IgG4相关系统性疾病的肾脏损害

IgG4相关系统性疾病(IgG4-related systemic disease,IgG4-RSD)是一组最近认识的临床综合征,可累及多个器官,胰腺最常受累,主要表现为血清IgG4升高,受累组织弥漫IgG4阳性浆细胞浸润。本文主要介绍IgG4-RSD累及肾脏时的临床表现、影像学、实验室检查、病理特点及治疗情况。     

IgG4相关性疾病伴肾脏损害病例报告并文献复习

目的:总结IgG4相关性疾病伴肾脏损害的临床特点。方法:回顾性分析4例IgG4相关性疾病伴肾脏损害患者的临床表现、病理特点、实验室检查、治疗及预后情况,并复习相关文献。结果:4例患者均为男性,年龄51~69岁,泌尿系统损害包括不同程度的血尿、蛋白尿、肾功能异常、梗阻性肾病。4例患者均同时存在泌尿系统外的多器官受累。所有患者均存在高球蛋白血症,血清Ig G及IgG4亚型显著升高。影像学表现可分为4类:肾皮质低密度影、肾脏弥漫增大、肾盂和/或输尿管积水、腹膜后纤维化。肾脏病理显示肾间质大量淋巴细胞、IgG4阳性的浆细胞浸润伴纤维化表现。患者对糖皮质激素治疗反应良好,临床症状及肾功能均明显改善。结论:累及泌尿系统的IgG4相关性疾病临床表现复杂多样,糖皮质激素治疗可较快缓解病情。     

重视IgG4相关性疾病的诊治刘朝君 辛星 董培源 孙汉英

IgG4相关性疾病(IgG4-Related Disease)是一种与IgG4阳性淋巴细胞-浆细胞密切相关的慢性、系统性、进行性的自身免疫性疾病,其主要特征有：IgG4阳性浆细胞浸润多种器官及组织；受累器官肿胀、纤维化、硬化；闭塞性脉管炎；伴或不伴有血清IgG4升高。由于IgG4-RD临床谱广泛,临床表现多样,目前尚无统一的诊断标准,患者可能因不同临床表现而于不同的专科就诊,故常误诊、漏诊。本文就近年来IgG4-RD的流行病学、发病机制、临床表现、诊断、治疗等方面的研究进展进行综述,以加深临床医生对该病的重视。     

IgG4相关性疾病9例临床特征分析

目的总结IgG4相关性疾病的临床特点以提高临床医师对该病的认知,做到早期诊断、规范治疗。方法选取2016年至2018年承德医学院附属医院收治的9例诊断为IgG4相关性疾病的患者为研究对象,观察其临床特点,分析其各项实验室检验结果、影像与病理学资料、治疗及预后。结果 9例IgG4相关性疾病患者中男性5例,女性4例,男女比例为1.25∶1。年龄35～68岁,平均54.89±10.03岁。其临床表现各异,可累及胰腺、胆囊、肺、肾、淋巴结、颌下腺、泪腺、关节、皮肤等多种组织器官。血清学表现为IgG4的升高及补体水平的降低,影像学表现为受累器官的肿大或占位。病理学表现为组织器官淋巴细胞、浆细胞浸润,IgG4及浆细胞增多。对糖皮质激素治疗反应良好,预后较好。结论 IgG4相关性疾病是一种系统性疾病,可累及多种组织器官,临床表现各异,需要结合临床表现、实验室检查、病理学检查及激素治疗效果等进行综合诊断     

IgG4相关疾病的实验室特征分析

目的探讨IgG4相关性疾病的共性和有意义的辅助诊断指标。方法回顾性分析福建医科大学附属第一医院2009年5月-2012年12月住院的10例Ig G4相关性疾病患者的临床资料。结果 10例患者中男7例,女3例,发病年龄41~75岁,平均年龄(55.8±13.6)岁,临床表现以黄疸(7/10)和体质量下降(4/10)为主,常见受累器官为胰腺(6/10)和胆管(5/10),均表现为多器官受累,患者血清IgG4均明显升高,影像学检查发现受累脏器呈弥漫性肿大或压迫狭窄,组织病理学检查表现为大量IgG4阳性淋巴细胞浸润并伴有明显纤维化改变,激素治疗后临床症状、影像学和实验室指标均有不同程度改善。结论 IgG4相关性疾病临床症状无特异性,血清IgG4可作为疑似病例的筛查方法。     

免疫球蛋白G4相关性肺疾病研究进展

近年来随着对免疫球蛋白G4(immunoglobulin G4,IgG4)相关性硬化性疾病的关注,越来越多的研究结果表明,IgG4相关性硬化性疾病是一组系统性疾病,除胰腺外,还可累及多个器官及组织,关于肺的IgG4相关性疾病也逐渐出现相关报道.IgG4相关性疾病以血清IgG4浓度升高、IgG4阳性浆细胞浸润及不规则纤维化为特征.     

血清IgG4检测在IgG4相关性疾病诊治中的作用

IgG4相关性疾病(IgG4-related disease,IgG4-RD)是一种新发现的以IgG4阳性淋巴细胞-浆细胞浸润为特点的累及多器官的系统性纤维炎症性病变.IgG4-RD受累器官广泛,临床表现多样,具有独特的病理学及血清学表现.但病理学检查存在诸多缺点:标本不易获取,尤其涉及到特定组织(如腹膜后组织、纵膈)、器官(如垂体、胰腺);为有创检查,患者不易接受等,使其在临床实施过程中受到一定限制.血清IgG4浓度升高作为IgG4-RD的诊断标准之一,其检测在临床应用方面有许多优点:方便、快捷、具有较高的敏感度和特异度,在疾病的诊治过程有一定的价值.     

IgG4相关性消化疾病及治疗研究进展

IgG4相关性疾病(IgG4-RD)是一组病因不明的表现为慢性纤维化的系统性自身免疫疾病,可波及全身器官,以IgG4升高以及IgG4阳性浆细胞浸润受累组织器官为特征,在消化系统中主要累及胰腺、肝脏、胆道、腹膜后等,糖皮质激素是目前公认的治疗IgG4-RD的一线用药,几乎所有的患者对于糖皮质激素治疗反应良好.     

IgG4相关性疾病

IgG4相关性疾病是一组以血清IgG4水平升高、受累组织IgG4阳性浆细胞浸润及纤维化为特征的、近几年才被人们认识的新疾病体.受累器官因纤维化、慢性炎症等出现增生肿大,从而导致相应的压迫阻塞症状或功能障碍.该病激素治疗效果较好[1-2].该病自发现至今有不少于10种名称,如IgG4相关的硬化性疾病、IgG4相关的自身免疫病、高IgG4疾病、IgG4阳性多器官淋巴增殖综合征(IgG4-positive multiorgan lymphoproliferative syndrome,IgG4+MOLPS)等[3],直到2010年才被统一命名为IgG4相关性疾病[4].本研究将从该病的认识过程、临床特点、病理特点、发病机制、诊断、治疗等方面作一综述,以便大家能逐渐认识和掌握该病.     

以自身免疫性胰腺炎和Mikulicz病为特征且伴有白细胞降低的IgG4相关性疾病1例报告

Ig G4相关性疾病(IgG4-related disease)是一类慢性、进行性炎症伴有纤维化的自身免疫性疾病,特征性的表现为血清IgG4水平升高,组织中大量IgG4阳性浆细胞浸润,席纹状纤维化以及闭塞性静脉炎[1-2],临床上病变几乎可以累及所有的器官和器官[3],受累器官通常表现为弥漫性的肿大。在消化系统中,唾液腺和胰腺是最常累及的器官。现将我们收治的以自身免疫性胰腺炎和Mikulicz病为特征且伴有白细胞降低的IgG4相关性疾病1例报告如下。     

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.     

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.     

Variabilité des concentrations plasmatiques de l’angiotensinogène et risque d’hypertension artérielle chez le rat transgénique

Aim

Genetic polymorphisms of the human angiotensinogen gene are frequent and may induce up to 30% increase of plasma angiotensinogen concentrations with a blood pressure increase of up to 5 mmHg. Their role for the pathogenesis of human arterial hypertension remains unclear. High plasma angiotensinogen levels could increase the sensitivity to other blood pressure stressors.

Methods

Male transgenic rats with a 9-fold increase of plasma angiotensinogen concentrations and male non-transgenic rats aged 10 weeks were treated or not with NG-Nitro-L-arginine-methyl ester for 3 weeks in their drinking water (n = 3/group). Systolic blood pressure and body weight were measured at baseline and at the end of the study when left ventricular weight and ventricular expression of angiotensin I-converting enzyme and procollagen Iα1 were determined (polymerase chain reaction).

Results

At baseline, transgenic rats had +18 mmHg higher bood pressure and –8% lower body weight compared to non-transgenic rats (P < 0.05) without significant changes for the vehicle groups throughout the study (P > 0.05). NG-Nitro-L-arginine-methyl ester increased blood pressure, left ventricular weight and left ventricular weight indexed for body weight by +41%, +17.6% and +18.6% (P < 0.05) in transgenic and +25%, +5.3% and +6.7% (P > 0.05) in non-transgenic rats compared to untreated animals, respectively. Cardiac gene expression showed no differences between groups (P > 0.05).

Conclusion

Increased plasma angiotensinogen levels may sensitize to additional blood pressure stressors. Our preliminary results point towards an independent role of angiotensinogen in the pathogenesis of human hypertension and associated end-organ damage.     

Morphological and immunocytochemical heterogeneity of cultured pinealocytes from one-week-and two-month-old rats: Planimetric and densitometric investigations

Abstract: In vitro preparations of rat pinealocytes are widely used for biochemical analyses of signal transduction processes. This paper deals with morphological and immunocytochemical features of such preparations. Special attention was paid to the problems of whether pinealocytes represent a heterogeneous cell population and how such heterogeneity may develop during ontogeny. The investigations were performed with cells which were obtained from the pineal organ of one-week-and two-month-old rats, attached to synthetic peptide-coated coverslips or tissue culture chamber slides, and maintained under in vitro conditions overnight. The attached cells were then fixed with paraformaldehyde. These preparations yielded monolayers of spherical cells of different sizes; most cells were isolated, but some of them were aggregated and formed small clusters. On the average, the cells from the one-week-old animals were smaller than the cells from the two-month-old animals. Immunocytochemical demonstration of S-antigen, a pinealocyte-specific marker, showed that the majority of the cells from two-month-old animals were intensely or moderately labelled. Pinealocytes from one-week-old animals were less S-antigen immunoreactive. Only very few cells (less than 1% displayed glial fibrillary acidic protein (GFAP)-immunoreactivity. Planimetric investigations of the cell size and semiquantitative densitometric investigations of the intensity of the S-antigen immunoreaction revealed that (i) pinealocytes kept in vitro form a heterogeneous cell population, and that (ii) this heterogeneity increases during postnatal development from one-week-old to two-month-old animals. Two groups of pinealocytes can be distinguished based on their developmental fate: pinealocytes of one group grow dramatically, but show only a moderate increase in S-antigen immunoreactivity, and pinealocytes of the other group retain their size, but display a distinct increment in S-antigen immunoreacti vitv.     

Effects of pinealectomy and melatonin administration on certain indices of ovarian hyperplasia and/or hypertrophy in rats with both ovaries intact or after unilateral ovariectomy

Abstract: In earlier studies from other laboratories it was shown that melatonin decreased ovarian weight in rats and inhibited compensatory hypertrophy of the remaining ovary after unilateral ovariectomy. This study was designed to examine the influence of melatonin on certain indices of ovarian hyperplasia and/or hypertrophy in adult female rats with both ovaries preserved and with either an intact pineal gland or with the pineal gland removed (pinealectomy, PX) or, finally, in sham-PX animals. Similar studies were conducted on rats after unilateral ovariectomy, referring the examined parameters to the remaining intact ovary. The studies included mitotic activity of granulosa layer cells and corpus luteum cells, ovarian weight, ovarian cross-sectional area, cross-sectional area of the granulosa layer of all the Graafian follicles and the cross-sectional areas of the corpora lutea, visible on the ovarian cross-section. On the basis of results, we conclude that: 1) the effect of PX on the processes of ovarian hyperplasia and hypertrophy may vary; analogously, exogenous melatonin administration may influence ovarian hyperplasia and hypertrophy in different ways; 2) PX and exogenous melatonin may, under certain conditions, exert similar biological effects, even synergistic effects; 3) melatonin inhibits ovarian growth processes, while the effects of PX are variable; 4) the results indicate that in experiments performed on rats, with the use of two control groups, i.e., intact and sham-PX, melatonin effects on these two groups may differ.