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| 1 | Transient elastography improves detection of liver cirrhosis compared to routine screening tests显示文摘AIM:To investigate the diagnostic significance oftransient elastography(TE) in a daily routine clinical setting in comparison to clinical signs,laboratory parameters and ultrasound.METHODS:TE,ultrasound,laboratory parameters and cutaneous liver signs were assessed in 291 consecutive patients with chronic liver disease of various aetiologies who underwent liver biopsy in daily routine.RESULTS:Sensitivity of TE for the detection of liver cirrhosis was 90.4%,compared to 80.1% for ultrasound,58.0% for platelet count and 45.1% for cutaneous liver signs(P < 0.0001 for comparisons with histology).AUROC for TE was 0.760(95%CI:0.694-0.825).Combination of TE with ultrasound increased sensitivity to 96.1% and AUROC to 0.825(95%CI:0.768-0.882).TE correlated with laboratory parameters of cirrhosis progression like albumin(r =-0.43),prothrombin time(r =-0.44),and bilirubin(r = 0.34; P < 0.001 for each).Particularly,in patients with Child Pugh score A or normal platelet count TE improved sensitivity for the detection of liver cirrhosis compared to ultrasound by 14.1%(P < 0.04) and 16.3%(P < 0.02),respectively.CONCLUSION:Transient elastography is superior to routine diagnostic tests allowing detection of liver cirrhosis in additional 10%-16% of patients with chronic liver disease that would have been missed by clinical examinations. | Thomas Gobel Janine Schadewaldt-Tümmers Lucas Greiner Christopher Poremba Dieter Haussinger Andreas Erhardt | 2015 | World Journal of Gastroenterology2015，21，3： | 8 |
| 2 | Partial external biliary diversion in bile salt export pump deficiency: Association between outcome and mutation显示文摘AIM To investigate the relation of two different mutations to the outcome of partial external biliary diversion(PEBD)in severe bile salt export pump(BSEP) deficiency.METHODS Mutations in the gene encoding BSEP leading to severe BSEP deficiency in two unrelated patients were identified by genomic sequencing. Native liver biopsies and transiently transfected human embryonic kidney(HEK) 293 cells expressing either wild-type or mutated BSEP were subjected to immunofluorescence analysis to assess BSEP transporter localization. Bile acid profiles of patient and control bile samples were generated by ultra-performance liquid chromatographytandem mass spectrometry. Wild-type and mutant BSEP transport of [~3H]-labeled taurocholate(TC) and taurochenodeoxycholate(TCDC) was assessed by vesicular transport assays.RESULTS A girl(at 2 mo) presented with pruritus, jaundice and elevated serum bile salts(BS). PEBD stabilized liver function and prevented liver transplantation. She was heterozygous for the BSEP deletion p.T919 del and the nonsense mutation p.R1235 X. At the age of 17 years relative amounts of conjugated BS in her bile were normal, while total BS were less than 3% as compared to controls. An unrelated boy(age 1.5 years) presenting with severe pruritus and elevated serum BS was heterozygous for the same nonsense and another missense mutation, p.G1032 R. PEBD failed to alleviate pruritus, eventually necessitating liver transplantation. BS concentration in bile was about 5% of controls. BS were mainly unconjugated with an unusual low amount of chenodeoxycholate derivatives(< 5%). The patients' native liver biopsies showed canalicular BSEP expression. Both BSEP p.T919 del and p.G1032 R were localized in the plasma membrane in HEK293 cells. In vitro transport assays showed drastic reduction of transport by both mutations. Using purified recombinant BSEP as quantifiable reference, per-molecule transport rates for TC and TCDC were determined to be 3 and 2 BS molecules per wild-type BSEP transporter per minute, respectively.CONCLUSION In summary, our findings suggest that residual function of BSEP as well as substrate specificity influence the therapeutic effectiveness of PEBD in progressive familial intrahepatic cholestasis type 2(PFIC-2). | Philipp Ellinger Jan Stindt Carola Droge Katharina Sattler Claudia Stross Stefanie Kluge Diran Herebian Sander HJ Smits Martin Burdelski Sebastian Schulz-Jürgensen Antje Ballauff Jan Schulte am Esch Ertan Mayatepek Dieter Haussinger Ralf Kubitz Lutz Schmitt | 2017 | World Journal of Gastroenterology2017，23，29： | 4 |
| 3 | Hepatic encephalopathy as a complication of liver disease显示文摘INTRODUCTIONHepatic encephalopathy ( HE) is a frequent complication of chronic liver disease .It is defined as a characteristic functional and reversible alteration of the mental state ,due to impaired liver function and / or increased portosystemic shunting . | Stephan vom Dahl Gerald Kircheis Dieter Haussinger | 2001 | World Journal of Gastroenterology2001，7，2： | 2 |
| 4 | Sorafenib ac- tivates CD95 and promotes autophagy and cell death via Src family kinases in gastrointestinal tumor cells显示文摘 | Park MA Reinehr R Haussinger D | 2010 | Mol Cancer Ther2010，9，8： | 1 |
| 5 | Association between duplex Doppler sonographic flow pattern in right hepatic vein and various liver diseases显示文摘 | Von Herbay A Frieling T Haussinger D | 2001 | J Clin Ultrasound2001，29，1： | 1 |
| 6 | Osmotic and oxidative/nitrosative stress in ammonia toxicity and hepatic encephalopathy 显示文摘 | Gorg B Schliess F Haussinger D | 2013 | Arch Biochem Biophys2013，536，2： | 1 |
| 7 | Cloning and molecular characterization of human high affinity antibody fragments against hepatitis C virus NS3 helicase 显示文摘 | Tessmann K Erhardt A Haussinger D | 2002 | J Virol Methods2002，103，1： | 1 |
| 8 | Cellular hydration state:an important determinant of protein catabolism in health and disease显示文摘 | Haussinger D Roth E Lang F | 1993 | Lancet1993，341，8856： | 1 |
| 9 | Efficiency of video-assisted thoracoscopic surgery for primary and secondary spontaneous pneumothorax显示文摘 | Passlick B Born C Haussinger K | 1998 | Ann Thorac Surg1998，65，： | 1 |
| 10 | The membrane - bound bile acid receptor TGR5 (Gpbar-1) is localized in the primary cilium of cholangiocytes显示文摘 | KEITEL V ULLMER C HAUSSINGER D | 2010 | Biol Chem2010，391，7： | 1 |
| 11 | Effects of hyper - and hypoosmolality on whole body protein and glucose kinetics in humans显示文摘 | Berneis K Ninnis R Haussinger D | 1999 | Am J Physiol1999，276，11： | 1 |
| 12 | Association between du- plex Doppler sonographic flow pattern in right hepatic vein and va- rious liver diseases 显示文摘 | van Herbay A Frieling T Haussinger D | 2001 | J Clin Ultrasound2001，29，1： | 1 |
| 13 | The role of cellular hydration in the regulation of cell function 显示文摘 | Haussinger D | 1996 | Biochem J1996，313，3： | 1 |
| 14 | Hepatic encephaiopathy显示文摘 | Kircheis G Haussinger D | 2012 | Dtsch Med Wochenschr2012，137，4： | 1 |
| 15 | Association between duplex Doppler soncgraphic flow pattern in right hepatic vein and various liver diseases显示文摘 | Von Herhay A Frieling T Haussinger D | 2001 | J Clin Ultrasound2001，29，1： | 1 |
| 16 | Hepatic and pancreatic stellate cells in focus显示文摘 | Kordes C Sawitza I Haussinger D | 2009 | Biol Chem2009，390，10： | 1 |
| 17 | Astroeyte swelling and protein tyrosine nitra- tion in hepatic eneephalopathy 显示文摘 | Haussinger D Sehliess F | 2005 | Neurochem Int2005，47，12： | 1 |
| 18 | Hepatic encephalopathy 显示文摘 | Haussinger D | 2010 | Acta Gastroen- terol Belg2010，73，4： | 1 |
| 19 | Pathogenetic mechanisms of hepatic encephalopathy显示文摘 | Haussinger D Schliess F | 2008 | Gut2008，57，： | 1 |
| 20 | Hepatic Encephalopathy in Chronic Liver Disease:a Clinical Manifestation of Astrocyte Swelling and Low grade Cerebral Edema显示文摘 | Haussinger D Kircheis G Fischer R | 2000 | J Hepatol2000，32，6： | 1 |
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