自身免疫性肝炎和原发性胆汁性肝硬化重叠综合征患者的临床特点

目的 了解自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(PBC)重叠综合征的临床特点。方法 对129例自身免疫性肝病患者的临床资料进行回顾分析,按国际评分标准,诊断AIH/PBC重叠综合征,并将其生物化学、自身抗体、肝穿刺结果与单纯的AIH、PBC患者病例资料比较。 结果 129例自身免疫性肝病患者中35例为AIH/PBC重叠综合征患者,占27.1％,以女性患者为主,男女比例为1:10,平均年龄(50.79±11.27)岁,其实验室检查具有AIH患者的特点,如:氨基转移酶、γ 球蛋白、免疫球蛋白G的明显升高,同时也具有PBC的特点,如:碱性磷酸酶、免疫球蛋白M的显著增高,自身抗体检测可见抗核抗体(74.3％)、抗线粒体抗体(68.6％)、M2抗体(45.7％)阳性;肝穿刺结果有界面坏死、浆细胞浸润及胆管不同程度的损害。 结论 AIH/PBC重叠综合征为独立于AIH、PBC存在的疾病,有必要对此及早诊断,寻找有效的治疗方法。     

自身免疫性肝病20例临床特点分析

目的　比较自身免疫性肝炎 (AIH)、原发性胆汁性肝硬化 (PBC)和原发性硬化性胆管炎 (PSC)的临床特点 ,以利于临床早期鉴别诊断。方法　分析我院 1998～ 2 0 0 1年 2 0例自身免疫性肝病 (AIH 9例 ,PBC 10例 ,PSC 1例 )的临床资料 ,并对其肝功能特点及自身抗体进行比较。结果　 2 0例患者中 ,男女之比为 2∶18,平均年龄 (5 4.7± 11.0 )岁。AIH与PBC患者各项指标比较 ,PBC肝功能异常以碱性磷酸酶升高为特点 ,其余指标比较差异无显著性 ;AIH免疫学指标以γ 球蛋白、主要是免疫球蛋白IgG升高为特点 ,而PBC以γ 球蛋白、主要是IgM升高为特点 ;AIH循环自身抗体以抗核抗体、抗平滑肌抗体为主 ,而PBC患者以抗线粒体抗体及其分型M2 为主。结论　AIH及PBC临床表现、肝功能检查有许多相似之处 ,但仍然有其各自特异的临床诊断依据     

自身免疫性肝病诊治面临的挑战

自身免疫性肝病(AILD)以循环中出现自身抗体、肝组织学炎症表现和血清免疫球蛋白水平升高为特点,主要包括自身免疫性肝炎(AIH)、原发性胆汁性胆管炎(PBC)、原发性硬化性胆管炎(PSC)、IgG4相关硬化性胆管炎(IgG4-SC)和重叠综合征。早期诊断和早期治疗可显著改善患者预后,提高生活质量。本文将重点评述我国AILD研究中取得的进展和遇到的难点问题。     

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.     

自身免疫性肝病临床及免疫学特征

目的 分析自身免疫性肝病的临床特征和免疫学特点,以提高对该类疾病的认识和诊断水平.方法 对164例自身免疫性肝病患者的临床症状、体征及免疫学检查资料进行回顾性分析.结果 原发性硬化性胆管炎(PSC)多发于青年男性,自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(PBC)及其重叠综合征(AIH-PBC)多发于中年女性;临床表现上AIH、PBC、PSC、AIH-PBC黄疸发生率分别为84%、78%、90%和67%,皮肤瘙痒的发生率分别为43%、56%、81%和60%.但AIH、PBC和PBC-AIH患者之间的年龄、性别、黄疸、皮肤瘙痒等症状无显著性差异(P均＞0.05).AIH首诊正确诊断率为8%(6/77),PSC为9%(1/11),PBC为13%(6/46).从发病到确诊PBC平均为38月,AIH为46月,PSC为31月,PBC-AIH重叠综合征确诊最难,平均需51月,常误诊为可能的AIH或单纯PBC.AIH、PBC、PSC和PBC-AIH肝外自身免疫性疾病的发生率分别为47%、11%、27%和24%,各自身免疫性肝病患者均有较高的自身抗体发生率.结论 自身免疫性肝病在临床上并不少见,患者常伴发肝外自身免疫性疾病及较高的自身抗体发生率;诊断需综合临床、生化、自身抗体和病理组织学等指标.     

自身免疫性肝炎

自身免疫性肝炎（AIH）是一种病因不明、可见于任何年龄段的进展性慢性肝炎，病情通常有波动。AIH的诊断需组织学形态改变、特征性临床表现、生化检查、血清免疫球蛋白异常升高和血清自身抗体的存在。变异综合征或重叠综合征常在AIH的基础上合并其他自身免疫性肝病的特征，如原发性胆汁性肝硬化（PBC）或原发性硬化性胆管炎（PSC）。另外，AIH需与其他慢性肝炎鉴别，及时恰当的治疗能延长患者生存期，提高生活质量并尽可能避免肝移植。     

西南地区三种自身免疫性肝病的临床特点分析

目的：分析西南地区各型自身免疫性肝病的临床特点,以期提高早期诊断率。方法回顾性分析比较第三军医大学西南医院收治的436例自身免疫性肝病患者的一般资料、临床表现、血清生化学、自身抗体、肝组织病理等指标的差异,其中包括自身免疫性肝炎（AIH）211例,原发性胆汁性肝硬化（PBC）145例,自身免疫性肝炎-原发性胆汁性肝硬化重叠综合征（OS）80例。结果患者平均发病年龄为49．7岁,男女比为1∶6;各组临床表现无显著差异;各组 ALT、GGT、TBil等血清学指标差异有统计学意义（P＜0．05）;AIH 组血清自身抗体以ANA（＋）为主,PBC 组中以AMA（＋）、AMA-M2（＋）为主,OS组兼有AIH 及PBC自身抗体的特点;各组肝组织病理主要表现为肝细胞变性、坏死、炎细胞浸润、小胆管炎症及纤维增生。结论在我国西南地区AIH、PBC、OS多为50岁左右的围绝经期女性。综合分析临床表现、血清生化学指标、免疫球蛋白亚型、自身抗体等指标有助于确诊不同类型的自身免疫性肝病,肝活组织检查为重要的辅助诊断手段。     

原发性胆汁性肝硬化／自身免疫性肝炎重叠综合征患者的临床和病理学研究

目的分析原发性胆汁性肝硬化（PBC）、自身免疫性肝炎（AIH）和PBC／AIH重叠综合征患者的临床和病理学特点。方法对105例自身免疫性肝病患者的临床资料进行分析,比较PBC／AIH重叠综合征和单纯PBC或AIH患者的临床表现和肝组织病理学变化。结果在105例患者中,包括11例PBC／AIH重叠综合征、60例PBC、33例AIH和1例原发性硬化性胆管炎（PSC）。PBC／AIH重叠综合征与PBC或AIH患者的性别、年龄、症状和并发症无明显差异（P〉0．05）,其实验室检查具有AIH的特点,如血清丙氨酸氨基转移酶、天冬氨酸氨基转移酶、免疫球蛋白IgG的明显升高,同时具有PBC的特点,如GGT、ALP、免疫球蛋白IgM的显著增高,但与PBC或AIH相比,无统计学差异（P〉0．05）;自身抗体检测可见抗核抗体、抗线粒体M2抗体和抗核心蛋白gP210抗体阳性,后两项抗体检出率明显高于AIH患者（P〈0．01）;肝组织病理学检查结果显示,PBC／AIH重叠综合征兼有PBC和AIH的特点,如界面炎和碎屑样坏死,汇管区浆细胞浸润,胆管不同程度的病变等。结论PBC／AIH重叠综合征的临床表现和肝组织病理学具有PBC和AIH的双重特征,应对此病充分认识,并探索有效的治疗方案。     

病毒性肝炎—总论

自身免疫性肝炎和原发性胆汁性肝硬化重叠综合征患者的临床特点——曾珍等(北京解放军第302医院感染三科100039)《中华肝脏病杂志》2005，13(1)：3-5[了解自身免疫性肝炎(AIH)、原发性胆汁性肝硬化(PBC)重叠综合征的临床特点。方法：对129例自身免疫性肝病患者的临床资料进行回顾分析，按国际评分标准，诊断AIH／PBC重叠综合征，并将其生物化学、自身抗体、肝穿刺结果与单纯的AIH、PBC患者病例资料比较。结果：129例自身免疫性肝病患者中35例为AIH／PBC重叠综合征患者，占27．1％，以女性患者为主，男女比例为l：10，平均年龄(50．79&;#177;11．27)岁，其实验室检查具有AIH患者的特点，     

自身免疫性肝病的研究进展与思考

正自身免疫性肝病(AILD)包括自身免疫性肝炎(AIH)、原发性胆汁性胆管炎(PBC)、原发性硬化性胆管炎(PSC)和免疫球蛋白G4相关硬化性胆管炎(IgG4-SC)。早期诊断并及时启动干预治疗对于AILD患者的预后及生活质量的改善至关重要。通常认为遗传易感个体在环境等因素的诱发下发病,AIH、PBC、PSC以及IgG4-SC在自身免疫反应的攻击对象、免疫应答、临床诊治和预后等方面均各     

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.     

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.     

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.