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New Insights into Genotype-phenotype Correlations in Chinese Facioscapulohumeral Muscular Dystrophy： A Retrospective Analysis of 178 Patients

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Chinese Medical Journal 2015年第13期128卷 1707-1713页

作者： Feng Lin zhi-qiang wang Min-Ting Lin Shen-Xing Murong Ning wang Department of Neurology and Institute of Neurology First Affiliated Hospital Fujian Medical University Fuzhou Fujian 350005 China

Background： Facioscapulohumeral muscular dystrophy （FSHD）, a common autosomal dominant muscular disorder, is caused by contraction of the D4Z4 repeats on 4q35. The complicated genotype-phenotype correlation among different ethnic population remains a controversial subject. We aimed to refine this correlation in order to provide new information for genetic counseling. Methods： Here, a cohort of[36 Chinese families including 178 affected individuals and 137 unaffected members were investigated. Genetic analyses were pertbrmed using the pl 3E-11, 4qA and 4qB probes after pulsed field gel electrophoresis separation and southern blotting. A I 0-grade FSHD clinical severity scale was adopted for clinical assessment. The genotype-phenotype correlation was established by linear regression analyses. Results： We observed a roughly inversed correlation between the short EcoRl fragment size and age-corrected clinical severity score in 154 symptomatic patients （P 〈 0.05）. Compared to male patients, a significant higher proportion of females in both asymptomatic carriers and severe patients showed larger variation in the size of short EcoRI fragment. A high incidence （ 19/42, 45.2%） ofasymptomatic （or minimally affected） carriers was found in familial members. Conclusions： Although the number of D4Z4 repeats is known as one of the critical influences on genotype-phenotype correlation, a rnajority ofphenotypic spectrum was still incompatible with their heterozygous contraction of the D4Z4 repeat, especial in female cases. Our resuhs suggest that there are multi-factors synergistically modulating the phenotypic expression.

关键词： Asymptomatic Facio-scapulohumeral Muscular Dystrophy Genetic Counseling Phenotype

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Effects of 1-MCP on Post-Harvest Physiology of Tomato

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Agricultural Sciences in China 2003年第6期2卷 663-669页

作者： SUN Xi-sheng, wang zhi-hua, LI zhi-qiang, wang Wen-hui, and ZHANG zhi-yun(Research Institute of Pomology, Chinese Academy of Agricultural Sciences , Xingcheng 125100 , P. R. China) Research Institute of Pomology Chinese Academy of Agricultural Sciences Xingcheng 125100 P. R. China

Tomato(Lycopersicon esculentum cv. 402)was used to investigate the effects of 1-MCP(1-methylcyclopropene)treatment on ethylene production, respiration, vitamin C, chlorophyll, titratable acid, change of fruit color and rot. The results indicated that 1-MCP strongly inhibited ethylene production and respiration of tomato at very low concentration, delayed the incidence of climacteric peak of ethylene production and respiration. 1-MCP also delayed the decrease of vitamin C, chlorophyll and titratable acid during shelf life and storage significantly. Shelf and storage life of fruit treated with 1-MCP were extended at least 10 days at ambient(20 - 22℃)and 15 days at storage(9 - 11℃)temperature, respectively. Efficacy of 1-MCP on shelf and storage life was improved by increasing its concentration. After treatment of 1-MCP, rots were markedly lower than that of untreated fruit. 1-MCP treatment delayed the fruit color change.

关键词： Tomato 1-MCP Physiology Storage

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A Historical Cohort Study on the Efficacy of Glucocorticoids and Riboflavin Among Patients with Late-onset Multiple AcyI-CoA Dehydrogenase Deficiency

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Chinese Medical Journal 2016年第2期129卷 142-146页

作者： Xin-Yi Liu zhi-qiang wang Dan-Ni wang Min-Ting Lin Ning wang Department of Neurology and Institute of Neurology First Affiliated Hospital Fujian Medical University Fuzhou Fujian 350005 China Fujian Key Laboratory of Molecular Neurology Fuzhou Fujian 350005 China

Background： Late-onset multiple acyl-CoA dehydrogenase deficiency （MADD） is the most common type of lipid storage myopathies in China. Most patients with late-onset MADD are well responsive to riboflavin. Up to now, these patients are often treated with glucocorticoids as the first-line drug because they are misdiagnosed as polymyositis without muscle biopsy or gene analysis. Although glucocorticoids seem to improve the fatty acid metabolism of late-onset MADD, the objective evaluation of their rationalization on this disorder and comparison with riboflavin treatment are unknown. Methods： We performed a historical cohort study on the efficacy of the two drugs among 45 patients with late-onset MADD, who were divided into glucocorticoids group and riboflavin group. Detailed clinical information of baseline and 1-month follow-up were collected. Results： After 1-month treatment, a dramatic improvement of muscle strength was found in riboflavin group （P 〈 0.05）. There was no significant difference in muscle enzymes between the two groups. Significantly, the number of patients with full recovery in glucocorticoids group was less than the number in riboflavin group （P 〈 0.05）. On the other hand, almost half of the patients in riboflavin group still presented high-level muscle enzymes and weak muscle strength after 1-month riboflavin treatment, meaning that l-month treatment duration maybe insufficient and patients should keep on riboflavin supplement for a longer time. Conclusions： Our results provide credible evidences that the overall efficacy of riboflavin is superior to glucocorticoids, and a longer duration of riboflavin treatment is necessary for patients with late-onset MADD.

关键词： Glucocorticoids Historical Cohort Study Late-onset Multiple Acyl-CoA Dehydrogenase Deficiency Lipid StorageMyopathy Riboflavin

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Thymic lipofibroadenomas:Three case reports

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World Journal of Clinical Cases 2023年第1期11卷 164-171页

作者： Mai-Qing Yang zhi-qiang wang Li-Qian Chen Su-Mei Gao Xing-Ning Fu Hai-Ning Zhang Ke-Xin Zhang Hong-Tao Xu Department of Pathology Weifang People's Hospital(First Affiliated Hospital of Weifang Medical University)Weifang 261041Shandong ProvinceChina Department of Orthopedics and Trauma Weifang People's Hospital(First Affiliated Hospital of Weifang Medical University)Weifang 261041Shandong ProvinceChina Department of Pathology The First Hospital and College of Basic Medical Sciences of China Medical UniversityShenyang 110001Liaoning ProvinceChina

BACKGROUND Thymic lipofibroadenomas are extremely *** this study,we investigated the clinicopathological characteristics of thymic *** SUMMARY This study included three patients with thymic *** retrospectively analyzed the patient data to determine the clinicopathological characteristics of thymic *** study included one man and two women[mean age,43(33–59)years].All patients were non-smokers and presented with well-defined anterior mediastinal *** cut surfaces of the tumors were solid,with a mixture of yellow and white *** evaluation of resected specimens showed scattered cord-like structures of epithelial cells embedded within abundant fibrotic and hyaline stroma admixed with variable quantities of adipose *** patient showed hyperplastic thymic tissue in a part of the *** Thymic lipofibroadenomas are an extremely rare type of benign thymic *** removal of lipofibroadenomas is usually curative.

关键词： Lipofibroadenoma Thymoma Rare thymic tumor Clinicopathological characteristics Thoracic tumor Case report

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Intraductal ultrasonography and endoscopic retrograde cholangiography in diagnosis of extrahepatic bile duct stones: a comparative study

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Hepatobiliary & Pancreatic Diseases International 2004年第1期3卷 129-132页

作者： En-qiang Linghu, Liu-Fang Cheng, Xiang-Dong wang, zhi-qiang wang, Yun-Sheng Yang, Wen Li, Feng-Chun Cai, Hong-zhi wang, Hong Du and Jiang-Yun Meng Beijing, China Department of Digestive Medicine, 301 Hospital, Beijing 100853, China Department of Digestive Medicine 301 Hospital Beijing 100853 China. lh_ eq@***

BACKGROUND: Intraductal ultrasonography (IDUS) is highly accurate in detection of extrahepatic bile duct stones. This study was to compare the accuracy of IDUS and endo- scopic retrograde cholangiography (ERC) in the diagnosis of extrahepatic bile duct stones. METHODS: Thirty patients suspected of extrahepatic bile duct stones on B ultrasonography, CT, or MRI were en- rolled for study. ERC was performed using a Fujinon duo- denoscope (ED-410XT, ED-410Xu), then IDUS was done by inserting a Fujinon microprobe (PL2220-15) through the endoscopic biopsy channel to detect the extrahepatic bile duct. Finally stones in the extrahepatic bile duct were detected and extracted by endoscopic sphincterotomy (EST). RESULTS: Among the 30 patients, 26 were diagnosed as having cholelithiasis accurately through ERC. In one pa- tient the stone detected by ERC was really floccule. Mis- diagnosis happened in 2 patients with extrahepatic bile duct stones. So the overall accuracy and sensitivity of ERC in the diagnosis of extrahepatic bile duct stones were 86.7% (26/30) and 92.9% (26/28) respectively. In contrast, IDUS showed the results of diagnosis were in consistent with those of EST stone extraction. Its accuracy and sensi- tivity in the diagnosis of extrahepatic bile duct stones were 100% (30/30) and 100% (28/28) respectively. CONCLUSION: IDUS which is superior to ERC in diagno- sing extrahepatic bile duct stones can avoid the visual error of ERC.

关键词： endoscopic retrograde cholangiography intraductal ultrasonography extrahepatic bile duct stones

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Pathologic response after preoperative therapy predicts prognosis of Chinese colorectal cancer patients with liver metastases

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Chinese Journal of Cancer 2017年第11期36卷 537-547页

作者： Yun wang Yun-Fei Yuan Hao-Cheng Lin Bin-Kui Li Feng-Hua wang zhi-qiang wang Pei-Rong Ding Gong Chen Xiao-Jun Wu Zhen-Hai Lu zhi-Zhong Pan De-Sen Wan Peng Sun Shu-Mei Yan Rui-Hua Xu Yu-Hong Li Sun Yat-sen University Cancer Center State Key Laboratory of Oncology in South China Collaborative Innovation Center for Cancer Medicine Guangzhou 510060 Guangdong P. R. China Department of Medical Oncology Sun Yat-sen University Cancer Center Guangzhou 510060 Guangdong P. R. China Department of Hepatobiliary Surgery Sun Yat-sen University Cancer Center Guangzhou 510060 Guangdong P. R. China Department of Colorectal Surgery Sun Yat-sen University Cancer Center Guangzhou 510060 Guangdong P. R. China Department of Pathology Sun Yat-sen University Cancer Center Guangzhou 510060 Guangdong P. R. China

Background: Pathologic response is evaluated according to the extent of tumor regression and is used to estimate the efficacy of preoperative treatment. Several studies have reported the association between the pathologic response and clinical outcomes of colorecal cancer patients with liver metastases who underwent ***, to date, no data from Chinese patients have been reported. In this study, we aimed to evaluate the association between the pathologic response to pre-hepatectomy chemotherapy and prognosis in a cohort of Chinese *** and methods: In this retrospective study, we analyzed the data of 380 liver metastases in 159 *** pathologic response was evaluated according to the tumor regression grade(TRG).The prognostic role of pathologic response in recurrence-free survival(RFS) and overall survival(OS) was assessed using Kaplan-Meier curves with the log-rank test and multivariate Cox models. Factors that had potential influence on pathologic response were also analyzed using multivariate logistic regression and Kruskal-Wallis/Mann-Whitney U ***: Patients whose tumors achieved pathologic response after preoperative chemotherapy had significant longer RFS and OS than patients whose tumor had no pathologic response to chemotherapy(median RFS: 9.9 vs.6.5 months, P = 0.009; median OS: 40.7 vs. 28.1 months, P = 0.040). Multivariate logistic regression and Kruskal-Wallis/Mann-Whitney U tests showed that metastases with small diameter, metastases from the left-side primary tumors,and metastases from patients receiving long-duration chemotherapy had higher pathologic response rates than their control metastases(all P < 0.05). A decrease in the serum carcinoembryonic antigen(CEA) level after preoperative chemotherapy predicted an increased pathologic response rate(P < 0.05). Although the application of targeted therapy did not significantly influence TRG scores of all cases of metastases, the addition of cetuximab to chemoth

关键词： Colorectal cancer Liver metastases Chemotherapy Pathologic response

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Pancreas-sparing duodenectomy with regional lymph node dissection for early-stage ampullary carcinoma: A case control study using propensity scoring methods

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World Journal of Gastroenterology 2015年第18期21卷 5488-5495页

作者： Bin Liu Jing Li Yong-Jiu Zhang Lu-Nan Yan Sheng-Yi You Wan-Yee Lau Hao-Ran Sun Shi-Yan Yan zhi-qiang wang Department of Critical Care Medicine General Hospital of Tianjin Medical University Department of Anesthesiology General Hospital of Tianjin Medical University Department of General Surgery Urumqi General Hospital of Lanzhou Military Region PLA Division of Liver Transplantation West China Hospital Sichuan University Department of General Surgery General Hospital of Tianjin Medical University Faculty of Medicine The Chinese University of Hong Kong Prince of Wales Hospital Department of Radiology General Hospital of Tianjin Medical University Institute of Clinical Basic Medicine China Academy of Chinese Medical Sciences

AIM:To investigate the outcomes of pancreas-sparing duodenectomy(PSD)with regional lymph node dissection vs pancreaticoduodenectomy(PD).METHODS:Between August 2001 and June 2014,228 patients with early-stage ampullary carcinoma(Amp Ca)underwent surgical treatment(PD,n=159;PSD with regional lymph node dissection,n=69).The patients were divided into two groups:the PD group and the PSD *** scoring methods were used to select patients with similar disease statuses.A total of 138 matched cases,with 69 patients in each group,were included in the final ***:The median operative time was shorter among the patients in the PSD group(435 min)compared with those in the PD group(481 min,P=0.048).The median blood loss in the PSD group was significantly less than that in the PD *** median length of hospital stay was shorter for patients in the PSD group vs the PD *** incidence of pancreatic fistula was higher among patients in the PD group vs the PSD *** 1-,3-,and 5-year overall survival and disease-free survival rates for patients in the PSD group were 83%,70%,44%and 73%,61%,39%,respectively,and these values were not different than compared with those in the PD group(P=0.625).CONCLUSION:PSD with regional lymph node dissection presents an acceptable morbidity in addition to its advantages over *** may be a safe and feasible alternative to PD in the treatment of earlystage Amp Ca.

关键词： Ampullary carcinoma Early stage Surgical treatments Prognosis Propensity scoring methods

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Right-sided colon cancer and left-sided colorectal cancer respond differently to cetuximab

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癌症 2015年第9期34卷 384-393页

作者： Feng wang Long Bai Tian-Shu Liu Yi-Yi Yu Ming-Ming He Kai-Yan Liu Hui-Yan Luo Dong-Sheng Zhang Yin Jin Feng-Hua wang zhi-qiang wang De-Shen wang Miao-Zhen Qiu Chao Ren Yu-Hong Li Rui-Hua Xu Department of Medical Oncology Sun Yat-sen University Cancer Center:State Key Laboratory of Oncology in South China Department of Medical Oncology Zhongshan HospitalFudan University Collaborative Innovation Center for Cancer Medicine

Introduction: Right-sided colon cancer(RSCC) and left-sided colorectal cancer(LSCRC) differ with respect to their biology and genomic patterns. This study aimed to examine whether the primary tumor location is associated with the response to cetuximab in patients with metastatic colorectal cancer(m CRC).Methods: Patients with mC RC treated with cetuximab and standard chemotherapy as first- or second-line treatments were compared with randomly chosen patients who were treated with chemotherapy alone between 2005 and *** main outcome measures were the overal response rate(ORR), progression-free survival(PFS), and overal survival(OS).The differences in the outcome were analyzed by using the chi-squared test, Student’s t test, and Kaplan-Meier ***: The treatment results of 206 patients with m CRC treated with cetuximab and standard chemotherapy as first- or second-line treatments were compared with those of 210 patients who were treated with chemotherapy alone. As a first-line treatment, cetuximab with chemotherapy was associated with a significantly higher ORR(49.4 % vs. 28.6 %, P = 0.005) as well as longer PFS(9.1 vs. 6.2 months, P = 0.002) and OS(28.9 vs. 20.1 months,P = 0.036) than chemotherapy alone in patients with LSCRC. However, cetuximab neither improved the ORR(36.4 % vs. 26.2 %, P = 0.349) nor prolonged PFS(5.6 vs. 5.7 months, P = 0.904) or OS(25.1 vs. 19.8 months, P = 0.553) in patients with RSCC. As a second-line treatment, cetuximab exhibited a tendency to improve the ORR(23.5 % vs. 10.2 %,P = 0.087) and prolong PFS(4.9 vs. 3.5 months, P = 0.064), and it significantly prolonged OS(17.1 vs. 12.4 months,P = 0.047) compared with chemotherapy alone in the patients with LSCRC. In contrast, as a second-line treatment,cetuximab neither improved the ORR(7.1 % vs. 11.4 %, P = 0.698) nor prolonged PFS(3.3 vs. 4.2 months, P = 0.761) or OS(13.4 vs. 13.0 months, P = 0.652) in patients with ***: The addition of cetuximab to chemotherapy in b

关键词： Colorectal neoplasms Cetuximab Left-sided Right-sided Chemotherapy

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A phase II clinical trial of toripalimab in advanced solid tumors with polymerase epsilon/polymerase delta(POLE/POLD1)mutation

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Signal Transduction and Targeted Therapy 2024年第10期9卷 4543-4553页

作者： Ying Jin Run-Jie Huang Wen-Long Guan zhi-qiang wang Zong-Jiong Mai Yu-Hong Li Jian Xiao Xing Zhang Qi Zhao Shi-Fu Chen Ming Liu Yan-Xia Shi Feng wang Rui-Hua Xu Department of Medical Oncology Sun Yat-sen University Cancer CenterState Key Laboratory of Oncology in South ChinaGuangdong Provincial Clinical Research Center for CancerSun Yat-sen UniversityGuangzhou510060People’s Republic of China Research Unit of Precision Diagnosis and Treatment for Gastrointestinal Cancer Chinese Academy of Medical SciencesGuangzhou510060People’s Republic of China Department of Oncology The Fifth Affiliated Hospital of Sun Yat-sen UniversityZhuhai519000People’s Republic of China Department of Medical Oncology Guangdong Provincial People’s Hospital(Guangdong Academy of Medical Sciences)Southern Medical UniversityGuangzhou510100People’s Republic of China Department of Medical Melanoma and Sarcoma Sun Yat-sen University Cancer CenterState Key Laboratory of Oncology in South ChinaCollaborative Innovation Center for Cancer MedicineSun Yat-sen UniversityGuangzhou510060People’s Republic of China Bioinformatic Platform Sun Yat-sen University Cancer CenterState Key Laboratory of Oncology in South ChinaCollaborative Innovation Center for Cancer MedicineSun Yat-sen UniversityGuangzhou510060People’s Republic of China HaploX Biotechnology Shenzhen518000People’s Republic of China

Patients carrying mutations in polymerase epsilon/polymerase delta have shown positive responses to immune checkpoint ***,prospective trials exploring the efficacy in those with polymerase epsilon/polymerase delta mutations are still lacking.A phase II clinical trial was initiated to evaluate the efficacy of toripalimab,a humanized IgG4K monoclonal antibody to human PD-1,in patients with advanced solid tumors with unselected polymerase epsilon/polymerase delta mutations but without microsatellite instability-high.A total of 15 patients were enrolled,14 of whom were assessed for treatment *** was a 21.4%overall response rate,with a disease control rate of 57.1%.The median overall survival and median progression-free survival were 17.9(95%CI 13.5-not reach)months and 2.5(95%CI 1.4-not reach)months,*** patients with exonuclease domain mutations,the objective response rate was 66.7%(2/3),with a disease control rate of 66.7%(2/3).For those with non-exonuclease domain mutations,the rates were 9.1%(1/11)and 54.5%(6/11),***,patients with PBRM1 gene mutations exhibited a high response rate to toripalimab at 75.0%(3/4).This study showed that neither the exonuclease domain mutations nor non-exonuclease domain mutations could fully predict the efficacy of immunotherapy,urging the need for more investigations to clarify potential immune sensitization differences within polymerase epsilon/polymerase delta mutation variants.

关键词： delta phase neither

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A New Solar Radio Spectrometer at 1.10-2.06 GHz and First Observational Results

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Chinese Journal of Astronomy and Astrophysics 2005年第4期5卷 433-441页

作者： Hui-Rong Ji Qi-Jun Fu Yi-Hua Yan Yu-Ying Liu zhi-Jun Chen Cheng-Ming Tan Cong-Ling Cheng De-Bang Lao Shu-Ke Li zhi-qiang wang Min-Hong Yu Jian-Nong Liu Li-Kang Zhang Ji-Yong Gao National Astronomical Observatories Chinese Academy of Sciences Beijing 100012 Hebei Semiconductor Research Institute Shijiazhuang 050051

An improved Solar Radio Spectrometer working at 1.10-2.06 GHz with much improved spectral and temporal resolution, has been accomplished by the National Astronomical Observatories and Hebei Semiconductor Research Institute,based on an old spectrometer at 1 2 GHz. The new spectrometer has a spectral resolution of 4 MHz and a temporal resolution of 5 ms, with an instantaneous detectable range from 0.02 to 10 times of the quiet Sun flux. It can measure both left and right circular polarization with an accuracy of 10% in degree of polarization. Some results of preliminary observations that could not be recorded by the old spectrometer at 1-2 GHz are presented.

关键词： instrumentation: spectrometer - sun: radio radiation

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