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| 1 | 感染性休克病人的血流动力学特点及意义显示文摘目的 了解感染性休克病人早期血流动力学改变的特点及与预后关系。方法 临床前瞻性研究 ,监测感染性休克早期血流动力学改变并分析与预后的关系。结果 共连续观察 4 8例病人 ,其中 2 5例(5 2 % )死亡。一些指标的水平在死亡者与生存者间差别较大 ,其中包括平均肺动脉压 (MPAP)、肺动脉楔压 (PAWP)、体 /肺循环阻力指数 (SVRI/PVRI)、左 /右心做功 (LCW/RCW )和左 /右心每搏动 (LCSW/RCSW)等血流动力学指标和氧合指数、动脉混合静脉血含氧量差 (a vDO2 )、全身性炎症反应综合征(SIRS)指标积分等。结论 感染性休克病情凶险 。 | 赵擎宇 贾清颜 Weigand MA Martin E Bardenheuer HJ | 2002 | 中华急诊医学杂志2002,11,3: | 16 |
| 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 | Malignant mesothelioma显示文摘 | Lerner HJ Schoenfeld DA Martin A | 1983 | Cancer1983,52,: | 1 |
| 4 | Genetic diversity in the protective antigen gene of Bacillus anthracis 显示文摘 | Lance BP Martin HJ Paul J | 1999 | J Bacter1999,181,23: | 1 |
| 5 | Manipulation of the toll-like receptor 7 signaling pathway by Epstein-Barr virus 显示文摘 | Martin HJ Lee JM Walls D etal | 2007 | J Virol2007,81,: | 1 |
| 6 | Anatomic and biome- chanical considerations of the craniovertebral junction 显示文摘 | Martin MD Bruner HJ Maiman DJ | 2010 | Neurosurgery2010,66,3: | 1 |
| 7 | Honokiol blocks store operated calcium entry in CHO cells expressing the M3muscarinic receptor: honokiol and muscarinic signaling 显示文摘 | Wang HJ Martin AG Chao PK | 2013 | Journal of biomedical science2013,20,1: | 1 |
| 8 | Synovial joint degeneration and the syndrome of osteoarthritis显示文摘 | Buckwalter JA Martin J Mankin HJ | | 0,,3: | 1 |
| 9 | The radio therapeutic injury - a complex wound显示文摘 | James WD Martin HJ | 2002 | Radio Ther Oncol2002,63,2: | 1 |
| 10 | Human preimplantation development in vitro is not adversely affected by biopsy at the8 -cell tage显示文摘 | Hardy K Martin KL Leese HJ | 1990 | Hum Reprod1990,5,6: | 1 |
| 11 | The photodegradation of vitamins A and E in parenteral nutrition mixtures during infusion 显示文摘 | Martin HJ | 2000 | Clin Nutr2000,19,5: | 1 |
| 12 | Anatomic and biomechanical considerations of the craniovertebral junction显示文摘 | Martin MD Bruner HJ Maiman DJ | 2010 | Neurosurgery2010,66,3: | 1 |
| 13 | Role of human aldo-keto-reductase AKR1B10 in the protection against toxic aldehydes显示文摘 | Martin HJ Maser E | | 0,,: | 1 |
| 14 | Enhanced bonding of chitosan to implant quality titanium via four treatment combinations显示文摘 | Martin HJ Schulz KH Bumgardner JD | 2008 | Thin Solid Films2008,516,18: | 1 |
| 15 | Comparison of pyruvate uptake by embryos derived from conception and non-conception natural cycles显示文摘 | Turner K Martin KL Woodward B J Lenton EA Leese HJ | 1994 | Hum Reprod1994,9,12: | 1 |
| 16 | Human preimplantation development in vitro is not adversely affected biopsy at the 8-cell stage 显示文摘 | Hardy K Martin KL Leese HJ | 1990 | Hum Reprod1990,5,6: | 1 |
| 17 | Manipulation of the toll-like receptor 7 signaling pathway by Epstein-Barr virus显示文摘 | Martin HJ Lee JM Walls D | 2007 | J Virol2007,81,18: | 1 |
| 18 | Is hand-held dynamometry use- ful for the measurement of quadriceps strength in older people? A com- parison with the gold standard Bodexdynamometry显示文摘 | Martin HJ Yule V Syddall HE | 2006 | Gerontology2006,52,3: | 1 |
| 19 | Transfemoral digital subtraction angiography for assessment of vertebral artery occlusion in rats显示文摘 | Martin E Bardenheuer HJ | 2000 | Stroke2000,31,: | 1 |
| 20 | Momordica charantia ex-tract, a herbal remedy for type 2 diabetes, contains a specific11 p-hydroxysteroid dehydrogenase type 1 inhibitor 显示文摘 | Blum A Loerz C Martin HJ | 2012 | J SteroidBiochem Mol Biol2012,128,12: | 1 |