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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 | Dysregulation of iron and copper homeostasis in nonalcoholic fatty liver显示文摘Elevated iron stores as indicated by hyperferritinemiawith normal or mildly elevated transferrin saturation a n d m o s t l y m i l d h e p a t i c i r o n d e p o s i t i o n a r e a characteristic finding in subjects with non-alcoholic fatty liver disease(NAFLD). Excess iron is observed in approximately one third of NAFLD patients and is commonly referred to as the 'dysmetabolic iron overload syndrome'. Clinical evidence suggests that elevated body iron stores aggravate the clinical course of NAFLD with regard to liver-related and extrahepatic disease complications which relates to the fact that excess iron catalyses the formation of toxic hydroxylradicals subsequently resulting in cellular damage. Iron removal improves insulin sensitivity, delays the onset of type 2 diabetes mellitus, improves pathologic liver function tests and likewise ameliorates NAFLD histology. Several mechanisms contribute to pathologic iron accumulation in NAFLD. These include impaired iron export from hepatocytes and mesenchymal Kupffer cells as a consequence of imbalances in the concentrations of iron regulatory factors, such as hepcidin, cytokines, copper or other dietary factors. This review summarizes the knowledge about iron homeostasis in NAFLD and the rationale for its therapeutic implications. | Elmar Aigner Gunter Weiss Christian Datz | 2015 | World Journal of Hepatology2015，7，2： | 6 |
| 3 | CT scans increase rotational accuracy in total knee arthroplasty显示文摘 | Grafinger R W Datz L Hitzl W | 2008 | Z Orthop Unfall2008，146，6： | 1 |
| 4 | Diagnostic algorithm: how to make use ofnew 2D, 3D and4D ultrasound technologies in breast imaging显示文摘 | Weismann CF Datz L | 2007 | Eur J Radiol2007，64，2： | 1 |
| 5 | Influence of design and bearing material on polyethylene wear particle generation in total knee replacement显示文摘 | Utzschneider S Paulus A Datz JC | | 0，，07： | 1 |
| 6 | Immune Response to Natural and Recombinant Antigens of Helicobacter pylori in Patients with Dyspeptic Complaints显示文摘 | S. Graessle G. Grabher G. Gapp E. Preuss C. Datz F. Sandhofer G. St?ffler | 1999 | European Journal of Clinical Microbiology & Infectious Diseases1999，，9： | 1 |
| 7 | Hedgehog signaling is in- volved in differentiation of normal colonic tissue rather than in tumor proliferation 显示文摘 | Alinger B Kiesslich T Datz C | 2009 | Virchows Arch2009，454，4： | 1 |
| 8 | Exercise increases solid meal gastric emptying rates in men显示文摘 | Moore J G Datz F L Christian P E | 1990 | Dig Dis Sci1990，35，4： | 1 |
| 9 | Bordetella infections in dogs and eats:Pathogenesis,clinical signs,and diagnosis显示文摘 | Datz C | 2003 | Compendium on Continuing Education For the Practicing Veterinarian2003，25，12： | 1 |
| 10 | Abdominal abscess detection:gallium, 111In, and 99Tcm-labelled leukocytes, and polyclonal and monoclonal antibodies显示文摘 | Datz FL | 1996 | Semin Nucl Med1996，26，1： | 1 |
| 11 | Diagnostic algorithm: how to make use of new 2D, 3D and 4D ultrasotmd technologies in breast imaging显示文摘 | Weismann CF Datz L | 2007 | Eur J Radiol2007，64，2： | 1 |
| 12 | 查看详情显示文摘 | Datz S | | 0，，03： | 1 |
| 13 | Low-cost air-cooled PEFC stacks显示文摘 | H Schmidt P Buchner A Datz K Dennerlein S Lang M Waidhas | 2002 | Journal of Power Sources2002，，2： | 1 |
| 14 | Hedgehog signaling is involved in differentiation of normal colonic tissue rather than in tumor proliferation显示文摘 | Alinger B Kiesslich T Datz C Aberger F Strasser F Berr F | 2009 | Virehows Arch2009，454，： | 1 |
| 15 | Liver Microsomal Triglyceride Transfer Protein Is Involved in Hepatitis C Liver Steatosis显示文摘 | Silvia Mirandola Stefano Realdon Jahangir Iqbal Martina Gerotto Francesca Dal Pero Gladis Bortoletto Moira Marcolongo Alessandro Vario Christian Datz M. Mahmood Hussain Alfredo Alberti | 2006 | Gastroenterology2006，，6： | 1 |
| 16 | Continuous Automated Pressure Ulcer Monitoring 显示文摘 | NANCY ANN DOBBS PAM SPANBAUER DAVID DATZ | 2007 | JOURNAL FOP NURSES IN STAFF DEVELOPMENT2007，23，3： | 1 |
| 17 | Factors influencing the awake-sleep difference in ambulatory blood pressure: main effects and sex differences显示文摘 | James GD Toledano T Datz G | 1995 | J Hum Hypertens1995，9，： | 1 |
| 18 | Association of the IL28B genotype with insulin resistance in patients with chronic hepatitis C显示文摘 | Albert Friedrich St?ttermayer Karoline Rutter Sandra Beinhardt Thomas-Matthias Scherzer Andreas Stadlmayr Harald Hofer Fritz Wrba Petra Steindl-Munda Michael Krebs Christian Datz Michael Trauner Peter Ferenci | 2012 | Journal of Hepatology2012，，3： | 1 |
| 19 | Hedgehog signaling is involved in differentiation of normal colonic tissue rather than in tumor proliferation 显示文摘 | Alinger B Kiesslich T Datz C | 2009 | Virchows Arch2009，454，4： | 1 |
| 20 | Hepatic steatosis in Wilson disease – Role of copper and PNPLA3 mutations显示文摘 | Albert Friedrich St?ttermayer Stefan Traussnigg Hans-Peter Dienes Elmar Aigner Rudolf Stauber Karoline Lackner Harald Hofer Judith Stift Friedrich Wrba Andreas Stadlmayr Christian Datz Michael Strasser Andreas Maieron Michael Trauner Peter Ferenci | 2015 | Journal of Hepatology2015，，1： | 1 |
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