1.Chinese expert consensus on blood support mode and blood transfusion strategies for emergency treatment of severe trauma patients (version 2024)
Yao LU ; Yang LI ; Leiying ZHANG ; Hao TANG ; Huidan JING ; Yaoli WANG ; Xiangzhi JIA ; Li BA ; Maohong BIAN ; Dan CAI ; Hui CAI ; Xiaohong CAI ; Zhanshan ZHA ; Bingyu CHEN ; Daqing CHEN ; Feng CHEN ; Guoan CHEN ; Haiming CHEN ; Jing CHEN ; Min CHEN ; Qing CHEN ; Shu CHEN ; Xi CHEN ; Jinfeng CHENG ; Xiaoling CHU ; Hongwang CUI ; Xin CUI ; Zhen DA ; Ying DAI ; Surong DENG ; Weiqun DONG ; Weimin FAN ; Ke FENG ; Danhui FU ; Yongshui FU ; Qi FU ; Xuemei FU ; Jia GAN ; Xinyu GAN ; Wei GAO ; Huaizheng GONG ; Rong GUI ; Geng GUO ; Ning HAN ; Yiwen HAO ; Wubing HE ; Qiang HONG ; Ruiqin HOU ; Wei HOU ; Jie HU ; Peiyang HU ; Xi HU ; Xiaoyu HU ; Guangbin HUANG ; Jie HUANG ; Xiangyan HUANG ; Yuanshuai HUANG ; Shouyong HUN ; Xuebing JIANG ; Ping JIN ; Dong LAI ; Aiping LE ; Hongmei LI ; Bijuan LI ; Cuiying LI ; Daihong LI ; Haihong LI ; He LI ; Hui LI ; Jianping LI ; Ning LI ; Xiying LI ; Xiangmin LI ; Xiaofei LI ; Xiaojuan LI ; Zhiqiang LI ; Zhongjun LI ; Zunyan LI ; Huaqin LIANG ; Xiaohua LIANG ; Dongfa LIAO ; Qun LIAO ; Yan LIAO ; Jiajin LIN ; Chunxia LIU ; Fenghua LIU ; Peixian LIU ; Tiemei LIU ; Xiaoxin LIU ; Zhiwei LIU ; Zhongdi LIU ; Hua LU ; Jianfeng LUAN ; Jianjun LUO ; Qun LUO ; Dingfeng LYU ; Qi LYU ; Xianping LYU ; Aijun MA ; Liqiang MA ; Shuxuan MA ; Xainjun MA ; Xiaogang MA ; Xiaoli MA ; Guoqing MAO ; Shijie MU ; Shaolin NIE ; Shujuan OUYANG ; Xilin OUYANG ; Chunqiu PAN ; Jian PAN ; Xiaohua PAN ; Lei PENG ; Tao PENG ; Baohua QIAN ; Shu QIAO ; Li QIN ; Ying REN ; Zhaoqi REN ; Ruiming RONG ; Changshan SU ; Mingwei SUN ; Wenwu SUN ; Zhenwei SUN ; Haiping TANG ; Xiaofeng TANG ; Changjiu TANG ; Cuihua TAO ; Zhibin TIAN ; Juan WANG ; Baoyan WANG ; Chunyan WANG ; Gefei WANG ; Haiyan WANG ; Hongjie WANG ; Peng WANG ; Pengli WANG ; Qiushi WANG ; Xiaoning WANG ; Xinhua WANG ; Xuefeng WANG ; Yong WANG ; Yongjun WANG ; Yuanjie WANG ; Zhihua WANG ; Shaojun WEI ; Yaming WEI ; Jianbo WEN ; Jun WEN ; Jiang WU ; Jufeng WU ; Aijun XIA ; Fei XIA ; Rong XIA ; Jue XIE ; Yanchao XING ; Yan XIONG ; Feng XU ; Yongzhu XU ; Yongan XU ; Yonghe YAN ; Beizhan YAN ; Jiang YANG ; Jiangcun YANG ; Jun YANG ; Xinwen YANG ; Yongyi YANG ; Chunyan YAO ; Mingliang YE ; Changlin YIN ; Ming YIN ; Wen YIN ; Lianling YU ; Shuhong YU ; Zebo YU ; Yigang YU ; Anyong YU ; Hong YUAN ; Yi YUAN ; Chan ZHANG ; Jinjun ZHANG ; Jun ZHANG ; Kai ZHANG ; Leibing ZHANG ; Quan ZHANG ; Rongjiang ZHANG ; Sanming ZHANG ; Shengji ZHANG ; Shuo ZHANG ; Wei ZHANG ; Weidong ZHANG ; Xi ZHANG ; Xingwen ZHANG ; Guixi ZHANG ; Xiaojun ZHANG ; Guoqing ZHAO ; Jianpeng ZHAO ; Shuming ZHAO ; Beibei ZHENG ; Shangen ZHENG ; Huayou ZHOU ; Jicheng ZHOU ; Lihong ZHOU ; Mou ZHOU ; Xiaoyu ZHOU ; Xuelian ZHOU ; Yuan ZHOU ; Zheng ZHOU ; Zuhuang ZHOU ; Haiyan ZHU ; Peiyuan ZHU ; Changju ZHU ; Lili ZHU ; Zhengguo WANG ; Jianxin JIANG ; Deqing WANG ; Jiongcai LAN ; Quanli WANG ; Yang YU ; Lianyang ZHANG ; Aiqing WEN
Chinese Journal of Trauma 2024;40(10):865-881
Patients with severe trauma require an extremely timely treatment and transfusion plays an irreplaceable role in the emergency treatment of such patients. An increasing number of evidence-based medicinal evidences and clinical practices suggest that patients with severe traumatic bleeding benefit from early transfusion of low-titer group O whole blood or hemostatic resuscitation with red blood cells, plasma and platelet of a balanced ratio. However, the current domestic mode of blood supply cannot fully meet the requirements of timely and effective blood transfusion for emergency treatment of patients with severe trauma in clinical practice. In order to solve the key problems in blood supply and blood transfusion strategies for emergency treatment of severe trauma, Branch of Clinical Transfusion Medicine of Chinese Medical Association, Group for Trauma Emergency Care and Multiple Injuries of Trauma Branch of Chinese Medical Association, Young Scholar Group of Disaster Medicine Branch of Chinese Medical Association organized domestic experts of blood transfusion medicine and trauma treatment to jointly formulate Chinese expert consensus on blood support mode and blood transfusion strategies for emergency treatment of severe trauma patients ( version 2024). Based on the evidence-based medical evidence and Delphi method of expert consultation and voting, 10 recommendations were put forward from two aspects of blood support mode and transfusion strategies, aiming to provide a reference for transfusion resuscitation in the emergency treatment of severe trauma and further improve the success rate of treatment of patients with severe trauma.
2. Differentially expressed genes in peripheral blood of patients with dermatomyositis complicated by interstitial lung disease or malignant tumors
Ke XUE ; Cheng QUAN ; Qian ZHAO ; Licheng DIAO ; Mengya CHEN ; Xuemei ZHU ; Jie ZHENG ; Hua CAO ; Hao LI
Chinese Journal of Dermatology 2020;53(1):23-29
Objective:
To investigate differentially expressed genes and related signaling pathways in patients with dermatomyositis/clinical amyopathic dermatomyositis (DM/CADM) complicated by interstitial lung disease or malignant tumors.
Methods:
From January 2017 to January 2018, 27 DM/CADM patients were enrolled from Department of Dermatology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, and divided into 3 groups according to the complications: 10 with interstitial lung disease, 8 with malignant tumors, and 9 without interstitial lung disease or malignant tumors. Meanwhile, 7 healthy controls were enrolled into this study. High-throughput RNA sequencing was performed to screen differentially expressed genes in peripheral blood in the above 4 groups. Then, these genes were subjected to gene ontology (GO) analysis and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichment analysis.
Results:
Compared with the healthy controls, 4 820 up-regulated genes and 137 down-regulated genes were identified in DM/CADM patients; GO analysis revealed 49 significantly enriched items in the DM/CADM patients, 37 (75.5%) of which were associated with biological processes; KEGG analysis showed that differentially expressed genes were mainly enriched in infection-, tumor- and immune-related pathways in DM/CADM patients. Compared with the patients without interstitial lung disease or malignant tumors, 272 up-regulated genes and 158 down-regulated genes were identified in the patients with interstitial lung disease; GO analysis revealed 157 significantly enriched items, 114 (72.6%) of which were associated with biological processes; KEGG analysis showed that differentially expressed genes were mainly enriched in bacterial infection- and autoimmune/inflammatory-related pathways in the patients with interstitial lung disease. Compared with the patients without interstitial lung disease or malignant tumors, 398 up-regulated genes and 68 down-regulated genes were identified in the patients with malignant tumors; GO analysis revealed 117 significantly enriched items, 94 (80.3%) of which were associated with biological processes; KEGG analysis showed that differentially expressed genes were mainly enriched in glycosylation-, metabolism- and tumor-related signaling pathways in the patients with malignant tumors.
Conclusions
Differences existed in transcriptomes and pathways between the DM/CADM patients and healthy controls, as well as between the patients with interstitial lung disease or malignant tumors and patients without these complications. Bacterial infection- and cytokine/chemokine-related pathways were significantly enriched in the patients with DM/CADM complicated by interstitial lung disease, while those pathways related to glycosylation, protein metabolism, angtigen presentation and cytotoxic effects of natural killer cells were significantly enriched in the patients with DM/CADM complicated by malignant tumors.
3.Effect of Five-tone Music Therapy on Anxiety, Depression and Quality of Life of Patients Treated with Chemotherapy After Gastric Cancer Radical Surgery
Xuemei LIN ; Xiaoming QUAN ; Yaoru LIN ; Guangyun HU
Journal of Guangzhou University of Traditional Chinese Medicine 2017;34(2):181-184
Objective To observe the effect of five-tone music therapy on relieving anxiety and depression and on improving the quality of life of patients receiving chemotherapy after gastric cancer radical surgery.Methods Ninety anxiety and depression in-patients treated with chemotherapy after gastric cancer radical surgery were randomly divided into observation group and control group,45 cases in each group.Patients of the two groups were treated with the same chemotherapy regimen and nursing,and additionally,the observation group was given fivetone musie therapy and the control group was given conventional music therapy.The intervention effects were evaluated by scoring with Self-rating Anxiety Scale (SAS),Self-rating Depression Scale (SDS) and questionnaire for the quality of life of gastric cancer patients (QLQ-STO22) at admission time and when discharging from hospital.Results The SAS and SDS standard scores and the scores of each dimension of QLQ-STO22 in the observation group were markedly decreased after intervention,the differences being statistically significant (P < 0.01 compared with those before intervention).And the SAS and SDS standard scores of the observation group after intervention were lower than those of the control group (P < 0.01).In the aspects of quality of life,the scores of all dimension of QLQ-STO22 except taste,appearance and hair loss in the observation group were lower than those in the control group (P < O.05).Conclusion Five-tone music therapy is effective on relieving anxiety and depression and on improving the quality of life of patients receiving chemotherapy after gastric cancer radical surgery.
4.Molecular mechanism of SCARB2-mediated attachment and uncoating of EV71.
Minghao DANG ; Xiangxi WANG ; Quan WANG ; Yaxin WANG ; Jianping LIN ; Yuna SUN ; Xuemei LI ; Liguo ZHANG ; Zhiyong LOU ; Junzhi WANG ; Zihe RAO
Protein & Cell 2014;5(9):692-703
Unlike the well-established picture for the entry of enveloped viruses, the mechanism of cellular entry of non-enveloped eukaryotic viruses remains largely mysterious. Picornaviruses are representative models for such viruses, and initiate this entry process by their functional receptors. Here we present the structural and functional studies of SCARB2, a functional receptor of the important human enterovirus 71 (EV71). SCARB2 is responsible for attachment as well as uncoating of EV71. Differences in the structures of SCARB2 under neutral and acidic conditions reveal that SCARB2 undergoes a pivotal pH-dependent conformational change which opens a lipid-transfer tunnel to mediate the expulsion of a hydrophobic pocket factor from the virion, a pre-requisite for uncoating. We have also identified the key residues essential for attachment to SCARB2, identifying the canyon region of EV71 as mediating the receptor interaction. Together these results provide a clear understanding of cellular attachment and initiation of uncoating for enteroviruses.
Acids
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chemistry
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Amino Acid Sequence
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Animals
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Capsid Proteins
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chemistry
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genetics
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metabolism
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Enterovirus A, Human
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genetics
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metabolism
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physiology
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HEK293 Cells
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Host-Pathogen Interactions
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Humans
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Hydrogen-Ion Concentration
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Lysosome-Associated Membrane Glycoproteins
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chemistry
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genetics
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metabolism
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Molecular Docking Simulation
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Molecular Sequence Data
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Protein Binding
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Protein Conformation
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Protein Interaction Mapping
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Protein Structure, Tertiary
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RNA, Viral
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genetics
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metabolism
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Receptors, Scavenger
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chemistry
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genetics
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metabolism
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Sequence Homology, Amino Acid
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Sf9 Cells
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Static Electricity
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Virion
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genetics
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metabolism
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Virus Attachment
5.Validation study of chronic kidney disease epidemiology collaboration predictive equations based on serum creatinine and cystatin C in Chinese chronic kidney disease patients
Xiuzhi GUO ; Yan QIN ; Ke ZHENG ; Ling QIU ; Wei CUI ; Xinqi CHENG ; Weiling SHOU ; Yan ZHU ; Qian DI ; Xuzhen QIN ; Huijuan HAN ; Guoqiang QUAN ; Jiafu FENG ; Xuemei LI
Chinese Journal of Laboratory Medicine 2012;35(9):798-804
Objective To validate the four chronic kidney disease epidemiology collaboration (CKD-EPI) predictive equations based on serum creatinine (SCr) and cystatin C (Cys C) in Chinese CKD patients,and try to develop the GFR predictive equations for Chinese CKD patients.Methods254 CKD patients were randomly selected from four Grade ⅢA hospitals in different regions in China from September 2007 to December 2010.Clearance of dual plasma sampling 99mTc-DTPA was used to measure glomerular filtration rate (rGFR) in 254 CKD patients.The serum concentration of Cr and Cys C were measured.CKD-EPI SCr equation,Cys C equation,Cys C equation adjusted for age,sex and race,SCr/Cys C combinated equation adjusted for age,sex and race were used to estimate GRF ( labeled as eGFR1,eGFR2,eGFR3 and eGFR4,respectively).The correlation,bias and precision of eGFRs were compared with rGFR by Wilcoxon signed rank test,intraclass correlation coefficient (ICC) and Spearman correlation analysis.The deviation degree between rGFR and different eGFRs was compared via Bland-Altman graph.The accuracy within 15%,25%,30% ( P15,P25,P30) and the staging correctness of eGFR against CKD at different stages was calculated.ResultsThe rGFR in 254 CKD participants was [ 48.07 (26.19 -92.97 )] ml · min -1·(1.73 m2) -1.The Spearman correlatiou coefficients (CC) of eGFR and rGFR varied within the range of 0.873 - 0.896 ( P =0.000 ).The intra-class CC ( ICC ) varied within the range of 0.920 - 0.942.The correlation of eGFR4 was the best.The absolute deviations of 4 eGFRs and deviation precision were eGFR4 <eGFR3 < eGFR2 < eGFR1.The 95% confidence intervals for the regression line of 4 eGFRs shown by Bland-Altman graphs were 92.5,87.3,83.0 and 76.1 ml · min-1 · ( 1.73 m2 ) -1,respectively,with the best result of eGFR4.For P30,the correctness of 4 eGFRs were eGFR4 > eGFR3 > eGFR2 > eGFR1,but no significant difference was found by Chi square test (x2 =6.448,P =0.092).The overall correctness rate in 4 eGFRs against CKD stages were 48.4% -57.5%,with the highest consistency of eGFR4,but their staging correctnessratewerenotideal(Kappa values were 0.405,0.348,0.366 and 0.463,respectively).Conclusions Compared with CKD-EPI SCr equation,no advantage was found in CKD-EPI Cys C equation.The Cys C equation adjusted by age and sex shows a little advantages over CKD-EPI Cys C equation in bias,precision,correlation and accuracy.The CKD-EPI SCr/Cys C combinated equation adjusted by age,sex and race has advantage over other three equations not only in bias,precision,correlation and accuracy,but also in staging correctness.However,the validation of this equation is still not fairly ideal for Chinese CKD patients.Based on these findings,it is essential for the Chinese CKD patients to develop SCr/Cys C combined predictive equation which adjusted by age,sex or other factors.(Chin J Lab Med,2012,35:798-804)
6.Human Bop is a novel BH3-only member of the Bcl-2 protein family.
Xiaoping ZHANG ; Changjiang WENG ; Yuan LI ; Xiaoyan WANG ; Chunsun JIANG ; Xuemei LI ; Youli XU ; Quan CHEN ; Lei PAN ; Hong TANG
Protein & Cell 2012;3(10):790-801
One group of Bcl-2 protein family, which shares only the BH3 domain (BH3-only), is critically involved in the regulation of programmed cell death. Herein we demonstrated a novel human BH3-only protein (designated as Bop) which could induce apoptosis in a BH3 domain-dependent manner. Further analysis indicated that Bop mainly localized to mitochondria and used its BH3 domain to contact the loop regions of voltage dependent anion channel 1 (VDAC1) in the outer mitochondrial membrane. In addition, purified Bop protein induced the loss of mitochondrial transmembrane potential (Δψm) and the release of cytochrome c. Furthermore, Bop used its BH3 domain to contact pro-survival Bcl-2 family members (Bcl-2, Bcl-X(L), Mcl-1, A1 and Bcl-w), which could inhibit Bop-induced apoptosis. Bop would be constrained by pro-survival Bcl-2 proteins in resting cells, because Bop became released from phosphorylated Bcl-2 induced by microtubule-interfering agent like vincristine (VCR). Indeed, knockdown experiments indicated that Bop was partially required for VCR induced cell death. Finally, Bop might need to function through Bak and Bax, likely by releasing Bak from Bcl-X(L) sequestration. In conclusion, Bop may be a novel BH3-only factor that can engage with the regulatory network of Bcl-2 family members to process intrinsic apoptotic signaling.
Amino Acid Sequence
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Animals
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Apoptosis
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Cell Line
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Cell Survival
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Humans
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Mice
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Mitochondria
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metabolism
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Mitochondrial Membranes
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metabolism
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Molecular Sequence Data
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Protein Structure, Tertiary
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Protein Transport
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Proto-Oncogene Proteins c-bcl-2
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chemistry
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metabolism
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Signal Transduction
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Time Factors
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Voltage-Dependent Anion Channel 1
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metabolism
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bcl-2 Homologous Antagonist-Killer Protein
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metabolism
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bcl-2-Associated X Protein
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metabolism
7.Studies on chemical constituents of Illicium simonsii.
Jifeng LIU ; Xuemei ZHANG ; Yao SHI ; Quan ZHANG ; Yunbao MA ; Jijun CHEN
China Journal of Chinese Materia Medica 2011;36(10):1311-1315
OBJECTIVETo study the chemical constituents of the Illicium simonsii.
METHODThe stems and leaves of I. simonsii were extracted with 95% EtOH. The EtOH extract was dispersed in H2O and extracted with petroleum, CHCl3 and BuOH, successively. The CHCl3 and BuOH fractions were isolated and purified by column chromatography on silica gel, Sephadex LH-20, Rp-C8 and Rp-C18. The isolated compounds were identified on the basis of spectral analyses (including MS, 1H-NMR, 13C-NMR).
RESULTFourteen compounds were isolated from the stems and leaves of I. simonsii, which were characterized as ficusesquilignan A (1), buddlenol C (2), buddlenol D (3), leptolepisol A (4), acernikol (5), aviculin (6), kaempferol (7), quercetin (8), quercetin 3-O-alpha-L-rhamnopyranosyl-(1 --> 6) -beta-D-glucopyranoside (9), taxifolin-3-O-beta-D-xylopyranoside (10), benzyl-2-O-beta-D-glucopyranosyl-2,6-dihydroxybenzoate (11), 2,4-dihydroxy-3,6-dimethyl-methylbenzoate (12), biondinin C (13), shikimic acid (14).
CONCLUSIONExcept compounds 9 and 14, all the other compounds were obtained from I. simonsii for the first time.
Drugs, Chinese Herbal ; chemistry ; isolation & purification ; Illicium ; chemistry ; Plant Leaves ; chemistry
8.A comparison study on histopathology and ultrastructure of lesions from patients with bullous ichthyosiform erythrodermia before and after treatment with acitretin
Xin TIAN ; Xuemei LI ; Xin ZHOU ; Yuqing HE ; Quan LUO ; Sanquan ZHANG ; Yuwu LUO ; Yumei LIU ; Li CUI ; Xibao ZHANG
Chinese Journal of Dermatology 2010;43(11):749-752
Objective To investigate the effect of acitretin on the histopathology and ultrastructure of lesions from patients with bullous ichthyosiform erythrodermia (BIE), and to explore mechanisms underlying the modulation of keratinization process by acitretin. Methods Lesional tissue was obtained from the back of 4 patients with BIE before and after the treatment with acitretin. Light microscopy and transmission electron microscopy were performed to observe histopathological and ultrastructural changes in these lesions. Results After treatment, the improvement in clinical manifestations was more than 75% in all the 4 patients, and reached 90% in 1 of the 4 patients. As histopathology and ultrastructural study showed, there was an obvious improvement in hyperkeratosis and continuity of extra cellular lamellar membrane, and a decrease in keratin deposition in prickle and granular layer, but no remarkable changes were observed for the proliferation of prickle cells or acantholysis. Conclusions Acitretin shows a favorable efficacy in clinical treatment of BIE,with histopathological and ultrastructural improvement mainly located in the stratum corneum. The modulation of keratinization process in keratinocytes by acitretin appears more apparent in granular and corneum layers.
9.Diagnosis and treatment of porcupine-like ichthyosis: a case report
Xibao ZHANG ; Xuemei LI ; Jianfang SUN ; Yuqing HE ; Xin TIAN ; Quan LUO ; Xiao XU ; Changxing LI ; Xin ZHOU ; Wei LI ; Li CUI ; Gengyun TANG ; Xinjing GAO
Chinese Journal of Dermatology 2010;43(11):801-804
A 10-year-old boy presented with a 3-year history of erythematous flat keratotic papules and brown-yellow, nail-like prominent keratotic plaques all over the body surface. Dermatological examination showed verrucous or nail-like prominence over multiple erythematous keratotic plaques on the head, face,trunk and limbs. The lesions, most of which confluenced, were covered with brown-yellow and greasy crusts,and gave a porcupine-like appearance. Skin biopsy of lesions from the back revealed epidermal hyperkeratosis,focal columnar parakeratosis, acanthosis, few acantholytic and dyskeratotic cells in stratum corneum, irregular upward proliferation of dermal papilla cells, and a superficial perivascular lymphocytic infiltration. A diagnosis of ichthyosis hystrix was established based on the histopathological findings. The boy was treated with oral acitretin and topical 0.1% acitretin cream for 8 years. The initial and maximum dose of oral acitretin was 0.5 mg·kg-1·d-1 and 1 mg·kg-1·d-1, respectively. Liver and kidney function, body height and weight were examined during the treatment, and no side effect was observed except for skin xerosis.
10.Phenotype, genotype and ultrastructural findings in a pedigree with autosomal recessive congenital ichthyosis
Xibao ZHANG ; Xuemei LI ; Yuqing HE ; Xin ZHOU ; Quan LUO ; Xin TIAN ; Ling LIN
Chinese Journal of Dermatology 2010;43(8):558-561
Objective To investigate the clinical phenotype, genotype and ultrastructural features in a pedigree with autosomal recessive congenital ichthyosis. Methods Patients were examined for clinical manifestation. PCR was carried out to amplify all the 15 exons and adjacent splice sites of TGM1 gene followed by bidirectional sequencing. Skin samples were taken by biopsy from the back of the proband, fixed in 3% glu-taraldehyde for transmission electron microscopy. Results The proband presented an intermediate clinical phenotype between lamellar ichthyosis (LI) and non-bullous congenital ichthyosiform erythroderma (NCIE), while his brother manifested as a collodion baby. A C551T heterozygous mutation which located in the third exon of TGM1 gene and resulted in a substitution of arginine by cysteine at codon 143 (R143C), was detected in the proband, his brother and father. Meanwhile, another heterozygous C-to-T transition at position 759 causing a substitution of serine by phenylalanine at codon 212 (S212F), was noted in the proband, his brother and mother. Electron microscopy revealed not only features of ichthyosis congenital type Ⅲ but also those of ichthyosis congenital type Ⅱ in lesions of the proband. Conclusions The patients in this pedigree carry compound heterozygous mutations, i.e. R143C, a hot missense mutation, as well as a de novo mutation S212F. The proband, who harbors mutations in the TGM1 gene, shows electron microscopic features characteristic not only of ichthyosis congenital type Ⅱ but also of ichthyosis congenital type Ⅲ.

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