What are the new contents of the guideline since 2010?A.Patients with primary and non-primary sclerosing cholangitis (PSC) are included in these guidelines for the diagnosis and management of cholangiocarcinoma.B.Define "related stricture" as any biliary or hepatic duct stricture accompanied by the signs or symptoms of obstructive cholestasis and/or bacterial cholangitis.C.Patients who have had an inconclusive report from MRI and cholangiopancreatography should be reexamined by high-quality MRI/cholangiopancreatography for diagnostic purposes. Endoscopic retrograde cholangiopancreatography should be avoided for the diagnosis of PSC.D. Patients with PSC and unknown inflammatory bowel disease (IBD) should undergo diagnostic colonoscopic histological sampling, with follow-up examination every five years until IBD is detected.E. PSC patients with IBD should begin colon cancer monitoring at 15 years of age.F. Individual incidence rates should be interpreted with caution when using the new clinical risk tool for PSC for risk stratification.G. All patients with PSC should be considered for clinical trials; however, if ursodeoxycholic acid (13-23 mg/kg/day) is well tolerated and after 12 months of treatment, alkaline phosphatase (γ- Glutamyltransferase in children) and/or symptoms are significantly improved, it can be considered to continue to be used.H. Endoscopic retrograde cholangiopancreatography with cholangiocytology brushing and fluorescence in situ hybridization analysis should be performed on all patients suspected of having hilar or distal cholangiocarcinoma.I.Patients with PSC and recurrent cholangitis are now included in the new unified network organ sharing policy for the end-stage liver disease model standard.J. Liver transplantation is recommended after neoadjuvant therapy for patients with unresectable hilar cholangiocarcinoma with diameter < 3 cm or combined with PSC and no intrahepatic (extrahepatic) metastases.
【更新要点】： 自2010年至今指南有哪些新内容？A.纳入原发性和非原发性硬化性胆管炎（primary sclerosing cholangitis，PSC）患者的胆管癌的诊断和管理指引。B.引入术语相关狭窄一词，定义为与梗阻性胆汁淤积和/或细菌性胆管炎的体征或症状相关的任何胆总管或肝管的胆道狭窄。C.对于磁共振成像和胰胆管造影结果不明确的患者，出于诊断目的，应复查高质量的磁共振成像/胰胆管造影。应避免为了诊断PSC进行内镜逆行胰胆管造影。D.对于未知炎症性肠病（inflammatory bowel disease，IBD）的PSC患者，应进行诊断性结肠镜组织学取样检查，每5年复查1次，直至检测到IBD。E.对于合并IBD的PSC患者，应从15岁开始进行结肠癌监测。F.PSC的临床风险新工具可用于风险分层，但应谨慎解释个体发生率。G.所有PSC患者应考虑参与临床试验；然而，如果熊去氧胆酸（13~23 mg·kg(-1)·d(-1)）耐受良好，且在治疗12个月后碱性磷酸酶(儿童中的γ-谷氨酰转移酶)和/或症状有显著改善，则可以考虑继续使用。H.所有疑似肝门部或远端胆管癌的患者均应进行内镜逆行胰胆管造影胆道细胞学刷检和荧光原位杂交分析。I.除了PSC和复发性胆管炎的患者，终末期肝病模型标准有统一的网络器官共享新政策。对于不可切除的肝门部胆管癌，直径< 3 cm或合并PSC且无肝内(外)转移的患者，建议新辅助治疗后进行肝移植。.
Keywords: Cholangiocarcinoma; Diagnosis; Follow-up; Inflammatory bowel disease; Primary sclerosing cholangitis; Therapy.