Cbl Ubiquitin Ligases Control B Cell Exit from the Germinal-Center Reaction

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两例Y染色体部分缺失胎儿的产前诊断

目的对两例Y染色体部分缺失胎儿进行产前诊断。方法采用常规G显带及C显带技术分析胎儿及父亲的核型,采用荧光原位杂交(fluorescence in situ hybridization,FISH)、染色体拷贝数变异检测技术(copy number varaition sequencing,CNV-seq)性别决定基因(sex region of Y chromosome,SRY)检测技术及无精子因子(azoospermia factor,AZF)检测技术检测胎儿DNAO结果2例胎儿羊水染色体在320〜400条带水平均提示46,XN,del(Y)(qll.2),Y染色体着丝粒探针FISH检测结果均提示Y染色体数目未见异常。2例胎儿父亲外周血染色体核型均未见明显异常。胎儿羊水DNA拷贝数检测提示一例胎儿Y染色体q 11.221-ql2处缺失12.88 Mb,涉及全部AZFb+AZFc区域;另一例胎儿Y染色体qll.21-ql2处缺失14.84 Mb,涉及全部AZF区域。2例胎儿羊水SRY基因检测提示SRY基因阳性,SKY基因编码区未检测到已报道的致病点突变。2例胎儿基因检测提示存在AZF部分或全部缺失。结论联合多种技术有助于明确诊断Y染色体结构异常。CNV-seq检测有利于快速筛查胎儿Y染色体微缺失,可做为对染色体核型分析的补充和验证的方法。     

影像存储和传输系统在疗养院的实施与应用

随着数字化医学影像技术和信息技术迅速发展,影像存储和传输系统(Picture Archiving and Communications System,以下简称PACS)在医疗机构中日益普及。PACS建设的目标旨在实现医学影像的计算机化和网络化管理[1],帮助影像科室医师进行影像诊断,更好地为临床医生的诊断决策提供参考,提高对疗养员的医疗服务品质。PACS是一     

Abdominal ventral rectopexy with colectomy for obstructed defecation syndrome: An alternative option for selected patients

BACKGROUNDAbdominal ventral rectopexy (AVR) with colectomy is controversial in the treatment of obstructed defecation syndrome (ODS). Literature data on this technique for ODS are very limited.AIMTo evaluate the safety and efficacy of AVR with colectomy for selected patients with ODS.METHODSConsecutive patients who underwent AVR with colectomy for ODS were identified prospectively from 2016 to 2017 in our department. Patient demographics, perioperative surgical results, and postoperative follow-up outcomes were collected and analyzed. Long-term follow-up was evaluated with standardized questionnaires. The severity of symptoms was assessed by the objective Wexner Constipation Score (WCS) and ODS Score. The quality of life was assessed by the Patients Assessment of Constipation Quality of Life score. Functional outcome was compared pre- and post-operatively for each patient. The primary outcomes were determined by the improvement in symptoms and quality of life. Secondary outcome measures were operating time, postoperative length of stay, morbidity and mortality, improvement of pelvic floor structure, and patient satisfaction.RESULTSFour patients underwent robotic-assisted surgery, and two patients underwent a laparoscopic-assisted procedure. The mean operating time for the robotic approach was 243 min (range 160–300 min), and the mean operating time for the laparoscopic approach was 230 min (range 220-240 min). The mean postoperative length of stay was 8.2 d (range 6-12 d). There was no conversion to open procedure and no postoperative mortality. No urinary retention, wound infection, prolonged ileus, pelvic infection and anastomosis leakage occurred. Six patients were followed up for 36 mo. The WCS, ODS, and Patients Assessment of Constipation Quality of Life score improved significantly postoperatively (P < 0.05). The WCS and ODS scores showed the best remission and stabilization at 6 to 12 mo after surgery. There was no recurrence or novel constipation after surgery. None of the patients used laxative medication.CONCLUSIONRobotic and laparoscopic-assisted ventral rectopexy with colectomy is a safe and effective procedure for selected patients with ODS. However, comprehensive preoperative evaluation and careful patient selection are essential.     

Angiotensin II regulates the expression of plasminogen activator inhibitor-1 in cultured endothelial cells. A potential link between the renin-angiotensin system and thrombosis.

Plasminogen activator-inhibitor C-1 (PAI-1) plays a critical role in the regulation of fibrinolysis, serving as the primary inhibitor of tissue-type plasminogen activator. Elevated levels of PAI-1 are a risk factor for recurrent myocardial infarction, and locally increased PAI-1 expression has been described in atherosclerotic human arteries. Recent studies have shown that the administration of angiotensin converting enzyme inhibitors reduces the risk of recurrent myocardial infarction in selected patients. Since angiotensin II (Ang II) has been reported to induce PAI-1 production in cultured astrocytes, we have hypothesized that one mechanism that may contribute to the beneficial effect of angiotensin converting enzyme inhibitors is an effect on fibrinolytic balance. In the present study, we examined the interaction of Ang II with cultured bovine aortic endothelial cells (BAECs) and the effects of this peptide on the production of PAI-1. 125I-Ang II was found to bind to BAECs in a saturable and specific manner, with an apparent Kd of 1.4 nM and Bmax of 74 fmol per mg of protein. Exposure of BAECs to Ang II induced dose-dependent increases in PAI-1 antigen in the media and in PAI-1 mRNA levels. Induction of PAI-1 mRNA expression by Ang II was not inhibited by pretreating BAECs with either Dup 753 or [Sar1, Ile8]-Ang II, agents that are known to compete effectively for binding to the two major angiotensin receptor subtypes. These data indicate that Ang II regulates the expression of PAI-1 in cultured endothelial cells and that this response is mediated via a pharmacologically distinct form of the angiotensin receptor.     

跨肺压监测设定呼吸机参数对腹腔高压模型猪血流动力学及氧代谢的作用

目的 评价通过跨肺压(Ptp)设定呼吸机参数对腹腔高压(IAH)模型猪血流动力学、氧代谢和呼吸力学的影响。方法 将6只家猪进行麻醉、气管插管后接呼吸机辅助呼吸,容量控制模式,呼吸机参数设置潮气量(VT) 10 ml/kg,呼吸频率16次/min,吸入氧浓度(FiO2)0.40,呼气末正压(PEEP)5 cm H2O(1 cm H2O=0.098 kPa)。置入动脉导管和Swan-Ganz导管,测定机械通气1h（基础值）的心率、平均动脉压(MAP)、心排血指数(CI)、中心静脉压(CVP)、肺动脉楔压(PAWP)等血流动力学指标,以及气道峰压( Ppeak)、平台压(Pplat)、胸腔内压(Pes)、肺顺应性(Cst)等呼吸力学指标,并行血气分析检查。通过腹腔内注入氮气法复制25 mm Hg(1 mm Hg =0.133 kPa)IAH模型,呼吸机参数不变,持续观察2h;随后置入食道测压管,调整PEEP使呼气末Ptp≥0（为正值）,其余呼吸机参数不变,持续观察2h。结果 6只家猪均制模成功,无一发生气压伤和死亡。与基础值比较,IAH后1h、2h模型猪心率（次/min）增快(134.3±5.8、127.3±3.3比117.7±1.5),MAP(mm Hg)、CVP(mm Hg)和PAWP(mm Hg)上升(MAP：120.7±3.8、117.3±4.8比100.4±6.6,CVP：7.3±0.3、7.6±0.9比5.6±0.2,PAWP:14.0±0.6、14.0±1.0比12.3±0.3),CI(L．min-1．kg-1)下降(0.150±0.019、0.137±0.014比0.179±0.021);Ppeak (cm H2O)、Pplat (cm H2O)、Pes(cm H2O)上升(Ppeak:46.3±2.3、47.0±3.2比11.0±1.6,Pplat：25.7±1.3、26.0±1.6比9.0±0.6,Pes：13.7±0.3、14.3±0.3比2.3±0.3),Cst (ml/cm H2O)下降（8.3±0.3、9.0±0.6比23.0±1.6）;动脉血氧分压(PaO2,mm Hg)和氧输送量(DO2,ml．min-·kg-1)均下降(PaO2：142.0±13.2、140.0±16．0比166.3±11.3,DO2：19.40±2.90、19.88±4.14比25.07±6.30),差异均有统计学意义（均P＜0.05）。与IAH常规机械通气同时间点各指标比较,通过食道压监测1h、2h时PaO2 (161.6±11.9、164．0±13.6)、DO2(21.90±6.21、21.16±2.78)以及Cst（12.0±1.6、12.7±2.9）均明显改善,CI(0.121±0.013、0.120±0.012)则进一步下降,反映组织灌注的血乳酸(mmol/L)明显下降(1.60±0.12比2.70±0.44,1.67±0.07比2.27±0.13),Pplat (31.3±3.4、31.7±3.2)显著升高,差异均有统计学意义（均P＜0.05）;而动脉血二氧化碳分压(PaCO2)、心率、MAP、CVP、PAWP则无明显变化。结论 IAH状态下通过Ptp设定呼吸机参数能改善模型猪的氧分压及Cst,对血流动力学指标影响较小。     

Bortezomib in treatment of extramedullary plasmacytoma of the pancreas

BACKGROUND:Extramedullary pancreatic plasmacy- toma treated with bortezomib is rarely reported. METHODS:We admitted a 53-year-old woman with an asymptomatic mass above the left clavicle for over three months,then an asymptomatic swelling of the pancreas was found.A biopsy on the mass and a fine needle aspiration of the pancreas were performed.The diagnosis of extramedullary plasmacytoma(EMP)was made.The patient was initially treated with combination chemotherapy consisting of vincristine,doxorubicin and dex...     

玉竹提取物A对小鼠免疫性肝损伤的保护作用

目的：探讨玉竹提取物A对小鼠免疫性肝损伤的保护作用，探讨其发挥此作用的可能的机制。 方法：①实验于2004-06／10在锦州医学院免疫实验室完成。选用健康雄性昆明小鼠50只。普通级。将50只健康雄性昆明小鼠随机分为5组：正常对照组、模型对照组、玉竹提取物A 0．5g／kg组、玉竹提取物A 1g／kg组、玉竹提取物A2g/／kg组，每组10只。②玉竹提取物A是玉竹的干燥根茎经水醇法提取而得。（固正常对照组和模型对照组小鼠腹腔注射生理盐水，玉竹提取物A0.5，1，2g／kg组小鼠分别腹腔注射相应浓度的玉竹提取物A，每次每只0．5mL，3次／d，连续注射4d。最后一次注射后8h，除正常对照组注射生理盐水外，其余各组均尾静脉注射20mg／kg的刀豆蛋白A0．5mL，制备免疫性肝损伤模型。（4）注射刀豆蛋白A8h后摘眼球取血低温保存，取肝待行光镜检查，以观察肝脏组织病理学改变（常规苏木精-伊红染色），取脾待行淋巴细胞增殖实验（采用四甲基偶氮唑盐法）和流式细胞术以检测T淋巴细胞亚群。各组小鼠血清谷丙转氨酶活性检测按购自南京建成生物研究所的谷丙转氨酶试剂盒说明进行。⑤组间计量资料差异比较采用方差分析。 结果：昆明小鼠50只均进入结果分析。①肝脏组织病理学改变：模型对照组可见明显免疫性肝损伤病理改变。玉竹提取物A3个实验组小鼠肝损伤病理改变均不同程度的好于模型对照组。（2）玉竹提取物A对血清谷丙转氨酶活性的影响：模型对照组血清谷丙转氨酶活性明显高于正常对照组（t=13．8，P〈0．01）。玉竹提取物A3个实验组血清谷丙转氨酶活性低于模型对照组，尤以玉竹提取物A2g／kg组与模型对照组差异最明显（1=5．62，P〈0．01），且存在剂量依赖关系。（参玉竹提取物A对T淋巴细胞转化增值的影响：刀豆蛋白A刺激体外培养的各组脾淋巴细胞，模型对照组的刺激指数明显高于正常对照组（t=9．02，P〈0．01），玉竹提取物A3个实验组的刺激指数明显低于模型对照组（t=6．99-11．06,P〈0．01），且存在剂量依赖关系。④玉竹提取物A对脾组织T淋巴细胞亚群的影响：各组，CD4^＋T，CD8^＋T淋巴细胞的数量和比例差异不明显（P〉0．05）。 结论：①玉竹提取物A具有抑制肝细胞破坏及改善肝脏微循环的作用。②玉竹提取物A可以显著抑制T淋巴细胞的转化增殖，并呈剂量依赖关系，玉竹提取物A发挥肝保护作用的一个机制可能就是显著抑制T淋巴细胞的转化增殖，减少肝损伤细胞因子的释放，抑制活化增殖的T琳巴细胞对肝细胞的直接细胞毒作用。③在肝损伤的早期可能只是大量免疫细胞聚集于肝脏并且各种分泌及破坏功能增强，而非其数量明显增加。     

The associations between psychological stress and psoriasis: a systematic review

Psoriasis is estimated to affect around 2–3% of the general population. More than one‐third of Australians report having a significant level of distress in their daily lives. Psychological stress has long been shown to play an important role in the natural history of psoriasis, but the details of this relationship remain to be clearly defined. We performed a systematic review of the literature with the aim of determining whether there is a temporal association between psychological stress as the predictor and onset and/or exacerbation of psoriasis as the outcome measure. Our secondary aim was to establish whether there is a relationship between the degree of psychological stress and clinical severity of psoriasis. Our systematic review demonstrates a probable temporal association between different measures of psychological stress and onset, recurrence, and severity of psoriasis. In the light of this, we suggest clinicians include “stress” as a trigger factor in their psoriasis assessment and consider psychological interventions as adjuncts, particularly in those who identify as “stress‐responders”.