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| 1 | Retrospective study on mixed neuroendocrine non-neuroendocrine neoplasms from five European centres显示文摘BACKGROUND Mixed neuroendocrine non-neuroendocrine neoplasm(MiNEN)is a rare diagnosis,mainly encountered in the gastro-entero-pancreatic tract.There is limited knowledge of its epidemiology,prognosis and biology,and the best management for affected patients is still to be defined.AIM To investigate clinical-pathological characteristics,treatment modalities and survival outcomes of a retrospective cohort of patients with a diagnosis of MiNEN.METHODS Consecutive patients with a histologically proven diagnosis of MiNEN were identified at 5 European centres.Patient data were retrospectively collected from medical records.Pathological samples were reviewed to ascertain compliance with the 2017 World Health Organisation definition of MiNEN.Tumour responses to systemic treatment were assessed according to the Response Evaluation Criteria in Solid Tumours 1.1.Kaplan-Meier analysis was applied to estimate survival outcomes.Associations between clinical-pathological characteristics and survival outcomes were explored using Log-rank test for equality of survivors functions(univariate)and Cox-regression analysis(multivariable).RESULTS Sixty-nine consecutive patients identified;Median age at diagnosis:64 years.Males:63.8%.Localised disease(curable):53.6%.Commonest sites of origin:colon-rectum(43.5%)and oesophagus/oesophagogastric junction(15.9%).The neuroendocrine component was;predominant in 58.6%,poorly differentiated in 86.3%,and large cell in 81.25%,of cases analysed.Most distant metastases analysed(73.4%)were occupied only by a poorly differentiated neuroendocrine component.Ninety-four percent of patients with localised disease underwent curative surgery;53%also received perioperative treatment,most often in line with protocols for adenocarcinomas from the same sites of origin.Chemotherapy was offered to most patients(68.1%)with advanced disease,and followed protocols for pure neuroendocrine carcinomas or adenocarcinomas in equal proportion.In localised cases,median recurrence free survival(RFS);14.0 months(95%CI:9.2-24.4),and median overall survival(OS):28.6 months(95%CI:18.3-41.1).On univariate analysis,receipt of perioperative treatment(vs surgery alone)did not improve RFS(P=0.375),or OS(P=0.240).In advanced cases,median progression free survival(PFS);5.6 months(95%CI:4.4-7.4),and median OS;9.0 months(95%CI:5.2-13.4).On univariate analysis,receipt of palliative active treatment(vs best supportive care)prolonged PFS and OS(both,P<0.001).CONCLUSION MiNEN is most commonly driven by a poorly differentiated neuroendocrine component,and has poor prognosis.Advances in its biological understanding are needed to identify effective treatments and improve patient outcomes. | Melissa Frizziero Xin Wang Bipasha Chakrabarty Alexa Childs Tu V Luong Thomas Walter Mohid S Khan Meleri Morgan Adam Christian Mona Elshafie Tahir Shah Annamaria Minicozzi Wasat Mansoor Tim Meyer Angela Lamarca Richard A Hubner Juan W Valle Mairéad G McNamara | 2019 | World Journal of Gastroenterology2019，25，39： | 13 |
| 2 | Epistaxis in end stage liver disease masquerading as severe upper gastrointestinal hemorrhage显示文摘AIM:To describe the prevalence,diagnosis,treatment,and outcomes of end stage liver disease(ESLD) patients with severe epistaxis thought to be severe upper gastrointestinal hemorrhage(UGIH).METHODS:This observational single center study included all consecutive patients with ESLD and epistaxis identified from consecutive subjects hospitalized with suspected UGIH and prospectively enrolled in our databases of severe UGIH between 1998 and 2011.RESULTS:A total of 1249 patients were registered for severe UGIH in the data basis,461(36.9%) were cirrhotics. Epistaxis rather than UGIH was the bleeding source in 20 patients. All patients had severe coagulopathy. Epistaxis was initially controlled in all cases. Fifteen(75%) subjects required posterior nasal packing and 2(10%) embolization in addition to correction of coagulopathy. Five(25%) patients died in the hospital,12(60%) received orthotopic liver transplantation(OLT),and 3(15%) were discharged without OLT. The mortality rate was 63% in patients without OLT.CONCLUSION:Severe epistaxis in patients with ESLD is(1) a diagnosis of exclusion that requires upper endoscopy to exclude severe UGIH;and(2) associated with a high mortality rate in patients not receiving OLT. | Marine Camus Dennis M Jensen Jason D Matthews Gordon V Ohning Thomas O Kovacs Rome Jutabha Kevin A Ghassemi Gustavo A Machicado Gareth S Dulai | 2014 | World Journal of Gastroenterology2014，20，38： | 3 |
| 3 | Genotype phenotype correlation in Wilson's disease within families-a report on four south Indian families显示文摘AIM: To study the genotype phenotype correlation in Wilson's disease (WD) patients within families. METHODS: We report four unrelated families from South India with nine members affected with WD. Phenotype was classified as per international consensus phenotypic classifi cation of WD. DNA was extracted from peripheral blood and 21 exons of ATP7B gene and flanking introns were amplified by polymerase chain reaction (PCR). The PCR products were screened for mutations and the aberrant products noted on screening were sequenced. RESULTS: Four separate ATP7B mutations were found in the four families. ATP7B mutations were identical amongst affected members within each family. Three families had homozygous mutations of ATP7B gene while one family had compound heterozygous mutation, of which only one mutation was identifi ed. We noted concordance between ATP7B gene mutation and Wilson's disease phenotype amongst members within each family. The age of onset of symptoms or of detection of asymptomatic disease, baseline serum ceruloplasmin and baseline urinary copper levels were also similar in affected members of each family. Minor differences in phenotype and baseline serum ceruloplasmin level were noted in one family.CONCLUSION: We report concordance between ATP7B mutation and WD phenotype within each family with > 1 member affected with WD. Homozygous ATP7B mutation was present in 3 of the 4 families studied. Our report supports allelic dominance as a determinant of WD phenotype. However, in one family with compound heterozygous mutation, there was a similar WD phenotype which suggests that there may be other factors determining the phenotype. | S Santhosh RV Shaji CE Eapen V Jayanthi S Malathi P Finny N Thomas M Chandy G Kurian GM Chandy | 2008 | World Journal of Gastroenterology2008，14，29： | 3 |
| 4 | Empagliflozin alleviates podocytopathy and enhances glomerular nephrin expression in db/db diabetic mice显示文摘BACKGROUND Modern guidelines recommend sodium-glucose cotransporter-2(SGLT2)inhibitors as the preferred antihyperglycemic agents for patients with type 2 diabetes and chronic kidney disease.However,the mechanisms underlying the renal protective effect of SGLT2 inhibitors are not fully understood.structure of podocytes and nephrin expression in glomeruli in db/db diabetic mice.METHODS We treated 8-wk-old male db/db mice with EMPA(10 mg/kg/d)or vehicle for 8 wk.Age-matched male db/+mice were included as non-diabetic controls.Parameters of body composition,glycemic and lipid control,and plasma concentrations of leptin,insulin and glucagon were assessed.We evaluated renal hypertrophy as kidney weight adjusted to lean mass,renal function as plasma levels of creatinine,and albuminuria as the urinary albumin-to-creatinine ratio(UACR).Renal structures were studied by light and transmission electron microscopy with a focus on mesangial volume and podocyte structure,respectively.Glomerular nephrin and transforming growth factor beta(TGF-β)were assessed by immunohistochemistry.RESULTS Severe obesity and hyperglycemia developed in db/db mice prior to the start of the experiment;increased plasma concentrations of fructosamine,glycated albumin,cholesterol,leptin,and insulin,and elevated UACR were detected.Mesangial expansion,glomerular basement membrane thickening,and increased area of TGF-βstaining in glomeruli were revealed in vehicle-treated mice.Podocytopathy was manifested by effacement of foot processes;nephrin-positive areas in glomeruli were reduced.EMPA decreased the levels of glucose,fructosamine and glycated albumin,UACR,kidney hypertrophy,mesangial expansion,glomerular basement membrane thickening,and glomerular TGF-βstaining,alleviated podocytopathy and restored glomerular staining of nephrin.CONCLUSION These data indicate that EMPA attenuates podocytopathy in experimental diabetic kidney disease.The anti-albuminuric effect of EMPA could be attributed to mitigation of podocyte injury and enhancement of nephrin expression. | Vadim V Klimontov Anton I Korbut Iuliia S Taskaeva Nataliya P Bgatova Maksim V Dashkin Nikolai BOrlov Anna S Khotskina Evgenii L Zavyalov Thomas Klein | 2020 | World Journal of Diabetes2020，11，12： | 2 |
| 5 | Modern synthetic methods for copper-mediated C(aql)-O,C(aryl)-N,and C(aryl)-S bond formation显示文摘 | LEY S V THOMAS A W | 2003 | Angew Chem Int Ed2003，42，： | 1 |
| 6 | The role of SO2 as an additive to organic Li-ion battery electrolytes显示文摘 | Ein-Eli Y Thomas S R Koch V R | 1997 | J Electrochem Soc1997，144，4： | 1 |
| 7 | Improving the nutritive value of full-fat rice bran for broiler chickens using a lipase-basedenzyme preparation显示文摘 | Tan S H Thomas D V Cumden B Je t al | 2000 | Asian Australasian Journal of Animal Sciences2000，13，3： | 1 |
| 8 | Gold and trace element zonation in pyrite using a laser imaging technique: Implications for the timing of gold in orogenic and Carlin- style sediment- hosted deposits 显示文摘 | R R Large L Danyushevsky C Hollit V Maslennikov S Meffer S Gilbert S Bull R Scott P Emsbo H Thomas B Singh J Foster | 2009 | Economic Geology2009，104，5： | 1 |
| 9 | Delay effect in models of population growth显示文摘 | Dang V G Yongwimon L Thomas I S | 2005 | J Math Appl2005，305，： | 1 |
| 10 | 显示文摘 | THOMAS M M MAURENE K V ERIC S C | 1997 | Cytometry1997，29，： | 1 |
| 11 | Age-associated defect in human TLR-1/2 function显示文摘 | van Duin D Mohanty S Thomas V | 2007 | J Immunol2007，178，2： | 1 |
| 12 | Bit error simulation for/4 DQPSK mobile radio communications using TwoRay and measurement-based impulse response models显示文摘 | Fung V Rappaport T S Thoma B | | 0，，03： | 1 |
| 13 | Central nervous system tuberculosis显示文摘 | Cherian A Thomas S V | 2011 | Afr Health Sci2011，11，1： | 1 |
| 14 | Measurement,analysis,and display of haptic signals during surgical cutting 显示文摘 | Stephanie G V H Vanessa C A O Thomas S | 2002 | Presence2002，11，6： | 1 |
| 15 | Self-incompatibility triggers programmed cell death in Papaver pollen显示文摘 | THOMAS S FRANKLIN-TONG V | 2004 | Nature2004，429，： | 1 |
| 16 | Effects of simultane- ous ozone exposme and nitrogen loads on carbohydrate concentrations, biomass and growth of young spruce trees(Picea abies) 显示文摘 | THOMAS V F D BRAUN S FLOCKIGER W | 2005 | Environ- mental Pollution2005，137，3： | 1 |
| 17 | Biocatalysis: applications and potentials for the chemical industry 显示文摘 | THOMAS S M DICOSIMO R NAGARAJAN V | 2002 | Trends in Biotechnology2002，20，6： | 1 |
| 18 | The environmental fate andeffects of antifouling paint biocides显示文摘 | Thomas K V Brooks S | 2010 | Biofouling2010，26，1： | 1 |
| 19 | Ganesh, Modelling and Assessment of Critical Risks in BOT Road Projects 显示文摘 | Thomas A V Satyanarayana N Kalidindi L S | 2006 | Construction Management and Economics2006，24，4： | 1 |
| 20 | Isolated myeloid sarcoma presenting in all four eyelids 显示文摘 | Thomas S A Durairaj V D | 2007 | Ophthal Plast Reconstr Surg2007，23，4： | 1 |
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