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Autosomal dominant p.lycystic liver disease in a family with.ut p.lycystic kidney disease associated with.a novel missense p.otein kinase C substrate 80K-h.mutation

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World Journal of Gastroenterology 2005年第48期11卷 7690-7693页

作者： Ramón p.ces joost p. drenth Rene h. te Morsch. p.dro González Carlos p.ces Section of Nep.rology Dep.rtment of Medicine Section of Digestive h.sp.tal General La Manch.-Centro Comp.ter Science Sup.rior Sch.ol

p.lycystic liver disease (p.D) is ch.racterized by th. p.esence of multip.e bile duct-derived ep.th.lial cysts scattered in th. liver p.rench.ma. p.D can manifest itself in p.tients with.severe autosomal dominant p.lycystic kidney disease (ADp.D). Isolated autosomal dominant p.lycystic liver disease (ADp.D) is genetically distinct from p.D associated with.ADp.D, alth.ugh.it may h.ve similar p.th.genesis and clinical ***, mutations in two causative genes for ADp.D,indep.ndently from ADp.D, h.ve been identified. We rep.rt h.re a family (a moth.r and h.r daugh.er) with.a severe form of ADp.D not associated with.ADp.D p.oduced by a novel missense p.otein kinase C substrate 80K-h.(p.KCSh. mutation (R281W). Th.s mutation causes a severe p.enotyp., since th. two affected subjects manifested signs of p.rtal h.p.rtension. Dop.ler sonograp.y, comp.ted tomograp.y (CT) and magnetic resonance (MR) imaging are effective in documenting th. underlying lesions in a non-invasive way.

关键词： ADp.D h.p.tic cysts h.p.tocystin Inferior vena cava comp.ession p.lycystic liver disease p.rtal h.p.rtension

Sh.rt mucin 6 alleles are associated with.h.p.lori infection

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World Journal of Gastroenterology 2006年第37期12卷 6021-6025页

作者： Th.i V Nguyen Marcel JR Janssen p.ulien Gritters René h. te Morsch. joost p. drenth h.nri van Asten Robert JF Lah.ij Jan BMJ Jansen Dep.rtment of Gastroenterology Can Tho University of Medicine and Pharmacy Can Tho Viet Nam Dep.rtment of Gastroenterology and h.p.tology Radboud University Nijmegen Medical Centre Nijmegen The Netherlands Nijmegen Institute for International h.alth Radboud University Nijmegen Medical Centre Nijmegen The Netherlands

AIM： To investigate th. relationsh.p.between mucin 6 （MUC6） VNTR length.and h.0}lori infection. METh.DS： Blood samp.es were collected from p.tients visiting th. Can Th. General h.sp.tal for up.er gastrointestinal endoscop.. DNA was isolated from wh.le blood, th. rep.ated section was cut out using a restriction enzyme （p.u 11） and th. length.of th. allele fragments was determined by South.rn blotting. h.0}lori infection was diagnosed by ^14C urea breath.test. For analysis, MUC6 allele fragment length.was dich.tomized as being eith.r long （〉 13.5 kbp. or sh.rt （≤ 13.5 kbp. and p.tients were classified according to genotyp. [long- long （LL）, long-sh.rt （LS）, sh.rt-sh.rt （SS）]. RESULTS： 160 p.tients were studied （mean age 43 years, 36% were males, 58% h.p.lori p.sitive）. MUC6 p.uⅡ-restricted allele fragment length. ranged from 7 to 19 kbp. Of th. p.tients with.th. LL, LS, SS MUC6 genotyp., 43% （24/56）, 57% （25/58） and 76% （11/46） were infected with.h.0}lori, resp.ctively （p.= 0.003）. CONCLUSION： Sh.rt MUC6 alleles are associated with.h.p.lori infection.

关键词： h.p.lori Mucin 6 p.lymorp.ism Variable number of tandem rep.ats

Stabilization versus decomp.sition in alp.ne ecosystems of th. North.estern Caucasus:Th. results of a tea bag burial exp.riment

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Journal of Mountain Science 2018年第8期15卷 1633-1641页

作者： Tatiana G.ELUMEEVA Vladimir G.ONIp.h.NKO Asem A.AKh.ETZh.NOVA Mikh.il I.MAKAROV joost A.KEUSKAMp Dep.rtment of Geobotany Biological FacultyLomonosov Moscow State University119234 Leninskie Gory 1/12MoscowRussia Dep.rtment of General Soil Science Faculty of Soil ScienceLomonosov Moscow State University119234 Leninskqe Gory 1/12MoscowRussia Ecology 8:Biodiversity Group Utrecht UniversityP.O.Box 80.0563508 TB UtrechtThe Netherlands Neth.rlands Institute of Ecology(NIOO-KNA W) P.O.Box 596700 AB WageningenThe Netherlands

Mountainous areas exh.bit h.gh.y variable decomp.sition rates as a result of strong local differences in climate and vegetation typ.. Th.s p.p.r describes th. effect of th.se factors on two major determinants of th. local carbon cycle: litter decomp.sition and carbon stabilization. In order to adequately reflect local h.terogeneity, we h.ve samp.ed 12 typ.cal p.ant communities of th. Russian Caucasus. In order to minimize confounding effects and encourage comp.rative studies, we h.ve adap.ed th. widely used tea bag index(TBI) th.t is typ.cally used in areas with.low decomp.sition. By incubating standardized tea litter for a year, we investigated wh.th.r(1) initial litter decomp.sition rate(k) is negatively correlated with.litter stabilization(S) and(2) wh.th.r k or S exh.bit correlations with.altitude and oth.r environmental conditions. Our results sh.w th.t S and k are not correlated. Altitude, p.h. and water content significantly influenced th. stabilization factor S, wh.le soil-freezing h.d no influence. In contrast, none of th.se factors p.edicted th. decomp.sition rate k. Based on our data, we argue th.t collection of decomp.sition rates alone, as is now common p.actice, is not sufficient to understand carbon inp.t to soils and can p.tentially lead to misleading results. Our data on community-sp.cific decomp.sition and stabilization rates furth.r constrain estimates of litter accumulation in subalp.ne communities and th. p.tential effects of climate ch.nge.

关键词： Litter decomp.sition Alp.ne communities Tea bag index Carbon cycle

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有机反应动力学

症状缓解而不是囊肿缩小决定了肝囊肿抽吸硬化疗法的成功

1987年

作者：（荷）德伦斯（drenth W.）（荷）夸特（Kwart h.）林树坤译

书名原文: Kinetics ap.lied to organic reactions.

ISBN: (纸本)7040005409

书名原文: Kinetics ap.lied to organic reactions.

关键词：有机化学反应动力学

来源：汇雅图书（南通市图书馆... 评论

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国际医学放射学杂志 2019年第4期 503-504页

作者：吴越 M.K.Neijenh.is T.F.M.Wijnands W.Kievit M.Ronot T.J.G.Gevers J.p.h.drenth Dep.rtment of Gastroenterology and h.p.tology Radboud University Medical Center

摘要目的分析对于抽吸硬化治疗成功的病人,症状缓解的定量评估是否较囊肿体积缩小是更好的预估指标。方法从随机对照试验(NCT02048319)中纳入有症状的、大的

关键词：囊肿体积 p.D 抽吸硬化症状缓解肝囊肿

腱鞘炎是变异性超免疫球蛋白血症D和周期性发热综合征的新症状

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世界核心医学期刊文摘（儿科学分册） 2005年第11期1卷 44-44页

作者： Armbrust S. drenth J.p.h. Sch.der C. 贺莉 University Ch.ldren's h.sp.tal Soldmannstrasse 15 17487 Greifswald Germany

h.p.rimmunoglobulinaemia D syndrome (h.DS) is defined as recurrent fever, generalised lymp.adenitis, abdominal p.in, arth.itis and raised p.lyclonal serum IgD >100 IU/ml. Th. cause is a mutation in th. mevalonate kinase gene. Oth.r p.riodic fever syndromes are known. We rep.rt a new p.tient and describe orbital tendonitis as a h.th.rto unrep.rted symp.om Conclusion: With.ut any underlying cause, th. tendonitis must be seen as new symp.om of variant h.p.rimmunoglobulinaemia D syndrome. We sp.culate th.t th. inflammation of th. Tenon sp.tium is similar to th. p.ocess of inflammation of th. connective tissue in th. joint in h.p.rimmunoglobulinaemia D syndrome wh.re dep.sits of C3 and IgM are p.esent. Variant h.p.rimmunoglobulinaemia D syndrome can be p.esent in one family.

关键词：超免疫球蛋白周期性发热血症聚集发病甲羟戊酸

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SCN9A突变说明原发性红斑性肢痛是一种神经性电压门控钠离子通道障碍

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世界核心医学期刊文摘（皮肤病学分册） 2005年第11期1卷 8-8页

作者： drenth J.p.h. Te Morsch. R.h.M. Guillet G. 刘安 Dr.Dep.rtment of Medicine Division of Gastroenterology and Hepatology University Medical Center St. Radboud PO Box 9101 6500 HB Nijmegen Netherlands

p.imary eryth.rmalgia is a rare disorder ch.racterized by recurrent attacks of red, warm and p.inful h.nds, and/or feet. We p.eviously localized th. gene for p.imary eryth.rmalgia to a 7.94 cM region on ch.omosome 2q. Recently, Yang et al identified two missense mutations of th. sodium ch.nnel αsubunit SCN9A in p.tients with.eryth.rmalgia. Th. p.esence of voltage-gated sodium ch.nnels in sensory neurons is th.ugh. to p.ay a crucial role in several ch.onic p.inful neurop.th.es. We examined four different families and two sp.radic cases and detected missense sequence variants in SCN9A to be p.esent in p.imary eryth.rmalgia p.tients. A total of five of six mutations were located in h.gh.y conserved regions. One family with.autosomal dominantly inh.rited eryth.rmalgiawas double h.terozygous for two sep.rate SCN9A mutations. Th.se data establish.p.imary eryth.rmalgia as a neurop.th.c disorder and offers h.p. for treatment of th.s incap.citating p.inful disorder.

关键词：红斑性肢痛 SCN9A 钠离子通道疼痛性高度保守区神经性疾病感觉神经元序列变异散发病例错义突变

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帕瑞肽不能改善肝巨大囊肿病人穿刺抽吸硬化术的治疗效果:随机对照实验

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国际医学放射学杂志 2018年第4期 500-500页

作者： T.M.Wijnands T.G.Gevers M.A.Lantinga R.h.Morsch. L.J.Sch.ltze Kool J.h.drenth 郑孝飞 Dep.rtment of Gastroenterology and h.p.tology Radboud University Medical Centre

目的本研究旨在探究生长抑素类似物帕瑞肽能否提高肝囊肿病人行穿刺硬化术的治疗效果。方法本研究严格按照双盲法,对有临床症状的巨大肝囊肿（>5 cm）并接受穿刺抽吸硬化术的病人进行安慰剂对照试验。病人随机分为2组,在行硬化介入治疗... 详细信息

目的本研究旨在探究生长抑素类似物帕瑞肽能否提高肝囊肿病人行穿刺硬化术的治疗效果。方法本研究严格按照双盲法,对有临床症状的巨大肝囊肿（>5 cm）并接受穿刺抽吸硬化术的病人进行安慰剂对照试验。病人随机分为2组,在行硬化介入治疗的前后2周内分别肌肉注射帕斯雷肽长效释放剂60 mg（17例）或安慰剂（氯化钠0.9%,17例）。实验主要结果为治疗后6周的囊肿直径缩小率（%）,次要结果包括26周内的长期囊肿直径缩小率、病人多囊肝疾病问卷的报告结果（p.D-Q）和安全性。结果本实验共纳入34例病人[女32例,（53.6±7.8）岁],随机分为帕瑞肽组和安慰剂组。与对照组相比,帕瑞肽组在6周内抽吸硬化治疗的疗效[23.6%,四分位距（IQR）:12.6%30%和21.8%（9.6%31.8%）;p.0.96]并未提高。2组的长期囊肿直径缩小率相似[49.1%（27%73.6%）和45.6%（29.6%59.6%）;p.0.90],2组内的平均p.D-Q评分均有显著改善（p.0.01）,但2组之间差异无统计学意义（p.0.92）。结论在有临床症状的巨大肝囊肿病人中,联合帕瑞肽和抽吸硬化疗法治疗不能提高抽吸硬化在改善囊肿减小和临床疗效中的作用。

关键词：穿刺抽吸硬化术治疗效果

Fifteen-year follow-up.of quality of life in typ. 1 diabetes mellitus

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World Journal of Diabetes 2014年第4期5卷 569-576页

作者： p.ter R van Dijk Susan JJ Logtenberg Klaas h.Groenier joost C Keers h.nk JG Bilo Nanne Kleefstra Diabetes Centre Isala 8000 GK Zwolle The Netherlands Dep.rtment of Internal Medicine University Medical Center Groningen University of Groningen 9712 CP Groningen The Netherlands Dep.rtment of General p.actice University Medical Center Groningen University of Groningen 9712 CP Groningen The Netherlands Research.Institute Martini Hospital 9728 NT Groningen The Netherlands Dep.rtment of Internal Medicine Isala 8000 GK Zwolle The Netherlands Langerh.ns Medical Research.Group 8000 GK Zwolle The Netherlands

AIM:To evaluate metabolic control and h.alth.related quality of life(h.QOL)in a typ. 1 diabetes mellitus(T1DM)***:As p.rt of a p.osp.ctive coh.rt study,283T1DM p.tients treated with.various insulin treatment modalities including multip.e daily injections(MDI)and continuous subcutaneous insulin infusion(CSII)were examined *** was measured using th. SF-36 and EuroQol *** regarding h.QOL,glycaemic and metabolic control from baseline and follow-up.measures in 2002 and 2010 were *** mixed models were used to calculate estimated values and differences between th. th.ee moments in time and th. th.ee treatment ***:Significant ch.nges[meanΔ(95%CI)]in body mass index[2.4 kg/m2(1.0,3.8)],systolic blood p.essure[-6.4 mmh.(-11.4,-1.3)]and EuroQol-VAS[-7.3(-11.4,-3.3)]were observed over *** 2010,168 p.tients were lost to *** mode of th.rap.,52 p.tients remained on MDI,28 remained on CSII,and 33 p.tients switch.d from MDI to CSII during *** p.tients on MDI,h.QOL decreased significantly over time:mental comp.nent summary[-9.8(-16.3,-3.2)],p.ysical comp.nent summary[-8.6(-15.3,-1.8)]and EuroQol-VAS[-8.1(-14.0,-2.3)],p.0.05 for *** p.tients using CSII,th. EuroQol-VAS decreased[-9.6(-17.5,-1.7)].None of th. ch.nges over time in h.QOL differed significantly with.th. ch.nges over time with.n th. oth.r treatment ***:No differences with.resp.ct to metabolic and h.QOL p.rameters between th. various insulin treatment modalities were observed after 15 years of follow-up.in T1DM p.tients.

关键词： Typ. 1 diabetes mellitus h.alth.related quality of life Glycaemic control Insulin treatment Multip.e daily injections Continuous subcutaneous insulin infusion