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Autoimmune cholangitis, immunoglobulin G4‐associated cholangitis (IAC), is a part of multiorgan IgG4‐related systemic disease, which was recognized as a new clinicopathological entity in recent years. IAC is defined as a biliary stricture that responds to steroid therapy, frequently is associated with other fibrosing conditions, especially autoimmune pancreatitis and is characterized by elevation of IgG4 in serum and infiltration of IgG4 positive plasma cells in bile ducts. Since IAC shares a number of clinical, biochemical, and imaging features with cholangiocarcinoma (CCA), it is often misdiagnosed as CCA, and unnecessary surgery was performed. In this compact review, we clarify the disease of IAC, summarize criteria for diagnosis of IAC, discuss the role of CA 19‐9, and provide key information to differentiate diagnosis of IAC from CCA. IAC should be highly suspected in unexplained biliary stricture associated with increased IgG4 (in serum especially in bile) and other organ involvement (kidney, retroperitoneum etc. especially pancreas in which there are abundant IgG4‐positive plasmocytes infiltration). Correct diagnosis of IAC will avoid unnecessary surgery because IAC responds well to steroid therapy. In a word, increased IgG4 levels, other organ involvement and response to steroids are keys to distinguishing IAC from CCA.  相似文献   
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ABSTRACT

Purpose/Aim: Although several prognostic factors for extrahepatic cholangiocarcinoma (EHC) have been reported, preoperative prognostic factors have yet to be established. We investigated the serum concentration of angiogenic, inflammatory, and nutritional parameters. Materials and Methods: Twenty-five patients with EHC were enrolled before starting treatment. Preoperative prognostic factors were identified using multivariate analyses. Results: The serum soluble intercellular adhesion molecule-1 (sICAM-1) levels were significantly higher in the patients with EHC (436.0 ± 43.2 ng/ml) than in the healthy volunteers (228.6 ± 22.0 ng/ml) (p <.001). In addition, the serum IL-6 levels were significantly higher in the patients (18.0 ± 5.6 pg/ml) than in the healthy volunteers (5.7 ± 0.8 pg/ml) (p <.05). The serum IL-6 and sICAM-1 showed a strong correlation (r = 0.559) in the patients with EHC (p <.01). The serum IL-6 (area under the curve = 0.764, p =.030, cut-off level = 11.6) and sICAM-1 (area under the curve = 0.818, p =.007, cutoff level = 322.6) were revealed to be useful as prognostic factors by the receiver operating characteristic curves. The high IL-6 group and the high sICAM-1 group showed poorer DSS than those of the respective low groups. In the multivariate analysis, IL-6 (hazard ratio: 1.050, 95% confidence interval: 1.002–1.100, p =.043) and sICAM-1 (hazard ratio: 1.009, 95% confidence interval: 1.002–1.015, p =.009) were independent prognostic factors for DSS. Conclusions: IL-6 and sICAM-1 were independent preoperative prognostic factors in EHC patients, causing continuous inflammation and malnutrition in collaboration with other pro-angiogenic factors.  相似文献   
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肝门部胆管癌是指原发于胆囊管开口以上,左、右二级肝管水平以下的肝门区胆管恶性肿瘤,占所有胆道恶性肿瘤的50%~70%。肝门部胆管癌根治性手术切除率低,病人预后差,生存期短。近年来,尽管在诊断和治疗方面取得了一定进展,但在术前胆道引流、门静脉栓塞、手术切除范围选择、联合血管切除重建、微创手术治疗和肝移植治疗等方面仍存在争议。  相似文献   
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目的 探讨单人经口胆道镜联合射频消融术(radiofrequency ablation,RFA)同台诊治不可切除肝外胆管癌的可行性和安全性。方法 回顾2013年1月至2022年1月期间在杭州市第一人民医院治疗的90例可疑肝外胆管癌患者资料,根据诊治过程最终纳入69例,分为常规分次组(n=34):先经内镜逆行胰胆管造影术(endoscopic retrograde cholangiopancreatography, ERCP)+细胞刷或单人经口胆道镜检查取得活检组织,待获得阳性病理结果再次行ERCP+RFA;同台诊治组(n=35):行ERCP经单人经口胆道镜检查胆道并对病灶行直视下活检,对术中快速病理结果确定为恶性肿瘤的患者同台行RFA。对比两组操作成功率、术后胆红素恢复情况、ERCP次数、术后不良事件发生率、住院时间及费用。结果 两组患者均成功完成内镜下RFA,操作成功率100.0%(69/69)。分次组和同台组总胆红素术后下降50%及以上的患者比例差异无统计学意义[52.94%(18/34)比57.14%(20/35),χ2=0.27,P=0.604]。分次组ERCP次数明显多于同台组[(2.59±0.50)次/人比(1.00±0.00)次/人],差异有统计学意义(t=3.13,P=0.002)。分次组和同台组术后总体不良事件发生率差异无统计学意义[(67.65%(23/34)比65.71%(23/35),χ2=2.83 ,P=0.626]。分次组住院时间明显长于同台组,差异有统计学意义[(17.41±9.13) d比(7.91±3.48) d,t=5.32,P=0.001]。分次组住院费用明显多于同台组,差异有统计学意义[(37 127.88±3 763.77)元比(23 980.69±4 767.15)元,t=6.61,P=0.001]。结论 单人经口胆道镜直视下诊断+活检联合RFA同台诊治不可切除肝外胆管癌可减少ERCP次数,并且不增加术后不良事件发生率,是一种安全有效且具有较高成本-效益比的诊治方法。  相似文献   
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Background and Aim: Cholangiocarcinoma (CCA) is a mucin‐producing cancer that has poor prognosis. Mucin 6 (MUC6) is a mucin that is normally co‐expressed with the trefoil factor family‐2 (TFF2) trefoil peptide. Both MUC6 and TFF2 have been reported to be involved in the progression of many types of cancers. The aim of this study was to determine the expression of MUC6 and TFF2 in CCA tissues and associate these results with clinical data. Methods: MUC6 and TFF2 were detected in CCA tissues by immunohistochemistry. The correlations of MUC6 and TFF2 expressions with clinical data were analyzed. Results: We determined the significant co‐expression of both proteins in serial CCA tissues. The high expressions of MUC6 and TFF2 were demonstrated in 37% and 31% of patients, respectively. The expression levels decreased in the advanced stage of CCA when clinical metastasis was exhibited. The high expression of either protein showed a correlation with prolonged postoperative survival time, but only a high expression of MUC6 is significantly correlated with a 5‐year survival rate. A multivariate Cox regression analysis revealed that a low expression of MUC6, high expression of TFF2, age of patients >56 years, tumor size >5 cm, and poorly‐differentiated histological type were independent, poor prognostic indicators for CCA. Conclusion: MUC6 showed a good correlation with the survival of CCA patients. It may be of value to propose that MUC6 is a good prognostic marker for CCA management.  相似文献   
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