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Malignant peripheral nerve sheath tumor with hemophilic syndrome and bone marrow fibrosis:A rare case report

World Journal of Clinical Cases 2023年第31期11卷 7673-7679页

作者： Hui Li Li Wang Ying-Hong Wu Gang Chen Hong-Xia Li Li-fen Fan Min gu Cai-Hong Jiang Department of Medical Oncology Ordos Central HospitalOrdos 017000Inner Mongolia Autonomous RegionChina Department of Pathology Ordos Central HospitalOrdos 017000Inner Mongolia Autonomous RegionChina Department of Neurology Baogang HospitalBaotou 014010Inner Mongolia Autonomous Region 014010China Department of Pharmacy Ordos Central HospitalOrdos 017000Inner Mongolia Autonomous RegionChina Department of Orthopedics Ordos Central HospitalOrdos 017000Inner Mongolia Autonomous RegionChina

BACKGROUND Malignant schwannoma is a rare tumor in the peripheral nervous system,accounting for approximately 5%to 10%of systemic soft tissue ***,malignant schwannoma occurring in the broad ligament of the uterus with hemophilic syndrome and bone marrow fibrosis is extremely rare in clinical ***,we report the first case of an patient diagnosed with malignant peripheral nerve sheath tumor(MPNST)of the broad ligament of the uterus with hemophilic syndrome and bone marrow fibrosis,and share our reference clinical diagnosis and treatment *** SUMMARY A patient was diagnosed with MPNST of the uterus harboring hemophilic syndrome and bone marrow *** received combination,and repeated imaging revealed further encountered rare complications(hemophilia syndrome and bone marrow fibrosis)after two cycles of ***,combined treatment with pazopanib,gemcitabine,and dacarbazine was ***,the patient succumbed to death at hospital after two *** This report firstly provided reference clinical practice for a patient with MPNST of the uterus harboring hemophilic syndrome and bone marrow *** case raises a reminder about the tolerance and safety of combination therapy,especially in young women.

关键词： Malignant peripheral nerve sheath tumor Hemophilic syndrome Bone marrow fibrosis Case report

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Rapid assembly of 1,3-indanedione-based spirocyclic tetrahydroquinolines for inducing human lung cancer cell apoptosis

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Green Synthesis and Catalysis 2022年第4期3卷 357-372页

作者： fen Tan Luojia Chen Yuncong Yuan Xiaoyu He Yiwen Su Shuqiang Cao Conghua Xie Meijia gu Youquan Zou Department of Radiation and Medical Oncology Zhongnan Hospital of Wuhan UniversitySchool of Pharmaceutical SciencesWuhan UniversityWuhan 430071China Hubei Key Laboratory of Purification and Application of Plant Anti-cancer Active Ingredients Hubei University of EducationWuhan 430205China Key Laboratory of Combinatorial Biosynthesis and Drug Discovery Ministry of EducationWuhan UniversityWuhan 430071China Hubei Key Laboratory of Tumor Biological Behaviors Hubei Cancer Clinical Study CenterZhongnan Hospital of Wuhan UniversityWuhan 430071China TaiKang Center for Life and Medical Sciences Wuhan UniversityWuhan 430071China

The rapid collection of a library of biologically relevant molecules is essential in new drug *** we show a variety of bioactive 1,3-indanedione-based spirocyclic tetrahydroquinolines that have been concisely and efficiently constructed under the guidance of biology-oriented synthesis(BIOS).Using Pd(PPh_(3))_(4) as a catalyst,various 2-arylidene-1,3-indanediones can react with vinyl benzoxazinanones under the mild condition to afford a new class of spirocyclic tetrahydroquinolines in moderate to excellent yields(45%-99%)with high stereocontrol(>95:5 diastereoselectivity).Their structures were unambiguously confirmed by X-ray single crystal *** biological evaluation revealed that two of the 1,3-indanedione-based spirocyclic tetrahydroquinolines show remarkable apoptosis-inducing activities over A549 human lung cancer cells,exceeding that of *** antitur activities were triggered by reactive oxygen species,commonly overproduced in cancer *** clinical demand for antitumor chemotherapy for lung cancer has encouraged an all-out search for analogues with lower toxcity,improved therapeutic index and increased activity.

关键词： Spirocyclic tetrahydroquinoline 1,3-Indanedione Lung cancer Apoptosis Mitochondria

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C-reactive protein and diabetic retinopathy in Chinese patients with type 2 diabetes mellitus

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International Journal of Ophthalmology(English edition) 2016年第1期9卷 111-118页

作者： Xiu-fen Yang Yu Deng Hong gu Apiradee Lim Torkel Snellingen Xi-Pu Liu Ning-Li Wang Amitha Domalpally Ronald Danis Ning-Pu Liu Department of Ophthalmology the Friendship HospitalCapital Medical University Beijing 100050 China Beijing Tongren Eye Center Beijing Ophthalmology and Visual Sciences Key Laboratory Beijing Tongren HospitalCapital Medical University Beijing 100730 China Department of Mathematics and Computer Science Faculty of Science and Technology Prince of Songkla UniversityPattani Campus Muang Pattani 9400 Thailand Sekwa Research Institute Beijing 100088 China Fundus Photograph Reading Center Department of Ophthalmology and Visual Sciences University of Wisconsin Madison 53705 Wisconsin USA

AIM：To investigate the relationship between Creactive protein（CRP）and diabetic retinopathy（DR）in a cohort of Chinese patients with type 2 diabetes mellitus（T2DM）.·METHODS：Community-based observational cohort *** were 1131 participants recruited from November 2009 to September 2011 in Desheng community in urban *** diagnosed T2DM were recruited and underwent a standardized evaluation consisting of a questionnaire,ocular and anthropometric examinations and laboratory *** presence and severity of DR were assessed by seven fields 30°color fundus *** were then classified into groups with no DR,any DR,or vision-threatening *** was analyzed from serum of study subjects.·RESULTS：A total of 1007 patients with T2DM were included for analysis,including 408（40.5%）men and 599（59.5%）*** median CRP level was 1.5 mg/L for women and 1.1 mg/L for men（=0.004,OR 0.37,95%CI0.18-0.74）.After adjusting for possible covariates,higher levels of CRP were associated with lower prevalence of any DR（=0.02,OR 0.55,95%CI 0.35-0.89）,but not associated with vision-threatening DR（=0.62,OR 0.78,95%CI 0.28-2.14）.After stratification by sex,the inverse association between CRP and DR was found to be statistically significant in men（=0.006,OR 0.35,95%CI0.16-0.73）,but not in women（=0.58,OR 0.88,95%CI0.29-1.16）.·CONCLUSION：The data drawn from a Chinese population with T2DM suggest that increasing CRP levels may be inversely associated with development of DR.

关键词： type 2 diabetes mellitus C-reactive protein diabetic retinopathy inflammation

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Nanomaterials-mediated lysosomal regulation:a robust protein-clearance approach for the treatment of Alzheimer’s disease

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Neural Regeneration Research 2025年第2期20卷 424-439页

作者： Mengqi Hao Jianjian Chu Tinglin Zhang Tong Yin Yuankai gu Wendanqi Liang Wenbo Ji Jianhua Zhuang Yan Liu Jie Gao You Yin Department of Neurology Second Affiliated Hospital of Naval Medical University(Shanghai Changzheng Hospital)ShanghaiChina School of Health Science and Engineering University of Shanghai for Science and TechnologyShanghaiChina Changhai Clinical Research Unit Shanghai Changhai HospitalNaval Medical UniversityShanghaiChina Department of Clinical Pharmacy Xinhua HospitalShanghai Jiao Tong University School of MedicineShanghaiChina Department of Neurology Shanghai East HospitalSchool of MedicineTongji UniversityShanghaiChina

Alzheimer’s disease is a debilitating,progressive neurodegenerative disorder characterized by the progressive accumulation of abnormal proteins,including amyloid plaques and intracellular tau tangles,primarily within the ***,crucial intracellular organelles responsible for protein degradation,play a key role in maintaining cellular *** studies have suggested a link between the dysregulation of the lysosomal system and pathogenesis of neurodegenerative diseases,including Alzheimer’s *** the normal physiological function of lysosomes hold the potential to reduce the pathological burden and improve the symptoms of Alzheimer’s ***,the efficacy of drugs in treating Alzheimer’s disease is limited,with major challenges in drug delivery efficiency and ***,nanomaterials have gained widespread use in Alzheimer’s disease drug research owing to their favorable physical and chemical *** review aims to provide a comprehensive overview of recent advances in using nanomaterials(polymeric nanomaterials,nanoemulsions,and carbon-based nanomaterials)to enhance lysosomal function in treating Alzheimer’s *** review also explores new concepts and potential therapeutic strategies for Alzheimer’s disease through the integration of nanomaterials and modulation of lysosomal *** conclusion,this review emphasizes the potential of nanomaterials in modulating lysosomal function to improve the pathological features of Alzheimer’s *** application of nanotechnology to the development of Alzheimer’s disease drugs brings new ideas and approaches for future treatment of this disease.

关键词： Alzheimer’s disease autophagy dysfunction lysosomal acidification lysosomal system nanomaterials neurodegenerative diseases

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Causes of severe neonatal hyperbilirubinemia: a multicenter study of three regions in China

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World Journal of Pediatrics 2021年第3期17卷 290-297页

作者： Xiao-Yue Dong Qiu-fen Wei Zhan-Kui Li Jie gu Dan-Hua Meng Jin-Zhen guo Xiao-Li He Xiao-Fan Sun Zhang-Bin Yu Shu-Ping Han Department of Neonatology Women's Hospital of Nanjing Medical University(Nanjing Maternity and Child Health Care Hospital)NanjingChina Department of Neonatology Guangxi Maternal and Child Health Care HospitalNanningChina Department of Neonatology Northwest Women’s and Children’s HospitalXi’anChina Department of Neonatology Yinchuan Maternal and Child Health Care HospitalYinchuanChina

Background Available evidence suggests that our country bear great burden of severe ***,the causes have not been explored recently in different regions of China to guide necessary clinical and public health *** This was a prospective,observational study conducted from March 1,2018,to February 28,*** hospitals in three regions of China participated in the *** from infants with a gestational age≥35 weeks,birth weight>2000 g,and total serum bilirubin(TSB)level≥17 mg/dL(342μmol/L)were prospectively *** A total of 783 cases were *** were identified in 259 *** major causes were ABO incompatibility(n=101),glucose-6-phosphate dehydrogenase deficiency(n=76),and intracranial hemorrhage(n=70).All infants with glucose-6-phosphate dehydrogenase deficiency were from the central south *** from the central south region had much higher peak total bilirubin levels[mean,404μmol/L;standard deviation(SD),75μmol/L]than those from the other regions(mean,373μmol/L;SD,35μmol/L)(P<0.001).Conclusions ABO incompatibility was the leading cause in the east and northwest regions,but cases in the central south region were mainly caused by both ABO incompatibility and glucose-6-phosphate dehydrogenase deficiency,and infants in this region had a much higher peak total bilirubin *** hemorrhage may be another common *** thorough assessments and rigorous bilirubin follow-up strategies are needed in the central south region.

关键词： Causes Hyperbilirubinemia Jaundice Neonate

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The role of autophagic and lysosomal pathways in ischemic brain injury

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Neural Regeneration Research 2013年第23期8卷 2117-2125页

作者： Zhaohua gu Yinyi Sun Kangyong Liu fen Wang Ting Zhang Qiang Li Liwei Shen Ling Zhou Liang Dong Nan Shi Qian Zhang Wei Zhang Meizhen Zhao Xiaojiang Sun Department of Neurology Shanghai Pudong New Area Zhoupu Hospital Department of Neurology the Sixth People's Hospital of Shanghai Shanghai Jiao Tong University Department of Neurobiology Suzhou University School of Medicine Department of Neurology the Fifth People's Hospital of Shanghai Fudan University

Autophagy is involved in neural cell death after cerebral ischemia. Our previous studies showed that rapamycin-induced autophagy decreased the rate of apoptosis, but the rate of apoptosis was in- creased after the autophagy inhibitor, 3-methyladenine, was used. In this study, a suture-occluded method was performed to generate a rat model of brain ischemia. Under a transmission electron microscope, autophagic bodies and autophagy lysosomes were markedly accumulated in neurons at 4 hours post brain ischemic injury, with their numbers gradually reducing over time. Western blotting demonstrated that protein levels of light chain 3-11 and cathepsin B were significantly in- creased within 4 hours of ischemic injury, but these levels were not persistently upregulated over time. Confocal microscopy showed that autophagy was mainly found in neurons with positive light chain 3 signal. Injection of rapamycin via tail vein promoted the occurrence of autophagy in rat brain tissue after cerebral ischemia and elevated light chain 3 and cathepsin B expression. However, in- jection of 3-methyladenine significantly diminished light chain 3-11 and cathepsin B expression. Results verified that autophagic and lysosomal activity is increased in ischemic neurons. Abnormal components in cells can be eliminated through upregulating cell autophagy or inhibiting autophagy after ischemic brain injury, resulting in a dynamic balance of substances in cells. Moreover, drugs that interfere with autophagy may be potential therapies for the treatment of brain injury.

关键词： neural regeneration brain injury autophagy lysosome light chain 3 cathepsin B cerebral ischemia neuron neuroprotection grants-supported paper neuroregeneration

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Tetrandrine citrate eliminates imatinib-resistant chronic myeloid leukemia cells in vitro and in vivo by inhibiting Bcr-Abl/β-catenin axis

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Journal of Zhejiang University-Science B(Biomedicine & Biotechnology) 2012年第11期13卷 867-874页

作者： Xiao-hua XU Yi-chao GAN Gen-bo XU Ting CHEN Hong ZHOU Jin-fen TANG Ying gu Fei XU Ying-ying XIE Xiao-ying ZHAO Rong-zhen XU Department of Hematology the Second Affiliated Hospital School of Medicine Zhejiang University Hangzhou 310009 China Department of Cancer Institute the Second Affiliated Hospital School of Medicine Zhejiang University Hangzhou 310009 China

Objective:To evaluate the effects of tetrandrine citrate, a novel tetrandrine salt with high water solubility, on the growth of imatinib (IM)-resistant chronic myeloid leukemia (CML) in vitro and in vivo, and reveal action molecular mechanisms. Methods:Cell viability in vitro was measured using methyl thiazolyl tetrazolium (MTT) assay. CML cell growth in vivo was assessed using a xenograft model in nude mice. Bcr-Abl and β-catenin protein levels were determined using Western blotting. Bcr-Abl messenger RNA (mRNA) was measured by reverse transcription polymerase chain reaction (RT-PCR). Flow cytometry (FCM) was used to determine cell cycle status. Results:Tetrandrine citrate inhibited the growth of IM-resistant K562 cells, primary leukemia cells, and primitive CD34 + leukemia cells, and their inhibition concentration that inhibited 50% of target cells (IC 50 ) ranged from 1.20 to 2.97 μg/ml. In contrast, tetrandrine citrate did not affect normal blood cells under the same conditions, and IC 50 values were about 10.12-13.11 μg/ml. Oral administration of tetrandrine citrate caused complete regression of IM-resistant K562 xeno-grafts in nude mice without overt toxicity. Western blot results revealed that treatment of IM-resistant K562 cells with tetrandrine citrate resulted in a significant decrease of both p210 Bcr-Abl and β-catenin proteins, but IM did not affect the Bcr-Abl protein levels. Proteasome inhibitor, MG132, did not prevent tetrandrine-mediated decrease of the p210 Bcr-Abl protein. RT-PCR results showed that tetrandrine treatment caused a decrease of Bcr-Abl mRNA. FCM analysis indicated that tetrandrine induced gap 1 (G 1 ) arrest in CML cells. Conclusions:Tetrandrine citrate is a novel orally active tetrandrine salt with potent anti-tumor activity against IM-resistant K562 cells and CML cells. Tetrandrine citrate-induced growth inhibition of leukemia cells may be involved in the depletion of p210 Bcr-Abl mRNA and β-catenin protein.

关键词： Chronic myeloid leukemia Imatinib-resistance Tetrandrine citrate Bcr-Abl protein β-catenin protein

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Effect and safety of sorafenib in patients with intermediate hepatocellular carcinoma who received transarterial chemoembolization: A retrospective comparative study

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World Journal of Clinical Cases 2018年第5期6卷 74-83页

作者： Xue-fen Lei Yang Ke Tian-Hao Bao Hao-Ran Tang Xue-Song Wu Zhi-Tian Shi Jie Lin Zhi-Xian Zhang Hou gu Lin Wang Department of Medical Oncology The Second Affiliated Hospital of Kunming Medical University Department of Hepatobiliary Surgery The Second Affiliated Hospital of Kunming Medical University The Mental Health Center of Kunming Medical University Department of Hepatobiliary Surgery The Second Affiliated Hospital of Kunming Medical University Department of Gastro- enterological Surgery The Second Affiliated Hospital of Kunming Medical University

AIM To evaluate the safety and efficacy of sorafenib plus transarterial chemoembolization(TACE) treatment for intermediate hepatocellular carcinoma(HCC).METHODS Sixty-seven patients with intermediate-stage [Barcelona Clinic liver cancer stage B(BCLC-B)] HCC who were treated with sorafenib plus TACE or TACE alone between 2009 and 2011 were included in the study. Follow-up was until 2014 or patient death. Two groups were defined in the experiment: The experimental group, treated with sorafenib plus TACE, and the control group, treated with standard TACE *** The Kaplan-Meier survival analysis showed that the median overall survival(m OS) of the experimental group was 35.2 mo, while that of the control group was 22.0 mo(P fenib plus TACE showed higher incidence rates of rash, hand-foot syndrome(HFS), and hypertension(P fenib plus TACE treatment for BCLC-B HCC significantly prolonged the m OS of patients compared to TACE treatment alone. The most common toxicities with sorafenib were rash(31.6%), HFS(39.5%) and hypertension(31.6%), but there were no intolerable adverse events. The Cox multivariate analysis showed that the survival of patients with BCLC-B HCC depended on the Child-Pugh classification, tumor diameter, and treatment with sorafenib plus TACE compared to TACE alone.

关键词： Sorafenib Hepatocellular carcinoma Transarterial chemoembolization Overall survival Adverse reaction

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QDPR gene mutation and clinical follow-up in Chinese patients with dihydropteridine reductase deficiency

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World Journal of Pediatrics 2014年第3期10卷 219-226页

作者： De-Yun Lu Jun Ye Lian-Shu Han Wen-Juan Qiu Hui-Wen Zhang Jian-De Zhou Pei-Zhong Bao Ya-fen Zhang Xue-Fan gu Department of Pediatric Endocrinology and Genetic Metabolism and Shanghai Institute for Pediatric Research Xinhua HospitalShanghai Jiaotong University School of MedicineShanghai 200092China

Background:This study aimed to investigate the mutation spectrum of the QDPR gene,to determine the effect of mutations on dihydropteridine reductase(DHPR)structure/function,to discuss the potential genotype-phenotype correlation,and to evaluate the clinical outcome of Chinese patients after ***:Nine DHPR-deficient patients were enrolled in this study and seven of them underwent neonatal *** gene mutations were analyzed and confi rmed by routine *** potential pathogenicity of missense variants was analyzed using Clustal X,PolyPhen program and Swiss-PDB Viewer 4.04_OSX software,*** clinical outcomes of the patients were evaluated after long-term ***:In 10 mutations of the 9 patients,4 were novel mutations(G20V,V86D,G130S and A175R),4 were reported by us previously,and 2 known mutations were identified.R221X was a hotspot mutation(27.7%)in our *** missense mutations probably had damage to *** patients in this series were treated with a good control of phenylalanine *** height and weight of the patients were normal at the age of 4 months to 7.5 *** patients,who underwent a neonatal screening and were treated early,showed a normal mental *** 2 patients diagnosed late,neurological symptoms were signifi cantly ***:The mutation spectrum of the QDPR gene is different in the Chinese *** mutations are related to severe *** determination of DHPR activity should be performed in patients with ***-defi cient patients who were treated below the age of 2 months may have a near normal mental development.

关键词： dihydropteridine reductase hyperphenylalaninaemia tetrahydrobiopterin

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Activity of HDV ribozymes to trans-cleave HCV RNA

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World Journal of Gastroenterology 2002年第4期8卷 694-698页

作者： Yue-Cheng Yu Qing Mao Chang-Hai gu Qi-fen Li Yu-Ming Wang Institute of Infectious Diseases of Chinese PLA,Southwest Hospital,Third Military Medical University,Chongqing 400038,China Institute of Infectious Diseases of Chinese PLA Southwest Hospital Third Military Medical University Chongqing 400038China. yuechengyu212001@***

AIM: To explore whether HDV ribozymes have the abilityto trans-cleave ***: Three HDV genomic ribozymes weredesigned and named RzC1, RzC2 and RzC3. Thesubstrate RNA contained HCVRNA 5'-noncoding regionand 5'-fragment of C region (5'-NCR-C). All theribozymes and HCV RNA 5'-NCR-C were obtained bytranscription in vibo from their DNA templates, and HCVRNA 5'-NCR-C was radiolabelled at its 5'-end Undercertain pH, temperature, appropriate concentration ofMg2+ and deionized formamide, these ribozymes wererespectively or simultaneously mixed with HCVRNA 5'-NCR-C and reacted for a certain time. The trans-cleavage reaction was stopped at different time points,and the products were separated with polyacrylamidegel electrophoresis (PAGE), displayed byautoradiography. Percentage of trans-deaved productswas measured to indicate the activity of HDV ***: RzC1 and RzC2 could trans-cleave 26 % and21.8 % of HCV RNA 5'-NCR-C under our reactionconditions with 2.5 mol. L-1 deionized formamiderespectively. The percentage of HCV RNA 5'-NCR-Ctrans-cleaved by RzC1, RzC2 or combined usage of thethree ribozymes increased with time, up to 24.9 %, 20.3 %and 37.3 % respectively at 90 min point. Almost noproduct from RzC3 was ***: HDV ribozymes are able to trans-cleavespecifically HCV RNA at certain sites under appropriateconditions, and combination of several ribozymesaiming at different target sites can trans-cleave thesubstrate more efficiently than using only one of them.

关键词：丁型肝炎病毒核酸重组丙型肝炎病毒 RNA活性

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