-
The Journal of Medical Investigation :... 2017Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an organ-specific autoimmune disease that predominantly affects middle-aged women and... (Review)
Review
Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an organ-specific autoimmune disease that predominantly affects middle-aged women and is characterized by the chronic progressive destruction of small intrahepatic bile ducts with portal inflammation and, ultimately, fibrosis. The serological hallmark of PBC is the presence of anti-mitochondrial autoantibodies (AMA). Several mechanisms have been proposed for immune-mediated bile duct damage in PBC, including the roles of T cells, B cells, other cell phenotypes, and AMA. A sign of fragility of biliary epithelial cells caused by apoptosis, senescence, and autophagy has also been noted. Several complex steps and mechanisms appear to be involved in the induction and progression of cholangitis and biliary degeneration in patients with PBC. J. Med. Invest. 64: 7-13, February, 2017.
Topics: Autoantibodies; B-Lymphocytes; Bile Ducts; Epithelial Cells; Female; Humans; Immunoglobulin M; Liver Cirrhosis, Biliary; Male; Mitochondria; T-Lymphocytes
PubMed: 28373632
DOI: 10.2152/jmi.64.7 -
Diagnostics (Basel, Switzerland) Jun 2020A biliary stricture is an area of narrowing in the extrahepatic or intrahepatic biliary system. The majority of biliary strictures are caused by malignancies,... (Review)
Review
A biliary stricture is an area of narrowing in the extrahepatic or intrahepatic biliary system. The majority of biliary strictures are caused by malignancies, particularly cholangiocarcinoma and pancreatic adenocarcinoma. Most malignant biliary strictures are unresectable at diagnosis. Treatment of these diseases historically required surgical procedures, however, the development of endoscopic techniques has provided alternative minimally invasive treatment options to improve patient quality of life and survival with unresectable disease. While endoscopic retrograde cholangiopancreatography with stent placement has been the cornerstone of biliary drainage for decades, cutting edge endoscopic developments, including radiofrequency ablation and endoscopic ultrasound-guided biliary drainage, offer new therapy options to patients that historically have a poor quality of life and a grim prognosis. In this review, we explore the endoscopic techniques that have contributed to revolutionary advancements in the endoscopic management of malignant biliary strictures.
PubMed: 32532018
DOI: 10.3390/diagnostics10060390 -
BMJ Open Gastroenterology May 2022Liver transplantation (LT) is the only curative therapy in patients with end-stage liver disease. Long-term survival is excellent, yet LT recipients are at risk of... (Review)
Review
Liver transplantation (LT) is the only curative therapy in patients with end-stage liver disease. Long-term survival is excellent, yet LT recipients are at risk of significant complications. Biliary complications are an important source of morbidity after LT, with an estimated incidence of 5%-32%. Post-LT biliary complications include strictures (anastomotic and non-anastomotic), bile leaks, stones, and sphincter of Oddi dysfunction. Prompt recognition and management is critical as these complications are associated with mortality rates up to 20% and retransplantation rates up to 13%. This review aims to summarise our current understanding of risk factors, natural history, diagnostic testing, and treatment options for post-transplant biliary complications.
Topics: Biliary Tract; Biliary Tract Diseases; Cholangiopancreatography, Endoscopic Retrograde; Humans; Liver Transplantation; Transplants
PubMed: 35552193
DOI: 10.1136/bmjgast-2021-000778 -
Seminars in Liver Disease Feb 2015Cholangiocytes, the epithelial cells lining the biliary tree, represent only a small portion of the total liver cell population (3-5%), but they are responsible for the... (Review)
Review
Cholangiocytes, the epithelial cells lining the biliary tree, represent only a small portion of the total liver cell population (3-5%), but they are responsible for the secretion of up to 40% of total daily bile volume. In addition, cholangiocytes are the target of a diverse group of liver diseases affecting the biliary tract, the cholangiopathies; for most of these conditions, the pathological mechanisms are unclear. MicroRNAs (miRNAs) are small, noncoding RNAs that posttranscriptionally regulate gene expression. Thus, it is not surprising that altered miRNA profiles underlie the dysregulation of several proteins involved in the pathobiology of the cholangiopathies, as well as showing promise as diagnostic and prognostic tools. Here the authors review recent work relevant to the role of miRNAs in the etiopathogenesis of several of the cholangiopathies (i.e., fibroinflammatory cholangiopathies and polycystic liver diseases), discuss their value as prognostic and diagnostic tools, and provide suggestions for further research.
Topics: Bile Duct Neoplasms; Bile Ducts, Intrahepatic; Biliary Atresia; Biliary Tract; Biliary Tract Diseases; Cholangiocarcinoma; Cholangitis, Sclerosing; Epithelial Cells; Humans; Liver Cirrhosis, Biliary; MicroRNAs
PubMed: 25632932
DOI: 10.1055/s-0034-1397346 -
Current Opinion in Pediatrics Jun 2015The purpose of this study is to review advances in both the pathogenesis and clinical management of biliary atresia. (Review)
Review
PURPOSE OF REVIEW
The purpose of this study is to review advances in both the pathogenesis and clinical management of biliary atresia.
RECENT FINDINGS
Immunologic studies have further characterized roles of helper T-cells, B-cells, and natural killer cells in the immune dysregulation following viral replication within and damage of biliary epithelium. Prominin-1-expressing portal fibroblasts may play an integral role in the biliary fibrosis associated with biliary atresia. A number of genetic polymorphisms have been characterized as leading to susceptibility for biliary atresia. Postoperative corticosteroid therapy is not associated with greater transplant-free survival. Newborn screening may improve outcomes of infants with biliary atresia and may also provide a long-term cost benefit.
SUMMARY
Although recent advances have enhanced our understanding of pathogenesis and clinical management, biliary atresia remains a significant challenge requiring further investigation.
Topics: Bile Ducts, Intrahepatic; Biliary Atresia; Child; Child, Preschool; Early Diagnosis; Humans; Infant; Infant, Newborn; Liver Transplantation; Portoenterostomy, Hepatic; Prognosis; Treatment Outcome
PubMed: 25944310
DOI: 10.1097/MOP.0000000000000214 -
American Society of Clinical Oncology... 2016Biliary tract cancer, or cholangiocarcinoma, arises from the biliary epithelium of the small ducts in the periphery of the liver (intrahepatic) and the main ducts of the... (Review)
Review
Biliary tract cancer, or cholangiocarcinoma, arises from the biliary epithelium of the small ducts in the periphery of the liver (intrahepatic) and the main ducts of the hilum (extrahepatic), extending into the gallbladder. The incidence and epidemiology of biliary tract cancer are fluid and complex. It is shown that intrahepatic cholangiocarcinoma is on the rise in the Western world, and gallbladder cancer is on the decline. Radiation therapy has emerged as an important component of adjuvant therapy for resected disease and definitive therapy for locally advanced disease. The emerging sophisticated techniques of imaging tumors and conformal dose delivery are expanding the indications for radiotherapy in the management of bile duct tumors. As we understand more about the molecular pathways driving biliary tract cancers, targeted therapies are at the forefront of new therapeutic combinations. Understanding the gene expression profile and mutational burden in biliary tract cancer allows us to better discern the pathogenesis and identify promising new developmental therapeutic targets.
Topics: Biliary Tract Neoplasms; Cholangiocarcinoma; Gallbladder Neoplasms; Humans; Molecular Targeted Therapy; Neoplasm Proteins; Signal Transduction
PubMed: 27249723
DOI: 10.1200/EDBK_160831 -
Current Opinion in Gastroenterology Jan 2012New knowledge on rotavirus infection in children and well established mouse models has renewed interest in whether rotavirus could cause biliary atresia, an idiopathic,... (Review)
Review
PURPOSE OF REVIEW
New knowledge on rotavirus infection in children and well established mouse models has renewed interest in whether rotavirus could cause biliary atresia, an idiopathic, obliterative infantile disease of bile ducts that is the primary indication for liver transplant in children.
RECENT FINDINGS
Studies in the rotavirus mouse model of biliary atresia indicate that infection of biliary epithelium is an inaugural event leading to biliary inflammation and obstruction, which is preceded by systemic spread of rotavirus, which also occurs during human rotavirus enteric infections. Viral factors, including rotavirus gene 4, are important for biliary infection and biliary atresia in mice. Specific host factors related to inflammatory processes (natural killer and T cells, interferon) are also critical, and a paucity of regulatory T cells in neonates may play a key role in pathogenesis in experimental biliary atresia. Rotavirus vaccination has substantially decreased rotavirus diarrheal disease worldwide and might enable demonstration of a cause-effect relationship between rotavirus infection and biliary atresia in humans.
SUMMARY
Rotavirus can be detected in the serum of mice and children and causes biliary atresia in neonatal mice. Approaches to re-examine whether rotavirus causes biliary atresia in children are discussed based on concepts from the mouse model of biliary atresia and rotavirus vaccination programs.
Topics: Animals; Bile Ducts; Biliary Atresia; Global Health; Humans; Incidence; Infant; Risk Factors; Rotavirus; Rotavirus Infections
PubMed: 22123643
DOI: 10.1097/MOG.0b013e32834c7ae4 -
The Korean Journal of Gastroenterology... Jan 2024Biliary tract cancers encompass a group of malignancies that affect the bile ducts and gallbladder and are associated with a poor prognosis, often due to late diagnosis... (Review)
Review
Biliary tract cancers encompass a group of malignancies that affect the bile ducts and gallbladder and are associated with a poor prognosis, often due to late diagnosis and limited treatment options. The incidence of biliary tract cancer has been increasing gradually, underscoring the need for a better understanding of its pathogenesis and potential risk factors. Research suggests that biliary tract cancer may develop through a combination of genetic and epigenetic alterations, as well as environmental factors. The role of microbial exposure and the human microbiome in the pathogenesis of biliary tract cancer is an emerging area of interest. Traditionally, the biliary tree was considered sterile under normal conditions, but recent studies have identified associations between specific microbiological patterns and inflammatory biliary diseases and cancer. The human microbiome plays a crucial role in maintaining host homeostasis and interacting with the host's immune system. Dysbiosis, or an imbalance in the microbiome composition, has been implicated in the development of various diseases, including cancer. Hence, dysbiosis in the biliary tract might trigger the pathogenesis of biliary tract cancer. Advances in next-generation sequencing technology have provided researchers with a more comprehensive view of the microbiota and their potential roles in health and disease, providing more evidence of the relationship between the microbiota and biliary tract cancer. This review summarizes the latest evidence of the microbiome that would be associated with biliary tract cancer.
Topics: Humans; Dysbiosis; Biliary Tract Neoplasms; Biliary Tract; Gallbladder Diseases; Microbiota
PubMed: 38268162
DOI: 10.4166/kjg.2023.135 -
Frontiers in Cellular and Infection... 2021The pathogenesis of choledocholithiasis is closely related to the role of bacteria. However, little is known about the predictive role of bile bacteria in clinical...
BACKGROUND
The pathogenesis of choledocholithiasis is closely related to the role of bacteria. However, little is known about the predictive role of bile bacteria in clinical conditions of patients and the compositional and functional characteristics of biliary microbiota in choledocholithiasis.
METHODS
To investigate the predictive value of biliary bacteria, clinical data of 488 patients with choledocholithiasis were collected. The predictive value of common bile bacteria to patients' clinical conditions was analyzed by logistic regression. Samples of bile and corresponding duodenal juice from 10 selected patients with choledocholithiasis were obtained, and the composition and function of microbial communities were analyzed based on rRNA sequencing and Tax4Fun.
RESULTS
The clinical conditions of patients with choledocholithiasis, such as recurrence, the severity of acute cholangitis, and duration of hospital stay were closely related to different species of bile bacteria as well as antimicrobial-resistant bacteria. Employing rRNA sequencing, the dominant phyla of biliary and duodenal microbiota were and . The top three core microbiota at the genus level were , and . accounted for the most abundant annotated species in both. Differences in composition between biliary and duodenal microbiota were not significant according to the alpha and beta diversities. Differential abundant features were not found in biliary microbiota indicated by A linear discriminant analysis effective size algorithm. The major pathways identified in biliary and duodenal microbiota were related to membrane transport, translation, replication and repair, carbohydrate and amino acid metabolism. However, no significant difference in those major pathways, as well as antimicrobial-resistance patterns, was observed between biliary and duodenal microbiota.
CONCLUSION
Our study first demonstrates the predictive contribution of biliary bacteria to the clinical conditions of patients with choledocholithiasis, and then it offers new insights into the compositional and functional features of biliary and duodenal microbiota. Similarities between biliary and duodenal microbiota support the theory of bacterial duodenal-biliary reflux in patients with choledocholithiasis. Meanwhile, when it is impracticable to obtain a bile sample, duodenal juice may be used as an alternative for bacterial culture and susceptibility tests.
Topics: Bile; Biliary Tract; Choledocholithiasis; Humans; Microbiota; RNA, Ribosomal, 16S
PubMed: 33996618
DOI: 10.3389/fcimb.2021.625589 -
Journal of Cystic Fibrosis : Official... Nov 2017Cystic fibrosis (CF) is a multi-organ, clinically diverse disorder caused by mutations in the cystic fibrosis transmembrane conductance receptor (CFTR). Awareness of... (Review)
Review
Cystic fibrosis (CF) is a multi-organ, clinically diverse disorder caused by mutations in the cystic fibrosis transmembrane conductance receptor (CFTR). Awareness of extra-pulmonary manifestations, including gastrointestinal and hepatobiliary disturbances, is an increasingly important part of providing high-quality care to patients with CF. Furthermore, biliary disorders, including gallbladder and bile duct disease, are common complications of CF. Therefore, a thorough understanding and efficient clinical evaluation of the gallbladder and biliary tree is an important aspect of integrated care for the patient with CF in order to prevent progression of undetected pathology. This best practice article summarizes the basis for gallbladder and bile duct pathology, describes the context and clinical presentation of biliary disease, and provides recommended approaches to delivery of high-quality care for patients with CF.
Topics: Biliary Tract Diseases; Cystic Fibrosis; Humans; Patient Care Management
PubMed: 28986023
DOI: 10.1016/j.jcf.2017.07.006