Objective: To explore disease characteristics of primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) and compare the differences between PSC with and without IBD. Methods: Study design was cross sectional. Forty-two patients with PSC who were admitted from January 2000 to January 2021 were included. We analyzed their demographic characteristics, clinical manifestations, concomitant diseases, auxiliary examination, and treatment. Results: The 42 patients were 11-74(43±18) years of age at diagnosis. The concordance rate of PSC with IBD was 33.3%, and the age at PSC with IBD diagnosis was 12-63(42±17) years. PSC patients with IBD had higher incidences of diarrhea and lower incidences of jaundice and fatigue than in those without IBD (all P<0.05). Alanine aminotransferase, total bilirubin, direct bilirubin, total bile acid and carbohydrate antigen 19-9 levels were higher in PSC patients without IBD than in those with IBD (all P<0.05). The positive rates for antinuclear antibodies and fecal occult blood were higher in PSC patients with IBD than in those without IBD (all P<0.05). Patients with PSC complicated with ulcerative colitis mainly experienced extensive colonic involvement. The proportion of 5-aminosalicylic acid and glucocorticoid application in PSC patients with IBD was significantly increased compared with that of PSC patients without IBD (P=0.025). Conclusions: The concordance rate of PSC with IBD is lower at Peking Union Medical College Hospital than in Western countries. Colonoscopy screening may benefit PSC patients with diarrhea or fecal occult blood-positive for early detection and diagnosis of IBD.
Humans ; Child ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Cholangitis, Sclerosing/therapy* ; Cross-Sectional Studies ; Inflammatory Bowel Diseases/diagnosis* ; Colitis, Ulcerative/complications* ; Diarrhea

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*

Humans ; Cholangitis, Sclerosing/diagnosis* ; Immunoglobulin G ; Hormones ; Diagnosis, Differential ; Autoimmune Diseases

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*

Background: Acute cholangitis (AC) in children is a rare but life-threatening infection. Symptoms vary from mild to severe disease. There are no local published data on pediatric AC. Objective: To determine the clinical, biochemical, ultrasonographic, microbiologic features, and treatment outcome of pediatric patients with definite AC. Methodology: Cross-sectional study using medical records of pediatric patients diagnosed with definite AC based on the Modified Tokyo Guidelines of 2018 admitted from January 2016 to June 2021. Results: Twenty-seven patients aged 0 to 18 years old (10.06 + 7.34), predominantly male (51.85%) were included. Choledocholithiasis (22%) and post-Kasai biliary atresia (22%) were the common underlying biliary conditions. Fever (88.89%) was the most frequent presenting symptom. Majority were classified as moderate AC (40.74%). Leukocytosis (mean 16×109/L), elevated inflammatory markers (93.33% with CRP >12mg/L and 100% with serum procalcitonin >0.25ng/mL), hyperbilirubinemia (total bilirubin 192.54±126.87umol/L) and elevated alanine transferases (mean 59 IU/L) were noted. Twenty-one out of 27 cases (87%) had a negative blood culture. Only 4 patients underwent bile culture, of which two (50%) grew Klebsiella pneumoniae resistant to empiric antibiotics. Dilated biliary ducts were observed on abdominal ultrasound in 92.59% of patients. Ampicillin-sulbactam (29.63%) was the most commonly utilized antibiotic. Discharge rate was high (88.89%). Conclusions AC affects all pediatric age groups but clinical presentations vary. Drug resistant organisms are a significant concern but despite this, favorable outcomes have been documented.
Cholangitis ; Child ; Choledocholithiasis

Cholangitis/therapy* ; Humans ; Liver Cirrhosis, Biliary/therapy*

Anastomosis, Surgical/adverse effects* ; Cholangitis/surgery* ; Gastroenterostomy/adverse effects* ; Humans ; Pancreatitis/complications* ; Suppuration/surgery*

In 2015, the first Chinese consensus on the diagnosis and management of primary sclerosing cholangitis was issued. In the past years, more data have emerged from the literature. Herein, the Autoimmune Liver Disease Group of the Chinese Society of Hepatology organized an expert group to review the evidence and updated the recommendations to formulate the guidelines. There are 21 recommendations on PSC clinical practice. To facilitate the differentiation between PSC and IgG4-related sclerosing cholangitis, 10 recommendations on IgG4-SC are also attached. These guidelines aim to provide a working reference for the management of PSC and IgG4-SC.
Autoimmune Diseases/diagnosis* ; Cholangitis, Sclerosing/therapy* ; Diagnosis, Differential ; Humans ; Immunoglobulin G

In 2015, the Chinese Society of Hepatology and Chinese Society of Gastroenterology issued a consensus on the diagnosis and management of primary biliary cholangitis (PBC). In the past years, more clinical studies have been reported in the field of PBC. To provide guidance to the clinical diagnosis and management of patients with PBC, the Chinese Society of Hepatology invited a panel of experts to assess the new clinical evidence and formulated the current guidelines which comprises 26 clinical recommendations.
Cholangitis/therapy* ; Consensus ; Gastroenterology ; Humans ; Liver Cirrhosis, Biliary/therapy*

BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) is the endoscopic modality of choice for the treatment of biliary and pancreatic diseases. However, patients with cirrhosis, particularly those with decompensated cirrhosis, are believed to be at increased risk for complications associated with ERCP. There is a paucity of literature describing the outcomes of ERCP for patients with cirrhosis. This study aimed to investigate the outcomes of ERCP for cirrhosis patients, especially adverse events, and evaluated its safety and efficacy. METHODS: We performed a multicenter, retrospective study of all patients at Beijing Friendship Hospital affiliated to Capital Medical University, Xijing Hospital affiliated to Air Force Military Medical University, Beijing Youan Hospital affiliated to Capital Medical University, and the Fifth Medical Center of the People's Liberation Army General Hospital from June 2003 to August 2019. The adverse events of inpatient ERCP for patients with ( n  = 182) and without liver cirrhosis (controls; n  = 385) were compared. RESULTS: A total of 567 patients underwent ERCP between January 2003 and December 2019 were enrolled in this study. Compared to patients without cirrhosis, patients with cirrhosis were at higher risk for postoperative complications (odds ratio [OR], 4.172; 95% confidence interval [CI], 1.232-7.031; P  < 0.001) such as postoperative pancreatitis (OR, 2.026; 95% CI, 1.002-4.378; P  = 0.001) and cholangitis (OR, 3.903; 95% CI, 1.001-10.038; P  = 0.036). The main indications for ERCP for patients with cirrhosis in this study included choledocholithiasis (101 cases; 55.5%), benign bile duct strictures (46 cases; 25.3%), and malignant bile duct strictures (28 cases; 15.4%). Among them, 23 patients (12.6%) underwent balloon dilation and 79 patients (43.4%) underwent sphincterotomy. Of the patients with cirrhosis, delayed bleeding occurred in ten patients (5.5%), postoperative pancreatitis occurred in 80 patients (44.0%), and postoperative cholangitis occurred in 25 patients (13.7%). An additional multivariate analysis showed that the total bilirubin (TBIL) level (OR, 4.58; 95% CI, 2.37-6.70) and Child-Pugh score of C (OR, 3.11; 95% CI, 1.04-5.37) were risk factors for postoperative complications in patients with cirrhosis. CONCLUSIONS Compared with the general population of patients undergoing ERCP, patients with cirrhosis were more prone to postoperative pancreatitis and cholangitis. TBIL levels and Child-Pugh scores were risk factors for postoperative complications in patients with cirrhosis.
Humans ; Cholangiopancreatography, Endoscopic Retrograde/adverse effects* ; Retrospective Studies ; Constriction, Pathologic ; Risk Factors ; Liver Cirrhosis/complications* ; Pancreatitis/etiology* ; Postoperative Complications/epidemiology* ; Cholangitis