应用层粘连蛋白免疫电镜技术研究杜氏肌营养不良症肌细胞基膜改变

应用免疫电镜技术，对ＤＭＤ和正常对照骨骼肌细胞基膜层粘连蛋白（ＬＮ）定位分布进行观察，表明ＬＮ定位于肌基膜，高电子密度反应产物呈粗带状均匀分布于肌细胞膜外层。ＤＭＤ肌基膜ＬＮ分布不均，外观粗糙，常出现双层或多层结构，并可见局部缺损。对其中２例ＤＭＤ进行ＬＮ抗体Ｆａｂ’－ＨＲＰ染色作进一步观察，也证实了ＤＭＤ肌基膜存在缺损改变，这为ＤＭＤ膜缺陷理论提供了新的病理形态学证据。     

应用免疫荧光技术诊断肌营养不良

目的 用免疫荧光技术对迪谢内（ＤＭＤ）、贝克（ＢＭＤ）、肢带－２Ｄ（ＬＧＭＤ２Ｄ）和肢带－２Ｃ（ＬＧＭＤ２Ｃ）型肌营养不良的致病基因编码产物ｄｙｓｔｒｏｐｈｉｎ、α－肌聚糖和γ－肌聚糖进行检测，为临床对肌营养不良的确诊和分型建立特异性的实验诊断手段。方法 用抗ｄｙｓｔｒｏｐｈｉｎ中央棒状区、Ｃ－末端和抗α、γ－肌聚糖单克隆抗体，借助免疫荧光技术对患者骨骼肌细胞膜上的致病基因表达产物进行研究。结果 在１５例临床拟诊为肌营养不良的患者中确诊９例为ＤＭＤ，４例ＢＭＤ，１例ＬＧＭＤ２Ｄ和１例ＬＧＭＤ２Ｃ。结论 分析骨骼肌细胞膜上ＤＭＤ、α－肌聚糖和γ－肌聚糖基因表达产物是诊断ＤＭＤ／ＢＭＤ、ＬＧＭＤ２Ｄ和ＬＧＭＤ２Ｃ型肌营养不良的可靠和特异手段。     

脂质沉积性肌病：附6例临床与肌肉病理观察

６例脂质沉积性肌病经肌肉组化检查确诊，其中２例做了电镜观察。年龄在３－３５岁之间，４例有家庭史，均在同辈人发病，属常染色体隐性遗传。肌活检油红Ｏ染色示大量脂滴在肌浆中沉积，Ⅰ型纤维中含量最多，Ⅱ型其次。电镜观察见大量无膜脂滴平行分布于肌原纤维间，线粒体增多，其嵴不清蜥。     

强直性肌营养不良的临床与肌肉病理研究

目的 研究强直性肌营养不良（ＤＭ）的临床与病理变化。方法 总结３例ＤＭ的临床特点，并对肌活检标本进行光镜和电镜检查。结果 ３例ＤＭ患者，年龄２５～４０岁，临床特点为缓慢进行的四肢无力，肌强直发作，腱反射迟钝对称，前额秃发。ＥＭＧ示肌源性损害，可见肌强直发放。肌活检光镜下可见肌纤维萎缩，肌核内移呈核链形成，肌纤维横纹尚存，晚期可见脂肪细胞浸润，电镜下可见肌纤维变性，溶解，Ｚ带破坏，线粒体肿胀、变性，     

X连锁肌营养不良症14例基因缺失的研究

应用９对引物的多重扩增对新疆地区的１４例Ｘ连锁肌营养不良症（ＭＤ）患者进行了基因分析。结果发现其中７例（５０％）有至少一个基因片段的缺失，且缺失热点集中于外显子４５～５１，这与国内外报道相近。同时发现，２例Ｂｅｃｋｅｒ型肌营养不良症的基因缺失均为一段，而５例Ｄｕｃｈｅｎｎｅ型肌营养不良症中的４例缺失各为２～３段。缺失片段多少是否与症状严重程度之间存在某种联系尚待研究论证。     

X连锁肌营养不良症14例基因缺失的研究

应用９对引物的多重扩增对新疆地区的１４例Ｘ连锁肌营养不良症（ＭＤ）患者进行了基因分析。结果发现其中７例（５０％）有至少一个基因片段的缺失，且缺失热点集中于外显子４５～５１，这与国内外报道相近。同时发现，２例Ｂｅｃｋｅｒ型肌营养不良症的基因缺失均为一段，而５例Ｄｕｃｈｅｎｎｅ型肌营养不良症中的４例缺失各为２～３段。缺失片段多少是否与症状严重程度之间存在某种联系尚待研究论证。     

运动神经元病的肌肉病理及其在诊断中的使用价值

本文报告６７例运动神经元病（ＭＮＤ）患者肌活检组织的光镜及酶组织化学检查的结果，其中２２例作过电镜观察；并与其它易混淆病（成年型脊髓性肌萎缩症、青年单侧上肢远端肌萎缩症、颈椎病和某些较慢性的周围神经病）７０例作对比研究。结果表明ＭＮＤ的肌肉病理有其特点，认为肌活检可作为ＭＮＤ诊断和鉴别诊断的重要辅助检查手段。     

X连锁肌营养不良症14例基因缺失的研究

应用９对引物的多重扩增对新疆地区的１４例Ｘ连锁肌营养不良症（ＭＤ）患者进行了基因分析。结果发现其中７例（５０％）有至少一个基因片段的缺失，且缺失热点集中于外显子４５￣５１，这与国内外报道相近。同时发现，２例Ｂｅｃｋｅｒ型肌营养不良症的基因缺失均为一段，而５例Ｄｕｃｈｅｎｎｅ型肌营养不良症中的４例缺失各为２￣３段。缺失片段多少是否与症状严重程度之间存在在某种联系尚待研究论证。     

亚急性联合变性四例磁共振成像特点

报道４例亚急性联合变性（ＳＣＤ）患者的临床特点及脊髓磁共振成像（ＭＲＩ）所见，观察病灶于治疗前后的变化并分析ＭＲＩ在诊断ＳＣＤ中的价值。例１男，６８岁，因双足麻木２周、双下肢无力１０天入院。查体：双下肢肌张力偏高，深感觉减退。血ＲＢＣ２．９９×１０１...     

骨骼肌冰冻切片和酶组织化学技术在肌病诊断中的应用

骨骼肌病理检查是肌病诊断中的一种重要检查方法，特别是近年来，骨骼肌冰冻切片和酶组织化学染色技术的开展和电镜检查的应用，使其在肌病诊断过程中，起着越来越重要的作用。本文总结了我科近３年来，采用液氮速冻固定，冰冻切片和酶组织化学染色技术，对７０例肌肉标本进行的病理学检查结果，分析讨论其病理改变及其在临床诊断中的价值。１资料和方法１１．１临床资料 男性４８例，女性２２例。年龄４－６６岁。临床表现有肌无力５６例，伴肌肉萎缩４３例，伴肌肉疼痛１１例，发作性肌无力６例。临床诊断：进行性肌营养不良症１１例，肌…     

不同病房模式对精神分裂症病情影响的研究

目的探讨不同病房模式对首发精神分裂症患者病情的影响及康复情况。方法开放式病房的患者为研究组68例,同时选择住封闭式病房患者为对照组130例,对两组患者的临床资料进行比较分析。结果对照组患者入院时焦虑抑郁及敌对猜疑出现明显变化（P＜0.05）;研究组患者在第1周时焦虑抑郁已经开始改善,第2周时病情、阳性症状、一般精神病理、敌对猜疑、激活性也出现明显变化（P＜0.05）;第4周时研究组病情及各因子、认知功能及自知力显著改善,与对照组比较存在统计学差异（P＜0.05）。结论开放式病房模式可以减少患者的负性影响,更快改善患者的认知功能,有利于患者自知力的恢复,使患者的病情达到更全面的康复。     

Certain predictions on the effectiveness of l-DOPA on parkinsonism

Summary Sixty nine patients with Parkinson's disease were treated with L-DOPA for more than a year. L-DOPA produced remarkable improvement in akinetic patients, moderate improvement in rigidity and slight improvement in tremor. The degree of improvement in akinesia, rigidity and tremor tended to be reversely related to the severity of the disease. Rigidity and akinesia improved better in young patients, but there was no correlation between age and the response of tremor to the treatment. Except for rigidity there was no correlation between the improvement and the duration of the disease.

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Zusammenfassung 69 Parkinsonpatienten wurden während mehr als 1 Jahr mit L-DOPA behandelt. Es wurde dadurch bei akinetischen Patienten eine beachtliche, bei Patienten mit Rigor eine mäßige und bei Patienten mit Tremor eine geringfügige Besserung erreicht. Das Ausmaß der Besserung war in allen drei Formen um so geringer, je ausgeprägter die Symptome bei Behandlungsbeginn waren. Rigor und Akinesie sprachen bei jungen Patienten besser auf die Therapie an, aber es bestand keine Korrelation zwischen dem Alter der Patienten und der Beeinflußbarkeit des Tremors durch die L-DOPA-Therapie. Außer für den Rigor bestand keine Korrelation zwischen dem Ausmaß der Besserung und der Dauer der Erkrankung vor Behandlungsbeginn.

     

Efficacy of milnacipran on poststroke depression on inpatient rehabilitation

Post-stroke depression (PSD) has a negative impact on rehabilitation following stroke. No satisfactory antidepressant treatment for PSD has yet been developed. The present study examined the effect of milnacipran, a serotonin and norepinephrine reuptake inhibitor, on PSD patients. Eleven PSD patients taking milnacipran in a rehabilitation hospital were compared to age-matched, sex-matched, and severity of depression at admission-matched PSD patients hospitalized during 2001 who did not take any antidepressant as historical control. Severity of depression was measured using self-rating depression scale for depression (SDS) assessed at admission and discharge after 3 months inpatient rehabilitation. Activities of daily living (ADL) and quality of life (QOL) were measured, respectively, by the functional independence measure (FIM) and a self-completed questionnaire for QOL (QUIK) as outcomes of rehabilitation. For the SDS score, the group taking milnacipran showed significant improvement compared to the control group in our study. FIM was improved in both groups. In the end QUIK did not change significantly in either group. We found no major side-effects of milnacipran among the patients. These results suggest that milnacipran is a safe and effective treatment for PSD for inpatients undergoing rehabilitation.     

散发神经鞘瘤22号染色体杂合子丢失发生的研究

目的探讨散发神经鞘瘤病人22号染色体（CHR22）杂合子丢失（LOH）情况及其与肿瘤增殖的关系。方法选取4个与NF2基因密切相关的多态性微卫星标记物,应用PCR方法研究54例神经鞘瘤的LOH情况。采用Ki-67和增殖细胞核抗原（PCNA）评估增殖指数。结果23例（42.6%）CHR22发生LOH,听神经鞘瘤发生率显著性高于脊神经鞘瘤（χ2=5.14,P〈0.05）。发生LOH者增殖指数明显高于无LOH者（Pki-67=0.0079,PPCNA=0.0021）。结论在散发神经鞘瘤中,CHR22LOH是常发事件;听神经瘤与脊神经鞘瘤LOH发生率有显著性差异,CHR22LOH与神经鞘瘤的增殖活动性有关。     

Influence of abciximab on the adhesion of platelets on a shielded plasma gradient prepared on polyethylene

INTRODUCTION: Thrombotic effects of biomaterial implants are mediated merely through activation of the platelet glycoprotein IIb-IIIa (GpIIb-IIIa) receptor. Consequently, platelet GpIIb-IIIa receptor inhibitors are successfully used during stent implantation procedures to prevent thrombosis. However, currently a new generation of stents contains surface coating, which changes the surface to more hydrophobic or hydrophilic. This change markedly affects the interaction of platelets and may influence the efficiency of GpIIb-IIIa inhibitors. MATERIALS AND METHODS: To study the influence of the wettability of biomaterials on the effectiveness of abciximab, 5-cm polyethylene gradients with contact angles of 100 degrees to 40 degrees were made by means of glow discharge. Fresh whole blood with or without abciximab was recirculated over this gradient. RESULTS: Inhibition of platelet adhesion by abciximab was maximal, but not complete, on the hydrophobic and moderate hydrophobic part of the gradient, with contact angles of 55 degrees to 90 degrees. Percentage inhibition by abciximab was maximal around 60 degrees. CONCLUSIONS: Intermediate hydrophobicity of currently applied stent materials, such as stainless steel, seems optimal in combination with abciximab. However, on hydrophobic and particularly on hydrophilic materials, abciximab is less effective.     

Clinical and experimental studies on the action of ethamsylate on haemostasis and on platelet functions

In a series of tests,the action of ethamsylate on haemostasis and on platelet functions was examined. After oral administration of the drug,a highly significant diminution of the bleeding time and of the blood loss from a standard wound was observed. This effect was demonstrated in healthy individuals and in patients suffering from platelet dysfunctions,and there was a distinct dose relation. There was also an increase of platelet adhesion,of PF 3 availability and of PF 4 release. The action of prostacyclin on the epinephrine induced platelet aggregation could be inhibited by ethamsylate. This offers a possible explanation for some of the effects on hemostasis. In addition,an action of ethamsylate on the platelet membrane was assumed since an in vitro increase of functions of the platelet membrane like PF 3 availability would not be explainable by a possible inhibition of prostacyclin.     

辣椒碱载药纳米微球抗脑胶质瘤作用的实验研究

目的探讨聚己内脂-聚乙二醇二嵌段共聚物(m PEG-PCL)制备的辣椒碱纳米微球的理化性质,通过实验评价其抗胶质瘤效果。方法通过开环聚合法和溶剂分散法制备负载辣椒碱的纳米微球,应用负载荧光素纳米粒子的肿瘤细胞摄取实验以及辣椒碱纳米微球的细胞毒性实验。结果实验显示载药微球具有缓释特性,细胞可通过胞吞作用将纳米微球摄入,负载NIR-797纳米微球可以很好地透过血-脑屏障,辣椒碱纳米微球对U251细胞的生长有明显的抑制作用。结论 m PEG-PCL为载体的辣椒碱纳米微球能够有效抑制胶质瘤细胞增殖,为脑胶质瘤药物治疗提供了一条极有前途的治疗途径。