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High-risk screening combined with family screening for Fabry disease in adult hemodialysis population-a family report of GLA IVS4+919G>A mutation in Fabry disease

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China medical Abstracts(Internal medicine) 2024年第2期41卷 113-113页

作者：陈冰蓉 Dept Nephrol Children's HospZhejiang Univ Med SchNatl Clin Child Health Res CenterHangzhou 310052

Objective To explore the combination of high risk screening and family screening for potential patients with Fabry disease in adult hemodialysis population,and to improve the diagnostic efficiency of the *** It was a cross-sectional investigation ***-risk screening for Fabry disease was performed on adult hemodialysis patients with end-stage kidney disease who were admitted to Yongkang First People's Hospital of Zhejiang Province between November 2022 and February *** blood paperα-galactosidase A(α-Gal A)detection assay was performedin males,or glycosphingolipids(Lyso-CL-3)detection assay was performed in *** genetic assay was performed for further diagnosis after abnormal screening *** screening was carried out on the family members of the confirmed Fabry disease patients,andα-Gal A activity and Lyso-GL-3 of peripheral blood were ***,urine routine,blood biochemistry,eye examination,hearing test,cranial magnetic resonance imaging,and electrocardiogram were performed to assess organ *** Among 244 hemodialysis patients,139(56.97%)were males and 105(43.03%)were females.

关键词： admitted GLA routine

Progress in investigating the pathogenesis of hepatopulmonary syndrome

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Hepatobiliary & Pancreatic Diseases International 2010年第4期9卷 355-360页

作者： Zhang, Zhao-Jie Yang, Chang-Qing Tongji Univ Sch Med Tongji Hosp Div Gastroenterol Shanghai 200065 Peoples R China Tongji Univ Sch Med Tongji Hosp Inst Digest Dis Shanghai 200065 Peoples R China

BACKGROUND: The pathogenesis of hepatopulmonary syndrome is complicated and remains unknown. This review aims to provide an updated knowledge about the pathogenesis of the syndrome. DATA SOURCES: Five medical databases, medLINE, Science-Direct, OVID, Springer Link, and Wiley Inter Science were searched for articles on 'hepatopulmonary syndrome', 'cirrhosis', 'angiogenesis', 'intestinal endotoxemia', 'nitric oxide', 'carbon monoxide', and other related subjects. RESULTS: Currently, imbalance between vasodilation and vasoconstriction, intestinal bacterial translocation, intestinal endotoxemia, and activation of the lung monocyte/macrophage system may play important roles in the pathogenesis of hepatopulmonary syndrome. Recent studies found that angiogenesis is also an important factor in the pathogenesis of experimental hepatopulmonary syndrome. CONCLUSION: Angiogenesis inhibition may be a potential approach for the treatment of hepatopulmonary syndrome in the future.

关键词： hepatopulmonary syndrome cirrhosis pathogenesis angiogenesis intestinal endotoxemia nitric oxide carbon monoxide

Effect of tacrolimus in idiopathic membranous nephropathy： a meta-analysis

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Chinese medical Journal 2014年第14期127卷 2693-2699页

作者： Santosh, Thapa Liu Hong Liu Bicheng Southeast Univ Sch Med Zhongda Hosp Inst Nephrol Nanjing 210009 Jiangsu Peoples R China

Background The efficacy and safety of immunosuppression for idiopathic membranous nephropathy （IMN） are still controversial. Recent studies showed tacrolimus is effective in the treatment of IMN. To evaluate the efficacy and safety of tacrolimus （TAC） for IMN, we conducted a meta-analysis of published medical literatures. Methods Studies addressing the effect of tacrolimus in IMN were searched on PUBmed, EMBASE, The Cochrane Library, and *** （March 2013）. Trials comparing tacrolimus with corticosteroid versus control group （cyclophosphamide with corticosteroid） were included. The quality of the studies was assessed using Jadad method. Statistical analyses were performed using Review Manager 5.2 and the results were summarized by calculating the risk ratio （RR） for dichotomous data or the mean difference （MD） for continuous data with 95% confident interval （CI）. Results A total of four studies （259 patients） were included. It was shown that therapy with tacrolimus plus corticosteroid had a higher complete remission rate compared to therapy with cyclophosplamide plus corticosteroid （RR=1.53, 95% CI： 1.05-2.24, P 〈0.05）, but not significant on total remission, partial remission and adverse effects. Also, no significant alterations were observed in proteinuria and serum albumin level between the two groups. During the entire follow-up period, serum creatinine level remained stable in both groups without ±50% increase in its level. Conclusions TAC is more effective than cyclophosphamide （CTX） by achieving complete remission in patients with IMN. Multi-ethnic RCTs are needed to evaluate its long-term efficacy and safety. Chin med J 2014;127 （14）： 2693-2699

关键词： tacrolimus idiopathic membranous nephropathy meta-analysis

Clinical characteristics of non-perianal fistulating Crohn＇s disease in China： a single-center experience of 184 cases

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Chinese medical Journal 2012年第14期125卷 2405-2410页

作者： WANG Ge-fei, Nanjing Univ Sch Med Dept Surg Jinling Hosp Nanjing 210002 Jiangsu Peoples R China

Background There is little information of non-perianal fistulating Crohn＇s disease in the consensus published by the European Crohn＇s and Colitis Organization in 2006 and 2010. This study was designed to demonstrate the clinical characteristics of non-perianal fistulating Crohn＇s disease among homogenous Chinese population. Methods One-hundred-and-eighty-four patients were retrospectively collected. All of these patients were diagnosed of Crohn＇s disease between February 2001 and April 2011. Results The male-to-female ratio was 2.7：1. The most common symptoms at onset were abdominal pain （88.0%）, diarrhea （34.7%）, and fever （28.3%）. The most common disease location and behavior at diagnosis were small bowel （56.0%） and penetrating （51.6%）. Among 324 non-perianal fistulae, the most common types were ileocolonic anastomotic （30.9%）, terminal ileocutaneous （19.7%）, and enteroenteric anastomotic （11.4%）. One-hundred-and-thirty- eight （75.0%） patients received antibiotics, and I^-Iactam （85.5%） and metronidazole （67.4%） are most frequently used. One-hundred-and-seventy-eight （96.7%） patients suffered 514 surgical operations, and the cumulative surgical rates after 1, 3, and 5 years were 38.0%, 52.2%, and 58.7% respectively. Nine patients died during the follow-up period, and the cumulative survival rates after 1, 3, and 5 years were 97.8%, 96.7%, and 96.2% respectively. Conclusions This study displayed the clinical characteristics of non-perianal fistulating Crohn＇s disease in our center. Large population-based studies are required for further investigation in China.

关键词： Crohn 'S disease, non-perianalfistulae inflammatory bowel disease, clinicalfeature,follow-up

Relative performance of two equations for estimation of glomerular filtration rate in a Chinese population having chronic kidney disease

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Chinese medical Journal 2012年第4期125卷 599-603页

作者： Li Jiang-tao Xun Chen Cui Chun-li Wang Hui-fang Wu Yi-tai Yun Ai-hong Jiang Xiao-feng Ma Jun Tongji Univ Tongji Hosp Dept Nephrol Sch Med Shanghai 200065 Peoples R China

Background The new Chronic Kidney Disease Epidemiology Collaboration （CKD-EPI） equation was developed to address the systematic underestimation of glomerular filtration rate （GFR） by the Modification of Diet in Renal Disease （MDRD） Study equation in patients with relatively well-preserved kidney *** of the new equation in the Chinese population is *** goal of the present study was to compare performance of these two equations in Chinese patients with chronic kidney disease （CKD）.Methods We enrolled 450 Chinese patients （239 women and 211 men） with CKD in the present *** renal dynamic imaging method was used to measure the referenced standard GFR （rGFR） for comparison with estimations using the two *** overall performance was assessed with the Bland-Altman method and receiver-operating characteristics （ROC） *** of the two equations in lower and higher estimated GFR （eGFR） subgroups was further *** Both eGFRs correlated well with rGFR （r=0.88,0.81,P〈0.05）.In overall performance,the CKD-EPI equation showed less bias,higher precision and improved accuracy,and was better for detecting *** the higher-eGFR subgroup,the CKD-EPI equation corrected the underestimation of GFR by the abbreviated MDRD *** The CKD-EPI equation outperformed the abbreviated MDRD equation not only in overall performance but also in the subgroups *** the present,the CKD-EPI equation appears to be the first-choice prediction equation for estimating GFR.

关键词： chronic kidney disease glomerular filtration rate abbreviated modification of diet in renal disease equation Chronic Kidney Disease Epidemiology Collaboration equation

STRUCTURE OF 1-EPICRASSICAUDINE

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Chinese Chemical Letters 1995年第2期6卷 109-110页

作者： Wang, FP Dai, XP Wang, JZ Zhang, R W China Univ med Sci Sch Pharm Dept Chem Med Nat Prod Chengdu 610044 Peoples R China

A new C-19-diterpenoid alkaloid. 1-epicrassicaudine, was isolated from the roots of Aconitum hemslevanum Pritz var. pengshinese. Spectral methods demonstrated that it has the structure represented by (1) an 1-epimer o...

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Correlation of urinary uric acid excretion with obesity and abdominal obesity in newly diagnosed patients with type 2 diabetes mellitus

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China medical Abstracts(Internal medicine) 2024年第1期41卷 29-29页

作者：王羽洁 Dept Endocrinol&Metab Shanghai 6th People's HospShanghai Jiaotong Univ Med SchShanghai 200233

Objective To investigate the association of uric acid excretion (UUAE) with obesity and abdominal obesity in newly diagnosed patients with type 2 diabetes mellitus(T2DM).Methods A total of 1 175 newly diagnosed T2DM patients hospitalized in the Department of Endocrinology and Metabolism of Shanghai Sixth People’s Hospital Affiliated to Shanghai Jiaotong University school of medicine were enrolled in this study from January 2006to December *** to the quartile of UUAE.

关键词： obesity T2DM patients

STUDIES ON THE TOTAL SYNTHESIS OF CIMICIFUGAMIDEFROM CIMICIFUGA DAHURICA

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Chinese Chemical Letters 1996年第6期7卷 545-548页

作者： Ding, PY Zhu, XX Cai, MS Yu, DQ BEIJING med UNIV SCH PHARMACEUT SCIDEPT ORGAN CHEMBEIJING 100083PEOPLES R CHINA CHINESE ACAD med SCI INST MAT MEDBEIJING 100050PEOPLES R CHINA

Cimicifugamide, a new natural compound isolated from the roots of Cimicifuga dahurica, was totally synthesized by a reaction sequence of seven steps in an overall yield of 31 %. D-Galactose was used as starting material, trifluoroacetoxy was used as leaving group at C-1. The target product was characterized by IR, MS, H-1-NMR C-13-NMR and elemental analysis.

关键词： TOTAL STUDIES ON THE TOTAL SYNTHESIS OF CIMICIFUGAMIDEFROM CIMICIFUGA DAHURICA

Efficacy and significance of various scores for pneumonia severity in the management of patients with community-acquired pneumonia in China

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Chinese medical Journal 2012年第4期125卷 639-645页

作者： Yang Yan Xu Feng Shi Li-yun Diao Ran Cheng Yu-sheng Chen Xi-yuan Jing Ji-yong Wang Xuan-ding Shen Hua-hao Zhejiang Univ Affiliated Hosp 2 Dept Resp Med Sch Med Hangzhou 310009 Zhejiang Peoples R China Zhejiang Univ Affiliated Hosp 2 Dept Intens Care Unit Sch Med Hangzhou 310009 Zhejiang Peoples R China Zhejiang Univ Affiliated Hosp 2 Dept Infect Prevent & Control Sch Med Hangzhou 310009 Zhejiang Peoples R China Hangzhou Normal Univ Div Immunol Hangzhou 310036 Zhejiang Peoples R China Zhejiang Univ Affiliated Hosp 2 Dept Internal Med Binjiang BranchSch Med Hangzhou 310009 Zhejiang Peoples R China Hangzhou Binjiang Hosp Hangzhou 310009 Zhejiang Peoples R China

Background Community-acquired pneumonia （CAP） remains one of the leading causes ot death from infectious diseases around the *** severe CAP patients are admitted to the intensive care unit （ICU）,and receive intense *** present study aimed to evaluate the role of the pneumonia severity index （PSI）,CURB-65,and sepsis score in the management of hospitalized CAP patients and explore the effect of ICU treatment on prognosis of severe *** A total of 675 CAP patients hospitalized in the Second Affiliated Hospital of Zhejiang University school of medicine were retrospectively *** ability of different pneumonia severity scores to predict mortality was compared for effectiveness,while the risk factors associated with 30-day mortality rates and hospital length of stay （LOS） were *** effect of ICU treatment on the outcomes of severe CAP patients was also *** All three scoring systems revealed that the mortality associated with the low-risk or intermediate-risk group was significantly lower than with the high-risk *** the risk level increased,the frequency of ICU admission rose in tandem and LOS in the hospital was *** areas under the receiver operating characteristic curve in the prediction of mortality were 0.94,0.91 and 0.89 for the PSI,CURB-65 and sepsis score,*** with the corresponding control groups,the mortality was markedly increased in patients with a history of smoking,prior admission to ICU,respiratory failure,or co-morbidity of heart *** differences were also identified in LOS between control groups and patients with ICU treatment,heart,or cerebrovascular *** regression analysis showed that age over 65 years,a history of smoking,and respiratory failure were closely related to mortality in the overall CAP cohort,whereas age,ICU admission,respiratory failure,and LOS at home between disease attack and hospital admission were identified as independent

关键词： community-acquired pneumonia pneumonia severity pneumonia severity index CURB-65 score sepsis score intensive care unit treatment