老年心肌肥厚患者心肌淀粉样变情况及对心功能指标和预后的影响研究

目的 探究老年心肌肥厚患者心肌淀粉样变(CA)情况及对血清N末端B型钠尿肽前体(NT-proBNP)、肌钙蛋白T(cTnT)、免疫球蛋白M(IgM)和预后的影响。方法 选取黄冈市中心医院收治的220例老年心肌肥厚患者,进行超声及组织病理检查,统计患者CA发生情况,将伴CA患者纳入CA组37例,不伴CA患者纳入非CA组183例,比较2组患者血清NT-proBNP、cTnT、IgM水平,随访1年,存活123例(生存组),死亡97例(死亡组)。结果 CA组血清NT-proBNP、cTnT水平高于非CA组,IgM水平低于非CA组(P<0.01);死亡组患者心功能Ⅲ～Ⅳ级、伴有CA、NT-proBNP、cTnT水平高于生存组,LVEF及IgM水平低于生存组(P<0.05,P<0.01)。Cox风险回归模型结果显示,心功能Ⅲ～Ⅳ级、伴有CA、血清NT-proBNP及cTnT升高,IgM降低是老年心肌肥厚患者死亡的独立影响因素(P<0.05,P<0.01);随访1年,CA组37例患者存活12例,1年生存率32.43%,中位生存时间9个月(95%CI:7.67～10.33...     

心脏磁共振心肌收缩分数评估老年心肌淀粉样变性患者预后的价值

目的 探讨心脏磁共振心肌收缩分数(MCF)评估老年心肌淀粉样变性患者预后的价值。方法 回顾性分析在我院确诊的老年心肌淀粉样变性患者54例,所有患者均进行心脏磁共振检查,根据患者临床结局分为存活组25例和死亡组29例。评估患者MCF与心脏磁共振参数及生化指标的相关性,Cox回归分析影响患者生存的独立预测因素,生存分析评价MCF预测患者预后的价值。结果 存活组MCF显著高于死亡组[(70.63±24.72)%vs(43.59±13.36)%,P=0.001]。随着MCF增加,LVEF水平呈上升趋势,左心室质量指数、舒张期最大左心室壁厚度、细胞外间质容积分数、肌钙蛋白T、N末端B型钠尿肽前体水平呈下降趋势。多因素Cox回归分析显示,MCF是影响患者生存的独立危险因素(HR=0.922,95%CI:0.866～0.981,P=0.011)。Kaplan-Meier曲线显示,MCF>57%的患者存活率显著高于MCF≤57%的患者(P<0.01)。结论 MCF是评估老年心肌淀粉样变性患者预后的有效影像学指标,可为识别高危患者及指导临床治疗提供帮助。     

心脏淀粉样变性的诊疗现状

心脏淀粉样变性(CA)是由于错误折叠的蛋白在心肌沉积引起的。这种淀粉样蛋白沉积可以单纯出现于心脏,也可于系统性淀粉样变性中受累而被发现。对高度可疑CA的心功能不全患者进行鉴别诊断以及确诊后对淀粉样沉积物进行精确生化分型至关重要。其中,以甲状腺素转运蛋白型(TTR)及单克隆免疫球蛋白轻链(AL)型的CA在临床上最为常见。本文旨在阐述CA诊断和治疗现状。     

原发型心肌淀粉样变性的诊断及治疗研究进展

<正>原发型心肌淀粉样变性(CA)是错误折叠的免疫球蛋白轻链在心肌的细胞外空间沉积,导致心脏功能障碍的恶性浆细胞疾病[1～4]。在美国,原发型CA每年的发病率约为1/100 000或2 500～5 000例新发病例[5]。我国尚无明确的流行病学统计。原发型CA早期以心脏舒张功能障碍为主,随着疾病的进展,最终表现为心脏的收缩功能障碍。心功能不全的严重程度是发病率和死亡率的主要决定因素[6]。原发型CA发病较晚,患者多为中老年人,平均年龄为65岁     

19例心肌淀粉样变性患者的临床特点及预后分析

目的:探讨心肌淀粉样变性(CA)患者的临床特征和预后。方法:回顾性分析19例CA患者的临床资料,对患者的生存影响因素进行相关性和Cox回归分析。结果:19例患者均出现心功能不全表现;肢体导联低电压10例;心脏核磁共振显像心肌呈不同程度延迟强化11例;就诊前有晕厥病史3例;19例患者中死亡9例,平均随访时间(24.2±4.5)个月,1年生存率为67.7%,2年生存率为56.4%,中位生存期为25.1个月。相关性分析表明,患者生存时间与入院时氨基末端B型脑钠肽前体(NT-proBNP)水平、晕厥病史之间存在负相关(r=-0.635,P=0.013;r=-0.632,P=0.002);单因素Cox回归分析,入院时NT-proBNP水平为CA预后的预测指标(P0.05)。结论:CA预后不佳,生存时间与入院时NT-proBNP水平、晕厥病史密切相关,入院时NTproBNP水平是CA患者预后的预测指标。     

心肌淀粉样变诊断进展

心肌淀粉样变（Cardiacamyloidosis,CA）是系统性淀粉样变性最常见的临床表现之一,临床上常以合并症或者单独发病的形式被发现。淀粉样变性可累及许多器官并最终导致器官衰竭,累及心脏时预后极差,因此有淀粉样变性疾病的患者均应排除CA。CA系淀粉样蛋白质物质沉积在心肌组织内,改变细胞代谢、钙转运、受体调节和细胞水肿等所致的一种限制性心肌病。CA较为少见,且临床表现多样化及缺乏特异性,易将其漏诊或误诊为肥厚型心肌病或其他病因的限制性心肌病。     

心肌淀粉样变性预后影响因素分析

  目的 分析心肌淀粉样变性患者临床表现、诊断治疗及预后情况。方法 回顾性分析1995—2005年18例经心内膜心肌活检（EMB）确诊为心肌淀粉样变性患者的临床特征及预后情况。结果 18例心肌淀粉样变性患者均存在心脏舒张功能下降,其中12例二尖瓣舒张早期血流峰速度/舒张晚期血流峰速度（E/A）>2.0,且心室舒张早期充盈减速时间（DT）<150 ms;12例存在心脏收缩功能受损,左室射血分数（LVEF）<50%,13例纽约心脏病学会心功能分级（NYHA）Ⅲ、Ⅳ级。18例心肌淀粉样变性患者1、2和5年生存率分别为67%、44%和17%。 Kaplan-Meier分析显示NYHA>Ⅱ级、E/A>2.0且心室舒张早期充盈减速时间（DT）<150 ms与病死率增加相关（log-rank P=0.026和 0.001）。心力衰竭前接受化疗者有生存期延长趋势。结论 随着发病时间延长,患者生存率逐渐下降,病死率上升。NYHA>Ⅱ级、E/A>2.0且DT<150 ms与病死率增加相关。        

多发性骨髓瘤合并心肌淀粉样变性诊治进展

多发性骨髓瘤（MM）是一种浆细胞异常增殖的恶性肿瘤,也叫浆细胞骨髓瘤,是由骨髓中异常增殖的浆细胞增生并分泌单克隆免疫球蛋白和（或）轻链（M蛋白）而导致相关的组织器官损伤。异常单克隆免疫球蛋白的轻链片段所形成的淀粉样物质在组织器官沉积而导致淀粉样变性。其中多发性骨髓瘤所致淀粉样变性中最重要的损害是心肌淀粉样病变,此类患者的发病机制复杂,心脏受损的临床表现具有多样性,常合并有其它多种组织及器官受累表现且缺乏特异性,容易误诊。本文拟对多发性骨髓瘤合并心肌淀粉样变性诊治进展做一综述。     

心肌淀粉样变的诊疗进展

心肌淀粉样变（CA）是淀粉样蛋白沉积并引起器官结构和功能障碍的一组疾病,其自然病程、累积器官和治疗随淀粉样蛋白来源的不同而有较大变化,主要有轻链型心肌淀粉样变（AL-CA）和转甲状腺素蛋白型心肌淀粉样变（ATTR-CA）2类。前者源于异常免疫球蛋白轻链,常累及心脏和肾脏;后者源自肝脏合成的转甲状腺素蛋白,又分为基因突变型（ATTRm-CA）和野生型（ATTRwt-CA）,除心脏外,也累及软组织及周围神经。CA的临床主要表现为左心室不大、射血分数保留的心力衰竭,且心脏受累及程度与患者预后具有显著相关性。组织活检是诊断CA,尤其是AL-CA的金标准。无创影像学技术是早期发现和诊断CA的重要手段,尤其心脏核素显像已被用于诊断ATTR-CA。CA的治疗包括心力衰竭的对症处理,以及靶向治疗淀粉样蛋白的产生过程。     

心肌淀粉样变性的临床特点分析

目的:分析心肌淀粉样变性患者的临床特点,以提高心肌淀粉样变性的早期诊断和治疗水平,提高患者生存率。方法:回顾性分析我院2015-01-2018-04行腹部、肾脏、心内膜等病理活检确诊的27例心肌淀粉样变性患者的临床表现、心电图、超声心动图、心脏磁共振(MRI)特点及治疗效果。结果:心肌淀粉样变性患者发病年龄晚,临床表现不一,以胸闷气促、下肢水肿等表现多见,常累及多个系统。心电图以肢体导联低电压、胸前导联R波递增不良及病理性Q波为主要表现;超声心动图表现为心房增大、心室壁增厚及收缩、舒张功能受限;心脏MRI表现为心室壁延迟强化。结论:心肌淀粉样变性患者缺乏特异性临床表现,诊断困难,治疗效果欠佳,预后差。因此,对于原因不明的心功能不全患者,高度怀疑心肌淀粉样变性时,在未进行心内膜活检情况下,应根据心电图、心脏超声及心脏MRI等检查及心外组织活检明确诊断,为早期诊断和治疗提供帮助。     

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.     

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.     

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.     

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.     

Changes in connexin43, the gap junction protein of astrocytes, during development of the rat pineal gland

Abstract: The abundance of gap junctions between rat pineal astrocytes formed by connexin43 (Cx43) was studied during development. Levels and distribution of Cx43 were measured by immunoblotting and indirect immunofluorescence, respectively. The amount of Cx43 in cells located within the gland was low until about the 7th postnatal day and increased to adult values between the 14th and 21st days postpartum. Although astrocytes, recognized by their vimentin immunoreactivity, were scarce before birth, they were abundant by the 7th postnatal day suggesting that the low levels of Cx43 found at this age corresponded to a low expression of this protein. Localization of the immunoreactivity to Cx43 and vimentin showed a close correlation, indicating that mature or immature pineal astrocytes form gap junctions made of Cx43. Since Cx43 levels attained their adult values at about the time the innervation and the functional state of the gland reached maturity (2–3 weeks after birth), it is proposed that astrocyte gap junctions are involved in the function of the adult rat pineal gland.     

Conservative management of perforated duodenal diverticulum： A case report and review of the literature

Duodenal diverticula are a relatively common condition. They are asymptomatic, unless they become complicated, with perforation being the rarest but most severe complication. Surgical treatment is the most frequently performed approach. We report the case of a patient with a perforated duodenal diverticulum, which was diagnosed early and treated conservatively with antibiotics and percutaneous drainage of secondary retroperitoneal abscesses. We suggest this method could be an acceptable option for the management of similar cases, provided that the patient is in good general condition and without septic signs.