体外循环对紫绀型先天性心脏病病人血小板的影响

检测１８例体外循环紫绀型先天性心脏病手术病人术前，术中及术后３，８天外周血血小数量和 附，聚集功能，探讨体外循环对血小板质和量的影响。结果显示，血小板数量和聚集功能在术后显著下降并持续至术后８天，血小板粘附功能显著下降，术后３天恢复。提示体外循环气血界面，人工材料非内皮表面可导致血小板激活，粘附，聚集面在量消耗，数量和功能显著下降。     

劳动复合方案对小鼠血浆心钠素和耐力的实验研究

昆明种小鼠56只，在两个海拔高度上（300m和5000m）各分为两个组（对照组和复合组），分别给予相应的处理因素后，检测其血浆ANP水平和游泳时间。结果表明，小鼠在海拔5000m生活48h后，其血浆ANP较海拔300m小鼠降低52．87％，而其游泳时间则增长91．59％；在两个海拔高度上，复合组血浆ANP都较对照组降低，而游泳时间都长于对照组．提示复合方案提高高原劳动能力的机理，可能与降低体内血浆ANP水平有关。     

三种基牙材料对铸造玻璃瓷冠碎裂影响的研究

目的:研究不同种类和强度基牙材料与瓷冠破折碎裂的内在关系.方法:选用计算机建立上颌中切牙的三维有限元模型,模拟陶瓷层、牙本质、核桩等结构.分析在天然齿基牙、钴铬合金基牙及复合树脂基牙情况下,铸造玻璃陶瓷全冠的瓷层内及残余牙本质的应力分布情况.结果:高弹性模量的钴铬合金能够降低瓷层及残余牙本质内的最大主应力和最小主应力峰值,防止瓷层破折碎裂;而弹性模量与牙本质相近的复合树脂桩降低应力峰值的作用不明显.结论:对于有牙髓活力的牙本质基牙,尽量保存牙髓活力后行全冠修复.对于行根管治疗术后的无髓牙,进行核桩冠修复时,采用高弹性模量的高熔合金,尽量避免使用弹性模量较小的复合树脂核.     

Similarities and differences in mechanisms of cardiotoxins, melittin and other myotoxins

Myonecrosis induced in vivo by cardiotoxin, melittin, and Asp49 and Lys49 phospholipase A₂ (PLA₂) myotoxins involves rapid lysis of the sarcolemma, myofibril clumping, and hypercontraction of sarcomeres. In contrast, skeletal muscle necrosis induced by crotamine and myotoxin a is much slower, consisting of mitochondrial and sarcoplasmic reticulum swelling, myofibril degeneration, and lack of sarcolemma or transverse tubule damage. The mechanisms contributing to the myonecrosis induced by these peptides were evaluated. Two cardiotoxins and two Lys49 PLA₂ myotoxins lysed primary cultures of human skeletal muscle within 24 hr at a concentration of 0.25 μM, while melittin, crotamine, and myotoxin a, and an Asp49 PLA₂ myotoxin were non-cytolytic at concentrations up to 5.0 μM, suggesting that cytolysis is not a good measure of myotoxicity. Crotamine and the Lys49 PLA₂ myotoxin altered Ca²⁺ ion flux in human heavy sarcoplasmic reticulum by opening the ryanodine receptor. Whole-cell patch-clamp studies demonstrated that administrating crotamine intracellularly increased Na⁺ currents. Free fatty acids, liberated by activation of tissue phospholipase C or by the PLA₂ activity of the myotoxins, were monitored for crotamine, myotoxin a and a Lys49 PLA₂ myotoxin in cell cultures in which the lipids had been radiolabeled. Only the Lys49 myotoxin produced significant amounts of fatty acid in cell cultures, supporting a potential role for fatty acid production only in the mechanism of sarcolemma-destroying myotoxins. These findings, coupled with those in the literature, support a hypothesis in which the myotoxins and/or products of lipase activity (e.g. fatty acids) are acting at a site existing on both the Na⁺ channel and a protein involved in Ca²⁺ release and probably serving a modulatory function for ion regulation. Based on the similarities in mechanisms between the toxins and fatty acids, the most likely site would be a fatty acid binding site on the protein (either similar to that on fatty acid binding proteins, or an acylated cysteine residue) or in the membrane.     

Proteolytic Processing Mechanisms in the Biosynthesis of Neuroendocrine Peptides: The Subtilisin-like Proprotein Convertases

The recent discovery of a novel family of precursor processing endoproteases has greatly accelerated progress in understanding the complex mechanisms underlying the maturation of prohormones, neuropeptides, and many other precursor-derived proteins. At least six members of this family have been found thus far in mammalian species, several having alternatively spliced isoforms, and related enzymes have been identified in many invertebrates, including molluscs, insects, nematodes, and coelenterates. The proprotein convertases are all dependent on calcium for activity and all possess highly conserved subtilisin-like domains with the characteristic catalytic triad of this serine protease (ordered Asp, His, and Ser along the polypeptide chain). Two members of this family, PC2(SPC2) and PC1/PC3(SPC3), appear to play a preeminent role in neuroendocrine precursor processing. Both convertases are expressed only in the brain and in the extended neuroendocrine system, while another important family member—furin/PACE (SPC1)—is expressed more ubiquitously, in almost all tissues, and at high levels in liver. SPC2 and SPC3 exhibit acidic pH optima and other properties which enhance their activity in the acidic, calcium-enriched environment of the dense-core secretory granules of the regulated pathway in neuroendocrine cells, while furin has a neutral pH optimum and is localized predominantly to the trans Golgi network where it is retained by a C-terminal transmembrane domain. Furin processes a wide variety of precursors in the constitutive pathway, such as those of growth factors, receptors, coagulation factors, and viral glycoproteins. Recent findings on the processing of proopiomelanocortin, proinsulin, proglucagon, and several other neuroendocrine precursors by SPC2 and SPC3 are discussed, along with information on the structure, properties, evolution, developmental expression, and regulation or the convertases. An inherited defect in the fat/fat mouse which affects the processing of proinsulin, and probably also many other prohormones, due to a point mutation in carboxypeptidase E has recently been identified and has begun to provide new insights into the functional integration of the individual processing steps.     

特发性肌炎免疫机制研究及临床意义

目的探讨多发性肌炎（PM）和皮肌炎（DM）患者骨胳肌组织损伤的免疫机制及临床意义。方法应用免疫荧光一步法和免疫组化SP法分析25例PM／DM肌组织中免疫球蛋白IgG、IgM、补体C3和浸润肌组织单核细胞的分布与定位。结果PM和DM肌组织中IgG、IgM、C3的阳性率分别为60％、33．3％、20％和70％、40％、50％，以IgG为主（P＜0．05），分布于肌组织血管壁、肌膜和肌浆中，补体C3在DM血管壁的分布有统计学意义（P＜0．05）；80％PM和7O％DM肌组织有单核细胞浸润，PM以T淋巴细胞为主，Ia＋活化TS细胞占多数，主要分布于肌内衣，DM以B淋巴细胞为主，多分布在肌柬衣血管周围。结论免疫反应在PM和DM发病机理中占重要地位，PM以T细胞介导的细胞毒作用为主，DM以体液免疫特别是补体介导的血管损害为主。     

儿童生殖细胞瘤全脑放射治疗中自适应消除摆位累积误差新技术探讨

目的 以影像引导放射治疗(image guided-radiotherapy, IGRT)为基础探讨儿童生殖细胞瘤全脑自适应放射治疗(以下简称放疗）(adaptive radio therapy,ART）可行性,为临床全脑ART提供依据。方法 回顾性研究首都医科大学附属北京天坛医院放射治疗科20例中枢神经系统生殖细胞肿瘤(germ cell tumors, GCTs)行全脑预防性照射的放疗患者。每例患者治疗前三次及后每周行一次锥形束电子计算机断层扫描(cone beam computed tomography,CBCT),共7次。记录整个治疗过程中左右(lateral, LAT)、进出(longitudinal, LNG)、升降(vertical, VRT)方向上的摆位误差值。原计划命名为Plan_A,将因摆位误差值产生新的治疗中心点(treatment center point,ISO)回归治疗计划系统,不改变射野权重、射野分布情况下进行体积剂量计算,用Plan Sum功能相加摆位累积误差产生新计划得到Plan_B。将摆位累积误差产生的新计划用Acuros XB算法重新快速优化后用Plan Sum功能相加得到Plan_C。对比Plan_A、Plan_B、Plan_C的靶区及危及器官(organ at risk, OAR)剂量变化并用Wilcoxon配对符号秩检验证统计学差异。结果与Plan_A相比,Plan_B 均匀性指均匀性指数（homogeneity index, HI）95计划靶区（planning target volume, PTV）、 HI98 PTV为1.058 4~1.084 7。适形度指数(conformity index, CI）PTV为0.934 4。 临床靶区（clinical target volume, CTV）D_min相对偏差为－7.22%,P值为0.000 7。OAR相对偏差为－0.1%~9.8%。与Plan_ A相比,Plan_ C的HI95 PTV、 HI98 PTV为1.056 9~1.078 8。CI PTV为0.936 5。CTV D_min相对偏差为－1.69%,P值为0.545 9。OAR相对偏差为－0.15%~3.40%。结论儿童生殖细胞瘤全脑放疗摆位累计误差对靶区及危及器官剂量差异有统计学意义,摆位累计误差导致靶区均匀性及适形度变差,CTV的D_min 变化超过5%,OAR受量增高。ART再计划可以让靶区均匀性和适形度及危及器官受量接近原计划。同时Plan_B虽然存在相对偏差,但是危及器官的绝对剂量在合理范围内。     

中性粒细胞/淋巴细胞比值对冠状动脉旁路移植术后心肌损伤的影响

探讨患者术前中性粒细胞/淋巴细胞比值（NLR）对非体外循环下冠状动脉旁路移植术（OPCABG）后心肌损伤以及临床预后的影响。方法 选取2014年1月—2019年12月我院收治的314名行OPCABG的患者,依据患者术前NLR的三分位数分为高比值组（NLR＞2.8,n=102）、中比值组（2.8≥NLR≥1.6,n=106）以及低比值组（NLR＜1.6,n=106）。分别检测患者术前基线、术后8 h及术后24 h肌酸激酶同工酶（CKMB）及肌钙蛋白（CTnI）水平。结果 高比值组患者术后心肌损伤发生率显著高于中比值组及低比值组（P＜0.05）。高比值组术后CKMB峰值显著高于中比值组［2.1(1.2～13.0) ng/mL vs2.0(1.0～7.3) ng/mL, P=0.047］及低比值组［2.1(1.2～13.0) ng/mL vs1.1(0.9～1.6) ng/mL, P＜0.001］。且高比值组患者术后CTnI峰值同样显著高于中比值组［0.075(0.010～0.185) ng/mL vs 0.020(0.000～0.103) ng/mL, P=0.011］,以及低比值组［0.075(0.010～0.185) ng/mL vs0.010(0.000～0.030) ng/mL, P＜0.001］。术前NLR较高是术后患者CTnI升高的独立危险因素（OR：2.809; 95%CI:1.326～5.954; P=0.007）。高比值组患者1年不良心血管事件发生率显著高于中比值组及低比值组（HR：1.80; 95%CI:1.16～2.79; Log Rank P=0.021）。结论 患者术前中性粒细胞/淋巴细胞比值升高是非体外循环冠状动脉旁路移植术后心肌损伤的独立危险因素,而且会增加患者不良心血管事件     

多囊性肾脏发育不良胎儿的遗传学病因、影像学表现及妊娠结局分析

目的 探究多囊性肾脏发育不良胎儿的遗传学病因、影像学表现及妊娠结局。方法 回顾性分析2017年5月—2023年5月扬州大学附属淮安市妇幼保健院收治的80例多囊性肾脏发育不良胎儿的临床资料,分析其遗传学病因、影像学表现及妊娠结局。结果 80例多囊性肾脏发育不良胎儿中检出11例（13.75%）染色体异常,其中染色体数目异常1例（1.25%）,10例（12.50%）存在染色体拷贝数变异（CNV）。80例多囊性肾脏发育不良胎儿中,39例（48.75%）为左侧多囊性发育不良肾,37例（46.25%）为右侧多囊性发育不良肾,剩余4例（5.00%）为双侧多囊性发育不良肾;单纯泌尿系统异常64例（80.00%）,合并其他系统异常16例（20.00%）,以心血管系统异常（8例）、肢体骨骼异常（3例）和、神经系统异常（3例）最常见。单纯性泌尿系统异常胎儿、合并其他系统异常胎儿的致病性CNV检出率分别为10.94%（7/64）和12.50%（2/16）,差异无统计学意义（P >0.05）。单纯性泌尿系统异常胎儿中,单侧多囊性发育不良胎儿、双侧多囊性发育不良胎儿的致病性CNV检出率分别为9.84%（6/61）和33.33%（1/3）,差异无统计学意义（P >0.05）。对80例多囊性肾脏发育不良胎儿进行随访,失访8例（10.00%）,人工终止妊娠31例（38.75%）,胎死宫内0例（0.00%）,活产41例（51.25%）,其中产后死亡1例（1.25%）。结论 多囊性肾脏发育不良胎儿采用染色体微阵列分析技术可提高遗传学检出率,影像学检查在多囊性肾脏发育不良诊断中具有一定价值,可为多囊性肾脏发育不良胎儿的产前诊断、遗传咨询、预后评估提供依据。