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.
Child ; Humans ; Cholangitis, Sclerosing/diagnosis* ; Constriction, Pathologic/complications* ; In Situ Hybridization, Fluorescence ; Cholangiocarcinoma/therapy* ; Liver Diseases/complications* ; Cholestasis ; Inflammatory Bowel Diseases/therapy* ; Bile Ducts, Intrahepatic/pathology* ; Bile Duct Neoplasms/therapy*

Drug-induced bile duct injury is a specific kind of drug-induced liver injury that has two main pathological types, namely ductopenia, or vanishing bile duct syndrome, and secondary sclerosing cholangitis. However, in recent years, the reports of new drugs that cause bile duct injury have been constantly increasing, and these drugs have different clinicopathological features and a novel pathogenesis. Therefore, this paper summarizes and analyzes the progress and challenges in the etiology, pathogenesis, diagnosis and treatment, and other aspects of drug-induced bile duct injury.
Humans ; Cholestasis/chemically induced* ; Cholangitis, Sclerosing/diagnosis* ; Chemical and Drug Induced Liver Injury/pathology* ; Bile Ducts/pathology*

BACKGROUND/AIMS: The mortality rate of pyogenic liver abscess (PLA) has decreased dramatically, but it remains a potentially life threatening disease. Most cases are cryptogenic or occur in elderly men with underlying biliary tract disease. Although several studies have addressed the characteristics and etiology of PLA, research on factors affecting PLA-associated mortality is lacking. This study intended to identify the clinical and radiological features, pathogens, complications, and predictors of mortality in Korean PLA patients. METHODS: The medical records of 231 PLA patients diagnosed at Yeungnam University Medical Center between January 2010 and January 2014 were analyzed. A diagnosis of PLA was made based on imaging studies and blood and abscess cultures. The clinical, radiological, and laboratory findings of patients were analyzed. RESULTS: The mean patient age was 64.0±12.9 years and the male to female ratio was 1.5:1. Klebsiella pneumoniae was the predominant organism isolated from hepatic abscesses (69.9%) and blood (74.2%). The most common complication was pleural effusion (35.8%) and most common co-infection was cholangitis (8.2%). The overall mortality rate of PLA was 6.9% (16/231), and was significantly higher in patients with a history of liver abscess (OR 5.970, 95% CI 1.207-29.529; p=0.028), bilirubinemia (>2 mg/dL) (OR 9.541, 95% CI 2.382-38.216; p=0.001), thrombocytopenia (<140×10(3)/µL) (OR 4.396, 95% CI 1.130-17.106; p=0.033), or anemia (<12 g/dL) (OR 13.277, 95% CI 1.476-119.423; p=0.021). CONCLUSIONS: The prognosis of PLA appears to be dependent on underlying pathologies and severity of condition. More aggressive treatment should be considered if a poor prognosis is expected.
Abscess ; Academic Medical Centers ; Aged ; Anemia ; Biliary Tract Diseases ; Cholangitis ; Coinfection ; Diagnosis ; Female ; Humans ; Hyperbilirubinemia ; Klebsiella pneumoniae ; Liver Abscess ; Liver Abscess, Pyogenic* ; Male ; Medical Records ; Mortality* ; Pathology ; Pleural Effusion ; Prognosis ; Retrospective Studies* ; Risk Factors ; Thrombocytopenia

Sclerosing cholangitis is a spectrum of chronic progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts, which can be classified as primary and secondary sclerosing cholangitis. Primary sclerosing cholangitis is a chronic progressive liver disease of unknown cause. On the other hand, secondary sclerosing cholangitis has identifiable causes that include immunoglobulin G4-related sclerosing disease, recurrent pyogenic cholangitis, ischemic cholangitis, acquired immunodeficiency syndrome-related cholangitis, and eosinophilic cholangitis. In this review, we suggest a systemic approach to the differential diagnosis of sclerosing cholangitis based on the clinical and laboratory findings, as well as the typical imaging features on computed tomography and magnetic resonance (MR) imaging with MR cholangiography. Familiarity with various etiologies of sclerosing cholangitis and awareness of their typical clinical and imaging findings are essential for an accurate diagnosis and appropriate management.
Adult ; Aged ; Aged, 80 and over ; Bile Ducts/*pathology ; Cholangiography/*methods ; Cholangitis/diagnosis/*pathology ; Cholangitis, Sclerosing/*diagnosis/pathology ; Cholestasis/diagnosis/*pathology ; Chronic Disease ; Constriction, Pathologic/diagnosis ; Diagnosis, Differential ; Female ; Humans ; Immunoglobulin G/immunology ; Liver/pathology ; Magnetic Resonance Imaging/methods ; Male ; Middle Aged ; Tomography, X-Ray Computed/methods

Approximately 3-15% of patients who have undergone removal of bile duct stones with endoscopic sphincterotomy have recurrence of stones which often presents as acute cholangitis. Despite better understanding on the factors and mechanisms underlying the recurrence of bile duct stones achieved during the past few decades, endoscopic removal still remains the mainstay of management for recurrent bile duct stones. Recently investigated and suggested management of recurrent bile duct stones are highlighted in this review.
Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis/pathology ; Gallstones/surgery/*therapy ; Humans ; Recurrence ; Risk Factors ; Sphincterotomy, Endoscopic ; Ursodeoxycholic Acid/administration & dosage

OBJECTIVE: To investigate the outcomes of percutaneous unilateral metallic stent placement in patients with a malignant obstruction of the biliary hila and a contralateral portal vein steno-occlusion. MATERIALS AND METHODS: Sixty patients with a malignant hilar obstruction and unilobar portal vein steno-occlusion caused by tumor invasion or preoperative portal vein embolization were enrolled in this retrospective study from October 2010 to October 2013. All patients were treated with percutaneous placement of a biliary metallic stent, including expanded polytetrafluoroethylene (ePTFE)-covered stents in 27 patients and uncovered stents in 33 patients. RESULTS: A total of 70 stents were successfully placed in 60 patients. Procedural-related minor complications, including self-limiting hemobilia (n = 2) and cholangitis (n = 4) occurred in six (10%) patients. Acute cholecystitis occurred in two patients. Successful internal drainage was achieved in 54 (90%) of the 60 patients. According to a Kaplan-Meier analysis, median survival time was 210 days (95% confidence interval [CI], 135-284 days), and median stent patency time was 133 days (95% CI, 94-171 days). No significant difference in stent patency was observed between covered and uncovered stents (p = 0.646). Stent dysfunction occurred in 16 (29.6%) of 54 patients after a mean of 159 days (range, 65-321 days). CONCLUSION: Unilateral placement of ePTFE-covered and uncovered stents in the hepatic lobe with a patent portal vein is a safe and effective method for palliative treatment of patients with a contralateral portal vein steno-occlusion caused by an advanced hilar malignancy or portal vein embolization. No significant difference in stent patency was detected between covered and uncovered metallic stents.
Adult ; Aged ; Aged, 80 and over ; Biliary Tract Neoplasms/surgery ; Cholangitis/etiology ; Cholestasis/*surgery ; Female ; Hemobilia/etiology ; Humans ; Kaplan-Meier Estimate ; Liver/blood supply/pathology/surgery ; Liver Neoplasms/surgery ; Male ; Middle Aged ; Palliative Care/methods ; Polytetrafluoroethylene ; Portal Vein/pathology/*surgery ; Retinal Vein Occlusion/*surgery ; Retrospective Studies ; Stents/*adverse effects ; Treatment Outcome

Biliary hamartoma and congenital hepatic fibrosis belong to fibrocystic disorders originating from ductal plate malformation. A 66-year-old man who had incidentally been diagnosed with biliary hamartoma two years ago presented to us with recurrent acute cholangitis. In the first episode, he had presented with septic shock and was treated with endoscopic retrograde cholangiopancreatography (ERCP) and cholecystectomy under the diagnosis of acute cholecystitis and cholangitis. However, during a two-month follow-up period, the patient experienced four episodes of acute cholangitis. Because he showed normal ERCP, and biliary hamartoma is usually asymptomatic, a liver biopsy was performed. Pathology revealed combined features of biliary hamartoma and congenital hepatic fibrosis, characterized as periportal fibrosis and intrahepatic ductular dysplasia. During follow-up for the last six months, he had experienced two episodes of acute cholangitis and was treated with antibiotics. A follow-up abdominal CT scan revealed aggravated hepatosplenomegaly compared to that of two years ago. We report a case of combined congenital hepatic fibrosis and biliary hamartoma and a literature review.
Aged ; Anti-Bacterial Agents ; Biopsy ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis* ; Cholecystectomy ; Cholecystitis, Acute ; Diagnosis ; Fibrosis* ; Follow-Up Studies ; Hamartoma* ; Humans ; Liver ; Pathology ; Shock, Septic ; Tomography, X-Ray Computed

IgG4-associated cholangitis can mimic hilar cholangiocarcinoma. Previously reported patients with IgG4-associated cholangitis mimicking cholangiocarcinoma had elevated serum IgG4 levels and long-segment biliary strictures. However, in the absence of other diagnostic criteria for malignancy, IgG4-associated cholangitis should remain a consideration among patients with normal serum IgG4 and a hilar mass suspicious for cholangiocarcinoma. The presence of a hilar mass and a malignant-appearing biliary stricture in two patients with normal serum IgG4 prompted further evaluation and subsequent concomitant liver and bile duct resection and reconstruction. The diagnosis of IgG4-associated cholangitis was established during the pathologic evaluation of the resected specimens. IgG4-associated cholangitis is a known imitator of hilar cholangiocarcinoma and should be considered in the differential diagnosis even among serologically IgG4-negative patients with a hilar mass prior to operative resection.
Aged ; Bile Ducts/pathology/surgery ; Cholangitis/blood/*diagnosis ; Diagnosis, Differential ; Humans ; Immunoglobulin G/*blood ; Klatskin Tumor/blood/*diagnosis ; Liver/pathology/surgery ; Male

Ulcerative colitis is an idiopathic chronic inflammatory bowel disease and its incidence in Korea has rapidly increased over the past two decades. Since ulcerative colitis is associated with increased risk for colorectal cancer, annual or biannual colonoscopy with four quadrant random biopsies at every 10 cm segments has been recommended for surveillance of colitic cancer in patients with long standing left-sided or extensive colitis. Recent epidemiologic data and meta-analysis suggest that the increment of colorectal cancer risk in ulcerative colitis was not larger than that of previous studies. Moreover, in addition to the extent and duration of colitis, other risk factors such as family history of colorectal cancer, primary sclerosing cholangitis, stricture, pseudopolyps, and histologic severity of inflammation have been recognized. As a result, updated guidelines provide surveillance strategies adjusted to the individual patient's risk for colitic cancer. Regarding surveillance method, target biopsy under panchromoendoscopy is preferentially recommended rather than random biopsy.
Cholangitis, Sclerosing/complications ; Colitis, Ulcerative/*complications ; Colon/pathology ; Colorectal Neoplasms/epidemiology/*etiology ; Humans ; Inflammatory Bowel Diseases/complications ; Polyps ; Risk Factors

Hepatocellular carcinomas (HCCs) in patients with Crohn's disease (CD) without underlying chronic hepatitis or liver cirrhosis are extremely rare. Previously reported cases occurred in patients who had developed CD at a young age and had been treated with immunosuppressive agents long-term. We herein report the first case of HCC in a 34-year-old patient with CD in Korea. The patient was treated with azathioprine for 14 years and had undergone repeated surgeries for CD. During the follow-up period, the patient was hospitalized for colon perforation and pericolic abscess formation. Computed tomography showed a liver mass, and HCC was diagnosed based on liver biopsy. The patient underwent right hemicolectomy for colon perforation and transcatheter arterial chemoembolization followed by radiofrequency ablation for the HCC. The present case is similar to previously reported cases with the exception of the liver pathology findings, which exhibited neither primary sclerosing cholangitis nor focal hepatic glycogenolysis.
Abscess ; Adult ; Azathioprine ; Biopsy ; Carcinoma, Hepatocellular* ; Catheter Ablation ; Cholangitis, Sclerosing ; Colon ; Crohn Disease* ; Follow-Up Studies ; Glycogenolysis ; Hepatitis, Chronic ; Humans ; Immunosuppressive Agents ; Korea ; Liver ; Liver Cirrhosis ; Pathology ; Infliximab