蓝色橡皮大疱痣综合征34例临床分析

目的 总结分析蓝色橡皮大疱痣综合征(BRBNS)的临床特征.方法 回顾性分析2001年来收治的4例和国内文献报道的30例BRBNS患者的临床资料,总结比较BRBNS的临床表现、家族史、内镜和影像学检查、病变部位、治疗方式、随访情况等.结果 BRBNS患者男女比例为1.8∶1,中位年龄为19.5岁.33例(97.1％)患者有消化道出血表现,发现消化道出血的中位年龄为9.0岁,其中9例(27.3％)以贫血为首发症状,1例并发肠套叠、肠坏死,同时伴有腹痛.2例患者有家族史.内镜和影像学检查以胃镜(85.3％)、结肠镜(73.5％)检出为主.病变主要累及皮肤(100.0％)和消化道(97.1％),其中在消化道的分布依次为胃(64.7％)、小肠(64.7％)、结直肠(58.8％)、食管(29.4％).治疗方法包括对症治疗、内镜治疗、外科手术等,内镜治疗多选用套扎、电凝电切,其中6例行内镜治疗的患者及4例行外科手术的患者短期随访未再出现消化道出血.结论 BRBNS病变主要累及皮肤和消化道,绝大多数并发消化道出血,内镜治疗和外科手术是目前疗效较好的治疗消化道出血的方式.     

成人皮肌炎合并消化道出血1例

皮肌炎(dermatomyositis,DM)是一种主要累及横纹肌,呈慢性非化脓性炎症改变,并具有特征性皮肤表现的自身免疫性疾病。小儿皮肌炎以广泛的血管炎为病理基础[1],继发消化道出血常见于文献报道。成人皮肌炎消化系统最常受累部位为食管上段骨骼肌,导致吞咽困难[2]。但成人皮肌炎合并严重的消化道病变,如消化道出血、穿孔、腹腔血肿等较为少见。本文报告1例成人DM合并消化道出血的病例,并进行相关文献复习。     

家族群发蓝色橡皮疱痣综合征1例

蓝色橡皮疱痣综合征（blue rubber bleb nevus syndrome, BRBNS）是以皮肤和内脏等多处海绵状血管瘤为表现的临床综合征。从幼儿到老人名年龄段皆可发病，但多数常在出现内脏并发症（如消化道出血，癫痫、咯血、视力障碍等）时才引起重视而就医。据国外资料显示，BRBNS皮肤血管瘤的出现率为100%，胃肠道血管瘤为74%，中枢神经血管瘤为17%，关节和肝血管瘤各为5%，眼内、肾、脾血管瘤各为2%，消化道血管瘤的分布；小肠100%，结肠74%，胃65%，本期刊出的2例病例报道，1例为直肠黏膜血管瘤，1例为食管黏膜血管瘤。但此2例均未作小肠镜检查。由于本病少见，且单纯以皮肤血管瘤就诊者很少，这就要求临床医师引起重视，仔细进行体格检查，一旦发现皮肤血管瘤（即使是不明显的1-2个），也需对重要脏器，如消化道，中枢神经系统等进行相应的检查。本期刊出的1例反复便血5年余，此次住院体检时发现右侧足背，右下腹，有腰部和肛周均有多个血管瘤，才确诊BRBNS，有关BRBNS消化道血管瘤的治疗，肠段血管瘤密集的需外科切除，分散，孤立病灶则应内镜下激光成硬化剂治疗。我们希望能对这2例患者进行消化道出血复发的随访，以及对血管瘤进行治疗的观察。[编者按]     

4例Peutz-Jeghers综合征诊治分析

<正>Peutz-Jeghers综合征(PJS)又称黑色素斑-胃肠多发性息肉症。本病为常染色体显性遗传病,特点是特定部位黏膜、皮肤色素沉着以及全胃肠道多发性息肉,据文献报道40%的患者有家族史。息肉病检为错构瘤,P-J息肉属于癌前病变,部分息肉可经不典型增生发展为癌,其肿瘤的发生率约为2%~3%[1,2]。息肉可发生于消化道任何部位,以小肠(64%)为主,依次为结肠(53%)、胃(49%)和直肠(32%)[3]。本文报道4例临床的资料并进行文献复习。     

国产胶囊内镜对不明原因消化道出血的诊断价值

[目的]探讨国产OMOM型智能胶囊(OMOM)在不明原因消化道出血(OGIB)患者中的诊断价值。[方法]对36例OGIB患者进行OMOM检查,总结并分析其临床资料。[结果]36例均完成检查,其中25例发现病变,阳性检出率69.4%;≥60岁患者以小肠血管畸形为主。检查过程中无任何不适及并发症。[结论]OMOM对OGIB诊断具有较高的诊断率及准确性,临床安全性好。     

113例胃隆起性病变各临床病理指标相关性研究

[目的]探讨胃隆起性病变患者性别、年龄、病变部位、起源、层次、病理类型、病变直径等临床病理指标特征及相关性。[方法]回顾性研究2016年10月～2018年10月在我院消化内科因胃隆起病变行内镜黏膜下剥离术(ESD)、内镜黏膜下挖除术(ESE)或内镜全层切除术(EFR)等内镜下治疗113例患者的临床资料,采用SPSS 19.0统计学软件,分析各指标特征及相关性。[结果]患者以40岁以上者居多(84.1%),不同性别发病差异无统计学意义。胃底部以间质瘤(72.7%)和平滑肌瘤(66.7%)居多,胃窦部以息肉(58.8%)、异位胰腺(61.1%)居多,病变多起源于固有肌层(58.4%)。起源于黏膜下层的病变直径以1～3 cm为主(67.6%),而固有肌层者直径则以≤2 cm居多(77.3%)。病变直径与起源层次具有显著相关性(P0.01),患者年龄与病理类型(P0.01)及病变部位(P0.01)具有显著相关性,起源层次与病变部位间亦有显著相关性(P0.05);病变部位与病理类型间存在显著相关性(P0.05)。[结论]胃隆起病变直径与起源层次有显著相关性,通过病变直径初判病变层次。内镜下病变部位与起源层次、病理类型间也存在显著相关性,内镜检查过程中可对胃隆起病变的性质和层次预判以利于进一步检查及治疗。后期需行大样本、多中心的前瞻性研究验证上述结论。     

克罗恩病并发急性下消化道大出血13例临床分析

目的 探讨克罗恩病(CD)并发急性下消化道大出血患者的临床特点及疾病转归.方法 收集1998年1月至2007年12月收治的CD并发急性下消化道大出血患者13例,对其临床及随访资料进行回顾性分析.结果 收治确诊的CD患者共230例,其中发生急性下消化道大出血者13例,占5.6%.13例患者中男女之比为3.3:1,其中4例以急性下消化道大出血为首发症状,10例病变累及小肠,能明确出血部位者8例(小肠出血4例,吻合口出血2例,结肠出血2例).13例首次大出血后,接受手术治疗4例,无死亡;接受内科保守治疗9例,死亡3例.随访10例,随访期6～94个月内,再发大出血8例,因再发大出血需手术2例,死亡1例.再发大出血而能继续存活的所有7例患者均以反复出血为惟一或主要临床表现,且病变均累及小肠.结论 部分CD患者可并发急性下消化道大出血,其诊断和治疗均存在一定困难,应引起临床医师的重视.     

隐源性消化道出血的病因及诊断分析

目的总结分析隐源性消化道出血的病因,探讨不同检查方法的诊断价值。方法对1992年至2004年的39例经手术治疗消化道大出血临床资料进行回顾性分析。结果39例患者均有病理诊断,所有病变以良性为主58.97%(23/39),病变部位以小肠居多89.74%(35/39)。与病理结果比,选择性动脉造影诊断符合率为56%(14/25),核素扫描定位诊断符合率为38.53%(13/34),小肠插管分段气钡造影诊断符合率为22.58%(7/31),术中内窥镜诊断符合率85.71%(6/7)。结论疑难消化道大出血病变多数在小肠,以良性病变为主,术前选择性动脉造影具有较高的定性和定位诊断价值。     

急性脑血管病患者继发早期痫性发作的影响因素研究

目的探讨急性脑血管病患者继发早期痫性发作的影响因素。方法选取2013年2月—2015年7月雅安职业技术学院附属医院神经内科收治的急性脑血管病患者100例,根据继发早期痫性发作情况分为继发组和无继发组,每组50例。比较两组患者临床资料,急性脑血管病患者继发早期痫性发作的影响因素分析采用多因素Logistic回归分析。结果继发组患者中男30例(占60.0%),女20例(占40.0%);年龄50岁者30例(占60.0%);40例(占80.0%)为出血性脑血管病,其中出血部位位于皮质者30例(占75.0%),多发病灶30例(占75.0%),大面积出血30例(占75.0%),均表现为癫痫持续状态。两组患者性别、年龄、吸烟率、饮酒率、糖尿病发生率、高血压发生率及冠心病发生率比较,差异无统计学意义(P0.05);两组患者病变性质、出血部位、病灶数量、大面积出血发生率、SSS评分30分者所占比例及既往有脑出血史者所占比例比较,差异有统计学意义(P0.05)。多因素Logistic回归分析结果显示,病变性质[OR=1.731,95%CI(1.004,2.986)]、出血部位[OR=2.038,95%CI(1.234,3.366)]、病灶数量[OR=1.989,95%CI(1.071,3.696)]、大面积出血[OR=2.065,95%CI(1.011,4.214)]、SSS评分[OR=2.149,95%CI(1.059,4.360)]及既往脑出血[OR=2.368,95%CI(1.056,5.301)]是急性脑血管病患者继发早期痫性发作的影响因素(P0.05)。结论病变性质、出血部位、病灶、大面积出血、SSS评分30分及既往有脑出血是急性脑血管病患者继发早期痫性发作的影响因素。     

应用胶囊内镜诊断不明原因消化道出血的临床价值探讨

[目的]探讨胶囊内镜(CE)对不明原因消化道出血(OGIB)的诊断价值。[方法]106例OGIB患者依据不同的临床表现分为隐性出血组和显性出血组,均采用CE检查,对诊断结果进行分析总结。[结果]106例OGIB患者中74例获阳性诊断(69.8%),显性出血组占50例(69.5%),隐性出血组占24例(70.6%),2组阳性诊断率比较,差异无统计学意义;阳性诊断结果中以血管病变居多,其次为小肠恶性肿瘤。[结论]CE检查诊断OGIB的价值较高,是一种安全、简便的诊断方法。     

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.     

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.     

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.     

Language of CTO interventions – Focus on hardware

The knowledge of variety of chronic total occlusion (CTO) hardware and the ability to use them represents the key to success of any CTO interventions. However, the multiplicity of CTO hardware and their physical character and the terminology used by experts create confusion in the mind of an average interventional cardiologist, particularly a beginner in this field. This knowledge is available but is scattered. We aim to classify and compare the currently used devices based on their properties focusing on how physical character of each device can be utilized in a specific situation, thus clarifying and simplifying the technical discourse.