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*

Aged ; Bile Duct Diseases ; complications ; diagnostic imaging ; pathology ; Bile Duct Neoplasms ; diagnosis ; Carcinoma, Hepatocellular ; complications ; diagnostic imaging ; pathology ; Diagnosis, Differential ; Humans ; Jaundice, Obstructive ; etiology ; Klatskin Tumor ; diagnosis ; Liver Neoplasms ; complications ; diagnostic imaging ; pathology ; Male ; Middle Aged ; Thrombosis ; complications ; diagnostic imaging ; pathology ; Tomography, X-Ray Computed

OBJECTIVETo investigate the pathogenesis of ischemic-type biliary lesions (ITBLs) in post-liver transplant patients and the possible therapeutic mechanisms of sirolimus.

METHODSThe clinic data of 32 post-liver transplant patients with ITBLs from May 2004 to December 2010 was analyzed. There were including 25 male and 7 female patients with a median age of 46 years (ranging from 19 to 61 years). Patients were divided into those who received sirolimus (sirolimus group) and those who did not (control group). The expression of IL-2, FoxP3, and IL-10 in the portal area, liver function indexes, and bile duct injury score were assessed pre-ITBL, when ITBLs were identified, and after 6 months of sirolimus treatment.

RESULTSCompared with pre-ITBL optical density (OD) values, there was a significantly increase in IL-2 OD(0.138 ± 0.050 in control group and 0.141 ± 0.052 in sirolimus group), but not FoxP3 and IL-10 OD in both groups at the time ITBLs were diagnosed. After 6 months of treatment, the IL-2, FoxP3, and IL-10 OD values in the control group were not different from those when ITBLs were diagnosed. There was a significant reduction in post-therapy IL-2 OD(0.107 ± 0.043, t = 2.087, P = 0.044), and a significant elevation in FoxP3(0.213 ± 0.039) and IL-10 OD(0.187 ± 0.048) in sirolimus group as compared with those when ITBLs were diagnosed(t = -3.822 and -4.350, both P < 0.01). There was a significant increase in serum levels of ALT, AST, total bilirubin, γ-glutamyl transpeptidase and ALP at the time ITBLs were diagnosed compared with pre-ITBL levels in both groups. After 6 months of treatment, the above indexes had not changed in the control group, but significantly improved in the sirolimus group, and the bile duct injury score in the sirolimus group had significantly decreased(4.4 ± 2.4, Z = -2.568, P = 0.010). The 1-year and 3-year graft survival rates in the control group were 6/13 and 5/13, respectively, and 17/19 and 13/19, respectively, in the sirolimus group (χ(2) = 7.166, P = 0.007; χ(2) = 5.398, P = 0.020, respectively).

CONCLUSIONSSirolimus can downregulate IL-2 expression and upregulate FoxP3 and IL-10 expression, thereby stimulating FoxP3+ Treg cells, suppressing immunopathological damage, and promoting epithelial repair in bile ducts.

Adult ; Bile Duct Diseases ; drug therapy ; Female ; Forkhead Transcription Factors ; metabolism ; Gene Expression Regulation ; drug effects ; Humans ; Interleukin-10 ; metabolism ; Interleukin-2 ; metabolism ; Ischemia ; diet therapy ; Liver Transplantation ; Male ; Middle Aged ; Postoperative Complications ; drug therapy ; Sirolimus ; therapeutic use ; Young Adult

IgG4-related systemic diseases are characterized by a diffuse or mass forming inflammatory reaction rich in lymphocytes and IgG4-positive plasma cells (lymphoplasmacytic infiltration), fibrosclerosis of variable organs and obliterative phlebitis. They usually involve various organs including the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate. However, most of them are accompanied by autoimmune pancreatitis, and good response to steroid treatment is one of the hallmarks of this disease. We report a case of an 67-year-old man with IgG4 associated sclerosing cholangitis, who was diagnosed by endoscopic retrograde cholangiopancreatography and successfully treated with steroid therapy.
Aged ; Anti-Inflammatory Agents/therapeutic use ; Autoimmune Diseases/complications/diagnosis ; Bile Ducts, Intrahepatic/pathology/ultrasonography ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis, Sclerosing/complications/*diagnosis/drug therapy ; Common Bile Duct/pathology/ultrasonography ; Humans ; Immunoglobulin G/*blood ; Immunohistochemistry ; Male ; Pancreatitis/complications/diagnosis ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed

Percutaneous access to the surgical bed after pancreaticoduodenectomy can be a challenge, due to the post-operative anatomy alteration. However, immediate complications, such as surgical bed abscess or suspected tumor recurrence, are often best accessed percutaneously, as open surgical or endoscopic approaches are often difficult, if not impossible. We, hereby, describe a safe approach that is highly replicable, in accessing the surgical bed for percutaneous intervention, following pancreaticoduodenectomy.
Abscess/radiography/therapy ; Bile Duct Neoplasms/pathology/radiography ; Biopsy/methods ; Catheterization/*methods ; Cholangiocarcinoma/pathology/radiography ; Drainage/instrumentation/*methods ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local/pathology/radiography ; Pancreatic Diseases/radiography/therapy ; *Pancreaticoduodenectomy ; Postoperative Complications/radiography/*therapy ; Radiography, Interventional/methods ; Retroperitoneal Space

Hepatic peribiliary cysts (HPCs) are characterized by cystic dilatations of the peribiliary glands located throughout the branches of the biliary systems. Specifically, they are mainly located along the hepatic hilum and major portal tracts. The natural history and prognosis of HPCs are uncertain. In fact, almost all HPCs have been discovered incidentally during radiological examination or autopsy, and they are considered to be clinically harmless. Recently, several cases of HPCs associated with obstructive jaundice or liver failure were reported in patients with pre-existing liver disease in several studies. However, until now there have been no reports of such a case in Korea. Herein, we report a case of HPCs that show a disease course with a poor prognosis. These HPCs developed in a 47-year-old man with progressive alcoholic liver cirrhosis.
Bile Duct Diseases/complications/*diagnosis/radiography ; Cholangiopancreatography, Magnetic Resonance ; Cysts/*complications/radiography ; Humans ; Jaundice, Obstructive/etiology ; Liver Cirrhosis, Alcoholic/complications/*diagnosis/radiography ; Male ; Middle Aged ; Tomography, X-Ray Computed

OBJECTIVE: To evaluate the risk of hepatectomy by detecting liver functional reserve preoperatively for patients with primary hepatolithiasis. METHODS: The clinical data of 134 patients with primary hepatolithiasis who underwent hepatectomy were reviewed. In terms of evaluation methods for preoperative liver functional reserve they were divided into a Child-Pugh group (group CP) and an indocyanine green group (group ICG). The preoperative and intraoperative parameters, and the incidence of postoperative complications were analyzed. RESULTS: Liver failure was more common in group CP (12.85%) than that in group ICG (1.56%, P<0.05). The overall complication rate in group CP (37.14%) was higher than in group ICG (18.75%, P<0.05). CONCLUSION ICG15 retention test is more accurate in evaluating liver functional reserve than Child-Pugh scoring system. It may predict the postoperative liver failure in patients with primary hepatolithiasis undergoing hepatectomy, decrease postoperative complications, and increase operation safety.
Adult ; Bile Duct Diseases ; surgery ; Bile Ducts, Intrahepatic ; surgery ; Cholelithiasis ; surgery ; Female ; Hepatectomy ; adverse effects ; Humans ; Liver Failure ; etiology ; prevention & control ; Liver Function Tests ; Male ; Middle Aged ; Patient Selection ; Postoperative Complications ; prevention & control ; Preoperative Care ; Retrospective Studies ; Risk Factors

BACKGROUNDThe prevalence of chronic pancreatitis has increased during recent years in Asia-Pacific areas as well as in China. The etiologies vary in different regions and periods. This study aimed to investigate the changing etiologies of chronic pancreatitis within 20 years at Peking Union Medical College Hospital in China.

METHODSRetrospective analysis of the etiologies of 636 cases of chronic pancreatitis at Peking Union Medical College Hospital from 1990 to 2010 was performed. Patients were divided into two groups according to two time periods (1990 - 2000 and 2001 - 2010). Statistical analysis was performed using the chi-square test.

RESULTSThe morbidity rate of chronic pancreatitis in China has recently increased. The main etiology changed from biliary diseases in the 1990s (decreased from 36.8% to 28.1%) to alcohol abuse after the year 2000 (increased from 26.5% to 36.8%). The main etiology of biliary diseases is stones in the cholecyst or bile duct, and the percentage of cholecystitis cases has increased. Autoimmune disease, including autoimmune pancreatitis, has increased quickly and currently accounts for 7.3% of cases because a greater number of autoimmune pancreatitis cases are being diagnosed. Approximately 9.5% of chronic pancreatitis cases are caused by multiple factors such as alcohol abuse and bile duct stones. Other factors include cholecystectomy and acute pancreatitis.

CONCLUSIONSThe main etiology of chronic pancreatitis has changed from biliary disease to alcohol abuse in recent years. Autoimmune factors have also obviously increased.

Adult ; Alcoholism ; complications ; Bile Duct Diseases ; complications ; China ; epidemiology ; Female ; Humans ; Male ; Middle Aged ; Pancreatitis, Chronic ; epidemiology ; etiology ; immunology ; Retrospective Studies ; Risk Factors

OBJECTIVETo explore the potential risk factors of intrahepatic cholangiocarcinoma (ICC) in China.

METHODA case-control study including 317 patients with pathologically confirmed ICC and 634 healthy individuals was conducted. The cases and controls were matched in age, sex and inhabitancy. Data were statistically analyzed by Chi-square test and conditional logistic regression.

RESULTSUnivariate analysis showed significant difference in HBsAg seropositivity, liver cirrhosis, hepatolithiasis, choledocholithiasis and schistosomiasis between ICC patients and healthy controls (P < 0.05). Multivariate analysis confirmed that HBsAg seropositivity, liver cirrhosis, hepatolithiasis and hepatic schistosomiasis were associated with ICC, and their adjusted odds ratio (95% confidence interval) were 10.265 (6.676-15.783), 13.101 (5.265-32.604), 18.242 (3.580-92.958), 18.435 (1.930-176.082), 15.102 (4.607-49.499) and 11.820 (3.522-39.668), respectively. The incidence of hepatic cyst, cholecystolithiasis, hepatic hemangioma, fatty liver, diabetes mellitus, smoking and drinking were not significantly different between ICC patients and controls.

CONCLUSIONSThe HBV infection, liver cirrhosis, hepatolithiasis and hepatic schistosomiasis may be the risk factors for ICC in China.

Adult ; Aged ; Bile Duct Neoplasms ; epidemiology ; etiology ; Bile Ducts, Intrahepatic ; Case-Control Studies ; Cholangiocarcinoma ; epidemiology ; etiology ; Cholelithiasis ; complications ; epidemiology ; Female ; Hepatitis B ; complications ; epidemiology ; Hepatitis B Surface Antigens ; blood ; Hepatitis B e Antigens ; blood ; Humans ; Liver Cirrhosis ; complications ; epidemiology ; Liver Diseases ; complications ; epidemiology ; Logistic Models ; Male ; Middle Aged ; Odds Ratio ; Risk Factors

OBJECTIVETo investigate the change of bile composition and its role in bile duct injury after orthotopic liver transplantation (OLT).

METHODSRats were randomly divided into 3 groups: group A (sham surgery), group B (OLT with 1 h cold preservation), group C (OLT with 12 h cold preservation). The arterialized rat liver transplantation model with biliary extra-drainage was used in group B and C. Animals were sacrificed at posttransplant 1, 3, 5, 7, 10 and 14 day. Routine bile chemistry and pathological assays were performed.

RESULTSCold preservation/reperfusion injury (CPRI) could repress the secretion of bile salts and phospholipid. However, in contrast with a rapid increase of bile salt secretion, the biliary secretion of phospholipid recovered more slowly, leading to an abnormal high bile salts/phospholipid ratio early after transplantation. Further analysis suggested that the secretion of bile salts correlated strongly with biochemical and histopathological signs of bile duct injury.

CONCLUSIONSCPRI can lead to great changes of graft bile composition, which plays a role in the pathogenesis of bile duct injury following liver transplantation.

Animals ; Bile ; metabolism ; Bile Acids and Salts ; metabolism ; Bile Duct Diseases ; etiology ; Bile Ducts, Intrahepatic ; pathology ; Cold Ischemia ; Disease Models, Animal ; Liver Transplantation ; Male ; Postoperative Complications ; etiology ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Reperfusion Injury ; complications ; metabolism ; pathology