微创介入治疗急性化脓性胆管炎28例分析

目的总结急性化脓性胆管炎患者微创介入治疗的效果。方法对解放军总医院2001年6月至2005年6月收治的急性化脓性胆管炎经微创介入治疗的28例患者的临床资料进行回顾性分析。结果行单纯内镜下逆行胰胆管造影(ERCP)22例,其中乳头切开同时进行鼻胆管引流或取石者13例,胆管支架内引流者9例,在胆管支架内引流者中病变位于肝门下者5例,肝门上者4例,以肝门下梗阻引流效果好,肝门上肿瘤引流后症状缓解,术后患者高热消失,体温恢复正常;内镜下治疗联合经皮经肝胆管外引流术(PTCD)4例,全部治愈;单纯PTCD治疗2例,缓解1例,治愈1例。所有患者均未发生严重并发症。结论ERCP、PTCD和内镜下治疗联合PTCD方法治疗胆系化脓性炎症,创伤小、效果好,值得推广。     

A Relapsing Remitting Lymphocytic Hypophysitis

Lymphocytic Hypophysitis is a rare autoimmune disease of the pituitary presenting mainly with features of a mass lesion and loss of pituitary function. Its cousre is quite unpredictable and its treatment is still controversial as experience in the field remains scarce.We describe a 45 year-old woman with a history of recurrent fever and meningeal symptoms who was referred 3 years later to our department for pituitary insufficiency Hormonal studies revealed an anterior pituitary deficiency and autoimmune thyroiditis. Pituitary hypertrophy as evidenced by magnetic resonance imaging showed complete regression upon hydrocortisone substitution therapy.Two years later, fever and meningeal symptoms recurred as well as pituitary hypertrophy. Cerebrospinal fluid analysis revealed an aseptic lymphocytic meningitis. Pituitary biopsy confirmed the diagnosis of LH. The patient was started on prednisone 70 mg per day. She improved clinically and radiologically and remained free of symptoms thereafter. At a recent follow up the MRI showed an empty sella turcica. Hereby we illustrate a yet unreported pattern of LH presenting as a recurrent aseptic meningitis. We also describe one of the long-term course of the disease which is still unpredictable.     

Clonal accumulation of Vβ5.1-positive cells in the liver of a patient with autoimmune cholangiopathy

Abstract: We describe a 43-year-old woman with clinical features compatible with autoimmune cholangiopathy recently reported by Ben Ari et al. She was negative for anti-mitochondrial antibody, positive for high titer anti-nuclear antibody with homogeneous pattern, high levels of serum immunoglobulin G and nearly normal levels of serum immunoglobulin M for more than five years. In the early stages of the disease, the elevations of serum transaminase, alkaline phosphatase and γ-glutamyl transpeptidase were well controlled by the administration of ursodeoxycholic acid. After five years of follow-up, she showed the second exacerbation of liver function tests, which then rapidly improved by prednisone administration. To analyze the antigen diversity recognized by T-cells in the liver, T-cell receptor repertoire was examined by immunohistochemistry. The liver biopsy obtained in the early stage showed clonal accumulation of Vβ5.1-positive cells in portal areas, which was found in patients neither with primary biliary cirrhosis nor autoimmune hepatitis. In conclusion, these data suggest that T-cell response in autoimmune cholangiopathy is different from those two autoimmune liver diseases, which may imply a distinct entity of the disease.     

Advanced cholangiocarcinoma in a patient with stage I primary sclerosing cholangitis

A 29‐year‐old woman presented with jaundice and fever in May 2001. Cholangiography showed multiple strictures and beading of the biliary tree, with a large stricture in the common bile duct and marked dilatation of the hilar bile ducts. Typical cholangiography findings and elevated hepatobiliary enzymes suggested primary sclerosing cholangitis (PSC). At the same time, computed tomography detected a 2‐cm tumor in the common bile duct, and angiography showed an encasement in the portal vein. Tumor markers, cytology, and biopsy were all negative for cancer. Although laparotomy showed a healthy liver and no lymph node metastasis was found, suggesting early‐stage PSC and a low likelihood of accompanying cholangiocarcinoma (CCA) reported so far, the tumor in the resected common bile duct was subsequently diagnosed as CCA. Therefore, pancreatoduodenectomy was performed combined with partial resection of the portal trunk. Histology also revealed invasion of the wall of the portal vein by cancer cells. The patient had a recurrence 5 months later and died 12 months after her operation. This is a rare case in which stage I PSC was complicated by advanced CCA.     

长链非编码RNA及其在自身免疫性疾病发生发展中的作用

长链非编码RNA(long non-coding RNA,lnc RNA)是一类长度超过200个核苷酸、不编码蛋白质的功能性RNA分子。lnc RNA通过表观遗传修饰与转录调控、转录后加工、翻译调控等多种机制,在细胞生命活动中发挥重要作用。最新研究表明,lnc RNA与免疫细胞的分化和激活密切相关,进而影响类风湿性关节炎、系统性红斑狼疮等多种自身免疫性疾病的发生发展,本文就lnc RNA在自身免疫性疾病中的研究进展进行综述。     

Primary sclerosing cholangitis in Japan—analysis of 192 cases

Primary sclerosing cholangitis is very rare in Japan. The aim of the present study was to identify the characteristics of such patients in Japan. A questionnaire was sent to the members of the Japanese Society of Gastroenterology and responses for 192 cases were analyzed. There was male predominancy (61%), and two peaks in the age distribution at diagnosis (20–30 years and 50–70 years). Bile duct damage was mainly intra + extrahepatic (69%) versus intrahepatic (17%) or extrahepatic (14%). The incidences of eosinophilia and positivity for anti-nuclear antibody were 27% and 30%, respectively. The incidence of associated inflammatory bowel disease was 21 % (38 ulcerative colitis and 2 Crohn's disease). Chronic pancreatitis, gallstones, and biliary cancers occurred in 15%, 12%, and 4%, respectively, of the 192 patients. Patients less than 40 years of age had a higher incidence than the patients 40 years old or more of damage intra + extrahepatic bile ducts (89% vs 56%) and of associated ulcerative colitis (36% vs 9%), whereas the incidence of chronic pancreatitis was lower in patients aged less than 40 years (4%). The characteristics of patients with primary sclerosing cholangitis in Japan differ from those in other countries in regard to age distribution and the incidence of complications, and at least two different groups of patients seem to exist in terms of the degree of bile duct damage and the incidence of complications.     

AUTOANTIBODIES AND ANTI-MOUSE ANTIBODIES IN THROMBOCYTOPENIC PATIENTS AS ASSESSed by DIFFERENT MAIPA assays

Two MAIPA (monoclonal antibody [MAb] immobilization of platelet antigen) assays were performed to determine (a) autoantibodies to platelet glycoproteins (GP) and (b) serum antibodies recognizing mouse MAbs used in the assay. In MAIPA I, control platelets were incubated simultaneously with human serum and a mouse MAb to a platelet glycoprotein (GP IIb–IIIa, Ib–IX, Ia–IIa, IV and p24). In MAIPA II, the control platelets were incubated first with the human serum and then, after washing, with the selected mouse MAb. A series of 25 patients with autoimmune thrombocytopenic purpura (ATP) associated or not with other autoimmune states were examined. Autoantibodies (both MAIPA I and MAIPA II positive) or anti-mouse Abs (MAIPA I positive and MAIPA II negative) were frequent in both groups of patients. Statistically significant differences existed in the incidence of anti-mouse Abs between patients (56.5%) and healthy donors (10%). This suggests that their production may be related to thrombocytopenias associated with autoimmune disease. We speculate that the presence of anti-mouse antibodies could reflect an abnormality in the immunological modulation of the idiotypic network.     

Occurrence of organ-specific and systemic autoimmune diseases among the first- and second-degree relatives of Caucasian patients with connective tissue diseases: report of data obtained through direct patient interviews

Studies have demonstrated a familial aggregation of systemic and organ-specific autoimmune diseases. The aim of the present survey was to obtain, by patient interviews, a preliminary estimate of the prevalence of systemic and organ-specific autoimmune diseases among the first- and second-degree relatives of Caucasian patients with connective tissue diseases (CTD) or inflammatory arthritis followed at our unit. Between June 2007 and January 2008, 626 patients and 85 controls (patients with osteoarthritis, osteoporosis, or fibromyalgia) were interviewed. Three hundred ten patients (50%) versus 21 controls (25%) were found to have at least one relative affected with an autoimmune condition (p < 0.0001). The most common conditions were organ-specific autoimmune diseases: 160 (34%) autoimmune thyroid (AT) disease, 112 (24%) psoriasis, 21 vitiligo, and 19 insulin-dependent diabetes mellitus. Systemic autoimmune diseases were reported in 126 relatives: rheumatoid arthritis (66 cases, 14%), 16 sacroileitis, and CTD (43 cases). A significant difference was observed in the prevalence of AT disease between the relatives of the patients and controls (3% versus 0.5%). In conclusion, these data confirm the high prevalence of autoimmune conditions, particularly of AT disease, among the relatives of patients.     

大鼠梗阻性胆管炎细胞免疫功能降低及中药锦红片的影响

目的:探讨梗阻性胆管炎大鼠细胞免疫功能降低的发生机制及清热通下中药锦红片的影响.方法:♂SD大鼠24只建立急性梗阻性胆管炎模型,随机分为模型组(n=8)、锦红片治疗组(n=8)和单纯胆管梗阻组(n=8),检测血浆IL-2,CD_3~ ,CD_4~ ,CD_8~ ,内毒素,胸腺指数,胸腺细胞凋亡指数及电镜下观察胸腺的超微结构及凋亡.结果:模型组IL-2,CD_3~ ,CD_4~ 和胸腺指数显著低于治疗组和单纯胆管梗阻组(IL-2:28.5±3.0 ng/L vs 33.9±3.6 ng/L,39.6±2.2 ng/L,P<0.05,P<0.01;CD_3~ :54.5%±5.5% vs 70.7%±4.8%,66.3%±7.1%,均P<0.01;CD_4~ :34.5%±8.3% vs 44.2%±3.3%,44.5%±4.2%,均P<0.01:胸腺指数:0.89±0.18 vs 1.10±0.13.1.12±0.24,均P<0.05),CD_8~ 3组间没有统计学差异,血浆内毒素和凋亡指数明显高于治疗组和梗阻组(内毒素:0.85±0.14 Eu/mL vs 0.53±0.10 EU/mL,0.49±0.11 EU/mL,均P<0.01;凋亡指数:25.7±5.1 vs 15.8±5.5.9.0±3.1.P<0.05,P<0.01),模型组胸腺可见较多典型的凋亡细胞,结果显示经中药干预治疗后,免疫功能、内毒素血症和胸腺细胞凋亡有所改善,接近单纯胆管梗阻组水平.结论:梗阻性胆管炎大鼠存在免疫功能降低,胸腺细胞异常凋亡.锦红片对维持免疫机能的稳定有积极的意义.     

抗可溶性肝抗原/肝胰抗原抗体阳性的肝病患者临床与实验室特征

目的研究肝功能异常伴抗可溶性肝抗原／肝咦抗原（SLA／LP）抗体阳性患者临床与实验室特点。方法收集肝功能异常患者血清5500例，分别完成相关自身抗体的检测。结果8例（0．15％）患者检测出sLA／LP抗体阳性，女性6例，以中老年女性为主，男性2例，年龄分别为27岁和33岁，8例中6例无甲、乙、丙、丁、戊型等病毒性肝炎现症感染证据，2例分别伴乙、丙型肝炎病毒标志物，无现症感染证据。8例中7例已发展至肝硬化，除sLA／LP抗体阳性外，抗核抗体均为阳性，部分患者伴抗平滑肌抗体，抗线粒体抗体，抗线粒体抗体亚型-丙酮酸脱氢酶复合物阳性。结论sLA／LP抗体在所检测患者中阳性率较低，以女性患者为主，多数临床与实验室特点符合自身免疫性肝炎特征，女性患者慢性肝病原因未名者应检测此抗体。