Haemophilia A and haemophilia B: molecular insights

This review focuses on selected areas that should interest both the scientist and the clinician alike: polymorphisms within the factor VIII and factor IX genes, their linkage, and their ethnic variation; a general assessment of mutations within both genes and a detailed inspection of the molecular pathology of certain mutations to illustrate the diverse cause–effect relations that exist; a summary of current knowledge on molecular aspects of inhibitor production; and an introduction to the new areas of factor VIII and factor IX catabolism. An appendix defining various terms encountered in the molecular genetics of the haemophilias is included, together with an appendix providing accession numbers and locus identification links for accessing gene and sequence information in the international nucleic acid databases.     

拆方研究补中益气汤中黄芪 甘草及全方的免疫调节作用

目的　检测补中益气汤中黄芪、甘草及全方对病态机体的免疫调节作用。方法　分 7组检测全方中黄芪、甘草及全方缺黄芪、缺甘草组方对利血平制备的脾虚小鼠的 5项非特异性免疫功能的影响进行研究。结果　黄芪能使Hb明显升高 ,单味甘草对某些免疫功能不起作用甚至起抑制作用 ,缺甘草组方作用低于全方 ,全方能明显提高机体免疫功能。结论　黄芪是方中主要升高Hb药物 ;方中甘草起到特殊的调节作用。     

颞下颌关节紊乱综合征(?)与龋因素的流行病学研究

目的:为探讨(?)因素、龋因素与颞下颌关节紊乱综合征(TMJDS)致病关系和作用机理。方法:以东北电力学院入学新生为研究对象。采用TMJDS、龋诊断标准和安氏分类等方法,对1065名大学生进行流行病学研究。结果:正常(?)TMJDS阳性率为6.81%,异常(?)TMJDS阳性率为48.32%,具有显著差异性。无龋者TMJDS阳性率为23.72%,龋病者TMJDS阳性率为39.58%,具有显著差异性。结论:异常(?)、龋病是TMJDS的致病因素之一。     

急性脑血管病肿瘤坏死因子-α的临床研究

目的：探讨肿瘤坏死因子-α（TNF-α）在急性脑血管病（ACVD）中的作用及其变化的临床意义。方法：选取95例ACVD患者，并设性别及年龄相匹配的血清对照组（20例）及脑脊液对照组（10例）。应用酶联免疫吸附法（ELISA）于发病后第1、3、7、14d测定血清和脑脊液中TNF-α。结果：①三种脑血管病患者血清TNF-α动态变化存在差异，脑出血和脑梗死组的峰值单间为发病后第3d，而蛛网膜下腔出血（SAH）组为第1d。②脑梗死患者血清TNF-α水平与梗死面积、神经功能缺损程度及病情恶化与否相关。③SAH组于发病后第1d、3d、7d脑脊液TNF-α值高于血清，14d时降至对照组水平并与血清无差异。结论：①脑梗死患者血清TNF-α的动态观察，可为临床预测梗死灶大小、神经功能缺损程度及病情恶化与否提供依据。②发病早期的脑梗死患者若CT未能显示病灶时，血清TNF-α的明显升高有助于临床诊断和治疗。③SAH患者脑脊液中TNF-α水平明显高于血清，进一步提示脑内神经组织可产生TNF-α。脑脊液中TNF-α的持续明显升高可能与SAH后脑血管痉挛有关。④TNF-α参与了ACVD的炎性反应过程，早期抑制TNF产生及抗炎性反应的治疗可能具有潜在的临床价值。     

Multidrug transport in human glioblastoma cells is inhibited by transforming growth factors type beta 1, beta 2, and beta 1.2

The transforming growth factors type beta 1, beta 2, and beta 1.2 suppress multidrug transport in human pat-1 glioblastoma cells and even in cells that strongly over-express mdr genes and are resistant to inhibition of multidrug transport by chemosensitizers. Thus, inhibition of multidrug transport by cytokines might be a new approach to increase cellular accumulation of chemotherapeutic agents in multidrug resistant glial tumor cells. Interestingly, a member of the more distantly related decapentaplegic subgroup of transforming growth factors, the bone morphogenetic protein BMP 2, did not inhibit multidrug transport.     

Sources of stress in Australian adolescents: factor structure and stability over time

Adolescent stress is an emerging area of importance in considerations of the health of young people. Exposure to stress predicts a range of both physical and mental health problems in adolescents and relates, as well, to the initiation of important health risk behaviours. Yet the measurement of stress in adolescents has been as fraught with methodological difficulty as it has been for adults. This paper examines the Adolescent Stress Questionnaire (ASQ) which was developed specifically to address the domain of stressors specific to adolescent experience and looks at three cohorts of responses to this questionnaire over time (1995, 1998 and 2001). It assesses the stability of the original 7‐factor structure of the ASQ over time, and while confirmatory factor analysis indicates that stability to be acceptable, it also suggests that either adolescent stressors themselves, or the language by which they are reported, vary sufficiently over time to warrant the refinement of the instrument. Copyright © 2002 John Wiley & Sons, Ltd.     

bFGF对人毛囊黑素细胞增殖、活化和黑素合成作用的研究

目的　研究碱性成纤维细胞生长因子 (bFGF)对人毛囊黑素细胞 (Hairfolloclemelanocyte ,HFM )增殖活化和黑素合成的影响。方法　体外培养正常人HFM ,观察不同浓度bFGF(0 3～ 1 2ng ml)对HFM形态、增殖、酪氨酸酶活性和黑素含量的影响。结果　bFGF处理HFM 7d后 ,HFM树突增多延长 ,酪氨酸酶活性和黑素含量增加显著高于对照组 (P <0 0 1) ,以 0 6ng ml组为著。结论　bFGF能诱导HFM增殖、酪氨酸酶活性和黑素合成增加     

8—2岁市区学龄儿童感觉统合失调与不良行为及家庭因素调查分析

目的：调查本市区小学儿童感觉统合失调现状与不良行为及家庭因素的关系。方法：用感觉统合诊断量表和自制儿童生长发育调查表对1775名学龄儿童进行心理行为问题调查。结果：感觉统合轻度失调23．4％，重度失调13．5％，男童失调发和率（42．86％）明显高于女童（31．36％）P＜0．0），前庭功能失衡发生率最高（21．30％）；统合失调组儿童不良行为发生率高达64．38％明显高于统合正常组（31．11％）P＜0．01，失调组不良行为各因子的发生率亦明显高于统合正常组P＜0．01；学习能力不足儿童与父母文化程度和不同职业有关P＜0．01。结论：儿童感觉纺合失调现状易成为成人心理障碍的潜在因素，应引起重视。     

抑制脑血栓形成的硫代磷酸酯脱氧寡核苷酸的亲和性研究

目的为抑制脑血栓形成,合成与TF基因启动子区切应力反应元件(SSRE)形成三链DNA的硫代磷酸酯寡核苷酸(TFO)。方法设计TFO序列14条,采用固相亚磷酰胺三酯固相法合成TFO。硫代磷酸酯修饰在TFO的3'末端进行。应用电泳迁移分析(EMSA)观察寡核苷酸和硫代脱氧寡核苷酸的亲和性。结果在设计合成的14条寡核苷酸中,与靶序列能形成三链DNA的TFO只有T21GTa、T14GTa和T15GTa,其Kd值分别为3.6×10-10、1.0×10-9和1.0×10-8(M),经硫代磷酸酯修饰后分别为:2.3×10-9、3.8×10-9和1.5×10-8。结论硫代磷酸酯修饰的T21GTa-ps、T14GTa-ps和T15GTa-ps能够与TF基因启动子SSRE的3个位点形成三链DNA。