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| 1 | Primary intestinal lymphangiectasia in an adult patient:A case report and review of literature显示文摘BACKGROUND Primary intestinal lymphangiectasia(PIL),first described in 1961,is a rare disorder of unknown etiology resulting in protein-losing enteropathy.The disease is characterized by dilatation and leakage of intestinal lymph vessels leading to hypoalbuminemia,hypogammaglobulinemia,and lymphopenia.Since the severity and location of lymph vessels being affected can vary considerably,the range of associated symptoms is wide from mild lower-limb edema to generalized edema,abdominal and/or pleural effusion,and recurrent diarrhea,among others.Although usually developing in early childhood,we present the case of a 34-yearold woman with PIL.Moreover,we performed a literature review systematically assessing clinical presentation,and provide a practical approach to facilitate diagnosis and therapy of PIL in adults.CASE SUMMARY Our patient presented with unspecific symptoms of abdominal discomfort,fatigue,nausea,and recurrent edema of the lower limbs.Interestingly,a striking collinearity of clinical symptoms with female hormone status was evident.Additionally,polyglobulia,hypoalbuminemia,hypogammaglobulinemia,and transient lymphocytopenia were evident.Due to suspicion of a bone marrow disease,an extensive diagnostic investigation was carried out excluding secondary causes of polyglobulinemia and hypoalbuminemia.The diagnosis of primary intestinal lymphangiectasia was established after 22 wk by histological analysis of biopsy samples obtained via enteroscopy.Consecutively,the patient was put on a high-protein and low-fat diet with medium-chain triglycerides supplementation leading to significant improvement of clinical symptoms until 2 years of follow-up.CONCLUSION PIL can be the reason for cryptogenic hypoalbuminemia,hypogammaglobulinemia,and lymphopenia in adulthood.Due to difficulty in correct diagnosis,treatment initiation is often delayed despite being effective and welltolerated.This leads to a significant disease burden in affected patients.PIL is increasingly been recognized in adults since the majority of case reports were published within the last 10 years,pointing towards an underestimation of the true prevalence.The association with female hormone status warrants further investigation. | Rudolf Huber Georg Semmler Alexander Mayr Felix Offner Christian Datz | 2020 | World Journal of Gastroenterology2020，26，48： | 6 |
| 2 | Differential role of Hedgehog signaling in human pancreatic(patho-)physiology:An up to date review显示文摘Since the discovery of the Hedgehog(Hh)pathway in drosophila melanogaster,our knowledge of the role of Hh in embryonic development,inflammation,and cancerogenesis in humans has dramatically increased over the last decades.This is the case especially concerning the pancreas,however,real therapeutic breakthroughs are missing until now.In general,Hh signaling is essential for pancreatic organogenesis,development,and tissue maturation.In the case of acute pancreatitis,Hh has a protective role,whereas in chronic pancreatitis,Hh interacts with pancreatic stellate cells,leading to destructive parenchym fibrosis and atrophy,as well as to irregular tissue remodeling with potency of initiating cancerogenesis.In vitro and in situ analysis of Hh in pancreatic cancer revealed that the Hh pathway participates in the development of pancreatic precursor lesions and ductal adenocarcinoma including critical interactions with the tumor microenvironment.The application of specific inhibitors of components of the Hh pathway is currently subject of ongoing clinical trials(phases 1 and 2).Furthermore,a combination of Hh pathway inhibitors and established chemotherapeutic drugs could also represent a promising therapeutic approach.In this review,we give a structured survey of the role of the Hh pathway in pancreatic development,pancreatitis,pancreatic carcinogenesis and pancreatic cancer as well as an overview of current clinical trials concerning Hh pathway inhibitors and pancreas cancer. | Eckhard Klieser Stefan Swierczynski Christian Mayr Tarkan Jager Johanna Schmidt Daniel Neureiter Tobias Kiesslich Romana Illig | 2016 | World Journal of Gastrointestinal Pathophysiology2016，7，2： | 5 |
| 3 | Role of interleukin-1 and its antagonism of hepatic stellate cell proliferation and liver fibrosis in the Abcb4^(-/-) mouse model显示文摘AIM: To study the interleukin-1(IL-1) pathway as a therapeutic target for liver fibrosis in vitro and in vivo using the ATP-binding cassette transporter b4^(-/-)(Abcb4^(-/-)) mouse model.METHODS: Female and male Abcb4^(-/-) mice from 6 to 13 mo of age were analysed for the degree of cholestasis(liver serum tests), extent of liver fibrosis(hydroxyproline content and Sirius red staining) and tissue-specific activation of signalling pathways such as the IL-1 pathway [quantitative polymerase chain reaction(q PCR)]. For in vivo experiments, murine hepatic stellate cells(HSCs) were isolated via pronasecollagenase perfusion followed by density gradient centrifugation using female mice. Murine HSCs were stimulated with up to 1 ng/m L IL-1β with or without 2.5 μg/m L Anakinra, an IL-1 receptor antagonist, respectively. The proliferation of murine HSCs was assessed via the Brd U assay. The toxicity of Anakinra was evaluated via the fluorescein diacetate hydrolysis(FDH) assay. In vivo 8-wk-old Abcb4^(-/-) mice with an already fully established hepatic phenotype were treated with Anakinra(1 mg/kg body-weight daily intraperitoneally) or vehicle and liver injury and liver fibrosis were evaluated via serum tests, q PCR, hydroxyproline content and Sirius red staining. RESULTS: Liver fibrosis was less pronounced in males than in female Abcb4^(-/-) animals as defined by a lower hydroxyproline content(274 ± 64 μg/g vs 436 ± 80 μg/g liver, respectively; n = 13-15; P < 0.001; MannWhitney U-test) and lower m RNA expression of the profibrogenic tissue inhibitor of metalloproteinase-1(TIMP)(1 ± 0.41 vs 0.66 ± 0.33 fold, respectively; n = 13-15; P < 0.05; Mann-Whitney U-test). Reduced liver fibrosis was associated with significantly lower levels of F4/80 m RNA expression(1 ± 0.28 vs 0.71 ± 0.41 fold, respectively; n = 12-15; P < 0.05; Mann-Whitney U-test) and significantly lower IL-1β m RNA expression levels(1 ± 0.38 vs 0.44 ± 0.26 fold, respectively; n = 13-15; P < 0.001; Mann-Whitney U-test). No gender differences in the serum liver parameters [bilirubin; alanine aminotransferase(ALT); aspartate aminotransferase and alkaline phosphatase(AP)] were found. In vitro, the administration of IL-1β resulted in a significant increase in HSC proliferation [0.94 ± 0.72 arbitrary units(A.U.) in untreated controls, 1.12 ± 0.80 A.U. at an IL-1β concentration of 0.1 ng/m L and 1.18 ± 0.73 A.U. at an IL-1β concentration of 1 ng/m L in samples from n = 6 donor animals; P < 0.001; analyses of variance(ANOVA)]. Proliferation was reduced significantly by the addition of 2.5 μg/m L Anakinra(0.81 ± 0.60 A.U. in untreated controls, 0.92 ± 0.68 A.U. at an IL-1β concentration of 0.1 ng/m L, and 0.91 ± 0.69 A.U. at an IL-1β concentration of 1 ng/m L; in samples from n = 6 donor animals; P < 0.001; ANOVA) suggesting an anti-proliferative effect of this clinically approved IL-1 receptor antagonist. The FDH assay showed this dose to be non-toxic in HSCs. In vivo, Anakinra had no effect on the hepatic hydroxyprolinecontent, liver serum tests(ALT and AP) and profibrotic(collagen 1α1, collagen 1α2, transforming growth factor-β, and TIMP-1) and anti-fibrotic [matrix metalloproteinase 2(MMP2), MMP9 and MMP13 ] gene expression after 4 wk of treatment. Furthermore, the hepatic IL-1β and F4/80 m RNA expression levels were unaffected by Anakinra treatment.CONCLUSION: IL-1β expression is associated with the degree of liver fibrosis in Abcb4^(-/-) mice and promotes HSC proliferation. IL-1 antagonism shows antifibrotic effects in vitro but not in Abcb4^(-/-) mice. | Florian P Reiter Ralf Wimmer Lena Wottke Renate Artmann Jutta M Nagel Manuel O Carranza Doris Mayr Christian Rust Peter Fickert Michael Trauner Alexander L Gerbes Simon Hohenester Gerald U Denk | 2016 | World Journal of Hepatology2016，8，8： | 3 |
| 4 | Organic Light‐Emitting Diodes with 30% External Quantum Efficiency Based on a Horizontally Oriented Emitter显示文摘 | Sei‐Yong Kim Won‐Ik Jeong Christian Mayr Young‐Seo Park Kwon‐Hyeon Kim Jeong‐Hwan Lee Chang‐Ki Moon Wolfgang Brütting Jang‐Joo Kim | 2013 | Adv Funct Mater2013，，31： | 1 |
| 5 | Functional Electrical Stimulation (FES) of denervated muscles: existing and prospective technological solutions显示文摘 | Winfried Mayr Christian Hofer Manfred Bijak | 2002 | Basic Appl Myol2002，，12： | 1 |
| 6 | 1,25-(OH) 2 -vitamin D 3 prevents activation of hepatic stellate cells in vitro and ameliorates inflammatory liver damage but not fibrosis in the Abcb4 ?/? model显示文摘 | Florian P. Reiter Simon Hohenester Jutta M. Nagel Ralf Wimmer Renate Artmann Lena Wottke Marie-Christine Makeschin Doris Mayr Christian Rust Michael Trauner Gerald U. Denk | 2015 | Biochemical and Biophysical Research Communicatio2015，，2： | 1 |
| 7 | The role of polycomb repressive complexes in biliary tract cancer显示文摘 | Christian Mayr Daniel Neureiter Andrej Wagner Martin Pichler Tobias Kiesslich | 2015 | Expert Opinion on Therapeutic Targets2015，，： | 1 |
| 8 | Cytotoxic effects of chemokine receptor 4 inhibition by AMD3100 in biliarytract cancer cells: Potential drug synergism with gemcitabine显示文摘 | Christian Mayr Daniel Neureiter Martin Pichler Frieder Berr Andrej Wagner Tobias Kiesslich Konrad Namberger | 2015 | Molecular Medicine Reports2015，，： | 1 |
| 9 | Chronic Infections and the Risk of Carotid Atherosclerosis: Prospective Results From a Large Population Study显示文摘 | Stefan Kiechl Georg Egger Manuel Mayr Christian J. Wiedermann Enzo Bonora Friedrich Oberhollenzer Michele Muggeo Qingbo Xu Georg Wick Werner Poewe Johann Willeit | 2001 | Circulation: Journal of the American Heart Association2001，，8： | 1 |
| 10 | Comparing two different arginine vasopressin doses in advanced vasodilatory shock: a randomized, controlled, open-label trial显示文摘 | Christian Torgersen Martin W. Dünser Volker Wenzel Stefan Jochberger Viktoria Mayr Christian A. Schmittinger Ingo Lorenz Stefan Schmid Martin Westphal Wilhelm Grander Günter Luckner | 2010 | Intensive Care Medicine2010，，1： | 1 |
| 11 | Role of histone deacetylases in pancreas: Implications for pathogenesis and therapy显示文摘In the last years, our knowledge of the pathogenesis in acute and chronic pancreatitis(AP/CP) as well as in pancreatic cancerogenesis has significantly diversified. Nevertheless, the medicinal therapeutic options are still limited and therapeutic success and patient outcome are poor. Epigenetic deregulation of gene expression is known to contribute to development and progression of AP and CP as well as of pancreatic cancer. Therefore, the selective inhibition of aberrantly active epigenetic regulators can be an effective option for future thera-pies. Histone deacetylases(HDACs) are enzymes that remove an acetyl group from histone tails, thereby causing chromatin compaction and repression of transcri-ption. In this review we present an overview of the currently available literature addressing the role of HDACs in the pancreas and in pancreatic diseases. In pancreatic cancerogenesis, HDACs play a role in the important process of epithelial-mesenchymal-transition, ubiquitin-proteasome pathway and, hypoxia-inducible-factor-1-angiogenesis. Finally, we focus on HDACs as potential therapeutic targets by summarizing currently available histone deacetylase inhibitors. | Eckhard Klieser Stefan Swierczynski Christian Mayr Johanna Schmidt Daniel Neureiter Tobias Kiesslich Romana Illig | 2015 | World Journal of Gastrointestinal Oncology2015，7，12： | 1 |
| 12 | Biliary tract cancer stem cells-translational options and challenges显示文摘Management of biliary tract cancer remains challenging. Tumors show high recurrence rates and therapeutic resistance, leading to dismal prognosis and short survival. The cancer stem cell model states that a tumor is a heterogeneous conglomerate of cells, in which a certain subpopulation of cells-the cancer stem cells-possesses stem cell properties. Cancer stem cells have high clinical relevance due to their potential contributions to development, progression and aggressiveness as well as recurrence and metastasis of malignant tumors. Consequently, reliable identification of as well as pharmacological intervention with cancer stem cells is an intensively investigated and promising research field. The involvement of cancer stem cells in biliary tract cancer is likely as a number of studies demonstrated their existence and the obvious clinical relevance of several established cancer stem cell markers in biliary tract cancer models and tissues. In the present article, we review and discuss the currently available literature addressing the role of putative cancer stem cells in biliary tract cancer as well as the connection between known contributors of biliary tract tumorigenesis such as oncogenic signaling pathways, micro-RNAs and the tumor microenvironment with cancer stem cells. | Christian Mayr Matthias Ocker Markus Ritter Martin Pichler Daniel Neureiter Tobias Kiesslich | 2017 | World Journal of Gastroenterology2017，23，14： | 0 |
| 13 | Toward flexible visual analytics augmented through smooth display transitions显示文摘Visualizing big and complex multivariate data is challenging.To address this challenge,we propose flexible visual analytics(FVA)with the aim to mitigate visual complexity and interaction complexity challenges in visual analytics,while maintaining the strengths of multiple perspectives on the studied data.At the heart of our proposed approach are transitions that fluidly transform data between userrelevant views to offer various perspectives and insights into the data.While smooth display transitions have been already proposed,there has not yet been an interdisciplinary discussion to systematically conceptualize and formalize these ideas.As a call to further action,we argue that future research is necessary to develop a conceptual framework for flexible visual analytics.We discuss preliminary ideas for prioritizing multi-aspect visual representations and multi-aspect transitions between them,and consider the display user for whom such depictions are produced and made available for visual analytics.With this contribution we aim to further facilitate visual analytics on complex data sets for varying data exploration tasks and purposes based on different user characteristics and data use contexts. | Christian Tominski Gennady Andrienko Natalia Andrienko Susanne Bleisch Sara Irina Fabrikant Eva Mayr Silvia Miksch Margit Pohl AndréSkupin | 2021 | Visual Informatics2021，5，3： | 0 |
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