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| 1 | New insights in bilirubin metabolism and their clinical implications显示文摘Bilirubin,a major end product of heme breakdown,is an important constituent of bile,responsible for its characteristic colour.Over recent decades,our understanding of bilirubin metabolism has expanded along with the processes of elimination of other endogenous and exogenous anionic substrates,mediated by the action of multiple transport systems at the sinusoidal and canalicular membrane of hepatocytes.Several inherited disorders characterised by impaired bilirubin conjugation(Crigler-Najjar syndrome typeⅠand typeⅡ,Gilbert syndrome)or transport(Dubin-Johnson and Rotor syndrome)result in various degrees of hyperbilirubinemia of either the predominantly unconjugated or predominantly conjugated type.Moreover,disrupted regulation of hepatobiliary transport systems can explain jaundice in many acquired liver disorders.In this review,we discuss the recent data on liver bilirubin handling based on the discovery of the molecular basis of Rotor syndrome.The data show that a substantial fraction of bilirubin conjugates is primarily secreted by MRP3 at the sinusoidal membrane into the blood,from where they are subsequently reuptaken by sinusoidal membrane-bound organic anion transporting polypeptides OATP1B1 and OATP1B3.OATP1B proteins are also responsible for liver clearance of bilirubin conjugated in splanchnic organs,such as the intestine and kidney,and for a number of endogenous compounds,xenobiotics and drugs.Absence of one or both OATP1B proteins thus may have serious impact on toxicity of commonly used drugs cleared by this system such as statins,sartans,methotrexate or rifampicin.The liverblood cycling of conjugated bilirubin is impaired in cholestatic and parenchymal liver diseases and this impairment most likely contributes to jaundice accompanying these disorders. | Eva Sticova Milan Jirsa | 2013 | World Journal of Gastroenterology2013，19，38： | 24 |
| 2 | Dubin-Johnson syndrome coinciding with colon cancer and atherosclerosis显示文摘Hyperbilirubinemia has been presumed to prevent the process of atherogenesis and cancerogenesis mainly by decreasing oxidative stress.Dubin-Johnson syndrome is a rare,autosomal recessive,inherited disorder characterized by biphasic,predominantly conjugatedhyperbilirubinemia with no progression to end-stage liver disease.The molecular basis in Dubin-Johnson syndrome is absence or deficiency of human canalicular multispecific organic anion transporter MRP2/cMOAT caused by homozygous or compound heterozygous mutation(s) in ABCC2 located on chromosome 10q24.Clinical onset of the syndrome is most often seen in the late teens or early adulthood.In this report,we describe a case of previously unrecognized Dubin-Johnson syndrome caused by two novel pathogenic mutations (c.2360_2366delCCCTGTC and c.3258+1G>A),coinciding with cholestatic liver disease in an 82-year-old male patient.The patient,suffering from advanced atherosclerosis with serious involvement of coronary arteries,developed colorectal cancer with nodal metastases.The subsequent findings do not support the protective role of Dubin-Johnson type hyperbilirubinemia. | Eva Sticova Milan Elleder Helena Hulkova Ondrej Luksan Martin Sauer Irena Wunschova-Moudra Jan Novotny Milan Jirsa | 2013 | World Journal of Gastroenterology2013，19，6： | 4 |
| 3 | Novel ABCB11 mutations in a Thai infant with progressive familial intrahepatic cholestasis显示文摘Progressive familial intrahepatic cholestasis(PFIC) type 2 is caused by mutations in ABCB11,which encodes bile salt export pump(BSEP).We report a Thai female infant who presented with progressive cholestatic jaundice since 1 mo of age,with normal serumγ-glutamyltransferase.Immunohistochemical staining of the liver did not demonstrate BSEP along the canaliculi,while multidrug resistance protein 3 was expressed adequately.Novel mutations in ABCB11,a four-nucleotide deletion in exon 3,c.90_93delGAAA,and a single-nucleotide insertion in exon 5,c.249_250insT, were identified,with confirmation in her parents. These mutations were predicted to lead to synthesis of truncated forms of BSEP.Immunostaining and mutation analysis thus established the diagnosis of PFIC type 2. | Suporn Treepongkaruna Amornphun Gaensan Paneeya Pienvichit Ondrej Luksan AS Knisely Pattana Sornmayura Milan Jirsa | 2009 | World Journal of Gastroenterology2009，15，34： | 3 |
| 4 | Relevance of low viral load in haemodialysed patients with chronic hepatitis C virus infection显示文摘AIM: To identify predictors of sustained virological response in hemodialysed patients treated by PEGinterferon α for chronic hepatitis C, genotype 1.METHODS: The sustained virological response(SVR) rate, IL28 B genotype, IFNL4 genotype, initial viral load(IVL) and other pretreatment variables in 39 endstage renal disease patients(ESRD) on maintenance haemodialysis(HD) infected with hepatitis C virus(HCV), genotype 1b, were compared with a control group of 109 patients with normal kidney function treated within the same period. All the patients were treatment nave and had well compensated liver disease. The ESRD patients received 135 μg of PEGylated interferon α-2a(Peg IFN-α) weekly and a reduced dose of ribavirin(RBV) was administered to 23/39 patients with an initial haemoglobin level > 10 g/d L. Control group patients were given standard doses of Peg IFN-α and RBV. SVR was assessed as HCV RNA negativity 24 wk post-treatment. A t-test or ANOVA were used for comparisons of the means and a χ2 testcompared the frequencies.Logistic regression was used to determine significant predictors of SVR.Cutoff values for continuous variables were obtained from Receiver Operating Characteristic analysis.RESULTS:The distribution of IL28B rs12979860 CC,CT and TT genotypes in the ESRD group was 28.2%,64.1%and 7.7%,respectively,and 19.3%,62.4%and18.3%in the controls.The IFNL4 genotype was in almost absolute linkage disequlibrium with IL28B.The proportion of patients with a low IVL(<600000 IU/m L)was significantly higher in the ESRD group than in the controls(28/39,71.8%vs 51/109,46.8%,P=0.009),as was the proportion of patients with low IVL in IL28B CC carriers compared with non-CC carriers in the ESRD group(10/11,90.9%vs 18/28,64.3%,P=0.0035).This difference was not found in the controls(7/22,31.8%vs 44/87,50.6%,P=0.9).The overall SVR rate was 64.1%(25/39)in the ESRD group and 50.5%(55/109)in the control group(P=0.19).11/11(100%)and 19/22(86.4%)IL28B CC patients achieved SVR in the ESRD and control groups,respectively.A statistically significant association between SVR and IL28B and IFNL4 variants was found in both groups.The ESRD patients who achieved SVR showed the lowest IVL[median 21000,interquartile range(IQR):6000-23000IU/m L],compared with ESRD individuals without SVR(1680000,IQR:481000-6880000,P=0.001),controls with SVR(387000,IQR:111000-1253000)and controls without SVR(905000,IQR:451000-3020000).In ESRD,an IVL<600000 IU/m L was strongly associated with SVR:24/28(85.7%)patients who achieved SVR had viraemia below this threshold.CONCLUSION:Haemodialysis decreases the viral load,especially in IL28B CC genotype carriers.A low IVL was the strongest predictor of SVR in ESRD patients identified in multivariate analysis. | Jan Sperl Sona Frankova Renata Senkerikova Magdalena Neroldova Vaclav Hejda Miroslava Volfova Dusan Merta Ondrej Viklicky Julius Spicak Milan Jirsa | 2015 | World Journal of Gastroenterology2015，21，18： | 2 |
| 5 | Shear requirelnents for load reversals on RC members显示文摘 | Gosain N K Brown RH Jirsa J O | 1977 | Journal of Structural Engineering /KSCE1977，103，7： | 1 |
| 6 | Shear in joints and other places 显示文摘 | Jirsa J O | 1995 | Recent Developments in Lateral Force Transfer in Buildings ACI1995，1573，： | 1 |
| 7 | The concept of cumula- tive ductility strength spectra and its use within per- formance-based seismic design 显示文摘 | Teran-Gilmore A Jirsa J Q | 2004 | ISET Journal of Earthquake Technology2004，41，1： | 1 |
| 8 | Shear behavior of full-scale reinforced concrete T-beams strengthened with CFRP strips and anchors显示文摘 | Kim Y Ghannoum W M Jirsa J O | 2015 | Construction & Building Materials2015，94，： | 1 |
| 9 | Shear strength of R/C Beam - column connections显示文摘 | Meinheit D F Jirsa J O | 1993 | Journal of Structural1993，，1： | 1 |
| 10 | Effects ofAlkali-silica Reaction and Delayed Ettringite Forma-tion on Anchorage of Prestressing Strands in Trape-zoidal Box Beams with Dapped Ends显示文摘 | LARSON N A’BAYRAK O’JIRSA J O | 2012 | PCI Journal?2012，57，3： | 1 |
| 11 | Enhancement of neural synchrony by time delay显示文摘 | Dhamala M Jirsa V K Ding M | 2004 | Phys Rev Lett2004，92，： | 1 |
| 12 | Maximum length sequences-auditory brainstem responses from children with auditory processing disorders 显示文摘 | Jirsa RE | 2001 | J Am Acad Audiol2001，12，3： | 1 |
| 13 | A damage model for practical seismic design that accounts for low cycle fatigue显示文摘 | Teran-Gilmore A Jirsa J Q | 2005 | Earthquake Spectra2005，21，3： | 1 |
| 14 | Behavior of concrete under compressive loadings显示文摘 | KARSAN I D JIRSA J O | 1969 | Journal of Structure Division1969，95，12： | 1 |
| 15 | Genetic background of cholesterol gallstone disease显示文摘 | Kosters A Jirsa M Groen AK | 2003 | Bioehim Biophys Acta2003，1637，1： | 1 |
| 16 | Shear in joints and other places显示文摘 | JIRSA J O | 1995 | Recent developments in lateral force transfer in buildings SP157-3 ACI1995，5973，： | 1 |
| 17 | Behaviour of concrete under compressive loadings 显示文摘 | Karsan I K Jirsa J O | 1969 | Journal of Structural Division ASCE1969，95，12： | 1 |
| 18 | Genetic background of cholesterol gallstone disease显示文摘 | Kosters A Jirsa M Groen A K | 2003 | Biochim Biophys Acta2003，1637，1： | 1 |
| 19 | The concept of cumulative ductility strength spectra and its use within performance- based seismic design 显示文摘 | Teran-Gilmore A Jirsa J Q | 2004 | ISET Journal of Earthquake Technology2004，41，1： | 1 |
| 20 | Behaviour of concrete under compressive loadings 显示文摘 | Karsan I K Jirsa J O | 1969 | Journal of the Structural Division ASCE1969，95，12： | 1 |
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