重叠综合征临床诊治现状

<正>自身免疫性肝病包括两大类:一类以肝细胞损伤为主,典型代表是自身免疫性肝炎(autoimmune hepatitis,AIH);另一类以胆汁淤积为主,典型代表是原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)、原发性硬化性胆管炎(primary sclerosing cholangitis,PSC)和自身免疫性胆管炎(autoimmune cholangitis,AIC)[1]。两大类自身免疫性肝病通常具有各自典型的临床表现、生化学、免疫学和病理学特征。然而,少数患者可兼有2种自身免疫性肝病的临床特点,这部分患者我们称之     

血清自身抗体检测对自身免疫性肝病的研究进展

自身免疫性肝病(autoimmune liver disease,AILD)是因自身免疫异常所致的以肝脏为相对特异性免疫病理损伤器官的一组疾病,主要包括自身免疫性肝炎(autoimmune hepatitis,AIH)、原发性胆汁性胆管炎(primary biliary cholangitis,PBC)、原发性硬化性胆管炎(primary sclerosing cholangitis,PSC)及重叠综合征。AILD与病毒感染、酒精、药物等所致的传统肝病不同,突出特点是血清中存在特异性的自身抗体。本文主要对AILD相关血清自身抗体的研究进展作一概述,以有助于临床诊断。     

自身免疫性肝病诊断和治疗指南

1概述 自身免疫性肝病与病毒感染、酒精、药物、遗传等其他因素所致肝病不同,是一组由于自身免疫异常导致的肝脏疾病,突出特点是血清中存在自身抗体,包括原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)、自身免疫性肝炎(autoimmune hepatitis,AIH)、原发性硬化性胆管炎以及其他自身免疫病[如系统性红斑狼疮(SLE)、干燥综合征(SS)等]肝脏受累等.本章重点介绍PBC和AIH.     

自身免疫性胆管炎

自身免疫性胆管炎(autoimmune cholangitis，AIC)是指临床特点、实验室榆查和病理改变具有自身免疫性肝炎和肝内胆管进行性非化脓性破坏的特征，血清中抗线粒体抗体(AMA)(-)、抗核抗体(ANA)( )的一类疾病。回顾性研究认为它无法归于传统上的自身免疫性肝炎(autoimmune hepatitis，AIH)、原发性胆汁性肝硬化(primary biliary cirrhosis，PBC)、原发性硬化性胆管炎(primary sclerosing cholangitis，PSC)。具有独特的临床生化和组织学特点 。     

自身免疫性肝病中的重叠综合征

自身免疫性肝病是一组具有一定自身免疫基础的炎症性肝病,包括自身免疫性肝炎(autoimmune hepatitis,AIH)、原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)、原发性硬化性胆管炎(primary sclerosing cholangitis,PSC)等,其共同特点是在肝脏出现病理性炎症损伤的同时、血清中可发     

类风湿关节炎合并原发性胆汁性肝硬化3例

<正>原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)是一种自身免疫性肝脏疾病,以胆道梗阻为特征,可合并干燥综合征、系统性硬化病、自身免疫性甲状腺炎等自身免疫性疾病,但合并类风湿关节炎(rheumatoid arthritis,RA)者少见,易被误诊。分析郑州大学第一附属医院2010年1月至2015年1月收治的3例RA合并PBC患者的临床资料,并结合国内外     

自身免疫性肝病的临床及病理研究现状

自身免疫性肝病(autoimmune liver disease,ALD)包括自身免疫性肝炎(autoimmune hepatits,AIH)、原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)和原发性硬化性胆管炎(primary sclerosing cholangitis,PSC),以及这三种疾病中任何两者之间的重叠综合征(overlap syndrome,OS)。肝穿刺组织病理检查是目前诊断各种慢性肝损伤的重要依据,在对自身免疫性肝病的诊断认识过程中,肝脏的组织病理学起重要作用。     

自身免疫性肝病重叠综合征的现状

自身免疫性肝病(autoimmune liver disease,A I L D)包括自身免疫性肝炎(a u t o i m m u n e hepatitis,AIH)、原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)和原发性硬化性胆管炎(primary sclerosing cholangitis,PSC).AILD重叠综合征(overlap syndrome,OS)是指患者同时兼具AIH和PBC或PSC两种疾病的临床表现、生物化学、血清学、组织学以及影像学特征的一种罕见疾病状态.AILD OS主要分为AIH-PBC OS和AIH-PSC OS.前者多见于成人,后者多见于儿童.OS若无治疗,最终可致肝硬化或肝衰竭.高剂量熊去氧胆酸(ursodeoxycholic acid)与免疫抑制剂类固醇和/或硫唑嘌呤(azathioprine)联合使用通常被用于治疗AIH-PBC OS和AIH-PSC OS.目前,肝移植仍是治疗终末期OS患者的唯一有效方法.     

重视自身免疫性肝病的临床诊治

自身免疫性肝病(autoimmune liver diseases)是一组自身免疫介导的肝胆系统损伤,根据临床表现、生化、影像学和组织病理学特点,可简单地分为以肝炎为主型,即自身免疫性肝炎(autoimmune hepatitis,AIH)以及以胆系损害及胆汁郁积为主型,即原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)和原发性硬化性胆管炎(primary sclerosing cholangitis,PSC).此外,还有这3种疾病中任意两者之间的重叠综合征(o-verlao syndromes),主要以AIH-PBC重叠综合征多见.以上几种疾病均可表现为严重的肝脏病变,并可进展至肝硬化[1].     

自身免疫性肝病相关自身抗体的研究进展

自身免疫性肝病(autoimmune liver disease, AILD)是一组由自身免疫反应引起的慢性肝胆系统损伤性疾病,早期诊断及治疗对该疾病发展及预后至关重要。自身抗体的检测对于该组疾病的诊疗具有重要临床价值。医学上对自身抗体检测技术的提高,对于AILD的诊断及鉴别工作都起到了非常大的作用。本文主要探讨自身免疫性肝炎(autoimmune hepatitis,AIH)、原发性胆汁性胆管炎(primary biliary cholangitis, PBC)、原发性硬化性胆管炎(primary sclerosing cholangitis, PSC)三种疾病的相关自身抗体,以期为今后的AILD诊断治疗提供具有建设性的参考意见。     

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.     

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.     

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.     

Presence of immunoreactive growth hormone and prolactin in the ovine pineal gland

Abstract: The use of antisera raised against bovine growth hormone (GH) and ovine prolactin (PRL) enabled the detection of related immunoreactive (ir) sequences of proteins in ovine pineal tissue. The isolation of PRL-like ir-material was accomplished using a 0.25 M ammonium sulphate (pH 5.5) extraction followed by ethanol precipitation, whereas the resulting 2.0 M ammonium sulphate (pH 7.0) precipitate contained a GH-like immunoreactivity. Gel chromatography of the GH-like immunoreactivity (Sephadex G-100) indicated the presence of several GH-like fragments ranging in the M_r range of 7,000 to 55,000. Analyses of the PRL-like ir-material found in pineal tissue on HPLC using a TSK 545-DEAE column led to the resolution into a single peak of immunoreactivity. A single peak of activity was also observed following chromatofocusing and hydrophobic interaction chromatography of the ir-peak from the TSK 545-DEAE column. The PRL-like ir-material inhibited the binding of [¹²⁵I]ovine PRL-S14 to anti-ovine PRL antibodies without showing an affinity for binding to anti-rat PRL or anti-bovine GH antibodies. Scatchard analysis of the binding of pineal PRL-like ir-material and pituitary ovine PRL-S14 to liver membranes from day-20 pregnant rats revealed similar affinity constants (K_a of 4.7 ± 0.2 × 10⁹ M^-1). In addition, the replication of Nb 2 Node rat lymphoma cells was stimulated by pineal PRL-like ir-material, an effect known to be specific for lactogenic hormones. The pineal PRL-like immunoreactivity appeared on sodium dodecyl sulfate polyacrylamide gels as a single major band of M_r 24,000. The functional status of PRL-and GH-like ir-material in the ovine pineal remains to be determined, but evidence is presented that the overall protein synthesis rate of the rat pineal responded to circulating concentrations of PRL.     

Microbiology of human immunodeficiency virus anorectal disease

PURPOSE: Individuals who are seropositive for the human immunodeficiency virus are at high risk for opportunistic infection and anorectal disorders. Little prospective information is available regarding anorectal pathogens in these patients. METHODS: One hundred sixty-three HIV-seropositive patients presented to the colorectal clinic between 1989 and 1992. Forty-seven (29 percent) patients were thought to have an infectious process and were prospectively studied using a standardized multiculture protocol. RESULTS: Mean age was 33 (range, 19–59) years. All were male; high-risk behavior accounted for 87 percent of HIV transmissions. Presenting complaints included anorectal pain (79 percent), pus per anum (28 percent), and blood per anum (26 percent). Examination revealed perianal tenderness (60 percent), condyloma (38 percent), perianal ulcers (38 percent), and anal fissures (34 percent). Sixty-six sets of cultures were performed; 28 patients had one set, 15 had two sets, and 4 had three sets. Thirty-two of these 47 patients (68 percent) had positive cultures including herpes (50 percent), cytomegalovirus (25 percent),Neisseria gonorrhoeae (16 percent), chlamydia (16 percent), acidfast bacilli (2 percent), and others (9 percent). Six of 32 patients with positive cultures had more than one organism cultured. Sixteen (50 percent) patients with positive cultures were treated medically, 8 (25 percent) were treated surgically and 8 (25 percent) were treated with both modalities. Sixty-one procedures were performed on 17 patients for condylomata. Eighteen patients had 20 procedures for abscesses, 50 percent of whom had positive cultures for other than common bowel flora; all improved. Fourteen patients underwent 33 procedures for perianal fistulas.Mycobacterium fortuitum was cultured from one patient who required 13 procedures for abscesses and fistulas. Forty-five (96 percent) patients were followed for an average of 12.5 months ±2.9 SEM (range, 1–94 months). Symptoms were improved or resolved in 22 of 32 (69 percent) patients with positive cultures and in 11 of 13 (84 percent) with negative cultures. CONCLUSIONS: Specific pathogens may often be identified in human immunodeficiency virus-seropositive patients with anorectal disorders if aggressively sought. Although patients without specific pathogens identified may be expected to improve with planned empiric treatment, positive identification allows more directed therapy.