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i.fluence of Fri.ti.n Dri.e Li.t Gears Constructi.n on the Length of Braki.g Di.tance

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Chi.ese Journal of Mechani.al Engi.eeri.g 2015年第2期28卷 363-368页

作者： LOONKWi. Poul Luubli. Factory of Passenger Li.ts Li.t serv.vi.e S. A. Lublli.

The fri.ti.n dri.e elevators the i.fluence of the braki.g di.tance has very hi.h si.ni.i.ance to meet certai. safety regulati.ns and *** the emergency braki.g the delay for the system a frame and a cabi. should be wi.hi. the range from 0.2 to 9.81 m/s~***,there are no speci.li.t li.eratures regardi.g the i.sues connected wi.h emergency braki.g of elevati.g devi.es *** results of the own empi.i.al research work are presented regardi.g the i.fluence of desi.n changes on the worki.g parameters of the fri.ti.n dri.e elevator ***100,KB 160,PP16,PR2000UD and CHP2000 types of safety progressi.e gears are ***100,KB 160,PP16,PR2000UD type progressi.e gears are already produced by European ***2000 type gears are establi.hed as the alternati.e opti.n for the already exi.ti.g *** uni.ue cam system has been used i. the CHP 2000 *** cam leverage gi.es the chance to unblock,i. a very easy way,the clamed gears after ***,i. i. a key aspect to perform laboratory tests over the braki.g process of a newly created *** proper value of the braki.g di.tance has a si.ni.i.ant i.fluence on the value of delay i. terms of bi.di.g *** i.fluence of loadi.g on the effecti.e braki.g di.tance and the value of the falli.g elevator cabi. speed are analyzed and the results are *** results presented are i.teresti.g from li.t devi.es operati.n and a new model of CHP 2000progressi.e gear poi.t of vi.w.

关键词： li.t gears fri.ti.n dri.e progressi.e safety gears braki.g di.tance emergency braki.g

Prader-Wi.li.Syndrome:16-Year Experi.nce i. Hong Kong

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Journal of Geneti.s and Genomi.s 2012年第4期39卷 191-194页

作者： i.an F.M. Lo Ho Mi.g Luk luksi.e@*** Tony M.F. Tong Kent K.S. Lai.Dani.l H.C. Chan Albert C.F. Lam Davi. K.H. Chan Edgar W.L. Hau Conni. O.Y. Fung Stephen T.S. Lam Cli. Genet serv Dept Hlth Kowloon Hong Kong Peoples R China

Prader-Wi.li.syndrome （PWS） i. an i.portant, well-recogni.ed syndromi. form of neurodevelopmental di.order. The i.ci.ence i. about 1 i. 15,000-25,000 li.e bi.ths, and i. affects both males and females （Vogels et al... 详细信息

关键词： PWS Prader-Wi.li.Syndrome

Δ~1-吡咯啉-5-羧酸合酶缺乏:神经变性、白内障以及结缔组织病表现并伴有高氨血症和鸟氨酸、瓜氨酸、精氨酸、脯氨酸减少

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世界核心医学期刊文摘（儿科学分册） 2005年第6期1卷 30-31页

作者： Baumgartner M. R. Rabi.r D. Nassogne M.-C. J. -M. Saudubray 王一飞 serv. des Maladi.s Metaboli.ues Hpital Necker-Enfants Malades 75743 Paris France

Δ1-pyrroli.e-5-carboxylate synthase （P5CS） catalyses the reducti.n of glutamate to Δ1-pyrroli.e-5-carboxylate, a cri.i.al step i. the bi.synthesi. of proli.e, orni.hi.e and argi.i.e. Recently, we reported a newly recogni.ed i.born error due to defi.i.ncy of P5CS i. two si.s, one presenti.g at bi.th wi.h hypotoni., dysmorphi. si.ns, pes planus and cloni. sei.ures. Both developed progressi.e neurodegenerati.n and peri.heral neuropathy, joi.t laxi.y, ski. hyperelasti.i.y and bi.ateral subcapsular cataracts. Thei. metaboli. phenotype i.cludes mi.d hyperammonaemi., hypo-orni.hi.aemi., hypoci.rulli.aemi., hypo-argi.i.ae- mi. and hypoproli.aemi.. i.corporati.n of 3Hproli.e i.to protei. was defi.i.nt i. fi.roblasts i.cubated wi.h 3H-glutamate. Both pati.nts are homozygous for the mi.sense mutati.n R84Q i. P5CS. Here, we descri.e the cli.i.al phenotype of the si.s i. detai. and show that a relati.e defi.i.ncy of urea cycle i.termedi.tes （orni.hi.e, ci.rulli.e and argi.i.e） duri.g fasti.g peri.ds results i. a paradoxi.al hyperammonaemi.. Furthermore, we show the results of orni.hi.e loadi.g tests and i.di.ect enzyme studi.s corroborati.g the bi.logi.al si.ni.i.cance of the defect i. P5CS i. vi.o. Conclusi.n: Themetaboli. phenotype of Δ1-pyrroli.e-5-carboxylate synthase defi.i.ncy i. easi.y mi.sed. The combi.ati.n of low levels of orni.hi.e, ci.rulli.e, argi.i.e and proli.e plus a tendency to hyperammonaemi. or one of the above together wi.h a cli.i.al phenotype of neurodegenerati.n wi.h peri.heral neuropathy and/or cataracts and connecti.e ti.sue mani.estati.ns should suggest thi. di.order. Early recogni.i.n would allow a therapeuti. tri.l wi.h ci.rulli.e and proli.e.

关键词：瓜氨酸血症神经变性鸟氨酸精氨酸血症结缔组织病合酶脯氨酸吡咯啉阵挛性惊厥尿素循环

The HY-1 satelli.e and ground system i. Chi.a

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Acta Oceanologi.a Si.i.a 2003年第2期22卷 301-308页

作者： Li. Ji.nqi.ng , Ji.ng Xi.gwei. Li. Mi.gsen Nati.nal Satelli.e Ocean Appli.ati.n serv.ce of State Oceani. Admi.i.trati.n, Bei.i.g 100081, Chi.a, State Ocean Adm Natl Satellite Ocean Applicat Serv Beijing 100081 Peoples R China

i.TRODUCTi.NWi.h the comi.g of the 21st century, we are faced the problems of the envi.onment, re-sources and populati.n. The ocean i. regarded as suppli.r of resources such as food, mi.erals,energy and space, and plays an i.portant role i. sustai.able economi. development. At thesame ti.e the ocean has a very i.portant effect on the worldwi.e envi.onmental changes.

关键词： HY-1 satelli.e ground system Chi.a

儿童假单胞菌性小汗腺炎提示急性淋巴细胞白血病(法语)

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世界核心医学期刊文摘（皮肤病学分册） 2005年第4期1卷 48-49页

作者： Laffi.te E. Hohl D. Pani.zon R.-G. 刘超 serv. de Dermatologi. et Venerologi. Ctr. Hosp. Universitaire Vaudois CH-1011 Lausanne Switzerland

i.troducti.n. We report the case of a Pseudomonas (P.) aerugi.osa eccri.e hi.radeni.i. i. a chi.d, or a "Pseudomonas Hot Foot Syndrome", reveali.g an acute lymphoblasti. leukemi.. Observ.ti.n. A 10 year-old gi.l consulted for the sudden onset of pai.ful and necroti. palmoplantar nodules i. a context of fever and shi.eri.g. Hi.tology of a cutaneous bi.psy found necrosi. of the eccri.e glands and, on culture, P. aerugi.osa. The blood count revealed pancytopeni. and the myelogram acute lymphoblasti. leukemi.. All the hemocultures and other mi.robi.logi.al samples were negati.e. The cutaneous si.ns had appeared 48 hours after bathi.g i. an aquati. amusement park. Di.gnosi. of Pseudomonas eccri.e hi.radeni.i.,or"Pseudomonas Hot Foot Syndrome"was retai.ed, although the local sani.ary authori.i.s were not able to demonstrate P. aerugi.osa contami.ati.n of the water i. the park. Comments. Lesi.ns evoki.g juveni.e Pseudomonas aerugi.osa eccri.e hi.radeni.i. wi.hout obvi.us traumati. factor must lead to the search for P. aerugi.osa contami.ati.n from water and the subsequent sani.ary and epi.emi.logi.al consequences. Furthermore, severe P. aerugi.osa cutaneous i.fecti.ns i. chi.dren must also lead to the search for an underlyi.g i.munosuppressi.n and notably an acute leukemi. process.

关键词：假单胞菌急性白血病造影片淋巴母细胞小汗腺坏死性结节铜绿色全血细胞减少掌跖流行病学方面

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黑色人种鳞状细胞癌患者:80例(法语)

世界核心医学期刊文摘（皮肤病学分册） 2005年第4期1卷 51-52页

作者： Di.ng M.-T. Di.p N.-N. Déme A. 刘艳 serv. de Dermatol. et de Venereol. CHU le Dantec BP 3001 Dakar Senegal

i.troducti.n. Squamous cell carci.oma i. a mali.nant epi.heli.l tumor of the ski., rarely observ.d i. "black"ski.. i. often compli.ates an acqui.ed or congeni.al pre-neoplasti. dermatosi.. We report on 80 cases of squamous cell carci.oma havi.g occurred i. black Afri.an pati.nts from Senegal. Pati.nts and methods. We retrospecti.ely analyzed all the medi.al fi.es of pati.nts followed-up i. the department for hi.tologi.ally confi.med squamous cell carci.oma from 1980 to 1999. Results. Ei.hty cases of carci.oma were collected. The mean age of the pati.nts was of 47 years (range: 1 to 85) and the sex rati. was of 1.35 (46 men and 34 women). A pre-neoplasti. dermatosi. was i.enti.i.d i. 65%of the pati.nts. Acqui.ed pre-neoplasti. dermatosi. was: heat burn scars (15 cases), chroni. di.coi. lupus erythematosus (6 cases) and chroni. phagedeni. ulcer (4 cases). Pre-neoplasti. genodermatoses were: albi.i.m (6 cases), xeroderma pi.mentosum (5 cases) and epi.ermodysplasi. verruci.ormi. (1 case). Lesi.ns were multi.le and cephali. i. pati.nts wi.h genodermatosi.. Death due to carci.oma occurred i. 8 pati.nts. Comments. Thi. large seri.s of squamous cell carci.oma emphasi.es: the rari.y of squamous cell carci.omas i. black pati.nts and the frequency of i.s occurrence on acqui.ed pre-exi.ti.g dermatosi.. For many years phagedeni. ulcers predomi.ated. Our results show the regressi.n of the latter and i.s i.crease on post-burn scars and chroni. lupus erythematosus. Pre-neoplasti. genodermatoses thatdetermi.emulti.lecancers,usuallylocatedonsun-exposed areas and occurri.g i. young pati.nts, are parti.ularly severe.

关键词：黑色人种鳞状细胞癌癌前皮肤病恶性上皮瘤崩蚀性溃疡烧伤瘢痕鳞癌患者遗传性皮肤病着色性干皮病医学领域

胆管闭锁症成年期的预后:对63例依靠自身肝脏存活20年以上的患者的研究

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世界核心医学期刊文摘（胃肠病学分册） 2005年第6期1卷 51-52页

作者： Lykavi.ri. P. Chardot C. Sokhn M. 杨瑗 serv. d’Hepatologi. Pedi.t. Hpital de Bicêtre 94275 Le Kremlin-Bicêtre France

To defi.e the long-term prognosi. of chi.dren undergoi.g the Kasai.operati.n for bi.i.ry atresi., a retrospecti.e study was undertaken compri.i.g 271 pati.nts operated between 1968 and 1983. Twenty years after surgery, 63 (23%) were ali.e wi.h thei. nati.e li.er. Serum bi.i.ubi. was normal i. 21 of these pati.nts, 12 also had normal serum ami.otransferase and γ-glutamyltransferase acti.i.i.s, all but 2 had si.ns of ci.rhosi., 44 had si.ns of portal hypertensi.n, 19 had late bacteri.l cholangi.i., and 6 had gallstones. Seven female pati.nts gave bi.th to 9 chi.dren, and 3 male pati.nts fathered 6 chi.dren. After age 20, 2 pati.nts di.d of li.er fai.ure and 14 underwent or are awai.i.g li.er transplantati.n. Twenty-year survi.al wi.h nati.e li.er was si.ni.i.antly better i. chi.dren wi.h bi.i.ry atresi. restri.ted to the hepati. ducts or wi.h cysts at the porta hepati.. i. conclusi.n, i. the long term, less than 18%of i.fants wi.h bi.i.ry atresi. who are treated wi.h correcti.e surgery may avoi. li.er transplantati.n, but even these pati.nts requi.e assi.uous li.elong care.

关键词：胆管闭锁肝衰竭门脉高压肝移植血清转氨酶肝管肝门

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胰腺转移癌:对22例患者的多中心研究

世界核心医学期刊文摘（胃肠病学分册） 2005年第3期1卷 29-30页

作者： Moussa A. Mi.ry E. Hammel P. G. Lesur 赵天智 serv. Hepato Gastroenterol./O. D. H.opital Ambroise Paré 92104 Boulogne Billancourt France

Ai.s of the study To evaluate the di.gnosi., treatment and outcome of pati.nts wi.h pancreati. metastases. Pati.nts and methods We retrospecti.ely revi.wed the records of pati.nts wi.h pancreati. metastasi. managed i. the Pari. area between 1990 and 2000. Results The seri.s analyzed i.cluded 22 pati.nts, 10 men and 12 women, mean age 61 years (range: 35-76). The pri.ary tumors were renal cell carci.oma (N = 10), colorectal cancer (N = 4), lung cancer (N = 4), breast cancer (N = 2), cutaneous melanoma (N = 1) and i.eal carci.oi. (N = 1). The mean i.terval between pri.ary treatment and presentati.n was 73.5 months (range: 2-151). Di.gnosi. was establi.hed because of cli.i.al symptoms (N = 15) or duri.g survei.lance (N = 7). Computed tomography (N = 19) and endoscopi. ultrasound (EUS) (N= 18) mai.ly showed soli.ary and hypodense/or hypoechoi. masses. Hi.tologi.al di.gnosi. was obtai.ed before surgery by EUS gui.ed fi.e needle aspi.ati.n(N =6), ultra sound gui.ed bi.psy (N = 3) or duodenoscopy (N = 3). Among 10 pati.nts wi.h pri.ary renal cell carci.oma, 7 were treated by surgery. Medi.n global survi.al was 33 months. Medi.n survi.al was 61 months i. the event of surgi.al treatment and 20 months i. the other pati.nts (ns). Mean survi.al depended on the type of pri.ary tumor, 61 months for renal cell carci.oma and 33 for colorectal cancer (P = 0.06). Conclusi.ns Most pancreati. metastases develop from renal cell carci.oma and can occur several years after nephrectomy. Hi.tologi.al di.gnosi. i. often obtai.ed before surgery. Surgi.al resecti.n must be di.cussed as i. can allow long term survi.al.

关键词：肾细胞癌结直肠癌原发肿瘤平均生存期手术切除术外科手术患者组织学诊断平均间隔低回声肿块黑色素瘤

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注意缺陷多动障碍儿童的铁缺乏

世界核心医学期刊文摘（儿科学分册） 2005年第6期1卷 21-22页

作者： Konofal E. Lecendreux M. Arnulf i. Mauren M.-C. 郭战宏 serv. Psychopathol. Enfant/Adol. Hpital RobertDebré 48 Boulevard Serurier 75020 Paris France Dr.

Background: i.on defi.i.ncy causes abnormal dopami.ergi. neurotransmi.si.n and may contri.ute to the physi.pathology of attenti.n-defi.i./hyperacti.i.y di.order (ADHD). Objecti.e: To evaluate i.on defi.i.ncy i. chi.dren wi.h ADHD vs i.on defi.i.ncy i. an age-and sex-matched control group. Desi.n: Controlled group compari.on study. Setti.g: Chi.d and Adolescent Psychopathology Department i. European Pedi.tri. Hospi.al, Pari., France Pati.nts: Fi.ty-three chi.dren wi.h ADHD aged 4 to 14 years (mean ±SD, 9.2 ±2.2 years) and 27 controls (mean ±SD, 9.5 ±2.8 years). Mai. Outcome Measures: Serumferri.i. levels evaluati.g i.on stores and Conners’Parent Rati.g Scale scores measuri.g severi.y of ADHD symptoms have been obtai.ed. Results: The mean serum ferri.i. levels were lower i. the chi.dren wi.h ADHD (mean ±SD, 23 ±13 ng/mL) than i. the controls (mean ±SD, 44 ±22 ng/mL; P i.i. levels were abnormal (i. 84%of chi.dren wi.h ADHD and 18%of controls (P i. addi.i.n, low serum ferri.i. levels were correlated wi.h more severe general ADHD symptoms measured wi.h Conners’Parent Rati.g Scale (Pearson correlati.n coeffi.i.nt, r = -0.34; P i.i.e defi.i.s (r = -0.38; P i.ns: These results suggest that low i.on stores contri.ute to ADHD and that ADHD chi.drenmay benefi. from i.on supplementati.n.

关键词：铁缺乏血清铁蛋白缺铁多巴胺能病理生理机制认知障碍症状严重程度儿童医院性别匹配青少年心理