Transplantation of an eight-organ multivisceraI graft in a patient with frozen abdomen after complicated Crohn's disease

To report an extended multivisceral transplantation (MVTx) including right kidney and ascending colon in a patient with complicated Crohn's disease (CD). A 36-year old female suffering from short bowel syndrome and frozen abdomen due to fistulizing CD after multiple abdominal operations underwent MVTx of eight organs including stomach, pancreatoduodenal complex, liver, intestine, ascending colon, right kidney, right adrenal gland, and greater omentum in November 2003. Immunosuppression consisted of alemtuzumab, tacrolimus and steroids. The patient was off parenteral nutrition by postoperative wk 3. She experienced one episode of pneumonia. The patient recovered completely and discharged 2.5 mo and was doing well 30 mo after MVTx. This is one of the very rare cases in which a complete mulitivisceral graft of eight abdominal organs was transplanted orthotopically.     

复合杂合parkin基因突变家族的不同表型

背景：Parkin基因（PRKN）突变可导致常染色体隐性遗传性早发帕金森病（EOPD）。目的：探讨EOPD白人家族PRKN突变的表现和基因型-表型关系。设计：对EOPD家族的3代20例成员进行基因分析，该家族有4例患者。应用直接基因组DNA测序、半定量聚合酶链反应、实时定量聚合酶链反应以及逆转录酶聚合酶链反应分析以确定PRKN突变。结果：4例早发患者（年龄30—38岁）被确定有PRKN复合杂合突变（T240M和EX5_6缺失），虽然PRKN的杂合T240M和纯合EX5_6缺失突变已有描述，但是据悉，本文为上述复合杂合突变的首次报道。患者的表型为典型常染色体隐性遗传性EOPD的表现，其特征是对左旋多巴治疗有效、相对缓慢的进展和运动障碍。所有杂合突变的基因携带者（T240M或EX5_6缺失）和1例56岁的复合杂合突变女性携带者（T240M和EX5_6缺失）无任何神经系统症状。结论：研究发现，PRKN基因复合杂合突变（T240M和EX5_6缺失）导致一个大的白人家族中4例成员发生常染色体隐性遗传性EOPD。另外1例成员具有相同的突变，比4例患者的平均发病年龄大10岁，并且无本病的临床表现。不完全的外显率对遗传咨询具有暗示作用，并且提示复杂的基因一环境交互作用在PRKN相关EOPD的发病机制中发挥作用。     

对比每天1次与每天3次妥布霉素治疗肺囊性纤维化(TOPIC研究):一项随机对照研究

背景:妥布霉素(3次/d)静脉注射广泛应用于慢性绿脓假单胞菌感染的肺囊性纤维化患者。本研究采用了随机双盲对照试验以评估妥布霉素1次/d与3次/d疗法应用于这些患者的安全性和有效性。方法:英国21个肺囊性纤维化诊疗中心的244例患者被随机分为妥布霉素1次/d治疗组及3次/d治疗组(均联合应用头孢他定),进行为期14d的治疗。用法为将妥布霉素加入0.9%的盐水中30m in内静脉滴注。主要结果为14d疗程结束后第一秒用力呼气量(FEV1)变化,用依据年龄、性别、身高的正常预测值的百分比表示。同时也测定了用基线值百分比表示的FEV1变化。次要结果还包…     

特纳综合征时碳水化合物代谢的异常:对葡萄糖耐量降低相关危险因素的分析

对103例正常的空腹和餐后葡萄糖浓度特纳综合征患者进行口服葡萄糖耐量试验(O G TT)。在试验中每30m in测试血糖、胰岛素、C肽以及胰岛素前体的浓度。利用内环境的稳态模型评估法(H OM A)和胰岛素敏感度定量检测指数(QUICK I),研究特纳综合征患者体内的胰岛素抵抗作用。2例和18例患者分别被新近诊断出患有糖尿病和葡萄糖耐量降低(IG T)。在O G TT试验中,与正常葡萄糖耐量(N G T)组患者不同(P<0.05),IGT组患者的平均血糖、胰岛素、C肽以及胰岛素前体的浓度都显著升高。与N GT组患者不同(P<0.05),IG T组患者的Q UICKI明显降…     

Differential Effects of Anesthetics on Mitochondrial KATP Channel Activity and Cardiomyocyte Protection

Background: Mitochondrial adenosine triphosphate-sensitive potassium (mitoKATP) channels play a pivotal role in mediating cardiac preconditioning. The effects of intravenous anesthetics on this protective channel have not been investigated so far, but would be of importance with respect to experimental as well as clinical medicine.

Methods: Live cell microscopy was used to visualize and measure autofluorescence of flavoproteins, a direct reporter of mitoKATP channel activity, in response to the direct and highly selective mitoKATP channel opener diazoxide, or to diazoxide following exposure to various anesthetics commonly used in experimental and clinical medicine. A cellular model of ischemia with subsequent hypoosmolar trypan blue staining served to substantiate the effects of the anesthetics on mitoKATP channels with respect to myocyte viability.

Results: Diazoxide-induced mitoKATP channel opening was significantly inhibited by the anesthetics R-ketamine, and the barbiturates thiopental and pentobarbital. Conversely, urethane, 2,2,2-trichloroethanol (main metabolite of [alpha]-chloralose and chloral hydrate), and the opioid fentanyl potentiated the channel-opening effect of diazoxide, which was abrogated by coadministration of chelerythrine, a specific protein kinase C inhibitor. S-ketamine, propofol, xylazine, midazolam, and etomidate did not affect mitoKATP channel activity. The significance of these modulatory effects of the anesthetics on mitoKATP channel activity was substantiated in a cellular model of simulated ischemia, where diazoxide-induced cell protection was mitigated by R-ketamine and the barbiturates, while urethane, 2,2,2-trichloroethanol, and fentanyl potentiated myocyte protection.     

围绝经妇女的子宫内膜绒毛膜癌

本文报道了1例发生于围绝经期的原发性子宫内膜绒毛膜癌合并腺癌的病例,并查阅了该患者的病程及组织病理学变化方面的资料,通过M edline检索查阅了其他的同种病例报道以及回顾了相关文献。β-H CG测定和子宫内膜刮片技术可为诊断绒毛膜癌提供依据,在患者临床状况恶化后立即给予多重化疗,疗效明显。5个月后该患者行经阴道子宫切除术和盆腔淋巴结切除术,最后治疗为经阴道镭疗法,手术切除标本组织病理学检查表明仅为腺癌,随访该患者18个月,并未发现任何肿瘤复发迹象。原发性子宫肿瘤非常罕见,其病因不明,治疗基础为综合化疗。而原发子宫绒毛…     

老年性黄斑变性视网膜色素上皮自体移植术的疗效:1项前瞻性试验研究

目的:评价1组老年性黄斑变性(A M D)并发黄斑中心凹脉络膜新生血管(fCN V)的连续性患者中,视网膜下手术联合自体视网膜色素上皮(RPE)细胞移植术的疗效。方法:本研究选择不能行激光或光动力疗法的fCNV患者,行视网膜下膜切除术,同时联合自体视网膜色素上皮细胞移植术。对照组采用单纯膜切除术。在术前、术后1个月、3个月及以后每隔3个月中,检测早期糖尿病视网膜病变研究和Jaege视力表的远、近最佳矫正视力、多焦ERG(m fERG)、中心视野、光学相干断层扫描(OCT)和眼底荧光造影。结果:最终有53只眼经过检查得到结果。其中,39只眼施行R …     

重庆市中药研究院

重庆市中药研究院创建于1930年，前身系国民政府经济工业部工业试验研究所。现为重庆市科委直属科研机构，下设有中药生药、中药种植、冬虫夏草、中药化学、中药制剂、中药药理毒理、实验动物研究所以及信息中心，附属有临床药理门诊部、附属制药厂、天然保健制品厂、医药公司，形成了中药科技与产业化的完整体系。     

瘢痕疙瘩瘙痒、疼痛与小神经纤维的功能:对照性研究

瘢痕疙瘩样瘢痕可引起瘙痒和疼痛,但对瘢痕疙瘩的这些症状的特征,却很少有了解。由于瘙痒和疼痛是由小神经纤维传导的,小神经纤维功能异常可以解释这种现象。作者探讨了瘢痕疙瘩样瘢痕的瘙痒和疼痛的特征,并探讨了瘢痕疙瘩的小神经纤维的功能。作者对瘢痕疙瘩的瘙痒和疼痛的强度及位置进行了持续 1年的研究,进一步通过无创、非正常的刺激分别引起异常的疼痛和反射来观察小神经纤维的功能。作者使用定量热感觉试验 (在瘢痕疙瘩皮损处、瘢痕疙瘩周边皮肤和对侧正常皮肤做为对照)进行测试。共 28例患者参与研究(男 13例,女 15例),平均年龄为 34…     

Current views of direct angioplasty in acute myocardial infarct.

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