THE CLINICAL AND NEURO-ELECTROPHYSIOLOGIC STUDY ON CORTICOBASAL DEGENERATION

目的：探讨皮质基底节变性(CBD)患者的临床和神经电生理特征。方法：对29例临床诊断为CBD患者，系统分析其临床症状、体征、常规化验检查、脑CT／MRI、SPECT以及神经电生理改变，并与其它疾病相对比。结果：结果显示，29例患者均出现不对称性帕金森样表现，皮层功能受损占79．3％，其它功能障碍占37．9％。21例患者脑CT或MRI显示不对称性脑萎缩，SPECT显示额顶区低灌注表现，以受损肢体的功能区较明显。EEG显示19例患者出现不对称性慢波，伴有肌阵挛的15例病人，表面肌电图显示患肢随意运动时可诱发巨大电位，皮层反应增强，SEP无特殊变化，运动诱发电位检查，26例患者中枢运动传导时间正常。结论：CBD是一种累及皮层和基底节、病程以缓慢进展为特征的变性性疾病。大部分患者可出现帕金森综合征样表现，皮层及其他运动功能障碍。脑影象学显示额顶叶萎缩。神经电生理检查表现为皮层兴奋性增强，而锥体束传导功能正常。     

Clinical characteristics of rapidly progressive leuko-araiosis

Introduction – 38 patients found to have either pure leuko-araiosis (LA) or LA combined with infarction(s) on computer tomography (CT) in 1989 were re-examined in 1992 in order to evaluate the progression of LA. The follow-up period averaged 3.2 years. Material and methods - The clinical and radiological data on patients in 1989 were collected from hospital records and re-evaluated. The patients were re-examined clinically (including 24 hour ambulatory blood pressure measurement), and neuroradiologically (CT) in 1992 for this study. Results – 11 (29%) patients were found to have significant (rapid) progression of the extent of LA on CT during the follow-up. At baseline, there was no significant difference in the mean number of brain infarctions between the groups with progressing (prLA) and non-progressing LA (nprLA) or between the number of cortical and central infarctions within these groups. At follow-up, the total number of infarctions had increased significantly in both groups, but it was mostly because of the increase in cortical infarctions in the prLA group (p = 0.043) and, conversely, the central ones in the nprLA group (p = 0.011). prLA was found to be related to heart failure (82% vs 37%, p = 0.029) and atrial fibrillation (55% vs 19%, p = 0.047), whereas nprLA was strongly associated with a sudden onset of symptoms (78% vs prLA 18%, p = 0.001) like a-true brain infarction. Other clinical factors, including mean blood pressure and heart rate, did not clearly differentiate between the groups. Conclusion - The results suggest that there are different subgroups of patients with LA associated with various vascular factors. The occurrence of LA is not related to the distribution of infarctions. The progression of LA is not related to the number of brain infarctions or to the simultaneous increase of infarctions on CT.     

Brain stem tuberculoma in adult patients: Diagnosis and treatment

A consecutive series of six adult patients ranging in age from 29 to 53 years is presented. The clinical and radiological features in each patient are described. Attention is drawn to the features demonstrated on computed axial tomography. In only one patient, the first encountered, was surgical excision undertaken and histological verification obtained. One patient died before any form of treatment could be instituted. The remaining four patients were treated with antituberculous chemotherapy alone and their progress monitored by sequential computed tomography. The excellent response and good outcome in this conservatively treated group are documented.     

各型成人高原心脏病的临床特点

本文报告了303例各型成人高原心脏病的临床分析结果。成人高原心脏病单纯型比混合型具有发病急、心脏传导系统损害重、病变累及全心及治疗反应较好的特点。混合型系继发于高原红细胞增多症与高原高血压的慢性心脏损害。其消化系统与神经系统损害重。心高型以左心损害为主,左室扩大、左室肥厚、主动脉增宽弯曲及心律失常率分别高于心红型(P<0.05～0.01);心红型以右心损害为主,右室扩大、右室肥厚、肺动脉段与圆锥隆突率分别高于心高型(P<0.05～0.01);心红高型全心损害常见而且严重。根据各型成人高原心脏病的病理机制而采用不同治疗方法对提高该病的治愈率有重要意义。     

海洛因依赖者支气管哮喘的临床特点及防治

目的：探讨海洛因依赖者支气管哮喘临床特点，为防治和治疗提供科学根据。方法：2004年1月至2005年1月1752例海洛因依赖者中伴发哮喘40例与随机抽取同期戒毒的100例患者进行回顾性分析与对照。结果：研究组病例的日滥用量、吸毒方式、吸毒年限与对照组病例相比均呈显著差异（P〈0．01）。研究组中：轻度发作24例（60％）；中度发作12例（30％）；重度发作3例（7．5％）；危重度1例（2．5％），哮喘多出现在脱毒中期；在有效脱毒的基础上，抗炎平喘，结合心理治疗与干预，轻度发作者多在4小时内缓解，中度发作者多在48小时内缓解，重度及危重度发作者多在3～5天内缓解。结论：长期大剂量烫吸海洛因是支气管哮喘发作的危险因素。应完善相关检查、及时发现，积极有效抗炎平喘，制定合理治疗方案，注意心理因素的影响。更重要的是，对海洛因依赖者进行教育，使他们了解哮喘知识、戒除毒品，才能从根本上预防和治疗此类并发症。     

Modulation of glial cell signaling by adenosine and pharmacological reinforcement

In view of the increasing evidence that a pathological glial activation plays a significant role in the development of neurodegenerative diseases, we investigated the underlying molecular signaling as a possible target for a pharmacological therapy. Here, we are particularly focusing on the endogenous modulation of the Ca²⁺ and cyclic nucleotide-dependent signaling by the nucleoside adenosine and its reinforcement by the xanthine derivative propentofylline (PPF). As an experimental model, we used cultured rat microglial cells and astrocytes that are immature, show a high proliferation rate, and resemble in several aspects pathologically activated glial cells. A prolonged increase of the cellular cAMP level favored the differentiation of cultured astrocytes and associated properties required for the physiological nerve cell function. On the other hand, a strengthening of the cyclic nucleotide-dependent signaling inhibited potentially neurotoxic properties of cultured microglial cells. Similar effects were obtained by treatment with propentofylline, which mimicked modulatory adenosine effects and increased the intracellular level of cAMP and cGMP. Such a pharmacological glial cell conditioning, obtained by modifying the strength and the timing of these second messengers, may provide a therapy of neurodegenerative diseases in which a pathological activation of microglial cells and astrocytes is discussed to play a pathogenic role.     

急性脑梗死患者血清CTRP-3、D-二聚体、sTREM2水平及相关临床特征与溶栓后出血性转化的关系

目的 探讨急性脑梗死患者血清补体C1q/肿瘤坏死因子相关蛋白3（CTRP-3）、D-二聚体、可溶性髓样细胞触发受体2（sTREM2）水平及相关临床特征与溶栓后出血性转化（HT）的关系。方法 回顾性分析2018年9月—2022年9月在青海省人民医院接受溶栓治疗的120例急性脑梗死患者的临床资料,根据患者溶栓后是否发生HT分为HT组（30例）、非HT组（90例）。比较两组患者的临床资料及血清CTRP-3、D-二聚体、sTREM2水平。采用多因素逐步Logistic回归分析急性脑梗死患者溶栓后发生HT的危险因素;绘制受试者工作特征（ROC）曲线,分析急性脑梗死患者溶栓后HT预测模型预测HT发生的价值。结果 HT组心房颤动（以下简称房颤）、大面积脑梗死、入院NIHSS评分≥ 15分占比高于非HT组（P <0.05）,血清CTRP-3水平低于非HT组（P <0.05）,D-二聚体、sTREM2水平高于非HT组（P <0.05）。血清CTRP-3、D-二聚体、sTREM2水平预测急性脑梗死患者溶栓后发生HT的敏感性分别为66.7%（95% CI：0.598,0.756）、70.0%（95% CI：0.607,0.812）、80.0%（95% CI：0.714,0.889）,特异性分别为73.3%（95% CI：0.636,0.821）、86.7%（95% CI：0.778,0.923）、86.7%（95% CI：0.747,0.942）。多因素Logistic逐步回归分析结果显示,房颤［O^R=1.237（95% CI：1.103,1.387）］、大面积脑梗死［O^R=2.338（95% CI：1.292,4.231）］、入院NIHSS评分≥ 15分［O^R=2.087（95% CI：1.231,3.538）］、CTRP-3 ≤ 269.265 μg/L ［O^R=3.006（95% CI：1.508,5.992）］、D-二聚体≥ 2.625 mg/L ［O^R=2.649（95% CI：1.374,5.107）］、sTREM2 ≥ 314.675 ng/L ［O^R=2.328（95% CI：1.411,3.841）］是急性脑梗死患者溶栓后发生HT的危险因素（P <0.05）。根据多因素Logistic逐步回归分析结果建立急性脑梗死患者溶栓后HT预测模型,Logit（P） = -33.887 + 0.213×房颤+ 0.849×大面积脑梗死+0.736×入院NIHSS评分+ 1.101×CTRP-3 + 0.974×D-二聚体+ 0.845×sTREM2;ROC曲线分析结果表明,预测模型预测HT发生的敏感性为93.3%（95% CI：0.841,0.991）,特异性为87.8%（95% CI：0.808,0.976）。结论 血清CTRP-3、D-二聚体、sTREM2水平与急性脑梗死患者溶栓后HT有关,预测价值较高,且急性脑梗死患者溶栓后HT预测模型预测HT优于各项指标单独预测。     

儿童急性白血病伴侵袭性肺部真菌感染的临床特点、真菌分布及影响因素分析

目的 分析儿童急性白血病伴侵袭性肺部真菌感染（IPFI）的临床特点、真菌分布及影响因素。方法 回顾性分析2018年1月—2022年12月安徽省儿童医院82例急性白血病患儿的临床资料,按照是否合并IPFI分为IPFI组（18例）和非IPFI组（64例）。比较两组患儿的一般临床资料;采用多因素一般Logistic回归模型分析儿童急性白血病伴IPFI的危险因素;分析IPFI组患儿的临床特征、真菌菌种分布。结果 18例IPFI患儿均有不同程度的发热,体温>38.5℃占比72.22%,咳嗽咳痰/白色黏痰占比83.33%;18例IPFI患儿中5例表现为结节实变影,7例表现为多发斑片状阴影,8例表现为散在斑片状阴影合并小结节,2例表现多发云雾状毛玻璃样高密度影、间质病变为主。16例获得真菌微生物学证据,血培养2例,肺泡灌洗液涂片1例,血或者肺泡灌洗液NGS检测13例,其中以毛霉菌（31.25%）、曲霉菌（25.00%）、近平滑假丝酵母菌（18.75%）为主;多因素一般Logistic回归分析结果显示,化疗方案含激素■、中性粒细胞缺乏时间≥10 d[■]、抗菌药物使用种类≥2种■均是儿童急性白血...     

肝脏子宫内膜异位症一例

肝脏子宫内膜异位症是以肝内存在异位子宫内膜为特征的一种罕见子宫内膜异位症类型,因其缺乏典型临床症状且影像学诊断困难,易被误诊,组织学检查目前仍是肝脏子宫内膜异位症诊断的金标准。现报告1例海军军医大学第三附属医院收治的患者,反复经期右上腹疼痛,经超声检查发现右肝占位性病变,术后病理证实为肝脏子宫内膜异位症。     

汗腺癌病理分级的预后意义

目的：探讨汗腺癌不同病理组织学分级对复发和转移及预后的影响。方法：参照Ｂｌｏｏｍ－Ｒｉｃｈａｒｄｓｏｎ乳腺癌分级标准对１０５例汗腺癌进行分级，并探讨其与复发、转移和预后间的关系。结果：在１０５例汗腺癌中，属分化型者４２例，属低分化型者６３例，两者复发率、多次复发率、区域淋巴结转移率和远处转移率及死亡率比较，提示在多次复发、远处转移方面差异有显著性，在预后方面差异非常显著。结论：将汗腺癌按腺管形成多少、核的多形性和核分裂数进行病理组织学分级，对选择治疗、估计预后具有很大的指导意义