|
|
|
题名
|
作者
|
年代
|
出处
|
被引量
|
| 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 | 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 |
| 3 | Calcium Regulates Key Components of Vascular Smooth Muscle Cell–Derived Matrix Vesicles to Enhance Mineralization显示文摘 | Alexander N. Kapustin John D. Davies Joanne L. Reynolds Rosamund McNair Gregory T. Jones Anissa Sidibe Leon J. Schurgers Jeremy N. Skepper Diane Proudfoot Manuel Mayr Catherine M. Shanahan | 2011 | Circulation Research2011，，1： | 2 |
| 4 | Comparison of CT Angiography With MR Angiography in the Preoperative Assessment of Living Kidney Donors显示文摘 | Thomas M. Gluecker Michael Mayr Jochen Schwarz Deniz Bilecen Thomas Voegele Juerg Steiger Alexander Bachmann Georg Bongartz | 2008 | Transplantation2008，，9： | 1 |
| 5 | Long-term follow-up after ovarian borderline tumor: Relapse and survival in a large patient cohort显示文摘 | Miriam S. Lenhard Stefanie Mitterer Carolin Kümper Petra Stieber Doris Mayr Nina Ditsch Klaus Friese Alexander Burges | 2009 | European Journal of Obstetrics and Gynecology2009，，2： | 1 |
| 6 | Neuropilin-1 Expression on Regulatory T Cells Enhances Their Interactions with Dendritic Cells during Antigen Recognition显示文摘 | Milka Sarris Kristian G. Andersen Felix Randow Luzia Mayr Alexander G. Betz | 2008 | Immunity2008，，3： | 1 |
| 7 | Phylogenetic definitions for 25 higher-level clade names of birds显示文摘Knowledge of the higher-level phylogenetic relationships of birds has grown substantially during the past two decades due to the application of genomic data.However,the nomenclature of higher-level taxa has not become more stable,due to the lack of regulation of taxon names above the level of superfamily by the ICZN,and the usage of rank-based nomenclature,which is not tied to clades in a phylogeny.Lack of regulation and the instability of rank-based nomenclature impede effective communication among systematists.We review support for higher-level avian clades using a set of 10 phylogenomic data sets,and identify clades that are supported by congruency of at least four of these.We provide formal definitions of the names of these clades based on the rules of the recently published PhyloCode.The names of 25 clades are here defined using minimum-crown-clade(n=23),minimum-clade(n=1)and maximum-crown-clade(n=1)definitions.Five new names are introduced here:Dinocrypturi,Pteroclimesites,Musophagotides,Phaethoquornithes and Pelecanes.We also review diagnostic apomorphies of the relevant clades,and identify known synonyms and homonyms.By establishing a formal link between higher-level taxon names and well-supported phylogenetic hypotheses,our phylogenetic definitions will provide a solid basis for the stabilization of avian higher-level nomenclature. | George Sangster Edward L.Braun Ulf S.Johansson Rebecca T.Kimball Gerald Mayr Alexander Suh | 2022 | Avian Research2022，13，2： | 0 |
您所在的机构暂未开通此部分的功能，请联系管理员