Golgi study on macular mutant mouse after copper therapy

Summary This study was undertaken to elucidate the clinical and neuropathological effects of copper administration on the macular mutant mouse. Its hemizygote, which is considered to be a model of Menkes kinky hair disease (MKHD), was injected intraperitoneally four times with 10, 20, 20 and 30 g of cupric chloride on days 4, 6, 8 and 10, respectively. The hemizygote's curly whiskers gradually straightened and the frequent tonic seizures and ataxia disappeared after the injections. The body weight also gradually increased. In the cerebral cortex, the dendritic arborization of the pyramidal neurons in both the normal littermate and the treated hemizygote developed with time and reached the maximum around day 60. In the treated hemizygote, however, the arborization of the dendrites was significantly poor in comparison with that in the normal littermate from day 20 to 90. In the cerebellum of the treated hemizygote, the abnormal Purkinje cells with the few somal sprouts, thick stem dendrite and/or poor arborization, which were seen in the non-treated hemizygote, were improved by day 30, while their focal dendritic swellings remained even on day 60. These results indicate that the copper therapy improves not only the clinical manifestations but also the neuropathological changes, especially in the cerebellum.Supported in part by Grant no. 86-05-02 from the National Center of Neurology and Psychiatry of the Ministry of Health and Welfare, Japan     

Prospect for enzyme therapy in glycogenosis II variants: a study on cultured muscle cells

Summary Impairment of skeletal muscle function is the common feature of distinct clinical forms of glycogenosis type II. In the present study, muscle cultures from different patients were used to investigate the cause of clinical heterogeneity and the feasibility of enzyme replacement therapy. The activity of acid -glucosidase appears to be the primary factor in determining the extent of lysosomal glycogen storage in muscle, and thereby the clinical severity of the disease. Neutral -glucosidases do not seem influencial. Correction of the enzymatic defect was achieved in skeletal muscle cultures from patients by administration of a high-uptake form of acid -glucosidase, purified from human urine. The enzyme reaches the lysosomes, including the glycogen storage vacuoles, and the lysosomal glycogen content is reduced to control level. In normal muscle cells 20% of the total cellular glycogen pool is segregated in lysosomal compartments. This percentage is higher than in fibroblasts, which may partly explain why muscles are more prone to store glycogen. The relevance of this study for enzyme therapy is discussed.     

11项心功能指标的计算方法及微机处理

本文用OMEGA 500型Υ相机和MCS 560计算系统,即SPECT,R波门控采集心血池图象,在此基础上进行曲线分析,编制BASIC程序计算11项心功能指标。临床应用结果表明:PFR、LVFF、FFR是舒张期功能的优选指标,其它指标对心功能评价也都有显著意义。     

Postoperative radiotherapy and radiotherapy combined with CCNU chemotherapy for treatment of brain gliomas

A prospective, randomized trial evaluates the effects of two postoperative treatment regimens on survival in 198 adult patients with supratentorial gliomas. All patients were irradiated with 6 000 rads after possibly radical removal of tumors. CCNU administration in the dosis of 100 mg/sq m of body surface every 6–8 weeks following surgery proved to have no significant effect on the survival of patients. The median survival time in patients receiving radiation therapy alone was 61±7 weeks, while in those receiving additional chemotherapy was 56±4 weeks. Tumor histological malignancy and patients age were found to be the only important prognostic factors, irrespective of the treatment modality. Address for offprints: T Trojanowski, Department of Neurosurgery, Medical School, Jaczewskiego 8, 20-950 Lublin, Poland     

冠心灵对犬心脏血流动力学和耗氧量的影响

冠心灵对健康麻醉开胸犬可减慢心律,降低血压,降低左室收缩压,减少dp/dtmax,减少左室作功指数,降低总外周血管阻力和冠脉阻力。冠心灵降低心肌耗氧量,降低心肌耗氧指数,而对心肌氧利用率影响不大。冠心灵保护缺血心肌的原理,可能在于降低心肌耗氧、耗能量。     

靶向B细胞治疗系统性红斑狼疮研究进展

系统性红斑狼疮(Systemic lupus erythematosus, SLE)是一种慢性、全身多系统受累的自身免疫性疾病。其病因和发病机制尚未完全明确,有研究表明B细胞过度增殖、活化是SLE发病至关重要的环节,也是造成全身多脏器损害的病理基础。因此B细胞成为SLE治疗的新型靶点,本综述主要围绕B细胞相关靶向治疗进展进行讨论,包括靶向B细胞表面抗原、靶向B细胞因子、共刺激阻断、酪氨酸蛋白激酶抑制剂等方面,重点介绍其已经结束或正在进行的临床试验以及实际上市后的临床应用。     

接受抗病毒治疗HIV/AIDS患者艾滋病心理痛苦现状及影响因素

目的：调查接受抗病毒治疗的HIV/AIDS患者艾滋病心理痛苦现状及影响因素。方法：2022 年3月—2022年6月,采用便利抽样法从北京地区艾滋病社会组织招募765例HIV/AIDS患者进行在线调查,调查内容为一般资料调查表和艾滋病心理痛苦量表。采用χ2检验、Fisher确切概率法和Logistic回归分析艾滋病心理痛苦的影响因素。结果：本次调查共收集有效数据765 份,被调查HIV/AIDS患者艾滋病心理痛苦检出率83.27%。Logistic回归分析结果表明没有固定工作（OR =5.004,95%CI =1.867～13.410,P =0.001）、接受抗病毒治疗1～3 年（OR =2.613,95%CI =1.354～5.044,P =0.004）、不按时服药（OR =31.277,95%CI =4.143～236.092,P =0.001）是艾滋病心理痛苦发生的危险因素,而已婚（OR =0.320,95%CI =0.187～0.550,P ＜0.001）、坚持使用安全套（OR =0.267,95%CI =0.127～0.563,P =0.001）、确诊时间长（OR =0.115,95%CI =0.024～0.550,P =0.007）、月收入＞1 0000元（OR =0.265,95%CI =0.095～0.737,P =0.011）是保护性因素。结论：接受抗病毒治疗的HIV/AIDS患者普遍存在艾滋病心理痛苦,其发生率较高,医护人员应重视艾滋病心理痛苦的评估,并及时对存在危险因素的患者进行干预,以期提高患者的生活质量。     

成人斯蒂尔病1例

成人斯蒂尔病起病原因及发生机制仍不清楚,临床主要表现为发热、关节痛、皮疹,且有白细胞、血清铁蛋白等指标增高,但缺乏针对该病的特定诊断指标,导致临床漏诊、误诊比率较高。通过查阅相关文献,发现本病内镜下报道资料较少。本文就1例成人斯蒂尔病病例及胃镜下表现进行报道,并结合AOSD相关诊断指标、治疗方面的最新进展进行归纳,旨在为临床诊疗本病寻求更精准的选择。     

急性冠脉综合征患者强化抗栓治疗研究进展

急性冠脉综合征（ACS）是冠心病的严重类型,ACS患者不仅病死率较高,还存在缺血事件（如缺血性卒中、心肌梗死）复发风险。血小板聚集及血栓形成是导致ACS的重要原因。为降低缺血事件的发生风险,临床推荐ACS患者接受阿司匹林联合强效P2Y12抑制剂的双联抗血小板治疗12个月。然而在标准双联抗血小板治疗下,ACS患者残余缺血风险（经抗栓治疗后仍残留的缺血事件发生风险）仍旧较高。因此为进一步降低缺血事件发生风险,临床对强化抗栓方案的研究也逐渐增多。本文通过总结强化抗栓治疗方案的作用机制及其最新研究进展,发现延长双联抗血小板治疗时间、三联抗血小板治疗、双通道抑制（抗血小板联合抗凝治疗）等强化抗栓治疗方案可降低缺血事件发生风险,为进一步指导临床个体化抗栓治疗及明确最佳抗栓策略提供了参考。     

老年高血压相关性衰弱的发病机制及运动干预机制的研究进展

随着我国老龄化进程的进一步加剧,衰弱作为常见的老年综合征已成为备受关注的公共卫生问题。高血压与衰弱常共存于老年人,导致出现多种不良健康结局。本文分析了老年高血压相关性衰弱的流行现状及发病机制,包括炎性反应、氧化应激反应、胰岛素抵抗及激素代谢紊乱等,总结了运动干预改善高血压相关性衰弱的可能作用途径,并发现目前关于高血压相关性衰弱的运动作用机制类研究仍然缺乏,而且不同类型的运动干预形式对机体的作用机制也存在一定差异性,未来可以进一步探讨不同的运动干预形式对高血压相关性衰弱的作用靶点及干预效果的不同。