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Redefining Budd-Chiari syndrome:A systematic review

World Journal of Hepatology 2016年第16期8卷 691-702页

作者： Naomi Shin Young H Kim Hao Xu Hai-Bin Shi Qing-Qiao Zhang jean paul colon pons Ducksoo Kim Yi Xu Fei-Yun Wu Samuel Han Byung-Boong Lee Lin-Sun Li Department of Radiology Univer-sity of Massachusetts Medical School Department of Interventional Radiology the Affiliated Hospital of Xuzhou Medical College Department of Radiology the First Affiliated Hospital of Nanjing Medical University Department of Radiology Boston University School of Medicine Department of Gastroenterology University of Massachusetts Medical School Department of Surgery George Washington University School of Medicine

AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same ***:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and ***:Of 1299 articles identified,26 were included in this *** BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and ***-BCS presents with chronic abdominal pain and abdominalwall *** neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management ***:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.

关键词： Budd-Chiari Hepatic vein outflow tract obstruction Membranous obstruction of the inferior vena cava Obliterative hepatocavopathy Hepatic vein thrombosis

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Pleomorphic leiomyosarcoma of the maxilla with metastasis to the colon:A case report

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World Journal of Gastrointestinal Endoscopy 2024年第6期16卷 361-367页

作者： Asma Alnajjar Abdulrahman Alfadda Abdullah Mohammad Alqaraawi Bader Alajlan jean paul Atallah Hussah Fahad AlHussaini Section of Gastroenterology Department of MedicineKing Faisal Specialist Hospital&Research CentreRiyadh 11211Saudi Arabia Department of Oncology King Faisal Specialist Hospital&Research CentreRiyadh 11211Saudi Arabia Department of Pathology King Faisal Specialist Hospital&Research CentreRiyadh 11211Saudi Arabia

BACKGROUND Pleomorphic leiomyosarcomas make up around 8.6%of all *** behave aggressively and often have poor *** can affect the gastrointestinal tract and *** date,pleomorphic leiomyosarcoma involving the mesocolon have been reported in nine *** SUMMARY The patient was a 44-year-old man with a history of pleomorphic leiomyosarcoma of the left maxilla with metastasis to the lung and *** most recent positron emission tomography-computed tomography(PET-CT)scan showed uptake in the ascending and transverse colons.A colonoscopy revealed a 5.0 cm×3.5 cm×3.0 cm pedunculated polyp in the ascending *** polyp was removed using hot snare polypectomy technique and retrieved with *** examination of the polyp showed a metastatic pleomorphic *** Uptake(s)on PET-CT in a patient with pleomorphic leiomyosarcoma should raise suspicion for metastasis.

关键词： Pleomorphic leiomyosarcoma colon polyp Hot snare polypectomy Maxillary Spindle cell tumor Case report

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Characterization of two rat models of cystic fibrosis—KO and F508del CFTR—Generated by Crispr-Cas9

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Animal Models and Experimental Medicine 2019年第4期2卷 297-311页

作者： Elise Dreano Marc Bacchetta Juliette Simonin Louise Galmiche Claire Usal Lotfi Slimani Jérémy Sadoine Laurent Tesson Ignacio Anegon jean-paul Concordet Aurélie Hatton Lucile Vignaud Danielle Tondelier Isabelle Sermet-Gaudelus Marc Chanson Charles-Henry Cottart INSERM 1151 INEMUniversitéde ParisParisFrance Département de Pédiatrie Gynécologie&Obstétrique et Département de Physiologie Cellulaire&MétabolismeUniversitéde GenèveGenèveSwitzerland Département de Pathologie APHPCHU Necker-Enfants MaladesParisFrance Centre de Recherche en Transplantation&Immunologie UMR 1064INSERMUniversitéde NantesNantesFrance Plateforme Trangénèse Rat&ImmunoPhénomique INSERM 1064&SFR François BonamyCNRS UMS3556NantesFrance Pathologie Imagerie&Biothérapies OrofacialesMontrougeFrance Plateforme Imageries du vivant Facultéde chirurgie dentaireUniversitéde ParisParisFrance INSERM U1154 CNRS UMR7196MNHNTACGENEParisFrance AP-HP Centre Maladie Rare Mucoviscidose et Maladies du CFTRAssistance Publique Hôpitaux de ParisHôpital Necker-Enfants MaladesParisFrance Facultéde Médecine de Paris Universitéde ParisParisFrance Facultéde Pharmacie de Paris Universitéde ParisParisFrance

Background: Genetically engineered animals are essential for gaining a proper understanding of the disease mechanisms of cystic fibrosis(CF). The rat is a relevant laboratory model for CF because of its zootechnical capacity, size, and airway characteristics, including the presence of submucosal ***: We describe the generation of a CF rat model(F508 del) homozygous for the ***508 del mutation in the transmembrane conductance regulator(Cftr) gene. This model was compared to new Cftr-/-rats(CFTR KO). Target organs in CF were examined by histological staining of tissue sections and tooth enamel was quantified by micro-computed tomography. The activity of CFTR was evaluated by nasal potential difference(NPD) and short-circuit current measurements. The effect of VX-809 and VX-770 was analyzed on nasal epithelial primary cell cultures from F508 del ***: Both newborn F508 del and Knock out(KO) animals developed intestinal obstruction that could be partly compensated by special diet combined with an osmotic laxative. The two rat models exhibited CF phenotypic anomalies such as vas deferens agenesis and tooth enamel defects. Histology of the intestine, pancreas, liver, and lungs was normal. Absence of CFTR function in KO rats was confirmed ex vivo by short-circuit current measurements on colon mucosae and in vivo by NPD, whereas residual CFTR activity was observed in F508 del rats. Exposure of F508 del CFTR nasal primary cultures to a combination of VX-809 and VX-770 improved CFTR-mediated ***: The F508 del rats reproduce the phenotypes observed in CFTR KO animals and represent a novel resource to advance the development of CF therapeutics.

关键词： animal models CFTR channel activity CFTR modulators cystic fibrosis primary cultures rat

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Identification of biomarkers of human pancreatic adenocarcinomas by expression profiling and validation with gene expression analysis in endoscopic ultrasound-guided fine needle aspiration samples

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World Journal of Gastroenterology 2006年第21期12卷 3344-3351页

作者： Henrik Laurell Michèle Bouisson Philippe Berthelémy Philippe Rochaix Sébastien Déjean Philippe Besse Christiana Susini Lucien Pradayrol Nicole Vaysse Louis Buscail INSERM U531 IFR31 Institut Louis BugnardBP 8422531432 Toulouse Cedex 4France Service de Gastroentérologie Centre Hospitaller de Pau PauFrance Laboratoire d'Anatomo-Pathologie Institut Claudius Regaud ToulouseFrance Laboratoire de Statistique et Probabilités Université paul Sabatier ToulouseFrance INSERM U531 IFR31 Institut Louis BugnardBP 8422531432 Toulouse Cedex 4France Service de Gastroenterologie et de la NutritionCHU RangueilToulouseFrance

AIM： To compare gene expression profiles of pancreatic adenocarcinoma tissue specimens, human pancreatic and colon adenocarcinoma and leukemia cell lines and normal pancreas samples in order to distinguish differentially expressed genes and to validate the differential expression of a subset of genes by quantitative real-time RT-PCR （RT-QPCR） in endoscopic ultrasound-guided fine needle aspiration （EUS-guided FNA） ***： Commercially dedicated cancer cDNA macroarrays （Atlas Human Cancer 1.2） containing 1176 genes were used. Different statistical approaches （hierarchical clustering, principal component analysis （PCA） and SAM） were used to analyze the expression data. RT-QPCR and immunohistochemical studies were used for validation of ***： RT-QPCR validated the increased expression of LCN2 （lipocalin 2） and for the first time PLAT （tissue-type plasminogen activator or tPA） in malignant pancreas as compared with normal pancreas. Immunohistochemical analysis confirmed the increased expression of LCN2 protein localized in epithelial cells of ducts invaded by carcinoma. The analysis of PLAT and LCN2 transcripts in 12 samples obtained through EUS-guided FNA from patients with pancreatic adenocarcinoma showed significantly increased expression levels in comparison with those found in normal tissues, indicating that a sufficient amount of high quality RNA can be obtained with this ***： Expression profiling is a useful method to identify biomarkers and potential target genes. Molecular analysis of EUS-guided FNA samples in pancreatic cancer appears as a valuable strategy for the diagnosis of pancreatic adenocarcinomas.

关键词： Pancreas colon Adenocarcinoma Gene expression profiling Endoscopic ultrasonography Ultrasound Fine needle aspiration

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Conversion of Unresectable to Resectable Liver Metastases from Colorectal Carcinoma Using Hepatic Arterial Chronomodulated Chemotherapy: A Case Report and Short Literature Review

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Surgical Science 2018年第10期9卷 358-366页

作者： Evgeny Y. Parnes Maria S. Sayapina Alexey A. Tryakin Mohamed Bouchahda Pasquale F. Innominato jean-Francois Morere Francis A. Levi Rene Adam The Central Clinical Hospital of Civil Aviation Moscow Russia N.N. Blokhin Russian Oncology Research Center Moscow Russia Department of Medical Oncology Assistance Publique H&ocirc pitaux de Paris H&ocirc pital paul Brousse Villejuif France INSERM UMRS 776 “Biological Rhythms and Cancers” Campus CNRS Villejuif France Cancer Chronotherapy Team Warwick Medical School Coventry United Kingdom Hepato-Biliary Center Assistance Publique H&ocirc pitaux de Paris H&ocirc pital paul Brousse Villejuif France

Background: The regional chronomodulated hepatic arterial infusion chemotherapy (HAIC) is an effective regimen for the treatment of patients with unresectable liver metastases from colorectal cancer, especially for the conversion into resectability. Aim: To demonstrate that chronomodulated HAI triplet chemotherapy according to OPTILIV protocol is well tolerated and displayed high antitumor activity in this heavily-pretreated patient. Case Presentation: A 54 years old patient from Russia was treated for a tumor in the ascending colon presented with 13 hepatic metastases ranging from 0.3 to 2.7 cm in diameter. He underwent a laparoscopic right hemicolectomy, 12 cycles of FOLFIRINOX combined to bevacizumab for the last 5 cycles, resulting in a partial response according to CT scan. It was decided to perform a two-stage hepatectomy at paul Brousse hospital: left partial hepatectomy allowed the excision of 9 lesions. Radio frequency ablation was performed in 2 nodular lesions. Afterwards, the patient received 5 cycles of chronomodulated triplet chemotherapy into the hepatic artery, according to the OPTILIV protocol design, yet without cetuximab, because of the KRAS mutation in the liver metastases, with a partial re-sponse. The patient could then undergo the second stage of the planned right hepatectomy, which turned out to be an R0 resection followed by receiving three courses of chronomodulated HAIC. Disease progression was documented after 3 months. Chronomodulated FOLFIRI chemotherapy was re-started intravenously, in combination with Aflibercept and it was associated with further disease progression. The genetic analysis of our patient’s cancer revealed a high level of MSI. The patient was included in the Phase 2 CheckMate-142 trial and received nivolumab 3 mg/kg every 2 weeks within 3 months. Treatment was discontinued due to ineffectiveness. Then the patient underwent radiotherapy geared towards reduction of pain. Afterwards, the patient died from the disease progr

关键词： The Regional Chronomodulated Hepatic Arterial Infusion Chemotherapy Metastatic Colorectal Cancer Conversion of Unresectable to Resectable Liver Metastases

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