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| 1 | Diagnostic criteria for acute liver failure due to Wilson disease显示文摘AIM: To describe the diagnostic criteria for acute liver failure due to Wilson disease (WD), which is an uncommon cause of acute liver failure (ALF). METHODS: We compared findings of patients presenting with ALF due to WD to those with ALF of other etiologies. RESULTS: Previously described criteria, such as low alkaline phosphatase activity, ratio of low alkaline phosphatase to total bilirubin or ratio of high aspartate aminotransferase (AST) to alanine aminotransferase (ALT), failed to identify patients with ALF due to WD. There were significant differences in low ALT and AST activities (53 ± 43 vs 1982 ± 938, P < 0.0001 and 87 ± 44 vs 2756 ± 2941, P = 0.037, respectively), low choline esterase activity (1.79 ± 1.2 vs 4.30 ± 1.2, P = 0.009), high urine copper concentrations (93.4 ± 144.0 vs 3.5 ± 1.8, P = 0.001) and low hemoglobin (7.0 ± 2.2 vs 12.6 ± 1.8, P < 0.0001) in patients with ALF caused by WD as compared with other etiologies. Interestingly, 4 of 7 patients with ALF due to WD survived without liver transplantation. CONCLUSION: In ALF, these criteria can help establish a diagnosis of WD. Where applicable, slit- lamp examination for presence of Kayser-Fleischer rings and liver biopsy for determination of hepatic copper concentration still remain important for the diagnosis of ALF due to WD. The need for liver transplantation should be evaluated carefully as the prognosis is not necessarily fatal. | Christoph Eisenbach Olivia Sieg Wolfgang Stremmel Jens Encke Uta Merle | 2007 | World Journal of Gastroenterology2007，13，11： | 14 |
| 2 | Copper toxicosis gene MURRl is not changed in Wilson disease patients with normal blood ceruloplasmin levels显示文摘瞄准:在为 MURR1 编码的基因为改变分析我们的威尔森疾病病人队(n=106 ) 。方法:有威尔森疾病的确定的诊断的病人但是正常血浆铜蓝蛋白血层次为我们的学习被选择(n = 14 ) 。有在 ATP7B 基因的二个已知的引起疾病的变化的病人没被包括。人的 MURR1 基因的三前 ons 被聚合酶链反应在 genomic DNA 的扩大以后定序。结果:我们的学习没揭示在威尔森疾病病人的 MURR1 顺序导致一个氨基酸变化的任何变化。在编码顺序的 472 bp 的多型性能被证实。结论:MURR1 基因不与正常浆液血浆铜蓝蛋白层次在威尔森疾病病人的致病起作用。 | Karl Heinz Weiss Uta Merle Mark Schaefer Peter Ferenci Joachim Fullekrug Wolfgang Stremmel | 2006 | World Journal of Gastroenterology2006，12，14： | 4 |
| 3 | Non-alcoholic fatty liver disease and COVID-19: Harmless companions or disease intensifier?显示文摘The pandemics of coronavirus disease 2019(COVID-19)and non-alcoholic fatty liver disease(NAFLD)coexist.Elevated liver function tests are frequent in COVID-19 and may influence liver damage in NAFLD,while preexisting liver damage from NAFLD may influence the course of COVID-19.However,the prognostic relevance of this interaction,though,is unclear.Obesity is a risk factor for the presence of NAFLD as well as a severe course of COVID-19.Cohort studies reveal conflicting results regarding the influence of NAFLD presence on COVID-19 illness severity.Striking molecular similarities of cytokine pathways in both diseases,including postacute sequelae of COVID-19,suggest common pathways for chronic low-activity inflammation.This review will summarize existing data regarding the interaction of both diseases and discuss possible mechanisms of the influence of one disease on the other. | Christoph G Dietrich Andreas Geier Uta Merle | 2023 | World Journal of Gastroenterology2023，29，2： | 2 |
| 4 | Cancer-related microangiopathic hemolytic anemia in patients with advanced gastric cancer: A retrospective single-center analysis显示文摘BACKGROUND Microangiopathic hemolytic anemia(MAHA) with thrombocytopenia and organ failure caused by tumor-associated thrombotic microangiopathy(TMA) is a lifethreatening oncological emergency. Rapid diagnosis and precise distinction from other forms of TMA is crucial for appropriate therapy, which aims at treating the underlying malignancy. However, the prognosis of patients with cancer-related(CR)-MAHA is limited. To date, less than 50 patients with gastric cancer and CRMAHA have been reported, mainly as single case reports, and detailed information on treatment strategies and outcome are scarce. We analyzed the characteristics and outcomes data of CR-MAHA patients with gastric cancer treated at our center between 2012 and 2019.AIM To gain knowledge about CR-MAHA and the course of disease.METHODS We retrospectively analyzed patients using an institutional prospectively maintained database. Patients who had CR-MAHA but other cancer types or cancer of unknown primary were excluded. The basic requirements for inclusion were: Histologically proven gastric adenocarcinoma;and clinical diagnosis of hemolytic anemia with schistocytes with or without thrombocytopenia. The observation period for each patient started with the first day of documented symptoms. The follow-up period for this analysis ended on February 1, 2020.RESULTS We identified eight patients with a median age of 54 years. Histologically, all patients had(partial) diffuse subtypes of gastric adenocarcinoma with partial or complete signet cell morphology. All patients had metastatic disease and one patient had a microsatellite instability-high(MSI-H) tumor. In three patients, clinical signs of MAHA preceded the diagnosis of cancer, and in two patients, CRMAHA indicated recurrent disease. All patients had severe hemolytic anemia and thrombocytopenia. Six patients experienced severe bone pain, and five patients had dyspnea. Systemic, 5-fluorouracil-based combination chemotherapy was initiated in six patients, which resulted in rapid initial response with significant improvement of clinical symptoms and blood values. Progression-free survival(PFS) of the whole cohort was 1.9 wk and median overall survival(OS) was 1.9 wk. For patients with chemotherapy, PFS was 9.0 wk and OS was 10.3 wk. The patient with the MSI-H tumor has been undergoing immunotherapy for more than 3 years.CONCLUSION The benefit of chemotherapy in CR-MAHA patients is limited. Immunotherapy for patients with MSI-H tumors may lead to long-term tumor control even in CRMAHA patients. | Anne Katrin Berger Michael Allgäuer Leonidas Apostolidis Anna Elisa Schulze-Schleithoff Uta Merle Dirk Jaeger Georg Martin Haag | 2020 | World Journal of Gastrointestinal Oncology2020，12，11： | 1 |
| 5 | Lentiviral gene transfer ameliorates disease progression in Long-Evans cinnamon rats: An animal model for Wilson disease显示文摘 | Uta Merle Jens Enckea Sabine Tuma Martin Volkmann Luigi Naldini Wolfgang Stremmel | 2006 | Scandinavian Journal of Gastroenterology2006，，8： | 1 |
| 6 | Serum ceruloplasmin oxidase activity is a sensitive and highly specific diagnostic marker for Wilson’s disease显示文摘 | Uta Merle Christoph Eisenbach Karl Heinz Weiss Sabine Tuma Wolfgang Stremmel | 2009 | Journal of Hepatology2009，，5： | 1 |
| 7 | Zinc Monotherapy Is Not as Effective as Chelating Agents in Treatment of Wilson Disease显示文摘 | Karl Heinz Weiss Daniel Nils Gotthardt Daniela Klemm Uta Merle Daniela Ferenci–Foerster Mark Schaefer Peter Ferenci Wolfgang Stremmel | 2011 | Gastroenterology2011，，4： | 1 |
| 8 | Efficacy and Safety of Oral Chelators in Treatment of Patients With Wilson Disease显示文摘 | Karl Heinz Weiss Florentine Thurik Daniel Nils Gotthardt Mark Sch?fer Ulrike Teufel Franziska Wiegand Uta Merle Daniela Ferenci–Foerster Andreas Maieron Rudolf Stauber Heinz Zoller Hartmut H. Schmidt Ulrike Reuner Harald Hefter Jean Marc Trocello Roderick | 2013 | Clinical Gastroenterology and Hepatology2013，，8： | 1 |
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