Ehlers-Danlos syndrome

A case of Ehlers-Danlos Syndrome in a 19 years old male involving cutaneous and skeletal systems is reported. A clinical scoring system to diagnose such cases is highlighted.     

湖南麦饭石卫生学评价

目的　为开发利用湖南 Ｆ６、 Ｆ７ 麦饭石矿物营养的有益资源。方法　毒理学试验,观察实验动物存活、生长状况;生物活性实验,证实麦饭石中微量元素对动植物的营养作用。结果　急性、８０ 天生长试验实际无毒,并能提高小鼠的游泳耐力,延长金鱼存活时间,花卉枯萎时间后移,伤口愈合时间缩短以及吸附水中氰化物、铅、汞、砷、镉和甲醇等有毒物质。结论　麦饭石作为矿物营养资源具有广阔的的应用前景。     

新型抗高血压药物氯沙坦的临床应用

氯沙坦是第一个被研究开发的ＡＮＧⅡ受体拮抗剂。该药口服后吸收良好,生物利用度为３３％ ,大约１４％ 的氯沙坦在体内形成活性更强的羧酸代谢物Ｅ－３１７４。存在体内的ＡＮＧⅡ受体有ＡＴ１、ＡＴ２、ＡＴ３ 三种,其生理效应主要由ＡＴ１ 受体介导发生,可出现强烈的血管收缩及醛固酮释放等多种效应。氯沙坦是非肽类拮抗剂,能高效、特异地与ＡＴ１ 受体结合,有效地抑制ＡＴ１ 受体介导的所有生理效应,发挥舒张血管、降低外周阻力的作用,减少后负荷及交感神经的过度兴奋,有效地治疗高血压病及心力衰竭,同时能够逆转左室肥厚。与其他降压药相比,既无药源性踝部水肿、心率增加的反应,又无频繁咳嗽的不良作用,且临床效果满意、耐受性好,它将在高血压病的治疗中占据显著地位。     

内源性一氧化碳对大鼠主动脉球囊损伤后新生内膜形成及丝裂素活化蛋白激酶活性的影响

目的：探讨内源性血红素加氧酶（ｈｅｍｅｏｘｙｇｅｎａｓｅ,ＨＯ）／ 一氧化碳（ｃａｒｂｏｎ ｍｏｎｏｘｉｄｅ,ＣＯ） 系统对大鼠主动脉球囊损伤后新生内膜形成及丝裂素活化蛋白激酶（ｍｉｔｏｇｅｎａｃｔｉｖａｔｅｄ ｐｒｏｔｅｉｎ ｋｉｎａｓｅ, ＭＡＰＫ） 激活的调节作用。方法：采用大鼠主动脉内膜球囊拉伤模型,观察ＨＯ 抑制剂锌卟啉９（ｚｉｎｃ ｐｒｏｔｏｐｏｒｐｈｙｒｉｎＩＸ,ＺｎＰＰ９）或其底物血红素左旋赖氨酸盐（ｈｅｍｅＬｌｙｓｉｎａｔｅ,ＨＬＬ）对血管壁细胞３ＨＴｄＲ掺入和ＭＡＰＫ 活性的影响,同时观察血管平滑肌ＨＯ活性和ＣＯ 生成的变化。结果：内膜拉伤后２ 周血管３ＨＴｄＲ 掺入和ＭＡＰＫ 活性明显增加,同时ＨＯ 活性和ＣＯ 的产生增加;ＺｎＰＰ９ 使血管的３ＨＴｄＲ掺入和ＭＡＰＫ 活性增加更为明显（ 分别比单独拉伤组增加３４ ．６％ 和３９．２％ ,均为Ｐ＜ ０．０１） ;而ＨＬＬ 使血管的３ＨＴｄＲ 掺入和ＭＡＰＫ 活性则明显降低（ 比单独拉伤组分别减少２９．４５ ％ 和３３．６ ％ ,均为Ｐ＜ ０．０１） 。结论：ＨＯ 活性上调或ＣＯ 产生增加是血管对机械拉伤的一种保护性应激反应;内源性ＨＯ／ＣＯ 系统直接     

强直性脊柱炎患者的食管运动功能

研究诱导型一氧化氮合酶 （ｉｎｄｕｃｉｂｌｅ ｎｉｔｒｉｃ 　ｏｘｉｄｅ ｓｙｎｔｈａｓｅ, ｉＮＯＳ）在电离辐射导致的神经细胞凋亡中的作用。 方法：以单剂量２．０ Ｇｙ γ射线照射出生后 ０ ｄ的 Ｗｉｓｔａｒ大鼠,用 ＡＢＣ法检测照射后第 ０．５、１、２、４、６、８、１２、２４、４８小时大脑皮质内ｉＮＯＳ的表达。结果：照射组各时间点均有不同程度的ｉＮＯＳ表达,第６小时达峰值。各对照组未见ｉＮＯＳ表达。结论：ｉＮＯＳ所具有的神经毒作用可能是电离辐射导致的发育中脑神经细胞凋亡的重要因素。     

聚丙烯酰胺凝胶电泳分析615纯系小鼠血清蛋白

目的：根据各种蛋白质在一定的泳动条件下,都有一定的相对迁移率不同的特点,分析６１５ 纯系小鼠血清中的蛋白质在聚丙烯酰胺凝胶上的分布,进行了定位分析。方法：采用高ｐＨ 不连续性聚丙烯酰胺凝胶电泳及蛋白质特殊染色法。结果：６１５ 鼠血清蛋白质在聚丙烯酰胺凝胶上可分离出１３ ～１５ 条区带,脂蛋白３ 条、白蛋白２ 条、糖蛋白５ 条、铜蓝蛋白１ 条、血红蛋白１ 条、结合珠蛋白３ 条。根据每种蛋白质的Ｒｍ 值定位顺序从阳极到阴极依次为１ ．糖蛋白、２．脂蛋白、３ ．白蛋白、４．脂蛋白、５ ．糖蛋白、６ ．巨球蛋白（ 根据其它资料） 、７ ．铜蓝蛋白、８ ．结合珠蛋白、９ ．Ｈｂ、１０ ．运铁蛋白（ 根据其它资料）、１１ ．脂蛋白、１２ ～１４ ．均为糖蛋白。光密度扫描６１５ 鼠血清蛋白质相对面积比分别为白蛋白为４８－４、α１ 球蛋白为６－９、α２ 球蛋白２３－８ 、β球蛋白为５－０ 、γ球蛋白为１３－８ 。结论：６１５ 鼠血清蛋白质的定位分析对于６１５ 鼠可移植性淋巴细胞型白血病鼠的蛋白质研究具有一定的实际意义,利用Ｒｍ 值来作蛋白质定位分析简单易行,关键在于掌握实验条件的恒定。     

nm23-H_1基因与突变型P~(53)基因在人乳腺病变组织中的表达

目的观察７６例乳腺病变组织中ｎｍ２３－Ｈ１及突变型Ｐ５３基因的表达情况,分析其与乳腺癌生物学行为及淋巴结转移的关系。方法应用ＳＰ免疫组织化学方法检测７６例乳腺病变组织中ｎｍ２３－Ｈ１及突变型Ｐ５３蛋白表达。结果ｎｍ２３－Ｈ１基因在５６例乳腺癌中表达率为４４．６４％,１０例乳腺纤维瘤均阳性,１０例纤维囊性病９例阳性,后二者均高于乳腺癌中阳性率（Ｐ＜０．００５,Ｐ＜０．０１）;Ｐ５３在乳腺癌中阳性率３５．７１％,在所有良性乳腺疾患中均阴性（Ｐ＜０．０２５）。同时发现ｎｍ２３－Ｈ１表达与乳腺癌组织分化程度及淋巴结转移呈负相关（Ｐ＜０．０２５,Ｐ＜０．０５）,与病理组织类型无关;Ｐ５３表达与乳腺癌淋巴转移正相关（Ｐ＜０．０５）,与组织类型及分化程度无关;ｎｍ２３－Ｈ１与Ｐ５３表达呈负相关（Ｐ＜０．０５）;另外,ｎｍ２３－Ｈ１表达缺失与Ｐ５３的阳性表达在乳腺癌淋巴结转移上有协同作用。结论ｎｍ２３－Ｈ１的表达缺失及Ｐ５３的阳性表达提示乳腺肿瘤恶性度高、淋巴转移潜能大,二者联合检测对乳腺癌患者预后的评价更为全面可靠。     

The Bournewood judgment: a way forward?

In this article we provide a commentary on the various reasonings behind the Law Lords' unanimous judgements in their recent decision (25 June 1998) in Regina v. Bournewood Community and Mental Health NHS Trust, Ex parte L. After summarizing the judgment and commenting on its important implications, we suggest a way forward. The 1995 Law Commission incapacity proposals, on which the 1997 consultation paper Who Decides? was based, do afford a mechanism which could resolve much of the practical difficulty which practitioners feared from the Appeal Court decision, whilst at the same time affording the sorts of rights which the House of Lords decision denies.     

Oesophagogastrectomy in the elderly high risk patients: role of effective regional analgesia and early mobilisation

BACKGROUND: Oesophagogastrectomy is the best available treatment for patients with carcinoma of the oesophagus or cardia. However, surgical resection may lead to increased mortality, morbidity and longer hospital stays in elderly (aged over 70 years) high risk patients. METHODS: To assess the impact of balanced pre-emptive and postoperative analgesia combined with early mobilisation in elderly patients undergoing oesophagogastrectomy we consecutively studied 52 patients (30 male, 22 female) of 75+/-4.2 years of age (mean+/-SD). Pre-emptive analgesia was by pre-incisional percutaneous paravertebral block combined with an opiate and a nonsteroidal anti-inflammatory drug (NSAID) premedication. Postoperative maintenance analgesia was by NSAID and continuous extrapleural intercostal nerve block. Following surgery all but three patients were returned to the ward. RESULTS: The hospital mortality rate was 7.6%. Morbidity caused by cardiovascular (27%), respiratory (23%) and cerebrovascular (19%) complications occurred in 19 patients, with two patients requiring ventilatory support. The mean hospital stay for the survivors was 10 days (range 8 to 30 days). All the survivors had their swallowing restored to normal and returned to their accustomed environment. CONCLUSIONS: These data suggests that surgical treatment can be achieved in the elderly high risk patients with acceptable mortality and morbidity. This is achieved by early mobilisation enabled by balanced pre-emptive and postoperative analgesia.