左心疾病相关性肺动脉高压临床研究进展

肺动脉高压是一大类进展性、致残率和致死率极高的疾病。左心疾病相关性肺动脉高压最为常见,其病情更重、预后更差、病死率更高,严重影响患者生活质量。目前该类肺动脉高压的流行病学数据不清楚、临床诊断标准不统一、病理生理机制不甚明确、分类方法存在争议、靶向药物治疗缺乏证据,国内外相关研究和治疗十分有限。现概述左心疾病相关性肺动脉高压的定义及分类、流行病学、病因及发病机制、病理生理机制,并详细阐述其诊断及特异性治疗的临床研究进展。     

老年性痴呆的治疗

老年性痴呆 ( AD)是一种常见的中枢神经变性疾病 ,由于原因未明和发病机制不清 ,目前尚无特异性治疗方法。临床上采用对症治疗、生物学治疗和对因治疗。生物学治疗包括神经介质替代疗法、神经营养因子、促神经细胞代谢药、神经细胞保护剂及神经移植。目前 ,对症治疗和生物学治疗是 AD治疗的主要方法。1　现行治疗方法评价1.1　对症治疗　各种精神症状如嗜睡、抑制、焦虑、攻击行为甚至成为植物人在 AD中常见 ,治疗中选择各种抗精神病药物是合理的。但是绝大部分抗精神病药物都有副作用 ,甚至使病情恶化。控制精神症状首先试用非药物疗法 …     

1996～2020年北京佑安医院遗传代谢性肝病住院患者流行病学特征分析

目的 了解1996～2020年北京佑安医院住院的遗传代谢性肝病(IMLD)患者的病因构成和变化趋势。方法 1996年1月～2020年12月期间我院收治的IMLD患者,分析疾病分布及患者性别和年龄分布特征。结果 共收治IMLD患者552人次,占住院肝病患者的3.6‰;在实际393例IMLD患者中,男性患病率高于女性,平均年龄为29岁;肝豆状核变性和先天性高胆红素血症分别为223例(56.7%)和136例(34.6%),共占比为91.3%;前后10年对比,近10年较为少见的病种住院人数逐渐增多,发病年龄略有升高(24.9岁对31.4岁,P<0.01),中、老年患者所占比例呈上升趋势。结论 成人IMLD并不罕见,疾病谱逐渐丰富,应该引起肝病医生的重视。     

遗传代谢性肝病患者临床和基因突变特征分析*

目的 总结过去20年洛阳市各大医院诊治的遗传代谢性肝病(IMLD)患者的临床特征。方法 2000～2021年期间洛阳市各大医院诊治的IMLD患者16例,总结、分析其临床特征。结果 在16例IMLD患者中,男性10例,女性6例,平均年龄为(26±15)岁;其中金属代谢障碍性疾病8例(肝豆状核变性7例、遗传性血色病1例),遗传性高胆红素血症3例(Gilbert综合征2例、Dubin-Johnson 综合征1例),遗传性胆汁淤积症4例【良性复发性肝内胆汁淤积症(BRIC) 2例、Alagille综合征1例、Citrin缺乏症1例】和糖原累积病1例;7例肝豆状核变性患者分别来自4个家族,主要为ATP7B基因突变;遗传性血色病为HFE基因突变,Gilbert综合征为UGT1A1基因突变,Dubin-Johnson综合征为ABCC2基因突变,BRIC为ATP8B1基因突变,Alagille综合征为JAG1基因突变,Citrin缺乏症为SLC25A13基因突变,糖原累积病为G6Pase基因突变。结论 IMLD以肝豆状核变性较常见,大多IMLD的诊断需要依靠临床资料综合和基因检测。     

分子靶向药物相关性脂膜炎的研究进展

脂膜炎是一种累及皮下脂肪的非化脓性炎症性疾病，发病原因不明，临床表现多样且缺乏特异性。药物相关的皮疹部分可表现为脂膜炎，但由于对此类疾病的认识不足，目前关于药物相关性脂膜炎的报道不多。随着分子靶向药物在临床治疗中得到越来越广泛地应用，这类药物诱发的脂膜炎越来越多地被观察到。本文从脂膜炎的定义、分子靶向药物相关性脂膜炎的临床表现以及治疗等方面进行综述。     

炎症性肠病基因学研究进展和应用价值

炎症性肠病(IBD)是发生于胃肠道的不明原因慢性非特异性炎症性疾病,其发生可能与肠黏膜免疫应答异常、肠道微生物、肠黏膜屏障损伤、遗传易感性以及环境等因素有关。近年来,IBD基因学研究取得巨大进展,并对临床转化和应用有重要帮助,尤其是能解释疾病发生原因和发病部位、判断疾病行为、预测药物治疗方案的有效性,从而服务于临床诊断和治疗。本文总结既往IBD基因学研究成果和新进展,客观阐述基因学研究在IBD疾病管理中的潜在应用价值。     

药物性肝损害伴自身抗体阳性患者临床特征及转归

随着自身抗体检测技术的提高及临床医帅对自身免疫性肝病认识程度的不断提高,肝功能异常伴自身抗体阳性特别是高滴度自身抗体阳性患者的诊断、鉴别诊断及治疗问题越来越受到临床医师的关注,因为自身抗体阳性虽是诊断自身免疫性肝病的主要条件之一,但在部分病毒性肝炎、药物性肝损害、酒精性及非酒精性脂肪性肝炎中也均可检测到自身抗体,特别是非特异性自身抗体,如抗核抗体、抗线粒体抗体及抗平滑肌抗体等,是一种疾病伴随自身免疫现象还是两种疾病共同存在,如何诊断及鉴别诊断,有时成为摆在临床医师面前较棘手的问题,也是临床亟待解决的问题,此节主要讨论药物性肝损害患者检测出自身抗体时如何诊断及治疗,是药物诱导了自身免疫样肝炎还是药物伴随自身免疫现象?如何与自身免疫性肝炎鉴别?因自身免疫性肝炎治疗需较长期应用激素及免疫抑制剂,而非自身免疫性肝炎患者短期应用或不应用上述治疗.如上述诊断及鉴别诊断不全面,使治疗不得当,会加重或延误病情,给患者带来痛苦.     

急性偏头痛的处理

多达3 000万美国人患有偏头痛.偏头痛对患者及其家庭的影响巨大,而对偏头痛的治疗又使家庭医生面临着诊断和治疗的难题.中止发作的治疗包括非特异性和特异性治疗.非特异性治疗包括镇痛药(阿司匹林、非甾体类消炎药和阿片类药物)、辅助治疗(止吐药和镇静药)和其他非特异性药物治疗(经鼻腔给利多卡因或皮质类固醇).偏头痛特异性中止发作治疗的药物包括麦角胺及其衍生物和曲坦类药.补充和替代治疗也可用来中止头痛发作或增强另一种用药程式的疗效.偏头痛急性发作期的治疗选择应以头痛严重程度、发作频率、伴随症状和共患疾病为依据.     

变应原特异性免疫治疗的研究进展

变应原特异性免疫治疗(Allergen-specific immunotherapy, ASIT)是现阶段唯一针对病因治疗且能长时间持续减轻过敏症状,改变自然病程的治疗方法。目前已在过敏性疾病的临床治疗中广泛应用,并取得良好的治疗效果。随着治疗药物和治疗途径的不断改良,ASIT在过敏性疾病的治疗中显示出不可比拟的优势。本文对ASIT作用机制、应用药物、给药途径和禁忌症方面进行综述,旨在为ASIT的临床治疗提供参考。     

6789例布鲁氏菌病患者的临床疗效分析

一个多世纪以来,国内外许多学者对布鲁氏菌病(以下简称布病)的治疗进行了广泛而深入的研究。笔者主要采用了抗菌方法、特异性抗原疗法和中医中药疗法对6789例布病患者进行了临床观察,结果如下:1 治疗药物分组与治疗方法1.1 链霉素十四环素类(四、金、土)或磺胺类及     

Three layer functional model and energy exchange concept of aging process

Relying on a certain degree of abstraction, we can propose that no particular distinction exists between animate or living matter and inanimate matter. While focusing attention on some specifics, the dividing line between the two can be drawn. The most apparent distinction is in the level of structural and functional organization with the dissimilar streams of ‘energy flow’ between the observed entity and the surrounding environment. In essence, living matter is created from inanimate matter which is organized to contain internal intense energy processes and maintain lower intensity energy exchange processes with the environment. Taking internal and external energy processes into account, we contend in this paper that living matter can be referred to as matter of dissipative structure, with this structure assumed to be a common quality of all living creatures and living matter in general. Interruption of internal energy conversion processes and terminating the controlled energy exchange with the environment leads to degeneration of dissipative structure and reduction of the same to inanimate matter, (gas, liquid and/or solid inanimate substances), and ultimately what can be called ‘death.’ This concept of what we call dissipative nature can be extended from living organisms to social groups of animals, to mankind. An analogy based on the organization of matter provides a basis for a functional model of living entities. The models relies on the parallels among the three central structures of any cell (nucleus, cytoplasm and outer membrane) and the human body (central organs, body fluids along with the connective tissues, and external skin integument). This three-part structural organization may be observed almost universally in nature. It can be observed from the atomic structure to the planetary and intergalactic organizations. This similarity is corroborated by the membrane theory applied to living organisms. According to the energy nature of living matter and the proposed functional model, the decreased integrity of a human body's external envelope membrane is a first cause of the structural degradation and aging of the entire organism. The aging process than progresses externally to internally, as in single cell organisms, suggesting that much of the efforts towards the restoration and maintenance of the mechanisms responsible for structural development should be focused accordingly, on the membrane, i.e., the skin. Numerous reports indicate that all parts of the human body, like: bones, blood with blood vessels, muscles, skin, and so on, have some ability for restoration. Therefore, actual revival of not only aging tissue of the human body's membrane, but the entire human body enclosed within, with all internal organs, might be expected. We assess several aging theories within the context of our model and provide suggestions on how to activate the body's own anti-aging mechanisms and increase longevity. This paper presents some analogies and some distinctions that exist between the living dissipative structure matter and inanimate matter, discusses the aging process and proposes certain aging reversal solutions.     

Unusual responses of nocturnal pineal melatonin synthesis and secretion to swimming: Attempts to define mechanisms

Abstract: The effect of swimming at night on rat pineal melatonin synthesis was compared with that of light exposure at night. Rats were forced to swim at 0030 hr (lights out at 2000 hr) and sacrificed by decapitation 15 and 30 min later, immediately after swimming. Other groups of animals were exposed to white light (650μW/cm²) for 15 and 30 min at same time. Swimming caused a rapid and highly significant drop in the melatonin content in the pineal gland; however, the activity of N-acetyltransferase (NAT), the supposed rate limiting enzyme in the melatonin production, was not changed. Despite the drop in pineal melatonin levels, serum concentrations of the indole remained elevated in the rats that swam. In contrast, melatonin levels in the pineal and serum of light exposed rats fell precipitously, accompanied by a significant suppression of NAT activity. Since we anticipated that the strenuous exercise associated with swimming may induce release of artrial natriuretic peptide (ANP) from the heart, which in turn could cause the release of pineal melatonin, in a second study we injected physiological saline intravenously to stretch the cardiac muscle and release ANP. Three milliliters of normal saline was injected during the day into the jugular vein of anesthetized rats that were pretreated with isoproterenol to stimulate pineal melatonin production. Animals were killed 15 min after the saline injection, and pineal NAT activity and pineal melatonin levels were measured. The saline injections caused no alteration in the elevated levels of either NAT or melatonin. These data suggest that the disparity in pineal NAT activity (which was high) and pineal melatonin (which was low), in animals swum at night, may not be caused by ANP which is released during strenuous exercise such as swimming.     

Melatonin and photoperiodic time measurement: Seasonal breeding in the sheep

Abstract: Well-established circadian physiology supports the view that photoperiodic time measurement utilizes the coincidence between the presence of light and a photosensitive phase of a 'biological clock' to alter reproductive status—the so-called external coincidence model of seasonal breeding. In this review, we examine the mechanism whereby photoperiod interacts with presumed suprachiasmatic nuclei activity to allow endogenous melatonin to normally synchronize reproductive activity to the optimal time of year. The Romney Marsh sheep is particularly explored as an experimental model. It is suggested that the on/off activity of seasonal reproduction may be a robust mechanism able to be predictably manipulated by the judicious use of the light/dark cycle and exogenous melatonin, but firmly based on circadian principles.     

The immunoneuroendocrine role of melatonin

Abstract: A tight, physiological link between the pineal gland and the immune system is emerging from a series of experimental studies. This link might reflect the evolutionary connection between self-recognition and reproduction. Pinealectomy or other experimental methods which inhibit melatonin synthesis and secretion induce a state of immunodepression which is counteracted by melatonin. In general, melatonin seems to have an immunoenhancing effect that is particularly apparent in immunodepressive states. The negative effect of acute stress or immunosuppressive pharmacological treatments on various immune parameters are counteracted by melatonin. It seems important to note that one of the main targets of melatonin is the thymus, i.e., the central organ of the immune system. The clinical use of melatonin as an immunotherapeutic agent seems promising in primary and secondary immunodeficiencies as well as in cancer immunotherapy. The immunoenhancing action of melatonin seems to be mediated by T-helper cell-derived opioid peptides as well as by lymphokines and, perhaps, by pituitary hormones. Melatonin-induced-immuno-opioids (MHO) and lymphokines imply the presence of specific binding sites or melatonin receptors on cells of the immune system. On the other hand, lymphokines such as -γ-interferon and interleukin-2 as well as thymic hormones can modulate the synthesis of melatonin in the pineal gland. The pineal gland might thus be viewed as the crux of a sophisticated immunoneuroendocrine network which functions as an unconscious, diffuse sensory organ.     

Response of rat pineal melatonin to calcium, magnesium, and lithium is circadian stage dependent

Abstract: Herein we documented the response of pineal melatonin production to electrolytes known to be effective on pineal function in view of a possible circadian stage dependence. We studied the release of melatonin by perifused rat pineal glands at 2 different circadian stages corresponding to the middle of the light and dark periods, i.e., respectively, 7 and 19 HALO (Hours After Light Onset, L:D = 12:12). The initial efflux rates were, as expected, much higher in the perifusates of glands removed from rats sacrificed during the dark phase than of those removed during the light phase. After 3 hr of perifusion, melatonin release reached similar levels which were found constant up to the 8th hr of perifusion, whatever the circadian stage. Perifusion of the glands with physiological concentrations for the rat of calcium (5.2 mmol/1) and magnesium (1.34 mmol/1) resulted in a stimulatory effect on the pineal glands removed from rats sacrificed in the middle of the dark period (19 HALO), whereas no effects were observed on the pineal glands removed from rats sacrificed during the light (7 HALO). Lithium (0.28 and 0.55 mmol/1) was ineffective on melatonin release in pineal glands removed 7 and 19 HALO. Our results show differences in the initial efflux rates of melatonin and in the response of perifused pineal glands to calcium and magnesium according to the circadian stage.     

Serological survey of HIV and syphilis in pregnant women in Madagascar

Objectives Peripartal transmission of human immunodeficiency virus (HIV) and Treponema pallidum, the causative agent of syphilis, leads to severe consequences for newborns. Preventive measures require awareness of the maternal infection. Although HIV and syphilis testing in Madagascar could be theoretically carried out within the framework of the national pregnancy follow‐up scheme, the required test kits are rarely available at peripheral health centres. In this study, we screened blood samples of pregnant Madagascan women for HIV and syphilis seroprevalence to estimate the demand for systemic screening in pregnancy. Methods Retrospective anonymous serological analysis for HIV and syphilis was performed in plasma samples from 1232 pregnant women that were taken between May and July 2010 in Ambositra, Ifanadiana, Manakara, Mananjary, Moramanga and Tsiroanomandidy (Madagascar) during pregnancy follow‐up. Screening was based on Treponema pallidum haemagglutination tests for syphilis and rapid tests for HIV, with confirmation of positive screening results on line assays. Results Out of 1232 pregnant women, none were seropositive for HIV and 37 (3%) were seropositive for Treponema pallidum. Conclusions Our findings are in line with previous studies that describe considerable syphilis prevalence in the rural Madagascan population. The results suggest a need for screening to prevent peripartal Treponema pallidum transmission, while HIV is still rare. If they are known, Treponema pallidum infections can be easily, safely and inexpensively treated even in pregnancy to reduce the risk of transmission.