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.     

石杉碱甲和乙促进小鼠的空间辨别学习和记忆

石杉碱甲和乙是从石杉科石杉属植物蛇足石杉[Huperzia scrrata(Thunb.)Trev.]中分得的二个新生物碱。“Y”迷宫实验表明,ip Hup-A 0.075～0.125 mg/kg或Hup-B 0.4～0.8mg/kg,均能明显促进小鼠的空间辨别学习,并能显著预防CO₂产生的短时识别障碍,促进记忆保持和记忆再现。ig Hup-A 0.1～0.3 mg/kg或Hup-B 0.8 mg/kg也有促进学习的作用。促进作用Hup-A>Phys>Hup-B。剂量与效应曲线呈倒U型。     

Ethical aspects of withdrawing/withholding renal replacement therapies on patients in acute renal failure in an intensive care unit

The majority of patients being treated for acute renal failure in intensive care units have multiple medical problems. Accordingly, the withdrawal of renal replacement therapies should be considered as part of a general decision about whether to initiate or continue with treatment per se. Several guidelines on withdrawing and withholding therapy have been produced and some common themes emerge: concerns to avoid euthanasia, potential for benefit, patient consent (shared decision‐making), team consensus/decision‐making, and the provision of appropriate palliative care and resource implications. Each of these is considered in turn, although the word limit for this paper does not permit detailed exposition.     

Active production of anti-human T-lymphotropic virus type I (HTLV-I) IgM antibody in HTLV-I-associated myelopathy

We investigated the presence of anti-human T-lymphotropic virus type I (HTLV-I) IgM in sera and cerebrospinal fluid from patients with HTLV-I-associated myelopathy (HAM) by Western blot analysis. Analyses of 36 serum samples revealed that most patients (31/36; 86.1%) had anti-HTLV-I IgM, whereas only four of 23 (17.4%) HTLV-I carriers had it. In studies of cerebrospinal fluid, anti-HTLV-I IgM was detected in 24 of 36 (66.7%) HAM patients, whereas none was detected in nine HTLV-I carriers. The differences were statistically significant (p less than 0.01). These results suggest that persistent active replication of HTLV-I occurs in the central nervous system as well as in the peripheral blood of HAM patients, and may contribute to the development of HAM.     

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.     

ABO血型与肝炎病毒感染关系的探讨

目的：探讨ABO血型与肝炎病毒感染的关系，方法：应用疾病关联分析方法对HBsAg和,抗－HCV阳性献血者与同期健康献血者的ABO血型资料进行比较分析。结果：ABO血型与HBsAg和抗－HCV阳性率之间无显著性相关关系（P＞0．05），结论：乙型肝炎和丙型肝炎病毒感染与ABO血型无显著性相关。     

青年急性冠脉综合征冠脉造特点及随访

目的:探讨青年急性冠状动脉综合征(ACS)冠状动脉(冠状)造影特点及其与远期心脏事件的关系。方法:比较青年ACS与老年ACS患者冠脉病变程度、范围,并进行心脏事件随访。结果:78例青年ACS患者有冠脉病变74例(94.9％),单支病变46例(58.9％);171例老年ACS患者有冠脉病变166例(97.1％),单支病变41例(23.9％)。平均随访9个月,青年ACS患者发生心脏事件者10例(12.8％),其中心绞痛再入院6例(7.7％);老年ACS患者发生心脏事件者47例(27.5％),其中心绞痛再入院30例(17.5％),两组比较差异有显著性(P<0.05)。结论:ACS患者冠状动脉粥样硬化青年时期之前已经发生,ACS预后与冠脉病变程度和年龄呈正相关。     

窒息鼠脑组织型纤溶酶原激活物活性变化与脑水肿的关系

目的：探讨窒息对鼠脑分泌组织型纤溶酶原激活物（TPA）的影响与脑水肿的关系。方法：通过“延迟剖宫产术”致胎鼠宫内窘迫，实验分空白对照组，窒息15min组，窒息30min组，窒息15min复氧30min组，窒息30min复氧30min五个实验组，每组各取8例测试脑组织TAP的活性及含水量，结果：窒息后鼠脑TPA活性与含水量均升高（P＜0．01），结论：窒息可致TAP活性增高，同时发生脑水肿，高活性的TPA可能是脑缺氧缺血致不可逆神经元损伤的一个重要媒介。     

Epidemiology of acute diarrhea in children living in Sicily

A longitudinal survey (February 1984 - January 1985) on the incidence of acute diarrhea episodes in a sample of 8,164 children (aged 0–12 years) living in southeastern Sicily was carried out by means of weekly telephone interviews with doctors practising in the territory. The incidence rate was 0.479 (0.472–0.485 95% C.I.) per child per year and the frequency of episodes was significantly higher (p < 0.001) in children aged 0–4 years (0.86). Diarrhea was more frequent in industrial areas than in rural ones, and almost half (45.1%) of the total episodes had a mild course. No death from diarrhea occurred and admission to hospital was reported for 8.4% of all cases.Corresponding author.     

完全性左束支传导阻滞伴快速心房纤颤并急性左心衰竭的抢救

目的总结完全性左束支传导阻滞伴快速心房纤颤并急性左心衰竭的治疗经验,以提高抢救成功率.方法对经抢救治疗的完全性左束支传导阻滞伴快速心房纤颤并急性左心衰竭9例(16次)患者进行治疗方面的回顾分析.结果9例(16次)患者14次抢救成功,成功率87.0%,但复发率较高,预后较差,有5例患者在抢救时或出院后1～2年内猝死.结论该组患者经传统"强心、利尿、扩血管",控制心房纤颤、心室率等治疗是远远不够的.应在未发生肺泡性肺水肿及心源性休克之前,尽早尽快地应用血管扩张剂及联合应用小剂量非洋地黄类正性肌力药物,并维持治疗24～72 h.其中以酚妥拉明加多巴胺和(或)多巴酚丁胺效果可能较佳,血管扩张剂硝酸酯类不及α-受体阻滞剂酚妥拉明疗效明显.