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Pathologica Jun 2021Autoimmune cholestatic liver diseases are rare hepato-biliary disorders characterized by a progressive, inflammatory destruction of bile ducts. Primary biliary... (Review)
Review
Autoimmune cholestatic liver diseases are rare hepato-biliary disorders characterized by a progressive, inflammatory destruction of bile ducts. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the main autoimmune cholestatic liver diseases. Both may evolve into secondary biliary cirrhosis and its complications. Therapeutic options are limited and liver transplantation remains the only definitive treatment for PBC and PSC. Most PBC and PSC patients have a typical presentation, which does not require liver biopsy. However, in routine clinical practice, important variants or specific subgroups that benefit from liver biopsy for proper management may be observed. Herein, we provide a general overview of clinical and pathological characteristic of PBC and PSC, highlighting the most important features for routine diagnostic practice.
Topics: Autoimmune Diseases; Cholangitis, Sclerosing; Humans; Liver Cirrhosis, Biliary
PubMed: 34294935
DOI: 10.32074/1591-951X-245 -
EBioMedicine Dec 2021Biliary Atresia is a devastating pediatric cholangiopathy affecting the bile ducts of the liver. In this review, we describe recent progress in the understanding of... (Review)
Review
Biliary Atresia is a devastating pediatric cholangiopathy affecting the bile ducts of the liver. In this review, we describe recent progress in the understanding of liver development with a focus on cholangiocyte differentiation and how use of technical platforms, including rodent, zebrafish and organoid models, advances our understanding of Biliary Atresia. This is followed by a description of potential pathomechanisms, such as autoimmune responses, inflammation, disturbed apical-basal cell polarity, primary cilia dysfunction as well as beta-amyloid accumulation. Finally, we describe current and emerging diagnostic opportunities and recent translation breakthroughs for Biliary Atresia in the area of emerging therapy development, including immunomodulation and organoid-based systems for liver and bile duct repair.
Topics: Animals; Bile Ducts; Biliary Atresia; Cell Differentiation; Disease Models, Animal; Epithelial Cells; Humans; Organoids
PubMed: 34781099
DOI: 10.1016/j.ebiom.2021.103689 -
Clinics in Liver Disease Aug 2022Biliary atresia is a rare disease but remains the most common indication for pediatric liver transplantation as there are no effective medical therapies to slow... (Review)
Review
Biliary atresia is a rare disease but remains the most common indication for pediatric liver transplantation as there are no effective medical therapies to slow progression after diagnosis. Variable contribution of genetic, immune, and environmental factors contributes to disease heterogeneity among patients with biliary atresia. Developing a deeper understanding of the disease mechanism will help to develop targeted medical therapies and improve patient outcomes.
Topics: Biliary Atresia; Child; Humans; Infant; Liver Transplantation
PubMed: 35868678
DOI: 10.1016/j.cld.2022.03.001 -
Hepatology Communications Jun 2023Primary biliary cholangitis (PBC) is a chronic cholestatic autoimmune liver disease characterized by a destructive, small duct, and lymphocytic cholangitis, and marked...
Primary biliary cholangitis (PBC) is a chronic cholestatic autoimmune liver disease characterized by a destructive, small duct, and lymphocytic cholangitis, and marked by the presence of antimitochondrial antibodies. The incidence and prevalence of PBC vary widely in different regions and time periods, and although disproportionally more common among White non-Hispanic females, contemporary data show a higher prevalence in males and racial minorities than previously described. Outcomes largely depend on early recognition of the disease and prompt institution of treatment, which, in turn, are directly influenced by provider bias and socioeconomic factors. Ursodeoxycholic acid remains the initial treatment of choice for PBC, with obeticholic acid and fibrates (off-label therapy) reserved as add-on therapy for the management of inadequate responders or those with ursodeoxycholic acid intolerance. Novel and repurposed drugs are currently at different stages of clinical development not only for the treatment of PBC but also for its symptomatic management. Here, we summarize the most up-to-date data regarding the epidemiology, prognosis, and treatment of PBC, providing clinically useful information for its holistic management.
Topics: Male; Female; Humans; Ursodeoxycholic Acid; Liver Cirrhosis, Biliary; Cholangitis; Prognosis; Cholestasis
PubMed: 37267215
DOI: 10.1097/HC9.0000000000000179 -
Seminars in Interventional Radiology Aug 2021The hepatobiliary system is known to have high anatomic variability, as studies have shown variant rates of over 40% among individuals. This review will describe biliary... (Review)
Review
The hepatobiliary system is known to have high anatomic variability, as studies have shown variant rates of over 40% among individuals. This review will describe biliary anatomy and the most common anatomic variants, knowledge of which is critical to ensuring safe and effective biliary interventions.
PubMed: 34393334
DOI: 10.1055/s-0041-1731085 -
Seminars in Interventional Radiology Dec 2016Differentiating benign and malignant biliary strictures is a challenging and important clinical scenario. The typical presentation is indolent and involves elevation of... (Review)
Review
Differentiating benign and malignant biliary strictures is a challenging and important clinical scenario. The typical presentation is indolent and involves elevation of liver enzymes, constitutional symptoms, and obstructive jaundice with or without superimposed or recurrent cholangitis. While overall the most common causes of biliary strictures are malignant, including cholangiocarcinoma and pancreatic adenocarcinoma, benign strictures encompass a wide spectrum of etiologies including iatrogenic, autoimmune, infectious, inflammatory, and congenital. Imaging plays a crucial role in evaluating strictures, characterizing their extent, and providing clues to the ultimate source of biliary obstruction. While ultrasound is a good screening tool for biliary ductal dilatation, it is limited by a poor negative predictive value. Magnetic resonance cholangiopancreatography is more than 95% sensitive and specific for detecting biliary strictures with the benefit of precise anatomic localization. Other commonly employed imaging modalities include endoscopic retrograde cholangiopancreatography with endoscopic ultrasound, contrast-enhanced CT, and cholangiography. First-line treatment of benign biliary strictures is endoscopic dilation and stenting. In patients with anatomy that precludes endoscopic cannulation, percutaneous biliary drain insertion and balloon dilation is preferred.
PubMed: 27904249
DOI: 10.1055/s-0036-1592325 -
Seminars in Interventional Radiology Aug 2021Biliary endoscopy is underutilized by interventional radiologists and has the potential to become an effective adjunctive tool to help both diagnose and treat a variety... (Review)
Review
Biliary endoscopy is underutilized by interventional radiologists and has the potential to become an effective adjunctive tool to help both diagnose and treat a variety of biliary pathology. This is particularly true in cases where endoscopic retrograde cholangiopancreatography fails or is not feasible due to surgically altered anatomy. Both preoperative clinical and technical procedural factors must be taken into consideration prior to intervention. In this article, clinical evaluation, perioperative management, and procedural techniques for percutaneous biliary endoscopy are reviewed.
PubMed: 34393344
DOI: 10.1055/s-0041-1731372 -
Journal of Clinical Medicine Apr 2023Endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic biliary drainage (PTBD) are currently first- and second-line therapeutic options,... (Review)
Review
Endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic biliary drainage (PTBD) are currently first- and second-line therapeutic options, respectively, for the relief of biliary obstruction. In recent years, however, endoscopic ultrasound-guided biliary drainage (EUS-BD) has become an established alternative therapy for biliary obstruction. There are multiple different techniques for EUS-BD, which can be distinguished based on the access point within the biliary tree (intrahepatic versus extrahepatic) and the location of stent placement (transenteric versus transpapillary). The clinical and technical success rates of biliary drainage for EUS-BD are similar to both ERCP and PTBD, and complication rates are favorable for EUS-BD relative to PTBD. As EUS-BD becomes more widely practiced and endoscopic tools continue to advance, the outcomes will likely improve, and the breadth of indications for EUS-BD will continue to expand.
PubMed: 37048819
DOI: 10.3390/jcm12072736 -
Seminars in Interventional Radiology Aug 2021Percutaneous biliary and gallbladder drainage is routinely performed by interventional radiology. These pathologies and techniques are well described in the literature... (Review)
Review
Percutaneous biliary and gallbladder drainage is routinely performed by interventional radiology. These pathologies and techniques are well described in the literature and in this issue. The purpose of this review is to focus on the recognition and management of complications. Percutaneous biliary drains can have a variety of complications which can range from minor skin issues to more serious and complex bleeding issues. Advancements in imaging and techniques improve the safety profile of percutaneous biliary and gallbladder interventions.
PubMed: 34393347
DOI: 10.1055/s-0041-1731375 -
World Journal of Gastroenterology Sep 2016Hepatobiliary and pancreatic ascariasis (HPA) was described as a clinical entity from Kashmir, India in 1985. HPA is caused by invasion and migration of nematode,... (Review)
Review
Hepatobiliary and pancreatic ascariasis (HPA) was described as a clinical entity from Kashmir, India in 1985. HPA is caused by invasion and migration of nematode, Ascaris lumbricoides, in to the biliary tract and pancreatic duct. Patients present with biliary colic, cholangitis, cholecystitis, hepatic abscesses and acute pancreatitis. Ascarides traverse the ducts repeatedly, get trapped and die, leading to formation of hepatolithiasis. HPA is ubiquitous in endemic regions and in Kashmir, one such region, HPA is the etiological factor for 36.7%, 23%, 14.5% and 12.5% of all biliary diseases, acute pancreatitis, liver abscesses and biliary lithiasis respectively. Ultrasonography is an excellent diagnostic tool in visualizing worms in gut lumen and ductal system. The rational treatment for HPA is to give appropriate treatment for clinical syndromes along with effective anthelmintic therapy. Endotherapy in HPA is indicated if patients continue to have symptoms on medical therapy or when worms do not move out of ductal lumen by 3 wk or die within the ducts. The worms can be removed from the ductal system in most of the patients and such patients get regression of symptoms of hepatobiliary and pancreatic disease.
Topics: Algorithms; Animals; Ascariasis; Ascaris lumbricoides; Biliary Tract; Biliary Tract Diseases; Cholangitis; Cholecystitis; Humans; India; Liver Diseases, Parasitic; Pancreatitis; Prevalence
PubMed: 27672273
DOI: 10.3748/wjg.v22.i33.7507