD who was admitted to the First Affiliated Hospital, Sun Yat-sen University in July 2020 to summarize the clinical features and diagnostic thinking of CBD.Results:The patient was a 59-year-old male, manifested rapidly progressive dysfunction of language and memory function. The aphasia was mainly featured as slow speech, reduced content and grammatical errors, and diagnosed as PPA, non-fluent grammatical variation. The imaging results showed the atrophy of the left frontal lobe, parietal lobe, basal ganglia and thalamus, coupled with the reduction in 18F-fluorodeoxyglucose radioactive uptake. The patient was finally diagnosed as possible CBD. Conclusions:PPA as the initial manifestation of CBD is very rare in clinical practice. The high non-specificity of clinical features and the lack of typical motor symptoms result in the difficulty of correct diagnosis of CBD. Timely functional imaging in nuclear medicine and reliable biomarkers help to facilitate early diagnosis of atypical CBD.

Objective:To discuss the clinical features of a family with Welander distal myopathy and analyze the genetic characteristics of the T-cell intracellular antigen 1 (TIA1) gene mutation in this family.Methods:The clinical data, electrophysiological and pathological examination results of some family members were collected, and the proband was tested by next generation sequencing techniques to detect possible pathogenic mutations. Sanger sequencing was performed in some family members for the gene mutations closely related to the clinical phenotype.Results:The proband, a 30-year-old man, manifested progressive weakness and muscle atrophy in distal limbs, followed by the involvement of muscles in proximal limbs. A gene mutation of c.91G>A was detected by genetic testing in the TIA1 gene, which was associated with Welander distal myopathy. The further Sanger sequencing revealed the same mutation site in the proband′s mother, one younger brother and his youngest uncle, who showed similar symptoms as the proband including muscle weakness and atrophy. The youngest brother of the proband was a mutation carrier without obvious symptoms, and his electromyography test showed myogenic injuries.Conclusions:Welander′s distal myopathy is a slowly progressing autosomal dominant disorder, characterized by weakness and muscle atrophy mainly in the extremities. In this family, the patients showed the onset in the extremities of the lower limbs and presented weakness and atrophy in distal and proximal limbs, with disease heterogeneity among patients. Genetic testing and the analysis of the family members confirmed the diagnosis of Welander′s distal myopathy and the pathogenic mutation c.91G>A in the TIA1 gene.

Objective　To explore the characteristics of musical cognition in aged Cerebral Small Vessel Disease (CSVD) patients.Methods　Thirty-five aged CSVD individuals as well as 32 elderly subjects were recruited from Jan to Sep 2019 in Department of Neurology,the Seventh Medical Center for PLAGH.Montreal Battery of Evaluation of Amusia (MBEA) was chosen to analyze musical cognition,including pitch,rhythm and memory scores.Meanwhile,demographic data and amount of cognitive assessments were also collected.Student t-test for two independent samples was used to compare the data between groups,Pearson correlations were chosen to analyze the relationship between musical cognition parameters and other variants.Results　Compared to control group,aged CSVD individuals showed significant different ability of musical cognition,reflected by lower scores in pitch,rhythm and memory (P<0.05).Rhythm score correlated with Digital Symbol Substitution Test (DSST) (r=0.374,P=0.002).Memory score correlated with DSST (r=0.278,P=0.023) as well as Choice Reaction Time (CRT) (r=0.323,P=0.008).Total score correlated with DSST (r=0.338,P=0.005) as well as CRT (r=0.247,P=0.044).Conclusion　Significant differences could be found in musical cognition between aged CSVD patients and elderly control,and could be helpful to reveal the executive dysfunctions in aged CSVD subjects.

Dual task walking (DTW) refers to the task of performing another attentional demand while walking naturally. It is quite common in our daily life, such as having a conversation while walking, or taking a call while crossing the street. People can simultaneously perform dual tasks normally. However, the performance of dual tasks will decline when the gait is impaired or cognitive dysfunction occurs.Neurodegenerative disease (NDD) is mainly characterized by motor and cognitive dysfunction, which seriously affects patients' ability of daily living activities. In recent years, studies on DTW in patients with NDD have found that decreased performance of DTW may be a feature of NDD patients, which may have important clinical value for early identification of NDD and predict disease progression. In the future, effective DTW training may improve the patients' ability of daily living activities, and it is important for the rehabilitation of NDD patients. This article mainly reviews the definition, common methods and mechanisms of DTW, as well as the clinical research findings and applications of DTW in patients with NDD, and provides theoretical basis for further clinical research.

Objective To establish a method to simultaneously determine syringing and isofraxidin by HPLC;To investigate the features of percutaneous absorption in vitro of Jiegugao blended and pasted by white vinegar, honey and vaseline; To discuss the mechanism of commonly used ointment matrices in Tujia Minority. Methods Rat abdomen skin in vitro was as transdermal barrier;the modified Franz diffusion pool was used to simulate human skin medication; the content of syringin and isoprofen was determined by HPLC; the percutaneous absorption equation was established and the related parameters, such as cumulative permeation rate and permeation rate, were calculated. Results When using Syncronis C18 (250 mm × 4.6 mm, 5 μm) as chromatographic column, acetonitrile-0.1%phosphoric acid as mobile phase, 1.0 mL/min as perfusion speed and 265 nm as determine wavelength, regression equation of syringingwas A=10 686.454 6C+1565.778 8 (r=1.000 0), regression equation of isofraxidin was A=12 297.305 4C-5913.729 9 (r=0.999 9). Cumulative permeation quantity of syringing in Jiegugao blended and pasted by white vinegar, honey, vaseline and blank were 7.549 2, 4.580 3, 3.890 8 and 5.378 4 μg?cm-2?h-1 respectively and permeation rate were 25.66%, 16.11%, 13.73% and 18.78%. Meanwhile, cumulative permeation quantity of isofraxidin were 2.536 9, 1.941 8, 1.178 2 and 2.293 6 μg?cm-2?h-1 respectively and permeation rate were 47.04%, 35.06%, 22.11%and 41.11%. Conclusion Using white vinegar as the ointment matrix can promote the percutaneous absorption of effective composition in Jiegugao blended. However, it will retard the percutaneous absorption of effective composition in Jiegugao when using honey and vaseline as the ointment matrices, but honey and vaseline can be used as a slow-release matrix.

Objective To study the spatial and temporal mode of infectious TB transmission in Guangxi Zhuang Autonomous Region (Guangxi).Methods Data related to infectious TB case (Include smear and/or culture positive patients) in Guangxi were collected from the National Notifiable Disease Reported System (NNDRS) from 2010 to 2015.Spatial-temporal analysis and prediction were performed by SaTScan 7.0.2,GeoDa 1.8.12,R program v 3.3.1 and SPSS 19.0 software,using the time series model,Moran' s I global and local spatial autocorrelation (Empirical Bayes adjustment).Kulldorff ' s space-time scan statistics displayed by R software was used to identify the temporal and spatial trend of TB.Results The total number of infectious TB cases,collected from NNDRS was 76 151,and showing a decreasing trend on annual incidence (value of Chi-square for Linear trend=3 464.53,P-value=0.000).The forecast value of TB cases in 2016 was 7 764 (4 971-10 557),with peak in March,analyzed through the Winters' multiplicative model.The Moran' s I global Statistics was greater than 0 (0.257-0.390).TB cluster seemed to have been existed for several years.The most significant hot spots seemed to be mainly located in the central and western parts of Guangxi,shown by local spatial autocorrelation statistics and the result from space-time scanning.Counties or districts that located in the east parts of Guangxi presented the low-low relation (significant cold spots).The situation of infectious TB seemed migratory.Conclusions Our data showed an annual decreasing trend of incidence on infectious TB with temporal concentration in spring and summer.Main clusters (hot spots) were found to be located in the central and western parts of Guangxi.Hopefully,our findings can provide clues to uncover the real mode of TB transmission at the molecular-biological level.

Bio-based polyamides are environment-friendly polymers. The precursors of bio-based polyamides come from bio-based materials such as castor oil, glucose and animal oil. Bio-based polyamides precursors include bio-based amino acids, bio-based lactams, bio-based diprotic acid and bio-based diamines. In this paper, we discussed the route of the precursors of bio-based polyamides that come from bio-based materials. We discussed the properties of bio-based polyamides. Bio-based PA11and bio-based PA1010 are well-known bio-based polyamides; we discussed the origin materials of the precursors, the route of manufacturing bio-based PA11 and PA1010, and their modifications status. The variety, classification and commercial production of bio-based polyamides were described in details, as well as bio-based polyamides development in China.
Animals ; Biotechnology ; Castor Oil ; China ; Glucose ; Nylons ; chemistry ; Polymers

Objective To observe the inhibitory effect of quercetin on the biofilm formation of Streptococcus mutans(Sm),to preliminarily reveal the possible underlying mechanisms,and to evaluate the cytotoxicity of quercetion to human dental pulp cells so as to provide the theoretical basis for the application of quercetin in oral biomaterials.Methods Quercetin storage solution was diluted to 0,3.125,6.25,12.5,25,50,100,200,400 and 800 mg/L,and added into Sm medium for 4 h and 24 h,crystal violet staining was used to evaluate the biofilm volume.In subsequent detections,three groups were set:control(0 mg/L),200 mg/L quercetin and 400 mg/L quercetin.Confocal laser scanning microscopy was used to observe the morphology of the biofilm;qPCR for gtfB,gtfC,comD,comE,and luxS were assessed to preliminarily investigate the mechanisms.Finally,the methyl thiazolyl tetrazolium(MTT) test using human dental pulp cells was used to investigate cytotoxicity.Results Quercetin could significantly inhibit up to (86.16±0.45)％ of the biofilm formation of Sm (Compared with the control group P=0.00) and effectively removed (43.04±0.53)％ of the mature biofilm(Compared with the control group P=0.00).Confocal laser scanning microscopy photographs showed that after co-incubated for 24 h,the dense biofilm structures of the experimental group were destroyed by quercetin both at 200 mg/L and 400 mg/L.Quercetin suppressedover 50％ of the expression of gtfB,gtfC,comD,comE(compared with the control group P＜0.05) and promoted the expression of luxS up to 2.18 ±0.24 and 2.84±0.26 after 4 h and 24 h,respectively(compared with the control group P＜0.05).Quercetin also exhibited acceptable compatibility for human dental pulp cells.Conclusions Quercetin could effectively reduce the biofilm formation of Sm by inhibiting the expression of the related genes,and exhibited no cytotoxicity for human dental pulp cells.Quercetin has good potential to be applied in oral biological materials.

ObjectiveTo assess the value of passive leg raising (PLR) test in predicting fluid responsiveness in patients with sepsis-induced cardiac dysfunction.Methods A prospective observational cohort study was conducted. Thirty-eight patients under mechanical ventilation suffering from sepsis-induced cardiac dysfunction admitted to Department of Surgical Intensive Care Unit of First Affiliated Hospital of Sun Yat-Sen University from September 2013 to July 2014 were enrolled. The patients were studied in four phases: before PLR (semi-recumbent position with the trunk in 45°), PLR (the lower limbs were raised to a 45° angle while the trunk was in a supine position), before volume expansion (VE, return to the semi-recumbent position), and VE with infusing of 250 mL 5% albumin within 30 minutes. Hemodynamic parameters were recorded in every phase. The patients were classified into two groups according to their response to VE: responders (at least a 15% increase in stroke volume,ΔSVVE≥15%), and non-responders. The correlations among all changes in hemodynamic parameters were analyzed by linear correlation analysis, and the receiver operating characteristic curve (ROC) was plotted to assess the value of hemodynamic parameters before and after PLR in predicting fluid responsiveness.Results Of 38 patients, 25 patients were responders, and 13 non-responders. There was no significant difference in the baseline and hemodynamic parameters at semi-recumbent position between the two groups. The changes in SV and cardiac output (CO) after PLR (ΔSVPLR andΔCOPLR) were significantly higher in responders than those of non-responders [ΔSVPLR: (14.7±5.7)%vs. (6.4±5.3)%,t = 4.304,P = 0.000;ΔCOPLR: (11.2±7.5)% vs. (3.4±2.3)%,t = 3.454,P = 0.001], but there was no significant difference in the changes in systolic blood pressure, mean arterial pressure, pulse pressure, and heart rate after PLR (ΔSBPPLR,ΔMAPPLR,ΔPPPLR andΔHRPLR) between two groups.ΔSVVE in responders was significantly higher than that of the non-responders [(20.8±5.5) % vs. (5.0±3.7) %,t = 8.347,P = 0.000]. It was shown by correlation analysis thatΔSVPLR was positively correlated withΔSVVE (r = 0.593,P = 0.000),ΔCOPLR was positively correlated withΔSVVE (r = 0.494,P = 0.002). The area under ROC curve (AUC) ofΔSVPLR≥8.1% for predicting fluid responsiveness was 0.860±0.062 (P = 0.000), with sensitivity of 92.0% and specificity of 70.0%; the AUC ofΔCOPLR≥5.6% for predicting fluid responsiveness was 0.840±0.070 (P = 0.000), with sensitivity of 84.0%and specificity of 76.9%; the AUC ofΔMAPPLR≥6.9% for predicting fluid responsiveness was 0.662±0.089, with sensitivity of 68.0% and specificity of 76.9%; the AUC ofΔSBPPLR≥6.4% for predicting fluid responsiveness was 0.628±0.098, with sensitivity of 76.0% and specificity of 61.5%; the AUC ofΔPPPLR≥6.2% for predicting fluid responsiveness was 0.502±0.094, with sensitivity of 56.0% and specificity of 53.8%; the AUC ofΔHRPLR≥-1.7%for predicting fluid responsiveness was 0.457±0.100, with sensitivity of 56.0% and specificity of 46.2%.Conclusion In patients with sepsis-induced cardiac dysfunction, changes in SV and CO induced by PLR are accurate indices for predicting fluid responsiveness, but the changes in HR, MAP, SBP and PP cannot predict the fluid responsiveness.